Helen: The Journal of Human Exceptionality - August 2022

ISSUE NO. 3 AUGUST 2022

The

LANGUAGE of

Disability

THE

5

ESSENTIAL CONCEPTS

THE BEGINNING OF

Choosing Kindness

By Dina Zuckerberg

of Developmental Medicine

Prader-Willi Syndrome Causes of Excessive Daytime Sleepiness

ENDORSED BY

helenjournal.org • HELEN | 1

Celebrating the ADA Challenging Health Care Inequities For 38 years, as one of the smallest federal agencies in all of government, NCD has people with disabilities across the United States and in our territories. The most on the need for robust civil rights protections for people with disabilities and we authored the initial draft of the Americans with Disabilities Act (ADA).

On July 26, 1990, President George H.W. Bush signed the ADA into law, recognizing the decades of struggle for Americans with disabilities to achieve equality in their daily lives. The landscape of the country changed for the better as a result.

Now, 32 years after the ADA, NCD is urgently calling on Congress and the Administration to act once again – this time, to address the longstanding health disparities and healthcare bias that people with disabilities continue to experience, and at times, at the cost of their lives.

Doctors can’t better treat people with whom they don’t have experience. People can’t get better care if they cannot get on an examination table. We cannot expect paint the fullest picture of the need if we’re not being counted.

Comprehensive disability clinical care curriculum and training; adoption of enforceable accessible medical and diagnostic equipment standards; designation of people with disabilities as medically underserved coupled with their designation as a health disparities population; and better federal disability health care data are four pillars that will help us turn the tide.

Andrés J. Gallegos Chairman, National Council on Disability

2 | HELEN • helenjournal.org

Contents 42

40

Pain, Individuals with IDD and Philadelphia Coordinated Health Care

Sleep and Prader-Willi Syndrome

By Vincent Siasoco, MD, MBA

A genetic disorder that affects multiple organ systems in the body and causes of excessive daytime sleepiness

Identifying pain as an issue or cause cannot always be conveyed by individuals with IDD, increasing the risk of diagnostic overshadowing.

By Althea A. Shelton, MD, MPH

16

The Five Essential Concepts of Developmental Medicine

By Philip May M.D., Henry Hood DMD, FADD, Matthew Holder MD, MBA, Rick Rader MD, FAAIDD, FAADM

A Medical Paradigm for Persons with Developmental Disabilities helenjournal.org • HELEN | 3

Contents Volume 1, Issue 3

34 HELEN is proud to announce that the American Association on Health and Disability (AAHD) has become an official collaborative partner along with AADMD, AADM and AADD.

24

The American Association on Health and Disability is a cross-disability national non-profit 501(c)(3) organization dedicated to ensuring health equity for children and adults with disabilities through policy, research, education and dissemination.

AAHD is the only national organization specifically dedicated to integrating public health and disability into the overall public health agenda.

COLUMNS

FEATURES

6 Nothing is Written

12 What Can Be Done to Reduce

Going Down in History

By Rick Rader, MD, FAAIDD, FAADM

11 Reflections

Helen Keller’s Splendor of“Hearing” Beethoven’s Ninth Symphony

30 AADMD

The Impact of Ableism on People with Disabilities

By H. Barry Waldman, DDS, MPH, PhD Rick Rader, MD, FAAIDD, FAADM Steven P. Perlman, DDS, MScD, DHL (Hon) Allen Wong, DDS, EdD, DABSCD

the Risk of Abuse?

By Nora J. Baladerian, PhD

24 The Language of Disability

By H. Barry Waldman, DDS, MPH, PhD David A. Ervin, BSc, MA, FAAIDD Steven P. Perlman, DDS, MScD, DHL (Hon) Rick Rader, MD, FAAIDD, FAADM Allen Wong, DDS, EdD, DABSCD

34 How and Why I Recently

Visited Ukraine - Part Two

By Seth Keller, MD

46 Ensuring Equitable Healthcare

The Beginning of Choosing Kindness

for People with IDD Is NOT the “Medical Model”

By Dina Zuckerberg

By Craig Escudé, MD, FAAFP, FAADMD

44 Journeys

ON THE COVER: Dina Zuckerberg and friend.

Photo by Rick Guidotti - positiveexposure.org 4 | HELEN • helenjournal.org

HelenJournal.org Helen: The Journal of Human Exceptionality is where people with disabilities, families, clinicians, and caregivers intersect for inclusive health.

Editor in Chief Senior Managing Editor Senior Editor for Special Projects Publisher Art Director American Academy of Developmental Medicine & Dentistry 3 Forester Avenue, #22 Warwick, NY 10990

Rick Rader, MD, FAAIDD, FAADM Vanessa B. Ira Steve Perlman, DDS, MScD, DHL(Hon) Allen Wong, DDS, EdD, DABSCD Alyse Knudsen All rights reserved. Copyright © 2022 American Academy of Developmental Medicine & Dentistry

Editorial Advisory Board Marc Bernard Ackerman, DMD, MBA, FACD Director of Orthodontics, Boston Children’s Hospital; Adjunct Assistant Professor, University of Pennsylvania School of Dental Medicine, PA Roberta Carlin, MA, MS, JD Executive Director, American Association on Health and Disability, Rockville, MD Renee Dease Specialist & Coordinator, Healthy Athletes Health Programs at Special Olympics International Steven M. Eidelman H. Rodney Sharp Professor of Human Services Policy & Leadership; Faculty Director, The National Leadership Consortium on Developmental Disabilities, University of Delaware David Ervin, BSc, MA, FAAIDD Chief Executive Officer, MAKOM, Rockville, MD David Fray, DDS, MBA Professor, Department of General Practice and Dental Public Health, University of Texas School of Dentistry, Houston, TX Rick Guidotti Founder and Director of Positive Exposure, New York, NY Matthew Holder, MD, MBA, FAADM CEO, Kramer Davis Health; Co-founder, AADMD; Co-founder, ABDM; Co-founder, Lee Specialty Clinic

Emily Johnson, MD Medical Director, Developmental Disabilities Health Center, Peak Vista Community Health Centers, Colorado Springs, CO Seth Keller, MD Neurologist, Co-president, National Task Group on Intellectual Disabilities and Dementia Practices Gary Liguori, PhD, FACSM Dean, College of Health Sciences, URI Academic Health Collaborative, Kingston, RI Joseph M. Macbeth President/Chief Executive Officer, National Alliance for Direct Support Professionals, Inc. Susan L. Parish, PhD, MSW Dean and Professor, College of Health Professions, Virginia Commonwealth University Richmond, VA Hoangmai H. Pham, MD, MPH President and CEO, Institute for Exceptional Care Hon. Neil Romano Former Assistant Secretary, United States, Department of Labor, Office of Disability Employment Policy, Chairman Emeritus, National Council on Disability, President, TRG-16 Vincent Siasoco, MD, MBA Assistant Professor, Dept. of Family Med | Pediatrics, Albert Einstein College of Medicine, Director of Primary Care, Rose F. Kennedy CERC, Montefiore, Med. Director, ADAPT Community Network, New York, NY

JoAnn Simons, MSW Chief Executive Officer, The Northeast Arc, Danvers, MA Michael Stein Co-founder & Executive Director, Harvard Law School Project on Disability, Cambridge, MA Justin Steinberg Special Olympics Northern California Athlete Member, AADMD Board of Directors Stephen B. Sulkes, MD Pediatrician, University of Rochester Medical Center, Rochester, NY Carl Tyler, MD Gerontologist, Research Director, Lakewood Family Health Center, Cleveland Clinic, OH Barbie Vartanian Parent Advocate; Director, Oral Health Advocacy & Policy Initiatives NYU Executive Committee, Project Accessible Oral Health H. Barry Waldman, DDS State University of New York Distinguished Teaching Professor, School of Dental Medicine, Stony Brook University, NY Dina Zuckerberg Director of Family Programs, MyFace, New York, NY

Helen: The Journal of Human Exceptionality neither endorses nor guarantees any of the products or services mentioned in the publication.

We strongly recommend that readers thoroughly investigate the companies and products or services being considered for purchase, and, where appropriate, we encourage them to consult a physician or other credentialed health professional before use and purchase.

helenjournal.org • HELEN | 5

NOTHING IS WRITTEN - EDITOR’S DESK Rick Rader, MD, FAAIDD, FAADM - Editor in Chief

1,500-year-old bones believed to be the oldest confirmed case of Down syndrome in the world.

Photo credit: Rivollat et al./Elsevier

W

hen I was a medical student in London, my genetics professor held classes in the London Underground. After all it was the epicenter of “The Commonwealth.”

The Commonwealth of Nations (the former name) was a 54-member humanitarian coalition of countries under the British Empire. Included in this portfolio was Canada, Australia, New Zealand, Tonga, Fiji, Western Samoa, India, Burma, Papua New Guinea, Malay, Sarawak, Brunei, Oman, Iraq, Egypt, Libya, Sudan, Kenya, Uganda, Northern and Southern Rhodesia, Tanganyika, Zanzibar, Mauritius, the Maldives, South Africa, Nigeria, Gold Coast, and Sierra Leone, among other countries during its reign.

London had an abundance of both residents and visitors from each of these countries. The London Underground (subway) was a genetics supermarket. Our professor would take us into “the Tube” to observe those individuals that had recognizable facial features reflective of a variety of genetic disorders.

6 | HELEN • helenjournal.org

Going DOWN in History

It would play out this way, with him saying, “Mr. Rader, third person on the left sitting next to the OAP carrying the tote from Harrod’s.” I quickly learned that OAP referred to an Old Age Pensioner.

Figuring it was better to offer something even if it was incorrect than to sit dumbfounded, I offered, “Might it be Noonan syndrome, Professor?” It was another case of me crashing and burning as we pulled into the Edgware Road station. My professor reminded me that the characteristic facial features of Noonan syndrome included a deep groove between the nose and the mouth, as well as widely-spaced eyes that are often a pale blue or blue-green color. Other unique features included low-set ears that may be positioned backward, a high arch in the mouth, smaller lower jaw, and excess neck skin and webbing.

My teacher was a walking encyclopedia and seemed to have a handful of neurons dedicated to cataloging most of the 7,000 known genetic disorders.

“I can see where you might have made that mistake, Mr. Rader, but you

should at least have included Ablepharon-Macrostomia syndrome in your short list.” “Yes Professor,” I thought to myself, that was going to be my next guess.

Every night during the three-week rotation I studied photos and took notes on their descriptions from “the Bible.”

Smith’s Recognizable Patterns of Human Malformation has long been known as the source to consult on multiple malformation syndromes of environmental and genetic etiology, as well as recognizable disorders of unknown cause. In short order I had more yellow highlights than not. I had bookmarks on both microdeletions and microduplication syndromes. I consistently confused Lenz Microphthalmmia syndrome for Mowart-Wilson syndrome (“How could I?”).

Finally, it dawned on me that even the distinguished Kaufman-McKusick syndrome looked more and more like Wiedeman Rautenstrauch syndrome (so much for my future in genetics).

There was never a trip to London’s Underground Genetics Tour where we

didn’t encounter an individual with Down syndrome. They were so recognizable that the Professor never asked us to identify the condition.

Down syndrome was first described by an English physician, John Langdon Down, in 1862. He used the term “mongoloid” to describe the condition, due to his opinion that children with Down’s syndrome shared similar physical features to people from the Blumenbach’s Mongolian race.

Down went on to describe his observations, “A very large number of congenital idiots are typical Mongols.

So marked is this that, when placed side by side, it is difficult to believe the specimens compared are not children of the same parents. The number of idiots who arrange themselves around the Mongolian type is so great, and they present such a close resemblance to one another in mental power, I shall describe an idiot member of this racial division, selected from the large number that have fallen under my observation.”

A case study published in the International Journal of Paleopathology showed photos of a skeleton buried near a church in Charlon-sur-Saone in eastern France. The remains feature a broad skull with flattened base and thinner skull bones, all telltale signs of Down syndrome.

It is believed to be the oldest confirmed case of Down syndrome in the world. The 1,500-year-old skeleton serves to not only reference the longstanding existence

of the disorder, but perhaps more important, how ancient civilizations viewed those with genetic disorders.

Lead researcher of the study, Maite Rivollat, commented that based on conditions related to its burial, it did not appear to be stigmatized by the community. “This Down syndrome child was not treated differently at death than others in the community.

We interpret this as meaning that the child was maybe not stigmatized during life, the first- time a Down syndrome individual has been so viewed in the context of the ancient community.” This observation is perhaps the most significant one related to the findings of the skeleton.

While my genetics rotation in the London Underground exposed me to a vast repertoire of human exceptionalities, we did not discuss how society embraced, responded to, or included them.

My own observation based on the body language of fellow passengers was they were keeping their distance, keeping their heads in newspapers, viewing their fingernails, and perhaps getting off the train before their preferred stop.

And while it was not the intention of the Underground Genetics Class, I learned more about human indignation, stigmatization and indifference than I learned about linkage disequilibrium mapping. I did not like what I learned.

There’s a lot to be learned from history and if the findings of the 1,500-year-old skeleton with Down syndrome accurately reflects a promise in the human capacity for tolerance, acceptance and inclusion than we need to “go Down in history.”

WHY HELEN?

Alone we can do so little; together we can do so much.

- Helen Keller HELEN: The Journal of Human Exceptionality pays tribute to Helen Keller. Ms. Keller is perhaps the most iconic disability rights advocate and an example of how an individual with complex disabilities found and used her stamina, perseverance, resilience and determination to accomplish great things.

Helen personifies the spirit, mission and vision of The Journal of Human Exceptionality.

Her remarkable life is a reflection of her determination; she inspired us with her words, “What I’m looking for is not out there, it is in me.”

“Nothing is written.”

This quote from T.E. Lawrence implies that nothing is inevitable, life consists of choices, and how the individual can make an impact on his/her destiny. The disability community continues to reinforce and remind me of this; hence the name for my monthly musings.

- Dr. Rick Rader helenjournal.org • HELEN | 7

Collaborative Organization Mission Statements Helen: The Journal of Exceptionality is proud to be endorsed by the nation’s leading organizations that advocate for people with Intellectual and Developmental Disabilities (IDD).

The AADMD is resolved: To assist in reforming the current system of healthcare so that no person with IDD is left without access to quality health services.

To prepare clinicians to face the unique challenges in caring for people with IDD. To provide curriculum to newly established IDD training programs in professional schools across the nation.

To increase the body and quality of patient-centered research regarding those with IDD and to involve parents and caregivers in this process.

It is the purpose of the American Academy of Developmental Dentistry (AADD) to establish postdoctoral curriculum standards for training dental clinicians in the care of patients with IDD, to establish clinical and didactic training materials and programs to promulgate these standards, and through its certifying entity – the American Board of Developmental Dentistry – to grant board-certification to those dentists who have successfully completed these training programs.

8 | HELEN • helenjournal.org

To create a forum in which healthcare professionals, families and caregivers may exchange experiences and ideas with regard to caring for patients with IDD. To disseminate specialized information to families in language that is easy to understand.

To establish alliances between visionary advocacy and healthcare organizations for the primary purpose of achieving better healthcare.

The American Academy of Developmental Medicine (AADM) is a medical society dedicated to addressing the complex medical needs of individuals with intellectual and developmental disabilities across the lifespan.

It incorporates clinician training and awareness, teaching, advocacy, research, board certification, health equity, interdisciplinary collaboration, inclusive care delivery models and shared decision making.

AAHD is dedicated to ensuring health equity for children and adults with disabilities through policy, research, education and dissemination at the federal, state and community level.

AAHD strives to advance health promotion and wellness initiatives for people with disabilities.

AAHD’s goal are to reduce health disparities between people with disabilities and the general population, and to support full community inclusion and accessibility.

Reducing barriers.

Improving access.

Driving change.

Together.

Delta Dental of Michigan, Ohio, and Indiana is the first dental benefits administrator in the nation to offer a Special Health Care Needs Benefit, which removes multiple barriers to oral health care for people with intellectual or developmental disabilities by covering additional dental visits, treatment delivery modifications and more.

Delta Dental and the Delta Dental Foundation are proud to support the American Academy of Developmental Medicine & Dentistry and congratulate them on their inaugural publication.

Together we champion people with disabilities.

Delta Dental of Michigan, Ohio, and Indiana www.vibrantcommunities.com www.deltadental.foundation

Delta Dental is a registered trademark of Delta Dental Plans Association. www.deltadental.com helenjournal.org • HELEN | 9

The future of wellness for people with IDD.

Terrence Davis

• Medical • Dental

• Behavioral

• Therapies • Prevention

Kramer Davis is proud to sponsor this inaugural edition of Helen.

10 | HELEN • helenjournal.org

Compassionate, interdisciplinary health care for people with IDD.

REFLECTIONS

The Splendor of “Hearing” Beethoven Helen Keller wrote the following letter to the New York Symphony Orchestra in March 1924 after hearing Beethoven’s “Ninth Symphony” over the radio.

Dear Friends: I have the joy of being able to tell you that, though deaf and blind, I spent a glorious hour last night listening over the radio to Beethoven’s “Ninth Symphony.” I do not mean to say that I “heard” the music in the sense that other people heard it; and I do not know whether I can make you understand how it was possible for me to derive pleasure from the symphony. It was a great surprise to myself. I had been reading in my magazine for the blind of the happiness that the radio was bringing to the sightless everywhere. I was delighted to know that the blind had gained a new source of enjoyment; but I did not dream that I could have any part in their joy. Last night, when the family was listening to your wonderful rendering of the immortal symphony someone suggested that I put my hand on the receiver and see if I could get any of the vibrations. He unscrewed the cap, and I lightly touched the sensitive diaphragm. What was my amazement to discover that I could feel, not only the vibration, but also the impassioned rhythm, the throb and the urge of the music! The intertwined and intermingling vibrations from different instruments enchanted me. I could actually distinguish the cornets, the roil of the drums, deep-toned violas and violins singing in exquisite unison. How the lovely speech of the violins flowed and plowed over the deepest tones of the other instruments! When the human voices leaped up thrilling from the surge of harmony, I recognized them instantly as voices more ecstatic, upcurving swift and flame-like, until my heart almost stood still. The women’s voices seemed an embodiment of all the angelic voices rushing in a harmonious flood of beautiful and inspiring sound. The great chorus throbbed against my fingers with poignant pause and flow. Then all the instruments and voices together burst forth – an ocean of heavenly vibration – and died away like winds when the atom is spent, ending in a delicate shower of sweet notes.

Of course this was not “hearing,” but I do know that the tones and harmonies conveyed to me moods of great beauty and majesty. I also sense, or thought I did, the tender sounds of nature that sing into my hand-swaying reeds and winds and the murmur of streams. I have never been so enraptured before by a multitude of tone-vibrations.

As I listened, with darkness and melody, shadow and sound filling all the room, I could not help remembering that the great composer who poured forth such a flood of sweetness into the world was deaf like myself. I marveled at the power of his quenchless spirit by which out of his pain he wrought such joy for others – and there I sat, feeling with my hand the magnificent symphony which broke like a sea upon the silent shores of his soul and mine.

Courtesy: The Auricle, Vol. II, No. 6, March 1924. American Foundation for the Blind, Helen Keller Archives. helenjournal.org • HELEN | 11

P

eople with disabilities, both as children and adults, are abused at rates higher than the generic population. However, it seems that this information is not well-distributed either among professionals serving them or among parent groups, in general. I have found that talking about abuse is terrifying for parents. It is not that they do not want to know about it or acknowledge it, it is that they want it not to happen to their children.

This brief article will focus on what can be done to reduce the risk of abuse happening to one’s child (minor or adult.) It is also important to address how to heal from abuse when it occurs. I believe that “knowledge is power,” and have worked for many years to educate parents of children and adults with disabilities, not only to the risks of abuse in general, but on what can be done to reduce the risk of abuse for their child.

Many programs across the country have as their motto, “End Abuse” or “No More Abuse,” and other phrases that I believe are more aspirational than practical. Thus, several years ago, I decided that rather than aim for the total elimination of abuse on the planet, I would endeavor to reduce the risk of abuse happening to individuals, then, if and when it does happen, have a better outcome. I have taught many parents and caregiving agencies this approach and have had positive feedback when abuse and abuse attempts have later occurred.

There are a few mottos that I have embraced in this approach, such as “Knowledge is Power” among others. The Girl Scout’s “Be Prepared!” is useful. I would like to share the foundational thinking and then provide some examples of how this can be effective in reducing the risk of abuse 12 | HELEN • helenjournal.org

Reduce

Overcoming fear and inertia to cr

in those most a children and adults with intellectual a

and improving healing when it does occur.

“Zoe” I like to use real-life stories to illustrate these concepts. The first is that of a nine-year-old girl who had been sexually abused over several years by close family members. She had been threatened by them not to tell her parents, and thus had kept her silence. However, both she (“Zoe”) and her sister (“Maria”), one day told their parents about the abuse. The mother and father were devastated, but primarily the mother, as these same family members had abused her when she was a child. She knew instantly

that her children were telling them the truth. Now, not only did the mother feel bad for her children, but guilty for somehow not protecting them. She had believed that they would never do that to her children, not knowing that in general, perpetrators never stop; they just find more victims. (In telling this story at training programs, some have “blamed the mother” for not knowing that abusers never stop, but I do not blame her, as such information is not normally provided in general magazines or articles on trauma). Trauma therapy was provided for the children.

For the mother, grief and trauma therapy was provided along with treatment for guilt (called, “irrational guilt” in

WHAT CAN BE DONE TO

the Risk of Abuse?

By Nora J. Baladerian, PhD

reate resiliency

and empowerment

at risk of sexual abuse victimization:

and developmental disabilities and other disabilities. such cases), to heal from this unbelievable travesty. I knew the children were feeling increasingly better. One day, Zoe had drawn a picture of her perpetrators in jail, then tore it into tiny pieces, then went to the restroom, flushed it down the toilet, and skipped joyfully back to the office! We did not schedule further appointments. I was so pleased for her and went home feeling good…until a thought hit me: “What about next time?” It was this question that caused me to design a risk-reduction program called: How to reduce the risk and impact of abuse. This approach is founded on how to respond to any “disaster” such as weather issues (storms, flooding,

snow, etc.)

I designed a plan structure and had Zoe and her family return to design a specific plan for Zoe (and her sister) to evade or better survive another sexual assault. Everyone in the family had a role. The plan framework is simple: • What should the family members learn before the next assault (attempt)?

• What should a sexual assault victim do during an assault?

• What should one do after an assault?

I called several rape treatment centers to find out their recommendations for how one would face an assault, but none had an answer for me. As a team,

we designed a plan for these three stages of an assault (before, during, after). These were to be reviewed, and practiced, using non-traumatic activities to build and strengthen Zoe’s communication and emergency response skills.

We also designed a plan for the parents: What should they be doing to support Zoe in “story-telling” skills, for example, telling/communicating the story of going to the store to buy bread. We detailed the parent’s role in responding to a report by Zoe. These skills were to be practiced over time, thus also increasing Zoe’s communication skills with the family. Each family member also had to make a plan, to be able to understand any report of abuse.

It was about 6 years later that I received a call from Zoe’s mother. My heart sank when I heard her voice, as I feared that Zoe had again been assaulted. The mother told me that Zoe had very recently been sexually assaulted by the school bus driver (a new driver). She said Zoe was so excited at “how well she did” and wanted me to know what a heroine she was! The mother reported that it was the driver’s first day on the job, and he had changed the route so Zoe would be dropped off last. Zoe implemented her “during” skills during the assault, and upon getting home implemented her “after” skills. “During” skills were to become a “human video recorder” to be able to later report what happened.

“After” skills were to immediately tell her mother what had happened, then prepare to go to the hospital for a forensic examination and interview with the police, among other post-assault recommendations to preserve evidence. The mother implemented her plan as well by calling the school, then the police, then taking Zoe to the hospital for treatment and forensic examination and evidence collection. helenjournal.org • HELEN | 13

I asked if Zoe and the family would he was angry and embarrassed. We he responded, “OK…and I’m going to like to come in for some trauma completed his trauma treatment and tell my mom.” He finally gave up and therapy. The mother laughed and said, he no longer felt bad about what had Daniel returned home, where he told “NO! Zoe is so proud of herself for happened to him, but, I thought, “what his aunt about this “creepy guy” and masterfully managing all of the steps about next time?” how he had evaded going with him. of her plan (before, during, after) that Those who work and live with indiThe aunt called the camp director to she does not need to come in! She is viduals with ID know that for the most report this. She later called me to let calling everyone to tell them me know that Daniel was how well she did in this territhe only child not molested ble situation. She wanted me by this “creepy guy.” Daniel to call you to let you know.” felt so proud of himself and It was particularly of note so empowered for having that because Zoe had immebeen able to successfully As a team, we designed a diately communicated to her manage a difficult situation. plan for these three stages mother what had occurred, and the mother, implementing her Since that time, I have of an assault (before, during, part in the plan, immediately assisted many families in deafter). These were to be recalled the school. The school signing risk reduction plans, immediately had called the poviewed, and practiced, using employing the communicalice, who (fortunately) arrived tion skills of the individual, non-traumatic activities to at the school before the bus and supporting the parents driver returned. Thus, evidence knowing that such things build and strengthen Zoe’s in was present and available for can and do happen, and they communication and collection in the bus and from can benefit from designing, the perpetrator. He was immeand practicemergency response skills. implementing, diately arrested and placed in ing a plan to survive and jail. This contributed greatly to survive well. In Zoe’s case, Zoe’s well-being even though she not only survived but she had been assaulted. felt empowered and wanted This taught me the importance and part, they are not “allowed” to say “everyone” to know what a great job value of having a plan for a disaster. I “No” to an adult or person in charge she had done, as well as Daniel! learned that the value of an individuof them (temporarily with changing I do not want readers to become alized approach is effective to address shifts, etc.). Perpetrators know this frightened but rather empowered to be communication and other issues that too, so we designed an intervention able to create a plan for their children may have interfered with a quick disin which he could say “Yes” to any (minors and adults) to know how to closure/reporting of abuse. request, even if it felt bad, following it respond to attempts to abuse and to with, “and I’m going to tell my Mom.” report this to their parents or other per“Daniel” We role-played with me asking him son who may be supporting them. I am Another teenager I worked with to give me money, go for a walk with in the process of re-editing my book (we’ll call him “Daniel,”) has Down me, etc., with his answer being “OK, called “The “Risk Reduction Guide syndrome and a mild intellectual disand I’m going to tell my Mom.” Book”© which provides additional ideas and plan guides for a variety of ability, and is verbal. He had also been Several months later, the aunt living and work situations. a victim of sexual assault and was called and told me the following.

After a recent conference in which I brought to the sessions by his aunt, as Daniel had gone to summer camp, provided training on designing a risk his mother and father were deceased. and upon returning told her that there reduction plan, the conference direcHe was only 13 or 14 and was known was a “creepy guy” who had many tor contacted me to tell me that he for being one who “does what he is times asked Daniel to go with him to had just received a call from a mother told” without much resistance, as are see something “really cool!” Daniel who had attended the program. Rather many young persons with intellectual followed his feeling, and said to him, disabilities (ID). However, we did talk “OK, and I’m going to tell my mom!” than focusing on abuse, she used the about his earlier assault how powerThe man said, “No, this is our secret… plan to address how to respond to her son’s seizures. She designed her plan less he had felt at first and how later you don’t have to tell her.” To which

“

14 | HELEN • helenjournal.org

and trained her son’s staff Nora J. Baladerian, PhD is a licensed psychologist how to implement the plan. and licensed marriage and family therapist (MFT) Only a few weeks later, he in Los Angeles, CA. She has worked with individagain had a significant seiuals with intellectual/developmental disabilities zure episode and the staff since 1974, beginning as a case manager at Harbor implemented their new Regional Center in Torrance, CA. She attended Anplan. She later reported tioch University to earn her PhD She augmented her to the conference director education by taking courses in human sexuality and that the first responders qualified as an AASECT sex educator, and provides told her that had her staff sex education programs to individuals with I/DD and not been so well trained, their families. She is bilingual (Spanish) and produces her son may not have presentations and translates most of her books. survived this episode.

I was so pleased that With funding from the US Dept of Justice, Office for she applied what she Victims of Crime in collaboration with the Arc of Riverhad learned to a comside County, she produced two law enforcement training Nora J. Balad erian, PhD pletely different but essential probDVD’s one on how to interview people with disabilities lem, and had great success, and by overcoming fear and and one on how to conduct an effective and sensitive first dread of a terrible problem successfully helped her son. response. In addition, she hosted ten national conferences on this topic. In her private practice she specializes in supporting crime victims with disabilities as well as the general population treating trauma victims primarily.

A Passion for Supporting the IDD Community

and Proud Supporter of HELEN: The Journal of Human Exceptionality Almost 30 years ago, Eunice Kennedy Shriver asked Dr. Steve Perlman to create a health program for the Special Olympics. “Healthy Athletes” is now the largest public health program in the world for children and adults with Intellectual/Developmental Disabilities.

HELEN: The Journal of Human Exceptionality is the next step to helping provide healthcare professionals, caregivers, families, and advocates with resources and education to better support this under-served, marginalized and invisible population.

Steve Perlman, DDS, MScD, DHL (Hon.) has extensive experience through his private practice and his role as Clinical Professor, Boston University School of Dental Medicine; has an Academic Appointment at the University of Pennsylvania School of Dental Medicine; is Co-founder and Past President of the AADMD. helenjournal.org • HELEN | 15

THE

FIVE

ESSENTIAL CONCEPTS

of Developmental Medicine:

A Medical Paradigm for Persons with Developmental Disabilities

By Philip B. May M.D. Henry Hood DMD, FADD Matthew Holder MD, MBA Rick Rader MD, FAAIDD, FAADM

Shortly after the landmark Surgeon General’s Report (2002), “Closing the Gap: A National Blueprint to Improve the Health of Persons with Mental Retardation,” the American Academy of Developmental Medicine and Dentistry (AADMD) was established. The Academy elected Dr. Phil May as its first president. Dr. May is both an internist and endocrinologist and has spent his career treating, researching, teaching and advocating for individuals with intellectual and developmental disabilities. Dr. May has mentored many clinicians who have become leaders in the field of developmental medicine. In 2006, Dr. May published “The Five Essential Concepts of Developmental Medicine.” It served as the framework for understanding the complex network of neurodevelopmental disabilities and disorders. HELEN Journal is pleased to provide this classic doctrine as a primer for the current crop of young clinicians and students coming up through the ranks as it is as relevant today as it was 16 years ago.

INTRODUCTION Any clinician working with any given patient population must have available to them a biomedically-based conceptual framework that provides a theoretical basis for the clinical diagnosis and treatment of that patient population. This is especially true for those clinicians who treat what is perhaps the most clinically challenging patient population imaginable –patients with neurodevelopmental disorders.

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The Five Essential Concepts provide the clinician with just such a conceptual framework. They teach developmental physicians, developmental dentists, and other healthcare professionals how to think about, how to talk about, and, most important, how to care for patients with neurodevelopmental disorders in an organized fashion.

This article was updated from its original version with the permission of its author, Philip B. May, MD

T

he major functions of the brain include memory and learning, control of motor function, control and routing of sensory input, and emotional regulation/control. These functions require that specific interrelated brain structures work together in a coordinated fashion. When these relationships are improperly developed or disrupted, brain dysfunction will occur.

Regardless of the cause of the brain dysfunction or age of onset, the primary categories of “symptoms” of brain dysfunction in either a child or an adult include: 1) cognitive dysfunction 2) neuromotor dysfunction 3) seizures 4) impulsive destructive behaviors and 5) sensory processing disorders. Any of these five categories of symptoms

can be mild, moderate, or severe, and can occur either singly or in various combinations.

In addition, the clinical presentations of these five categories vary from person to person. For example, cognitive dysfunction may present as mild dyslexia or severe mental retardation, neuromotor dysfunction may present as mild spastic hemiplegia or severe choreoathetosis, seizures may present as brief “absence” spells or severe generalized tonic-clonic convulsions, and behavior problems may present as mild impulsivity or severe bizarre self-injurious episodes.

By convention, we also call these four primary symptom complexes the “complications” of “neurodevelopmental disorders”.

Brain dysfunction that first becomes apparent in childhood (Childhood Onset Brain Dysfunction or C.O.B.D.) can be caused by a large variety of genetic and acquired conditions which

are known as “neurodevelopmental disorders” – see below for classification (by convention, we refer to brain conditions – either genetic or acquired – that manifest during the time period between initial embryological brain formation until brain development is complete at approximately age 21 years, as “neurodevelopmental disorders.” Neurodevelopmental disorders are conditions (genetic or acquired) which prevent and/or disrupt normal development of brain function and/ or structure: they are associated with measurable chemical or structural abnormalities which lead to various clinical symptoms, including cognitive impairment, seizures, neuromotor dysfunction and abnormal behaviors. They are often (but not always) associated with substantial functional limitations in the activities of daily living—a.k.a. disability).

We now know that certain genes that control complex processes are required for normal brain anatomy and function. Brain dysfunction will occur if any one of these genes is absent or does not function properly in the brain of the developing embryo.

Gene abnormalities may occur, either because of a spontaneous mutation in the earliest stages of embryological development, or as the result of inheritance from the mother or father.

In addition to genetic etiologies, brain dysfunction may also result from a multitude of adverse environmental influences during the prenatal, perinatal or postnatal periods. helenjournal.org • HELEN | 17

At times, the precise etiology of through the State Medicaid program. any adult individual with a neurodeChildhood Onset Brain Dysfunction Benefits vary from state to state. In velopmental disorder, depending on (COBD) cannot be determined. In the precise etiology of the neurodevel- general, the “diagnosis” of “develthese cases of COBD of unknown opmental disability” requires that the opmental disorder, may also demonetiology (i.e., the precise neurodevelstrate, “syndrome-specific” conditions. onset of the condition occurs before opmental disorder is not known), it is age 18 years, is chronic, will require For example, if the specific etiology important to continue to search for the of the neurodevelopmental disorder lifelong services, and is associated exact cause for the reasons discussed with substantial functional limitations is Down syndrome, in addition to the below. in most areas of daily living. To detersymptoms of brain dysfunction, the Some children with these early person with Down syndrome may also mine the diagnosis of developmental genetic and/or acquired brain disability, a psychologist deficits will be stillborn. performs two different Others will die in childhood. tests. The first is a test of However, depending on the intellectual or cognitive nature and severity of the function (e.g. the StanChildren who survive early neurodevelopmental disorford-Binet IQ test) and problems and become adults der that is responsible for the the second is a test of COBD, many will survive into will likely demonstrate one or functional limitation (e.g. adulthood and more and more the Vineland test). While more of the five primary appear to be experiencing a neurodevelopmental disnormal life span. orders are the underlying medical complications of the Those children who surcause of “developmental neurodevelopmental disorder: vive early problems and who disabilities,” determieventually become adults nation of the precise 1) cognitive dysfunction will likely demonstrate one neurodevelopmental 2) neuromotor dysfunction or more of the four primary disorder is not usually medical complications of the a part of the determina3) seizures, and/or neurodevelopmental disorder: tion of “developmental 1) cognitive dysfunction 2) 4) abnormal impulsive behavior disability.” Sub-categoneuromotor dysfunction 3) ries of developmental 5) sensory disorders. seizures, and/or 4) abnormal disability include mental impulsive behavior. retardation, epilepsy, Depending on which of these four experience “congenital heart disease,” cerebral palsy, and autism. These are primary complications is present all conditions that result from neurodebecause congenital heart disease is a and its severity (some have all four), velopmental disorders, has usually not complication often associated with the adult who suffers from a neubeen determined (although we feel it Down syndrome. rodevelopmental disorder may also should be).

It is therefore important to distindemonstrate a number of “secondary” It appears that one barrier to access guish the primary (complications) conditions – or health “consequencto quality health services for persons and secondary (consequences) sympes” – that result from those “primary” with developmental disabilities relates toms of brain dysfunction (which are complications. to a language issue – specifically, the the same whether onset of the brain For example, as shown in the figure Developmental Disabilities Labels dysfunction is in childhood or during on page 20 of this article, because adulthood) from the syndrome-specific (DDLs). These labels are specificalneuromotor dysfunction (a primary conditions which may be multi-system ly mental retardation (or intellectual “complication”) can cause immobility, and etiologically unrelated to brain disability), cerebral palsy, epilepsy the adult with a neurodevelopmental and autism. The people we serve have dysfunction. disorder may develop inactivity osteotraditionally been labeled with one of “Developmental Disability”: The porosis, cancer of the esophagus, and these DDLs. The problem that octerm developmental disability is a chronic lung disease (all secondary curs when a physician conceptualizes term that is confusing to most physiconditions—or health “consequences” cians. The term has legal, social, and a person with one of those existing of neuromotor dysfunction). medical aspects. If a person meets the DDLs, is that the physician will be less In addition to the “complications likely to consider the multiple health criteria for having a developmental and consequences” of neurodevelopconditions that the person might have. disability, he or she becomes eligimental disorders as discussed above, This has also been called “diagnostic ble for certain government services

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of Developmental Medicine:

FIVE

THE

THE

FIVE

ESSENTIAL CONCEPTS CRITICAL Questions

1. Childhood Onset Brain Dysfunction (COBD) - Symptoms of brain dysfunction that first occur before the age of 22 years (the age when brain development is complete).

The most common symptoms of brain dysfunction are cognitive dysfunction, motor dysfunction, behavior dysfunction and seizures.

Does this patient demonstrate any signs or symptoms of brain dysfunction?

2. Neurodevelopmental Disorder - The specific medical disorder or disease that causes the childhood onset brain problems (e.g. Down syndrome, Angelman syndrome, Fragile X, Perinatal hypoxic/ischemic brain injury, congenital rubella syndrome).

If brain dysfunction is present, what is the specific neurodevelopmental disorder responsible?

3. Complications (of neurodevelopmental disorders) - Refers to cognitive dysfunction, motor dysfunction, behavior dysfunction, and seizures: The symptoms of neurodevelopmental disorders, or the clinical manifestations of COBD. What complications of the neurodevelopmental disorder does this patient demonstrate?

4. Consequences (of the “complications”) - Refers to the medical conditions that result from the four complications (e.g. osteoporosis is a consequence of severe motor dysfunction, oral health disorders result from cognitive dysfunction).

What are the health consequences that have resulted from those complications?

5. Syndrome-specific complications - Refers to health conditions that are associated with a given specific neuro-developmental disorder. For example, thyroid dysfunction with Down syndrome, renal angiomyolipomata with Tuberous Sclerosis or obesity with Prader-Willi syndrome.

Does my patient demonstrate any syndrome-specific health complications? overshadowing” (Reiss). If instead of conceptualizing the person as someone with “mental retardation” – or some other DDL, the physician instead understood the patient to be suffering from a brain disorder, which was in turn caused by an underlying specific genetic or acquired neurodevelopmental disorder, and which is also associ-

ated with “complications” (cognitive, motor, behavior, seizures) and “consequences” and syndrome-specific health complications he/she would be less to overlook any related medical condition.

This “conceptualization” is a “mental training process” that, with practice, will train the physician to

consider all the issues that should be considered in order to provide comprehensive, quality health service to his patients with chronic brain dysfunction. You do not need to think this way if you are a psychologist or social worker, perhaps … but it is “essential” for the health professional to “conceptualize” his patient differently. helenjournal.org • HELEN | 19

These concepts need to be internalized by the physician, dentist, optometrist, nurse – in fact all health professionals – so that comprehensive quality health service may be provided to persons with developmental disabilities. The American Academy of Developmental Medicine and Dentistry calls this thinking construct the “Five Essential Concepts of Developmental Medicine.”

In order to avoid confusion, and to understand the “medical” approach to the individual who is determined by the government to meet the criteria for “developmental disability,” it is important therefore that the physician have a clear understanding of the Five Essential Concepts of Developmental Medicine, as explained on page 22.

Each concept should stimulate the practitioner to ask a critical question (the Five Critical Questions, which correspond to the Five Essential Concepts). We believe that the Five Essential Concepts offer a conceptual framework that provides the physician with an orderly approach to the evaluation and management of those health conditions that frequently occur in their patients with developmental disabilities.

Furthermore, in order for the health professional to be better able to provide comprehensive and quality health services to their patients with developmental disabilities, it is important to distinguish the concept of developmental disability from that of neurodevelopmental disorder.

“Developmental disability” refers to “childhood-onset” (i.e. before the age of 21 years) “disability” (i.e. persistent/permanent substantial functional limitation in the activities of daily living-ADLs) secondary to dysfunction/disease of the central nervous system (primarily the brain).

Sub-categories of developmental disability include mental retardation, epilepsy, cerebral palsy and autism.

The person with a developmental disability, because of persistent substantial functional limitation in 20 | HELEN • helenjournal.org

the activities of daily living (such as eating, bathing, dressing, toileting, etc), becomes eligible for a variety of government services which provide education, vocational support, recreational programs, and residential opportunities. Forty years ago, these services were centralized in large state run facilities; however, today, most people with developmental disabilities receive them from various community-based provider agencies. In contrast to the excellent community-based educational, vocational, recreational, and residential services that have been made available to persons with developmental disabilities, there has been difficulty “mainstreaming” medical services as effectively as has been the case with these other services.

As former Surgeon General David Satcher stated in the introduction to his comprehensive 2002 report on the problem of health disparities and unmet health needs of persons with developmental disabilities, “as our system of care for those with mental retardation evolved, our attention to their health lessened.” Difficulty mainstreaming medical care appears to be in part related to the fact that health

professionals are not well-trained or experienced when it comes to the health conditions frequently encountered in persons (especially adults) with severe developmental disabilities. It also appears to be related to the fact that, for the most part, health professionals play only a small role in designing service policy, since policy tends to emphasize educational, vocational, recreational and residential services and supports, rather than health concerns. This “social” paradigm is certainly appropriate for those individuals who have milder forms of developmental disabilities and are physically and mentally healthy. Unfortunately, individuals with developmental disabilities who are medically fragile or complex and who therefore require a greater emphasis on health services, often find that community-based health professionals lack critical experience regarding their specific health needs. Some states have recently undertaken initiatives designed to correct this problem. These initiatives include the creation of collaborations between medical/dental schools (where research is generated and physician/dentist training occurs), with

The diagram below demonstrates these concepts graphically.

The center hexagon represents the neurodevelopmental disorder that leads to childhood onset brain dysfunction (COBD).

The four rectangles represent the “complications,” each with a few examples of health consequences.

Sensory Disorders sensory input dysregulation

community-based hospitals and clinics so severely that ulcerations of the skin quality of life, even when the cogni(where most services are provided) resulted. Numerous consultations with tive dysfunction remains unchanged. and human services agencies (where dermatologists failed to provide an ac- This report also demonstrates the valresponsibility for service resides). curate diagnosis or effective treatment ue of the PKU diet, even when mental “Neurodevelopmental disorder” in retardation is present. of the skin rash. contrast to “developmental disability” As previously discussed, regardless When Robert was 42 years old, he refers to the specific medical disease of the exact diagnosis, most people was evaluated by a medical team that or condition that causes the develwith neurodevelopmental disorders was attempting to provide accurate opmental disability in the first place. will have one or more of five general “neuro-developmental” diagnoses to Down syndrome, PKU, Fragile X syn- institutionalized residents who had not “complications,” namely: cognitive drome, Angelman syndrome, Prader been previously diagnosed. The amino disability, neuromotor dysfunction, Willi syndrome, William syndrome, acid analysis that was part of the eval- seizures, abnormal behaviors, or sencongenital rubella syndrome, and sory processing disorders. These five uation process revealed that Robert’s the syndrome of perinatal complications of neurodehypoxic/ischemic encephavelopmental disorders, delopathy are all examples of pending on which of the five neurodevelopmental disorcomplications are demonders. Neurodevelopmental ‘Neurodevelopmental disorder’ strated by a given individual disorders can be either with a neurodevelopmental in contrast to ‘developmental “genetic” or “acquired.” In disorder, can additionalorder to provide compredisability’ refers to the specific ly lead to a multitude of hensive and quality health health “consequences.” For medical disease or condition services for patients with example, an individual who developmental disabilities, it that causes the developmental is disabled from hypoxis important for the providic-ischemic encephalopathy disability in the first place. er of healthcare services to (an “acquired” neuro-deknow the precise etiology of velopmental disorder), may the neurodevelopmental disorder (i.e. demonstrate severe motor dysfunction serum phenylalanine level was 34 mg syndrome). This is because knowing (cerebral palsy). As a “consequence” % (normal <4.0 mg %). Thus, the dithe exact cause then directs the health agnosis of phenylketonuria was made. of immobility from motor dysfunction, professional towards syndrome-assothe patient may have difficulty pracBecause of reports in the medical ciated conditions that will need to be ticing good oral hygiene and therefore literature which suggest that dietary evaluated, monitored and/or treated. develop periodontitis. In addition, the phenylalanine restriction may result The case history below illustrates this lack of movement/exercise may also in improvement of behavioral probpoint. lead to osteoporosis and dyslipidemia. lems in persons with phenylketonuria, Robert (not his real name) is a Thus, the “complications” of neuroRobert was placed on a special “PKU 54-year-old man who was diagnosed developmental disorders frequently diet” that restricted his total dietary with severe mental retardation of phenylalanine intake to 250 to 500 mg lead to various health “consequencunknown etiology when he was a es,” which makes it more difficult to per day. This dietary change resulted young child. By early adolescence, in a normal serum pheynlalanine level provide comprehensive quality health Robert demonstrated severe hyperfor Robert. Over the subsequent one to services to patients with neurodevelactivity and self-injurious behavior. two years, Robert’s behavior improved opmental disorders.

He would “bang his head” against the In conclusion, in order for health dramatically. His skin rash cleared, he wall, a behavior which led to many professionals to provide compreno longer required a helmet to protect cuts and bruises. He was placed in a hensive quality healthcare to his/her his head from headbanging, and he state institution by his parents because was tapered off thioridizine complete- patients with developmental disabiliof this severe behavior which could ties, they must be educated regarding ly. He has remained off all psychonot be managed at home. By the time the precise diagnosis of neurodeveltropic medication to this day. Robert he was 20 years old, Robert received clearly demonstrates the principle that opmental disorders as well as the large doses of thioridazine (Mellaril) evaluation and management of the an accurate etiologic neuro-developand wore a large protective helmet in “complications” that result from that mental diagnosis in individuals with order to reduce the effects of his self neurodevelopmental disorder, and also a diagnosis of “mental retardation injurious behavior. He also developed the health “consequences” that result of unknown etiology” can result in a a rash of his legs which he scratched dramatic improvement in that person’s from those complications.

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A Brief Classification of

“Neuro-Developmental Disord “GENETIC” DISORDERS (ABNORMAL GENE/GENES): A. METABOLIC: Missing enzyme leads to build-up of toxic metabolite (secondary brain damage) and/or deficiency of essential end product needed for neuronal function (primary brain damage).

“Primary” metabolic causes: l. Lipids: Sulfatide-lipidosis (Metachromatic Leukodystrophy) 2. Mucopolysaccharides: (Sanfilippo Syndrome) “Secondary” metabolic causes: l. Amino Acids: Phenylalanine accumulation (Phenylketonuria), Homocysteine accumulation (Homocystinuria) 2. Purines/pyrimidines: Uric acid accumulation (Lesch-Nyhan Syndrome)

B. STRUCTURAL MALFORMATIONS: Cellular derangement or malfunction of brain tissue, usually with structural abnormalities of the brain and often other body parts. No known enzyme deficiency or chromosomal abnormality.

1. Neurodysplasia: a. Tuberous Sclerosis; b. Neurofibromatosis 2. Neuro-dysgenesis: neurulation, prosencephalic segmentation, neuronal proliferation, neuronal migration, organization, myelination: a. Lissencephaly (some types); b. Schizencephaly (some types); c. Septo-Optic Dysplasia (some types) 3. Unknown: a. Sturge Weber Syndrome; b. Rett Syndrome-MEC-P2 mutation C. CHROMOSOMAL ABNORMALITIES: 1. Triplet Repeat Disorders a. Fragile X Syndrome (C-X); b. Myotonic Dystrophy (C-19) 2. Aneuploidies: a. Down syndrome; b. Trisomy 8 Mosaic Syndrome; c. Trisomy 13; d. Trisomy 18 3. Microdeletions: a. William’s Syndrome (C-7); b. Prader-Willi Syndrome (paternal C-15); c. Angelman’s Syndrome (maternal C-15); d. Smith-Magenis Syndrome (C-I7); e. Rubenstein-Taybi Syndrome (C-16); f. Velo-Cardio-Facial Syndrome (C-22) 4. Rearrangements: a. Ring 22 syndrome

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“ACQUIRED” DISORDERS:

ders”

A. PRENATAL DISORDER l. Infections (TORCH, Rubella) 2. Toxins (alcohol, maternal PKC) 3. Traumatic (ischemia-hypoxia) 4. Structural Malformations: a. Arnold-Chiari Malformation; b. Dandy-Walker Cyst c. Agenesis of Corpus Callosum; d. Septo-Optic Dysplasia (some types); e. Lissencephaly (some types) B. PERINATAL DISORDER l. Traumatic(hypoxia-ischemia); 2. Toxins (Kernicterus) 3. Prematurity/Low Birth Weight C. POST-NATAL DISORDER 1. Infection (measles); 2. Traumatic; 3. Metabolic (hypothyroid) 4. Neoplasm; 5. Toxins (lead)

“SENSORY” DISORDERS: 1. Sensory Registration 2. Sensory Interpretation 3. Response Organization 4. Response Execution

AUTHORS Philip B. May, MD is an internist in Louisville, Kentucky.

He received his medical degree from University of North Carolina at Chapel Hill School of Medicine and has been in practice for more than 20 years.

Matthew Holder, MD is recognized as an international leader in the emerging field of developmental medicine. In 2002, he took over the American Academy of Developmental Medicine and Dentistry (AADMD). He is now the CEO of Kramer Davis Health.

Henry Hood, DMD, FADD Chief Clinical Officer, Kramer Davis Health, Co-Founder / Past President, American Academy of Developmental Medicine and Dentistry, Co-Founder, American Academy of Developmental Dentistry.

Rick Rader, MD, FAAIDD, FAADM, DHL (hon) is Director, Habilitation Center, Orange Grove Center; President, American Association on Health and Disability; Member, National Council on Disability; Board, American Academy of Developmental Medicine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals. helenjournal.org • HELEN | 23

The Language of Dis

O

HOW TERMS ABOUT DISABILITES HAVE CHANGED THROU

ver the last several decades, the language of disability has changed. And, once it changed, it changed again.

And again. In 2007, the organization known today as the American Association on Intellectual and Developmental Disabilities changed its name to drop the widely-agreed-pejorative ‘mental retardation’ as a response to self-advocates advocating for many, many years to stop using ‘the R word.’ There has also been a shift toward person-first language. This convention demands that we refer to people first – a person with a disability versus a disabled person. Person-first language has its roots in the 1960s but was embraced more fully by the disability community in the late 1980s. Okay, so fair enough.

We use person-first language, we drop the ‘R word’, and all is okay in the world. Yes? Not quite. Or, at least not for everyone. In the autism community, for example, autism is embraced as part 24 | HELEN • helenjournal.org

of a person’s identity. In that context, referring to an autistic person acknowledges that part of the person’s identity. For many, reference to them as a person with autism (person-first language) is anathematic to their preference for identity-first language.

The prominent Autistic Self Advocacy Network (ASAN) puts it this way: “Autism is a part of us. It is a disability that we will have our whole lives, not an illness that might go away. And it is not a bad thing – we are proud to be autistic! We think saying ‘person with autism’ can reinforce stigma or send the message that autism is a scary thing. By saying ‘autistic person’ or ‘autistic community,’ we are emphasizing that autism is an important part of who we are.”

(ASAN, n.d.)

There’s also the increasingly controversial use of ‘special needs’ that has come to refer to people – children mostly, but also adults – with intellectual and/or developmental disabilities

(IDD) (Gernsbacher, et al., 2016).

Many people with IDD and others bristle at the use of this language.

They argue that their needs are no more special than anyone else’s. Each person looks a bit different to other people, and everyone (everyone!) has needs.

A 2021 USA Today article entitled “‘I am not ashamed’: Disability advocates, experts implore you to stop saying ‘special needs’” (Oliver, June 11) refers to the National Center on Disability and Journalism (NCDJ), a service of the Walter Cronkite School of Journalism and Mass Communication at Arizona State University, Disability Language Style Guide, in which the term ‘special needs’ should be “avoided when describing a person with a disability” because “the word ‘special’ in relationship to those with disabilities is now widely considered offensive because it euphemistically stigmatizes that which is different” (NCDJ, 2021). An increasing aware-

By H. Barry Waldman, DDS, MPH, PhD David A. Ervin, BSc, MA, FAAIDD Steven P. Perlman, DDS, MScD, DHL (Hon) Rick Rader, MD, FAAIDD, FAADM

Allen Wong, DDS, EdD, DABSCD

sability

UGHOUT THE YEARS ness for the need for change was the Realities sight, out of mind. Societal attitudes of motivating initiative by The New the day, which persisted into the first Generally speaking, prior to the York Times and the NCDJ to create half of the 20th century, fostered this mid-1800s, people with intellectua fellowship to develop early career segregating style of management, a al disability, cerebral palsy, autism, journalists with an expertise in and and/or epilepsy resided at home and reflection of the belief that disability improve media coverage of disability and disease were one-and-the-same were cared for by their families. Life issues. Yes, language matters. expectancy for individuals with severe and that people with disabilities were The specialized words and redefective and deviant. This gave spective meanings to rise to eugenics, lobotomies and describe people with more. The language of the day disabilities have been was, well, awful. changing along with There is no hard, fast rule that Idiot, imbecile and feechanges in society’s ble-minded were terms used to attitudes. Very old terms is so inviolate as to give us clear describe people with intellectual to describe people with widely in the 19th and compelling guidance but disability IDD include, believe it century. In 1866, A Manual for or not, idiot, imbecile this: Ask. the Classification, Training, and and moron. Fortunately, Education of the Feeble-Minded, language has evolved, Imbecile, and Idiotic was puband people with disabilities simply disabilities was not nearly as long as it lished in London (Duncan & Millard, want to be recognized for their abiliis today. Beginning in the mid-1800s, 1866). This volume essentially codities and aspirations. With that in mind, institutions were built in the US by fied the use of these terms to describe what do we do with the conundrum of state and local governments to house a wide range of “definite mental and disability language? How do we navbodily deficiencies” (p. 2). American people with IDD. These institutions igate person-first versus identity-first physician, Walter Fernald, in his 1912 were usually built on the outskirts language, and why does it matter? of or altogether out of town – out of

“

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speech to the Massachusetts Medical Society, referred to “the feeble-minded [as] a parasitic, predatory class, never capable of self-support or of managing their own affairs. The great majority ultimately become public charges in some form. They cause unutterable sorrow at home and are a menace and danger to the community. Feeble-minded women are almost invariably immoral, and if at large usually become carriers of venereal disease or give birth to children who are as defective as themselves” (Massachusetts Medical Society, 2016).

In 1927, the Supreme Court of the United States, in a decision written by Justice Oliver Wendell Holmes, Jr., ruled that compulsory sterilization of women with intellectual disabilities was permissible “for the protection and health of the state.” In his opinion, Justice Holmes infamously wrote “it is better for all the world, if instead of waiting to execute degenerate offspring for a crime, or to let them starve for their imbecility, society can prevent those who are manifestly unfit from continuing their kind. Three generations of imbeciles are enough” (Carrie Buck v. John Hendren Bell, Superintendent of State Colony for Epileptics and Feeble Minded, 1927).

Indeed, for centuries, society treated people with IDD as objects of fear and pity. The prevailing attitude was that such individuals were incapable of participating in or contributing to society and that they must rely on welfare or charitable organizations (Paul Burtner College of Dentistry, University of Florida, n.d.). Unfortunately, segregating from society further stigmatized people with disabilities and resulted in lives of isolation, disconnection from family and community, and in terrible social and developmental outcomes.

It’s little wonder, in such circumstances, that the words used to described people with IDD were as demeaning and dehumanizing as they were.

In 1966, President Lyndon B. Johnson established the President’s 26 | HELEN • helenjournal.org

Committee on Mental Retardation. Its purpose was to ensure the right of a “decent, dignified place in society” for people with mental retardation.

In 2009, Rosa Marcellino, a nineyear-old girl with Down syndrome expressed that she did not like being called ‘retarded.’ Rosa brought to a boil what had been a simmering movement led by people with IDD for years. Working with her parents, they advocated for the nationwide removal of the ‘R word,’ In 2010, President Barack Obama signed Rosa’s Law

‘defective’) to mental retardation, from ‘trainable mentally handicapped’ to ‘educable mentally retarded’, and, more recently – thankfully – intellectual disabilities or intellectual and/or developmental disabilities. All along, people with IDD have increasingly influenced the terms we use. In the 21st century, influence has changed to expectation, and (finally!) people with disabilities are creating and defining the convention. From the USA Today article referenced above: “[Lawrence] Carter-Long, commu-

(Public Law 111-256) which changed ‘mental retardation’ to ‘intellectual disability’ in US federal law. The law was a key component in the groundswell of advocacy efforts promoting inclusive, people first language for people with intellectual disabilities.

The President’s Committee changed its name to the President’s Committee for People with Intellectual Disabilities, as it is currently known. Words have significance, power and authority, and the ‘R word’ was abandoned because of negative public sentiment.

nications director for the Disability Rights Education & Defense Fund, says the word disabled connects members of the community ‘to each other, our common history, and to the lineage of all those who fought, protested and persisted so that one day we could be proud of disability history too.’ [Lisette] Torres-Gerald says there is power in the word disabled. ‘I am not ashamed to be disabled; I consider it a difference that allows me to view the world in a different way than other people’ (Oliver, 2021).”

In a compelling 2021 interview (Ladau, 2021) with well-known self-advocate, Julie Petty, when asked how she chooses to refer to herself, she said this:

Who Decides?

Terminology has continued to evolve. From mental deficiency (and referring to people as ‘deficient’ or

“

There’s also the increasingly controversial use of ‘special needs’ that has come to refer to people – children mostly, but also adults – with intellectual and/or developmental disabilities (IDD) (Gernsbacher, et al., 2016). Many people with IDD and others bristle at the use of this language.

They argue that their needs are no more special than anyone else’s.

“I think language has evolved over time – I used to be strict about being called a person first because I feel I deal with ableism all the time, and people see my disability, and that’s what they see first. So therefore, they don’t treat me maybe like a person; they treat me as a child or talk down to me. So, I prefer people-first language. But as I said, I have evolved, and we have evolved as a disability community. And I don’t mind being called a ‘disabled woman.’ I’m proud to reclaim my disability, as you were saying. But don’t call me ‘cerebral palsy girl,’ you know? Don’t call me by my disability. So, I go back and forth between people-first language and identity-first. But I think people

should be able to choose, and I always say everybody has their name. Just call people by their name!”

Self-Advocates Becoming Empowered (SABE), a national self-advocacy organization whose leadership and Board of Directors are comprised of people with IDD and other disabilities, publish a People First Language Manual in which the organization quite unequivocally states “we will not stand for any other languige except people first languige” [sic] (p. 2) (SABE, n.d.).

Renowned autistic advocate, Lydia Brown, offers all of us sage guidance, which drives at the heart of who gets to choose what language: When in doubt, ask.

“Different communities of actually-disabled people or people with disabilities have different preferences.

But as with any group, especially any group of marginalized people, it’s the people who we’re talking about who should be dictating what they’re called. The same word that’s empowering for some people might be retraumatizing for others.

“If you don’t know the preference of the person and don’t have a chance to ask them, go with the majority opinion of the community, because that’s a safe bet. If you do have a chance to ask, ask and use that. And if you’re referring to a group of people who have different preferences, use the language of the majority of the group.” (Callahan, 2018) The Right Words That we will err and cause unknowing offense is as certain as sunrise in the East. The authors of this article, who have dedicated their lives to supporting the dental health of people with IDD, will stumble in choosing the right words.

We all will. This much we know. There is no hard, fast rule that is so inviolate as to give us clear and compelling guidance but this: Ask. Some people with disabilities will indeed prefer identity-first language.

For them, it is ‘disabled people.’ Others will prefer person-first language.

For them, it’s ‘people with disabilities.’ For others, it may be something different altogether. The elegance here is that unlike in the early, darker days of the 19th and well into the 20th centuries, we are finally awakening to people with disabilities/disabled people choosing the words the world uses to describe them.

Our history proves positive that the language we choose can have a profound impact on the actions that helenjournal.org • HELEN | 27

follow. To refer to people as imbeciles and a ‘parasitic, predatory class, never capable of self-support or of managing their own affairs’ gave rise to institutionalization and the eugenics movement, all of which was possible when describing people as less than human.

This history compels us to be thoughtful in the language we choose; and, this history and the trauma it brings to a community of our fellow human beings with IDD and other disabilities settles fully and finally who gets to choose the right words. When in doubt, ask. When you do, listen carefully and embrace the answer, knowing it might change with next person you ask or the next time you ask the same person. That’s all okay. It’s their choice, after all.

REFERENCES Autistic Self Advocacy Network. (n.d.). About ASAN--FAQ. Retrieved July 28, 2022, from https://autisticadvocacy.org/about-asan/faq/ Callahan, M. (2018, July 12). ‘Autistic person’ or ‘person with Autism’: Is there a right way to identify people? News@Northeastern. other, Northeastern University. Retrieved July 13, 2022, from https://news.northeastern.edu/2018/07/12/unpacking-the-debate-over-person-first-vs-identity-first-language-in-the-autism-community/ Carrie Buck v. John Hendren Bell, Superintendent of State Colony for Epileptics and Feeble Minded (United States Supreme Court May 2, 1927).

Duncan, P.M. & Millard, W. (1866). A Manual for the classification, training, and education of the Feeble-minded, imbecile, & idiotic. Longmans, Green, and Co., London.

Gernsbacher MH, Raimond HR et al. Special needs is an ineffective euphemism. Cognitive Research: Principles and Implications. Published online 2016 Dec 19. doi: 10.1186/s41235-0160025-4 Ladau, E. (2021, April 21). A Conversation about Disability and Language with Julie Petty. A Conversation About Disability and Language with Julie Petty. other, Disability and Philanthropy Forum. Retrieved July 13, 2022, from https://disabilityphilanthropy.org/resource/a-conversation-about-disability-and-language-with-julie-petty/ Lightner, L. (n.d.). Special Needs vs Disabled? The “new” term to say instead of Special Needs.

Available from: https://adayinourshoes.com/disabled-instead-of-special-needs/ Massachusetts Medical Society (2019, October 16). The burden of Feeble-Mindedness. Retrieved July 28, 2022, from https://www.massmed.org/About/MMS-Leadership/History/The-Burden-of-Feeble-Mindedness/ Oliver, D. (2021, June 11). ‘I Am Not Ashamed’: Disability Advocates, Experts Implore You to Stop Saying ‘Special Needs’. Available from https://www.usatoday.com/story/life/health-wellness/2021/06/11/disabled-not-special-needs-experts-explain-why-never-use-term/7591024002/ Paul Burtner College of Dentistry, University of Florida. (n.d.). Society’s attitude toward people with disabilities. UF monogram. Retrieved July 28, 2022, from https://paul-burtner.dental.ufl.edu/ oral-health-care-for-persons-with-disabilities/societys-attitude-toward-people-with-disabilities Self Advocates Becoming Empowered. (n.d.). People first language manual - SABE USA. Position Statements--SABE USA. Retrieved July 28, 2022, from https://www.sabeusa.org/wp-content/ uploads/2014/01/People-First-Language-Manual.pdf Walter Cronkite School of Journalism and Mass Communication, Arizona State University. (2021, August). Disability Language Style Guide. National Center on Disability and Journalism. Retrieved July 28, 2022, from https://ncdj.org/style-guide/#mentallyretarded

28 | HELEN • helenjournal.org

David A. Ervin, BSC, MA, FAAIDD, is CEO of Makom, a community provider of supports to people with intellectual and developmental disabilities (IDD) in the Washington, DC metropolitan area. With 35 years in the field, Mr. Ervin has extensive professional experience working in and/or consulting to organizations and governments in the U.S. and abroad. He is a researcher, published author and speaks internationally on health, wellness and healthcare for people with IDD and other areas of expertise.

H. Barry Waldman, DDS, MPH, PhD is SUNY Distinguished Teaching Professor Department of General Dentistry, Stony Brook University, NY. Steven P. Perlman, DDS, MScD, DHL (Hon) is Clinical Professor of Pediatric Dentistry, The Boston University Goldman School of Dental Medicine.

Rick Rader, MD, FAAIDD, FAADM, DHL (hon) is Director, Habilitation Center, Orange Grove Center; President, American Association on Health and Disability; Member, National Council on Disability; Board, American Academy of Developmental Medicine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals; Board, National Task Group on Intellectual Disabilities and Dementia Practices.

Allen Wong, DDS, EdD, DABSCD is a professor and the director of the Dugoni School of Dentistry AEGD and Hospital Dentistry program in the San Francisco area, and is the Global Clinical Advisor to the Special Olympics Special Smiles. He is current president of the American Academy of Developmental Medicine and Dentistry (AADMD).

Proud to be a founding sponsor of Helen: The Journal of Human Exceptionality Even prouder of all we’re accomplishing to improve access to care for the millions of underserved people with special needs across the country.

helenjournal.org • HELEN | 29

AADMD

The

Impact of Ableism

on People with Disabilities

By H. Barry Waldman, DDS, MPH, PhD Rick Rader, MD, FAAIDD, FAADM Steven P. Perlman, DDS, MScD, DHL (Hon) Allen Wong, DDS, EdD, DABSCD

30 | HELEN • helenjournal.org

W

hat is Ableism?

“Racism, sexism, and other forms of discrimination are in the news fairly often. As a developed society, people tend to look at forms of discrimination as problems. This is particularly true if the discrimination is institutionalized throughout government. Institutionalization is a sociological term that describes when a concept becomes intertwined with a government, society, or other institution… (It is) a form of discrimination that is not discussed (frequently) in the media…

This is ableism, which is a form of discrimination against people with disabilities. Ableism can take the form of discrimination or personal prejudice and can hinder the lives of people with disabilities.”1 “The word is ‘ableist’ because it is a way of looking at the world as if everybody had more or less functional bodies and minds. These able-bodied individuals become the norm. When someone who is not able to engage in certain activities comes along (someone with disabilities), he or she can feel as if the world has left them out…

People without disabilities don’t typically have to think of things like the disabled do, and so neglect to address their needs.”1 The first known use of the word “ableist” was in 1981, which makes it a relatively new concept. Ableism can be intentional or unintentional.

People can be an ableist (prejudiced towards people with disabilities) unintentionally because ableism is so common that people may not notice that it exists. Some people describe ableism as a form of oppression. In

some countries, there are laws that are made to protect people with disabilities from ableist discrimination. The Convention on the Rights of Persons with Disabilities also prohibits such discriminations.

• A common way that people with disabilities are abused is by ‘disability bullying’. People think of bullying as something that just happens to children at school and is not serious.

This is not true. Bullying can occur to people of all ages and cause longterm psychological problems such as low self-esteem. Some people commit suicide because they have been bullied. Mencap is a charity in the United Kingdom that helps people with intellectual disabilities. They asked over 500 children with disabilities about bullying in a survey. Eight out of ten children with a learning disability are scared to go out because they are worried they will be bullied.2 Healthcare Services • More than 90% of people (both male and female) with developmental disabilities will experience sexual abuse at some point in their lives.

Forty-nine percent (49%) of them will experience ten or more abuse incidents.3 • More than 1.3 billion people live with some form of disability. That’s 17% of the global population, making it the largest minority group worldwide. Yet the employment rate for people with disabilities is half that of the non-disabled.4 • People with disabilities often experience ableism along with racism, sexism, homophobia, transphobia, income inequality, religious intolerance,

class disadvantage and other forms of oppression.

• These forms of prejudice and discrimination both stack one upon another and intertwine. Depending on numerous factors like race, sexuality, gender, social background, education, wealth (and poverty), these intersections work differently for each person with disabilities.5 • Many people with disabilities, at least to some extent, had to deal with ableism along with other prejudices.

Some may find those other prejudices more harmful and dangerous to them than ableism, as bad as ableism itself can be. Others come to realize that ableism, racism, and other forms of oppression are linked and inseparable. Ableism cannot be adequately addressed without also contending with the many other structural prejudices that continue to shape people’s mindsets, as well as our laws, policies, and everyday practices.5 Lack of People with Disabilities in Positions of Power and Influence One reason why structures of ableism are so prevalent, may be that disability policies and priorities are still dominated by people who aren’t themselves disabled.

• “There are still very few elected officials with disabilities, or important appointees in government. This isn’t necessarily because voters won’t vote for candidates who are disabled. The greater barriers are likely much deeper, involving physical, logistical, and procedural issues; not just to voting, but to all forms of political involvement.

• Many people with disabilities can’t

helenjournal.org • HELEN | 31

attend campaign events because they are physically inaccessible to them.

If they can attend, many can’t follow what’s happening and participate because the information itself isn’t accessible. People with disabilities may not have the resources or financial infrastructure to run for office, for town council, much less for a state legislature or Congress.

• While the lives of people with disabilities are directly affected by numerous structural barriers, they make it difficult for people with disabilities to influence policies that could reduce those barriers. It’s a vicious cycle that is only beginning to be disrupted by people with disabilities in policy-making, activism or elected office; e.g., Sen. Duckworth, a veteran, double amputee, and wheelchair user who helped introduce the ASAP Act to fix long overdue transportation barriers.

• This sobering list of barriers with which they must contend isn’t meant to be discouraging or demotivating but a reminder that “disability awareness” and activities alone can’t dismantle structural ableism. Structural ableism is what contributes most to the lack of basic freedom, mobility and financial stability of people with disabilities.

The Perspective of Healthcare Providers Ableism has long been a serious concern in healthcare policy, and the COVID-19 pandemic has exaggerated and highlighted its prevalence. Studies frequently show how patients with disabilities are considered a “headache” for the healthcare system.

“In a 2020 study, 83.6% of healthcare providers preferred patients without disabilities to those with disabilities. It is important to assess the impact of such views on care for these individuals and healthcare policies

32 | HELEN • helenjournal.org

(emphasis added). One example is the crisis standards of care. Though these standards have been in place before the COVID-19 pandemic, crisis standards of care have come to the forefront of healthcare policy due to their timely relevance. In order to maximize space in hospitals, Several states allow healthca ‘states, localities, and providers, in times of crisis, t individual hospitals… explicitly ruled out triage based on the perceive treatment for people quality of life of the patient with certain ‘pre-existing conditions,’ which tends to be perceived choosing ‘healthy’ lower for those with disabiliti people with no disabilities to live, and ‘sicker’ people with disabilities to die. This policy is especially concerning since, according to the CDC, people with disabilities are at a heightened risk for contracting COVID-19. Additionally, in the second wave of the COVID-19 pandemic in the U.K., people with intellectual disabilities were told that they will not be resuscitated if they become ill with COVID-19. These examples illustrate how the devaluation of people with disabilities’ lives is done for logistical reasons and considered ethically sound.”6 “Another policy example that clearly demonstrates ableism

“

are to ed ts, d as ies.

is that of ventilator rationing for hospitals during the pandemic. New York State instituted explicit guidelines for the distribution of ventilators, naming specific exclusion criteria for access to the ventilators. These criteria were made on the basis of a patient’s functional status, presence of disabilities, and prognosis. Some examples given for functional statuses included recent cardiac arrests, hypotension, and currently needing a ventilator. In England, Scotland and Wales, it is permitted for an abortion to be performed on the basis of the fetus having physical or mental abnormalities.”6 “A 2021 study of over 700 physicians in the United States found that only 56.5% ‘strongly agreed that they welcomed patients with a disability into their practices.’ The same study also found that 82.4% of these physicians believed that people with a significant disability had a lower quality of life than those without disabilities. Data from the 1994-1995 National Health Interview Survey-Disability Supplement has shown that those with disabilities have lower life expectancies than those without. While this can be explained by a myriad

of factors, one of these is the ableism experienced by those with disabilities in clinical settings. Those with disabilities may be more hesitant to seek care when needed due to barriers created by ableism, such as dentist chairs that aren’t accessible or offices filled with bright lights and noises that can be triggering.”6 Several states, including Alabama, Arizona, Kansas, Pennsylvania, Tennessee, Utah, and Washington allow healthcare providers, in times of crisis, to triage based on the perceived quality of life of the patients, which tends to be perceived as lower for those with disabilities. In Alabama, healthcare providers are allowed to exclude patients with disabilities from treatment who require assistance with various daily tasks.6

H. Barry Waldman, DDS, MPH, PhD is SUNY Distinguished Teaching Professor Department of General Dentistry, Stony Brook University, NY.

The Future “Ableism shapes more than just the physical environment. It also shapes our digital and technological imaginations—notions of who will ‘benefit’ from the development of Artificial Intelligence (AI) and the ways that those systems are designed and implemented are a product of how we envision the ‘proper’ functioning of bodies and minds.”7 …How do we reprogram this insidious movement?

Allen Wong, DDS, EdD, DABSCD is a professor and the director of the Dugoni School of Dentistry AEGD and Hospital Dentistry program in the San Francisco area, and is the Global Clinical Advisor to the Special Olympics Special Smiles. He is current president of the American Academy of Developmental Medicine and Dentistry (AADMD).

Steven P. Perlman, DDS, MScD, DHL (Hon) is Clinical Professor of Pediatric Dentistry, The Boston University Goldman School of Dental Medicine.

Rick Rader, MD, FAAIDD, FAADM, DHL (Hon) is Director, Habilitation Center, Orange Grove Center; President, American Association on Health and Disability; Member, National Council on Disability; Board, American Academy of Developmental Medicine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals; Board, National Task Group on Intellectual Disabilities and Dementia Practices.

REFERENCES 1. Study.com Ableism: definition & examples. Available from: com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-more-than-attitudes-andawareness/?sh=25bba0c019c0 https://www.forbes.com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-morethan-attitudes-and-awareness/?sh=25bba0c019c0 2. Lipsett A. Eight out of 10 disabled children bullied report finds. Available from: https://www. theguardian.com/education/2007/jun/18/schools.children 3. Valenti-Hein, D. & Schwartz, L. The Sexual Abuse Interview for Those with Developmental Disabilities. Available from: https://www.mass.gov/service-details/prevalence-of-violence 4. The People Space. The biggest source of untapped talent is ...people with disability. Available from: https://www.thepeoplespace.com/ideas/articles/biggest-source-untapped-talent-people-disability 5. Pulrang A. Fighting ableism is about much more than attitudes and awareness. Available from: https://www.forbes.com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-morethan-attitudes-and-awareness/?sh=1240fc0619c0 6. Ableism. Available from: https://en.wikipedia.org/wiki/Ableism 7. Ableism, Techno-ableism, and Future AI Available from: https://ieeexplore.ieee.org/document/9035527

helenjournal.org • HELEN | 33

Seth and a new friend

How and Why PART TWO

I Recently Visited

UKRAINE

By Seth Keller, MD Photos by Seth Keller, MD

W

hat a sight.

It was pretty obvious that there was significant disruption in Ukraine well before we even crossed the border. Mile after mile, there were 18-wheeler trucks lined up, trying to get into Ukraine. This was created due to security concerns. Because of this great delay, many supplies, including fuel, have been in significantly in short supply. Halyna was driving her Toyota Land Cruiser Prado, which made our trip comfortable and actually she helped us to navigate across the border into Ukraine from Romania pretty easily despite what all of the many truck drivers had to deal with. We spent many hours together. Eric and Halyna have worked together for many years with Disability Rights International (DRI). Halyna is

34 | HELEN • helenjournal.org

an amazing advocate and very passionate for the mission. She also spoke of her recent challenges due to the war, in which she and her mother and two young children had been displaced from Kviv.

I was provided a great amount of detail on the focus of our visits to the various institutions, as well as the work we were going to do in coordination with the BBC. A BBC crew visited Ukraine with Eric and Marisa a few months before and had reported the findings in the news. I watched a video on YouTube before I left for the Ukraine trip so I did have a good sense of what I was also about to see: “Disabled children ‘abandoned’ in Ukrainian institutions - BBC News”. Many of the same crew were returning

everywhere! Evidence of soldiers casually walking around the streets was quite prominent, but I never felt scared or worried until the first of several air raid sirens went off. I watched the locals to see how they’d react to these never-ending high-pitched sounds; they seemed to not even notice them.

They continued to walk around with-

she helped Helyna with a number of the logistics for the mission. It was pretty obvious that Eric, Helyna and Tetiana had a strong working relationship and used their camaraderie to focus on the importance of their mission.

Later that evening, we met up with our BBC friends at a local restaurant serving BBQ. The beer, food and com-

A military checkpoint

to Ukraine to expand upon the story, and we were planning on meeting with them for dinner later that first night in Ukraine.

The main base of operation was from the city of Chernivtsi, a city of approximately 250,000 located in the far southwest of Ukraine and a 1 ½ drive from the Romanian border.

The capital of Ukraine, Kviv, is about an eight-hour drive northeast from where we were. Chernivtsi probably looks like most metropolitan cities in Europe, except for all signs being in Cyrillic. There were also a lot of sign posts, billboards and flags of Ukraine

out any change. As it turns out, the sirens would go off, even if there was a possible air strike many miles away.

The town of Chernivtsi to date had not had a direct attack.

We stayed at a pretty nice hotel, which created a weird combination of emotions in light of our mission and the fact that there was a real war taking place in the country, even though it was being fought many miles far to the east.

We met up later with the final member of our DRI team Tetiana Biriukova. Tetiana had also worked with DRI before as a Ukrainian interpreter and

pany were all amazingly terrific. The chatter was light and actually quite jovial, but the mood would quickly become a lot darker the next morning as we began the first official day of our journey into the institutions of Ukraine.

Halyna was instrumental in helping to set up and arrange a good number of our daily visits. She had made contact with the directors of the various institutions and orphanages that we visited. The selection of sites was based on proximity to where we were staying, the decision of the directors who let us in, plus the type of facility helenjournal.org • HELEN | 35

which we were to see. The various facilities housed different ages of those with IDD; babies, young children and adults. We also knew that there were a good number of people in these facilities who had been displaced from other facilities which had been closer

visited was always a very interesting and cautionary adventure. Helyna was always the lead. She would walk up to the door, fence or whatever barrier separated us from the entrance onto the institutions. Even though the visits were all scheduled and planned ahead

Being that this was my first go at a site visit, I sat back observed how the DRI team and the BBC would conduct their business; then they helped inform me about how I could lend my support to the cause.

The weather turned out to be beautiful even though we all prepared for heavy rain from the initial forecasts. The facility staff had a dozen or so young children all lined up in strollers and, across from them, were older children who were sitting, standing and laying in and around a “playground.”

What I saw can be described on a number of different levels. I am a caregiver at heart and wish to find the humanity and hope that is around.

Aside from the many years working in the IDD field and meeting and helping to support self-advocacy, families and community care, I have been supported by my good friend, Rick Guidotti of Positive Exposure (Positive Exposure - The spirit of Left to right: Seth Keller, Richard Engel, Eric Rosenthal, Halyna Kurlyo, and Tetiana Biriukova difference). Rick has used digital media to use imagto the battle further to the east. of time, it seemed always as if we ery and in giving voice to those with Our first institution was in a small were aliens. The director of the facility disabilities. With this in mind, I began town about an hour or so away. The (who often was the one meeting us at to take picture after picture of many GPS helped Helyna get close to the fa- the entrance) would look around, ask of the people I met. I giggled, made cility but, as it turned out, and I guess more questions, and usually pick up weird faces, and put on music on my not surprisingly so, many facilities their cell phone and call someone, I phone and gave the thumbs up. I guess are often hidden away and not easily guess, to look for guidance on what I could’ve danced but didn’t want found on any sort of map. It was so to do with us - whether to let us in or to scare anyone. Even though many odd that the facility, as it turned out, turn us way. With several more nods, were nonverbal and certainly didn’t was just a block away from where we smiles and gesturing, we always got understand English, I was thrilled to were parked next to a really beautiful inside. We were often advised initially see their smile and hear their laughter. church and small park. It was as if the to be careful on what videos and picThey were kids who deserved a life in facility was invisible, almost hidden tures we took of the residents and the the community and with family! from the small town and its local resgrounds. The directors were told that Sadly, there really is a dark side to idents. Clearly it was segregated and our investigation would help to get the what we saw. Many were extremely not a part of the community in any real word out that there were great inadefrail and way too thin. Many were also sense. quacies of care, and that this attention left alone with no sense of stimulation.

“Knocking on the door” and getmay, in some way or another, lead to Those who appeared to have cerebral ting into each of the facilities that we reforms and supports. palsy, and there were many, did not 36 | HELEN • helenjournal.org

have any idea that their muscles and Ukraine. and also interviewed us. They are joints had EVER received the attention The lack of services that are needputting together a piece that, by time required to prevent their extremities ed to start from the earliest days of this article is out, will most likely be and joints from becoming forever frolife, and in most cases, must continue shown. zen and totally immobile. The kind of throughout their lives, leads to a very An amazing highlight was a meeting pain and discomfort that many of them high mortality rate in infants, as well Richard Engel. Richard is the Chief feel is unimaginable. as early deaths among adults. My spe- Foreign Correspondent for NBC News Being a neurologist, I had the oppor- cialty as a neurologist is working with who has been in Ukraine covering tunity to meet and chat with the medithe geriatric population, but there were the war. As it turns out, he has a son cal staff at each of the facilities that we none to be seen, as they don’t live long Henry, who is 6 years old, with Rett met and was even asked to consult on enough. As it turns out, most die from syndrome (Richard Engel Shares a few “cases.” I got a first-hand view either aspiration pneumonia or heart Health Update on 6-Year-Old Son and a real sense of how Ukrainian failure, all of which are preventable.

Henry | PEOPLE.com). Eric reached healthcare is being provided. out to him and he jumped at the First off, in the other faciliopportunity to cover our story ties we visited, we met those and report on it. His crew drove with IDD of various ages. five hours from the east to meet The youngest was probably Sadly, there really is a dark us, and we drove five hours to the several months old and the to meet him. The tiny village side to what we saw. Many east oldest was a little over 40. where we met probably had never We saw a true representation seen Americans before. Richard were extremely frail and of the IDD population there. interviewed Eric, and then he went way too thin. Many were No one used feeding tubes, onto another institution directly those with spina bifida did also left alone with no sense after meeting with us. His piece not appear to have had any appeared on the Today Show on of stimulation. type of corrective surgery.

June 12, 2022: “How To Help The child with hydrocephaDisabled Ukrainian Children Left lus did appear to have a shunt Behind During War” (today.com). in their brain, however, there was no I’m going to skip over the visits we I have seen this video many times now follow up or management protocol for made over the course of the week, as and shared it with so many others. long-term care. Those with cerebral they were all similar in scope, but at a But what can be done? Now, and by palsy and significant spasticity were different location, and with different whom? I will pick share some answers left to develop irreversible bone and staff. The travel from place to place al- to this and more in Part 3 of the story. joint deformities. It was also obvious lowed us to drive through broad areas It will also include a meeting we had that oral health care was pathetic. I can of rural Ukraine and get an opportuni- with the Chernivtsi local authorities. only imagine what their mouths must ty to see everyday life. This included feel like, such pain and discomfort. driving around and through manned Many who appeared to have autism and unmanned Ukrainian military Seth M. Keller, MD is a board-certified were rocking back and forth, and sevcheckpoints. Also, the steely eyes solneurologist in private practice with eral that we saw were tied up to a bed diers gave us through the car window, Neurology Associates of South Jersey. or a bench for their ”safety” according and how they looked at our passports He specializes in the evaluation and to the staff. No one had access to any with great scrutiny. On one occasion, care of adults with Intellectual and blood testing or imaging. These would I had to hand over my cell phone and Developmental Disabilities (IDD) with be done only in what they deemed to show them whether I had taken any neurologic complications. Dr. Keller be real emergencies at hospital, very wrongful pictures or not. I saw tanks is on the Executive Board of the Arc far away. Social isolation and lack of on a flatbed truck. I also can’t forget of Burlington County, as well as on a smile and personal attention were that we had to have our car windows the board for The Arc of New Jersey absent. Not enough staff, no great down most of the time, since we had Mainstreaming Medical Care Board. training. Abuse and neglect come in to drive with extra gasoline-filled cans Dr. Keller is the Past President of the different forms and measures. A culin the back of the Toyota, as most gas American Academy of Developmental ture which devalues people with IDD stations were closed. The gas fumes Medicine and Dentistry (AADMD). He from birth, segregates them, marginwere just a backstory to our journey. is the co-president of the National Task alizes them, will end up experiencing The BBC crew interviewed the staff, Group on Intellectual Disabilities and all that I observed was happening in took lots of pictures of the grounds Dementia Practices (NTG).

“

helenjournal.org • HELEN | 37

.

The National Task Group on Intellectual Disabilities and Dementia Practices

NEEDS Your Help!

The NTG wants to understand where families and caregivers of individuals with IDD who have dementia get their information and what information would be of most use to them.

Take a brief survey and help NTG with their mission to aid and advocate around issues related to dementia among adults with IDD and their caregivers.

Visit www.SurveyMonkey.com/r/TJ8T5DV OHR-8F

7/2006

Subject Recru

itment Letter

tional (NTG) is a na and Dementia vocate around s tie ili ab is D Intellectual d to aid and ad vers.

Task Group on ce organization chartere sabilities and their caregi an The National di st l si ua as ct l technica ith intelle advocacy and dementia among adults w dementia for to d te la re information on rs to help es e bl issu la ai av e regive pand th initiative to ex out to family and other ca n might be of dertaking an ng atio un hi is rm ac fo re G T in is t N d ha he an w T rs ion and cies, and othe their informat families, agen here they get w nd ta rs de us better un ted key areas rela em. tapping some most use to th ns tio ur es yo qu ound to ey with might send ar ete it. The 15-item surv iate it if you loped a brief pl ec m ve pr co de ap to ve d ha e’ em e W th W ey and ask d its access. rv ouldn’t take an su sh n e d io th t at an ou V rm to info m/r/TJ8T5D this notice ab co rs y. ve ke gi on re ca ym ww.surve network of ted at https://w survey is loca utes to complete. e form min ly in aggregat more than 10 ill be used on w n io at rm fo e in tifiers and th k for any iden n initiative.

We do not as io at , rm our info ho is a friend to help us plan and anyone w le ib ss po as on sponses as so n participate. g to get all re r, or other care partner ca We are lookin be em Committee e, family m mily Support advocate, mat also se cont act Fa org. You can ea g. pl nt e eis .th ar w ey w rv w ve su at ha is u ed th yo ss be acce ns about n.edu if If any questio the-ntg.org. The NTG can erge@jefferso .h el ad at t ec oj t@ familysuppor ary Investigator of this pr im contact the Pr out the project itself. ab any questions

ue: Dear Colleag

Thanks, bera Marianne Bar mittee Support Com ily m Fa ir, ha C up k Gro National Tas

38 | HELEN • helenjournal.org

Take the NTG Survey TODAY! Please complete your survey by September 16, 2022.

THE SANTA FE GROUP IS A 501(C)(3), ACTION-ORIENTED THINK TANK WITH A PASSION TO IMPROVE LIVES THROUGH ORAL HEALTH

SAVE THE DATE!

TUESDAY, SEPTEMBER 6 – WEDNESDAY, SEPTEMBER 7, 2022

Achieving Equitable Oral Health for Those with Intellectual and Developmental Disabilities: A Promise, A Pledge, A Plan Hosted by the University of the Pacific, Arthur A. Dugoni School of Dentistry San Francisco, CA Join us for the Santa Fe Group’s Fall Program, a two-day gathering that celebrates the 25th anniversary of the Santa Fe Group and honors co-founder Dr. Arthur A. Dugoni.

• Hear from nationally known experts in the field of oral health and advocacy • Build understanding of healthcare disparities from a medical perspective, implicit bias in healthcare, the basics of developmental disabilities and more • Engage in breakout sessions to discuss issues of inclusion, health concerns and other topics

“ Early detection gave us time to adapt together, as a family.”

If you’re noticing changes, it could be Alzheimer’s.

Talk about visiting a doctor together.

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• Participate in live simulations of patient experiences with selfadvocates and parents • Discuss overcoming barriers to care, including physical, financial, insurance,attitudes, training and access • Learn about communication, etiquette and empathy; transitioning the patient for a lifetime of health and building the oral health team of the future to care for people with IDD

AND MUCH MORE TO BE ANNOUNCED!

REGISTRATION OPENS SOON! FOR MORE INFORMATION CONTACT ALLEN.WONG@AADMD.ORG. WE HOPE TO SEE YOU IN SEPTEMBER! santafegroup.org

*Breakfast, luncheon, and refreshments will be served on both days

helenjournal.org • HELEN | 39

Prader-Willi Syndrome: Causes of Excessive Daytime Sleepiness

By Althea A. Shelton, MD, MPH

P

rader-Willi Syndrome (PWS) is a genetic disorder that affects multiple organ systems in the body. It is recognized as one of the known leading genetic causes of morbid obesity.1 It is present in approximately 1/15,000 individuals.1 Individuals with PWS have appetite dysregulation and thus never feel satiated; they are always hungry. They also experience issues with body temperature regulation and excessive daytime sleepiness. This is due to hypothalamic dysfunction.1 As stated above, a common symptom that is prevalent in patients with PWS is excessive daytime sleepiness (EDS), also known as hypersomnia.

Hypersomnia can be debilitating to patients with PWS. In a study of 60 adults with PWS, 67% reported excessive daytime sleepiness.2 Oftentimes, when patients with PWS present to a medical provider with hypersomnia, the first thought is obstructive sleep apnea due to obesity. However, the underlying cause/causes of hypersomnia in PWS is much more complex.

There are several sleep disorders that cause hypersomnia, including sleep disordered breathing and central hypersomnia. The term sleep disordered breathing involves disorders of breathing that occur primarily in sleep. The most common type of sleep disordered breathing problem seen in PWS patients is obstructive sleep apnea (OSA). Central hyper40 | HELEN • helenjournal.org

somnias are sleep disorders related to the central nervous system (brain) that cause excessive daytime sleepiness despite sufficient nighttime sleep and other sleep disorders (such as OSA) being effectively treated. One of the most common central hypersomnia is narcolepsy. Narcolepsy is a sleep disorder characterized by abnormal regulation of the sleep-wake cycle. The characteristic symptoms of narcolepsy include hypersomnia, cataplexy (loss of muscle tone with strong emotion), hypnogogic/hypnopompic hallucinations (hallucinations associated with drowsiness) and sleep paralysis. Automatic behavior (repetitive and common behaviors done at night) and disrupted nighttime sleep also commonly occur.

Narcolepsy type 1 (formerly narcolepsy with cataplexy) is caused by the degeneration of the neurons that make the arousal hormone called “hypocretin”.3 These neurons are in the hypothalamus.

Thus, the cerebrospinal fluid (CSF)

of narcolepsy patients has low or no evidence of hypocretin because the hypothalamus no longer has the neurons that produce it. Think of hypocretin as an “manager” of the arousal system.

There are several neurohormones that aid in keeping us alert, but they don’t consistently work without hypocretin around.

It is true that the incidence of OSA in patients with PWS is higher than seen in neurotypical individuals. It has been reported to be as high as 80%.4 In addition, the presentation of OSA can be worse in patients with PWS. They tend to have longer respiratory pauses (apneas) and thus lower oxygen decreases in sleep before they arouse from sleep.5 However, even after OSA has been treated, individuals with PWS can continue to experience significant hypersomnia. Their presentation has been described as “narcolepsy-like”.

In fact, patients with PWS have been found to have lower levels of

:

hypocretin in the CSF, but not as low PWS have a characteristic behavioral some individuals with PWS, modafinil as a narcoleptic patient. In this same profile that can include tantrums, stub- has helped both behavioral and sleep study, it was found that the lower the bornness, insistence on sameness and concerns.10 CSF hypocretin levels, the sleepier the compulsivity.7 Then again, who isn’t Prader-Willi Syndrome is a genetic patient was as measured on the Epmore irritable when they are sleepy disorder that rarely gets mentioned and hungry? Imagine how difficult it worth Sleepiness Scale (a commonly in the media. I have treated sleep used validated scale of sleepiness).6 In must be to feel this way all the time. disorders in patients with PWS for TREATMENT another study, evaluating adults with over 11 years now. The importance PWS, narcolepsy of diagnosing and treating unwas diagnosed in derlying sleep disorders often The hypothalamus maintains 35%, hypersomnia gets overlooked. There should 12% and a suggesbe routine sleep surveillance for homeostasis of our body. It is tive, but unclear all children with PWS. Ideally, involved in controlling our sleepdiagnosis was their care should be managed in a present in 53%.2 multi-disciplinary clinic, but if not wake rhythms, thirst, appetite, Isolated cataplexy available, communication among and the maintenance of our body all the medical specialists needs to is extremely rare and PWS is one of temperature among other things. be open and frequent. the few disorders Dr. Althea A. Shelton, MD, MPH is that can present an Associate Professor of Neurology at Growth hormone therapy is a stanwith isolated cataplexy. Diagnosing Vanderbilt University Medical Center, dard of care for individuals with PWS. narcolepsy in individuals with PWS specializing in sleep medicine. She is inIt is FDA approved for the treatment can be challenging. According to the volved in the diagnosis andDr. treatment of of children. Guidelines require a International Classification of Sleep Allen Wong pediatric sleep disorders and the teachsleep study prior to the initiation of Disorders (ICSD-3), other underlying ing of neurology residents and fellows. growth hormone and then 8-10 weeks causes of sleep fragmentation, such post-initiation.8 Adenotonsillectomy is She is board certified in neurology by as OSA, must be treated to diagnose the American Board of Psychiatry and narcolepsy. So, if an individual has the gold-standard for OSA in chilNeurology (2008) and board certified untreated/undertreated OSA, the defin- dren. Research has shown that adein sleep medicine (2011). She is also a itive diagnosis of narcolepsy cannot be notonsillectomy is safe and effective in member of the Vanderbilt Kennedy Cenmade.3 Thus, diagnosing and treating children with PWS. Although, they are ter. She is the sleep neurologist in the OSA in patients with PWS is importlikely to have residual OSA even after Vanderbilt Multi-disciplinary Prader ant. Because without treating OSA, it treatment.9 PAP therapy is an option to Willi Syndrome Clinic. cannot be excluded as the main cause treat refractory OSA along with weight of hypersomnia. management. Modafinil is an effective This article is included in the upcomUntreated hypersomnia has been treatment in many individuals with ing monograph “Sleep and Sleep associated with an increase in chalhypersomnia in the PWS population.

Disorders in People with Disabilities” lenging behavior in children and adoCare should be exercised due to a published by HELEN: The Journal of lescents with PWS. Individuals with possible increased risk of anxiety. In Human Exceptionality.

REFERENCES al. Decline of CSF orexin (hypocretin) levels in Prader-Willi syndrome.

1. Butler MG, Miller JL, Forster JL. Prader-Willi Syndrome - Clinical Genetics, Diagnosis and Treatment Approaches: An Update. Current Pedi- American Journal of Medical Genetics Part A. 2016;170a(5):1181-6.

7. Richdale AL, Cotton S, Hibbit K. Sleep and behaviour disturbance atric Reviews. 2019;15(4):207-44.

2. Ghergan A, Coupaye M, Leu-Semenescu S, Attali V, Oppert JM, Arnulf in Prader-Willi syndrome: a questionnaire study. Journal of Intellectual Disability Research : JIDR. 1999;43 (Pt 5):380-92.

I, et al. Prevalence and Phenotype of Sleep Disorders in 60 Adults With 8. Tauber M, Diene G, Molinas C. Growth Hormone Treatment for Prader-Willi Syndrome. Sleep. 2017;40(12).

3. American Academy of Sleep M. The international classification of sleep Prader-Willi Syndrome. Pediatric endocrinology reviews : PER. 2018;16(Suppl 1):91-9. disorders. 2014.

9. Lee CH, Hsu WC, Ko JY, Yeh TH, Lin MT, Kang KT. Adenotonsil4. Sedky K, Bennett DS, Pumariega A. Prader Willi syndrome and oblectomy for the Treatment of Obstructive Sleep Apnea in Children with structive sleep apnea: co-occurrence in the pediatric population. Journal Prader-Willi Syndrome: A Meta-analysis. Otolaryngology--head and of clinical sleep medicine : JCSM : official publication of the American neck surgery : OfficialJjournal of American Academy of OtolaryngoloAcademy of Sleep Medicine. 2014;10(4):403-9. gy-Head and Neck Surgery. 2020;162(2):168-76.

5. Arens R, Gozal D, Omlin KJ, Livingston FR, Liu J, Keens TG, et al.

Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. 10. De Cock VC, Diene G, Molinas C, Masson VD, Kieffer I, Mimoun E, et al. Efficacy of modafinil on excessive daytime sleepiness in Journal of Applied Physiology (Bethesda, Md : 1985). 1994;77(5):2224Prader-Willi syndrome. American Journal of Medical Genetics Part A. 30.

6. Omokawa M, Ayabe T, Nagai T, Imanishi A, Omokawa A, Nishino S, et 2011;155a(7):1552-7.

helenjournal.org • HELEN | 41

PA I N

Individuals with IDD & Philadelphia Coordinated Health Care

By Vincent Siasoco, MD, MBA

J

ane was a 30-year-old female with autism, profound intellectual disabilities, overweight, anxiety, and impulse control disorder who recently moved into a new group home two months prior from out of state, where she lived with her elderly parents. The first week she moved in, staff reported that she had ongoing behavioral outbursts. They eventually subsided after that week as she became more adjusted to her new surroundings, according to staff.

At the end of that month, she was introduced to unfamiliar staff and a new individual that had recently moved in. Behavioral issues again began to arise by the beginning of the following month. Jane was brought to her new PCP for an initial evaluation with the plan to follow up with a psychiatrist. After completing a thorough history and examination by her PCP, it was identified that she had her menstrual periods regularly every month, and in the past, took Ibuprofen from her parents to address the cramps.

At the time she moved in, this infor42 | HELEN • helenjournal.org

mation was not passed on. Ibuprofen was placed on the medication list at her group home and the behaviors had resolved.

The International Association for the Study of Pain (IASP) defines pain as the following, “An unpleasant sensory and emotional experience associated

nonhuman animal experiences pain.”1 As in the case above, identifying pain as an issue or cause cannot always be conveyed or recognized in individuals with intellectual and developmental disabilities (IDD). Thus, this increases the risk of diagnostic overshadowing in this population.

People have presumed, since an individual has IDD, they may have a higher threshold for pain because they are Identifying pain as an issue not expressing it. Evior cause cannot always be dence shows, however, that “individuals with conveyed or recognized in intellectual and develindividuals with intellectual opmental disabilities and developmental disabilities. experience acute and chronic pain with at least the same frequenwith, or resembling that associated cy as the rest of the population.”2 In addition, reports have also shown that with, actual or potential tissue damthey “are more likely to have physical age.” The IASP goes on to include and psychological co-morbidities, that, “Verbal description is only one resulting in a higher risk of experiencof several behaviors to express pain; inability to communicate does not ne- ing pain and having more frequent or severe pain.”3 gate the possibility that a human or a

“

Pain is subjective with self-reporting as the “gold-standard.” This becomes a challenge to clinicians and caregivers when this is difficult to convey and information difficult to obtain. “The inability to express the subjective experience of pain through traditional communication modalities such as understanding or expressing language or speech may provide a significant challenge for clinicians to adequately identify and treat pain in individuals with ID, especially in more severe forms.”4 This lack of understanding can unfortunately result in pain being underdiagnosed and not being managed adequately.

Fortunately, the Philadelphia Coordinated Health Care’s booklet, “Management of Pain for Individuals with Intellectual Disabilities and Autism Spectrum Disorder” was published.5 It was first published in 2002 and has since been updated in 2022. The Philadelphia Coordinated Health Care (PCHC) was created in 1989 to support Philadelphians with an intellectual disability (ID) and/or autism.

The mission of PCHC, “is to enhance access to community physical and mental health care through education, public health outreach, advocacy, and empowerment as well as to improve health care outcomes for individuals with an intellectual disability (ID) and/or autism.”

The booklet provides a valuable tool to guide clinicians and caregivers to help identify and manage pain in individuals with IDD.

To view or download a copy of “Management of Pain for People with Intellectual Disabilities (ID) and Autism Spectrum Disorder (ASD), visit: https://pmhcc.app.box.com/s/042sjnbilm0h1wwjhsrgu8j9wa498ygz Vincent Siasoco, MD, MBA is an Assistant Professor, Department of Pediatrics (Developmental Medicine) at the Albert Einstein College of Medicine, New York, NY. He is a Board member at AADMD and Editorial Advisory Board member, for Helen: The Journal of Human Exceptionality.

REFERENCES 1. International Association for the Study of Pain. https://www.iasp-pain. org/ 2. Chantel C. Barney, et al. Challenges in pain assessment and management among individuals with intellectual and developmental disabilities, Pain Rep. 2020 Jul-Aug; 5(4): e821.

3. Doody, O, Bailey, M. Pain and pain assessment in people with intellectual disability: Issues and challenges in practice. Br J Learn Disabil.

2017; 45: 157– 165. https://doi.org/10.1111/bld.12189 4. Tjong E, Keane J, Goldstein L., Managing Pain in Adults with Intellectual Disabilities. Pract Pain Manag. 2019;19(2).

5. Management of Pain for People with Intellectual Disabilities and Autism Spectrum Disorder. https://www.pchc.org/images/PDFs/Publications/Physical-Health/Management-of-Pain-Booklet.pdf

Every person has the right to live well. ™

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Sevita is proud to be a Founding Sponsor of the AADMD’s New Publication, Helen: The Journal of Human Exceptionality helenjournal.org • HELEN | 43

JOURNEYS

Photo by Rick Guidotti

44 | HELEN • helenjournal.org

Photo by Rick Guidotti

The Beginning of Choosing

Kindness

A

s a society, we are brought up to believe that staring is rude, that it is not polite. If you stare at someone, you may hurt their feelings. Often, when we see people who look different than we do we will divert our eyes. Or, we might look right through them, pretending not to notice that they stand out.

Our impulse to stare at them might stem from fear or nerves. For me, I think it is more about curiosity, wanting to understand, rather than to judge. We learn at a very young age to categorize people. Are you short or tall? Do you have brown eyes or blue? So, when we see someone who has a prominent scar or is missing a limb, the inclination is to let our eyes linger on this one feature, something we are not accustomed to seeing in everyday life.

I was born with a craniofacial difference, and as a child I didn’t like it when kids stared at me. I would look away. I did not want to talk about my differences; I wanted to blend in.

However, that started to change when I was in my early twenties. I joined a group called the Inner Faces, and all of its members had craniofacial differences like me. Together, we wrote and performed a show telling the story of our lives. For many of us it was the first time stepping on stage and publicly sharing our stories of growing up with craniofacial differences. After each performance, we had “talkback” sessions with the audience and I recall someone saying that it felt like she had permission to stare and really see us, something that she wouldn’t normally

By Dina Zuckerberg

feel comfortable doing.

It has taken me many years, but I now realize that it is naive to think that people, especially children, won’t notice differences. We all have things that make us unique and I think noticing them can actually be healthy. What it isn’t, is having a negative reaction to these attributes.

I am the Director of Family Programs at myFace, a leading non-profit organization that helps children and adults with craniofacial differences lead full and productive lives. One of the things that I do is share my own story with students from kindergarten through high school and encourage them to choose kindness and be an active upstander instead of a passive bystander.

One of the things that we explore is how to address the anxiety or distress that some might experience as a result of seeing someone different than themselves. There have been laughs.

There have been stares. There have even been tears, but I believe children, while sometimes careless, are not naturally cruel. There is one thing we tell them repeatedly, and it has been well received at every single school we’ve been to: “It is okay to notice someone who looks different. But you have the power to choose your words and choose a kind reaction to difference.”

We teach the kids to ask respectful questions and use thoughtful words.

Rather than staring, or laughing, or asking, “What’s wrong with you?” we suggest alternative questions like, “May I ask you about your face?”

“May I ask why you’re different?”

“May I ask about your scar?”

We explain that many of our myFace kids are happy to share this information, while others would prefer to get to know someone first, and that’s okay, too. When we say this, there seems to be a feeling of relief from both the kids - and the adults - in the audience because it validates their observations and questions.

But how do you react when there is no time for a conversation? A parent once asked my colleague after a presentation, “What is a kind reaction if you see someone who looks different but it’s not on the playground or at school? What if you’re never going to see that person again, but you get caught staring?”

My colleague thought for a moment because our focus is largely on relating to people with differences in long-term settings. What if you react to someone different on the street? On the subway?

At a stoplight? How do you choose kindness if your interaction is only going to be fleeting?

Her response was, “Most of all, don’t ignore the person. Don’t look away. Don’t deliberately pretend they aren’t there. That erases a basic element of human interaction that we all crave.” If you have time, say hello.

And here’s something I truly think would have an impact. If you get caught staring, smile and say, “I’m sorry for staring.”

That’s it. That’s the beginning of choosing kindness. Smiling.

Dina Zuckerberg is Director of Family Programs at myFace.org. helenjournal.org • HELEN | 45

Let’s Get Something Straight:

Ensuring Equitable Healthcare for People with IDD is NOT the ‘Medical Model’

T

By Craig Escudé, MD, FAAFP, FAADM

healthcare suddenly takes on a derogaequitable access to qualified nursing, he Medical Model is a term tory meaning when applied to people medical and other supports, sufficient used to describe an outdated with IDD. Anyone who seeks medical to ensure they receive the same funcway of viewing intellectual care is appropriately called a “patient.” tional supports, preventive care, and and developmental disabilAnd definitely, let’s not throw around ongoing medical care as people who ities (IDD) and is associated with the the term Medical Model any time do not have a disability. approach taken during the years when someone talks about the importance Attaining disability clinical comlarge numbers of people with IDD of equitable access to evidence-based, petency among healthcare providers lived in institutions, where doctors and preventive and ongoing healthcare for nurses were the primary drivers of ser- and ensuring equitable access to people with or without IDD. vice delivery. The Medical Model sees healthcare for people with disabilities We should never disability as a “defect” back to the medwithin the person The Social Model of disability calls go ical model of disand contends that the ability, but advocatfor changing the environment to person needs to be for well-trained, “cured” or “fixed” in better meet the needs of everyone. ing disability-compeorder for them to have tent clinicians and a good quality of life. supporters of people with IDD who is in direct opposition to the Medical Seeing a disability as a deficit can lead can recognize, prevent, and appropriModel, as is identifying health risks to lower self-esteem and perpetuate ately respond to health issues is a far and educating supporters about how the mindset among clinicians that cry from the errant views of the past to recognize them and mitigate them. people with IDD have a lower quality and are grand steps to reducing health Educating healthcare providers and of life and do not deserve the same level of healthcare that people without other supporters to meet the healthcare disparities and achieving health equity for all. needs of people with IDD is exactly IDD receive. The Medical Model can what the Social Model of disability promote feelings of sympathy, pity, or calls for; changing the environment shame towards people with IDD, and Dr. Craig Escudé, MD, FAAFP, to better meet the needs of everyone. often, people are unaware of this bias.

FAADM is a board-certified Fellow Having access to disability-competent The Medical Model has been reof the American Academy of Family healthcare providers who recognize jected by many in favor of the Social Physicians and the American AcadModel of disability. This model focus- the sometimes, unique health needs emy of Developmental Medicine and es on the intersection between disabili- of people with IDD, who understand is the President of IntellectAbility. He ties and the environment within which the link between adverse behaviors has more than 20 years of clinical and the need to look for underlying, the person lives and promotes the creation of environments that are more treatable medical conditions, and who experience providing medical care for people with IDD and complex medical recognize that people with IDD are accessible for all. The Social Modand mental health conditions. He is people first, will go a long way to el calls for an end to discrimination improving overall health, wellness and the author of “Clinical Pearls in IDD through education, accommodation, Healthcare” and developer of the quality of life for people with IDD. and the universal design of structures Let’s not say that the term “medical” “Curriculum in IDD Healthcare”, an and environments to facilitate accessieLearning course used to train cliniis related to the medical model. Let’s bility for everyone. Within the Social cians on the fundamentals of healthnot say that using the term “patient” Model, there is an understanding that care for people with IDD. to describe a person who is receiving people with disabilities should have

“

46 | HELEN • helenjournal.org

Founding Sponsors Comprehensive and collaborative support for the disability community demands partnerships, coalitions and alliances.

The Founding Sponsors of HELEN: The Journal of Human Exceptionality are making it possible to provide valuable information to healthcare professionals, those living with disabilities, their families, and caregivers.

Steve Perlman, DDS, MScD, DHL (Hon.) For information about becoming a Founding Sponsor of HELEN, please contact Vanessa Ira at vira@helenjournal.org. helenjournal.org • HELEN | 47

People-centered care means all your health professionals work together.

Join AADMD - Health professionals united in one voice for inclusive health.

The American Academy of Developmental Medicine & Dentistry (AADMD) is a non-profit, membership organization of interdisciplinary health professionals — including primary physicians, medical specialists, dentists, optometrists, nurses and other clinicians — committed to improving the quality of healthcare for people with intellectual & developmental disabilities (IDD). We make a difference through membership, education and advocacy.

Associate memberships available for non-clinicians as well.

Patients with IDD shouldn’t have to advocate alone.

48 | HELEN • helenjournal.org

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