Helen: The Journal of Human Exceptionality - August 2022 by Helen The Journal of Human Exceptionality

ISSUE NO. 3 AUGUST 2022 ENDORSED BY THE BEGINNING OF By Dina Zuckerberg LANGUAGE Choosing KindnessCONCEPTSESSENTIAL5THE of Developmental Medicine Prader-Willi Syndrome Causes of Excessive Daytime Sleepiness The of Disability

Comprehensive disability clinical care curriculum and training; adoption of enforce able accessible medical and diagnostic equipment standards; designation of people with disabilities as medically underser ved 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.

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Celebrating the ADA Challenging Health Care Inequities

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 countr y changed for the better as a result.

Now, 32 years af ter the ADA, NCD is urgently calling on Congress and the Adminis tration to ac t 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.

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 protec tions for people with disabilities and we authored the initial draf t of the Americans with Disabilities Ac t (ADA).

Doc tors 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 expec t paint the fullest pic ture of the need if we’re not being counted.

Andrés J. Gallegos Chairman, National Council on Disability

helenjournal.org • HELEN | 3 Contents 16 The Five DevelopmentalConceptsEssentialofMedicine By Philip May M.D., Henry Hood DMD, FADD, Matthew Holder MD, MBA, Rick Rader MD, FAAIDD, FAADM A Medical Paradigm for Persons withDisabilitiesDevelopmental Pain, Individuals with IDD and CoordinatedPhiladelphiaHealthCare By Vincent Siasoco, MD, MBA Identifying pain as an issue or cause cannot always be conveyed by individuals with IDD, increasing the risk of diagnostic overshadowing. 42 40 Sleep and Prader-Willi Syndrome By Althea A. Shelton, MD, MPH A genetic disorder that affects multiple organ systems in the body and causes of excessive daytime sleepiness

4 | HELEN • helenjournal.org Contents COLUMNS FEATURES 24 34 Ensuring Equitable Healthcare for People with IDD Is NOT the “Medical Model”

4430 AADMD The Impact

By Craig Escudé, MD, FAAFP, FAADMD

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

By Rick Rader, MD, FAAIDD, FAADM

ON THE COVER: Dina Zuckerberg and friend. Photo by Rick Guidotti - positiveexposure.org

Volume 1, Issue 3

David A. Ervin, BSc, MA, FAAIDD Steven P. Perlman, DDS, MScD, DHL (Hon) Rick Rader, MD, FAAIDD, FAADM Allen Wong, DDS, EdD, DABSCD and Why I Recently Visited Ukraine - Part Two By Seth Keller, MD 34 What Can Be Done to the Risk of Abuse?

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.

Reduce

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

11 Reflections Helen Keller’s Splendor of“Hearing” Beethoven’s Ninth Symphony 6 Nothing is Written Going Down in History

12 Journeys The Beginning of Choosing Kindness By

By H. Barry Waldman, DDS, MPH, PhD

on People with Disabilities

24 How

By Nora J. Baladerian, PhD Dina Zuckerberg of Ableism

The Language of Disability

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.

Vanessa B. Ira Steve Perlman, DDS, MScD, DHL(Hon) Allen Wong, DDS, EdD, DABSCD

Gary Liguori, PhD, FACSM Dean, College of Health Sciences, URI Aca demic Health Collaborative, Kingston, RI

Editor in Chief Senior Managing Editor

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

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Seth Keller, MD Neurologist, Co-president, National Task Group on Intellectual Disabilities and Demen tia Practices

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.

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

American Academy of Developmental Medicine & Dentistry 3 Forester Avenue, #22 Warwick, NY 10990

Renee Dease Specialist & Coordinator, Healthy Athletes Health Programs at Special Olympics Interna tional Steven M. Eidelman

H. Barry Waldman, DDS State University of New York Distinguished Teaching Professor, School of Dental Medi cine, Stony Brook University, NY Dina Zuckerberg Director of Family Programs, MyFace, New York, NY

Senior Editor for SpecialArtPublisherProjectsDirector

HelenJournal.org

H. Rodney Sharp Professor of Human Services Policy & Leadership; Faculty Director, The National Leadership Consortium on Develop mental Disabilities, University of Delaware

Emily Johnson, MD Medical Director, Developmental Disabili ties Health Center, Peak Vista Community Health Centers, Colorado Springs, CO

Susan L. Parish, PhD, MSW Dean and Professor, College of Health Profes sions, 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 Em ployment Policy, Chairman Emeritus, National Council on Disability, President, TRG-16

Rick Rader, MD, FAAIDD, FAADM

Vincent Siasoco, MD, MBA Assistant Professor, Dept. of Family Med | Pe diatrics, Albert Einstein College of Medicine, Director of Primary Care, Rose F. Kennedy CERC, Montefiore, Med. Director, ADAPT Community Network, New York, NY

Barbie Vartanian Parent Advocate; Director, Oral Health Ad vocacy & Policy Initiatives NYU Executive Committee, Project Accessible Oral Health

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

Editorial Advisory Board

Stephen B. Sulkes, MD Pediatrician, University of Rochester Medical Center, Rochester, NY

David Ervin, BSc, MA, FAAIDD Chief Executive Officer, MAKOM, Rockville, MD

Joseph M. Macbeth President/Chief Executive Officer, National Alliance for Direct Support Professionals, Inc.

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

Carl Tyler, MD Gerontologist, Research Director, Lakewood Family Health Center, Cleveland Clinic, OH

It would play out this way, with him saying, “Mr. Rader, third person on the left sitting next to the OAP carry ing the tote from Harrod’s.” I quickly learned that OAP referred to an Old AgeFiguringPensioner.itwas better to offer some thing 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 fea tures 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 encyclo pedia 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 Ableph aron-Macrostomia syndrome in your short list.” “Yes Professor,” I thought to myself, that was going to be my nextEveryguess.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 Microph thalmmia syndrome for Mowart-Wil son syndrome (“How could I?”).

Going DOWN in History1,500-year-old bones believed to be the oldest confirmed case of Down syndrome in the world. Photo credit: Rivollat et al./Elsevier

The Commonwealth of Nations (the former name) was a 54-member hu manitarian 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, Ugan da, 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 Under ground (subway) was a genetics super market. Our professor would take us into “the Tube” to observe those individuals that had recognizable facial features reflective of a variety of genetic disorders.

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

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

6 | HELEN • helenjournal.org NOTHING IS WRITTEN Rick Rader, MD, FAAIDD, FAADM - Editor in Chief - EDITOR’S DESK

WHY HELEN? Alone we can do so little; together we can do so much. - Helen Keller “Nothing is written.”

Down syndrome was first described by an English physician, John Lang don Down, in 1862. He used the term “mongoloid” to describe the condi tion, 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 ob servations, “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 chil dren of the same parents. The number of idiots who arrange themselves around the Mon golian type is so great, and they pres ent such a close mentalanothertosemblancereoneinpower, I shall describe an idiot member of this racial division, selected from the large number that have fallen under my observa tion.”Acase study published in the International Journal of Paleo pathology 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 con firmed case of Down syndrome in the world. The 1,500-year-old skeleton serves to not only refer ence 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 syn drome individual has been so viewed in the context of the ancient commu nity.” This observation is perhaps the most significant one related to the findings of the skeleton.

And while it was not the intention of the Underground Genetics Class, I learned more about human indig nation, stigmatization and indiffer ence 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.”

HELEN: The Journal of Human Exceptionality pays tribute to Helen Keller. Ms. Keller is per haps the most iconic disability rights advocate and an ex ample of how an individual with complex disabilities found and used her stamina, perse verance, resilience and deter mination to accomplish great things. Helen personifies the spirit, mission and vision of The Jour nal of Human Exceptionality. Her remarkable life is a re flection of her determination; she inspired us with her words, “What I’m looking for is not out there, it is in me.”

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This quote from T.E. Lawrence implies that nothing is inevita ble, 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. didn’t encounter an individual with Down syndrome. They were so recog nizable that the Professor never asked us to identify the condition.

- Dr. Rick Rader

distance,theyfellowbodyvationembraced,societyrespondedto,orincludedthem.Myownobserbasedonthelanguageofpassengerswaswerekeepingtheirkeepingtheirheads

While my genetics rotation in the London Underground exposed me to a vast repertoire of human exceptionalities, we did not discuss how

in newspapers, viewing their fingernails, and perhaps getting off the train before their preferred stop.

The American Academy of De velopmental Medicine (AADM) is a medical society dedicated to addressing the complex med ical needs of individuals with intellectual and developmental disabilities across the lifespan. It incorporates clinician training and awareness, teaching, ad vocacy, research, board certifi cation, health equity, interdisci plinary collaboration, inclusive care delivery models and shared decision making.

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Collaborative Organization Mission Statements

To establish alliances between visionary advo cacy and healthcare organizations for the prima ry purpose of achieving better healthcare.

It is the purpose of the Ameri can Academy of Developmen tal Dentistry (AADD) to establish postdoctoral curriculum stan dards 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 Devel opmental Dentistry – to grant board-certification to those den tists who have successfully com pleted these training programs.

To assist in reforming the current system of healthcare so that no person with IDD is left with out access to quality health services.

The AADMD is resolved: AAHD is dedicated to ensuring health equity for children and adults with disabilities through policy, research, education and dissemination at the feder al, state and community level. AAHD strives to advance health promotion and wellness initia tives for people with disabilities. AAHD’s goal are to reduce health disparities between peo ple with disabilities and the gen eral population, and to support full community inclusion and accessibility.

To prepare clinicians to face the unique chal lenges in caring for people with IDD. To provide curriculum to newly established IDD training programs in professional schools across theTonation.increase the body and quality of pa tient-centered research regarding those with IDD and to involve parents and caregivers in this process.

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

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).

Together.

Delta Dental is a registered trademark of Delta Dental Plans Association.

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 www.vibrantcommunities.comIndianawww.deltadental.foundation

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.

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Reducing Improvingbarriers.access.Drivingchange.

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10 | HELEN • helenjournal.org The future of wellness for people with IDD. Terrence Davis • •MedicalDental•Behavioral•Therapies•Prevention Compassionate, interdisciplinary health care for people with IDD.Kramer Davis is proud to sponsor this inaugural edition of Helen.

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 Sympho ny.” 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 sing ing 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 har monious 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.

Courtesy: The Auricle, Vol. II, No. 6, March 1924. American Foundation for the Blind, Helen Keller Archives.

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

The Splendor of “Hearing” Beethoven

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.

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Overcoming fear and inertia to create in those most at children and adults with intellectual and Reduce

People 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 profes sionals 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 theirThischildren.briefarticle will focus on what can be done to reduce the risk of abuse happening to one’s child (mi nor 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 feed back 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 oth ers. 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 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 maga zines or articles on trauma). Trauma therapy was provided for the children. For the mother, grief and trauma ther apy was provided along with treatment for guilt (called, “irrational guilt” in

helenjournal.org • HELEN | 13 such cases), to heal from this unbe lievable 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 de sign 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.)

• What should a sexual assault vic tim do during an assault?

• What should one do after an assault?Icalled several rape treatment cen ters 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 activi ties to build and strengthen Zoe’s com munication and emergency response skills.Wealso 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 communica tion skills with the family. Each family member also had to make a plan, to be able to understand any report of abuse.

at risk of

WHAT

: and developmental

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)?

create

and

and other

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 assault ed. The mother told me that Zoe had very recently been sexually assault ed 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 driv er’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-as sault 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. CAN BE DONE TO By Nora J. Baladerian, PhD resiliency empowerment sexual abuse victimization disabilities disabilities. the Risk of Abuse?

The man said, “No, this is our secret… you don’t have to tell her.” To which he responded, “OK…and I’m going to tell my mom.” He finally gave up and Daniel returned home, where he told his aunt about this “creepy guy” and how he had evaded going with him. The aunt called the camp director to report this. She later called me to let me know that Daniel was the only child not molested by this “creepy guy.” Daniel felt so proud of himself and so empowered for having been able to successfully manage a difficult situation. Since that time, I have assisted many families in de signing risk reduction plans, employing the communica tion skills of the individual, and supporting the parents in knowing that such things can and do happen, and they can benefit from designing, implementing, and practic ing a plan to survive and survive well. In Zoe’s case, she not only survived but felt empowered and wanted “everyone” to know what a great job she had done, as well as Daniel!

After a recent conference in which I provided training on designing a risk reduction plan, the conference direc tor contacted me to tell me that he had just received a call from a mother who had attended the program. Rather than focusing on abuse, she used the plan to address how to respond to her son’s seizures. She designed her plan

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

“Daniel”

I do not want readers to become frightened but rather empowered to be able to create a plan for their children (minors and adults) to know how to respond to attempts to abuse and to report this to their parents or other per son who may be supporting them. I am in the process of re-editing my book called “The “Risk Reduction Guide Book”© which provides additional ideas and plan guides for a variety of living and work situations.

It was particularly of note that because Zoe had imme diately communicated to her mother what had occurred, and the mother, implementing her part in the plan, immediately called the school. The school immediately had called the po lice, who (fortunately) arrived at the school before the bus driver returned. Thus, evidence was present and available for collection in the bus and from the perpetrator. He was imme diately arrested and placed in jail. This contributed greatly to Zoe’s well-being even though she had been assaulted. This taught me the importance and value of having a plan for a disaster. I learned that the value of an individu alized approach is effective to address communication and other issues that may have interfered with a quick dis closure/reporting of abuse.

I asked if Zoe and the family would like to come in for some trauma therapy. The mother laughed and said, “NO! Zoe is so proud of herself for masterfully managing all of the steps of her plan (before, during, after) that she does not need to come in! She is calling everyone to tell them how well she did in this terri ble situation. She wanted me to call you to let you know.”

Another teenager I worked with (we’ll call him “Daniel,”) has Down syndrome and a mild intellectual dis ability, and is verbal. He had also been a victim of sexual assault and was brought to the sessions by his aunt, as his mother and father were deceased. He was only 13 or 14 and was known for being one who “does what he is told” without much resistance, as are many young persons with intellectual disabilities (ID). However, we did talk about his earlier assault how power less he had felt at first and how later he was angry and embarrassed. We completed his trauma treatment and he no longer felt bad about what had happened to him, but, I thought, “what about next time?”

We role-played with me asking him to give me money, go for a walk with me, etc., with his answer being “OK, and I’m going to tell my Mom.”

Those who work and live with indi viduals with ID know that for the most part, they are not “allowed” to say “No” to an adult or person in charge of them (temporarily with changing shifts, etc.). Perpetrators know this too, so we designed an intervention in which he could say “Yes” to any request, even if it felt bad, following it with, “and I’m going to tell my Mom.”

Several months later, the aunt called and told me the following. Daniel had gone to summer camp, and upon returning told her that there was a “creepy guy” who had many times asked Daniel to go with him to see something “really cool!” Daniel followed his feeling, and said to him, “OK, and I’m going to tell my mom!”

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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.

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.

Nora J. Baladerian, PhD is a licensed psychologist and licensed marriage and family therapist (MFT) in Los Angeles, CA. She has worked with individ uals with intellectual/developmental disabilities since 1974, beginning as a case manager at Harbor Regional Center in Torrance, CA. She attended An tioch University to earn her PhD She augmented her education by taking courses in human sexuality and qualified as an AASECT sex educator, and provides sex education programs to individuals with I/DD and their families. She is bilingual (Spanish) and produces presentations and translates most of her books.

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With funding from the US Dept of Justice, Office for Victims of Crime in collaboration with the Arc of River side County, she produced two law enforcement training DVD’s one on how to interview people with disabilities and one on how to conduct an effective and sensitive first 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.

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.

A Passion for Supporting the IDD Community and Proud Supporter of HELEN: The Journal of Human Exceptionality and trained her son’s staff how to implement the plan. Only a few weeks later, he again had a significant sei zure episode and the staff implemented their new plan. She later reported to the conference director that the first responders told her that had her staff not been so well trained, her son may not have survived this episode. I was so pleased that she applied what she had learned to a com pletely different but essential prob lem, and had great success, and by overcoming fear and dread of a terrible problem successfully helped her son.

Nora J. Baladerian, PhD

A Medical Paradigm for Persons with Developmental Disabilities

FIVE of Developmental Medicine: ESSENTIAL CONCEPTSTHE

16 | HELEN • helenjournal.org 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 diagno sis and treatment of that patient popu lation. This is especially true for those clinicians who treat what is perhaps the most clinically challenging patient population imaginable –patients with neurodevelopmental disorders.

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 devel opmental 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 Develop mental Medicine.” It served as the framework for understanding the complex network of neurode velopmental 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. 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.

INTRODUCTION

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

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

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The major functions of the brain include memory and learning, control of motor function, control and routing of sensory input, and emotional regula tion/control. These functions require that specific interrelated brain struc tures 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 prima ry 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.Inaddition, the clinical presenta tions of these five categories vary from person to person. For example, cognitive dysfunction may present as mild dyslexia or severe mental retar dation, 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 “neurodevelop mental 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 classifica tion (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 disor ders.” 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 cogni tive impairment, seizures, neuromotor dysfunction and abnormal behav iors. 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 embryolog ical 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, perina tal or postnatal periods.

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retardation,disabilityriesdisability.”tionadisorderneurodevelopmentalnationdisabilities,”causeordersneurodevelopmentalWhiledisaretheunderlyingof“developmentaldetermiofthepreciseisnotusuallypartofthedeterminaof“developmentalSub-categoofdevelopmentalincludementalepilepsy, cerebral palsy, and autism. These are all conditions that result from neurode velopmental disorders, has usually not been determined (although we feel it should be). It appears that one barrier to access to quality health services for persons with developmental disabilities relates to a language issue – specifically, the Developmental Disabilities Labels (DDLs). These labels are specifical ly mental retardation (or intellectual disability), cerebral palsy, epilepsy and autism. The people we serve have traditionally been labeled with one of these DDLs. The problem that oc curs when a physician conceptualizes a person with one of those existing DDLs, is that the physician will be less likely to consider the multiple health conditions that the person might have. This has also been called “diagnostic any adult individual with a neurode velopmental disorder, depending on the precise etiology of the neurodevel opmental disorder, may also demon strate, “syndrome-specific” conditions. For example, if the specific etiology of the neurodevelopmental disorder is Down syndrome, in addition to the symptoms of brain dysfunction, the person with Down syndrome may also experience “congenital heart disease,” because congenital heart disease is a complication often associated with Down syndrome. It is therefore important to distin guish the primary (complications) and secondary (consequences) symp toms of brain dysfunction (which are the same whether onset of the brain dysfunction is in childhood or during adulthood) from the syndrome-specific conditions which may be multi-system and etiologically unrelated to brain dysfunction.“Developmental Disability”: The term developmental disability is a term that is confusing to most physi cians. The term has legal, social, and medical aspects. If a person meets the criteria for having a developmental disability, he or she becomes eligi ble for certain government services At times, the precise etiology of Childhood Onset Brain Dysfunction (COBD) cannot be determined. In these cases of COBD of unknown etiology (i.e., the precise neurodevel opmental disorder is not known), it is important to continue to search for the exact cause for the reasons discussed below.Some children with these early genetic and/or acquired brain deficits will be stillborn. Others will die in childhood. However, depending on the nature and severity of the neurodevelopmental disor der that is responsible for the COBD, many will survive into adulthood and more and more appear to be experiencing a normal life span. Those children who sur vive early problems and who eventually become adults will likely demonstrate one or more of the four primary medical complications of the neurodevelopmental disorder: 1) cognitive dysfunction 2) neuromotor dysfunction 3) seizures, and/or 4) abnormal impulsiveDependingbehavior.onwhich of these four primary complications is present and its severity (some have all four), the adult who suffers from a neu rodevelopmental disorder may also demonstrate a number of “secondary” conditions – or health “consequenc es” – that result from those “primary” complications. For example, as shown in the figure on page 20 of this article, because neuromotor dysfunction (a primary “complication”) can cause immobility, the adult with a neurodevelopmental disorder may develop inactivity osteo porosis, cancer of the esophagus, and chronic lung disease (all secondary conditions—or health “consequences” of neuromotor dysfunction).

through the State Medicaid program. Benefits vary from state to state. In general, the “diagnosis” of “devel opmental disability” requires that the onset of the condition occurs before age 18 years, is chronic, will require lifelong services, and is associated with substantial functional limitations in most areas of daily living. To deter mine the diagnosis of performsdisability,developmentalapsychologisttwodifferent tests. The first is a test of intellectual or cognitive function (e.g. the Stan ford-Binet IQ test) and the second is a test of functional limitation (e.g. the Vineland test).

In addition to the “complications and consequences” of neurodevelop mental disorders as discussed above, Children who survive early problems and become adults will likely demonstrate one or more of the five primary medical complications of the neurodevelopmental disorder: 1) cognitive dysfunction 2) neuromotor dysfunction 3) seizures, and/or 4) abnormal impulsive behavior 5) sensory disorders. “

2. Neurodevelopmental Disorder - The specific medical disorder or disease that causes the child hood onset brain problems (e.g. Down syndrome, Angelman syndrome, Fragile X, Perinatal hy poxic/ischemic brain injury, congenital rubella syndrome). If brain dysfunction is present, what is the specific neurodevelopmental disorder responsible?

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3. Complications (of neurodevelopmental disorders) - Refers to cognitive dysfunction, motor dys function, 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?

ESSENTIAL CONCEPTS Questions CRITICALTHE FIVE THE FIVE

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 neurodevelopmen tal disorder, and which is also associ

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?

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?

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 dys function and seizures. Does this patient demonstrate any signs or symptoms of brain dysfunction? ated with “complications” (cognitive, motor, behavior, seizures) and “con sequences” 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 com prehensive, quality health service to his patients with chronic brain dys function. 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 “concep tualize” his patient differently. of Developmental Medicine:

The diagram below demonstrates these concepts graphically.

dysregulationsensoryDisordersSensoryinput

Furthermore, in order for the health professional to be better able to pro vide comprehensive and quality health services to their patients with devel opmental disabilities, it is important to distinguish the concept of develop mental disability from that of neurode velopmental“Developmentaldisorder.disability” refers to “childhood-onset” (i.e. before the age of 21 years) “disability” (i.e. per sistent/permanent substantial function al limitation in the activities of daily living-ADLs) secondary to dysfunc tion/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 developmen tal disability, because of persistent substantial functional limitation in These concepts need to be inter nalized by the physician, dentist, optometrist, nurse – in fact all health professionals – so that comprehensive quality health service may be provided to persons with developmental disabil ities. The American Academy of De velopmental Medicine and Dentistry calls this thinking construct the “Five Essential Concepts of Developmental Medicine.”Inorderto 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 physi cian with an orderly approach to the evaluation and management of those health conditions that frequently occur in their patients with developmental disabilities.

The four rectangles represent the “complications,” each with a few examples of health consequences. professionals are not well-trained or experienced when it comes to the health conditions frequently encoun tered in persons (especially adults) with severe developmental disabil ities. 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 pol icy tends to emphasize educational, vocational, recreational and residen tial services and supports, rather than health concerns. This “social” para digm is certainly appropriate for those individuals who have milder forms of developmental disabilities and are physically and mentally healthy. Un fortunately, individuals with develop mental disabilities who are medically fragile or complex and who therefore require a greater emphasis on health services, often find that communi ty-based health professionals lack crit ical experience regarding their specific health needs. Some states have recent ly undertaken initiatives designed to correct this problem. These initiatives include the creation of collabora tions between medical/dental schools (where research is generated and physician/dentist training occurs), with 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, rec reational 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 communi ty-based provider agencies. In contrast to the excellent community-based educational, vocational, recreational, and residential services that have been made available to persons with devel opmental 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 main streaming medical care appears to be in part related to the fact that health

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

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developmentalserviceshensiveorder“genetic”disordersders.neurodevelopmentallopathyhypoxic/ischemicperinatalencephaareallexamplesofdisorNeurodevelopmentalcanbeeitheror“acquired.”Intoprovidecompreandqualityhealthforpatientswithdisabilities, it is important for the provid er of healthcare services to know the precise etiology of the neurodevelopmental disorder (i.e. syndrome). This is because knowing the exact cause then directs the health professional towards syndrome-asso ciated conditions that will need to be evaluated, monitored and/or treated. The case history below illustrates this point.Robert (not his real name) is a 54-year-old man who was diagnosed with severe mental retardation of unknown etiology when he was a young child. By early adolescence, Robert demonstrated severe hyper activity and self-injurious behavior. He would “bang his head” against the wall, a behavior which led to many cuts and bruises. He was placed in a state institution by his parents because of this severe behavior which could not be managed at home. By the time he was 20 years old, Robert received large doses of thioridazine (Mellaril) and wore a large protective helmet in order to reduce the effects of his self injurious behavior. He also developed a rash of his legs which he scratched so severely that ulcerations of the skin resulted. Numerous consultations with dermatologists failed to provide an ac curate diagnosis or effective treatment of the skin rash. When Robert was 42 years old, he was evaluated by a medical team that was attempting to provide accurate “neuro-developmental” diagnoses to institutionalized residents who had not been previously diagnosed. The amino acid analysis that was part of the eval uation process revealed that Robert’s serum phenylalanine level was 34 mg % (normal <4.0 mg %). Thus, the di agnosis of phenylketonuria was made. Because of reports in the medical literature which suggest that dietary phenylalanine restriction may result in improvement of behavioral prob lems in persons with phenylketonuria, Robert was placed on a special “PKU diet” that restricted his total dietary phenylalanine intake to 250 to 500 mg per day. This dietary change resulted in a normal serum pheynlalanine level for Robert. Over the subsequent one to two years, Robert’s behavior improved dramatically. His skin rash cleared, he no longer required a helmet to protect his head from headbanging, and he was tapered off thioridizine complete ly. He has remained off all psycho tropic medication to this day. Robert clearly demonstrates the principle that an accurate etiologic neuro-develop mental diagnosis in individuals with a diagnosis of “mental retardation of unknown etiology” can result in a dramatic improvement in that person’s quality of life, even when the cogni tive dysfunction remains unchanged. This report also demonstrates the val ue of the PKU diet, even when mental retardation is present. As previously discussed, regardless of the exact diagnosis, most people with neurodevelopmental disorders will have one or more of five general “complications,” namely: cognitive disability, neuromotor dysfunction, seizures, abnormal behaviors, or sen sory processing disorders. These five complications of neurode velopmental disorders, de pending on which of the five complications are demon strated by a given individual with a of(cerebraldemonstratevelopmental(anic-ischemicisexample,healthlydisorder,neurodevelopmentalcanadditionalleadtoamultitudeof“consequences.”Foranindividualwhodisabledfromhypoxencephalopathy“acquired”neuro-dedisorder),mayseveremotordysfunctionpalsy).Asa“consequence”immobilityfrommotordysfunction, the patient may have difficulty prac ticing good oral hygiene and therefore develop periodontitis. In addition, the lack of movement/exercise may also lead to osteoporosis and dyslipidemia. Thus, the “complications” of neuro developmental disorders frequently lead to various health “consequenc es,” which makes it more difficult to provide comprehensive quality health services to patients with neurodevel opmental disorders. In conclusion, in order for health professionals to provide compre hensive quality healthcare to his/her patients with developmental disabili ties, they must be educated regarding the precise diagnosis of neurodevel opmental disorders as well as the evaluation and management of the “complications” that result from that neurodevelopmental disorder, and also the health “consequences” that result from those complications.

‘Neurodevelopmental disorder’ in contrast to ‘developmental disability’ refers to the specific medical disease or condition that causes the developmental disability in the first place. “

helenjournal.org • HELEN | 21 community-based hospitals and clinics (where most services are provided) and human services agencies (where responsibility for service resides). “Neurodevelopmental disorder” in contrast to “developmental disability” refers to the specific medical disease or condition that causes the devel opmental disability in the first place. Down syndrome, PKU, Fragile X syn drome, Angelman syndrome, Prader Willi syndrome, William syndrome, congenital rubella syndrome, and the syndrome of

22 | HELEN • helenjournal.org “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: 2.(MetachromaticSulfatide-lipidosisLeukodystrophy)Mucopolysaccharides: (Sanfilippo Syndrome) “Secondary” metabolic causes: l. Amino Acids: Phenylalanine accumulation 2.(Homocystinuria)Homocysteine(Phenylketonuria),accumulationPurines/pyrimidines:Uricacidaccumulation(Lesch-NyhanSyndrome) 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 A Brief Classification of “Neuro-Developmental Disorders”

DISORDERS: 1.

Henry Hood, DMD, FADD Chief Clinical Officer, Kram er Davis Health, Co-Founder / Past President, American Academy of Developmental Medicine and Dentistry, Co-Founder, American Academy of Developmental Den Ricktistry.Rader, MD, FAAIDD, FAADM, DHL (hon) is Direc tor, Habilitation Center, Orange Grove Center; President, American Association on Health and Disability; Member, National Council on Disability; Board, American Acad emy of Developmental Medicine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals. “ACQUIRED” DISORDERS: A. PRENATAL DISORDER l. Infections (TORCH, Rubella) Toxins (alcohol, maternal PKC) Traumatic (ischemia-hypoxia) 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) PERINATAL DISORDER l. Traumatic(hypoxia-ischemia); Toxins (Kernicterus) Prematurity/Low Birth Weight POST-NATAL DISORDER Infection (measles); 2. Traumatic; 3. Metabolic (hypothyroid) Neoplasm; 5. Toxins (lead) “SENSORY” Sensory Registration Sensory Interpretation Response Organization Response Execution

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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 Developmen tal Medicine and Dentistry (AADMD). He is now the CEO of Kramer Davis Health.

Disorders”

HOW TERMS ABOUT DISABILITES HAVE CHANGED THROUGHOUT

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Over 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 Associa tion on Intellectual and Developmental Disabilities changed its name to drop the widely-agreed-pejorative ‘mental retardation’ as a response to self-advo cates 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 ev eryone. In the autism community, for example, autism is embraced as part of a person’s identity. In that context, referring to an autistic person ac knowledges 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 Language of Disability

The prominent Autistic Self Advocacy Network (ASAN) puts it this way: “Autism is a part of us. It is a dis ability 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 commu nity,’ we are emphasizing that autism is an important part of who we are.” (ASAN,There’sn.d.)also the increasingly con troversial use of ‘special needs’ that has come to refer to people – children mostly, but also adults – with intellec tual 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.A2021 USA Today article enti tled “‘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 Communi cation 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

David A. Ervin, BSc, MA, FAAIDD

By H. Barry Waldman, DDS, MPH, PhD

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

THROUGHOUT THE YEARS “

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 soci ety and that they must rely on welfare or charitable organizations (Paul Burt ner College of Dentistry, University of Florida, n.d.). Unfortunately, segregat ing 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 circumstanc es, 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 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 (Public Law 111-256) which changed ‘mental retardation’ to ‘intellectual disability’ in US federal law. The law was a key component in the ground swell 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 Disabil ities, as it is currently known. Words have significance, power and author ity, and the ‘R word’ was abandoned because of negative public sentiment. Who Decides? Terminology has continued to evolve. From mental deficiency (and referring to people as ‘deficient’ or ‘defective’) to mental retardation, from ‘trainable mentally handicapped’ to ‘educable mentally retarded’, and, more recently – thankfully – intellec tual 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 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.’

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:

26 | HELEN • helenjournal.org 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. Fee ble-minded women are almost invari ably immoral, and if at large usually become carriers of venereal disease or give birth to children who are as de fective as themselves” (Massachusetts Medical Society, 2016).

[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).”

Self-Advocates Becoming Empow ered (SABE), a national self-advocacy organization whose leadership and Board of Directors are comprised of people with IDD and other disabili ties, 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,Renownedn.d.).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 actu ally-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 em powering for some people might be retraumatizing for others.

“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 lan guage. 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!”

“If you don’t know the preference of the person and don’t have a chance to ask them, go with the majority opin ion 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 re ferring 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.’ Oth ers will prefer person-first language. For them, it’s ‘people with disabili ties.’ 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 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 develop mental 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. “

Rick Rader, MD, FAAIDD, FAADM, DHL (hon) is Director, Habilitation Center, Orange Grove Center; Presi dent, American Association on Health and Disability; Member, National Council on Disability; Board, Ameri can Academy of Developmental Med icine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals; Board, Nation al Task Group on Intellectual Disabili ties and Dementia Practices.

H. Barry Waldman, DDS, MPH, PhD is SUNY Distinguished Teaching Pro fessor Department of General Dentist ry, Stony Brook University, NY.

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/s41235-016Ladau,0025-4 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, from Paulness/2021/06/11/disabled-not-special-needs-experts-explain-why-never-use-term/7591024002/StopOliver,den-of-Feeble-Mindedness/JulyMassachusettsAvailableLightner,tion-about-disability-and-language-with-julie-petty/https://disabilityphilanthropy.org/resource/a-conversaL.(n.d.).SpecialNeedsvsDisabled?The“new”termtosayinsteadofSpecialNeeds.from:https://adayinourshoes.com/disabled-instead-of-special-needs/MedicalSociety(2019,October16).TheburdenofFeeble-Mindedness.Retrieved28,2022,fromhttps://www.massmed.org/About/MMS-Leadership/History/The-BurD.(2021,June11).‘IAmNotAshamed’:DisabilityAdvocates,ExpertsImploreYoutoSaying‘SpecialNeeds’.Availablefromhttps://www.usatoday.com/story/life/health-wellBurtnerCollegeofDentistry,UniversityofFlorida.(n.d.).Society’sattitudetowardpeople

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 Olym pics Special Smiles. He is current president of the American Academy of Developmental Medicine and Dentistry (AADMD).

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 expe rience working in and/or consulting to organizations and governments in the U.S. and abroad. He is a researcher, published author and speaks inter nationally on health, wellness and healthcare for people with IDD and other areas of expertise.

2022,

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. Posi tion Statements--SABE USA. Retrieved July 28, 2022, from https://www.sabeusa.org/wp-content/ Walteruploads/2014/01/People-First-Language-Manual.pdfCronkiteSchoolofJournalismandMassCommunication, Arizona State University. (2021, August). Disability Language Style Guide. National Center on Disability and Journalism. Re trieved July 28, 2022, from https://ncdj.org/style-guide/#mentallyretarded 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 institution alization 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

28 | HELEN • helenjournal.org

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

Autistic Self Advocacy Network. (n.d.). About ASAN--FAQ. Retrieved July 28, 2022, from Callahan,https://autisticadvocacy.org/about-asan/faq/M.(2018,July12).‘Autisticperson’ or ‘person with Autism’: Is there a right way to identify people? News@Northeastern. other, Northeastern University. Retrieved July 13, 2022, from ty-first-language-in-the-autism-community/https://news.northeastern.edu/2018/07/12/unpacking-the-debate-over-person-first-vs-identi

helenjournal.org • HELEN | 29 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.

By H. Barry Waldman, DDS, MPH, PhD

Steven P. Perlman, DDS, MScD, DHL (Hon) Allen Wong, DDS, EdD, DABSCD

30 | HELEN • helenjournal.org AADMD Impact of Ableism The on People with Disabilities

Rick Rader, MD, FAAIDD, FAADM

What is discriminationand“Racism,Ableism?sexism,otherformsofarein 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 govern ment. Institutionalization is a sociolog ical term that describes when a concept becomes intertwined with a govern ment, 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“Theword 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 (some one with disabilities), he or she can feel as if the world has left them out… People without disabilities don’t typ ically have to think of things like the disabled do, and so neglect to address their needs.”1

Lack of People with Disabilities in Positions of Power and Influence

helenjournal.org • HELEN | 31

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.

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 (preju diced 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 disabili ties from ableist discrimination. The Convention on the Rights of Persons with Disabilities also prohibits such •discriminations.Acommonway that people with disabilities are abused is by ‘disability bullying’. People think of bullying as something that just happens to chil dren 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 bul lied. Mencap is a charity in the United Kingdom that helps people with intel lectual 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

• More than 90% of people (both male and female) with developmen tal disabilities will experience sexual abuse at some point in their lives. Forty-nine percent (49%) of them will experience ten or more abuse inci dents.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 world wide. Yet the employment rate for people with disabilities is half that of the non-disabled.4

• People with disabilities often ex perience ableism along with racism, sexism, homophobia, transphobia, in come inequality, religious intolerance, class disadvantage and other forms of •oppression.Theseforms 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 intersec tions work differently for each person with disabilities.5

• “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 deep er, involving physical, logistical, and procedural issues; not just to voting, but to all forms of political involve •ment.Many people with disabilities can’t

• Many people with disabilities, at least to some extent, had to deal with ableism along with other prejudices. Some may find those other prejudic es 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 insepa rable. Ableism cannot be adequately addressed without also contending with the many other structural preju dices that continue to shape people’s mindsets, as well as our laws, policies, and everyday practices.5

Healthcare Services

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 “head ache” for the healthcare system. “In a 2020 study, 83.6% of health care providers preferred patients without disabilities to those with dis abilities. It is important to assess the impact of such views on care for these individuals and healthcare policies (emphasis added). One example is the crisis standards of care. Though these standards have been in place before the COVID-19 pandem ic, crisis standards of care have come to the forefront of healthcare policy due to their timely relevance. In order to maximize space in hospitals, ‘states, localities, and individual hospitals… explicitly ruled out treatment for people with certain ‘pre-ex isting wavetionally,COVID-19.riskarepeopleaccordingconcerningpolicydisabilities‘sicker’abilitiespeoplechoosingconditions,’‘healthy’withnodistolive,andpeoplewithtodie.Thisisespeciallysince,totheCDC,withdisabilitiesataheightenedforcontractingAddiinthesecondoftheCOVID-19 pandemic in the U.K., people with intellec tual 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 disabil ities’ lives is done for logistical reasons and considered ethically sound.”6“Another policy example that clearly demonstrates ableism “ Several states allow healthcare providers, in times of crisis, to triage based on the perceived quality of life of the patients, which tends to be perceived lower for those with disabilities.

32 | HELEN • helenjournal.org 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 finan cial 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 nu merous 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-mak ing, 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.

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 Dentist ry (AADMD). healthcaretoperceivedpatients,asdisabilities.

of factors, one of these is the ableism experienced by those with disabilities in clinical settings. Those with disabil ities 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.”6Severalstates, including Alabama, Arizona, Kansas, Pennsylvania, Ten nessee, Utah, and Washington allow healthcare providers, in times of crisis, to triage based on the perceived qual ity of life of the patients, which tends to be perceived as lower for those with disabilities. In Alabama, healthcare providers are allowed to exclude pa tients with disabilities from treatment who require assistance with various daily tasks.6 The Future “Ableism shapes more than just the physical environment. It also shapes our digital and technological imagi nations—notions of who will ‘benefit’ from the development of Artificial In telligence (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 insidi ous movement?

1. Study.com Ableism: definition & examples. Available com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-more-than-attitudes-and-from: awareness/?sh=25bba0c019c0 https://www.forbes.com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-morethan-attitudes-and-awareness/?sh=25bba0c019c0

helenjournal.org • HELEN | 33 is that of ventilator rationing for hospitals during the pandemic. New York State instituted explicit guide lines for the distribution of ventilators, naming specific exclusion criteria for access to the ven tilators. These criteria were made on the basis of a patient’s function al status, presence of disabilities, and progno sis. Some examples giv en 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 abnor malities.”6“A2021 study of over 700 physicians in the United States found that only 56.5% ‘strongly agreed that they wel comed patients with a disability into their practices.’ The same study also found that 82.4% of these phy sicians believed that people with a significant disability had a lower quality of life than those without disabilities. Data from the 1994-1995 National Health Inter view bewithout.expectanciesitiesthatSupplementSurvey-DisabilityhasshownthosewithdisabilhavelowerlifethanthoseWhilethiscanexplainedbyamyriad

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

H. Barry Waldman, DDS, MPH, PhD is SUNY Distinguished Teaching Pro fessor Department of General Dentist ry, Stony Brook University, NY.

2. Lipsett A. Eight out of 10 disabled children bullied report finds. Available from: https://www. 3.theguardian.com/education/2007/jun/18/schools.childrenValenti-Hein,D.&Schwartz,L.TheSexualAbuseInterview 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: 5.abilityhttps://www.thepeoplespace.com/ideas/articles/biggest-source-untapped-talent-people-disPulrangA.Fightingableismisaboutmuchmorethanattitudesandawareness.Availablefrom: https://www.forbes.com/sites/andrewpulrang/2021/06/14/fighting-ableism-is-about-much-morement/90355277.6.than-attitudes-and-awareness/?sh=1240fc0619c0Ableism.Availablefrom:https://en.wikipedia.org/wiki/AbleismAbleism,Techno-ableism,andFutureAIAvailablefrom:https://ieeexplore.ieee.org/docu

REFERENCES

Rick Rader, MD, FAAIDD, FAADM, DHL (Hon) is Director, Habilitation Center, Orange Grove Center; Presi dent, American Association on Health and Disability; Member, National Council on Disability; Board, Ameri can Academy of Developmental Med icine and Dentistry; National Medical Advisor, National Alliance for Direct Support Professionals; Board, Nation al Task Group on Intellectual Disabili ties and Dementia Practices.

What 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

By Seth Keller, MD

How and WhyPART TWO UKRAINEI Recently Visited

Seth and a new friend

Photos by Seth Keller, MD

34 | HELEN • helenjournal.org

helenjournal.org • HELEN | 35 an amazing advocate and very pas sionate 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: “Dis abled children ‘abandoned’ in Ukrainian institutions - BBC News”. Many of the same crew were returning to Ukraine to expand upon the story, and we were planning on meeting with them for dinner later that first night in Ukraine.Themain 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 everywhere! Evidence of soldiers ca sually 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 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 tak ing place in the country, even though it was being fought many miles far to the east. We met up later with the final mem ber of our DRI team Tetiana Biriuko va. Tetiana had also worked with DRI before as a Ukrainian interpreter and 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 relation ship and used their camaraderie to fo cus 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 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 con tact 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 A military checkpoint

36 | HELEN • helenjournal.org which we were to see. The various fa cilities 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 to the battle further to the east. Our first institution was in a small town about an hour or so away. The GPS helped Helyna get close to the fa cility but, as it turned out, and I guess not surprisingly so, many facilities are often hidden away and not easily found on any sort of map. It was so odd that the facility, as it turned out, was just a block away from where we were parked next to a really beautiful church and small park. It was as if the facility was invisible, almost hidden from the small town and its local res idents. Clearly it was segregated and not a part of the community in any real sense. “Knocking on the door” and get ting into each of the facilities that we 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 of time, it seemed always as if we were aliens. The director of the facility (who often was the one meeting us at the entrance) would look around, ask more questions, and usually pick up their cell phone and call someone, I guess, to look for guidance on what to do with us - whether to let us in or turn us way. With several more nods, smiles and gesturing, we always got inside. We were often advised initially to be careful on what videos and pic tures we took of the residents and the grounds. The directors were told that our investigation would help to get the word out that there were great inade quacies of care, and that this attention may, in some way or another, lead to reforms and supports.

Left to right: Seth Keller, Richard Engel, Eric Rosenthal, Halyna Kurlyo, and Tetiana Biriukova

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 support ed by my good friend, Rick Guidotti of Posi tive Exposure (Positive Exposure - The spirit of difference). Rick has used digital media to use imag ery and in giving voice to those with disabilities. With this in mind, I began to take picture after picture of many of the people I met. I giggled, made weird faces, and put on music on my phone and gave the thumbs up. I guess I could’ve danced but didn’t want to scare anyone. Even though many were nonverbal and certainly didn’t understand English, I was thrilled to see their smile and hear their laughter. They were kids who deserved a life in the community and with family! Sadly, there really is a dark side to what we saw. Many were extremely frail and way too thin. Many were also left alone with no sense of stimulation. Those who appeared to have cerebral palsy, and there were many, did not

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 beauti ful even though we all pre pared 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.”

helenjournal.org • HELEN | 37

have any idea that their muscles and joints had EVER received the attention required to prevent their extremities and joints from becoming forever fro zen and totally immobile. The kind of pain and discomfort that many of them feel is Beingunimaginable.aneurologist, I had the oppor tunity to meet and chat with the medi cal staff at each of the facilities that we met and was even asked to consult on a few “cases.” I got a first-hand view and a real sense of how Ukrainian healthcare is being provided. First off, in the other facili ties we visited, we met those with IDD of various ages. The youngest was probably several months old and the oldest was a little over 40. We saw a true representation of the IDD population there. No one used feeding tubes, those with spina bifida did not appear to have had any type of corrective surgery. The child with hydrocepha lus did appear to have a shunt in their brain, however, there was no follow up or management protocol for long-term care. Those with cerebral palsy and significant spasticity were left to develop irreversible bone and joint deformities. It was also obvious that oral health care was pathetic. I can only imagine what their mouths must feel like, such pain and discomfort. Many who appeared to have autism were rocking back and forth, and sev eral that we saw were tied up to a bed or a bench for their ”safety” according to the staff. No one had access to any blood testing or imaging. These would be done only in what they deemed to be real emergencies at hospital, very far away. Social isolation and lack of a smile and personal attention were absent. Not enough staff, no great training. Abuse and neglect come in different forms and measures. A cul ture which devalues people with IDD from birth, segregates them, margin alizes them, will end up experiencing all that I observed was happening in Ukraine.Thelack of services that are need ed to start from the earliest days of life, and in most cases, must continue throughout their lives, leads to a very high mortality rate in infants, as well as early deaths among adults. My spe cialty as a neurologist is working with the geriatric population, but there were none to be seen, as they don’t live long enough. As it turns out, most die from either aspiration pneumonia or heart failure, all of which are preventable.

The BBC crew interviewed the staff, took lots of pictures of the grounds and also interviewed us. They are putting together a piece that, by time this article is out, will most likely be shown.Anamazing highlight was a meeting Richard Engel. Richard is the Chief Foreign Correspondent for NBC News who has been in Ukraine covering the war. As it turns out, he has a son Henry, who is 6 years old, with Rett syndrome (Richard Engel Shares Health Update on 6-Year-Old Son Henry | PEOPLE.com). Eric reached out to him and he jumped at the opportunity to cover our story and report on it. His crew drove five hours from the east to meet us, and we drove five hours to the east to meet him. The tiny village where we met probably had never seen Americans before. Richard interviewed Eric, and then he went onto another institution directly after meeting with us. His piece appeared on the Today Show on June 12, 2022: “How To Help Disabled Ukrainian Children Left Behind During War” (today.com). I have seen this video many times now and shared it with so many others. But what can be done? Now, and by whom? I will pick share some answers to this and more in Part 3 of the story. It will also include a meeting we had with the Chernivtsi local authorities.

Sadly, there really is a dark side to what we saw. Many were extremely frail and way too thin. Many were also left alone with no sense of stimulation. “

I’m going to skip over the visits we made over the course of the week, as they were all similar in scope, but at a different location, and with different staff. The travel from place to place al lowed us to drive through broad areas of rural Ukraine and get an opportuni ty to see everyday life. This included driving around and through manned and unmanned Ukrainian military checkpoints. Also, the steely eyes sol diers gave us through the car window, and how they looked at our passports with great scrutiny. On one occasion, I had to hand over my cell phone and show them whether I had taken any wrongful pictures or not. I saw tanks on a flatbed truck. I also can’t forget that we had to have our car windows down most of the time, since we had to drive with extra gasoline-filled cans in the back of the Toyota, as most gas stations were closed. The gas fumes were just a backstory to our journey.

Seth M. Keller, MD is a board-certified neurologist in private practice with Neurology Associates of South Jersey. He specializes in the evaluation and care of adults with Intellectual and Developmental Disabilities (IDD) with neurologic complications. Dr. Keller is on the Executive Board of the Arc of Burlington County, as well as on the board for The Arc of New Jersey Mainstreaming Medical Care Board. Dr. Keller is the Past President of the American Academy of Developmental Medicine and Dentistry (AADMD). He is the co-president of the National Task Group on Intellectual Disabilities and Dementia Practices (NTG).

38 | HELEN • helenjournal.org . 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. The National Task Group on Intellectual Disabilities and Dementia Practices NEEDS Your Help! Take the NTG TODAYSurvey! Visit www.SurveyMonkey.com/r/TJ8T5DV Subject Recruitment Letter OHR-8F7/2006

Thanks,Marianne Barbera Chair, Family Support Committee National Task Group Please complete your survey by September 16, 2022.

Dear Colleague: The National Task Group on Intellectual Disabilities and Dementia (NTG) is a national advocacy and technical assistance organization chartered to aid and advocate around issues related to dementia among adults with intellectual disabilities and their caregivers. The NTG is undertaking an initiative to expand the available information on dementia for families, agencies, and others and is reaching out to family and other caregivers to help us better understand where they get their information and what information might be of most use to them. We have developed a brief 15 item survey with questions tapping some key areas related to information and its access. We’d appreciate it if you might send around to your network of caregivers this notice about the survey and ask them to complete it. The survey is located at https://www.surveymonkey.com/r/TJ8T5DV and shouldn’t take more than 10 minutes to complete. We do not ask for any identifiers and the information will be used only in aggregate form to help us plan our information initiative. We are looking to get all responses as soon as possible and anyone who is a friend, advocate, mate, family member, or other care partner can participate. If any questions about this survey arise please contact Family Support Committee familysupport@the ntg.org. The NTG can be accessed at www.the ntg.org. You can also contact the Primary Investigator of this project at adel.herge@jefferson.edu if you have any questions about the project itself.

helenjournal.org • HELEN | 39 THE SANTA FE GROUP IS A 501(C)(3), ACTION-ORIENTED THINK TANK WITH A PASSION TO IMPROVE LIVES THROUGH ORAL HEALTH FOR MORE INFORMATION CONTACT ALLEN.WONG@AADMD.ORG. WE HOPE TO SEE YOU IN SEPTEMBER! REGISTRATION OPENS SOON! SAVE THE DATE! Achieving Equitable Oral Health for Those with Intellectual and Developmental Disabilities: A Promise, A Pledge, A Plan TUESDAY, SEPTEMBER 6 – WEDNESDAY, SEPTEMBER 7, 2022 Hosted by the University of the Pacific, Arthur A. Dugoni School of Dentistry San Francisco, CA AND MUCH MORE TO BE ANNOUNCED! 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 • 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 santafegroup.org *Breakfast, luncheon, and refreshments will be served on both days “ Early detection gave us time to adapt together, as a family.” If you’re noticing changes, it could be aTalkAlzheimer’s.aboutvisitingdoctortogether. ALZ.org/TimeToTalk

Prader-Willi Syndrome:

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 presen tation has been described as “narcolep sy-like”.Infact, patients with PWS have been found to have lower levels of 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 hallucina tions (hallucinations associated with drowsiness) and sleep paralysis. Auto matic behavior (repetitive and common behaviors done at night) and disrupted nighttime sleep also commonly occur. Narcolepsy type 1 (formerly narco lepsy with cataplexy) is caused by the degeneration of the neurons that make the arousal hormone called “hypocre tin”.3 These neurons are in the hypo thalamus. Thus, the cerebrospinal fluid (CSF)

By Althea A. Shelton, MD, MPH

Causes of Excessive Daytime Sleepiness

40 | HELEN • helenjournal.org of narcolepsy patients has low or no evidence of hypocretin because the hy pothalamus no longer has the neurons that produce it. Think of hypocretin as an “manager” of the arousal system.

Prader-Willi Syndrome (PWS) is a genetic dis order that affects mul tiple organ systems in the body. It is recognized as one of the known leading ge netic causes of morbid obesity.1 It is present in approximately 1/15,000 individuals.1 Individ uals with PWS have appetite dysregulation and thus never feel satiated; they are always hungry. They also experience issues with body temperature regulation and excessive day time sleepiness. This is due to hypo thalamic dysfunction.1 As stated above, a common symp tom 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 exces sive 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 un derlying 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 disor dered 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 hyper

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

This article is included in the upcom ing monograph “Sleep and Sleep Disorders in People with Disabilities”

Dr. Allen Wong hypocretin in the CSF, but not as low as a narcoleptic patient. In this same study, it was found that the lower the CSF hypocretin levels, the sleepier the patient was as measured on the Ep worth Sleepiness Scale (a commonly used validated scale of sleepiness).6 In another study, evaluating adults with PWS, narcolepsy was diagnosed in 35%, hypersomnia 12% and a sugges tive, but presentdiagnosisunclearwasin53%.2IsolatedcataplexyisextremelyrareandPWSisoneofthefewdisordersthatcanpresentwithisolatedcataplexy.

5. Arens R, Gozal D, Omlin KJ, Livingston FR, Liu J, Keens TG, et al. Hypoxic and hypercapnic ventilatory responses in Prader-Willi syndrome. Journal of Applied Physiology (Bethesda, Md : 1985). 1994;77(5):22246.30.Omokawa M, Ayabe T, Nagai T, Imanishi A, Omokawa A, Nishino S, et PWS have a characteristic behavioral profile that can include tantrums, stub bornness, insistence on sameness and compulsivity.7 Then again, who isn’t more irritable when they are sleepy and hungry? Imagine how difficult it must be to feel this way all the time.

2. Ghergan A, Coupaye M, Leu-Semenescu S, Attali V, Oppert JM, Arnulf I, et al. Prevalence and Phenotype of Sleep Disorders in 60 Adults With Prader-Willi Syndrome. Sleep. 2017;40(12).

Dr. Althea A. Shelton, MD, MPH is an Associate Professor of Neurology at Vanderbilt University Medical Center, specializing in sleep medicine. She is in volved in the diagnosis and treatment of pediatric sleep disorders and the teach ing of neurology residents and fellows. She is board certified in neurology by the American Board of Psychiatry and Neurology (2008) and board certified in sleep medicine (2011). She is also a member of the Vanderbilt Kennedy Cen ter. She is the sleep neurologist in the Vanderbilt Multi-disciplinary Prader Willi Syndrome Clinic.

7. Richdale AL, Cotton S, Hibbit K. Sleep and behaviour disturbance in Prader-Willi syndrome: a questionnaire study. Journal of Intellectual Disability Research : JIDR. 1999;43 (Pt 5):380-92.

REFERENCES

1. Butler MG, Miller JL, Forster JL. Prader-Willi Syndrome - Clinical Genetics, Diagnosis and Treatment Approaches: An Update. Current Pedi atric Reviews. 2019;15(4):207-44.

helenjournal.org • HELEN | 41

TREATMENT Growth hormone therapy is a stan dard of care for individuals with PWS. It is FDA approved for the treatment of children. Guidelines require a sleep study prior to the initiation of growth hormone and then 8-10 weeks post-initiation.8 Adenotonsillectomy is the gold-standard for OSA in chil dren. Research has shown that ade notonsillectomy is safe and effective in children with PWS. Although, they are likely to have residual OSA even after treatment.9 PAP therapy is an option to treat refractory OSA along with weight management. Modafinil is an effective treatment in many individuals with hypersomnia in the PWS population. Care should be exercised due to a possible increased risk of anxiety. In al. Decline of CSF orexin (hypocretin) levels in Prader-Willi syndrome. American Journal of Medical Genetics Part A. 2016;170a(5):1181-6.

8. Tauber M, Diene G, Molinas C. Growth Hormone Treatment for Prader-Willi Syndrome. Pediatric endocrinology reviews : PER. 2018;16(Suppl 1):91-9.

10. De Cock VC, Diene G, Molinas C, Masson VD, Kieffer I, Mi moun E, et al. Efficacy of modafinil on excessive daytime sleepiness in Prader-Willi syndrome. American Journal of Medical Genetics Part A. 2011;155a(7):1552-7.

9. Lee CH, Hsu WC, Ko JY, Yeh TH, Lin MT, Kang KT. Adenotonsil lectomy for the Treatment of Obstructive Sleep Apnea in Children with Prader-Willi Syndrome: A Meta-analysis. Otolaryngology--head and neck surgery : OfficialJjournal of American Academy of Otolaryngolo gy-Head and Neck Surgery. 2020;162(2):168-76.

published by HELEN: The Journal of Human Exceptionality.

Syndrome:

3. American Academy of Sleep M. The international classification of sleep disorders. 2014. 4. Sedky K, Bennett DS, Pumariega A. Prader Willi syndrome and ob structive sleep apnea: co-occurrence in the pediatric population. Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine. 2014;10(4):403-9.

Diagnosing narcolepsy in individuals with PWS can be challenging. According to the International Classification of Sleep Disorders (ICSD-3), other underlying causes of sleep fragmentation, such as OSA, must be treated to diagnose narcolepsy. So, if an individual has untreated/undertreated OSA, the defin itive diagnosis of narcolepsy cannot be made.3 Thus, diagnosing and treating OSA in patients with PWS is import ant. Because without treating OSA, it cannot be excluded as the main cause ofUntreatedhypersomnia.hypersomnia has been associated with an increase in chal lenging behavior in children and ado lescents with PWS. Individuals with

The hypothalamus maintains homeostasis of our body. It is involved in controlling our sleepwake rhythms, thirst, appetite, and the maintenance of our body temperature among other things. some individuals with PWS, modafinil has helped both behavioral and sleep concerns.10Prader-Willi Syndrome is a genetic disorder that rarely gets mentioned in the media. I have treated sleep disorders in patients with PWS for over 11 years now. The importance of diagnosing and treating un derlying sleep disorders often gets overlooked. There should be routine sleep surveillance for all children with PWS. Ideally, their care should be managed in a multi-disciplinary clinic, but if not available, communication among all the medical specialists needs to be open and frequent.

Individuals with IDD PAIN Philadelphia Coordinated Health Care & Identifying pain as an issue or cause cannot always be conveyed or recognized in individuals with intellectual and developmental disabilities. “

As in the case above, identifying pain as an issue or cause cannot always be conveyed or recognized in individuals with intellectual and de velopmental 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 not expressing it. Evi dence shows, however, that “individuals with intellectual and devel opmental leastchronicexperiencedisabilitiesacuteandpainwithatthesamefrequen cy as the rest of the population.”2 In addition, reports have also shown that they “are more likely to have physical and psychological co-morbidities, resulting in a higher risk of experienc ing pain and having more frequent or severe pain.”3 By Vincent Siasoco, MD, MBA

42 | HELEN • helenjournal.org

Jane was a 30-year-old female with autism, profound intellec tual 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 psy chiatrist. After completing a thorough history and examination by her PCP, it was identified that she had her men strual periods regularly every month, and in the past, took Ibuprofen from her parents to address the cramps. At the time she moved in, this infor mation was not passed on. Ibuprofen was placed on the medication list at her group home and the behaviors had resolved.TheInternational Association for the Study of Pain (IASP) defines pain as the following, “An unpleasant sensory and emotional experience associated with, or resembling that associated with, actual or potential tissue dam age.” The IASP goes on to include that, “Verbal description is only one of several behaviors to express pain; inability to communicate does not ne gate the possibility that a human or a nonhuman animal experiences pain.”1

SevitaHealth.com

Fortunately, the Philadelphia Coordinated Health Care’s booklet, “Management of Pain for Individuals with Intel lectual 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 Philadel phians 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.”

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

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

helenjournal.org • HELEN | 43

4. Tjong E, Keane J, Goldstein L., Managing Pain in Adults with Intel lectual Disabilities. Pract Pain Manag. 2019;19(2).

3. Doody, O, Bailey, M. Pain and pain assessment in people with intel lectual disability: Issues and challenges in practice. Br J Learn Disabil. 2017; 45: 157– 165. https://doi.org/10.1111/bld.12189

Every person has the right to live well.™

Sevita is proud to be a Founding Sponsor of the AADMD’s New Publication, Helen: The Journal of Human Exceptionality Vincent Siasoco, MD, MBA is an Assistant Professor, De partment 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

To view or download a copy of “Management of Pain for People with Intellectual Disabilities (ID) and Autism Spectrum Disorder (ASD), https://pmhcc.app.box.com/s/042sjnbilmvisit:0h1wwjhsrgu8j9wa498ygz Pain is subjective with self-reporting as the “gold-stan dard.” This becomes a challenge to clinicians and care givers 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 understand ing can unfortunately result in pain being underdiagnosed and not being managed adequately.

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

44 | HELEN • helenjournal.org JOURNEYS

Photo by Rick Guidotti

helenjournal.org • HELEN | 45 As 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, pre tending 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 dif ference, 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 dif ferences 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 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 notic ing them can actually be healthy. What it isn’t, is having a negative reaction to these attributes. I am the Director of Family Pro grams 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.Oneofthe 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 nat urally 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 pow er 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 informa tion, 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 andButquestions.howdoyou react when there is no time for a conversation? A par ent once asked my colleague after a presentation, “What is a kind reaction if you see someone who looks differ ent 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 ele ment 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. of Choosing

By Dina Zuckerberg Kindness

The Beginning

Let’shelenjournal.orgGetSomething

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

The Medical Model is a term used to describe an outdated way of viewing intellectual and developmental disabil ities (IDD) and is associated with the approach taken during the years when large numbers of people with IDD lived in institutions, where doctors and nurses were the primary drivers of ser vice delivery. The Medical Model sees disability as a “defect” within the person and contends that the person needs to be “cured” or “fixed” in order for them to have a good quality of life. Seeing a disability as a deficit can lead to lower self-esteem and perpetuate the mindset among clinicians that people with IDD have a lower quality of life and do not deserve the same level of healthcare that people without IDD receive. The Medical Model can promote feelings of sympathy, pity, or shame towards people with IDD, and often, people are unaware of this bias. The Medical Model has been re jected by many in favor of the Social Model of disability. This model focus es on the intersection between disabili ties and the environment within which the person lives and promotes the creation of environments that are more accessible for all. The Social Mod el calls for an end to discrimination through education, accommodation, and the universal design of structures and environments to facilitate accessi bility for everyone. Within the Social Model, there is an understanding that people with disabilities should have equitable access to qualified nursing, medical and other supports, sufficient to ensure they receive the same func tional supports, preventive care, and ongoing medical care as people who do not have a disability. Attaining disability clinical com petency among healthcare providers and ensuring equitable access to healthcare for people with disabilities is in direct opposition to the Medical Model, as is identifying health risks and educating supporters about how to recognize them and mitigate them.

Educating healthcare providers and other supporters to meet the healthcare needs of people with IDD is exactly what the Social Model of disability calls for; changing the environment to better meet the needs of everyone. Having access to disability-competent healthcare providers who recognize the sometimes, unique health needs of people with IDD, who understand the link between adverse behaviors and the need to look for underlying, treatable medical conditions, and who recognize that people with IDD are people first, will go a long way to improving overall health, wellness and quality of life for people with IDD.

And definitely, let’s not throw around the term Medical Model any time someone talks about the importance of equitable access to evidence-based, preventive and ongoing healthcare for people with or withoutWeIDD.should never go back to the med ical model of dis ability, but advocat ing for tentdisability-compewell-trained,cliniciansand supporters of people with IDD who can recognize, prevent, and appropri ately respond to health issues is a far cry from the errant views of the past and are grand steps to reducing health disparities and achieving health equity for all. Dr. Craig Escudé, MD, FAAFP, FAADM is a board-certified Fellow of the American Academy of Family Physicians and the American Acad emy of Developmental Medicine and is the President of IntellectAbility. He has more than 20 years of clinical experience providing medical care for people with IDD and complex medical and mental health conditions. He is the author of “Clinical Pearls in IDD Healthcare” and developer of the “Curriculum in IDD Healthcare”, an eLearning course used to train clini cians on the fundamentals of health care for people with IDD.

Let’s not say that the term “medical” is related to the medical model. Let’s not say that using the term “patient” to describe a person who is receiving healthcare suddenly takes on a deroga tory meaning when applied to people with IDD. Anyone who seeks medical care is appropriately called a “patient.”

46 | HELEN •

Straight:

“ The Social Model of disability calls for changing the environment to better meet the needs of everyone.

By Craig Escudé, MD, FAAFP, FAADM

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

48 | HELEN • helenjournal.org 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. aadmd.org

Helen: The Journal of Human Exceptionality - August 2022

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