Biopsychosocial Aspects of Chronic Conditions

A CHES Publication

Fall/Winter 2019 I Volume 14

Biopsychosocial Aspects of

CHRONIC

CO N DITIO N S

Making a Choice for Inhibitor Treatment l Your Joint Health is Important l Stressing Out? How about Using the 80/20 Rule? l American with Disabilities Act (ADA) & Section 504: Overview & Reasonable Accommodations l 10 Mindfulness Tips l and more!

Follow us to keep abreast of our programs and announcements facebook.com/ CHES.Programs

instagram.com/ CHES_Programs

LifeLines for HealthSM Disclaimers The views and opinions of our writers are not a reflection of Comprehensive Health Education ServicesTM, Inc. (CHES) or its sponsors.

This newsletter is designed to provide a forum for community members to express their views from an open and honest platform. It is meant to provide a sharing of knowledge and experience to help one another. Nothing in this newsletter is meant to replace the advice of your HTC, medical professional team or insurance provider. You are always urged to seek the opinion of a healthcare professional for treatment and your specific insurance provider for information.

We take your privacy very seriously. We would never disclose your personal health information without your express written consent. We would never sell nor make available our secure database to anyone.

Articles and pictures may not be reproduced, published, and/or placed on websites without the express written permission of CHES. In every publication of LifeLines for HealthSM, we will provide links to other websites that are not owned or controlled by CHES or its sponsors. We cannot be responsible for privacy practices of other website owners, nor can we be responsible for the accuracy of the information provided.

Letter From the Editors Welcome! As we close out a very busy 10th year of providing program experiences to the inhibitor community, we remain inspired and humbled by the grace, resilience, and dignity in how you live your daily lives.

This fall our Feature article takes a dive into the psychosocial effects of managing a chronic condition. Conversations surrounding mental health in the US are finally moving to the forefront. Here at CHES, these conversations occur consistently and have always been interwoven into our programs and LifeLines for Health.

Managing your emotional or mental health, is just as important as managing your physical health. The stressors associated with a chronic condition are numerous.

Everything from appointments, medication protocols and administration, insurance, and the daily demands create significant stress. In Family Matters, read about the 80/20 Rule to assist you in putting less pressure on yourself and reducing stress.

We hear questions all the time about what accommodations and protections are afforded to school age children. What’s the Plan outlines some of the most common accommodations and situations that could be considered discriminatory.

Mindfulness is a valuable tool for bringing yourself back to the moment to slow a continually racing brain. Tear out the graphic in Mind-Body Connection to practice 10 simple exercises to slow you down.

There isn’t a ONE way approach to inhibitor treatment that works for everyone. In Bloodlines, read the experiences of two members of the inhibitor community who have shared their approaches to tolerizing and treating inhibitors. CHES neither endorses or, recommends any inhibitor treatment, as we are an education company not physicians. As educators, we believe in transparent sharing of all information to assist you in making well-informed decisions for yourself, your child or loved ones. As always, we encourage you as consumers to do your homework, avail yourself of studies, and know the risks before you make any decision that could produce positive or negative outcomes in the years to come.

The decision to change treatment, remain with what works, or try any of the new and upcoming products is a personal decision that should be made between yourself, your physician, trusting family/ friends and your child if they are old enough to understand.

Maintaining joint health is crucial for those with a bleeding disorder.

What’s New, shares strategies for prevention of joint damage.

Community Chatter highlights our After the Shock, Leverage, Mom’s Uninhibited, and Momentum programs this year written from our participants' perspectives. We hope their words will encourage you to try one of them next year or motivate you to continue attending! Once you become a member of the CHES family, we don’t let you go!

As we prepare for winter’s imposed rest period, we hope you emerge ready to face the spring with renewed energy. Do you have an idea, comment or suggestion? We really want to hear from you!

- Janet Brewer & Eric Lowe jbrewer@ches.education elowe@ches.education

“What mental health needs is more

sunlight, more candor, more unashamed conversation about illnesses that affect not only individuals, but their families as well."

'

- Glenn Close

To our extended CHES family, thanks for an unforgettable 2019! w CHES

CONTENTS

Integrity, Accuracy, Empathy...

FEATURE 12 I Biopsychosocial Aspects of Chronic Illness The connection between chronic conditions and mental health are astonishing. Sociology expert, Anna Maria Bell explains the symptoms that almost all individuals or caregivers with a chronic condition face, from depression or anxiety to suicide and everything in between.

CONTENTS COMMUNITY CHATTER

WHAT’S NEW

3 I MUM: Rewarding Surrender

24 I Your Joint Health Is Important

Mother of two boys (one with hemophilia and an inhibitor), Stephanie Shea did something incredibly hard - something others just like her would find equally difficult. Caregivers too often neglect their own needs because they put themselves last. For just one weekend, she took time for herself... a rewarding decision she came to appreciate.

Ask anyone with severe hemophilia who has surpassed his/her 20's and they'll most likely tell you that it's far better to care for your joints early in life than to repair them after the damage is done. Follow these tips for a healthier set of joints.

7 I Leverage: Taking Time On Heard of taking "time off"? How about "time on"? The combination of Donovan Guerrero's tenacity and his recent experiences at our adult inhibitor camp, Leverage has sparked a new level of drive in his aspirations.

8 I After the Shock: Camp Fosters Healing, Friendship, & Play We, once again, hear from Stephanie Shea and her family's variety of experiences at CHES' inhibitor camp, After the Shock for children of all ages.

11 I Momentum: The Old Guard Ken Martin has seen a lot, heard a lot, and been through a lot as a survivor of the many eras in hemophilia. This summer, he attended Momentum, a symposium specifically for men with inhibitors. Ken explains why programs like this are meaningful even to veterans like himself.

MIND BODY CONNECTION 21 I 10 Mindfulness Tips Mindfulness expert, Krystyn Strother provides ten of her best mindfulness tips that we can use at home or on the go.

FAMILY MATTERS 28 I Stressed Out? How About Using the 80/20 Rule?

What's the 80/20 rule? In a nutshell, relieving the stresses and responsibilities in your life by 20%. But that's easier said than done. Dr. Gary McClain explains the different techniques to teach you in an effective, healthy way.

BLOODLINES 34 I Making a Choice for Inhibitor Treatment You've done the research, attended the sessions, spoken to your treaters. But how many life stories have you been told on what families are doing to tolerize that pesky inhibitor? We're sharing two of them right here to help!

WHAT’S the PLAN? 38 I American with Disabilities Act (ADA) & Section 504: Overview & Reasonable Accommodations There's a lot to remember - we get it. That's why we've compiled some good info with lots of lists from discriminatory actions to helpful accommodations for your kiddos.

2

n

LIFELINES for HEALTH

n

Fall/Winter 2019

CONTENTS

Rewarding Surrender W

hen I first heard about the MUMSM event (Moms’ ‘ Uninhibited Meeting), I made up a zillion reasons why I wouldn’t be able to go. The dates weren’t right with tentative summer plans we had already made, it was too close to other events we had already committed to, my son might need me (his port had been acting up), a friend had mentioned she may potentially come to town that weekend....yada, yada, yada. Excuses. All of them.

The truth was, I was scared to leave my 5-year-old son.

My husband is amazing and completely capable of taking care of our two boys, daily factor infusions and all. But if something did happen to my boy, I would want to BE there - to reassure and hug and comfort and do all those things that us mamas do. But it’s often all of those things that us mamas do that can lead to burnout and feeling depleted. And when we’re exhausted and not taking care of ourselves, it’s often the case that we don’t have anything left to give to those we love around us.

by Stephanie Shea

arrive, I took the time to chat with some of the other moms.

And then I had a very happy surprise when I met someone with whom I had only ever connected with online. She was my first ever hemophilia mom friend and she reached out to me online through social media mere days after my son was first diagnosed (when my head was still spinning from the shock). She was a huge support to me then and I was thrilled to finally meet her in person. And I had no idea she was going to be there, such an awesome surprise!

Continued on page 6

Being a caregiver for someone with a chronic condition such as hemophilia can be frustrating at times and exhausting. It’s often emotionally, physically, and mentally draining. It may limit your social life and can cause financial problems. Burnout occurs when the stress and burden from these negative effects become overwhelming, negatively affecting your life and health.

When I received the email about the MUM event, I was definitely experiencing burnout. Before I had children, I was a professional counselor/therapist, so I was very aware of the signs and symptoms. Yet I still had a hard time agreeing to the trip. My husband finally -lovingly- nudged me to commit to it (and I’m so grateful he did!)

The event was held at a beautiful hotel in downtown Santa Fe. When I first arrived and checked in to my room, I felt at a loss. What do I do with myself when I don’t have two little humans to look after?! I realized my predicament and committed to sinking into that uncomfortable space for the weekend in hopes of not only recharging my mama batteries but rekindling my relationship with myself.

The event started with a welcome session before dinner as everyone began trickling in from different places all over the U.S. Since I was one of the first to

3

n

LIFELINES for HEALTH

n

Fall/Winter 2019

COMMUNITY CHATTER

LIVE IN THE BLEED-FREE MOMENT …with FEIBA® prophylaxis patients can have more bleed-free days as compared to on-demand treatment.

72

%

REDUCTION

Every joint bleed has the potential to do permanent damage1,2 Median ABR with prophylaxis vs. on-demand3* 28.7 median ABR with on-demand treatment3 629 bleeding episodes occurred during on-demand treatment3

in median ABR with prophylaxis treatment3

7.9 median ABR with prophylaxis treatment3 196 bleeding episodes occurred during prophylaxis treatment3

N O B L E E DS OCCURRED IN 3 OUT OF 17 PATIENTS on FEIBA prophylaxis in a clinical study3†

*Based on the results from the FEIBA PROOF clinical study of 36 hemophilia A and B patients with inhibitors receiving FEIBA for prophylaxis or on-demand treatment for 12 months.3 † Of those patients who achieved zero bleeding events, 2 out of 3 completed the study.4

FEIBA is the ONLY FDA-approved treatment indicated for use in hemophilia A and B patients with inhibitors for routine prophylaxis.3

FEIBA [Anti-Inhibitor Coagulant Complex] Indications and Detailed Important Risk Information for Patients What is FEIBA? FEIBA is an Anti-Inhibitor Coagulant Complex approved for use in hemophilia A and B patients with inhibitors for: • Control and prevention of bleeding episodes • Use around the time of surgery • Routine prophylaxis to prevent or reduce the frequency of bleeding episodes.

FEIBA is NOT for use in the treatment of bleeding episodes resulting from coagulation factor deficiencies without inhibitors to factor VIII or factor IX.

Detailed Important Risk Information for FEIBA WARNING: EVENTS INVOLVING CLOTS THAT BLOCK BLOOD VESSELS • Blood clots that block blood vessels and their effects have been reported during post-marketing surveillance following infusion of FEIBA, particularly following administration of high doses (above 200 units per kg per day) and/or in patients at risk for forming blood clots.

• If you experience any of these side effects, call your doctor right away.

Who should not use FEIBA?

What other important information should I know about FEIBA? (continued) The safety and efficacy of FEIBA for breakthrough bleeding in patients receiving emicizumab has not been established. Events of thrombotic microangiopathy (TMA), a condition where blood clots and damage occur in small blood vessels, were reported in an emicizumab (Hemlibra®) clinical trial where patients received FEIBA with emicizumab as part of a treatment plan for breakthrough bleeding. If you are on emicizumab and are taking or anticipate taking FEIBA for a breakthrough bleeding episode, tell your doctor immediately because there are specific safety considerations and you must be closely monitored by your hemophilia treater or treatment center.

Allergic reactions, including severe, sometimes fatal allergic reactions that can involve the whole body, can occur following the infusion of FEIBA. Stop using FEIBA promptly and call your doctor or get emergency treatment right away if you get a rash, hives or welts, experience itching, tightness of the throat, vomiting, abdominal pain, chest pain or tightness, difficulty breathing, lightheadedness, dizziness, nausea or fainting.

Because FEIBA is made from human plasma it may carry a risk of transmitting infectious agents, such as viruses, variant Creutzfeldt-Jakob disease (vCJD) and, theoretically, the Creutzfeldt-Jakob disease (CJD).

What are the possible side effects of FEIBA? The most common side effects observed during the prophylaxis clinical study were low number of red blood cells, diarrhea, bleeding into a joint, positive test for hepatitis B surface antibodies, nausea, and vomiting.

You should not use FEIBA if: • You had a previous severe allergic reaction to the product • You have Disseminated Intravascular Coagulation (DIC), or signs of small blood vessel clots throughout the body • You have sudden blood vessel clots or blocked blood vessels, (such as, heart attack or stroke)

The serious side effects seen with FEIBA are allergic reactions and clotting events involving blockage of blood vessels, which include stroke, blockage of the main blood vessel to the lung, and deep vein blood clots.

What other important information should I know about FEIBA?

Talk with your doctor about the possibility of formation of blood clots when taking drugs that may prevent clot breakdown such as tranexamic acid, and aminocaproic acid.

There have not been adequate studies of the use of FEIBA and rFVIIa (NovoSeven®), or emicizumab together, or one after the other. Use of drugs that may prevent clot breakdown within approximately 6 to 12 hours after the administration of FEIBA is not recommended.

Events involving blood clots blocking blood vessels (such as blood clot in vein, blood clot in the lung, heart attack, and stroke) can occur with FEIBA, particularly after receiving high doses (above 200 units per kg per day) and/or in patients with risk factors for clotting.

At first sign or symptom of a sudden blood vessel clot or blocked blood vessel (such as chest pain or pressure, shortness of breath, fever, altered consciousness, vision, or speech, limb or abdomen swelling and/or pain), stop FEIBA administration right away and seek immediate emergency medical treatment.

Infusion of FEIBA should not exceed a single dose of 100 units per kg body weight and daily doses of 200 units per kg of body weight. Maximum injection or infusion rate must not exceed 2 units per kg of body weight per minute.

Call your doctor right away about any side effects that bother you during or after you stop taking FEIBA. What other medications might interact with FEIBA?

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.

Please see next page for Important Facts about FEIBA, including BOXED WARNING on blood clots, and discuss with your doctor.

References: 1. Pergantou H, Matsinos G, Papadopoulos A, Platokouki H, Aronis S. Comparative study of validity of clinical, X-ray and magnetic resonance imaging scores in evaluation and management of haemophilic arthropathy in children. Haemophilia 2006;12(3):241-247. 2. Gringeri A, Ewenstein B, Reininger A. The burden of bleeding in haemophilia: is one bleed too many? Haemophilia. 2014;20(4):459-463. 3. FEIBA Prescribing Information. 4. Antunes SV, Tangada S, Stasyshyn O, et al. Randomized comparison of prophylaxis and on-demand regimens with FEIBA NF in the treatment of haemophilia A and B with inhibitors. Haemophilia. 2014;20(1):65-72.

Copyright © 2019 Takeda Pharmaceutical Company Limited. 300 Shire Way, Lexington, MA 02421. 1-800-828-2088. All rights reserved.

TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited.

FEIBA is a registered trademark of Baxalta Incorporated, a Takeda company.

S51823 09/19

Important Facts about FEIBA®: This leaflet summarizes important information about FEIBA. Please read it carefully before using this medicine. This information does not take the place of talking with your healthcare provider.

What is the most important information I should know about FEIBA?

What should I tell my healthcare provider before using FEIBA?

FEIBA can cause blood clots, including clots in the lungs, heart attack, or stroke, particularly after high doses of FEIBA (above 200 units per kg per day) or in people with a high risk of blood clots. Patients that have a risk of developing blood clots should discuss the risks and benefits of FEIBA with their healthcare provider since FEIBA may cause blood clots.

You should tell your healthcare provider if you • have or have had any medical problems • take any medicines, including prescription and non-prescription medicines, such as over-the-counter medicines, supplements or herbal remedies • have any allergies • are breastfeeding. It is not known if FEIBA passes into your milk and if it can harm your baby • are pregnant or planning to become pregnant. It is not known if FEIBA may harm your unborn baby.

The safety and efficacy of FEIBA for breakthrough bleeding in patients receiving emicizumab (Hemlibra®) has not been established. Events of thrombotic microangiopathy (TMA), a condition where blood clots and damage occur in small blood vessels, were reported in an emicizumab clinical trial where patients received FEIBA with emicizumab as part of a treatment regimen for breakthrough bleeding. If you take, or anticipate taking, FEIBA with emicizumab, tell your doctor, since you will need to be closely monitored by your hemophilia treating physician, preferably at the hemophilia treatment center (HTC). At first sign or symptom of a sudden blood vessel clot or blocked blood vessel (such as chest pain or pressure, shortness of breath, fever, altered consciousness, vision, or speech, limb or abdomen swelling and/or pain), stop FEIBA administration right away and seek immediate emergency medical treatment.

Allergic reactions, including severe, sometimes fatal allergic reactions that can involve the whole body, can occur following the infusion of FEIBA. The symptoms include rash, hives or welts, itching, tightness of the throat, vomiting, abdominal pain, chest pain or tightness, difficulty breathing, lightheadedness, dizziness, nausea, and fainting. Other infusion reactions, such as chills, fever, and high blood pressure have also been reported. If signs and symptoms of severe allergic reactions occur, stop using FEIBA immediately and call your doctor or get emergency treatment right away.

Because FEIBA is made from human plasma, it may carry the risk of transmitting infectious agents, for example, viruses, including Creutzfeldt-Jakob disease (CJD) agent, and the variant CJD agent.

Although steps have been taken to minimize the risk of virus transmission, there is still a potential risk of virus transmission.

FEIBA may interfere with some blood tests for red blood cell antibodies, such as the Coombs test.

What is FEIBA used for?

FEIBA is used for people with hemophilia A and B with inhibitors to control and prevent bleeding episodes, around surgery, or routinely to prevent or reduce the number of bleeding episodes (prophylaxis). It is NOT used to treat bleeding conditions without inhibitors to factor VIII or factor IX.

Who should not use FEIBA? You should not use FEIBA if you • are allergic to any ingredients in FEIBA, including factors of the kinin generating system • have a condition called disseminated intravascular coagulation (DIC), which is small blood clots in various organs throughout the body • currently have blood clots or are having a heart attack.

Make sure to talk to your healthcare provider about your medical history.

Tell your healthcare provider if you are pregnant or breastfeeding because FEIBA may not be right for you.

What are the possible side effects of FEIBA? The most frequent side effects of FEIBA are: low red blood cell count, diarrhea, bleeding into a joint, hepatitis B surface antibody positivity, nausea, and vomiting. The most serious side effects of FEIBA include: allergic reactions, including anaphylaxis, blood clot events, including those blocking blood flow to the brain (stroke), the lungs (pulmonary embolism), and the veins (deep vein thrombosis or DVT). Always immediately talk with your healthcare provider if you think you are experiencing a side effect. These are not all the side effects possible with FEIBA. You can ask your healthcare provider for information that is written for healthcare professionals.

What other medications might interact with FEIBA? Talk with your doctor about the possibility of formation of blood clots when taking drugs that may prevent clot breakdown such as tranexamic acid and aminocaproic acid. There have not been adequate studies of the use of FEIBA and rFVIIa (NovoSeven® RT), or emicizumab together, or one after the other. Use of drugs that may prevent clot breakdown within approximately 6 to 12 hours after the administration of FEIBA is not recommended. For additional information on potential drug interaction with emicizumab, see the “What is the most important information I should know about FEIBA?” section.

What else should I know about FEIBA? You should be trained on how to do infusions by your healthcare provider or HTC. Many people with hemophilia A or B with inhibitors learn to infuse their FEIBA by themselves or with the help of a family member.

Report any side effects or problems following FEIBA administration to your hemophilia treating physician right away.

Medicines are sometimes prescribed for purposes other than those listed here. Do not use FEIBA for a condition for which it is not prescribed. Do not share FEIBA with other people, even if they have the same symptoms that you have.

The risk information provided here is not comprehensive.

To learn more, talk about FEIBA with your healthcare provider or pharmacist. The FDA-approved product labeling can be found at http://www.feiba.com/us/forms/feiba_pi.pdf or by calling 1-877-825-3327.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088. ©2019 Takeda Pharmaceutical Company Limited. 300 Shire Way, Lexington MA 02421.

1-800-828-2088. All rights reserved.

TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited.

FEIBA is a registered trademark of Baxalta Incorporated, a Takeda company. All other trademarks appearing herein are the property of their respective owners. Issued: 12/2018 S47996 06/19

Connecting with other moms at the event was definitely the highlight of the weekend for me. Hemophilia can be such an isolating condition to live with and when you throw an inhibitor into the mix, it can feel even more isolating within an already isolated community. I feel fortunate because I do have a wonderful group of friends and tons of support outside of the hemophilia community. Yet, sometimes I still feel isolated. When we’re going through a lot of hemophilia related issues, it tends to be forefront on my mind. And if I don’t talk about it to the people who I spend my time with, I don’t feel authentic or as if I’m able to truly connect. And although I have friends who will listen and provide support, there’s a part of me that knows they will never truly understand what I’m experiencing.

That is not the case with hemophilia moms and especially other mamas of kiddos with inhibitors. I often feel an automatic connection, a knowingness that we truly get what it’s like to walk in the others’ shoes. I also often learn more from other members of the community than I do from medical professionals and this weekend was no exception.

Connecting with other mothers has been so incredibly healing for me and the MUM event was invaluable for that.

We had a “rap” session that was a bit of a free for all in terms of subject matter. We talked about the things that we felt were necessary to talk about. We talked about the biggest challenges that we face in raising a child with intense medical issues. We discussed new treatments and how we feel about them. We spoke about how we make decisions for our children and how we advocate for them.

We talked about how we manage (or don’t manage) the stress we face in our lives. We spoke about anything and everything related to mothering a child with an inhibitor and it was very satisfying and therapeutic.

After dinner on the first night, most women walked a few blocks into downtown Santa Fe and checked out the sights. There was live music and wine-tasting and art and antique cars and all sorts of interesting things to see and explore. But the best part of the night? And the best part of the entire event? Simply being with other mothers who know what it’s like to have the extra challenges of raising a child with hemophilia and inhibitors. Thank you CHES for allowing me this opportunity!

Supported by

For more info on MUM, visit ches.education/mum

6

n

LIFELINES for HEALTH

n

Fall/Winter 2019

Taking Time On

by Donovan Guerrero

A

ction. The willpower that it requires to take risks is vital to growing as a person. Sometimes I struggle with this. I think it is imperative for young adults like myself to do this to gain independence.

This past September I was blessed with an opportunity to apply for a program called LeverageSM. I had heard very little about this camp specially designed for adults with inhibitors. My mother actually filled out part of the application without my knowledge. I was shocked to get a call from Eric at CHES, never having met him, asking if I’m still interested in the program. I was unfamiliar with the two facilitating organizations, GutMonkey and CHES. Within three weeks of that phone call, I was packing my gear and preparing to go to Portland, Oregon.

The first lesson that I learned was the elephant-riderpath-theory (a concept that explains the conflicting struggles within all of us. Developed by the Heath brothers in a book called Switch, it’s the idea that everyone has a figurative elephant along with a rider inside him/herself. The elephant (relative to our emotions) is often controlled by the rider (our thought of logic.) But at times, our elephant (or emotions) can derail from its path by outside factors.

And at this point, it’s our rider’s responsibility to motive our elephant to return to its path.) This lesson resided with me the most. There was not one significant moment that I could point to as being a pivotal moment during this program. I think it was more of an accumulation of different challenges and tasks that I can focus on. To name a few in particular, would have to be the rock-climbing tower, building didgeridoos, and the high ropes course. I felt as though these were the biggest barriers I overcame. From the burning of my forearms on the tower, frustration of my didgeridoo cracking all over, and recovering from the splits on the stirrups obstacle on the high ropes course. My elephant wanted to give up – tell me to stop climbing, stop trying, stop caring. But the elephant-rider-path-theory really pushed me to succeed.

Personally, I think about when I do great things or go to amazing places, I always end up asking myself the same question when I return home: “What’s next?” For example, I like setting goals. One goal I made a few months prior was to get my boating license so I can operate a boat that has 25 horsepower or more. I’ve almost completed the course currently and hoping to pass the test soon after. My current In collaboration with:

boat does not require a boating license since it only has 7.5 horsepower. I did it to learn the “rules of the road” so to speak and so I’m already ahead of the game when I get a big boat in the future. When the time comes, I’m sure I will evaluate my decision and think to myself, “Yay, you passed the test!” Then when I buy a nice boat, I will think, “Well,

now I guess I should take a fishing trip to Alaska for salmon, or Florida for smallmouth bass, or even the Great Lakes for perch and crappie.”

It’s that drive that I have to learn and share. For me, I want to keep setting goals and I don’t want to meet them, I want to surpass them. To go above and beyond. Like the difference from getting an “A” or a “B” on a test. I’m at a point in my life where I graduated high school and I’m not attending college this year. I want to go next year but I wanted to take time off and become a more mature person, learn more on my own and gain independence, and work to save up for college. All of these facets would contribute to working on my mental health as well. What I really want to do is take the remainder of the year head on. Take a “gap year” on. Leverage definitely gave me the support and the confidence to do that. I have big plans ahead of me. Taking action to put them in motion is the crux of the issue. I want to be a leader in some fashion.

Thank you to Takeda pharmaceuticals for making this event possible. Thank you to CHES and GutMonkey for putting on a great Leverage 2019.

Supported by:

For more info on Leverage, visit ches.education/leverage

COMMUNITY CHATTER

Camp Fosters

Sho c k

SM

by Stephanie Shea

inhibitor camp

Healing, Friendship, & Play

I

n June, we had the opportunity to attend CHES' After the ShockSM inhibitor camp. It was a hard decision for my family as it happened to fall on the same weekend as our local chapter’s annual camp. Although my 5-year-old son Dakoda’s inhibitor isn’t “active”, we decided to go to After the Shock as we felt we might relate more with this community. And I’m so glad we did.

tennis court, watching Dakoda and his little brother Phoenix “play” tennis. We started talking with a father who we’ve met before at other events. Dakoda and Phoenix wanted to run to the upper fields to play soccer with their camp buddy.

The other

One of the biggest blessings that we have found on our hemophilia journey has been the community....and the love and support we get from them.

At times, we have felt isolated among the “normal” hemophilia population. Not all, but many that I’ve connected with, seem to infuse factor prophylactically a couple to a few times a week with no issues and they have been fortunate enough not to experience any major traumas.

Those of us that have experienced inhibitors know that it’s an entirely different ballgame for us. Although Dakoda’s inhibitor has been considered partially tolerized for almost 2 years, his half-life is very short, so he still needs daily infusions, aka, needle pokes. Daily infusions can definitely take their toll on a family, not to mention the task of emotionally healing from past traumas (head bleeds, prolonged bleeding from ports, joint bleeds, etc.). Most families with inhibitors know this path all too well.

On the first day of camp, we learned that every family was assigned a camp “buddy” who would stay with the kids for most of the day, even while parents went to educational classes. On the first day, my husband and I were on the

8

n

LIFELINES for HEALTH

n

Fall/Winter 2019

father we were talking with could clearly see the anxiety on our faces (as we have very rarely let Dakoda be with anyone other than us or one or two very trusted family members). The father of the boy was several years older than Dakoda and he shared his own experiences with anxiety. He encouraged us to let go and trust that Dakoda would be ok without us. During the conversation, my husband and I both teared up and were greatly touched. It set the stage for us to loosen our grip for the rest of the weekend and it felt like a turning point in both of our personal healing journeys.

Discover more about IXINITY

®

Visit IXINITY.com

Aptevo BioTherapeutics LLC, Berwyn, PA 19312 IXINITY® [coagulation factor IX (recombinant)] and any and all Aptevo BioTherapeutics LLC brand, product, service and feature names, logos, and slogans are trademarks or registered trademarks of Aptevo BioTherapeutics LLC in the United States and/or other countries. © 2018 Aptevo BioTherapeutics LLC.

All rights reserved.

Everyone in the inhibitor community seems to just “get it”.

They get that we’ve all seen our children in pain, we’ve all seen them suffer and we’ve all been through a lot of trauma, which often results in fear and anxiety.

Aside from the immense emotional value we received from talking and sharing with other parents, the camp itself was incredible. Set on a lake in the

CM-FIX-0258

Pocono mountains in Pennsylvania, the scenery of Camp Zeke was breathtaking. We were surrounded by lush green trees and grass and it was so nice to be immersed in nature.

We have attended many national and local events in the hemophilia and inhibitor community, and they have all taken place indoors, mainly in small windowless classrooms.

The difference at After the Shock was staggering. The kids were able to be how kids are meant to be - running carefree and playing in nature. Even when the parents were in educational sessions or talks, the rooms had huge windows overlooking the lake and giant trees, giving us the sense that we were in a forest.

There's so much to do at Camp Zeke, that there was never a dull moment. There was the lake, of course and all the activities that came with it (kayaking, fishing, swimming) with an outdoor pool, nature trails, a full theater, art studios, and so much more!

The educational sessions were great too! In addition to Current Treatment of Inhibitors and separate “rap sessions” for the Moms and Dads, there was also Aqua Therapy and Yoga for Relaxation. My personal favorite though was The Bio-psychosocial Effects of a Chronic Condition. The speaker was knowledgeable about trauma as well as the

COMMUNITY CHATTER

intensity and daily challenges that come with managing a chronic condition. I feel that both my husband and I greatly benefited from this session. We took home tools and techniques that have enhanced our lives, especially in times of stress and anxiety.

When recalling the weekend, we spent at After the Shock, there was a special incident that stood out for me. Dakoda wanted to play basketball, but a group of older kids were in the middle of a game on the court. Dakoda just hopped on and started “playing”. Instead of having him move off the court and wait for a turn like many kids their age would, they slowed down the pace of their game and included him.

When I witnessed this, my heart expanded, and I teared up again. I know that most kids with hemophilia and inhibitors have probably been excluded at some point in their lives and instead of doing the same thing to a younger kiddo, they made the point to include him (despite it diminishing the speed and flow of their game!) Every child at camp seemed to exhibit such kindness and compassion and it made me so grateful to be a part of this community.

The final night was special, with a talent show and a wooden wish boat launch to close out the weekend. My boys have already asked me when we can go back to the camp in Pennsylvania. And they’re not the only ones, I can’t wait until next year!

Supported in part by: DIAMOND

SILVER KEDRION Biopharma

10

n

LIFELINES for HEALTH

n

Fall/Winter 2019

by Kenneth Martin

The

M

any, and I do mean many, years ago I would go to the original Camp Bold Eagle and events through the local chapter, and my brother and I would be the lone people with an Inhibitor. There was talk about new treatments and I would ask, “Does it work for inhibitors?”, and the answer was always the same - “No.” Not until the early 90’s was there specific treatment for inhibitor patients. Even then the effectiveness of those treatments for me were barely measurable. I participated in trials and studies and again, the results did not include an opportunity to change things for myself.

Old Guard

there before me to rely on anymore, but there are a few who are in similar situations in their life like myself. Programs like Momentum help me, help others and provide an opportunity for me to receive what I might need at a given time. Whether it be new ways of doing things, new ways of keeping myself healthy, both physically and mentally, this program teaches, helps, and nurtures my well-being to keep me moving forward in my life.

I was formally diagnosed with an inhibitor at age 5, but it was Dr. Jean Lusher’s opinion that I had always had an inhibitor to Factor VIII. I had been dealing with, living with, succeeding with (to an extent), my inhibitor for years.

I was married with two boys, working full time and had a life outside of hemophilia; even with having over 40 bleeds a year.

Due to the dark ages in hemophilia in the late 1980s and 1990s, I retreated from the community to concentrate on my family which was fine by me because there wasn’t anything available for me or my specific condition for nearly twenty years. Then new products, new treatment regimens for people without From right to left, top to bottom: Matt Compton, Gage Louviaux, Harp Maan, Ken Martin, Eric Lowe, Austin Mireles, Benjamin inhibitors became the norm in the Denman, Tom Sitzler, Gabe Anspaugh, and Chris Maddix community. Mysteriously, the number of inhibitor patients increased. So much so that in 2005, there was an actual program created Supported by JUST FOR INHIBITOR PATIENTS! My wife and I attended in 2006 and met a lot of other patients who were dealing with being newly diagnosed with an inhibitor and how it was changing their life. I told them that I had never remembered not having an inhibitor.

From that early program, other resources have evolved.

CHES is one of them. It allows for adults to interact and relate their experiences, troubles, triumphs to each other and most importantly… just listen. I actually cherish these new opportunities to meet and become friends with others who are going through the same things that I am going through. Unfortunately, there aren’t many that have been

COMMUNITY CHATTER

Biopsychosocial Aspe Chronic Illness H

aving a chronic medical diagnosis, can be extremely challenging. Along with those challenges, caregivers and those managing the diagnosis often face periods of isolation, depression, and anxiety.

Because of this fact, taking care of your mental health becomes as essential, as taking care of your physical health.

Mental health encompasses many things including emotional, psychological, and social well-being. Your mental health will affect how you handle stress, relate to others and make choices. Mental health may have a biological factor or be caused by life experiences including trauma and abuse. As we explore the literature surrounding chronic conditions and mental health, namely depression and anxiety, we see a substantial cause and effect scenario. Some research suggests that due to constant issues with managing pain, the cost, as well as the stress and worry of managing the condition causes mental health complications. Other research asserts that there is an increased likelihood for a chronic condition to develop and manifest, if a person is diagnosed with mental health concerns as a result of not taking care of one’s medical needs including non-compliance with medication, attending doctor’s appointments and following medical recommendations.

Living with a chronic condition can become all consuming. Taking the time to manage multiple appointments, infusion regimens (often including their own, their children or loved ones), managing the pain and subsequent doctor’s appointments involves many hours of planning and juggling. Because of this fact, paying attention to the signs of depression and anxiety often take a back burner to day-to-day responsibilities, leaving some undiagnosed and most definitely untreated.

12

n

LIFELINES for HEALTH

n

Fall/Winter 2019

By: Anna Maria Bell, LICSW, LCSW-C, LISW

ects of

FEATURE

With the genetic component of some chronic conditions, fear and dread is often fueled by familial examples of family members living, coping and managing it. If these “examples” have not been positive in nature or have resulted in failing health, issues with pain management, increase in bleeding episodes, multiple hospitalizations, multiple surgeries, and an overall decrease in quality of life, then the perspective on living with the condition can cause fear and dread leading to anxiety symptoms.

Take for example the chronic condition of hemophilia.

Because of the genetic predisposition for it, caregivers have little to no adjustment time before their role as caregiver begins. (According to Schwartz, Powell & Eldar-Lissai, 2017), since people are born with hemophilia, family members are initiated into the caregiving role as soon as the family becomes aware of the diagnosis. Because of this, caregivers need to equip themselves to be able to manage multiple comorbidities, while also taking care of themselves mentally because caregiving is physically, emotionally, and financially demanding. (Miravitlles, Pena- Longobardo, Oliva-Moreno & Hidalgo-Vega, 2015).

Depression and Anxiety Depression and anxiety present themselves in different ways. Coping with a disorder causes the person to have thought processes that, if gone untreated, can lead to mental health complications. Some of those thought processes may include: •

What did I do to deserve this (why me?)

•

I am not equipped enough to handle this

•

I feel unfulfilled due to this diagnosis

•

My body is flawed

•

Why is my life so complicated?

Without proper coping mechanisms these thoughts can manifest into the symptomatology of depression and anxiety.

Anxiety is the way people respond to certain objects, stimuli or situations. These responses often involve feeling dread and fear. With a chronic condition, this fear and dread can often be centered around effect management, including quality of life issues as well as life expectancy.

14

n

LIFELINES for HEALTH

n

Fall/Winter 2019

Depression is the persistent feeling of sadness or loss. It often manifests itself with a wide range of behavioral and physical symptoms including thoughts of suicide. These symptoms can be broken up into several categories including mood, behavioral, sleep, physical, and cognitive symptoms.

The following provides a list of symptomatology: Mood: anxiety, apathy, general discontent, guilt, hopelessness, loss of interest or pleasure in activities, mood swings, or sadness Behavioral: agitation, excessive crying, irritability, restlessness, or social isolation Sleep: early awakening, excess sleepiness, insomnia, or restless sleep Physical body: excessive hunger, fatigue, loss of appetite, weight gain or weight loss Cognitive: lack of concentration, slowness in activity, repeatedly going over thoughts or thoughts of suicide

In persons managing a chronic condition, the feelings of loss of control over their own body, along with physical complications from managing it can often result in feelings of isolation, agitation, apathy, hopelessness thereby presenting as depression. As the focus of managing the disorder takes precedence, it often prevents the affected individual or their caregivers, from seeking out treatment, for depression and anxiety.

A team led by researchers at the Munson Medical Center, and supported by Pfizer, conducted a survey to better understand the incidence and impact of anxiety and depression among hemophilia patients. The study recruited 200 participants, of whom 80.5% had hemophilia type A and 19.5% had hemophilia type B, between 2013 and 2014 at national or state conferences for people with hemophilia in the United States. Among the participants, 68% had hemophilia classified as severe. The survey revealed that 186 (93%), of the participants experienced symptoms consistent with depression, and 184 (92%), had anxiety.

Among these persons, 52 (28%), reported moderate-tosevere depression, while 23 (14%) had moderate-to-severe anxiety.

What is most significant about this study is that more than half of the respondents indicated no history of “diagnosed” depression. This adds to the argument that this population, although exhibiting symptoms, often moderate to severe, are still going undiagnosed and thereby untreated. With this knowledge the researchers suggest that patients and caregivers managing chronic conditions, in this case Hemophilia, should undergo routine screenings at their HTC’s or doctor’s appointments. Most HTC’s have social worker’s on staff who are available and licensed to complete these assessments. As medical models are expanding their focus, including the “whole person” philosophy of mind/body wellness, hopefully the stigma surrounding mental health diagnosis can be removed and these screenings will become common practice.

Experienced Consistent Symptoms Depression/Anxiety (out of entire study group of 200 individuals) Depression - 93%

Depression

Anxiety

Anxiety - 92%

Reported Moderate-to-Severe Depression/Anxiety (out of those who experienced symptoms as indicated above) Depression - 28%

Depression

Anxiety

Anxiety - 14%

FEATURE

Complications of Pain Management More than 25 million U.S. adults have some level of daily pain, and 10.5 million have considerable pain every day. Managing hemophilia often involves treating pain by utilizing medication. Often these medications are prescribed as a daily course of treatment. For more than 25 years, opioids for the treatment of acute pain have been strongly encouraged, in the past 2 decades, the treatment of chronic pain using long-term opioid therapy has become more common. Although prescribed with intentions of increased quality of life and freedom from constant pain, this regimen of pain management has resulted in unintended consequences. The dependency on these medications have caused addiction rates in this population to skyrocket as well as all the complicating factors associated with addiction. These include financial hardships, family conflicts, an increase in rates of depression and sadly an increase in suicidal rates.

The correlation between addiction and mental health has been researched and proven over the years. As the communities of people who suffer from chronic conditions battle to find ways to live a “normal” life as pain free as

16

n

LIFELINES for HEALTH

n

Fall/Winter 2019

possible, it is important to closely monitor dependency and to also seek out treatment from certified addiction counselors who help to develop alternative methods and strategies to managing pain, free from addiction. Some emotional/behavioral symptoms of drug dependency include: •

Loss of control over the amount and frequency of use

•

Craving and compulsive using

•

Continued use in the face of adverse consequences

•

Missing work/school

•

Isolating/secretive about activities

•

Relationship/marital problems

•

Irritability/argumentative

•

Defensiveness

•

Inability to deal with stress

•

Confusion

•

Blaming others for their problems

Survivor’s Guilt Survivor guilt is a mental condition that occurs when a person believes they have done something wrong by surviving a traumatic event when others did not, often feeling self-guilt. In the hemophilia community, survivor’s guilt is often not spoken about. At most national conferences they honor those who have passed on from complications associated with hemophilia, at “Remembrance Services”. These services are well attended, full of amazing and beautiful stories of love, sacrifice, and ultimate loss of loved ones.

Some of the stories are about fathers, sons, moms, brothers and sisters. Then there are those stories of 5 uncles and a dad, 6 siblings and a father, whole generations of families who have fought the valiant fight and lost the battle with disease. Then you hear the remaining family discuss,

“why not me, when all my brothers died?” or “why not me, when I lost my dad and all my uncles?” These are poignant examples of survivor’s guilt. This guilt adds to the symptoms of depression and anxiety. Often resulting in self-loathing, increased sadness and feelings of isolation.

As those suffering with chronic conditions deal with this survivor’s guilt, the desire to attach and form relationships often is compromised due to the feeling of inevitable loss or that they may be next so why get close to anyone. Survivor’s guilt may also induce non-compliance with treatment regimens with a subconscious desire to “be with the lost loved one” or “I am going to be next so why not speed up the process”. These feelings may appear extreme but are realistic to the person who is experiencing them. Survivor’s often have both depression (sadness, apathy) while also experiencing anxiety (fear and dread), at the same time. As a survivor who is living with a chronic disorder, managing these feelings through therapeutic intervention is paramount.

Suicide Suffering debilitating pain every day may be so unbearable that some decide to take their own lives. In a study, led by Dr. Emiko Petrosky from the U.S. National Center for Injury Prevention and Control, in 8.8 percent of suicides in 2014, there was some type of history of chronic pain. This was a significant increase from previous studies in 2003 which only indicated a 4.7 percent history of chronic pain. Among suicide victims with chronic pain where toxicology results were available, opioids were much more likely to be present at the time of death than in those without pain. In addition, if a suicide note was present, more than two-thirds mentioned a pain condition as well as long suffering from the pain as a direct contributor to the suicide crises.

These startling statistics highlight the importance of mental health intervention for anyone attempting to manage a chronic condition. It also highlights the need to improve pain treatment, not only for the direct effect on management but also as a “method to raise hope” in persons coping with chronic pain due to a chronic condition. For those who may have or have had thoughts of suicide, it is imperative that you seek out intervention from someone who is licensed to provide services in crises situations. Some of the warning signs or things to look out for include: •

Inability to perform daily tasks like bathing, brushing teeth, brushing hair, changing clothes

•

Rapid mood swings, increased energy level, inability to stay still, pacing

•

Suddenly depressed, withdrawn; suddenly happy or calm after period of depression

•

Increased agitation, verbal threats, violent, out-of-control behavior, destroys property

•

Isolation from school, work, family, friends

•

Paranoia

•

Increased or new substance usage

•

Constant sleeping or Insomnia

FEATURE

18

n

LIFELINES for HEALTH

n

Fall/Winter 2019

Common warning signs of suicide include: •

Giving away personal possessions

•

Talking as if they’re saying goodbye or going away forever

•

Taking steps to tie up loose ends, like organizing personal papers or paying off debts

•

Stockpiling pills or obtaining a weapon

•

Preoccupation with death

•

Sudden cheerfulness or calm after a period of despondency

What to do if someone is in crisis/at risk of suicide: If safe, keep them talking. Ask things that require more than a yes and no response like: •

Tell me what emotions you are feeling?

•

What happened to make you feel this way?

•

What can I do to help you right now?

•

“Let’s go get something to eat or spend the day together.”

(Diverting their attention is extremely beneficial.)

Remember, asking questions without judgement is essential.

Here are some things to ask: • •

•

Ask them if they have a therapist or counselor they would like to reach out to.

Tell them you would be happy to assist them to get to the hospital or crisis clinic and are willing to stay during the assessment process (this is a scary step for some people so having someone there with them is extremely beneficial) Ask them if they are thinking about completing suicide.

An example of what to say could be: “Based on what you’ve shared with me, I’m wondering if you’re thinking about suicide?” If they answer yes, find out more details and ask them if they have a plan such as, “Do you have a plan for how you would do it?”

What can I do if I recognize my Family or Friends have Mental Health Challenges?

•

Remember only 44% of adults with diagnosable mental health problems and less than 20% of children and adolescents receive needed treatment.

Do something!!

•

Friends and family can be important influences to help someone get the treatment and services they need.

•

Treat people with mental health diagnoses with respect, compassion, and empathy

•

Don’t define them by their diagnosis or use labels such as "crazy"

•

Express your concern and support

•

Remind your friend or family member that help is available and that mental health problems can be treated

•

Ask questions, listen to ideas, and be responsive when the topic of mental health comes up

How to start a conversation with Adults about Mental Health •

Can you tell me more about what is happening?

How you are feeling?

•

Can we talk about what you are experiencing? If not, who are you comfortable talking to?

•

Have you had feelings like this in the past or are these new feelings? When did you notice the change?

•

I’m here to listen. How can I help you feel better?

•

I’m worried about your safety. Can you tell me if you have thoughts about harming yourself or others?

How to start a conversation with children or adolescents about Mental Health •

Communicate in a straightforward manner. Don’t beat around the bush.

•

Speak at a level that is appropriate to a child or adolescent’s age and developmental level. The developmental level is the most important.

•

Discuss the topic when your child feels safe and comfortable

•

Watch for reactions during the discussion and slow down or back up if your child becomes confused or looks upset (nonverbal cues are important)

•

Listen openly and let your child tell you about his or her feelings and worries

Never say things like: •

“We all go through tough times like these. You’ll be fine.”

•

“It’s all in your head. Just snap out of it.”

•

“You think you have problems, well look what happened to me.”

•

“Get some sleep and things will look differently tomorrow.”

•

“Grow up and stop feeling sorry for yourself.”

FEATURE

Treatment Options

References

Seeking treatment through therapeutic intervention should not be an after-thought, but viewed as a holistic approach to wellness. It should not be a surprise or something you, “just get to when I have time”, especially if you are living with a chronic condition. Developing coping strategies and having a safe and non-judgmental place to share the emotions, the loss, the sorrows of managing and having a chronic condition, is extremely beneficial.

It is important to remember that therapy is not a “one size fits all” but instead in this day-and-time, there are many different modalities and options for treatment that are available for you to consider. If the idea of “talk” therapy does not appeal to you, why not try art therapy or music therapy? The most important thing to remember is that seeking treatment should not be stigmatized but instead viewed as a way to live a healthier, more productive, and fulfilled life. Here are some things to consider when seeking therapy:

Miravitlles, M., Pena-Longobardo, L., Oliva-Moreno, J.& Hidalgo-Vega, A. (2015).

Caregivers’ burden in patients with COPD. International Journal of Chronic Obstructive Pulmonary Disease. 10. 347-356. doi: 10.2147/COPD.S76091

•

Therapy is not a “one size fits all”.

•

If one therapist just doesn’t feel right and you are unable to develop a trusting relationship, don’t be discouraged, try someone else.

•

If Traditional “talk” Therapy is not comfortable there are plenty of therapy modalities. Try as many as necessary until you find the one that “fits” you and helps you with your concerns.

•

Choosing a therapy modality can be difficult but so worth it. Finding a therapist that specializes in a particular area (i.e. play therapy, art therapy, dance therapy, telephone/ video health) can really lead to great success and make a huge difference when seeking help.

Pertrosky, E., Webb, R., Kontopantelis, E., Doran, T., Qin, P., Creed, F., & Kapur, N. (2014).

Suicide Risk in Primary Care Patients with Major Physical Disease. Archives of General Psychiatry 69(3). 256-264.

Schwartz, C., Powell, V., & Eldar-Lissai, A. (2017). Measuring Hemophilia Caregiver Burden: Validation of the Hemophilia Caregiver Impact Measure. Quality of Life Research. 26. 2551-2562.

DOI: 10.1007/s11136-017-1572-y.

Witkop, M., Lambing, A., Nichols, C., Munn, J., Anderson T., & Tortella, B. (2019).

Interrelationship Between Depression, Anxiety, Pain, and Treatment Adherence in Hemophilia: Results from a US Cross-sectional Survey. Patients Preferences and Adherence. 13.1577-1587.

Doi: 10.2147/PPA.S212723.

The most important thing to remember is that seeking help is beneficial. There is no shame in reaching out to a professional. Managing a chronic condition is extremely difficult but with the right coping strategies and support network, you can live, thrive, and have a fulfilled life.

Anna Maria Bell is a graduate of The Catholic University of America Master’s Program in Social Work with a concentration in Gerontology and a graduate of Capella University with a Master’s Degree in Public Service Leadership. She is currently ABD at Walden University where she is pursuing a PhD in Human Services. She is Clinically Licensed to Practice in the District of Columbia, Maryland, Virginia and the State of Ohio. She provides counseling to various populations including couples, children, adolescents, women, and geriatric populations. Anna joined the Hemophilia Foundation of America consultancy staff in 2015 as a facilitator/speaker, conducting trainings, seminars and facilitations. Anna conducts various workshops throughout the Washington DC Metropolitan Area.

20

n

LIFELINES for HEALTH

n

Fall/Winter 2019

FEATURE

ips

Krystyn Strother is the former program director at HUSH Meditation, strategic designer/author of the HUSH meditation curriculum, is a certified meditation instructor, co-founder of NOMAD, “Adventures in Wellness”, and yoga instructor. Krystyn’s yoga classes range from Vinyasa to Yin. In addition to her regularly scheduled classes, Krystyn guest teaches at several yoga teacher training programs throughout the country, speaks at conferences on mindfulness and stress reduction practices, teaches specialized workshops, facilitates yoga + adventure retreats, and conducts continuing education classes for currently registered RYTs. Krystyn holds a certificate of completion in the Yoga of Awareness For Chronic Pain, an evidence-based program sponsored by the Department of Anesthesiology at OHSU. Read more about Krystyn at krystynstrother.com

21

n

LIFELINES for HEALTH

n

Fall/Winter 2019

MIND BODY CONNECTION

Photo courtesy of krystynstrother.com and Stark Photography

Mindfulness 0 1

by Krystyn Strother

REAL LIFE. REAL BLEED PROTECTION.*

AdynovateRealLife.com

HEMOPHILIA A IS A PIECE OF YOU. NOT ALL OF YOU. ADYNOVATE® is a treatment that can be personalized to fit your lifestyle so you have more time to spend doing the other things that also make you, you.

It has a simple, twice-weekly dosing schedule on the same 2 days every week.1,2

*In clinical trials, ADYNOVATE demonstrated the ability to help prevent bleeding episodes using a prophylaxis regimen.

No actual patients depicted.

ADYNOVATE twice-weekly prophylaxis prevented or reduced the number of bleeds2 ADYNOVATE was proven in 2 pivotal clinical trials to prevent or reduce the number of bleeding episodes in children and adults when used regularly (prophylaxis)2 • Children Under 12 Years: This study evaluated the efficacy of ADYNOVATE twice-weekly prophylaxis and determined the ability to treat bleeding episodes for 6 months in 66 children under 12 years old who received 40–60 IU/kg of ADYNOVATE prophylaxis treatment2 – During the 6-month study in children under 12, those receiving twice-weekly prophylaxis treatment experienced a median† overall ABR‡ of 2.0 – 0 bleeds in 38% (25 out of 66 patients) during 6 months on twice-weekly prophylaxis Median is defined as the middle number in a list of numbers arranged in numerical order.

ABR=annualized bleed rate, the number of bleeds that occur over a year.

Per-protocol patients were assigned to the prophylactic group and treated with their originally assigned dose for the entire duration of the study.

†

• Adolescents and Adults 12 Years and Older: This study evaluated the efficacy of ADYNOVATE in a 6-month study that compared the efficacy of a twice-weekly prophylactic regimen with on-demand treatment and determined hemostatic efficacy in the treatment of bleeding episodes in 137 patients. These adolescents and adults were given either ADYNOVATE prophylaxis twice-weekly at a dose of 40–50 IU/kg (120 patients) or on-demand treatment with ADYNOVATE at a dose of 10–60 IU/kg (17 patients). The primary study goal was to compare ABR between the prophylaxis and on-demand treatment groups2 – 95% reduction in median overall ABR (41.5 median ABR with on-demand [17 patients] vs 1.9 median ABR with prophylaxis [120 patients]) – 0 bleeds in 40% (40 out of 101 per-protocol§ patients) during 6 months on twice-weekly prophylaxis

‡ §

ADYNOVATE Important Information What is ADYNOVATE? • ADYNOVATE is an injectable medicine that is used to help treat and control bleeding in children and adults with hemophilia A (congenital factor VIII deficiency).

• Your healthcare provider (HCP) may give you ADYNOVATE when you have surgery.

• ADYNOVATE can reduce the number of bleeding episodes when used regularly (prophylaxis).

ADYNOVATE is not used to treat von Willebrand disease.

DETAILED IMPORTANT RISK INFORMATION Who should not use ADYNOVATE? Do not use ADYNOVATE if you: • Are allergic to mouse or hamster protein.

• Are allergic to any ingredients in ADYNOVATE or ADVATE® [Antihemophilic Factor (Recombinant)].

Tell your HCP if you are pregnant or breastfeeding because ADYNOVATE may not be right for you.

What should I tell my HCP before using ADYNOVATE? Tell your HCP if you: • Have or have had any medical problems.

• Take any medicines, including prescription and non-prescription medicines, such as over-the-counter medicines, supplements or herbal remedies.

• Have any allergies, including allergies to mice or hamsters.

• Are breastfeeding. It is not known if ADYNOVATE passes into your milk and if it can harm your baby.

• Are or become pregnant. It is not known if ADYNOVATE may harm your unborn baby.

• Have been told that you have inhibitors to factor VIII (because ADYNOVATE may not work for you).

What important information do I need to know about ADYNOVATE? • You can have an allergic reaction to ADYNOVATE. Call your healthcare provider right away and stop treatment if you get a rash or hives, itching, tightness of the throat, chest pain or tightness, difficulty breathing, lightheadedness, dizziness, nausea or fainting.

• Do not attempt to infuse yourself with ADYNOVATE unless you have been taught by your HCP or hemophilia center.

What else should I know about ADYNOVATE and Hemophilia A? • Your body may form inhibitors to factor VIII. An inhibitor is part of the body’s normal defense system. If you form inhibitors, it may stop ADYNOVATE from working properly.

Talk with your HCP to make sure you are carefully monitored with blood tests for the development of inhibitors to factor VIII.

What are possible side effects of ADYNOVATE? • The common side effects of ADYNOVATE are headache and nausea. These are not all the possible side effects with ADYNOVATE. Tell your HCP about any side effects that bother you or do not go away.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.

Please see Important Facts about ADYNOVATE on the following page and discuss with your HCP. For Full Prescribing Information, visit www.adynovate.com.

References: 1. Valentino LA. Considerations in individualizing prophylaxis in patients with haemophilia A. Haemophilia. 2014;20(5):607-615. 2. ADYNOVATE Prescribing Information.

Copyright © 2019 Takeda Pharmaceutical Company Limited. 300 Shire Way, Lexington, MA 02421. 1-800-828-2088.

All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited. ADVATE and ADYNOVATE are registered trademarks of Baxalta Incorporated, a Takeda company.

S50751 08/19

How should I use ADYNOVATE? (cont’d) Call your healthcare provider right away if your bleeding does not stop after taking ADYNOVATE.

What should I tell my healthcare provider before I use ADYNOVATE?

Patient Important facts about

You should tell your healthcare provider if you:

ADYNOVATE [Antihemophilic Factor (Recombinant), PEGylated]

• Have or have had any medical problems.

This leaflet summarizes important information about ADYNOVATE. Please read it carefully before using this medicine. This information does not take the place of talking with your healthcare provider, and it does not include all of the important information about ADYNOVATE. If you have any questions after reading this, ask your healthcare provider.

• Have any allergies, including allergies to mice or hamsters.

®

What is the most important information I need to know about ADYNOVATE? Do not attempt to do an infusion to yourself unless you have been taught how by your healthcare provider or hemophilia center.

You must carefully follow your healthcare provider’s instructions regarding the dose and schedule for infusing ADYNOVATE so that your treatment will work best for you.

What is ADYNOVATE? ADYNOVATE is an injectable medicine that is used to help treat and control bleeding in children and adults with hemophilia A (congenital Factor VIII deficiency). Your healthcare provider may give you ADYNOVATE when you have surgery. ADYNOVATE can reduce the number of bleeding episodes when used regularly (prophylaxis).

ADYNOVATE is not used to treat von Willebrand disease.

Who should not use ADYNOVATE? You should not use ADYNOVATE if you: • Are allergic to mice or hamster protein • Are allergic to any ingredients in ADYNOVATE or ADVATE® [Antihemophilic Factor (Recombinant)] Tell your healthcare provider if you are pregnant or breastfeeding because ADYNOVATE may not be right for you.

How should I use ADYNOVATE? ADYNOVATE is given directly into the bloodstream.

You may infuse ADYNOVATE at a hemophilia treatment center, at your healthcare provider’s office or in your home. You should be trained on how to do infusions by your healthcare provider or hemophilia treatment center. Many people with hemophilia A learn to infuse their ADYNOVATE by themselves or with the help of a family member.

Your healthcare provider will tell you how much ADYNOVATE to use based on your individual weight, level of physical activity, the severity of your hemophilia A, and where you are bleeding.

Reconstituted product (after mixing dry product with wet diluent) must be used within 3 hours and cannot be stored or refrigerated. Discard any ADYNOVATE left in the vial at the end of your infusion as directed by your healthcare professional.

You may have to have blood tests done after getting ADYNOVATE to be sure that your blood level of factor VIII is high enough to clot your blood.

• Take any medicines, including prescription and non-prescription medicines, such as over-the-counter medicines, supplements or herbal remedies.

• Are breastfeeding. It is not known if ADYNOVATE passes into your milk and if it can harm your baby.

• Are pregnant or planning to become pregnant. It is not known if ADYNOVATE may harm your unborn baby.

• Have been told that you have inhibitors to factor VIII (because ADYNOVATE may not work for you).

What are the possible side effects of ADYNOVATE? You can have an allergic reaction to ADYNOVATE. Call your healthcare provider right away and stop treatment if you get a rash or hives, itching, tightness of the throat, chest pain or tightness, difficulty breathing, lightheadedness, dizziness, nausea or fainting.

The common side effects of ADYNOVATE are headache and nausea. Tell your healthcare provider about any side effects that bother you or do not go away.

These are not all the possible side effects with ADYNOVATE. You can ask your healthcare provider for information that is written for healthcare professionals.

What else should I know about ADYNOVATE and Hemophilia A? Your body may form inhibitors to Factor VIII. An inhibitor is part of the body’s normal defense system. If you form inhibitors, it may stop ADYNOVATE from working properly.

Consult with your healthcare provider to make sure you are carefully monitored with blood tests for the development of inhibitors to Factor VIII. Medicines are sometimes prescribed for purposes other than those listed here. Do not use ADYNOVATE for a condition for which it is not prescribed. Do not share ADYNOVATE with other people, even if they have the same symptoms that you have.

The risk information provided here is not comprehensive.

To learn more, talk with your health care provider or pharmacist about ADYNOVATE. The FDA-approved product labeling can be found at www.ADYNOVATE.com or 1-877-825-3327.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088. ©2018 Shire US, Inc., Lexington, MA, 02421. All rights reserved. 1-800-828-2088.

BAXALTA®, ADVATE®, and ADYNOVATE® are trademarks of Baxalta Incorporated, a wholly owned, indirect subsidiary of Shire plc.

Patented: see https://www.shire.com/legal-notice/product-patents Baxalta US Inc. Lexington, MA 02421 USA U.S. License No. 2020 Issued 05/2018 S47033

24

n

LIFELINES for HEALTH

n

Fall/Winter 2019

YOUR JOINT HEALTH IS IMPORTANT EVERY JOINT BLEED MATTERS 70 to 80% of all bleeds in hemophilia occur in the joints.1 If not treated promptly, even a single bleed can lead to permanent joint or muscle damage.2 This can be true even for people with mild hemophilia.2

IS IT A BLEED?3 During the early stages of a bleed, you may feel a tingling sensation in the joint. It may feel warm, or the skin over the joint may feel warmer than skin nearby.

After a short time, the joint may swell up and become stiff or painful. However, you may not see a bruise with a joint bleed.

What’s important is to go by what you feel, not necessarily what you see.

Blood during a joint bleed, which may lead to permanent damage

SCAN TO TAKE A CLOSER LOOK INSIDE A JOINT

WHAT’S NEW?

PREVENT JOINT DISEASE BEFORE IT HAPPENS The most effective way to take care of your joints and protect them from future damage remains holding to your prescribed treatment routine.4 For decades, prophylaxis with factor has been the most widely used treatment for hemophilia, with a proven track record of reducing bleeds, preserving joint health, and, in some cases, resolving target joints.1,5 Even patients with joint damage can benefit from factor prophylaxis because it can help preserve joint structure and function so that things don’t get worse.4

PROPHYLAXIS with FACTOR is the infusion of factor on a routine basis in order to prevent bleeding.4

TARGET JOINTS are joints with multiple bleeds in a short period of time, leading to chronic inflammation.1,4

A care team member of a hemophilia patient evaluates his knee joint with an ultrasound machine to monitor any changes that may need treatment or attention.

KEEPING FIT SUPPORTS HEALTHY JOINTS Staying active—even if it’s just taking short walks around the neighborhood—is a great way to keep your joints healthy, helping the muscles and tendons that surround them stay strong.3 And consider this: every additional pound you gain puts 4 times the amount of stress on your knees.6 So maintaining a healthy weight with exercise goes a long way toward keeping your joints healthy.

The World Federation of Hemophilia encourages participating in physical activity appropriate to your ability and interest to help keep joints healthy and reduce the risk of bleeds.7 You also can stay proactive by having regular appointments with your care team to monitor the current state of your joint health.

Not all physical activities are appropriate for all individuals. Please consult with your doctor prior to engaging in any physical activity.

References: 1. Quon D. Joint health in hemophilia: latest approaches and advances. Medscape. https://www.medscape.org/viewarticle/908267. Published February 5, 2019. Accessed May 6, 2019. 2. Alavian S, Norton C, Othman S. Living with mild haemophilia and care after a bleed—a qualitative study. J Haem Pract. 2016;3(1):14-23. doi:10.17225/jhp00063. 3. The Hemophilia, von Willebrand Disease & Platelet Disorder Handbook.

Hemophilia of Georgia. http://www.hog.org/handbook/article/3/33/joint-bleeds. Accessed May 10, 2019. 4. Manco-Johnson MJ, Soucie JM, Gill JC; Joint Outcomes Committee of the Universal Data Collection US Hemophilia Treatment Center Network. Prophylaxis usage, bleeding rates, and joint outcomes of hemophilia, 1999 to 2010: a surveillance project. Blood. 2017;129(17):2368-2374. doi:10.1182/blood-2016-02-683169.

5. Mullins ES, Stasyshyn O, Alvarez-Román MT, et al. Extended half-life PEGylated, full-length recombinant factor VIII for prophylaxis in children with severe hemophilia A. Haemophilia. 2017;23(2):238-246. 6. 51 ways to be good to your joints. Arthritis Today website. http://www.arthritis.org/living-with-arthritis/pain-management/joint-protection/joint-health.php. Accessed May 10, 2019. 7. World Federation of Hemophilia. Fitness and physical activity. WFH Guidelines for the Management of Hemophilia. 2nd ed. 2012. http://www1.wfh.org/publications/files/pdf-1494.pdf. Accessed May 10, 2019. 8. National Hemophilia Foundation. Playing it safe: bleeding disorders, sports, and exercise. HANDI Information Resource Center. 2017. https://www.hemophilia.org/sites/default/files/document/files/Playing-It-Safe_0.pdf. Accessed May 5, 2019. 9. Broderick CR, Herbert RD, Latimer J, et al. Association between physical activity and risk of bleeding in children with hemophilia. JAMA. 2012;308(14):1452-1459. 10. National Hemophilia Foundation. Role of new prolonged half-life clotting factors in hemophilia: new clotting factors for dosing schedule flexibility. HANDI Information Resource Center. 2014. https://www.hemophilia.org/sites/default/files/document/files/PHLBrochure.pdf. Accessed May 10, 2019.

Copyright © 2019 Takeda Pharmaceutical Company Limited. 300 Shire Way, Lexington, MA 02421. 1-800-828-2088. All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited.

S51791 09/19

26

n

LIFELINES for HEALTH

n

Fall/Winter 2019

STAY AWARE AND TAKE CONTROL OF YOUR JOINT HEALTH Monitoring your joint health with your care team is a good way to stay aware of any early signs of disease and take the treatment steps necessary to keep your joints healthy long term. Your care team may provide deeper insight from clinical examination, x-rays, ultrasounds, and magnetic resonance imaging (MRIs).1 Nowadays, an ultrasound may be routinely used to identify early changes in the joints, which can help guide treatment. Further, if you have joint pain, an ultrasound can help your care team understand if the pain is from a bleed or due to other causes, such as arthritis.1

It may seem like a challenge now, but your commitment to staying on top of your treatment plan through monitoring with a care team, prophylaxis with factor, and staying active will help keep your joints in step for a healthy future.

REMEMBER FACTOR COVERAGE WHEN YOU’RE ACTIVE Prophylaxis with factor is recommended by the National Hemophilia Foundation (NHF) before beginning your exercise routine.8 Keeping Factor in your body above a meaningful level helps provide coverage to protect your joints.9 Prepare for physical activity by planning your factor treatment schedule beforehand to help lower the risk of a bleed.8,10

Infuse with factor to keep joints in the safe zone

Low Bleeding Risk

100%

Factor levels that are safe for physical activity*

Factor Level (%)

75%

50%

Limited factor levels— use caution with high-risk physical activity

25%

Little protection from bleeds—not safe for strenuous physical activity

0 Infusion

Day 3

Infusion

Day 6

Infusion

Day 9

Infusion

In order to keep your joints as healthy as you can, continue your prescribed prophylaxis routine.

And make sure to talk with your doctor about when to modify your infusions based on your activity level.1

High Bleeding Risk

Day 12

Chart adapted from: Role of New Prolonged Half-Life Clotting Factors in Hemophilia brochure from NHF, 2014. *Not all physical activities are appropriate for all individuals. Please consult with your doctor prior to engaging in any physical activity.

VISIT BLEEDINGDISORDERS.COM FOR MORE INFORMATION ABOUT HEMOPHILIA AND INDIVIDUALIZED FACTOR THERAPY. Commitment to our community is always a priority for Takeda Hematology (previously part of Shire and Baxalta). As a leader in hemophilia research, Takeda continues to innovate on your behalf, developing programs and services that support your efforts each step of the way. Takeda is focused on providing advanced hematology treatments for today and innovating for the future.

WHAT’S NEW?

28

n

LIFELINES for HEALTH

n

Fall/Winter 2019

by Dr. Gary McClain, PhD

80

T U O ?

20

How About

USING the

80 20 Rule?

I

t’s only human nature to have high expectations for how we want life to look like day to day and what we want to accomplish. In a word: perfection. But life has a way of getting in the way of our intentions. Nobody knows that better than someone who is living with a bleeding disorder.

A question for you: Have you taken a look at your expectations lately? Starting with what you are expecting yourself to accomplish?

My clients often talk to me about how their lives aren’t measuring up to their expectations. They feel let down by others, not getting enough of what they need. They feel that they are letting others down by not being able to give enough. Or they feel they have let themselves down by not doing enough.

Here are a couple of examples: A client I’ll call Joe is balancing a full-time job, part-time college, and the responsibilities that go along with a bleeding disorder. To say that he pushes himself hard to be the best person he can be is an understatement. He recently told me about an experience he had that brought home to him how trying to do everything can lead to stress.

“I had a rough week at work, and I had a test to study for over the weekend. On Friday night, a friend called and said she was inviting a few friends over to help her set up her and her roommate’s new apartment. She told me how she really wanted me to be there.

FAMILY MATTERS

“Again, I had a lot to do and I also needed to get some rest. But I said yes anyway.”

“How did that turn out for you?” I asked.

“I spent the day with her and her friends, and I was exhausted that evening. I also banged my knee helping her move a couch, which I shouldn’t have been doing, and ended up with a bleed. Along with a lot of stress. So, I did pretty badly on my test.”

A client I’ll call Connie, is a mother of a child with a bleeding disorder. She told me about her own struggles with attempting to be perfect.

“I have a full-time job. I am a single parent. On Friday night, my son had a bleed that took us to the emergency room. He was fine but it was a hard

30

n

LIFELINES for HEALTH

n

Fall/Winter 2019

night and a late night, to say the least.

“I got a call early Saturday morning from my mom. She told me that she and one of her sisters were planning to stop by during the afternoon.

She laughed and told me she had bragged to my aunt about my amazing chocolate chip cookies.”

“Nothing like additional pressure,” I said.

“Exactly,” she answered. “So, I rushed around to make sure the bathroom and the living room were spotless and got the cookies in the oven. I was so stressed. I don’t know how I managed to keep my smile on while they were here. Needless to say, I collapsed after they left.”

Are my clients’ experiences anything like yours?

to or don’t know how to, or just won’t. Or because you won’t let them.

So, here’s an idea to consider: Given that we are dealing with imperfect beings, what if, instead of expecting perfection, you expected imperfection?

What if you started to ask yourself what parts of your life need to be functioning at 100%, and where you might begin to loosen up on your expectations? Averaging out your expectations to, let’s say, around 80%?

Adjusting your expectations for yourself, and the people around you, to a more realistic 80 instead of 100 percent would mean allowing for the human factor in yourself and others. It might also mean a whole lot less disappointment. Not to mention a whole lot less stress.

Self-Expectations… Self-Criticism… Stress…

Sure, it’s only human to expect to always perform at your best. To think you need to always be available to other people. As well as to have high expectations of how others should feel or act towards you. Especially people who are close to you, friends and family, or the healthcare professionals that you work with.

One of the most frustrating things about living with a bleeding disorder is adjusting to the constant challenges to maintain your own self-care or watching over (AKA enforcing) the self-care of a child with a bleeding disorder.

A whole boatload of responsibilities and demands, none of which you asked for. Along with the need to rely more on others to do their part, assuming you let anyone else help out (more of that later).

Here’s what I’ve learned from my clients, as well as in my own experience with friends and family dealing with chronic conditions: Expecting 100% can be a set-up for a letdown.

Behind the science and technology of healthcare are humans. And humans aren’t perfect. Healthcare professionals aren’t always so responsive. Delays are going to happen, along with the restrictions and inconveniences of managed care.

Yes, you’re human. With good days and bad days, good intentions, and your own limitations.

Your family members are also human. And that means they aren’t always so supportive, because they aren’t able

And what if you embraced this idea so much that you instituted a new rule at your house? The 80/20 rule. With you and your family all adjusting expectations for themselves and each other accordingly.

Ready to give the 80/20 rule a chance?

Here are ideas for how to loosen up your grip and giving yourself, and others, some breathing room.

Focus your energy on what needs to be in place to take the best care of yourself and your child. Taking care of your health is priority number one – medication, diet, rest, and anything else you need to do to manage your condition.

As well as the health of your child with a bleeding disorder.

So, if you’re looking for a starting place, this is one aspect of your life where striving for 100% makes sense. After all, your self-care is the cornerstone of your life.

Take a look at your priorities. Assuming you aren’t superwoman/man, maintaining 100% self-care and care for your children may require making some adjustments in the other areas of your life. Eighty out of 100 doesn’t have to mean an across the board cut but doing some reallocating to take the pressure off yourself. This might mean taking a look at your to-do list, the one you’ve written down or the one you keep in your head and making some decisions on what needs to be at the top and what needs to be at the bottom, what can be postponed. Think of it this way: When everything you have to do is fighting for top priority, you turn your mind into a battleground. How’s that for stress?

FAMILY MATTERS

Say no to the go, go, go. Let’s start with your family. Sure, you want to give 100% to your family. But parents often tell me that they run themselves ragged with work around the house, trying to participate in community or school activities, being constantly available for extended family events, while also trying to have real quality time with their children.

If that’s you, then it might be time to look at where you can tighten up and where you can lighten up. For example, Joe could have just said to his friend, “Sorry, but I need to sit this one out.”

Watch out for that four-letter word: NEED. What do you “need” to commit to?

Something that you really want to do or something that you feel like you should do?

Do you need to go to that meeting or commit to that weekend activity? If you look at your commitments from the 80/20 perspective, you might find that good enough, and not perfect, can leave you with more balance in your life. Less stress. Here’s a way to look at what’s needed and what’s not. At the risk of repeating myself: Maintaining your optimal health and the health of your children definitely falls into the need category, and therefore front and center in that 80% of your life that gets your best effort. No, no slacking there. However…

Say yes to a little mess. Could your house be a little less spotless, with the laundry waiting an extra day, the lawn waiting a few days? 80 out of 100 might mean a less than perfect house, but also give you more time to spend enjoying your family. While taking better care of yourself by getting a little more rest and a little less stress? And what if you asked other family members to help out, giving up some of your control and giving them a chance to give you a hand? Is there an aunt or uncle or a grandparent who would jump at the chance to come over and help out for an afternoon? For example, could Connie have asked her mom to pick up some cookies on the way to her house? Or to postpone her visit until Sunday?

32

n

LIFELINES for HEALTH

n

Fall/Winter 2019

Lighten up, for crying out loud. And while we’re on the topic, are you demanding 100 out of 100 from the people around you? Keep in mind that expectations for other people can lead to disappointment. Other people aren’t always going to act the way we think they should or hope they would. Everybody has days when they aren’t on their A-game, when they don’t feel good, when they aren’t so supportive. Instead of expecting the people of your life to be at 100, how about cutting them some slack, too?

Given that you are dealing with human beings here, 80 out of 100 isn’t so bad. Patience helps.

And what about you? Try going a little easier on yourself.

When you hear that voice of self-criticism start to zero in on what you should have don’t and didn’t, or do perfectly, remind yourself of the 80/20 rule. Give yourself credit for doing the best you can.

Speaking of should… Every time the word “should” creeps into your self-talk, ask yourself: “Who says?” Asking this question is a way to assess when the need to be perfect – and to live up to unrealistic expectations for yourself and others – is interfering with your well-being. In other words, stop “should-ing” all over yourself. (With the holidays on their way, you might want to build the “Who says?” test into your daily routine.)

Loosen up on that tight grip. Show some compassion toward yourself, and it will be a lot easier to show compassion toward others. Think 80/20! If you’re taking better care of yourself, you have that much more to give to others, and to give it joyfully and not out of exhaustion. And remember, we are all in this together.

Gary McClain, PhD, is a therapist, patient advocate, and author, specializing in helping clients deal with the emotional impact of chronic and life-threatening illnesses, as well as their families and professional caregivers. He works with them to understand and cope with their emotions, to learn about their lifestyle and treatment options, to maintain compliance with medical regimens, to communicate effectively with the medical establishment, to communicate better with other family members, and to listen to their own inner voice as they make decisions about the future. He writes articles for healthcare publications and websites, facilitates discussions in social health communities, and conducts workshops on living with chronic conditions, Chronic Communication Skillssm. He maintains a Website, www.JustGotDiagnosed.com.

FAMILY MATTERS

Making a

CHOICE for

W

ith so many changes and advances in the bleeding disorders community, it can leave many of us confused and uncertain about some of our treatment choices. You may have even asked yourself or your medical care team, "what products can I try?", or "which ones will even work for me?". Most healthcare professionals agree that inhibitor eradication/tolerization is still the preferred choice of care over bypassing agents, new or old. We've hand-picked two of our dearest friends (and their families) to share with you their experiences in inhibitor tolerization (IT.)

34

n

LIFELINES for HEALTH

n

Fall/Winter 2019

r o t i b i h In t n e m t Trea

Taha Amir Janet: What was your historic inhibitor B/U?

Taha: From about the age of 7 or 8 to until the age of 33 I had inhibitors. I believe my highest inhibitor BU was when I was a teen, which was about 1,000 BU. Janet: How many times have you tried IT and with what?

Units per kilo/frequency?

Taha: Ever since I was a kid, my hematologist always

wanted me to try Immune Tolerance. In the early 1990’s, there was not enough data and research about IT treatments and my parents were a little scared and nervous, so they decided not to go forward.. The first time I tried IT was when I was 33 years old. My BU had been under 5.0 for a couple of years, so my hematologist thought it would be a good idea for me to try IT. However, this came with several disclaimers. The first disclaimer was that I had a very slim chance of eradicating or getting rid of the inhibitor because of how long I have had them and my age. The second disclaimer was that I had to be 100% compliant with the treatment. However, he said that if I did not try, then I would not know the outcome.

FVIII product because it seemed to work for the people I trusted, and I had to start somewhere.

Janet: How is your current regiment working for you?

Taha: So far, this is definitely my “go to” factor for IT. I

started off with 100 IU per kilo, every day, for about six months. Then my hematologist lowered the dose to 75 IU per kilo, every day, for about 3 months. I was still being monitored very closely to make sure that my inhibitor did not come back. Currently I am on 25 IU per kilo, every other day and it is working great! I have been able to do more activities that I was not able to do before. If it continues to go well, my hematologist will consider lowering the frequency to three times a week.

Janet: Would you consider participating in a clinical trial for emerging products?

Taha: I know there are many emerging trials coming out

or in trials, but personally I would not consider them at this point. I am grateful to the people who do get on the trials, because without them there would not be better treatments or cures.

Janet: What choices for IT were presented to you by your care team?

Taha: My hematologist first suggested I try a

plasma-derived FVIII with vWF complex product, because it had good success with other patients.

He made me an appointment for the following month, so I could come into his office and get baseline bloodwork done.

When I had the second visit, he suggested using a recombinant FC Fusion extended half-life product. My partner and I were confused as to why, because the previous few times, he recommended for me to be on a plasma-derived FVIII with vWD complex product.

Janet: How else did you educate yourself about inhibitor treatment choices?

Taha: Fortunately, we knew of a few people

who guided us in our decision making, and we were able to talk to people who were using the product successfully.

Janet: Ultimately, why did you make your current product choice?

Taha: In the end, I chose the plasma derived,

BLOODLINES

The Walker Family Janet: What was your child’s historic inhibitor B/U?

The Walker's: Our son’s highest titer was 120 BU. Most

of the time while on ITT his titer has been under 10 BU. However, each time he failed IT, his titer went historically higher.

Janet: How many times have you tried IT and with what?

Units per kilo/frequency?

The Walker's: Our son, J, has tried IT many times with

various different products. We initially started out with 100U/kg of the recombinant product he developed his inhibitor on when he was 2.5 years old. We tried a couple rounds of IT with that for 4-5 years, then moved to a plasma derived FVIII- vWF complex at 100 U/kg based on the Factor

36

n

LIFELINES for HEALTH

n

Fall/Winter 2019

VIII. His titer was measuring 0 BU for several years on that, but during a growth spurt at age 9, his high titer came back.

He began bleeding almost continually, and we took a break in IT by treating prophylactically with a bypassing agent.

After his titer slowly came back down below 10 BU close to a year later, we restarted IT with the PD FVIII- vWF complex again. It came down but spiked back up again around age 12 during puberty. After age 12, he was on a constant inhibitor titer roller coaster ride. Although we consistently infused daily, his titer was continually changing.

We went through different periods of doing prophy with two bypassing agents to attempt to control bleeding while still doing IT. Based on other patients’ experiences we heard at the summits, we decided to try a combination of our previous FVIII-vWF complex and rFVIII together in the same syringe.

The reasoning behind this combination was to get more FVIII into the bloodstream than vWF, as the vWF would bind to multiple FVIII molecules and protect it. We used 200U/kg FVIII at this time. It was much less fluid volume but still did not tolerize the inhibitor. After his titer went up again and discontinuing FVIII to allow his titer to drop, we restarted IT when he was 14 with a recombinant PEGylated (extended half-life)product at 200U/kg, but his peak titer was not much higher than the rFVIII product we were using before, so we quickly switched for the final IT try to a Fc fusion extended half-life product at 200u/kg. He was using this for IT for a year and it did work in bringing his titer down.

Janet: What choices for IT were presented to you by your care team?

The Walker's: Our care team wanted us to return to

his original recombinant VIII product when he was age 2.5. We were offered to be on a randomized IT study where inductees were given either daily treatment or 3 times a week treatment. Ultimately, we chose not to go on it, because we felt strongly that J should be on daily treatment.

After not making progress with that we and our care team easily concluded that we needed to switch to a plasma derived product. We let them choose for us because we did not have any strong opinions about one being better than the other after much research. When it stopped working for him, we were given more freedom to choose the next product. Multiple times we were pushed to try rituximab as an immunosuppressant however, we declined due to low long-term success rates and the high risk of side effects.

We also developed an individualized, independent care plan of how to treat bleeds with our care team, so that when J started a bleed, we knew the procedure. If after treating for the first couple of days we were not seeing improvement, we would contact them for further help.

Janet: How else did you educate yourself about inhibitor treatment choices?

The Walker's: We researched by reading the latest

studies via medical journals and talked to pharmaceutical manufacturing representatives and as many medical

professionals as we could. The most helpful way we educated ourselves was by going to the Inhibitor Summits and Inhibitor Family Camps, where we could talk to other families and hear what was working for them.

Janet: Ultimately, why did you make your current product choice?

The Walker's: We could see that J’s quality of life was

deeply affected being tied to lengthy, daily bypassing agent infusions that were not preventing bleeds and joint damage.

Although his titer had historically gotten to a manageable level where we could treat with Factor VIII, his titer was never completely gone and his half-life never went over 6 hours to indicate complete tolerance. So, for us, although IT helped us manage the inhibitor for many years, it wasn’t the ultimate end to his battle with inhibitors.

We felt that trying the bi-specific, antibody product would give him the best chance to experience fewer bleeds, less joint damage throughout his life span and improve his quality of life. We were not able to access the Phase 3 trials, but switched shortly after it was approved for inhibitor patients.

Janet: How is your current regiment working for you?

The Walker's: Our current weekly injection regimen works amazingly. He has had approximately 3 bleeds in the past year and a half, where previously he had bleeding issues 2-4 times a month and now he rarely bleeds. Overall, he has spent much less time on crutches and using his wheelchair.

Technically his inhibitor titer has increased in numbers now that we stopped IT, but we are better able to manage the bleeding and daily life issues. We feel he has progressed immensely in regards to his quality of life and ability to be an independent, fully functioning, young man.

Janet: Would you consider participating in a clinical trial for emerging products?

The Walker's: Yes, we would consider it and make the

best choice for our individual situation based on the current information available.

It's important to know that each individual reacts differently to medications, and results can vary; especially when it comes to IT since it’s an attempt to manipulate the immune system. CHES reminds you to speak with your medical care team before changing, discontinuing, or beginning a new regimen. If your doctor isn’t open to discussing or considering certain treatment options, it may be helpful to refer them to another treatment center or doctor who has experience with that specific product or treatment method. And remember, CHES makes no claims nor prescribes any medical advice, as we are strictly an education company. It is our intention to inform others of all options so they can make the best decisions for themselves.

BLOODLINES

American with Disabilities Act (ADA) & Section 504

Overview & Reasonable Accommodations

by Janet Brewer, M.Ed & Lisa Cosseboom, M.Ed., CAGS

S

ection 504 of the Rehabilitation Act of 1973: The 504 section of this law was enacted to provide people with disabilities protection against discrimination by any program, activities or schools (as well as colleges and universities) that receive federal funding. The Office of Civil Rights (OCR) are responsible for enforcing institutions to provide reasonable accommodations for “qualified individuals.”

American with Disabilities Act (1990): This amendment in 1990 widened the breadth of coverage for people with disabilities. The act notes that its purpose is “to provide a clear and comprehensive national mandate for the elimination of discrimination against individuals with disabilities.” Part of this act prohibits a “public entity” such as a public school and non-parochial private schools from discriminating against students with a disability.

•

Affording a qualified student with a disability an opportunity to participate in or benefit from the aids, benefits, or services that are not equal to that afforded other students.

•

Providing aids, benefits, or services to a qualified student with a disability that are not as effective as those provided other students.

•

Providing different or separate aids, benefits, or services to a qualified student with a disability unless necessary to provide aids, benefits, or services that are as effective as those provided others.

•

Aiding or perpetuating discrimination by providing significant assistance to an agency, organization, or person that discriminates on the basis of a disability.

•

Denying qualified persons with disabilities the opportunity to participate as a member of a planning or advisory board because of their disability.

•

Limiting a qualified student with a disability from the enjoyment of any right, privilege, advantage, or opportunity enjoyed by other students.

What is considered discriminatory?

•

38

Denying a qualified student with a disability the opportunity to participate in or benefit from the aids, benefits, or services that are afforded other students.

n

LIFELINES for HEALTH

n

Fall/Winter 2019

How is a disability determined?

The law states that a person must: •

Have a physical or mental impairment which substantially limits one or more major life activities (those basic activities that the average person in the general population can perform with little or no difficulty)

•

Has a record of such impairment

•

Is regarded as having such an impairment

The term “substantially limits” one major life activity can be episodic or in remission and still be considered a disability and have accommodations in place for when it is active; and the determination must be made without regard to the ameliorative effects of mitigating measures.

What does this mean?

The 504 Team must identify mitigating measures being used by a student and determine how the disability would impact the major life activity in the absence of the mitigating measure. For example, a student with an

inhibitor who happens to not be actively bleeding, or is currently managing any bleeding disorder with infusions (any type of medication is a mitigating factor), the Team must determine whether the impairment would substantially limit a major life activity if removed. Each mitigating measure must be identified and used to determine what the impairment would look like if the mitigating measure was removed. Additionally, the Office of Civil Rights has determined that health plans and emergency plans are considered mitigating measures.

Examples of mitigating measures: •

Medication, medical supplies, equipment, or appliances; low-vision devices (which do not include ordinary eyeglasses or contact lenses); prosthetics, including limbs and devices; hearing aids and cochlear implants or other implantable hearing devices; oxygen therapy equipment and supplies; use of assistive technology;

•

Reasonable accommodations or auxiliary aids or services; and

•

Learned behavioral or adaptive neurological modifications.

WHAT’S the PLAN?

Disability Categories: •

Specific Learning Disability

•

• Blindness

•

Other Health Impairment (bleeding disorders would fall here)

Speech & Language Impairment

•

Visual Impairment

•

Orthopedic Impairment

•

Traumatic Brain Injury

•

Autism Spectrum Disorder

•

Multiple Disabilities

•

Emotional Disturbance

•

•

Intellectual Disability

• Deafness Hearing Impairment

The purpose of a 504 plan is to provide access or remove barriers to participation. It provides students the same rights and services as their non-disabled peers. For students with bleeding disorders, a major life activity could include walking, writing, sitting or standing. Under a 504 plan a student can access related services as determined by the Team. These include the following: Speech & Language Therapy: •

Occupational Therapy

•

Physical Therapy

• Counseling •

Rehabilitative Counseling

•

School health services

• Transportation Reasonable Accommodations under a 504 plan: •

Schedule of Physical Education activities

•

Extra set of books at home

•

Extended travel time around building

•

Extended time for homework/make-up work

•

All medically related absences are excused with no loss of credit

•

Field trips to include a nurse

•

Permanent pass to the school nurse

•

Medications, needles and supplies maintained in the nurse’s office

•

Staff in-service

•

Tutoring due to absences

•

Physical education credits for participating in outside physical therapy

•

Audio or video of class lectures

•

Regular email contact from teachers regarding missed assignments

•

Note takers/Copies of teacher notes

•

Homework posted on the school district internet (Class Dojo/Google Classroom)

•

Permanent pass for school elevator

•

Walkie-Talkies for recess

•

Medically-related absences excused with no loss of credit

•

Individualized Health Care Plan

What about after-school programming?

Any program that is operated or funded by a school or contracted by a school is required for ensuring reasonable accommodations for children on a 504 or Individualized Health Plan. The school district, family and after-school program should work to ensure consistency between the school plan and what accommodations the student needs in the afterschool setting.

Parents should plan on advocating for their child in the school system as bleeding disorders are rare and school staff may be unfamiliar with their needs or what accommodations will be needed. After reasonable requests for 504 eligibility, if a family has a complaint contact the Office for Civil Rights at: https://www2.ed.gov/about/offices/list/ocr/qa-complaints.html

40

n

LIFELINES for HEALTH

n

Fall/Winter 2019

FACTOR REPLACEMENT REFLECTS THE PROTECTION WITHIN

For people with hemophilia, Factor treatment temporarily replaces what’s missing.1,2 With a long track record of proven results, Factor treatment works with your body’s natural blood clotting process to form a proper clot.2-6 Brought to you by Takeda, dedicated to pursuing advancements in hemophilia for more than 70 years.7

Stay empowered by the possibilities.

References: 1. Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388:187-197. 2. Canadian Hemophilia Society. Factor replacement therapy. http://www.hemophilia.ca/en/bleeding-disorders/hemophilia-a-and-b/the-treatment-of-hemophilia/ factor-replacement-therapy/. Accessed May 18, 2018. 3. Franchini M, Mannucci PM. The history of hemophilia. Semin Thromb Hemost. 2014;40:571-576. 4. Hvas AM, Sørensen HT, Norengaard L, Christiansen K, Ingerslev J, Sørensen B. Tranexamic acid combined with recombinant factor VIII increases clot resistance to accelerated fibrinolysis in severe hemophilia A. J Thromb Haemost. 2007;5:2408-2414.

5. Antovic A, Mikovic D, Elezovic I, Zabczyk M, Hutenby K, Antovic JP. Improvement of fibrin clot structure after factor VIII injection in haemophilia A patients treated on demand. Thromb Haemost.

2014;111(4):656-661. 6. Berg JM, Tymoczko JL, Stryer L. Many enzymes are activated by specific proteolytic cleavage. In: Biochemistry. 5th ed. New York, NY: WH Freeman; 2002. https://www.ncbi. nlm.nih.gov/books/NBK22589/. Accessed May 18, 2018. 7. Shire. Shire’s 70+ year commitment to the hemophilia community. https://www.shire.com/en/newsroom/2018/january/7sossj. Accessed June 6, 2018.

Copyright © 2019 Takeda Pharmaceutical Company Limited. 300 Shire Way, Lexington, MA 02421. 1-800-828-2088.

All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited. S46132 03/19

89 E. Washington Street Hanson, MA 02341-1125

CHES Mission To Inspire awareness and selfreliance for patients with chronic health conditions, their families, and their communities.

Editors in Chief Janet Brewer, M.Ed Eric Lowe

Editor Janet Brewer, M.Ed

Publication Designer Eric Lowe

Contributing Writers Taha Amir Anna Bell, LICSW, LCSW-C, LISW Janet Brewer, M.Ed Lisa Cosseboom, M.Ed., C.A.G.S Donovan Guerrero Eric Lowe Kenneth Martin Dr. Gary McClain, PhD Stephanie Shea Krystyn Strother Amy & Eric Walker

Stay Connected

__________________________________________________

Subscribe for Free If you have received this newsletter from another individual, and you wish to receive your own personal copy, please contact us via any of the following methods.

Email: info@ches.education Office: 781.878.8561 Online: https://ches.education/communications-profile-form __________________________________________________

Online Version Giving a contribution to support the newsletter:

View this publication online for access to helpful links, cited resources, and other great add-ons at: https://ches.education/newsletter/ __________________________________________________ DEAR HEALTH CARE PROVIDERS, PLEASE CIRCULATE TO PATIENTS.