Lifelines for Health (vol 12): Understanding Complex PTSD Trauma

Fall/Winter 2018 I Volume 12

A CHES Publication

Inhibitor Family Camp: “HOME” Coming l Leverage 2018: Walking the Wire Exploring the Possibilities of Factor l Anxious? How About Running Toward Your Anxiety? l Shining Light on School System Disagreements An Overview of Mindfulness and Research-Based Physiological Benefits

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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 continue the celebration of our 5th year of LifeLines for Health’s evolution to a full-fledged magazine specific to the inhibitor community, we remain humbled by the privilege to provide it. We will also be celebrating our 10th year in 2019 as a national organization that provides education and support to the smaller subsets of the bleeding disorders community.

There is no way we would have been able to accomplish any of this without our generous sponsors and YOU, our readers and participants. CHES’ mission is to inspire awareness and selfreliance for patients with chronic health conditions, their families, and their communities. Accuracy, empathy and integrity are the core principles of our company.

We conclude our two-part series on PTSD in this edition.

PTSD is a very real diagnosis for those coping with acute medical challenges. As you read through our Feature, can you identify situations that resonate with you?

Perhaps as a child, if you are an adult reader, a parent currently in the trenches, or a sibling trying to help but managing disappointment and fear? How do these feelings or behaviors manifest themselves in your everyday life? Anxiety is a powerful emotion, especially when it comes to managing chronic illness within a family. In Family Matters, read about ways to use your anxiety to propel you forward or how to sit with it and not let it control your life.

What’s the Plan takes a different approach this edition by answering some of the questions we hear during our program sessions. Do you have other questions we didn’t answer? Send us an email at info@ches.education.

Ever wonder if there is science behind Mindfulness? In 2011, CHES began offering mindfulness as a topic in our programming.

In Mind-Body Connection, discover the proven health benefits of this practice and perhaps find ways you can weave some exercises into your daily life. You will be glad you did!

Are there other benefits associated with factor treatment? This question has come into the limelight as Hemlibra, a non-factor bypass product has entered the mainstream of treatment. In Bloodlines and What’s New, read about some ground- breaking research, and talking points for your health care professional as you weigh all treatment choices available to you. 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 wellinformed decisions for yourself, your child or loved ones.

(Concluded on pg 3)

2019 PROGRAM GUIDE

Event opportunities for the inhibitor community

CHES has been serving the needs of those with rare bleeding conditions since 2009. As long time members of the bleeding disorder community, our mission is to inspire awareness and self-reliance for patients with chronic health conditions, their families, and their communities.

Below is a brief overview of the various programs we are offering to the inhibitor community in 2019. More details on each of our programs can be found on our website: www.CHES.education

Friday, June. 21st - Monday, 24th, 2019 Camp Zeke - Lakewood, PA

After the Shock provides parents and caregivers of diagnosed children (ages 0-18) with hemophilia and inhibitors the support they truly need.

CHES understands at a personal level the obstacles and challenges that coincide with raising a child with inhibitors. We strive to equip every new parent with the tools to navigate his/her child through everything from hospital stays to home infusion. This weekend program is packed full of education and support. We play, learn, and grow while we build stronger relationships.

LEVERAGE

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Date: Wed, Sept. 4th - Sun, 8th, 2019 YMCA Camp Collins - Gresham, OR

Leverage is a pioneering, national program for young adults from ages 18 and up that have hemophilia with an inhibitor. The program consists of life changing experiences that allow participants to challenge themselves in ways they never thought possible through a series of outdoor adventure, experiential education activities, including fishing, rafting, reflection, and various ropes courses with adaptations in place.

The Ultimate Inhibitor Adventure

Friday, Sept. 27th - Monday, Oct. 30th, 2019 The Painted Turtle - Lake Hughes, CA

Inhibitor Family Camp is specially designed to meet the needs and limitations of children (ages 6-18) with both hemophilia & inhibitors.

Immediate family members are invited because we understand that chronic illness affects the entire family. We play, learn, and grow while we build a stronger community.

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Friday, July 19th - Sunday, 21st, 2019 Santa Fe, New Mexico

Momentum is a men’s only, national program for those ages 18 and up that continue to live with both - hemophilia and an inhibitor. The program provides opportunities to not only speak with specialists familiar with inhibitors, but most of all, amongst themselves about issues and challenges important to them.

The CHESTM logo and program logos are registered trademarks of Comprehensive Health Education Services, LLC. The use of these marks are restricted in part or their entirety without expressed written consent.

Integrity, Accuracy, Empathy...

FEATURE 10 I Understanding Complex PTSD Trauma Charlie P. Gilbert II, LCSW explains that life with an inhibitor can impose a number of stressful experiences which can lead to repressed memory triggers, depression, and ultimately - PTSD. As a solution, Gilbert discusses a variety of approaches like reflection, P.A.C.E., and his very own Charlie’s Challenges.

CONTENTS COMMUNITY CHATTER

WHAT’S the PLAN?

4 I Inhibitor Family Camp: “HOME” Coming

27 I Shining Light on School System Disagreements

Mother of 3 boys, and veteran IFC (Inhibitor Family Camp) attendee, Crystal James reflects on this year’s experience at camp, and explains why they keep returning each year.

8 I Leverage 2018: Walking the Wire Gabe Anspaugh must have read Gilbert’s feature article on inhibitor trauma as he faced his own repressed memory trigger at this year’s outdoor experience program - Leverage (for adults with active inhibitors.) Find out just what that memory was, how it unwelcomingly returned, and what he did to overcome it.

BLOODLINES 18 I Exploring the Possibilities of Factor: Factor Replacement Reflects the Protection Within With all the new options in the bleeding disorder community, it’s easy to forget about the foundation blocks that have fulfilled a void for so many. This is a simple reminder of the many roles that factor plays, and perhaps there’s a lesson on a few benefits that we never really thought of.

FAMILY MATTERS 22 I Anxious? How About Running Toward Your Anxiety?

We live in a world of uncertainty, stress... and a whole lot of medical challenges. Add them all up and you’ve got yourself a big ball of anxiety. Dr. Gary McClain understands this so well that he has formulated over 10 techniques to use at home, take in the car, practice in the hospital, or bring wherever else you see fit.

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This article might just be the most interesting in the history of What’s the Plan? as we’ve gathered real-life scenarios of accommodation conflicts, civil rights, and more between students (their parents) and the school system. Our expert, Lisa Cosseboom, CAGS explains what options you and your student have.

WHAT’S NEW 29 I The Future’s So... BRIGHT This has been a whirlwind of a year, so let’s take a moment to collect our thoughts. Our very own Janet Brewer, M.Ed reviews some of the highlights of this year’s biggest developments that have and (most likely) will continue to make a big impact in the future of an uncertain world.

MIND BODY CONNECTION 34 I An Overview of Mindfulness and Research-Based Physiological Benefits Mindfulness expert, Krystyn Strother explains the scientific research behind mindfulness meditation. The benefits of mindfulness promote awareness and understanding of our thought processes, as well as physiological benefits such as weight loss, managing inflammation, and emotional wellbeing.

Learn more on how to apply these benefits in your life.

Discover Learn more about our focus on the rare bleeding disorder community “Our goal is to help better the daily lives of patients living with hemophilia with inhibitors or severe von Willebrand disease.”

Discover us at hemabio.com or call 1-855-717-HEMA (4362). © 2017 HEMA Biologics, LLC HB061-0617

HEMA-930 HEMA_JA_BLOOD_horiz_FINAL v2.indd 1

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Letter From the Editors (conclusion from inside page) Community Chatter highlights our Leverage and Inhibitor Family Camp programs this year written from our participants’ perspective. 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 say goodbye to the last remnants of fall’s splendor and get ready to hunker down 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

“It is in your moments of decision that your destiny is shaped” - Tony Robbins

jbrewer@ches.education l elowe@ches.education

CONTENTS

Inhibitor Family Camp

TM

“ HOME” Co m i n g

by Crystal James

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nhibitor Family Camp to my family and I means love and acceptance. We live in a world that’s harsh and sometimes rather distant to the reality of what hemophilia is. Camp is a place where we as parents can watch our children be carefree and the children themselves can build relationships amongst their peers and build confidence within themselves.

Families are able to see within what can be missing as a whole when we step out of our normal environments. We get a chance to escape the everyday routine of life and let the enjoyment of being a structured family unit flourish as one.

I feel as though IFC gives my boys a sense of self confidence. They get a chance to meet peers older than them such as camp assistants and counselors that also have the same disorder that they battle every day. Meeting these older peers with the same challenges, gives them a sense of self-fulfillment. It provides the boys’ positive reinforcement that growing up with hemophilia is okay and they can do anything in this world that they want.

Sponsored by an educational grant from (Continued on page 6)

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COMMUNITY CHATTER

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 Shire, 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. ©2018 Shire US Inc., Lexington, MA 02421.

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

Shire and the Shire logo are registered trademarks of Shire Pharmaceutical Holdings Ireland Limited or its affiliates. S40507 06/18

Inhibitor Family Camp is specially designed to meet

the needs and limitations of children (ages 6-18) with both hemophilia & inhibitors. Immediate family members are invited because we understand that chronic illness affects the entire family. We play, learn, and grow while we build a stronger community.

2019 SCHEDULE

Scheduled: Friday, September 27th - Monday, the 30th 2019

Location: The Painted Turtle, Lake Hughes, CA Registration opens: Friday, June 6th, 2019

More info at CHES.education/inhibitor-family-camp

I also think Inhibitor Family Camp is a place where you can feel relieved within the hemophilia community. All of us as families were brought to this amazing place for the same reason. As crazy as life can be dealing with the horrors of this disorder every day, day in and day out, camp seems to be a place where these horrors don’t exist. All of the attending families are comfortable with one another. These children from all different walks of life join together as one and we become one big happy family for a whole entire weekend.

Camp is also a place where we learn how to deal with the mental aspects of living with a child that has hemophilia with an inhibitor. I find it amazing that we get the chance to challenge our intellect and obtain knowledge on how to deal with the harsh reality of what hemophilia can do to your family members and the siblings living with it as well. We need that aspect of teaching for us as parents because it can be tough having to deal with this disorder and having other children.

For me, altogether Inhibitor Family Camp means - we grow substantially as a family. We gain confidence as well as independence. We gain memories and friends for a lifetime. We look forward to the year passing so that we can make these memories grow with many more. If I truly had to sum up camp to one word, I would say camp is like home because (in my eyes) home is where the heart is, and Inhibitor Family Camp is full of just that. Love for a lifetime. I will always love for my family to be a part of this experience. Thank you.

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COMMUNITY CHATTER

DO YOU OR SOMEONE YOU LOVE HAVE HEMOPHILIA A WITH AN INHIBITOR TO FACTOR VIII? YOU MIGHT BE INTERESTED IN OUR CLINICAL RESEARCH STUDY. Factor VIII inhibitors are antibodies that people with hemophilia A can develop in response to their Factor VIII treatment. They interfere with Factor VIII and prevent it from working the way it should. The development of FVIII inhibitors is one of the most serious complications for people with hemophilia A. The INITIATE clinical study is examining the potential advantages of utilizing a patient-specific laboratory test called batch-selection to decrease the time to success in Immune Tolerance Induction (ITI) for hemophilia A patients with inhibitors to FVIII.

SPEAK TO A STUDY REPRESENTATIVE

For more information, or if you would like to be considered for participation in the study, please contact us at 916-734-3880. We will be available to discuss any concerns or answer any questions.

Or go to www.initiateusa.com to learn more.

LEVERAGE 2018 Walking th

I

was diagnosed with inhibitors when I was 15 years old.

Up until that point I lived my life pretty normally. Yes, I had hemophilia, but I wasn’t going to let it get in the way of how I lived my life. However, the first little bit of time with inhibitors was quite tough on my mental state. What could I do, and what couldn’t I do? And could I even get back to where I was?

As time went on, I again discovered what I could do and what would be more difficult for me.

Back in February, I was on a

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by Gabriel Anspaugh

e r i eW

slackline at a rock-climbing gym. (I know, a hemophiliac with an inhibitor at a rock-climbing gym - sounds like the beginning of a bad joke, right?)

Moving on, a slackline is essentially a tightrope that has a lot of slack, so it’s more wobbly. I was determined to prove that I could do this. I ended up rolling my ankle pretty severely. This injury put me in the hospital for a week and in a wheelchair for many more. As I was going through this, it was pretty hard for me to look at the positive

Fall/Winter 2018

side of this situation since there really wasn’t one when losing the ability to walk.

However, there was an upside to this accident. Eight months later, this life lesson came full circle when I attended an educational, adventure, camp program for individuals with inhibitors called Leverage in Oregon.

As I was at Leverage, one activity we did was a high ropes course. The amazing instructors had suggested to us that we each find a specific goal to accomplish. I wanted to complete every element of that course.

My first instinct was to go straight for the most difficult ones to complete. In my nature of being a very physically, active person, these tasks were fairly easy for me. But I knew that the last element was going to be a challenge. It was a slackline with ropes hanging at separate points to hold onto. My experience with a slackline from less than a year prior was the most difficult for me to accomplish. It’s funny how such an easy obstacle to complete was the most difficult for me to overcome. That horrible fall, the time spent in the hospital, and that debilitating wheelchair were all running through my mind throughout the entire experience. I watched all of my blood brothers (who I had grown to know and love) go through this obstacle with no problem and just keep going. However, when I stepped out and was about a quarter of the way through this element, I actually had to stop, take a knee, breath, and wrap my mind around everything. I knew I couldn’t fall because I had a safety line. And I knew that the likelihood of injury was low. But why was this so hard for me? Once I caught my breath enough to keep going, I just had to keep my eyes on the trees ahead and try not to focus on the memories of what was the worst injury of my life. Once I got to the next platform, I had to fight off tears as I realized that I just conquered something that had crippled me for a better part of the year.

In collaboration with

Sponsored by an educational grant from

So, what was one of the biggest things that I took away from Leverage 2018? …the memory of conquering something that was once so disabling to me.

COMMUNITY CHATTER

UND ER STANDING

Complex

PT SD

Trauma

“The” Inhibitor Traumas

D

o you remember yours? That first time that it really hit you that your child had a life-threatening inhibitor.

Maybe it was when the Doctor or Nurse told you in the HTC clinic that the “normal” treatments to stop the bleeding weren’t working and that it was clear that your child’s body was rejecting the known factor solutions. Or it could have been when you nervously waited in the chaotic

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Emergency Room with dozens of other parents while a Doctor somewhere behind a curtain in another room tried to figure out what to do with your child who couldn’t stop bleeding, swelling and crying. And then there was the time that you with others had to hold down your child, kicking and screaming, to infuse or draw labs. The abject powerlessness of a parent to protect or save their child is painful in itself. You probably recall the de-personalization that you felt when it seemed as though you were outside

By: Charlie P. Gilbert II, LCSW

of your own body, observing and suspending reality. Take a moment and think about that time. You’ll know which incident it was because it’s written indelibly on your memory. Or maybe you have several such events to choose from.

you that all the new modern treatments would prevent the joint damage and crippling changes that Dad had and your child would be “normal” like the other kids at school. But this, this is horrible! Few people would doubt that these experiences constitute a traumatic exposure.

There’s nothing that prepares you for this. Yes, Uncle Billy and Pops both had hemophilia and an inhibitor, but that was their life and your child’s future is now threatened. You knew that this wouldn’t be easy, despite everyone telling

The memories are back there and perhaps you’ve managed to erase them from the daily journal of your thoughts and behaviors but in the darkest recesses of your mind they are hiding. The events have left little land mines

FEATURE

for that unsuspecting moment when they trigger the anger, panic, powerlessness, fear or hurt that comes from your exposures to the earlier threat to your child’s existence and your impotent role as a parent. It might be on another visit to the hospital years later when someone minimizes the effects of a bleed. Or it could be when the school assumed that your child couldn’t participate in the class musical or other event.

In the case of PTSD, clinicians look for a traumatic event. Here however, we find in inhibitor care and treatment a series of small and repetitive traumas that over time coalesce into an ongoing chronic stressor leading to physical, developmental and interpersonal negative outcomes. These include avoidance of medical care and treatment or withdrawal or hypersensitivity to other threats in the environment.

The Brain’s Response

We know, from the research of the ACEs (Adverse Childhood Experiences) events in children’s lives, that abuse, neglect and other severe trauma, experienced or witnessed predicts very negative outcomes later in adult life. In this study of the early lives of more than 17,000 adults, the CDC and Kaiser Permanente Health Appraisal Clinic found that ten forms of childhood trauma were associated with negative psychological and physical outcomes. Unfortunately, the forms of trauma most studied failed to capture the inevitable and repeated traumatization of children and their families by medical and surgical procedures. In response to this oversight, professional bodies have begun to look at new terminology to describe this problem. Complex trauma and Complex PTSD are two of the newer terms created to recognize the various symptoms of this developmental trauma.

So what will it take to recognize the lasting effect these events have had on your mind? We know that the most traumatic events and stressors lead to a cascade of neuroendocrine responses in the body. These changes alert and prepare us to take on the stresses and challenges we’re facing. But, if constantly repeated, they also lead to permanent changes in our brain structure in which we develop high arousal levels leading to anxiety, fear, hypervigilance, avoidance, and compulsive, intrusive thoughts and memories. Some clinicians will find these symptoms giving rise to a diagnosis of depression or PTSD. In Depression they observe a loss of hope and the inability to find enjoyment. Acute Inhibitor care at a very young age can also result in traumatic exposures which isolate family members, change their roles and may produce an anxious attachment style in the child, leading to future difficulty forming interpersonal relationships.

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The changes that occur in the brain following these occurrences become permanent and represent considerable

risk to health and life for parents and children going forward. Parenting also affects children’s’ brain development in lasting ways. As children grow with the ever-present threat of severe physical outcomes of a momentary lapse in judgement, they take their cues from the coping practices of their parents. In effect, they surrender their own coping choices to the dominant style of their parents’ management of the stressors that develop out of the child’s inhibitor.

This is also quite evident in our other children, the siblings of the affected child, when they witness the overpowering acuity of inhibitor related activity. Priorities shift, plans are given up, goals are set aside, events cancelled, hours are spent in clinical offices or emergency rooms.

Needed attention shifts from the unaffected siblings to your child with the inhibitor.

The brothers and sisters get to see their significant adults at their most stressed and find in that experience meaning about their own role in the family. At the same time the older and younger sibs are required to take on adult responsibilities and more mature coping responses. Their childhood can fade away into a “parentified” identity with accompanying expectations from other family members. The siblings help with the caregiving, sacrifice their own events and happiness, and can become surrogate adults in the home. Of course, it’s quite possible that they will reject the role entirely and become the focus of resistance to the family process. Their acting out can send a powerful message about their complaints. The stressors experienced by siblings, while lacking a diagnostic terminology, lead to many of the same symptoms and developmental impediments found in PTSD. More than a few siblings of children with inhibitors have shared that in crisis they become “invisible.” Some even hide there.

Overcoming the Trauma So what, if anything can we do about this? How can we help our traumatized children, siblings, partners, and ourselves to avoid the negative outcomes of the repeated traumatizing exposures? There are certainly a great many approaches

to reduce, mitigate and overcome the symptoms and risks of the trauma. In the summer issue of Lifelines for Health Dr. Gary McClain told us about the effects of repeated traumatic exposures on the individual with an inhibitor and on his caregivers as well. He encouraged us to improve our coping, manage our anxiety, remain calm and get support.

I’d like to add several important ideas that could help us to address the symptoms and improve outcomes for our affected children, siblings, partners and for ourselves. The following approaches are designed to be useful for anyone experiencing the effects of repeated and chronic traumatic exposures and needing to overcome adversity. Some of these you may find are helpful while others don’t make sense to you at this time.

Some of you may have tried and found them not helpful. Others you might see as difficult or moving you out of your normal responses. I would ask you not to discard them as they may be more effective at another time or with another member of the family. Here are “Charlie’s Challenges.”

First let’s remember the lesson of why we have families. A family is the laboratory for the biopsychosocial development of its members. Each person in our family is on a trajectory of development towards happiness, satisfaction, cognitive/ intellectual development, interpersonal effectiveness and meaning in life. We can get there and help our family members to get there by understanding and supporting each other. There are some rules for accomplishing this. One is that we need to understand the meaning of events, emotions, and behavior not just in ourselves and family members but in others around us. This requires us to set aside our emotions for a moment and consider the significance of what is happening, what we and our family members are feeling. The poet, Maya Angelou told us that people don’t really remember what we said or did but “…how we made them feel.” If we lose our “cool,” we become reactive and not reflective. Aside from saying things that serve to alienate others, we set a problematic example for our children and impede their acceptance and appropriate response to the stressors.

FEATURE

Our children are smart and they often know exactly how we’re feeling even if we didn’t verbalize it directly. But they more frequently act out their feelings rather than articulate them. We must help them to find the words and sometimes we give them the words by recognizing and validating their feelings. They often need help to really see and understand what is going on around them, especially in threatening environments such as schools, emergency rooms and clinics. To understand their risk, they will take their cue from their parents and other adults behavior. If they’ve got an angry, upset or fearful parent they will adapt quickly to fight, flight or freeze. Their arousal level will peak with uncertainty about what to do next. In that setting they may mistake or over exaggerate the threat that exists and they won’t be able to see any positive benefits of treatment and compliance. They won’t recognize or adopt the process of reflection and choosing the best approach. So, we must protect them from our negative emotions and model the self-regulation that will instill confidence and serve them well in this and future crises.

But reflection serves us as well. An important aspect of coping with stress is recognizing and accepting events and their meaning to us. As we learn to identify our triggers we can adopt an improved “reflective functioning,” that simultaneously engages our emotional and cognitive brain structures. Rather than respond immediately to each insult and stressor that appears in our environment we need to step back and ask ourselves, “What is this?” and “Do I own this?” This will help us to modify our perception of the problem and reduce its power. If we can adopt this approach we can avoid the outlay of emotional energy on the past and move on to a more productive approach of understanding and coping with the stressors. These changes can re-train the brain to be less responsive to triggers and reduce our need to be overly vigilant.

Some parents have pursued other behavioral and cognitive approaches that have proven helpful in defusing their emotional outbursts. Quite a few people have discovered that they need a “time out” in response to re-experiencing the thoughts and emotions from the original trauma.

They are inclined to focus on a

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breathing exercise to clear their mind and control their physiological response. Others may go directly to meditation and find that doing so routinely, before entering a stressful venue, helps to reduce their anxiety.

The old cliché of “living in the moment” rather than the “painful past” gives us a chance to think clearly and positively. Sometimes we’ll find that we “own” only a part of the stressor and that a painful part belongs to some other person or is part of a different problem. Yes, we certainly learn from the past, but if our negative emotions focus only on the past, they cloud our vision of what will make a significant change in coping with the issue. At other times we may find that we need to take responsibility for our actions and emotions and this requires making amends to our loved ones. The act of offering apology and asking for forgiveness can lift a burden from ourselves and from our partners and children.

and improved social relations. Other research points to positive affect reducing the likelihood of adolescent substance abuse and risky behaviors. Helping our loved ones to find the positive in a difficult situation can make a big difference in their feeling of security and hope for a better future.

Another critical aspect of ownership is that as parents, we can’t take ownership of health problems and behavior from our children.

If we want our children to learn to manage their lives and become resilient they have to “own” their life with all of its assets and liabilities.

Moving towards a more effective outcome, we know that decisionmaking is improved by positive thinking. Problem-solving is enhanced and positive affect reduces psychological distress and alters neuroendocrine, inflammatory, and cardiovascular activity. It leads to experiencing less pain

It can be awfully hard for parents to not take complete responsibility for our children’s health and treatment issues but doing so takes away the motivation to be effective managers of their own lives. It deprives them of the feelings

FEATURE

of competence and confidence in directing their own position in the family and in the world in general. Yes, we love and want to spare them all the mistakes in judgement, but they find out who they are by participating in the process. As Billy Joel reminds us about mistakes, “…they’re the only things that you can truly call your own.” One of the hardest parts of parenting is supporting our children even when they chose poorly and against our best advice.

Helping our children overcome the trauma experience is often foremost on parents’ minds. One mindful approach for parents to adopt that has proven effective is PACE. Developed most recently by Dan Hughes and Jonathan Baylin, it incorporates Playfulness, Acceptance, Curiosity, and Empathy. When discussion of the event or stressor is experienced by children in a lessserious or lighthearted manner they Playfulness feel less threatened and the deAcceptance escalation allows for more objectivity.

Acceptance is a means by which Curiosity judgement is set aside and parent and Empathy child can work together to understand the problem and adapt a response.

Curiosity serves to facilitate a pattern of discovery and understanding of the stressor and encourages the child to explore and make meaning of their own inner lives.

Finally, a consistent empathic approach to our stressed-out children helps to reduce defensive posturing and enables children to feel visible and valued.

PACE

Another benefit of letting go of the painful past is that we can avoid re-enactment of the trauma or re-traumatizing members of the family. If we visit that crisis every time we or they are triggered by some offense or challenge, the original insult is re-enforced and brought to foreground of our emotional coping responses. This tears away at our resilience and blocks our adaptation to effective management of the issue. The same is true for our children, as they relive all the emotional baggage and hurt that originally caused them pain. Rather, we try to avoid the baggage of over analyzing, blaming, and fixing that we are

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inclined to do to protect our loved ones from their pain and their development of an emotional boundary against it. As we and our children battle with the challenges of life we develop a pattern of coping with stressors that leads to a mature repertoire of adaptive mechanisms that will carry us towards that success that we want and need. If we can remove that baggage from our relationships, we will be much more likely to develop the “challenge orientation” and independence that equips us with strength, determination and motivation to be successful.

One last look at our response to the trauma of the inhibitor experience, involves training ourselves to listen more carefully with empathy even when we are hearing painful and negative comments. We can easily get caught up in the rolling catastrophe of an ER visit when a physician, nurse, or child welfare advocate want to second guess causation or a remedy for a problem we’ve been coping with on a daily basis. If we hear this often enough we respond emotionally and tend to stop listening and block the sometimes unspoken meaning of it. It might be coming from a sibling of our affected children in the form of a complaint about fairness or it could be a silent acceptance of their secondary status in the family. We have to remember that all feelings are real and listening for the meaning of comments rather than just the exact details of the spoken words will help us to keep on the path of open communication. When we develop a curiosity for the meaning behind other’s statements and behavior we can begin to truly understand their motivation and needs. We are in effect “PACEing” ourselves.

Our brain’s ability to adapt and develop new connections is referred to as plasticity and it continues throughout our lives. Those triggers and traumatic stress symptoms which have been created in our minds can be altered. If we take on the challenges to develop different responses to the stressors of having an inhibitor in the family, we can reduce the power of the symptoms and retrain our minds to overcome any adversity. Change is coming and if we get behind it in a positive way we’ll have more positive and resilient outcomes.

Here are several overarching and specific goals to keep in mind

Charlie’s Challenges: 1.

2.

3.

4.

5.

6.

7.

8.

Monitor and regulate our own affect and model self-regulation for others Observe how we’ve made the other persons feel Recognize, verbalize and validate the feelings of our loved ones Develop a reflective response to stressors: “What is this?”

Determine who owns the stressor, don’t take what isn’t yours from anyone Let go of the “painful past” and focus on the positive in the moment Avoid re-enacting the crisis and re-traumatizing ourselves and others Learn to listen with empathy without being triggered

Readings: American Psychiatric Association, 2013, Diagnostic and Statistical Manual of Mental Disorders, 5th ed., Arlington, VA. Brooks, R., Goldstein, S., 2001, Raising Resilient Children, Contemporary Books, Chicago, IL. Hughes, D., Baylin, J., 2012, Brain-Based Parenting, Norton, NYC. Kelley, L., Clement, P., 2010, Managing Your Child’s Inhibitor: A Practical Guide for Parents, LA Kelley Communications.

Masten, A., 2014, Ordinary Magic: Resilience in Development. The Guilford Press NYC.

Charles P. Gilbert II, LCSW is a Clinical Psychiatric Specialist with the Hemophilia Treatment Center of Central Pennsylvania in Hershey PA for the past 33 years. A Licensed Clinical Social Worker, he also maintains a small private practice in Palmyra, PA, working with individuals and families to overcome adversity in their lives. Over the course of his career Charlie has spoken and conducted workshops at various hemophilia and psychiatric meetings and Inhibitor Summits throughout the United States. He has always enjoyed working in the hemophilia community and greatly admires the resilience of all the families he has met.

FEATURE

Expl ring the Possibilities of Factor Factor Replacement Reflects the [Subhead] Protection Within Factor replacement reflects the protection within

[Header] Exploring the possibilities of Factor

By: Mike Denne, Shire

By Mike Denne, Shire To effectively manage your hemophilia, it is important to have an established treatment regimen that is backed by a long track record of results.1,2 Used for the treatment of hemophilia for more than 50 years,3 factor replacement therapies today have proven efficacy and established safety profiles.1 How factor treatment works When you have hemophilia, your body is missing certain proteins called factors. Without these proteins, your body is unable to produce a clot that is able to effectively stop or control a bleed. 4 Factor treatment replaces the missing proteins in your blood, working within the natural bloodclotting process to help create a stable clot.4,5 The natural blood-clotting process is quite complicated and involves a delicate balance of activation (on) and inactivation (off) steps.

When a bleed occurs, the blood-clotting process is turned on, activating the factor proteins to help stop the bleed.

Once a bleed is controlled, the process is turned off, inactivating the factor proteins.5,6

Factor replacement has the additional benefit of providing an appropriate level of bleed control through this inactivation step, which means there is a lower chance of developing a clot (called thrombotic risk).5,6 Factor treatment has decades of real-world use that demonstrate a low risk of developing these thrombotic events with factor.7

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A comprehensive treatment approach Factor treatment is the only treatment that can be used in multiple scenarios to meet different treatment needs.

•

Prophylaxis treatment is the routine infusion of factor with the goal of preventing bleeding episodes before they happen.4 On-demand treatment is infused to control bleeds once they begin. 4

•

Factor can also be used to help prevent and manage bleeds during or after surgery.8

•

Because factor is infused intravenously (IV), the proteins for clotting are immediately available for use, allowing for bleed protection when you need it.9 Achieving an individual peak Factor level10* 100

TARGET PEAK FACTOR LEVELS %

When developing your treatment plan, your healthcare provider (HCP) will take a close look at your lifestyle and other activities, as these can have an impact on your bleeding risk.10 According to the World Federation of Hemophilia, the need for prophylaxis should be assessed before participating in any high-risk activities, like sports that require physical contact with other players or equipment.8

80 60 40

20

0 0

DOSING REGIMEN

*This chart is a visual representation only. It does not show actual patient data.

Maintaining optimum factor coverage is important during times of certain physical activity, trauma, and surgery.11,12 By understanding how each person’s body responds to Factor, your HCP can adjust dose and time(s) of infusion, to ensure that you are receiving optimum coverage whenever you need it.13 Why joint health matters Prophylaxis is not just about preventing bleeds. Did you know that prophylaxis also helps prevent long-term joint damage?14 All bleeding, even bleeding you may not feel or see (defined as subclinical or microbleeding), can result in significant joint damage.15 Your joint health is very important—remember that every bleed matters.14,15 A range of clinical studies and long-term data have shown that starting prophylaxis early with Factor replacement can prevent joint damage and preserve joint health in some patients with hemophilia A.15-19 Staying involved and sticking to your prophylaxis routine is currently the most effective strategy in protecting your joints and reducing bleeds.20

BLOODLINES

Partner with your doctor To help stay on track with your treatment regimen, work with your HCP on setting clear treatment goals.21 These goals can motivate you to strive toward achieving positive long-term outcomes.

Having an incentive is also a nice way to help yourself stay on track. 22 An optimal treatment plan can be successfully maintained with an appropriate amount of effort from both the HCP and the patient.20,21 To effectively manage your hemophilia, be sure to ask your HCP about the established treatment that many patients continue to rely on. With decades of documented success in managing, controlling, and reducing bleeds in more than 200 clinical studies, Factor treatment remains a trusted and well-studied treatment option in people with hemophilia.1-3

Nearly

200

clinical studies2

Used for over

50 YEARS

3

BLEEDREDUCTION benefits1

Commitment to our hemophilia community is always a priority for Shire. As a leader in hemophilia research, Shire continues to innovate on your behalf, developing programs and services that support your efforts each step of the way. Shire is focused on providing advanced hematology treatments for today and innovating for the future.

Visit www.bleedingdisorders.com to learn more about how factor works and all the ways it can help patients like you living with hemophilia.

References: 1. Franchini M, Mannucci PM. The history of hemophilia. Semin Thromb Hemost. 2014;40:571-576.

2. NIH Clinical Trials Registry. Ongoing and complete clinical trials using Factor in patients with hemophilia. https://clinicaltrials.gov/ct2/results/details?term=Factor+VIII&recr=Closed&fund=2. Accessed July 17, 2018.

3. Center for Biologics Evaluation and Research. User fee billable biologic products and potencies approved under Section 351 of the PHS Act. http://www.fda.gov/AboutFDA/CentersOffices/OfficeofMedicalProductsandTobacco/CBER/ucm122936.htm.

Accessed July 30, 2018.

4. Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388:187-197.

5. 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/?rendertype.

Accessed July 24, 2018.

6. Colvin BT. Physiology of haemostasis. Vox Sang. 2004;87(suppl 1):S43-S46.

7. Coppola A, Franchini M, Makris M, Santagostino E, Di Minno G, Mannucci PM. Thrombotic adverse events to coagulation Factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies. Haemophilia. 2012;18:e173-e187.

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8.

9.

10.

11.

12.

13.

14.

15.

16.

17.

18.

19.

20.

21.

22.

World Federation of Hemophilia. Guidelines for the Management of Hemophilia. 2nd ed. World Federation of Hemophilia, Montreal, Quebec, 2012.

Canadian Hemophilia Society. Factor replacement therapy. http://www.hemophilia.ca/en/bleedingdisorders/hemophilia-a-and-b/ the-treatment-of-hemophilia/Factor-replacement-therapy/. Accessed September 20, 2017.

Valentino LA. Considerations in individualizing prophylaxis in patients with haemophilia A. Haemophilia.

2014;20:607-615.

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.

Iorio A, Iserman E, Blanchette V, et al. Target plasma Factor levels for personalized treatment in haemophilia: a Delphi consensus statement. Haemophilia. 2017;1-10.

Collins PW, Fischer K, Morfini M, et al. Implications of coagulation Factor VIII and IX pharmacokinetics in the prophylactic treatment of haemophilia. Haemophilia. 2011;17:2-10.

Bertamino M, Riccardi F, Banov L, Svahn J, Molinari AC. Hemophilia care in the pediatric age. J Clin Med. 2017;6(54):1-13.

Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med. 2007;357(6):535-544.

Price VE, Hawes SA, Chan AKC. A practical approach to hemophilia care in children. Paediatr Child Health.

2007;12(5):381-383.

Manco-Johnson MJ, Soucie JM, Gill JC; for the 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.

Khawaji M, Astermark J, Von Mackensen S, Akesson K, Berntorp E. Bone density and health-related quality of life in adult patients with severe haemophilia. Haemophilia. 2011;17:304-311.

Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. Haemophilia.

2012;18:8-16.

Berntorp E. Differential response to bypassing agents complicates treatment in patients with haemophilia and inhibitors. Haemophilia. 2009;15:3-10.

Schrijvers LH, Uitslager N, Schuurmans MJ, Fischer K. Barriers and motivators of adherence to prophylactic treatment in haemophilia: a systematic review. Haemophilia. 2013;19:355-361.

Thornburg CD, Duncan NA. Treatment adherence in hemophilia. Patient Prefer Adherence. 2017;11:16771686.

Š2018 Shire US Inc., Lexington, MA 02421. All rights reserved. 1-800-828-2088.

SHIRE and the Shire Logo are registered trademarks of Shire Pharmaceutical Holdings Ireland Limited or its affiliates.

S42106 08/18

BLOODLINES

Anxious?

How About Running Toward Your

? y t e i x An

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by Dr. Gary McClain, PhD

L

iving with a chronic condition gives you all kinds of reasons to feel anxious. Do I need to go through a list with you? Probably not. Humans don’t do well with uncertainty. And chronic conditions can bring all kinds of uncertainty to your life. Having to face a test or a procedure. Adjusting to changes in your daily life.

Thinking about the future. And stress. All of this can lead to anxious feelings. So, what do you do about those anxious feelings? Suffer until they hopefully go away on their own? Try a technique and hope it works? You’ve got more options than that. Coping with anxiety begins with how you think about anxious feelings as well as preparing yourself for the anxiety that might come your way.

FAMILY MATTERS

Here’s how to get started:

Look for the lesson

Accept that anxiety is part of life When I was in training to be a therapist, one of my teachers said this at the beginning of a lecture on working with anxious clients: “You’re anxious. So what!” Look around you at the world news, your community, your family and friends. You could say that any given day brings lots of reasons for anyone to feel anxious. That doesn’t mean you have no choice but to be continuously overwhelmed with anxiety. Not at all. The point is that unless you’re comatose, you’re probably going to experience some anxious feeling from time to time, or often. It’s normal, and you’re not alone.

Is there something you can learn from those anxious feelings? In other words, could some of that anxiety be a message from your mind that it’s time to take better care of yourself or to make a change for the better in your life?

Successful people often talk about how they use their anxiety to motivate themselves to work a little harder to be excellent. Clients living with chronic conditions often tell me that feeling a little bit anxious helps motivate them to stay compliant with their treatment and self-care routine.

This isn’t to say that’s all that anxiety is good for you. But if there’s lesson there, then learning it might help you to reduce some of that anxiety.

Don’t get anxious about getting anxious

Lean in to your anxiety Here’s a mindfulness exercise for you. Visualize yourself hiding in your house with all the doors and windows locked and anxiety standing outside on the porch, pounding on the door to get in. And then, imagine opening the door and saying: “Come on in, anxiety. Let’s sit down and talk.” If the anxiety is not going away on its own, you might as well see if the two of you can come to an understanding.

Nobody likes being anxious. But I often find with my clients is that they dislike anxiety so much and are so afraid of being overwhelmed by their anxious feelings, that when they feel anxiety coming on, or worry that they might get anxious later, they get anxious about the anxiety. They do this by worrying about how bad their anxiety might be.

They create anxious stories about what might happen.

They get anxious about how they’re going to cope with the anxiety. And guess what? They end up creating more anxiety for themselves. Watch out for negative self-talk that helps to create more anxiety.

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Talk to yourself When anxious thoughts and feelings start building up in your mind, talk yourself off the ceiling. Remind yourself that it’s normal to be anxious sometimes. Ask yourself what you can do for yourself to reduce your anxious feelings. When your mind tosses those scary stories and images at you, talk back to them: “Bad things can happen but so can good things. I can’t predict the future.” When you stop fighting anxiety, you free yourself up to focus your energy on what you can do about it.

Calm yourself down Think about what helps you when you’re feeling anxious. Imagining a relaxing place like the beach?

Taking a walk? Listening to music? Reading a book? It might help to put a tool kit together with the calming techniques that work for you, and have them ready for when you feel anxious.

Be proactive Take a look at recent days when you have felt less anxious to find clues for how to avoid anxiety. Was there something you did or didn’t do that day that might have helped you to have a more relaxed outlook? Were you compliant with medication? Following your diet? Staying active? In touch with supportive people? One of the best ways to cope with anxiety is to not set yourself up to feel that way by building anxiety prevention into each and every day. Again, apply the lessons that anxiety can teach you. As they say, an ounce of prevention is worth a pound of cure.

Talk to somebody Get some perspective. Sit down with someone who can listen without judging you or telling you what to do.

Talk about what’s making you feel anxious. Sometimes just saying it out loud can help you to see what’s real and what’s being manufactured by your imagination. Ask for some encouragement. And maybe some accountability if you want to make some changes for the better.

And reach out for help if you need it If you are feeling overwhelmed by anxiety, if it is interfering with your ability to do what you need to do to take care of yourself, if it is getting in the way of your relationships, or if it is making it hard for you to make decisions, then it may be time to reach out to a mental health professional. Talk to your doctor if you aren’t sure where to start. Don’t go through this alone.

Anxious feelings are part of being human. Lean in to your anxiety. Listen for the lesson anxiety can teach you.

And take good care of yourself.

FAMILY MATTERS

Remember that feelings may not represent reality. The feelings of the moment can make the world around you look pretty foggy. You may see things that aren’t real, like what someone else’s motives look like, or where a situation seems to be going. All the more reason to take a pause until the fog dissipates.

Keep Calm Withdraw if you need to. Move yourself out of the center of the action. Physically, mentally, or both. Step back. You can even say something like, “I need a moment.”

Use your breathing. That initial emotional rush can be overwhelming. Notice how you’re breathing. Little short breaths? Not breathing at all? Take a series of deep, calming breaths. In through your nose, out through your mouth.

Do things that calm you down. Listen to music. Take a walk. Read. Do some relaxation exercises. Anything that helps you to stay calm, that helps you feel connected to your center. Calming yourself down is essential if you are experiencing symptoms of PTSD. Don’t fight your feelings. Okay, so you’re having a lot of feelings. The worst way to cope with emotions is by pushing them down or trying to pretend they aren’t there. Your feelings are your feelings. Good feelings, feelings you aren’t so proud of. Give yourself permission to feel them all.

Try to be patient with yourself. Emotions are part of being human. Our way of coping with strong emotions is hard wired into us, based on years of practice. Some of us shut down, some of us wear our emotions on our sleeve. Learning how to cope with emotions in a healthier manner takes practice. And keep in mind, if you’re living with a chronic condition, you have that much more on your plate. So be patient with yourself.

Most of All, Stay Supported…

If you’re living with a bleeding disorder and an inhibitor, you already know the value of staying connected to your support network. If you are experiencing strong emotions, and especially if you suspect you are experiencing PTSD, it is important to get emotional support, professional support, family support, friends, and other members of the inhibitor community. The same if you are a caregiver. If you are a family member of someone you suspect may be experiencing PTSD, it’s important to get support for both your loved one and for yourself. Don’t go through this alone!

You, your chronic condition, and your mental health.

You’re dealing with a lot. And one stressful situation followed by another can take a big toll on your mental health. If it’s all starting to wear you down, do the bravest thing in the world and ask for HELP. PTSD, like other mental health conditions, is treatable.

Gary McClain, PhD, LMHC, 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 and Chronic Communicationssm. He maintains a Website, www.

JustGotDiagnosed.com.

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FAMILY MATTERS

Shining L ght

by Lisa Cosseboom, M.Ed. & CAGS School Psychologist & Special Education Evaluation Team Chairperson

on

F

or this article, we asked our inhibitor families to share stories of challenges they faced with their local school systems. We thank you for sharing! We applaud your tireless efforts to access equals rights to a free appropriate public education for your child. It can be a frustrating landscape to navigate! Our school psychologist, Lisa Cosseboom shares some solutions for challenging situations.

1

My child is in a wheelchair temporarily. Can the school suggest he stay home until the wheelchair is no longer needed?

a 504 plan for him before he enters high school, but the school refuses to write one.

NO! You cannot exclude a child from school based upon a disability, temporary or otherwise. If it were a permanent condition, the school district must provide wheelchair accessibility including transportation, handicap bathroom access, elevator, ramps etc.

Additionally, the school must provide alternative/modified physical education during which the child is unable to participate fully.

3

My child is in private school. What accommodations or services can be provided for my child there?

Unless receiving any type of federal funding, the school is not required to provide accommodations and/or services.

However, you can certainly request a meeting with administration to see if they would be willing to entertain providing accommodations as needed. Sometimes, approaching a school with a positive attitude and reasonable request will work. (I.e. you get more with sugar than spice.)

4

2

As part of an agreement with our public school, my child attends a private school at their expense as it met his needs better than the public system. We have been trying to get

27

Unfortunately, unless that private school is receiving federal funding in any capacity (i.e. Title 1 funding) they are not required to follow the Americans with Disabilities Act regulations. If he were entering a public high school, the school district would be required to hold a 504 eligibility meeting.

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My child sees the adjustment counselor at our school for anxiety issues related to his bleeding disorder. Can the school system charge me personally for those services?

Absolutely NOT! Services provided in the public school system, by a public school employee are covered by your taxes! If your child is seeing someone outside of the

WHAT’S the PLAN?

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.

5

All rights reserved.

public schools for counseling in school by an agency, then your insurance would cover the cost.

The ADA coordinator at our elementary school invalidated my child’s 504, indicating that absences and injuries would be evaluated on a case by case basis to prevent abuse of services.

This is a clear civil rights violation. Public schools are required to provide appropriate accommodations to a qualified student who has a disability so that child can participate and access their education. Any child who has a disability that “substantially limits one or more major life activities” which includes education. They cannot “invalidate” a 504 plan because of injury or absences. I would suggest you meet with the principal or higher administrator in the district. If possible, I would suggest you acquire an advocate.

CM-FIX-0258

has a bleed and can manage on her own. How can I get the school to put a 504 in place?

Regardless of your daughter’s independence in caring for her bleeding disorder, she should have a plan in place.

A meeting should be held for qualification and you will need to provide medical documentation. There could be times that your daughter may need to access the nurse without question from teachers or scrutiny.

6

The school my daughter attends, put a 504 on hold for my daughter because she doesn’t go to the nurse when she

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WHAT’S the PLAN?

?

?

?

?

?

he bleeding disorders community is currently experiencing one of the most rapid growth periods since the 1990’s when recombinant products hit the market.

Corporate buyouts bring changing companies, some of whom are completely new players to the bleeding disorder community. New products that provide the convenience of subcutaneous administration are here, with more coming, gene therapy for FIX may be just around the corner, with FVIII not far behind.

Women’s bleeding symptoms are finally being recognized. It is truly a dizzying pace and we do this for a living! With each of these advances however, the benefit-risk profile for any and all new products and treatments must be first and foremost in our minds.

So, what is going on?

Gene Therapy A cure in our lifetime was the refrain when my brother was born in 1970. Multiple attempts at gene therapy have occurred in the last 4 decades, but it is clearly looking to become a treatment option in the next five years, especially for Hemophilia B. Companies such as Spark and BioMarin are on the leading edge.

?

No one is saying yet that hemophilia will be cured. Currently, gene therapy — which uses a virus to deliver a new gene to cells — can only be used once. If it stops working, the patients lose the benefits. For now, “we are anticipating that this is a once-in-a-lifetime treatment,” said Dr. Steven Pipe, director of the hemophilia and coagulation disorders program at the University of Michigan and a lead investigator of a clinical trial conducted by the biotech company BioMarin (https://www.nytimes. com/2018/08/13/health/hemophilia-genetherapy.html)

? ?

?

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Many questions exist however, regarding its long term-safety and efficacy. The FDA has a draft document that’s currently open for comments, entitled Human Gene Therapy for Hemophilia at:

?

?

?

The Future’s T So... BRIGHT ?

by Janet Brewer, M.Ed

Fall/Winter 2018

https://www.fda.gov/downloads/ BiologicsBloodVaccines/ GuidanceComplianceRegulatoryInformation/ Guidances/CellularandGeneTherapy/

WHAT’S NEW?

The document covers considerations for product development, consideration for FVIII/FIX activity measurement assessed by different clinical laboratory assays, expedited programs, communication with the FDA, considerations for preclinical studies, and considerations for clinical trials to include:

procedures, should be part of the approval process. Our community has a well-documented history of what can go wrong when products that are considered “life changing” are introduced into our treatment regiments.

• Efficacy Endpoints • Study Population • Study Monitoring

For many in the inhibitor community, Hemlibra has proven to be a game-changer in care. There is no doubt it has decreased bleeding and consequently increased quality of life for inhibitor patients where nothing else has worked.

The convenience of subcutaneous treatment after years of port therapy and complications has led to unprecedented recommendations for its use.

• Study Design • Statistical Considerations • Patient Experience

Increased patient monitoring efforts that are wellestablished and enforced with clear cut guidelines for what constitutes an adverse event with proper reporting

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Hemlibra

Genentech announced the release of Hemlibra to hemophilia A patients without an inhibitor on October 4, 2018. This comes less than a year after its approval on November 18, 2017 for the treatment of hemophilia A patients with an inhibitor.

During Dr. Stacy Croteau (Boston Children’s Hospital) and Dr. Lindsey George’s presentation, Update on New Therapies and Gene Therapy Trials in Hemophilia for physicians at NHF, October 18th-21st, 2018, they explained, emicizumab/Hemlibra is a hot topic.

The HAVEN 3 noninhibitor patient study also shows that “emi” (emicizumab) is better than treating on demand. However, there were adverse events occurring in at least 5 percent of participants including injection site reaction, headache and diarrhea. Dr. Croteau also concludes that there is “significant laboratory monitoring challenges and (a) lack of understanding of effective hemostasis provided by emi alone and that more information is needed concerning those who have had major trauma or surgery.”

Dr. Glen Pierce concludes that, “questions remain; while it mimics FVIII in bringing FIX and X together, it doesn’t simulate other functions of FVIII.” The American Thrombosis and Hemostasis Network (ATHN) announced the launch of ATHN7: Hemophilia Natural History Study Overview at NHF this October. The study’s intent is to follow a group of people over time to collect health information from patients to understand how bleeding and clotting disorders like hemophilia develop and to design effective treatments.

According to Dr. Tyler Buckner, one of the lead researchers at Hemophilia and Thrombosis Center, University of Colorado, “ATHN 7 is looking to find out whether non-factor replacement products are safe when used as a preventative measure for patients who have inhibitors as well as for those who don’t. We’re also going to be evaluating the effectiveness of non-factor products*, bypassing agents, or coagulation factor replacement products when used for prevention and for surgery in patients with hemophilia.” - NHF Daily Want to participate? Hemophilia A or B patients of any severity with or without an inhibitor are eligible through their hemophilia treatment center over a multiple year period. *For the purposes of this study, “non-factor products are those that became commercially available in the US after January 1, 2017.”

My Life Our Future Until the end of 2017, My Life Our Future (MLOF), originally supported by ATHN, Biogen, Bloodworks Northwest and NHF gathered genotypic data from 9,000+ hemophilia A and B patients and carriers. Your support of this invaluable data collection contributed to the MLOF Research Repository, the largest hemophilia scientific resource of its kind in the world.

Over 4,000 genetic variations in hemophilia were discovered as a result of this research, which will hopefully answer questions such as, which variants increase the risk of inhibitor formation, or why bleeding severity varies from person to person? Some of the research projects that will be explored using this repository include: Inhibitor Development, Bleeding Specific to Hemophilia A and Hemophilia B, FVIII clearance, and Carriers.

Doctors Hong Yang and Zuben Suana of the FDA’s Center for Biologics Evaluation and Research (CBER) will utilize MLOF data to create a mathematical model to predict the body’s immune response to treatment. Once validated, it could be used to evaluate new FVIII drugs, project the risk of the development of antibodies, and help determine appropriate treatment for newly diagnosed patients. Eventually, it may also help inform development of specific treatments for people with different types of hemophilia A. - NHF Daily October 13, 2018

WHAT’S NEW?

Talk to your healthcare provider to FIND OUT IF KOĀTE IS RIGHT FOR YOUR CHILD.

© 2017 Kedrion Biopharma Inc. All Rights Reserved. June 2017 KT-0514-00-2017B

Want to know more about MLOF? Visit www.MyLifeOurFuture.org

It is truly an exciting time in the bleeding disorders community! Since 2017, CHES has had the great fortune to collaborate with Dr. Diane Nugent, CHOC and her team from The Center for Inherited Blood Disorders in Orange, California and Hematology Advanced Diagnostic Lab (HADL) to offer whole gene molecular sequencing for our FVII patients at the Factor 7 Retreat initiated in 2012.

The overarching goal was to provide molecular diagnosis and variant identification for patients and family Dr. Diane Nugent, CHOC (Children’s members with Hospital of Orange County) FVII deficiency to speaking at CHES’ Factor 7 Retreat

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ascertain why bleeding symptoms and severity levels do not correspond. The discoveries made as a result of this project have led to abstract presentations at both World Federation of Hemophilia (WFH) and International Society of Thrombosis and Hemophilia (ISTH) 2018. This preliminary research clearly indicates that the factor VII levels do not correlate with the bleeding phenotype and that there are mutations that are more closely associated with increased bleeding, regardless of factor level.

So, what does this have to do with hemophilia and inhibitors you may ask? There is still so much we do not know in the world of bleeding disorders and many think we are just scratching the tip of the iceberg. We are caught up in a wave of new science, new studies, new products which are all very exciting! New advancements also create multiple unknowns. In this time of significant change, continue to do your research, reflect on our community’s history, ask questions and don’t hesitate to speak up if a decision just doesn’t feel right for you. The choice to use any product or protocol is yours, alone. After all, it is your own or your child’s body you are making decisions about and those choices can have benefits or consequences for a lifetime.

WHAT’S NEW?

Overview of

Mindfulness and

by Krystyn Strother

Research-Based Physiological Benefits In past issues we’ve talked about the general benefits mindfulness can have on our general well-being, reducing stress, coping with chronic pain, but what about the physiological effects of mindfulness?

L

et’s define mindfulness as a moment-to-moment awareness of your environment and experience without judgement. It is important to highlight that mindfulness, when we talk about it this way, is not a character of your personality, it is an active state that you engage in. In other words, being a “mindful” person is not the same as practicing mindfulness. For our intents and purposes, mindfulness is an intentional practice, be it 30 seconds or 30 minutes.

Photo courtesy of krystynstrother.com and Stark Photography

When our “thoughts and feelings are observed as temporary events in the mind, instead of a reflection of oneself or reality,” we are better, and more easily, able to access calmness, clarity, and concentration (Russell, et al., 2018).

The majority of research focuses on the effect mindful meditation has on the brain and body and explores the connection between the two. Below is a highlight of compelling research showing the physiological effects formal and informal mindfulness meditation practices have.

How Does Mindfulness Affect the Body?

In 2010, Hoffman et al. looked at 39 studies exploring the use of mindfulnessbased stress reduction and mindfulnessbased cognitive therapy and concluded that practicing mindfulness may be useful in changing physiological processes that are at the root of many clinical issues, stress. They saw a pattern that was consistent with other evidence that mindfulness meditation increased positive physiological experiences, decreased anxiety, and decreased negative physiological experiences.

Mindfulness has the ability to initiate a relaxation response in the body. When we experience a stressful situation, the brain begins a cascade of events that affect the body called the stress response. The brain, signaling the rest of the body through the autonomic nervous system, initiates a host of involuntary functions such as increased breathing and heartbeat, and elevated blood pressure. That signal from the brain triggers a release of hormones from the adrenals that intensify the physiological changes already taking place and affects the endocrine, sympathetic nervous and immune systems. Physically, the stress

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response shows up as increased breathing, heartbeat, and elevated blood pressure. Those hormones have other effects on the body potentially contributing to insomnia, digestive disorders, mental health issues, heart disease, fertility, and weight gain.

getting the body back to its baseline levels after a stressful event. The heart and respiratory rates are lowered, blood pressure drops, and tension in the muscles is released. All of those stress hormones that were released-those are greatly reduced as well.

Think about this cascade being initiated every time you encounter something stressful. It is no wonder chronic stress has such a profound impact on our health and wellness!

Mindfulness can give rise to the relaxation response by engaging the part of the nervous system responsible for

Research shows that mindfulness meditation not only promotes awareness and understanding of one’s own thought process but also has many broad-ranging benefits from weight loss, managing inflammation, and emotional wellbeing.

MIND BODY CONNECTION

Manage Inflammation & Prevent Disease Looking at recent research; a number of leading institutions are finding a link between the brain and our immune systems. Scientists at the Center for Healthy Minds University of Wisconsin-Madison have been studying the mind-body connection and the idea that the brain and the immune system work in conjunction with one another. Their theory is that the brain influences the body’s inflammatory responses, and our ability to train the brain to intervene on those responses might prevent some chronic disease.

Weight Loss & Disordered Eating Many studies have shown that mindfulness meditation has a great effect on the area of the brain that regulates emotions and perspective, making it easier to let negative thoughts come and go without dwelling on them. Furthermore, there are a handful of studies pointing to mindfulness meditation as an “effective intervention for emotional eating.” A 2016 study conducted

by UMass Medical School Center found that mindfulness meditation helped reduce emotional eating behavior.

Cellular Changes A study published in the journal Cancer by the Tom Baker Cancer Center in Alberta, Canada suggests that mindfulness meditation can influence, “key aspects of your biology.” In this particular study, researchers were looking at the effect of mindfulness on telomere length.

Telomeres are protective lengths of protein at the ends of chromosomes and can be used to determine how quickly cells age. The subjects that participated in mindfulness training on a weekly basis over a threemonth period showed preserved telomere length, while the subjects that did not learn or practice mindfulness had shorter telomeres. While more research is currently being done, the idea here is that folks in this study who engaged in mindfulness meditation may have more resilient cells.

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Emotional Reactivity Many of the physiological benefits of mindfulness meditation take place within the body in ways that aren’t immediately recognizable. A 2007 study reported a decrease in emotional reactivity among participants that had anywhere from one month to 29 years of mindfulness meditation practice.

Mindfulness practices stimulate and increase the density of neurons in the area of the brain that regulates emotions and decreases neuron density in the region of the brain responsible for initiating the stress response. That means, the more we engage in mindfulness practices the greater our ability to regulate emotions without initiating the stress response.

Studies suggest that our minds and bodies can affect one another, and that the benefits of mindfulness meditation have profound physiological and psychological effects. More research is being done to better understand how these benefits are achieved in the body.

There are many paths to approach mindfulness and what works for you may not work for your neighbor, partner, or child. Yoga, Tai Chi, formal and informal mindful meditation are just a handful of ways to practice mindfulness. Consider mindfulness meditation as a preventative strategy to help manage how your brain responds to events taking place in the body.

1.

Russell, L., Ugalde, A., Milne, D., Krishnasamy, M., O, E., Austin, D. W., . . . Livingston, P. M. (2018). Feasibility of an online mindfulness-based program for patients with melanoma: Study protocol for a randomised controlled trial. Trials, 19(1). doi:10.1186/s13063-018-2575-x

2.

Hofmann, S. G., Sawyer, A. T., Witt, A. A., & Oh, D. (2010). The effect of mindfulness-based therapy on anxiety and depression: A meta-analytic review. Journal of Consulting and Clinical Psychology, 78(2), 169-183. http://dx.doi.org/10.1037/a0018555

3.

https://centerhealthyminds.org/news/mind-over-matter-mappingthe-two-way-street-of-the-brain-and-immune-system?utm_ content=63584016&utm_medium=social&utm_source=twitter

4.

https://www.ncbi.nlm.nih.gov/pubmed/27890474?utm_ content=63208938&utm_medium=social&utm_source=twitter

5.

https://www.sciencedirect.com/science/article/pii/ S019566631630825X?via%3Dihub

6.

https://www.sciencealert.com/world-first-evidence-suggests-thatmeditation-alters-cancer-survivors-cells

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 evidencebased program sponsored by the Department of Anesthesiology at OHSU. Read more about Krystyn at krystynstrother.com

MIND BODY CONNECTION

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 Gabriel Anspaugh Janet Brewer, M.Ed Charlie P. Gilbert II, LCSW Lisa Cosseboom, M.Ed., C.A.G.S Mike Denne, Shire Crystal James Dr. Gary McClain, PhD Krystyn Strother

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