Lifelines for Health (vol 10): Clinical Trials 101: Understanding the Process and the Terminology

A CHES Publication

Winter 2017 I Volume 10

Revolutionary Solutions for Needle Phobia

New Sub-Q Treatment is FDAApproved for Inhibitors

POWER

PAIN

Importance of Bypassing Agents

When Someone You Love Hurts.

How Can You Help?

Chronic Pain and Mindfulness The Individualized Health Care Plan.

What’s It All About?

Discover

Learn more about our focus on the rare bleeding disorder community HEMA Biologics™ is a privately held biopharmaceutical company exclusively focused on meeting the needs of patients living with the rarest bleeding disorders, supporting the broader community that cares for them, and bringing them meaningful products and services.

Our two investigational product candidates are in development for the potential treatment of hemophilia A or B with inhibitors and severe von Willebrand disease.

Our primary goal is to advance the treatment and management of these conditions in the US to positively affect patients’ lives.

“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 HEMA014-1216

Letter From the Editors Does it seem like the years are going by faster and faster? It is hard to believe we are getting ready to close out 2017! LifeLines for Health became a winter edition, as opposed to our planned fall edition due to a technology glitch. We promise you though it is still jammed with information to enhance your lives. Inhibitor Family Camp, Leverage and Momentum programs this year continue to provide us with valuable insight on what YOU, our inhibitor family needs. It is our honor to provide these programs to our community.

We remain humbled by your experiences, your resilience and your grace as you meet each challenge head on. In 2018, we are preparing to add an additional family camp program for those with a child 0-18 with an active inhibitor. Sadly, we are saying good-bye to Victory Junction to enable us to offer After the Shock. The Serious Fun Network’s primary camper age minimum restricts us from offering camp to those under 6. Through After the Shock, we endeavor to create that inhibitor family support for those struggling with a new diagnosis.

CHES’ mission is to inspire awareness and self-reliance for patients with chronic health conditions, their families, and their communities. Accuracy, empathy and integrity are the core principles of our company. Our programs are meant to augment the wonderful resources currently available in this community and are free of charge to attendees. There is no marketing or sales representatives at any of our inhibitor programs, just the way you like them!

Pain management is an all-consuming part of our daily lives in the inhibitor community.

Tighter restrictions on narcotics prescriptions penalize those that truly need them. We have heard many stories of addiction in this community, for medications to ease pain, which become easy to abuse. Most of this edition is structured around how to manage pain for yourself or assist a loved one. Our Feature includes the cognitive cycle pain creates by affecting emotions, behaviors and thoughts. The Mind-Body Connection offers mindfulness strategies well-based in research that “take the suffering out of pain”. It is how we think of pain that dictates how we live it-definitely a new way to think of it. What’s New focuses on exciting tools that have been developed, some available commercially that assist with needle phobia using ice and vibration. A virtual reality system that uses distraction in a very innovative way!

In Family Matters, practical, daily strategies offer us ways to assist our loved ones when all we want to do is help, but that help may not come across as helpful. If you have a school age child, do they have an IEP? 504 plan? Individualized Health Care Plan? IEP’s and 504 plans are well known. There are multiple resources on how to obtain one, knowing the difference between the two, even sample 504s to assist in the process. What’s the Plan discusses a written plan that instructs school personnel on the correct procedures and protocols that must be implemented when your child has a bleed. If you don’t have one, you need one!

As individuals managing an inhibitor, we are constantly evaluating treatments to manage an inhibitor. Genentech’s announcement for FDA approval and manufacturing for their new product, HemLibra in November, finds our community in a state of excitement and caution. In Bloodlines, we review the well-studied history, efficacious use of aPCC therapy. CHES neither endorses or recommends any inhibitor treatment, as we are an education company, not physicians. 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, trusted family/friends and your child if they are old enough to understand.

Lastly, we leave you with the inspirational side of life with an inhibitor. Community Chatter brings our writer’s personal perspective about events and programs specific to the inhibitor community. We look forward to their reflections in every edition.

As we enter 2018 in a world that seems upside down, continue effecting change where you can. When you receive emails from your local chapter, HFA or NHF, take the time to make the calls. Our very future as a community is in the balance. Be cautiously optimistic, but never forget the lessons painfully learned.

Best wishes for a beautiful holiday season and a new year of hope, peace and community.

Do you have an idea, comment or suggestion? We really want to hear from you! Share your thoughts at info@comphealthed.com.

- Janet Brewer & Eric Lowe “If a way to the better there be, it lies in taking a full look at the worst.” - Thomas Hardy jbrewer@comphealthed.com l elowe@comphealthed.com

Did YOU Know?

In 2018, CHES plans to present a new inhibitor program entitled After the ShockSM, and splitting another, LeverageSM, into two - adding a total of 2 new programs for the inhibitor community next year.

(1) After the Shock will be our first inhibitor-based program for individuals with inhibitors who are under the age of 6. The entire immediate family is invited to attend.

(2) Leverage is an adventure-based camp for individuals with inhibitors ages 18 and up. Although this program is designed for those who live with it, feedback tells us that the planned activities leave some individuals feeling hesitant to attend. Therefore, we’re offering a shorter introductory version, Leverage 1.0 and renaming the traditional Leverage program Leverage 2.0.

A calendar of our events is on pages 6 and 7. More info on all of our programs is available on comphealthed.com.

LifeLines for HealthSM Disclaimers The views and opinions of our writers are not a reflection of Comprehensive Health Education ServicesSM, 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.

Integrity, Accuracy, Empathy...

FEATURE 14 I Pain and the Power to HEAL Pain Psychologist, Georgia Panopoulas, PhD, LP explains how pain is processed in the body, and how it’s perceived in the mind.

Discover how cognitive thoughts, emotions, and relationships are all connected to pain, and how these connections can be used to manage pain with many different approaches.

CONTENTS COMMUNITY CHATTER 8 I LEVERAGE: a Molehill Practice for a Mountainous Adventure Justin Levesque (with an inhibitor) was selected to embark on a colossal voyage. But he had serious reservations about his safety and performance. See how Leverage reassured him.

11 I Taha’s Moment: Finding “Normal” Taha Amir understands seclusion all too well, thanks to his lifelong inhibitor. Discover his pathway to a new outlook on life through Momentum, a program for men with inhibitors.

12 I What Inhibitor Family Camp Means to the Cook Family Cheryl Cook has brought her family to Inhibitor Family Camp (IFC) three times now. Each with its own special milestones.

She shares with us what IFC has meant to her and her family.

13 I Family Tradition with Modern Change Inhibitor Family Camp was started back in 2010. Every family reunion comes with its own set of traditions. Read how three of our IFC families upgraded a tradition in a big-hearted way.

BLOODLINES 22 I Importance of Bypassing Agents In this exciting time of so many new and improved products, don’t forget the essentials! For the inhibitor population, bypassing products can still be a lifeline to survival.

26 I New Sub-Q Treatment is FDAApproved for Inhibitors It has been the talk of the community. The study drug formerly known as ACE910 is now FDA-Approved, frequently referred to as the Game Changer.

27 I Eloctate Could Be an Option for ITI This FVIII, extended half-life drug stirred up a lot of excitement when it hit the market, promising longer coverage with less infusions. But it had no place in the inhibitor population.

Fortunately, some studies are saying otherwise.

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FAMILY MATTERS 28 I When Someone You Love Hurts.

How Can You Help?

When someone is hurting, it can be difficult just to approach him/her. We don’t want to make things worse by being bothersome or saying the wrong thing. Dr Gary McClain has some pointers to get you through these challenges.

WHAT’S the PLAN? 37 I The Individualized Health Care Plan. What’s It All About?

You have your IEP or 504 in place, so you’re all set, right?

Wrong. It’s natural to focus on the safeguards that will help your child learn just as his/her classmates. But don’t forget about treatment plans for medical needs in the school.

WHAT’S NEW 42 I Virtual Reality: A Painless Distraction Needle phobia is a reoccurring challenge for parents, patients, and medical staff. Nationwide Children’s Hospital took that challenge head-on by developing a virtual reality system to distract their pediatric patients.

44 I ITP Poker Club This support program was created by the Platelet Disorder Support Association for children with Immune Thrombocytopenia to cope with needle phobia. Tools used include poker chips, distraction cards, and a device called the Buzzy.

46 I Tired of Needles? How Buzzy Can Help The Buzzy is a device designed to confuse nerve sensories during infusions and blood drawls. This is how it works...

MIND BODY CONNECTION 38 I Chronic Pain and Mindfulness Meditation and yoga expert, Krystyn Strother explains the connection between the mind and perceived pain, and offers up an exercise for all to try.

CONTENTS

e r o m r e v o c s Di about IXINITY

®

Visit IXINITY.com Aptevo BioTherapeutics LLC, Berwyn, PA 19312 © 2017 Aptevo BioTherapeutics LLC. All rights reserved. CM-FIX-0112

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.

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 bleeding disorders community in 2018. Please save the dates. More details can be found on our website: comphealthed.com

FACTOR 7 RETREATSM DURATION: 3-day event ATTENDEES: Anyone with a confirmed diagnosis of FVII deficiency and their immediate family Inspired by the LadyBugs Foundation, we are proud to offer the only national program of its kind for those affected by Factor 7 Deficiency (FVIId). This unique event offers tracks for individuals with FVIId, parents and caregivers, teens, and children so that support and education are tailored specifically to the needs of each group.

LEVERAGE

Friday, April 6th - Sunday, the 8th, 2018 Marriott at City Center - Newport News, VA Learn more at comphealthed.com/fvii

SM

The Ultimate Inhibitor Adventure LEVERAGESM DURATION: 5-day event

ATTENDEES: Young adults ages 18 and up living with an active inhibitor or those who have tolerized within 3 years from the event date In collaboration with GutMonkey , this program delivers truly lifechanging experiences by challenging attendees through a series of outdoor adventure activities paired with reflection, discussion, training, and planning for the future.

TM

Learn more at comphealthed.com/Leverage

AFTER THE SHOCKSM DURATION: 3-day event ATTENDEES: Children ages 0-18 living with an active inhibitor and their immediate families

Leverage 1.0 (beginners - excludes extreme activities) Monday, May 9th thru Friday, the 15th, 2018 YMCA Camp Collins - Gresham, OR Leverage 2.0 Saturday, Sept. 8th thru Wednesday, the 12th, 2018 Location: TBA

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.

Friday, June 22nd - Monday, the 25th, 2018 Camp Zeke - Lakewood, PA Learn more at comphealthed.com/after-the-shock

2018 SAVE the DATES

Event opportunities for members of the inhibitor community MOMENTUMSM DURATION: 3-day event ATTENDEES: Men ages 18 and up living with an active inhibitor or those who have tolerized within 3 years from the event date Friday, July 20th thru Sunday, the 22nd, 2018 St. Louis, MO Learn more at comphealthed.com/Momentum

By bringing together adult members of the inhibitor community in an atmosphere of acceptance and unity, this program provides opportunities to share experiences with others and discuss critical topics specific to their needs.

LADYBUGSSM DURATION: 3-day event ATTENDEES: Women ages 16 and up with a bleeding disorder, or who are mothers, caregivers, carriers, and/or siblings of someone with a bleeding disorder

Friday, August 24th thru Sunday, the 26th, 2018 Charleston, SC Learn more at comphealthed.com/ladybugs

At it core, LadyBugs is uniquely tailored to the needs of women whose lives have been affected by bleeding disorders. The program is designed to empower women though education, support, laughter, and tears to help find their voices when it comes to making decisions about their health and the health of their loved ones.

INHIBITOR FAMILY CAMPTM DURATION: 4-day event ATTENDEES: Children ages 6-18 living with an active inhibitor or those who have tolerized within 3 years from the event date (and their immediate families) From arrival to departure, this event is packed with fun, adventure, and education-all in a sharing and supportive environment.

Campers can look forward to meeting other families, enjoying outdoor activities, attending educational classes, and making lifelong friends.

Friday, Sept. 28th - Monday, Oct. the 1st, 2018 The Painted Turtle - Lake Hughes, CA Learn more at comphealthed.com/ifc

GLANZMANN’S THROMBASTHENIA SYMPOSIASM DURATION: 3-day event ATTENDEES: Anyone with Glanzmann’s Thrombasthenia (GT) and immediate family

Friday, Nov. 2nd - Sunday, the 4th, 2018 South Carolina

The GT Symposia is intended to bring those affected by this incredibly rare bleeding disorder together to establish a community and build a support system with others who truly understand their unique needs.

Learn more at comphealthed.com/glanzmanns-thrombasthenia-symposia 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.

LEVERAGE: a Molehill Practice for a

By Justin Levesque

Mountainous Adventure S

ome things in life never change, like living with severe hemophilia. Treatment options have come and gone and of course, there’s the promise of new therapies on the horizon. But on a fundamental level, I’ll always have these mutated genes to keep me company. It wasn’t until recently though that the perception of my bleeding disorder was turned upside down with the sudden development of a high-titre inhibitor at twenty-five (I’m now thirty-one).

Things were really rough those first few years. With lots of bleeds and a dash of emotional defeat, I only exacerbated the impact of my perceived epic misfortune by isolating

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from others, imposing limitations upon myself, and no longer seeking out life’s great adventures.

It’s a longer story than this space allows but eventually, having an inhibitor (and everything that comes with it) became integrated into everyday life and I got back to sustaining a creative practice of making artworks and photographs. Most photographers dream of the opportunity to travel to remote places and make pictures. While it’s a dream I also shared, I was always deeply worried about my ability to do so; this fear now bigger since I developed an inhibitor.

an Innovative CHES/GutMonkey Program

Last year I came across a “Call For Applicants” to The Arctic Circle artist residency program. The Arctic Circle residency is a highly competitive program that brings together thirty international artists of all disciplines, scientists, architects, and educators who collectively explore remote and fascinating destinations aboard an ice-class Tall Ship and takes place in the international territory of Svalbard, a mountainous Arctic archipelago just 10 degrees from the North Pole. On a whim, I applied thinking the chances were pretty slim. But a few months later, I was beyond stunned to learn of my acceptance and that I was now invited to go north in June 2017.

Of course, I was excited. So excited.

And while I still couldn’t believe it,

I bombarded myself with questions about the reality of my body in the Arctic: Can someone with a inhibitor go to the Arctic? Should I disclose my disorder to the program beforehand and potentially jeopardize the opportunity?

What if the activities are beyond my physical capability but everyone else is doing it? Do I push myself and risk a bleeder catastrophe? Will I miss out on seeing parts of the Arctic landscape if I don’t do every adventure presented to me?

a revolutionary, new program for young adults with inhibitors. This 5-day adventure delivers heart-pounding excitement that puts your inner strength to the test! Find out what you’re made of, and develop new skills that can help you become who you want to be.”

Supported by educational grants from

It was about this time that I was encouraged to participate in CHES’s Leverage inhibitor adventure program co-facilitated by GutMonkey at YMCA Camp Collins in the heavily wooded forests of Portland, Oregon. Leverage is described by CHES as, “Introducing

Photo courtesy of Justin Levesque

COMMUNITY CHATTER

It probably sounds silly but I initially declined because “That’s too.. adventurous for someone with an inhibitor.” I’m not sure why my brain was OK with winging it in the Arctic while scoffing at the Pacific Northwest but the absurdity of my rationalization was thankfully pointed out to me. I prepared to fly to Oregon just a few weeks shy before heading North. It was, hands down, the best decision I could have made.

At Leverage, you’re surrounded by other folks with a bleeding disorder and an inhibitor just like you. It couldn’t be more relaxed and no one has to explain why you’re different than a “normal” bleeder. There’s structured adventurous activities (more on that later) and plenty of free time to explore the camp’s beautiful and serene natural environment. To top it off, GutMonkey and CHES’ supportive staff brings truly impactful educational opportunities that speak authentically to the difficulty of the lived bleeder experience and provide new skills and techniques to navigate that Inhib Life.

When it came time for adventure, there was a wide gamut of activities for us to participate in. I don’t use that word lightly, participate. From learning to throw a tomahawk to floating 60 feet in the air (they call it the Flying Squirrel) to rapelling down the side of a building with nothing but open ravine below you, the only thing that mattered was to participate. What I learned was that participation didn’t always mean being the person strapped in or required to use their body in some performative way.

Participation was also helping to keep the ropes organized, shouting “You got

this!” and just being present for your fellow campers.

If something was too beyond my comfort zone, I was affirmed and supported in my choice to pass on a particular activity. I relearned how to say, “No thanks” and be OK with that choice.

No one judged me there but I also realized if anyone did, or rather, if anyone else in the world were to, that it didn’t matter.

I was doing what I had to do for me. It was both practice and a practice. I got to try all this “saying no” stuff out, to hear the words coming out of my own mouth and gain comfort in their sound. And further, it was a mindset that developed while at Leverage. To be present in my own body, to pursue and be OK with my own motivations as reconciled with my known limitations. I was asked to push myself within reason but not become some kind of unattainable superhero.

I can’t stress enough how important all of these lessons would become and how often I would think back to this week in the woods while I explored the frozen Arctic Ocean. While some folks hiked every glacier they set their eyes on, I resolved to climb one. While some folks lugged every single piece of heavy gear to shore, I rotated one piece of photo gear for each landing where I could really focus on using that tool. While some folks didn’t think at all about their bodies and found themselves injured and quite sore, I was always listening to my body with an acute awareness to make choices accordingly and with confidence.

Thank you CHES. Thank you GutMonkey. Without Leverage, I’d have been frozen with fear instead of finding myself in the middle of a glacier just shy of the North Pole.

Justin Levesque specializes in the critical analysis of images and their impact on social norms and community expectations. He lives in Portland, Maine where he runs his own design studio, Shop Geometry. He also serves as Creative Director for The Hemophilia Alliance of Maine and is Co-Director of the FOLX program which celebrates and acknowledges creativity in the bleeding disorders community.

His website can be found at onedynamicsystem.com

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Photos courtesy of Justin Levesque

an Innovative CHES Program

Taha’s Moment:

Finding “Normal”

By Taha Amir

I

am a 32-year-old male with severe hemophilia A. I moved to the United States, from Saudi Arabia when I was about 7 years old. Within that year, the hematologist told my parents that I had inhibitors - and they were lost, confused, and devastated. My parents knew what hemophilia was because my mom’s brothers also had it. But inhibitors were unheard of. The hematologist said that my body does not accept the “regular” factor, and I needed a bypassing agent called FEIBA. My brother was born in 1992, and he also has hemophilia, but not inhibitors, thankfully. To me, he was considered a “normal” person.

As a kid growing up with hemophilia with an inhibitor, I always stood out. I had bruises, swollen joints, and I walked with a limp. If I wasn’t walking, I was either using a wheelchair, crutches, cane, or a walker. I felt left out because the other kids were playing and walking better than me.

My pediatric hematologist suggested that I attend a hemophilia camp, which I did. The first time I went, I was nervous and scared because I wasn’t in my safety net and away from my parents for the first time. Even at the hemophilia camp, I stood out. I was still “different” from them because I had inhibitors. The “normal” hemophiliacs were still better than I was. Despite my struggles at camp, I attended a couple times after that because I was accepted.

It felt like they knew me!

Each time I have gone to the Momentum event, I have learned something new and met some amazing men who actually understand my struggles. The retreat is a threeday event over the weekend. The first day was dinner and a small informative talk by Dr. Dustin Sulak about the uses of medicinal cannabis for both acute and chronic pain. The second day started off with breakfast, a few more educational talks from treatment products to pain management, followed by lunch and more great information like infusion tips and tricks and practical career paths. One of the hot topics of this year’s retreat was health insurance and the current changes by NHF’s Michelle Rice. In the evening, the group went out for dinner and entertainment.

Most of the previous events that I’ve attended were primarily catered to “just normal hemophiliacs.”

At Momentum, I feel accepted and better about myself. It has really changed the way I look at events for hemophilia with inhibitors.

Supported by an educational grant from

As a teen, I really didn’t attend any hemophilia events because it seemed like I was always bleeding. My younger brother, who also has hemophilia without inhibitors, was able to do more than I could. I felt as if I was the only one who had a different kind of hemophilia, which I did.

As I graduated high school, I decided to change my outlook on life. I attended a hemophilia conference, and met some amazing friends whom I still keep in contact with. At one of these events, I met the co-founder of CHES. She introduced herself and what CHES does for the hemophilia community - especially the inhibitor patients/community. One of the programs they offer is called Momentum, a men’s inhibitor retreat.

So far, I have attended Momentum twice. In July, I had started Immune Tolerance Therapy (ITT) in April 2017. By the time I went to the event, my inhibitor level was 0. Even though it had been 0 for a few months, the hematologists still hadn’t declared tolerization. I still have to do daily infusions of factor for the next few months.

Photo courtesy of Taha Amir

COMMUNITY CHATTER

What Inhibitor Family Camp

Means to the Cook Family

By Cheryl Cook

There was this voice on the other end of the phone, (I could barely hear because of the tears I was crying - saying we would not be coming.) It was another mom. The mom who said this camp was something she knew people needed; this was Janet Brewer (of CHES.) She told me to get Jake on that plane. She knew what I was going through and assured me that she would be there. She had survived through these same times and would help us get through them. She said she believed this would be something that Jake would never forget. She was so right. That weekend CHANGED Jake’s life!

H

ow do I even begin to tell anyone what Inhibitor Family Camp means to our family? How do you explain that it was a life-changing event?

My son is a mild hemophiliac, but we didn’t even know what an inhibitor was until he was diagnosed with one.

Our life changed in a minute. I heard about this camp from someone who had been there before, so I looked into it. We registered and “yeah!” we heard we were going!!

However, I was told by many that Jake should not go. His needle phobia required five (5) adults to hold him down for every infusion. We had health care companies that refused to work with us. We were giving infusions everyday. He was a mess; I was a mess. After much thought, I called to let CHES know we would not be coming to camp this year.

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When we got back from camp, none of his doctors or nurses believed this was the same kid. He was, but something had changed. I am still not sure what exactly happened to my 7-year-old that weekend, but he had a different attitude and outlook. He was better able to accept this inhibitor diagnosis. He said it was because he was able to hang out with kids who “got” it. He said he felt that if they could do it, he could do it. We still have some stumbling blocks, but each time we go, it gets better.

The second year we were able to go, my son learned to self-infuse! Wow, what one year can do! The third year, he was excited about seeing his friends. He looks forward to hearing that registration has opened. He looks forward to seeing who is going to be there. He gets to go to Inhibitor Family Camp and be a kid; be with other kids, just like him, and no one is looking at his port. No one is wondering why he has it, or what is wrong with him? He’s able to just be a kid.

I asked him to describe what this camp means to him, and he turned around and said one word, ”JOY!” All I can say is, “thank you!”

Family Tradition,

an Innovative CHES Program

Change with

Modern

Supported by an educational grant from

F

Pictured: Tracy Ziminski with sons, Jack and Matthew, and Colin Cox with children, Nikos, Arija, and Maksis.

lying saw dust, splashing paint, and lubed up wheels have all become a tradition at Inhibitor Family Camp (IFC) at The Painted Turtle in Lake Hughes, CA. We marked our 6th year of IFC at The Painted Turtle, and it was highly decorated with a new spin, literally. CHES was honored by three of our Inhibitor Family Camp families, the Cox’s, the Katzman’s, and the Ziminski’s by donating a brand new, aluminum track (to replace the old, wooden model) for the annual pinewood derby on behalf of CHES’ name.

Missing in our photo credit is Renee Cox. Jim Ziminksi and the Katzman family were unable to attend and missed greatly. CHES and The Painted Turtle proudly thank these families for their friendship, kind hearts, and especially their generosity. -Eric Lowe

COMMUNITY CHATTER

POWER

PAIN

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FEATURE

By: Georgia Panopoulas, PhD, LP - Pain Psychologist and Janet Brewer, M.Ed

A

few days ago, I came across an interesting article in the Minneapolis Star Tribune, and a very timely article, in retrospect. The author of the article titled “Opioids: Epidemic is the symptom, not the disease,” argued that “one of the reasons we have an opioid epidemic is because we have a pain epidemic.”

There is no arguing that pain, both acute and chronic, is prevalent in the United States and around the globe. Although trends in the prevalence of pain disorders are generally lacking, one study found that for chronic, debilitating low back pain (LBP), prevalence increased from 3.9% in 1992 to 10.2% in 2006. Increases were seen across all adult ages, for females and males, and across race. Reasons for the observed increase in prevalence rates were unclear, however, researchers suspected that increasing rates of obesity, changes in psychosocial and physical work demands, increase prevalence of depression, and increases in symptom awareness and reporting contributed to these increases in prevalence rates.

Pain in the Bleeding Disorders Community Would these findings stand true within the bleeding disorders community? Witkop et al. (2017) argued that as life expectancy of persons with hemophilia (PWH) increases, pain and specifically joint pain is a major problem affecting adult PWH. Their study (the Hemophilia Experiences, Results and Opportunities study; HERO) found 85% of participants experienced pain during the 6-month period prior to completing the survey. Twenty percent of PWH (77% with Hemophilia A, 23% with Hemophilia B, and 9% with inhibitors, median age of 34 years) reported experiencing acute pain only, 34% reported experiencing chronic pain, and 32% reported experiencing both acute and chronic pain. Ankles (37%), knees (24%), and elbows (19%) were identified as the most painful joint. By comparison, 11.2 % of the general population in the United States experiences chronic pain, with LBP being the most common, followed by headache or migraine, neck pain, and facial pain.

In 2014, the Food and Drug Administration (FDA) sponsored a public forum titled “Patient-Focused Drug Development for Hemophilia A, Hemophilia B, von Willebrand Disease, and Other Heritable Bleeding Disorders” in an effort to learn from those affected about their treatment options and treatment concerns. A major topic of concern was pain management (both acute and chronic).

FEATURE

Definition of Pain

Pain Management Requires a Variety of Approaches

For the purpose of this article, pain is defined by the International Association for the Study of Pain (IASP) as: “An unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage.” IASP also added that pain is a subjective experience. Acute pain typically lasts less than 3 to 6 months.

In PWH, acute pain is common during joint or muscle bleeds.

As such, treatment requires an interdisciplinary approach focusing on physical, cognitive and emotional aspects of pain, in addition to focusing on pain and its impact on relationships. Opioids alone cannot successfully treat pain.

In fact, successful treatment with opioids is considered to be 40-50% relief in pain (according to a scale of pain from 0-10, 0 representing “no pain,” and 10 representing “the worst pain imaginable”). Thus, successful treatment using opioids alone would result in a decrease of pain from 10/10 to 5-6/10. Opioids cannot eradicate the pain, much less “treat” the cognitive, emotional, and relational aspects of pain. Additionally, opioids are not without their negative side effects which include sedation, dizziness, nausea, vomiting, constipation, physical dependence, tolerance and respiratory depression. Long term use can result in allodynia and hyperalgesia (thus pain increases over time).

From a psychological perspective, opioids and marijuana are dissociative drugs, numbing physical and emotional distress, making it more difficult for individuals to stay connected with loved ones and to find purpose and meaning in life.

Chronic pain is defined as pain that persists longer than 1 month beyond the normal healing period or that is associated with a pathological process (e.g., arthropathy) that causes continuous or recurrent pain over months or years.

Chronic Pain Syndrome (CPS) effects approximately 25% of people with chronic pain. CPS occurs when people have symptoms beyond pain alone, like depression, anxiety, sleep disturbance, which interfere with daily functioning.

Why is pain so difficult to treat? The pain experience is subjective by nature. Contrary to 17th century thought that pain was either physical or psychic in origin, physical and mental aspects of pain as inseparable was the focus of medical research by the early part of the 19th century.

By the 20th century, the Gate Control Theory of Pain emphasized the role of sensation, affect, and cognition “interacting with one another to provide perceptual information that ultimately influences the motor mechanisms characterizing pain,” (Olson, 2013). Further, as pain evolves from acute to chronic, it alters neural pathways, impacting mood and interfering with sleep.

Adobe Stock, by Joshya

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Managing Physical Aspects of Pain Managing the physical aspects of pain may require use of factor (in the case of acute pain, or chronic pain with acute exacerbation, or flare), RICE (rest, ice, compression, elevation), breathing exercises, relaxation exercises, gentle stretching, etc., in addition to certain medications. The goal is to manage the physiological arousal that often results from pain, but that only serves to feed right back into the pain experience. Pain is perceived by the brain as a potential threat or danger to the system. As such, pain activates the sympathetic nervous system (SNS), the fight or flight response, resulting in increases in blood pressure, heart rate, fuel availability, adrenaline, oxygen circulation to the organs, blood clotting, pupil size, while decreasing fuel storage, digestion, and salivation.

Rest, which is essential for healing, becomes more difficult. Activation of the parasympathetic nervous system (PNS) is essential to counteracting the effects of the SNS. Activation of the PNS can be accomplished through meditation, massage, deep breathing, being in nature, playing with children or animals, yoga, tai chi, mindful movement. Finding the balance between relaxation and reactivation can strengthen the relaxation response and serve as an immediate defense against the effects of pain.

Cognition and Pain Managing the cognitive aspects of pain may at first seem trivial, unimportant. Can pain management be as simple as mind over mood? Of course not. And in fact, we discussed previously that it’s not mind over mood, but mind and mood.

Our pain is based on our perceptions, our perceptions are based on what we know, and what we know is interpreted based on what we have experienced in the past. Not knowing if our pain is a result of a bleed can be very disconcerting. Do we treat? Do we ignore the pain and push through? Are we thinking realistically about the situation or are we engaging in distorted, unrealistic, or irrational thoughts like:

Distorted Thoughts All-or-nothing thinking

Jumping to conclusions

You used to play baseball on the weekends before you developed chronic pain. Now you find yourself thinking, “if I can’t play baseball, I can’t enjoy the sport anymore at all”

Mind Reading: You assume you know why someone else does what he or she does and do not bother to check.

0vergeneralization

Fortune telling: You “know” that things will turn out badly. You predict it as an established fact.

If you woke up in more pain, you might think, “I’ll never be able to enjoy anything anymore.”

Emotional reasoning:

Mental filtering

Labeling:

You are preparing lunch for some friends and discover that you don’t have an essential ingredient. All you can think about is how the whole lunch will be ruined.

Disqualifying the positive A friend comes over to visit and tell you that you look great. Your immediate thought is: “I don’t feel great. She doesn’t understand” Magnification and minification: You exaggerate the importance of a negative event or mistake. You take positive personal qualities or events and deny their importance.

If you feel that something is right, it must be true.

Instead of seeing yourself as an individual who has a pain problem, you find yourself saying, “I’m defective, imperfect and without any redeeming qualities.”

Personalization: You and your spouse go out to eat at a fancy restaurant, but the service and food are poor. You find yourself feeling responsible for ruining your evening by choosing that restaurant.

“Should” statements: You attempt to browbeat or motivate yourself with “I should know better”.

Irrational Beliefs • • • • • • • • • •

It is an absolute necessity for an adult to have love and approval from peers, family and friends.

You must be unfailingly competent and almost perfect in all you undertake.

Certain people are evil, wicked and villainous, and should be punished.

It is horrible when people and things are not the way you would like them to be.

External events cause most human misery - people simply react as events trigger their emotions You should feel fear or anxiety about anything that is unknown, uncertain or potentially dangerous.

It is easier to avoid than to face life’s difficulties and responsibilities.

You need something other or stronger or greater than yourself to rely on.

The past has a lot to do with determining the present.

Happiness can be achieved by inaction, passivity, and endless leisure.

(Adapted from Margaret Caudiull’s Managing Pain Before It Manages You) If you recognize any of these thought patterns, you are not alone. But once you recognize them, try to change them, not by thinking positively (for positive thinking can be as distorted as our negative thinking), but by thinking realistically/ rationally/with the evidence.

FEATURE

Emotional Well-being The importance of managing emotional aspects of pain cannot be overstated. The prevalence rates of depression and anxiety in PWH are indeed higher than what we would expect in the general population and higher and/ or comparable to rates of emotional distress in persons with other chronic pain conditions (e.g., lower back pain, headache/migraine, neck pain, facial pain). Sadness, anger, fear are real and justifiable emotional responses to chronic illness and chronic pain, but can be managed. First identify what you are feeling. I like having a cheat sheet of “feeling words.”

Ultimately, however, our feelings fall into one of four categories: mad, glad, sad, and scared. What are you feeling? What do you attribute the feelings to? Something that just happened or something that happened in the past?

MAD

GLAD Comfortable Proud Caring Friendly Pleased/Loving Worthwhile Ecstatic Keyed up Thrilled Excited Happy Gay Enthusiastic Joyful Cheerful Calm Independent Amorous

Hostile Resentful Rage Aggressive Outraged Rebellious Ornery Rabid Burned up Frustrated Annoyed Disturbed Disgusted Angry Ticked off Hate Smothered P’d off Upset Irritated Belligerent

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Can you do anything about the situation that is contributing to how you feel? If not, can you allow yourself to feel without trying to change the feeling (i.e., mindfulness)? Can you allow yourself to feel anger, sadness, grief and loss, forgiveness toward yourself and others, and to accept these feelings as they are, real and valid? Or do we too quickly assign judgment to these feelings?

Keep in mind that feelings of grief, sadness, loss apply to both the parent/caregiver and the person with pain.

Guilt can be a source of great pain for mothers given that hemophilia is a x-linked chromosomal disorder. “It is my fault my son suffers because I gave it to him”. Or, as the person in pain, “I am a burden to my wife/children because I can’t do the things I once did which falls on them.”

SAD Helpless Bored Despairing Embarrassed Disappointed Lonesome Hurt Sorry Guilty Lost Blue Melancholy Rejected Down in dumps Burned out Forlorn Blah

SCARED Ashamed Curious Anxious Horrified Petrified Terrified Apprehensive Tense Freaked out Insecure Frightened Shy Shamed Worried Fearful Afraid Mistrusting Confused Defensive Jealous Uptight Inadequate

Pain and Relationships As much as we would like to think that pain only impacts the person with pain, we know otherwise. Pain impacts those we love deeply. Family and friends want to help, they want to “fix it.” They can’t. They become frustrated and, at times, it seems as if they are frustrated with the person who has pain. Their response to the person with pain can be perceived as “punishing.” Or maybe overly “helpful.” The research suggests that these responses are common, but not helpful especially when attempting to manage chronic pain. So, what are family members to do? “Distracting” responses can be most helpful. What does that mean?

Offer understanding, validation. But more than that, I encourage family and friends to ask, “What do we need to do to get through this.” The “we” refers to the person

in pain along with the family member or friend. This, of course, is a work in progress and requires communication, willingness to try new things, and reflection. When it all gets overwhelming, there is the propensity to shut down and pull away. Parents watching their children withdraw is painful. Trying to help seems to make it worse because the child may not want to talk about it. Ultimately, we may seek counseling and/or use mood enhancing medications to get us through. As much as we’d like to put the bleed, or the flare, or the crisis behind us, it would serve us better if we talked about how we got through, what helped, what didn’t, and what we might want to do different next time, because there will always be a next time.

FEATURE

The Power to Heal In 2016, I attended the NHF Inhibitor Summits. Dr. Tyler Buckner and I presented several sessions on pain management (an overview of strategies for managing acute and chronic pain). Dr. Buckner presented information about various classes of medications including but not limited to opioids and medical cannabis. The utility of these medications, as indicated previously, was variable at best.

But the participants, those living with inhibitors, shared their favorite pain coping strategies with the group and, once again, I left the Summits truly believing that I learned more from the Summit participants than they learned from me. The following is a list of pain coping strategies identified by participants (including PWH, parents/ caregivers, siblings, significant others):

Assistive Devices •

Buzzy (see related articles in what’s new on page 46)

•

Poke program*check out on our website in reference section

•

Smart phone apps, i.e. - Healing Buddies Comfort Kit http://www.healingbuddiescomfort.org/

•

Virtual reality (see related articles in what’s new on page 42)

Distraction-Passive and Active •

Blow bubbles

•

Read/Listen to Stories

•

Listen to or create music

•

Watch movies

•

Play games

•

Utilize social media

Emotional Strategies

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Calming Techniques

•

Change your thoughtspositive self-talk and reinforcement

•

Coping

•

Counseling

•

Grief and loss acceptance

•

Humor

•

Pain Education

•

Set realistic goals/expectation

Movement •

Balance, coordination, ball therapy

•

Mini Breaks

•

Change position

•

Tai Chi

•

Dance

•

Walk/pace/rocking

•

Exercise/Gentle stretching

•

Yoga

Mindfulness

Physical Comfort Measures

•

Attention Management

•

Breathing

•

Guided imagery

•

Meditation

•

Food

•

Progress muscle relaxation/body scan

•

Self-hypnosis

•

Set realistic expectations of self/others

•

Acupuncture

•

Hot/Cold packs/pads

•

Acupressure

•

Loose, soft clothing

•

Beverages

•

Massage

•

Biofeedback

•

Pillows/positioning

•

Contrast baths-Epsom salts

•

RICE

•

Cuddle

•

Sleep

•

Dim lighting, noise reduction

•

TENS (Transcutaneous electrical nerve stimulation)

•

Essential oils, salves, rubs/ aromatherapy

•

Weight Management-anti-inflammatory, gluten, soy, soda, sugar free diet

This list is far from exhaustive! But these strategies are tried and true. Will we eradicate the pain? Can we eradicate the pain? Should we eradicate the pain? Remember, pain is not all in our head, but that’s where pain messages are processed and where we can begin to alter neural pathways and change the suffering.

FEATURE

Managing Inhibitors in Hemophilia Treatment: The Importance of Bypassing Agents

Bypassing Agents are a Factor option with a long history of use in treating hemophilia patients with inhibitors

By Mike Denne

Treatment of inhibitors is one of the greatest challenges in hemophilia today Up to 33% of people with severe hemophilia A develop an inhibitor, or antibody, to treatment, while 3% to 13% of patients with moderate to mild hemophilia develop an inhibitor.1,2 (Inhibitors are less common among people with hemophilia B, affecting only 1% to 6% of individuals.1) These inhibitors stop, or block, Factor VIII (FVIII) or Factor IX (FIX) function in your blood-clotting process. Bypassing Agents offer an alternative treatment approach because they go around, or bypass, the need for FVIII or FIX.3

Bypassing Agents supplement other Factors in the blood-clotting process This chart is a simplified visualization of Bypassing Agent activity in the blood-clotting process.

*Thrombin is one of the last proteins involved in the blood-clotting process. It is necessary for clot formation.

Help maintain a delicate balance The natural blood-clotting process is complicated, involving a delicate balance of on-and-off (activation and inactivation) steps.4-6 You can compare the on-and-off steps to the way a light switch turns on and off: once a bleed occurs, the blood-clotting process turns on, as with a light switch, and Bypassing Agents are available to help control the bleed. Once the bleed is controlled, the process turns off again, as when a light switch turns off. Factor activity is inactivated and unused Bypassing Agent Factors are eliminated from your blood.59 These on-and-off steps are important because they help your body form a proper clot and then inactivate the Factors when they are no longer needed.8-11

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It’s important to note that turning “off,” or inactivation, is an important step in the blood-clotting process: Unchecked thrombin generation could lead to risk of blood clots over 11,12 time

Bypassing Agents: 2 options to address different responses to treatment There are 2 Bypassing Agent treatment options available for people with inhibitors and you may respond differently to each of them.8,14

*These patients either responded to both Bypassing Agents or did not respond to either Bypassing Agent.

In one study, 60%-67% of people responded in the same way to each Bypassing Agent. However, up to 40% of people responded to only one Bypassing Agent and not the other in the first 12 hours after the infusion. Variability in patient response reduced to 7.3% after 48 hours.15

There are serious risks of blood clots that block blood vessels with the use of Bypassing Agents.8 You should be monitored for the development of blood clots. If you experience any side effects while using a Bypassing Agent, call your health care provider right away.

Work with your healthcare provider to develop an individualized treatment plan that is right for you

Use of Bypassing Agents in different scenarios Bypassing Agents are infused intravenously, making the proteins needed for clotting immediately available for use.9,16-18 Bypassing Agents may be used in the following scenarios:

PROPHYLAXIS Infuse routinely to prevent bleeds16

ON DEMAND Infuse when needed or to control breakthrough bleeds16

SURGERY Infuse to prevent/manage bleeds during or after surgery10,19

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BLOODLINES

Ability to adjust dose and frequency for life’s planned and unplanned events Your health care provider can tailor the dose and frequency of Bypassing Agent treatment to meet your individual needs, including on-demand and surgical needs.

For example, your health care provider can provide a lower dose and/or lower treatment frequency for a mild bleed or minor surgery. Conversely, your health care provider can provide a higher dose and/or higher treatment frequency in response to a severe bleed or major surgery.8,10,19

Talk with your health care provider about Bypassing Agents, including tailoring your treatment based on your individual needs.

Bypassing Agents are backed by a long history of use With up to 40 years of use, and substantial bleed reduction benefits, Bypassing Agents are considered a well-studied and established treatment option for hemophilia patients with inhibitors.8,20-22

EXPLORE THE POSSIBILITIES: To learn more about treating hemophilia inhibitors with Bypassing Agents, talk with your health care provider or visit bleedingdisorders.com.

REFERENCES: 1. DiMichele DM. Inhibitor treatment in haemophilias A and B: inhibitor diagnosis. Haemophilia. 2006;12(suppl 6):37-42.

2. Hay CRM. Factor VIII inhibitors in mild and moderate-severity haemophilia A. Haemophilia. 1998;4(4):558-563.

3. DiMichele DM. World Federation of Hemophilia website. Inhibitors in hemophilia: a primer. http://www1.wfh.org/publication/files/pdf-1122.pdf. Published 2008. Accessed October 5, 2017.

4. Lenting PJ, van Mourik JA, Mertens K. The life cycle of coagulation factor VIII in view of its structure and function. Blood. 1998;92(11):3983-3996.

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/. Accessed September 19, 2017.

6. Johari V, Loke C. Brief overview of the coagulation cascade. Dis Mon. 2012;58:421-423.

7. Retzios AD. The new and improved (?) activated factor VII molecules. Bay Clinical R&D Services. 2001:1-41.

8. Shapiro AD, Hedner U. Advances in bypassing agent therapy for hemophilia patients with inhibitors to close care gaps and improve outcomes. Ther Adv Drug Saf. 2011;2(5):213-225.

9. Ananyeva NM, Lee TK, Jain N, Shima M, Saenko EL. Inhibitors in hemophilia A: advances in elucidation of inhibitory mechanisms and in inhibitor management with bypassing agents. Semin Thromb Hemost. 2009;35(8):735-751.

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10. Leissinger CA. Prevention of bleeds in hemophilia patients with inhibitors: emerging data and clinical direction.

Am J Hematol. 2004;77(2):187-193.

11. Wolberg AS. Plasma and cellular contributions to fibrin network formation, structure and stability. Haemophilia.

2010;16(suppl 3):7-12.

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

13. Hoffman M, Monroe DM. A cell-based model of hemostasis. Thromb Haemost. 2001;85:958-965.

14. Hoffman M, Dargaud Y. Mechanisms and monitoring of bypassing agent therapy. J Thromb Haemost.

2012;10:1478-1485.

15. Astermark J, Donfield SM, DiMichele DM, et al. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FENOC Study. Blood. 2007;109(2):546-551.

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

17. Hedner U. Recombinant activated factor VII: 30 years of research and innovation. Blood Rev. 2015;29(suppl 1):S4-S8.

18. Hemophilia Federation of America. Treatment of hemophilia. http://www.hemophiliafed.org/bleedingdisorders/ hemophilia/treatment/. Accessed September 20, 2017.

19. Santagostino E, Escobar M, Ozelo M, et al. Recombinant activated factor VII in the treatment of bleeds and for the prevention of surgery-related bleeding in congenital haemophilia with inhibitors. Blood Rev. 2015;29(suppl 1):S9-S18.

20. Mehta R, Parameswaran R, Shapiro AD. An overview of the history, clinical practice concerns, comparative studies and strategies to optimize therapy of bypassing agents. Haemophilia. 2006;12(suppl 6):54-61.

21. National Hemophilia Foundation. Medical and Scientific Advisory Council (MASAC) recommendation regarding the use of bypassing agents in patients with hemophilia A or B and inhibitors. MASAC document #167. 2006.

22. NIH Clinical Trials Registry. Ongoing and complete clinical trials using bypassing agents in hemophilia patients with inhibitors. https://clinicaltrials.gov/ct2/results/ details?term=hemophilia+a+with+inhibitors%2C+hemophilia+b+with+inhibitors&recr=Closed&cond=hemophilia +a+with+inhibitors%2C+hemophilia+b+with+inhibitors. Accessed September 19, 2017.

Š2017 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. S34496 10/17

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BLOODLINES

New Sub-Q Treatment is for Inhibitors

O

n November 11th 2017, Genentech announced that the study drug, widely known as ACE910 (emicizumab), had been FDA-approved for prophylaxis treatment in children and adults with hemophilia A and inhibitors to prevent or lessen bleeding episodes. This approval snapped a streak of nearly 20 years for the inhibitor population on newly-approved treatment products.

Almost 1 in 3 hemophilia A patients that are treated with factor VIII replacement therapy tend to develop inhibitors against factor VIII, which can lead to a higher risk for bleeds and long-term joint damage. The FDA-approved drug, now named Hemlibra, bypasses that problem as it is a bispecific factor IXa- and factor X-directed antibody. This means its use can bring together the two proteins, which will activate the natural clotting cascade. Hemlibra also requires only once a week use as an injection under the skin.1

Hemlibra, has had positive results from two studies, HAVEN 1 (NCT02622321) for patients 12 years of age or older with hemophilia A with inhibitors, and HAVEN 2 (NCT02795767) for patients younger than 12 years with hemophilia A with inhibitors. Results indicated that Hemlibra significantly reduced bleeds in both adult and pediatric patients with hemophilia A. “People with hemophilia A who develop inhibitors face significant challenges preventing and controlling bleeds and typically require infusions of medicine multiple times a week, which can be especially difficult for young children and their families,” Dr. Guy Young, professor of pediatrics at University of Southern California Keck School of Medicine,

FDA-Approved

by Eric Lowe

said in a press release. “This new medicine has been shown to reduce the frequency of bleeds compared to the currently available medicines and only needs to be injected once a week. This could make a meaningful difference for these children.”1 Results Phase 3 of the HAVEN 1 study (in patients 12 or older) showed an 87% bleed-reduction when using Hemlibra compared to those who did not treat prophylactically, and a 79% bleed-reduction was reported for those who did treat prophylactically with a different bypassing agent (BPA) before switching to Hemlibra.

Similarly, the HAVEN 2 study (in patients younger than 12) also showed an 87% bleed-reduction when beginning prophylactic treatment with Hemlibra, and a 99% bleedreduction when changing their prophylactic treatments from a BPA to Hemlibra.

Common side effects of treatment were injection site reactions, headache, and joint pain (arthralgia).1 One death has occurred in the HAVEN 1 study back in February 2017. The patient received bypassing agents (BPA) after experiencing a serious rectal hemorrhage.2 The patient’s repeated use of the BPA in combination with the remaining residual amounts of Hemlibra in the patient’s system caused threat of serious thrombotic events. After discontinuation of the (BPA), the patient showed improvement. But Roche said the hemorrhage therapy was complicated because the patient declined blood transfusions. The trial investigator determined that the cause of death was the rectal hemorrhage, not emicizumab.2 You may read more details on this using the #2 resources link below.

Looking Ahead Hemlibra’s availability in the US is currently emerging and evolving.

HAVEN 3 and HAVEN 4 studies are currently underway to evaluate the treatment’s efficacy and safety at other doses and schedules. HAVEN 3 (NCT02847637) is being conducted in patients 12 and older for those with hemophilia A without inhibitors with doses given once every 1-2 weeks.

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HAVEN 4 (NCT03020160) is being conducted in patients 12 years or older, with or without inhibitors, with doses given every four weeks after an initial one-month “loading” period.

1. https://hemophilianewstoday. com/2017/11/17/fda-approves-hemlibraby-genentech-to-treat-hemophilia-apatients-with-factor-viii-inhibitors/?utm_ source=Hemophilia+News&utm_ campaign=58c1c52d86-RSS_EMAIL_ CAMPAIGN&utm_medium=email&utm_ term=0_ab10fdd11a-58c1c52d86-72167405 2. https://hemophilianewstoday.com/2017/02/24/ death-of-hemophiliac-in-clinical-trail-raisesmore-concern-about-emicizumab/

Did you know?

Eloctate Could Be an Option for

by Eric Lowe

forImmune Tolerance Induction (ITI)

I

n March of 2016, results were released from a study announcing the reduction of immunogenicity (the immune response to a foreign protein) using Eloctate when evaluated in a mice model. It concluded that:

•

•

Eloctate resulted in lower rates of inhibitors when used at therapeutically relevant doses (50-100 IU/ kg) compared to equivalent doses of traditional recombinant factor 8. However, at higher doses (250 IU/kg), Eloctate and traditional recombinant factor 8 resulted in similar inhibitor development rates.

Eloctate demonstrated tolergenic effects, whereby pretreating at therapeutically relevant doses (50 IU/ kg) can lessen an immunogenic response when dosed at the higher 250 IU/kg range compared to equivalent regimens with traditional factor 8.

The full details and outcomes on this study can be found at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4936482/ In May 2014, a new study (NCT02196207) was posted to evaluate if inhibitors are reduced when Eloctate is begun in children with severe Hemophilia A before the first bleed (preemptive - or also known as Previously Untreated Patients (PUP study)) and continued weekly to prevent bleeds (prophylaxis).

In July 2017, this study was concluded (although not yet released to the general public) and presented at the International Society on Thrombosis and Haemostasis (ISTH) Annual Congress in Berlin, Germany this summer.

While we cannot release any details of the outcomes in study NCT02196207, we can say that the results and conclusions were notable enough to initiate 2 new studies in the relation between Eloctate and ITI. The first new study’s (NCT03093480) purpose is to describe the time to tolerization with Eloctate in participants within a maximum of 12 months of ITI treatment. And the other study (NCT03103542) is meant to describe the outcome of ITI treatment performed with Eloctate within a time frame of 60 weeks in patients with hemophilia A who have failed previous attempts at tolerization.

Although the details in this article are broad and limited, we will continue to include more information on their outcomes in upcoming Lifelines for Health. If the use of Eloctate for ITI treatment interests you, ask your healthcare provider, and direct them to study NCT02196207 for results and guidance in a treatment regimen that’s right for you or your child. http://www.ClinicalTrials.gov

BLOODLINES

When Someone You Love Hurts

How Can You

HELP?

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A

while back, a politician said something to the effect of, “I feel your pain.”

These days, that line is generally used in a humorous sense. But if you have a partner or child who is living with pain, I suspect that line conjures up all kinds of thoughts and feelings for you. Most of them not very funny.

by Dr. Gary McClain, PhD

That’s because watching someone you love struggle with pain is anything but humorous. As a partner, as a parent.

You know how hard things are for them, you can see it in their face, the way they move, and in the way they speak.

You want to say something to make them feel better. You might find yourself saying to your partner: “You’re gonna be just fine. And soon.” Or to your child: “”Where does it hurt?

Let me make it better for you.”

And the way in which your loved one responded may not have been so gratifying. They may felt frustrated, or angry, because you were promising something you clearly couldn’t deliver on. Sure, you were just trying to be helpful. But it didn’t feel that way to them.

I think it is not an overstatement to say that having a loved one with chronic pain can really make you feel helpless.

Of course, you can’t take their pain away. Even if that’s what you wish you could do. But between doing absolutely nothing, and ridding them of their pain, there are actually a lot of things you can do to help a partner or child living with chronic pain.

FAMILY MATTERS

When Someone You Love Hurts. How Can You Help? (Continued from page 28)

Here are some ideas to consider: Get Informed Together Make it known that you are on stand-by. Let your partner or child know that you are standing by to help in not only gathering information, but making sense of it, whenever they are ready. Kids are especially in need of reassurance, so feel free to repeat as needed.

Do your own information-gathering. It’s not realistic to remain uninformed even if your loved one is locking you out of this process. Find information on your own so that you can educate yourself on how to be a better healthcare partner. And active information-gathering is a great way to help you cope with your own helpless feelings.

Let your loved one know you are getting informed. You don’t need to surf the Web in secret. Gently inform your loved one that you want to be as educated as possible and are doing your own research.

Offer to share your information. As you get educated, offer to share what you are learning. Use statements like: “I just found out about an interesting new treatment. Do you want to know about it?” or “I saw a list of foods that might help you. Any interest?” If you are a parent, your information-sharing may be needed to help explain any limits you need to set.

Encourage Adherence Look for teachable moments. The long lectures get old after awhile. You might instead be alert for moments when you can gently point out your loved one’s lack of adherence.

For example, if you notice they have missed a medication dosage, you can quietly remind them and ask if they would like you to help them remember. Parents may find using teachable moments especially helpful because younger kids and teens can be especially resistant to feeling lectured.

FAMILY MATTERS

Use some “patient” education. If you find yourself scolding your loved one, or getting angry, or giving orders, you may also find that you’re not making a whole lot of progress. Instead, assume good intention on their part rather than a desire to cause you frustration and annoyance. Who knows? Your loved one may get the overall concept of self-care but not be sure how to actually make it happen. Kids often need additional guidance in establishing self-care routines. How about asking if you can make a suggestion before you launch in with one? And then, focus on what they could have done and not what they didn’t do.

Choose your battles. As you have probably learned already, when people feel pushed into doing something, they become more resistant. That’s true for your partner and it’s true for your children.

So if, out of your own desire to help, you are turning everything into a battle of wills, your partner or kids may put up a wall. Be mindful of what is most important – like taking medication on schedule – versus what be less important. Use your judgment here.

When You Don’t Know What To Do… Ask. It’s sad to me that so many people don’t take the time to ask questions. They assume to know already. Or they just don’t think about what their loved one needs. The simple question – “What can I do for you?” – shows how much you care. By asking it, you’re already one step toward helping your loved one feel more supported.

Listen. With an open mind. You might be surprised at what you hear from your loved one.

Maybe you’ll learn that a simple gesture would help him/her have a better day. But you may also learn that your partner or child needs a lot more from you than you expected. You might hear about how overwhelmed they feel at times, or a lot of the time.

A few disappointments or resentments might come up. Be open to what your loved one has to say.

Get specific I have found that both caregivers and patients avoid talking about the support process itself, and what they are expecting of each other. Family members are often afraid that if they bring up the subject of support, their partner or child may assume that they are feeling burdened with their

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support tasks, or that they don’t want to help them anymore and are trying to find a way to break the news. In turn, your loved one may fear burdening you, but also doesn’t want to be made to feel incompetent by receiving too much care. Keep in mind that grown-ups, younger children, and teens can all feel disempowered because “hovering” over them can be interpreted as not trusting them to do what they can do to take care of themselves. Furthermore, when needs and expectations are not clear, this can result in missteps that can lead to tension.

Figure out a path forward. Talk about how you see your role — what you can and want to give, and what you think they need — as well as your

partner’s or child’s expectations. While defining your role is going to be a work in progress, open communications can help you to build a solid foundation for moving forward, and for protecting your own health and well-being. Keep talking!

Watch the Silver Lining Talk Don’t be a positive thinking police bully. Being told to “think positive” or to “stop feeling that way” is a tactic of the positive thinking police. Jumping in with a “yes but” and showering your loved one in rainbows and puppy dogs is another tactic. Denying feelings doesn’t make them go away.

Your loved one has the right to feel bad. Be sensitive to what your loved

one needs to hear and doesn’t need to hear. They are living with this chronic pain. Some days are going to be harder than others. On a bad day, go into asking questions and listening mode. Keep in mind, you may be asked to step aside and just be quiet.

When your partner says, “I’ll be okay, honey,” or your child says, “please leave me alone for awhile,” keep an eye out but back off.

Let your loved one know he/she doesn’t have to sit all alone with negative feelings. Be someone who can listen without telling them how they should be feeling or judging them.

Encourage them to vent! When you release feelings into the light of day, they lose their power over you.

FAMILY MATTERS

Give Your Loved One Their Space Individuals living with chronic pain are facing a wide range of emotions.

Many of these feelings are uncomfortable — a feeling, like fear or anger, may be so uncomfortable that they may not be able to even acknowledge feeling this way, let alone begin to express these feelings to someone else. Your loved one may feel so emotionally overwhelmed that they may shut down. And that’s scary for you to witness. Gently ask your partner or child how he or she is feeling, not only physically but emotionally, let them know you are here to listen. Remind them as needed, but don’t push.

Be willing to step aside. Keep in mind that, at least initially, some individuals are more comfortable opening up to people who are not their family members, and with whom they are less involved on a daily basis, like a counselor or support group member.

Don’t take this personally. Your partner may feel the need to protect you from his or her feelings, and so might your child. Give your loved one space to cope in a way that works for them.

Take Care of Yourself Take ownership of your own helplessness. Human beings love being in control, and we love it so much that

we tell ourselves we have control even when it is obvious that we don’t. And in a caregiving situation, that need to be in control can result in running ourselves into the ground trying to meet every possible need of someone we love to the point that we are running on empty. What parent hasn’t felt this way, right? In the process of depleting ourselves, we also risk alienating the people we care about by taking their own sense of control away from them.

Find your own support system. If you totally deplete yourself, you aren’t going to be helpful to anyone. Yes, I know you’re superhuman, but you’re still human. Find a safe place to talk about your own emotions — your fears and frustrations — and to get feedback and advice. Don’t be afraid to ask for help in coping with being a caregiver.

And don’t be afraid to talk about how you feel, even the feelings that you aren’t so comfortable with, like anger.

A trusted friend, a family member, a counselor, or a member of the clergy can be beneficial.

Express your own emotions. You may not feel comfortable admitting to emotions like fear, out of concern that they may come across as having a negative or pessimistic attitude.

While it is realistic not to sound alarms, expressing your own concerns can help to create an atmosphere of honesty.

If fear, for example, has become the “elephant in the room,” getting it out

in the open can relieve the tension that results from talking around the emotions that are most likely on everybody’s mind. While you want to encourage and support your partner or child, he or she most likely wants to do the same thing for you. Open the door for both of you.

And recognize where you don’t have control. Here’s the hardest one of all.

When you love someone, you also want the best for them. But nobody likes to be told what to do. Even if they probably need to be told what to do.

Be a support, a cheerleader, and throw in some tough love when you need to.

What you can do is be a supportive partner or parent. But you can’t make the pain go away. So focus on the goal of helping to motivate your partner or child to do everything they need to do to take the best possible care of him/ herself. Be a team!

You and your loved one. Here is some final advice that is guaranteed to make every day a better one. Begin and end the day with three words: “I love you.”

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

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The Individualized Healthcare Plan:

by Ann Marie M. Minichiello

What’s It All About?

The Individualized Healthcare Plan The Individualized Healthcare Plan (also known as IHP or IHCP) is a common tool used for students with special health needs. When you send your child off to school, you want to know that his or her medical needs will be met while there, and an IHP is just the place to start. A child with a bleeding disorder should have this plan in place and it should be developed by your school or district nurse. According to the National Council of State Boards of Nursing (2005), the development of an IHP must be the responsibility of a licensed nurse. However, in the absence of a school nurse, it is recommended that there still be a plan developed by caregivers, student, healthcare providers and designated school personnel to ensure continuity of care in the school setting.

The IHP is a document based on the nursing process and in essence, is a variation of a nursing care plan. It includes a nursing assessment, diagnosis, interventions, and student outcomes. It is meant to outline the health services that your child will receive in school. It should include how, when and where these services will be provided at school.

It should be reviewed and updated annually or as often as the student’s health status changes. An IHP is considered a standard for good nursing practice.

Although an Individualized Healthcare Plan is a standard of school nursing care and should be in place for any student with mild to severe health care needs, there is no law that states your child has the right to this plan.

WHAT’S the PLAN?

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The Individualized Healthcare Plan. What’s It All About? (Continued from page 37) It is designed to address only medical and nursing issues that do not impact student learning. It does not address access to education in any way; it is strictly a formalized plan to address the medical issues of a student. It does however, provide documentation of the nursing process and can be used in a legal proceeding, should the school nurse’s conduct or performance ever result in such a case. That being said, if a legally protected plan is what you want for your child, then an IEP or 504 Plan would be the appropriate and necessary route to pursue.

If it is determined that your child is eligible for special education services, then an IHP can be included in the health portion of an IEP. An IHP should also be part of a 504 plan that your child qualifies for due to a medically related health condition. When the Americans with Disabilities Act of 1990 (ADA) was first written, it was considered to be fairly strict, but certain health conditions were not

always viewed as disabling. A health condition has not always guaranteed qualification as having a disability under the 1990 Americans with Disabilities Act. For instance, it could have been argued that an individual with a bleeding disorder who successfully treated prophylactically might not have been considered to have a disability because his or her medical condition did not substantially limit a major life activity. In 2008, the ADA Amendments Act sought to expand the meaning of a disability and make the law more inclusive of those who may not have qualified previously as having a disability. As a result of this legislation, a school district must consider the effect of the disability without the use of “mitigating measures” such as medication (i.e. worst case scenario). As a result of this change, those with a medically related health condition have the right to a legally binding agreement with the school by having a 504 plan which should include an IHP for specific nursing care procedures.

Emergency Plan: Just as an IHP can be added to, or part of a 504 Plan, so too can (and should) an Emergency Care Plan (ECP) be part of the equation. An ECP is a document that requires a plan for the worst-case scenario - the emergency situations - that none of us like to think about, but that we always have in the back of our minds as parents of children with special health needs. Unlike the IHP which should be written in “nursing language,” the ECP should be clear and succinct so it is can be read quickly and easily in an emergency situation. It should be in layman’s terms, because it is not just for the school nurse but for any school staff member to implement in an emergency. It can even be formatted like a basic chart:

If you see this….

Do this….

Child is limping

This is also a plan that can be passed on to Emergency Medical Services providers (EMS).

WHAT’S the PLAN?

An IHP and an ECP are both plans that strictly address the medical issues that may arise for the student during the school day. Unlike an IEP or 504 Plan, the IHP and ECP do not have a standardized format. The format may vary by school district or even by school. While considered standard, good nursing practice, you may not find that this type of plan is the “norm” in your school district or with your school nurse. In such a case, it is important for parents to advocate for this plan for your child at school. Your healthcare provider can assist you by acting as a liaison

with the school or district nurse or by giving you a plan that you can discuss with the district’s health team. In the end, an ECP is the bare minimum of what should be in place for any child with special health needs. An IHP is a more lengthy and detailed plan that focuses on nursing procedures for your child and can incorporate an ECP. However, if you feel that your child requires more accommodations (and a legally binding agreement) that a nursing care plan does not offer, then you should seek out an evaluation for services provided under a 504 Plan.

The Basics of a Well-Developed IHP: •

should always include up to date emergency contact information

•

should include a review date

•

should always be signed by a parent/guardian and school nurse

•

should include six components 1. Assessment: gives the background information on the student/health issue 2. Nursing Diagnosis: the school nurse’s analysis/summary of the health issue and how the nurse can contribute for care of the student 3. Goals/Outcomes: clear, concise outcomes for student healthcare plan 4. Nursing Interventions: details how the nurse will meet the goals 5. Implementation: put the plan in practice 6. Evaluation: reviewing student goals and whether they have been attained; review interventions and adjust as needed based on status of the student’s health

•

The Emergency Care Plan should be part of the IHP - it must include what emergent scenarios could arise as a result of the student’s health condition and what actions must be taken in response - for any member of the school staff (not just the school nurse)

Remember, there is no standardized format for the IHP. You may see many different versions of this type of document. Your medical provider must submit written orders for any medical treatment that may be performed at school.

A blank IHP sample form is attached for you reference.

References and additional resources: http://oley.org/?page=IHP_IEP_Difference https://www2.ed.gov/about/offices/list/ocr/504faq.html

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Example IHP

Individualized Healthcare Plan

Student Name____________________________________________________DOB_______________ Address: School: Home Phone: Grade: Parent/Guardians: Teacher: Parent Cell Phone: School Nurse: Parent Work Phone: IHP Date: Doctor Phone: Review Date: Written By: Parent/Guardian Signature:_________________________________________Date:_________________ School Nurse Signature:___________________________________________Date:_________________ Assessment

Nursing Diagnosis

Goals

Nursing Interventions

WHAT’S the PLAN?

This issue’s What’s New section is focusing on a trifecta of new coping methodologies for needle phobia. We present to you 3 different programs and/or devices to help children of all ages in tolerating infusions and blood drawls, including virtual reality, a device called the “Buzzy”, and a program entitled the “Poke-R Club”.

Virtual Reality:

by Abbie Roth and Dr. Amy Dunn, MD Nationwide Children’s Hospital

A Painless Distraction

A

first-of-its-kind virtual reality experience – Voxel Bay – distracts patients with hemophilia through engagement with an immersive environment of penguins, pirates and hermit crabs during infusions and other procedures.

problem – a targeted experience that was designed and created in tandem with clinical expertise and research.”

“Every component of Voxel Bay and the headset was designed with the needs and goals of the pediatric patient with hemophilia in mind,” says Jeremy Patterson, lead for User Experience Technology Research and Development at Nationwide Children’s. “The true magic of Voxel Bay is in the entire experience that was crafted around a specific

“We wanted to do more than make something fun and cute,” says Amy Dunn, MD, director of Pediatric Hematology at Nationwide Children’s and assistant professor of Pediatrics at The Ohio State University College of Medicine.

“The aspect of clinical utility is paramount, and we were able to conduct a clinical trial with the system to measure the impact it had on our patients. We were able to show that we could incorporate our virtual reality platform into a busy hemophilia clinic without lengthening time in clinic. Additionally, the medical team, parents and children really saw what a difference Voxel Bay made in the patient experience.”

This bench-to-bedside effort exemplifies the results that can be obtained when the clinical staff and the design team work closely together.

According to Charmaine Biega, RN, nurse clinician and leader of the clinical trial, the feedback from clinic staff, patients and parents has all been positive.

“The time difference for blood draws and factor infusions after adding Voxel Bay to the clinic visit was less than one minute,” she says. “But the difference in patient experience was dramatic. It was a 180-degree change for some of our most challenging patients.”

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Six-year-old Brody is one of the patients to benefit from the integration of Voxel Bay into the clinic and part of the early pilot study.

“Brody just started getting his treatments through IV on a regular basis and was having a really rough time,” says Biega. “But the first time he used the game in clinic, he was so completely engaged in the game when the IV was administered, he just barely flinched. The difference in how patients react during a procedure when they are playing these interactive games is remarkable.”

Biega uses a tablet to interact with the game and see exactly what her

patients are seeing in their completely customized headset. Being able to have interaction between the nurse and patient was an important feature clinicians challenged the design team to create.

Since the pilot and rollout of Voxel Bay in the Comprehensive Hemophilia Clinic at Nationwide Children’s, the program has been catching the attention of others. Voxel Bay has been the recipient of several honors and awards, including being a nominated finalist at South by Southwest (SXSW) Interactive for 2017, receiving the #WhatsGood award at SXSW, being named the Children’s Miracle Network Achievement of the Year and being a

2017 Most Wired Children’s Hospitals Innovation Finalist.

“A lot of people we’ve talked with are excited about what could be next,” says Dr. Dunn. “If you think about how we can take what we’ve done, what we’ve learned, and expand it to other parts of the hospital or other parts of the patient experience, it’s a really exciting prospect.”

An exciting prospect that takes time, effort and funding to do right.

According to Dr. Dunn, the Voxel Bay team is actively pursuing partnerships and clinical research funding to explore the use of the platform to help patients in a variety of clinical situations.

Link to longer version of the article, including Brody’s story, video and additional images: http://pediatricsnationwide.org/2016/10/14/ hemophilia-virtual-reality/ Link to article about the creative process leading to the VR platform: http://pediatricsnationwide.org/2017/09/12/creative-reality/

WHAT’S NEW?

The ITP Poke-R Club was created by the PDSA for children with ITP (Immune Thrombocytopenia) to better cope with needle phobia - something the bleeding disorders community is all too familiar with. The PDSA submitted a research poster in the National Organization for Rare Disorders (NORD) Rare Diseases & Orphan Products Breakthrough Summit 2016, which was ranked in the top 3 abstracts of 40. The content for this article was pulled directly from the poster and formatted for this publication.

ITP Poke-R Club:

A Clinical Support Program Bringing Comfort and Support to Kids Coping with Immune Thrombocytopenia

Poster Authored by: Nancy Potthast, Alexandra Kruse, Jody Shy, Caroline Kruse Platelet Disorder Support Association, Cleveland, Ohio

Objective PDSA created the Poke-R Club to empower kids with Immune Thrombocytopenia by alleviating the fear and anxiety associated with needles while giving them a program and community to call their own. This study aimed to determine the efficacy and impact of the Poke-R Club on pediatric patients with ITP.

O

PDSA created the Poke-R Club to empowe fear and anxiety associated with needles w own. This study aimed to determine the effi with ITP.

Methods Surveys pertaining to demographic and clinical information, and program efficacy were distributed to 46 children enrolled in the Poke-R Club. As of June 2016, 46 children (50% female) ages 2-15 earned 910 poker chips (equivalent to 910 needle pokes).

Results Twelve children enrolled in the ITP Poke-R Club (26%, n=46) responded to PDSA surveys. The majority of respondents (58%) were 6-10 years old, diagnosed with ITP for 1-5 years (67%). All learned about the Poke-R club through PDSA; none heard about it through their hematologist, nurse, hospital, clinic or BUZZY®.

On average, children ranked their fear of pokes and anxiety level around treatment as a 6/10 (answers ranged 0-10) before introduction to the Poke-R club, and 3/10 (answers ranged 0-8) after enrollment (Figure 1); median difference was 3 between needle phobia scores before and after Poke-R club introduction (range 0-8) (p=0.002). The window of time during which participants worried about treatments, draws, or shots ranged from never worrying, to more than 2 days before, with most patients worrying the most 10 minutes before venipuncture.

Seventy-five percent of respondents had their blood drawn over 11 times in the past year. The number of times

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a child received an ITP treatment requiring a poke ranged from 11+ times to no treatment received in the past year requiring venipuncture.

A majority of respondents claimed the best part of the program was BUZZY (67%) or that it made the child feel special (58%) (Figure 2). Eight of 12 respondents liked everything about the program, three found the distraction cards unhelpful, one said BUZZY was the least useful. to demographic and clin Parents rated the efficacy ofSurveys BUZZY pertaining in pain management to 46 children enrolled in the Poke-R Club. between 0/10 to 5/10 (avg= 2) Figure 3). Eleven parents earned 910 poker chips (equivalent to 910 claimed their child only feels a small discomfort during

M

pokes but Buzzy and the Poke-R club make treatment and draws more bearable, one parent said their child couldn’t tell the difference with or without BUZZY and treatment still hurts. Eleven children appreciate the Poke-R club because they don’t feel as alone, they look forward to the prizes, and ITP, bearable; Immune Thrombocytopenia, venipun the club makes the pokes more one patient said the Poke-R Club does not make a difference to her (Figure 4). Overall, the Poke-R Club program was rated 9/10 on average (Figure 5).

K

Twelve children enrolled in the ITP Poke-R majority of respondents (58%) were 6-10 ye learned about the Poke-R club through PDS nurse, hospital, clinic or BUZZY®.

Each child receives a drawstring pouch, chip, scorecard, and BUZZY device with their first needle stick and receives additional chips and distraction cards with each “poke.”

They track their chips on a scorecard and once the child collects 10 chips, they receive a prize from PDSA. The Program Overview included with each kit has additional tips and suggestions for medical professionals and parents on easing pain and fears. Many members share on PDSA’s private Facebook group their positive experiences within the club.

PDSA partnered with BUZZY for pain relief (a vibrating ice pack that confuses the body’s nerves thereby dulling injection pain). Previous studies demonstrated a correlation between BUZZY usage during treatments or diagnostic tests requiring venipuncture and a decrease in pain scores

Conclusions Most programs for child hematology/oncology patients are created for those with cancer. PDSA designed the ITP Poke-R Club to empower kids with ITP by alleviating needle phobia and giving patients a support group of their own.

and lower anxiety levels during the procedure (Moadad 2015; Bahorski 2015; Canbulat 2015). In our study, BUZZY was proven to be the best part of the Poke-R Club program, although parents claimed that BUZZY was only partly efficacious in their child’s pain management.

Needle phobia significantly decreased after enrollment in the Poke-R Club, demonstrating that PDSA has accomplished its goal in minimizing the anxiety correlated with treatments, draws, and pokes, although Buzzy doesn’t necessarily eliminate pain from venipuncture. The program had a positive impact on pediatric patients with ITP, and is most effective for children and preteens who have chronic ITP (lasting more than a year).

In the future, community building within the Poke-R Club could be increased if enrollment in the Poke-R Club was encouraged by doctors and nurses. PDSA would like to expand the program to include more children for a greater impact and survey a larger sample size.

For more info on the Poke-R Club, go to: https://pdsa.org/resources/itp-poke-r-club.html

WHAT’S NEW?

Tired of Needles?

by Amy Baxter, MD President and CEO of MMJ Labs

How Buzzy® Can Help

B

leeding disorders are different from other chronic medical conditions – some are short duration, some lifelong, and some like inhibitors can last… who knows? What all of these have in common are lots and lots of needles. Fortunately, there are new devices proven to help with pain and fear, and new research in the field for what works.

While the media focuses on needle phobia, there is a less drastic but equally draining phenomenon impacting people

requiring frequent pokes. “Needle Fatigue” is different from phobia – on the outside, there’s no drama, no fainting, no fuss, but on the inside a low-intensity dread that can delay medicines for days. Needle fatigue is less of an “Oh, no!” and more “I’m so over it.” Research showed 94% of insulin pump users still had physiologic fear symptoms with each insertion.1 Ongoing fight or flight responses like increased heart rate, blood pressure, and cortisol release can burn you out, even when your mind is willing.

For young children, pain is punishment and scary, so addressing fear is an important first step to making shots less of a big deal. Children are

less fearful when they know what’s happening and feel in control. If your child is afraid of needles (and 68% now are2), remember to address pain, fear, and focus.

Whether fear or fatigue, new research shows a device called Buzzy® can help with pain and fear at any age, and works even better when focusing on DistrACTION cards.

Buzzy(R) is a cold vibrating needle pain management device that uses the scientific principles of the gate control theory of pain. Just like cold running water stops a burned finger from hurting, putting the vibrating Buzzy® with ice “wings” between the brain and the pain overwhelms the body’s pain nerves.

Not recommended for children under 18 months

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Over 20 clinical trials have been done with Buzzy® – the most recent showed that Buzzy® reduced the pain of an IV in children 70% by itself, and 88% when used with DistrACTION cards.3 Adults tend to be able to tolerate the ice better, for about 10% better results.

For an IV or factor, place Buzzy® where the tourniquet will go (or use Buzzy® AS the tourniquet) with the ice wings attached and the vibration turned on. Start to clean the site as usual, and access while Buzzy® is still in place to continually jam the pain nerves. Since warm blood is flowing toward Buzzy,® and you never put Buzzy® on the veins, the dilation from the vibration works like a nurse patting a vein. Studies show getting access with Buzzy® is easier than without, and equivalent to using LMX numbing cream.

The Ladybuzz® is a mini held in place with our comfort strap. The striped one is our XL, which actually has a slot so the tourniquet can go through the middle.

When introducing Buzzy,® let kids touch Buzzy,® or press Buzzy® on a hand or forearm, and lightly scratch the area distal (downstream) to the Buzzy.® “See how cold this is, and see how you can’t feel so much any more?”

Seeing for themselves and agreeing with you helps the child feel in control.

Placing the child in a “position of comfort”, e.g. with the parent’s arm around them, or facing them on a lap for younger children, also increases children’s security.

Let a child choose from multiple options to focus elsewhere during the shot. One option is to play a DistrACTION Card game: have the parent ask questions on the cards while the child looks at them. The combination of a task and focus decreases pain. DistrACTION cards have been proven in 4 studies to decrease IV pain by half by themselves.

Fortunately, as needle phobia has kept pace with the addition of new vaccines, there is also new research on techniques to decrease the drama. A Canadian group published the updated

Buzzy® XL Personal - Striped

1.

guidelines to reduce needle anxiety at various ages to which we’ve put into easy to understand PDF cards on our “Buzzyhelps/for/children” page. https://buzzyhelps.com/for/children Clinical trials on the use of Buzzy® can be found at: https://buzzyhelps.com/research/ buzzy-clinical-trials

Buzzy® XL Personal - LadyBuzz®

[Kruger DF Diabetes met 2015:8;49-56 ]

2. Taddio, Ipp, Thivakaren et al. Survey of the prevalence of immunization non-compliance due to needle fears in children and adults.

Vaccine. 2012 Jul 6;30(32):4807-12.

3. Inal, Kellici. Pediatr Emerg Care. 2017 Sep 5.

4. Sahiner, Inal, Akbay. J Perianesth Nurs. 2015 Jun;30(3):228-35. The effect of combined stimulation of external cold and vibration during immunization on pain and anxiety levels in children.

5. Taddio, McMurtry, et al. Reducing pain during vaccine injections: clinical practice guideline. CMAJ. 2015 Sep 22;187(13):975-82.

WHAT’S NEW?

Chronic Pain and Mindfulness

by Krystyn Strother

“Between stimulus and response there is a space. In that space is our power to choose our response. In our response lies our growth and our freedom.”

- Viktor E. Frankl

D

id you know that your thoughts, mood, and emotions affect the way you perceive pain and your ability to heal? What if, rather than trying to block out the pain and taking painkillers every day, it was more effective to explore the sensations of pain and illness? Sounds crazy right, but decades of research is proving that this may be the most powerful tool in fighting chronic pain.

The Pain Game. What I mean by the pain game is this pattern of experiencing pain and the brain launching into ways to get rid of the pain, how much we dislike the pain, wishing the pain away, judgements about ourselves and a whole lot of negative thoughts. This creates frustration and stress and a general sense of being stuck.

Our brain reacts automatically when pain arises and there isn’t much we can do about the initial reaction. There is, however, something we can do about that reaction. We can learn to respond as opposed to react in a way that calms the mind around the sensation and relate differently to the pain.

Awareness. This is where the practice of mindfulness comes into play. Instead of focusing on how badly we want the pain to stop, we pay attention to our pain with curiosity and without judgement. However, because our brains were built to think and to constantly judge our environment as a means to navigate safely throughout our day, this exploratory and non-judgmental practice doesn’t always come easy.

Researchers are finding that when we focus on negative thoughts and judgements, the pain becomes worse and adds fuel to feelings of anxiety and depression. Taking this into consideration, it becomes essential to engage with our pain in a different way.

When we practice focused awareness on pain, we discover a more accurate perception of pain. One that can show us that while we perceive we are in pain all day long, there are peaks and valleys to the pain levels we experience.

Mindfulness meditation, the practice of “paying attention in a particular way; on purpose, in the present moment, and nonjudgmentally”, is at the core of new treatments for chronic pain and illness and has been shown in clinical trials to reduce chronic pain by 57% 1,2.

Mindfulness works to unravel the relationship we make between awareness and thinking. “It is only awareness itself that can balance out all of our various inflammations of thought and the emotional agitations and distortions that accompany the frequent storms that blow through the mind, especially in the face of a chronic pain condition,” writes Jon Kabat-Zinn, Ph.D, in his book The Mindfulness Solution to Pain.

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Acceptance. Mindfulness applied to chronic pain asks us to develop presence of mind, acceptance of our symptoms and a willingness to learn from pain and other challenging experiences. The looks I usually get from people when I explain this to them are of the “you’ve got to be kidding me” kind, but let me tell you from experience and a plethora of science to back this up…it works.

“Acceptance doesn’t mean resignation. It means understanding that something is what it is and that there has to be a way through it.” –Michael J. Fox In a study done by Lance McCracken at the University of Chicago in 1997, 160 adults with chronic pain were questioned regarding pain and acceptance. It showed that, “greater acceptance of pain was associated with reports of lower pain intensity, less pain-related anxiety and avoidance, less depression, less physical and psychosocial disability, and more daily up time.”2

You don’t have to commit hours of your day to this practice, even just a couple of minutes each day will be helpful. Practice by yourself or with your loved ones and keep track of your pain sensation levels and frequency. Remember, this doesn’t happen overnight and takes consistency. Many of the studies showing dramatic changes in pain perception were done over an 8-week time period.

A typical meditation involves focusing on different parts of the body and simply observing with the mind’s eye what you find. This allows you to observe painful sensations as they arise, and to let go of struggling with them. When you do

this, something remarkable happens: your suffering begins to lessen.

1.

John Kabat-Zinn

2.

Cherken, D. C. (2016, March 22). Use of Mindfulnessbased Stress Reduction Results in Greater Improvement of Chronic Low Back Pain Compared to Usual Care - For The Media - JAMA Network. Retrieved September 02, 2017, from https:// media.jamanetwork.com/news-item/use-of-mindfulnessbased-stress-reduction-results-in-greater-improvement-ofchronic-low-back-pain-compared-to-usual-care/

Body Scan Meditation The idea with a body scan meditation is to gently focus your awareness on each region of the body, watching and noticing what you feel or find, and then move on to the next area. Your mind will wander or get stuck in one area.

You may start to judge the feelings or get caught up in your pain game story. Notice when that happens, take a deep breath, and move on to the next area.

Lie on something or sit, get as comfortable as you can. Place your hands and arms in any comfortable position. Close your eyes and feel your body get heavy. Focus on your natural breath as it flows in and out of the body. Is the breath deep or shallow? Smooth or ragged?

Remember, one is not better than the other, we are just observing.

Feel the rhythm of the breath in as much detail as you can.

Notice your head, the space it takes up. Do you feel any part of your breath moving around your head and face? Focus on different areas of your head: ears, nose, mouth, and eyes. Think about their shape, what their function is.

Move your awareness to the neck and shoulders, arms and chest.

What do you sense in these areas?

Are they warm or cold? Heavy feeling or light? If you have aches or pains here what is the quality of that pain? Sharp, dull, throbbing?

Name the quality of the pain in your mind and then move on. Do your best not to start a conversation with yourself about it. If your pain is intense, begin right around the edges of the pain and slowly move inward. Come back to the rhythm of your breath in this area.

Move your awareness to the middle of the back and explore sensation. Observe what you find. Move your awareness to the lower back and pelvis, taking your time and simply noticing.

Let your awareness move to the legs and notice what you find.

What, if anything aches here? Is it sharp or dull? Gently probe the edges and move inward. If it becomes too intense, broaden the focus of

your awareness so that you hold the discomfort in a wider space.

Observe the rhythm of your breath.

Does that make it less intense?

Observe the body as a whole, integrated unit, breathing as one.

Gently open your eyes and notice your surroundings. Picking one thing to focus on as you come back to your room can be helpful to anchor you.

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

Publication Designer Eric Lowe

Contributing Writers Taha Amir Dr. Amy Baxter, MD Janet Brewer, M.Ed Cheryl Cook Mike Denne Dr. Amy Dunn, MD Justin Levesque Eric Lowe Dr. Gary McClain, PhD Ann Marie M. Minichiello Georgia Panopoulas, PhD, LP Abbie Roth Krystyn Strother

Contributing Materials Nancy Potthast, Alexandra Kruse, Jody Shy, Caroline Kruse Platelet Disorder Support Association (PDAS) - Cleveland, OH

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