What's So Special About Rare?

LifeLines for HealthSM Disclaimers

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Letter From the Editors

Here’s hoping that as we put the winter behind us and march into summer, that in person programming may be part of our future. We were grateful to see so many of you via our winter/spring virtual webinar series each Thursday evening. It was wonderful to see faces that are like family at HFA Symposium in April!

Programming in 2022 may continue to look a little different. We will follow CDC guidelines and are hoping for the best! nSpiration Foundation, as a 501 c 3 nonprofit, will collaborate with CHES to present After the Shock: an Inhibitor Family Camp, One Drop: THE Rare Bleeding Disorders Consortium, (which combines the previous F7 Retreat and GT Symposia programs) LadyBugs, and Momentum. Resources are limited to fund air travel, which is what makes nSpiration Foundation an important partner. It has the ability as a non-profit to accept donations and submit grants to a wider variety of resources. Please consider donating to continue the programs that mean so much to you.

In this issue of LifeLines for Health the focus is on ultra-rare bleeding disorders; a segment of the community that has been our focus since 2012. The F7 Retreat began in 2012 and has remained a very valuable resource for those with FVII deficiency. In 2016, we hosted the Glanzmann’s Thrombasthenia Symposia to expand the services available to the ultra-rare bleeding disorder community. Our friend, Dr. Gary McClain shares on a topic that is getting a lot of attention, unconscious bias and how that can affect your perception of people and even healthcare. Looking for ways to refuel and stay healthy this summer? Megan Allen tells us how in MindBody Connection. Have you heard about FAIR? Females are Important to be Recognized is a national movement that unites national stakeholders to coordinate efforts for the diagnosis, treatment, and care of women with bleeding disorders. We are excited to be a part of this initiative as proud sponsors of the LadyBugs program for ALL women with bleeding disorders.

Want more information about live programs and events near you? Go to www.nspiration.foundation. Be sure to check out our website at www. ches.education, like us on Facebook, and follow us on Instagram to stay apprised of how to keep up to date and connected to programs. If you have an idea, comment or suggestion, don’t hesitate to let us know at info@ches.education. We love hearing from you!

Enjoy all that the summer has to bring! Outdoor spaces, fresh air, sunshine and connecting with others. We look forward to seeing you soon!

- Janet Brewer & Eric Lowe

“I think tolerance, acceptance and love is something that feeds every community.” —Lady Gaga

FEATURE

9 I What's So Special About Rare?

It's been a long and difficult path for individuals and families managing bleeding disorders. Luckily, the quality of life has greatly improved for Hemophilia A and B, as well as vonWillebrand's Disease, which make up the majority of the bleeding disorders population. But the remaining 15-20% living with other various factor/platelet deficiencies is still decades behind in treatment and care. Progression is slow, but focus is growing as HCP's and organizations like nSpiration, CHES, and many others are rising to fight for equality.

CONTENTS

WHAT’S NEW

6

I nSpiration Wins HAF Innovation Grant

The Hemophilia Alliance Foundation awarded the nSpiration foundation an innovation grant to help support nSpiration's project for SHEmophiliaTM, a passion for women with bleeding disorders to achieve equitable care.

7 I FAIR Time For Women

FAIR (Females Are Important to be Recognized) is the new Women’s Coalition for Equality in Bleeding Disorders Care. With a new name comes a refreshed approach to channel all resources in an effort to improve the care and needs of women with bleeding disorders.

FAMILY MATTERS

27 I Do You Know How That Makes Me Feel? Recognizing

and Responding to Unconscious Bias

Unconscious bias can be described as a judgement or assumption placed on someone or something based on his or her own teachings and experiences. Despite its unintentional demeanor, unconscious bias can still leave us feeling victimized if we're on the receiving end. Learn how to reduce unconscious bias as well as coping methods for yourself and teaching others.

BLOODLINES

35 I Product Updates at a Glance

New medications are always developing in the pipeline. Take a look at updates on a few of the most promising products.

MIND BODY CONNECTION

37 I

Fueling for the Summer

After an overwhelmingly strong response to a springtime webinar with registered dietitian, Megan Allen, we asked her for some pointers on feeding our families during the busy seasons of the year. And she did not disappoint with a serving of quick and organized grocery shopping, to a side of meals on the road, and topped with a variety of shortcuts!

WHAT'S THE PLAN?

43 I

504 or IEP: Making Sense of It All

With over 20 years of experience in the world of special education, Janet Brewer, M.Ed explains two education plans to determine the right choice for any child.

CONTENTS

2022Education Programs

Virtual programming

We'd like to thank all who attend our spring webinar series concentrating on the needs of the bleeding disorder community.

To receive info on additional upcoming programs and webinars visit: https://nspiration.foundation

Comprehensive Health Education Services has been serving the needs of those with rare bleeding conditions since 2009.

programs

August 19-22, 2022

Camp Zeke - Lakewood, PA

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.

More details on our programs can be found on our website: www.ches.education

July 22-24, 2022

Westin Detroit Airport Hotel

We are excited... for our continued partnership with the Cold Agglutinin Disease Foundation (CADF) and their virtual conference series that spanned from January to June 2022.

Cold A-what? You ask?

Cold Agglutinin is a rare blood disorder that causes hemolysis (when healthy red blood cells are mistakenly destroyed by the body causing symptoms such as anemia, shortness of breath and bluish discoloration of the hands and feet.) Cold temperatures can trigger symptoms, including air conditioning.

Think you may know someone with CAD or want to know more about it? Check out the CADF website at https:// coldagglutinindisease.org/

Nov. 2-4, 2022

Westin Detroit Airport Hotel

We are thrilled to partner with this amazing group of individuals to assist in their mission to provide education and support for those managing CAD.

Making me for what matters most. MY DECIDING FACTOR:

• VONVENDI® is the first and only treatment approved for routine use (prophylaxis) in adults with severe Type 3 VWD who previously received on-demand therapy

• VONVENDI is also approved for on-demand and surgical bleed management in adults with all types of VWD

• VONVENDI replaces VWF (the main issue behind VWD), and may be used with or without recombinant factor VIII as instructed by your healthcare provider

Are you ready to ask about VONVENDI for your VWD? Visit VONVENDI.com to learn more, and talk to your healthcare provider.

VONVENDI [von Willebrand factor (Recombinant)]

Important Information

What is VONVENDI?

VONVENDI is used in adults (age 18 years and older) diagnosed with von Willebrand disease to:

• treat and control bleeding episodes

• prevent excessive bleeding during and after surgery

• reduce the number of bleeding episodes when used regularly (prophylaxis) in adults with severe Type 3 von Willebrand disease receiving on-demand therapy

Detailed Important Risk Information

Who should not use VONVENDI?

You should not use VONVENDI if you:

• Are allergic to any ingredients in VONVENDI.

• Are allergic to mice or hamsters.

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

How should I use VONVENDI?

Your first dose of VONVENDI for each bleeding episode may be administered with a recombinant factor VIII as instructed by your healthcare provider.

Your healthcare provider will instruct you whether additional doses of VONVENDI with or without recombinant factor VIII are needed.

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

You should tell your healthcare provider if you:

• Have or have had any medical problems.

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

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

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

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

• Have been told that you have inhibitors to von Willebrand factor (because VONVENDI may not work for you).

• Have been told that you have inhibitors to blood coagulation factor VIII.

What else should I know about VONVENDI and von Willebrand Disease?

Your body can form inhibitors to von Willebrand factor or factor VIII. An inhibitor is part of the body’s normal defense system. If you form inhibitors, it may stop VONVENDI or factor VIII from working properly. Consult with your healthcare provider to make sure you are carefully monitored with blood tests for the development of inhibitors to von Willebrand factor or factor VIII.

What are the possible side effects of VONVENDI?

You can have an allergic reaction to VONVENDI.

Call your healthcare provider right away and stop treatment if you get a rash or hives, itching, tightness of the throat, chest pain or tightness, difficulty breathing, lightheadedness, dizziness, nausea or fainting.

Side effects that have been reported with VONVENDI include: headache, nausea, vomiting, tingling or burning at infusion site, chest discomfort, dizziness, joint pain, joint injury, increased liver enzyme level in blood, hot flashes, itching, high blood pressure, muscle twitching, unusual taste, blood clots and increased heart rate.

Tell your healthcare provider about any side effects that bother you or do not go away.

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

Please see the VONVENDI Consumer Brief Summary on the following page and talk to your healthcare provider.

Erica VONVENDI Use: On-Demand & Surgery

VWD=von Willebrand disease; VWF=von Willebrand factor.

Important facts about VONVENDI® [von Willebrand factor (Recombinant)]

This leaflet summarizes important information about VONVENDI Please read it carefully before using this medicine This information does not take the place of talking with your healthcare provider

What is VONVENDI?

VONVENDI is a recombinant medicine used to replace low levels or not properly working von Willebrand factor in people with von Willebrand disease Von Willebrand disease is an inherited bleeding disorder in which blood does not clot normally.

VONVENDI is used in adults (age 18 years and older) diagnosed with von Willebrand disease to:

• Treat and control bleeding episodes

• Prevent excessive bleeding during and after surger y

• Reduce the number of bleeding episodes when used regularly (prophylaxis) in adults with severe Type 3 von Willebrand disease receiving on-demand therapy.

Who should not use VONVENDI?

You should not use VONVENDI if you:

• Are allergic to any ingredients in VONVENDI

• Are allergic to mice or hamsters

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

What should I tell my doctor before I use VONVENDI?

You should tell your healthcare provider if you:

• Have or have had any medical problems

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

• Have any allergies, including allergies to mice or hamsters

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

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

• Have been told that you have inhibitors to von Willebrand factor (because VONVENDI may not work for you)

• Have been told that you have inhibitors to blood coagulation factor VIII

What is the most important information I need to know about VONVENDI?

VONVENDI can cause blood clots particularly in patients with known risk factors for blood clots Discuss this risk with your healthcare provider

You can have allergic reactions to VONVENDI. Symptoms may include generalized itching; rash or hives; rapid swelling of the skin or mucous membranes; chest pain or tightness; tightness of the throat; low blood pressure; shock; drowsiness; nausea; vomiting; tingling, prickling, burning, or numbness of the skin; restlessness; wheezing and/or difficulty breathing; lightheadedness; dizziness; or fainting If symptoms occur, stop using VONVENDI immediately and get emergency treatment right away

Your body can form inhibitors to von Willebrand factor or factor VIII An inhibitor is part of the body’s normal defense system If you form inhibitors, they may stop VONVENDI or FVIII from working properly Consult with your healthcare provider to make sure you are carefully monitored with blood tests for the development of inhibitors to von Willebrand factor or factor VIII

What are the possible side effects of VONVENDI?

Side effects that have been reported with VONVENDI include: headache, nausea, vomiting, tingling or burning at infusion site, chest discomfort, dizziness, joint pain, joint injur y, increased liver enzyme level in blood, hot flashes, itching, high blood pressure, muscle twitching, unusual taste, blood clots and increased heart rate. These are not all the possible side effects with VONVENDI. You can ask your healthcare provider for information that is written for healthcare professionals.

Tell your healthcare provider about any side effects that bother you or do not go away

What else should I know about VONVENDI and von Willebrand Disease?

Consult with your healthcare provider to make sure you are carefully monitored with blood tests to measure levels of von Willebrand factor and factor VIII so they are right for you.

You may infuse VONVENDI at a hemophilia treatment center (HTC), at your healthcare provider’s office or in your home You should be trained on how to do infusions by your healthcare provider or HTC Many people with von Willebrand disease learn to infuse VONVENDI by themselves or with the help of a family member

Call your healthcare provider right away if your bleeding does not stop after taking VONVENDI

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

The risk information provided here is not comprehensive. To learn more, talk with your healthcare provider or pharmacist about Vonvendi. The FDA approved product labeling can be found at https:\\www shirecontent com/PI/PDFs/VONVENDI USA ENG pdf or call 1-877-TAKEDA-7 (1-877-825-3327)

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

Copyright ©2022 Takeda Pharmaceuticals U S A , Inc

300 Shire Way, Lexington, MA 02421 1-877-TAKEDA-7 (1-877-825-3327)

All rights reser ved

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

VONVENDI is a registered trademark of Baxalta Incorporated, a Takeda company

Revised: 01/2022

US-VON-0601v1 0 01/22

©2022 Takeda Pharmaceuticals U.S.A., Inc. 300 Shire Way, Lexington, MA 02421. 1-877-TAKEDA-7 (1-877-825-3327). All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited. VONVENDI is a registered trademark of Baxalta Incorporated, a Takeda company.

US-VON-0657v1.0 04/22

by Janet Brewer, M.Ed

WINS

Hemophilia Alliance Foundation Innovation Grant

nSpiration is thrilled to announce they are a winner of a Hemophilia Alliance Foundation Innovation 2022 Award for our project for women with bleeding disorders “What is SHEmophiliaTM?”.

The HAF Innovation grant’s goal is “to encourage larger scale, creative, and unique projects, and to serve the community with resources that will improve the lives of patients with inheritable blood disorders.”

Our project, “What is SHEmophilia?”, is a national collaborative program to raise awareness of women’s existing barriers for adequate healthcare.

The year-long project will consist of videos produced by Quennel Worthy of Blurred Vision Entertainment, podcasts with Patrick James Lynch of Believe Ltd, and web-based resources.

HAF said “Your application scored well and we are enthusiastic about the project. The continued and ongoing need for education for women with bleeding disorders is important and we look forward to the short films and new website and know that this will be shared across the country.”

We are massively grateful to HAF for supporting “What is SHEmophilia?” and are thrilled to get started on this exciting project.

For more information about the project, or nSpiration itself, please contact us by using the QR code.

WHAT IS shemophilia?

emales re mportant to be ecognized

FAIR Advisory Committee

Janet Brewer, CHES/ (nSpiration)

Lew Wyman-Collins

Kimberly Haugstad, UpEquity

Stormy Johnson

Patrick James Lynch, Believe Ltd.

John Martinez, BioMatrix Specialty Pharmacy

Dr. Danielle Nance

Rocio Nunez, Latino Hemophilia Foundation

Dawn Rotellini, NHF

Dr. Robert Sidonio

FAIR Time For Women

On June 1, 2022, nearly 50 national stakeholders from the bleeding disorders community were invited to collaboratively support women in the bleeding disorders community to accomplish three specific goals; enable diagnosis, treatment, and access to medication for women with bleeding disorders. Originally formed under the name Women’s Coalition for Equality in Bleeding Disorders Care, the idea was conceptualized by Education and Advocacy Director, Ashley Gregory, Hemophilia Foundation of Northern California, and Ray Dattoli, lifelong community member and advocate. FAIR seeks to unite our nationwide stakeholders by utilizing currently existing initiatives such as HFA’s Bleeders Bill of Rights (2015), NHF MASAC Recommendations, patient advocacy organizations, physicians, and industry to achieve these goals and ensure the voices of women with bleeding disorders experiencing lack of access to diagnosis, care, and treatment are heard.

https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/ masac-document-264-masac-recommendations-regarding-diagnosis-and-management-ofinherited-bleeding-disorders-in-girls-and-women-with-personal-and-family-history-of-bleeding 03/04/2021

https://www.hemophilia.org/healthcare-professionals/guidelines-on-care/masac-documents/ masac-document-269-standards-and-criteria-for-the-care-of-persons-with-congenitalbleeding-disorders

Women’s Health Services and Equitable Access and Diagnosis and Treatment at HTC’s 04/29/2022

What's Special About RARE?

so

By: Janet Brewer, M.Ed

Although hemophilia A, hemophilia B, and vonWillebrand Disease are characterized as rare bleeding disorders (affecting fewer than 200,000 in the US), and account for 80-85% of the bleeding/coagulation disorders, rare factor deficiencies such as FI, FII, FV, FVII, FX, FXI, FXII and FXIII and rare platelet disorders such as Glanzmann’s Thrombasthenia, Bernard-Soulier, and Platelet Storage Pool Disorders are considered to be ultra-rare, making up for 15% of bleeding/coagulation disorders.

Given their low incidence, little is known about them, and establishing research studies is challenging because of the small number of patients.

Until recently, those affected by them have struggled with obtaining a diagnosis, treatment, proper care, and understanding,

BUT we still have a long way to go.

The majority of ultra-rare bleeding disorders follow an autosomal recessive inheritance pattern, meaning a child inherits one mutated copy of a gene from each parent. The parents are typically carriers and do not have the disorder.

General features of autosomal recessive deficiency of coagulation factors

APTT - activated partial thromboplastin time

APTT - activated partial thromboplastin time l PT - prothrombin time l TT - thrombin time

PT - prothrombin time

*Including dysfunctional proteins

TT - thrombin time

Web Source:

ASH Publications, Inherited Bleeding Disorders (March 2015) Roberta Palla, Flora Peyvandi, and Amy D. Shapiro https://ashpublications.org/blood/article/125/13/2052/33968/Rare-bleeding-disorders-diagnosisand-treatment

Symptoms of a Bleeding Dis rder

Joint/Muscle bleeds

Increased bleeding after minor injury or surgery

Excessive bruising

Excessive gum bleeding

Heavy menstrual bleeding

Excessive nose bleeds

HTC Population Profile Patient Characteristics

RARE FACTOR DEFICIENCIES (excluding FVIIId & FIXd)

Data Reported from 1/1/2012 through 3/31/2022

Note. This table excludes individuals with Factor VIII deficiency (hemophilia A) and Factor IX deficiency (hemophilia B). “Factors V & VIII, combined” refers to an autosomal condition, which occurs when there is a defect in a gene on one of the first 22 pairs of chromosomes called autosomes. Hemophilia A and B are X-linked conditions, which occur when there is a defect on the 23rd pair of chromosomes which determine one’s sex.

* Counts of five or fewer have been suppressed to protect patient confidentiality. Additional cells may be suppressed to prevent derivation of these counts by subtraction.

† The HTC Population Profile contains 47 transsexual individuals. For confidentiality purposes, the number of transsexual patients is too small to report by year or other characteristics. Transsexual patients have been included in the counts of male and female according to the sex assigned to them at birth since hemophilia and von Willebrand disease, the most common congenital bleeding disorders, affect the sexes differently. On August 10, 2020, the responses for this question changed such that “Male to Female” (i.e., transsexual) and “Female to Male” were removed and “Intersex” was added. The number of intersex patients is too small to report by year or other characteristics; therefore, these counts have been excluded to protect patient confidentiality.

Web Source: CDC (Centers for Disease Control and Prevention)

https://www.cdc.gov/ncbddd/hemophilia/communitycounts/datareports/2022-03/table-4-rare-factors.html

HTC Population Profile Patient Characteristics PLATELET DISORDERS

Data Reported from 1/1/2012 through 3/31/2022

* Counts of five or fewer have been suppressed to protect patient confidentiality. Additional cells may be suppressed to prevent derivation of these counts by subtraction.

Bernard Soulier syndrome

Glanzmann thrombasthenia

Gray platelet syndrome

HermanskyPudlak syndrome

Platelet function disorder, hereditary (nonspecific)

Platelet Release defect Platelet storage pool disease

Thrombocytopenia, hereditary

† The HTC Population Profile contains 47 transsexual individuals. For confidentiality purposes, the number of transsexual patients is too small to report by year or other characteristics. Transsexual patients have been included in the counts of male and female according to the sex assigned to them at birth since hemophilia and von Willebrand disease, the most common congenital bleeding disorders, affect the sexes differently. On August 10, 2020, the responses for this question changed such that “Male to Female” (i.e., transsexual) and “Female to Male” were removed and “Intersex” was added. The number of intersex patients is too small to report by year or other characteristics; therefore, these counts have been excluded to protect patient confidentiality.

Web Source: CDC (Centers for Disease Control and Prevention)

https://www.cdc.gov/ncbddd/hemophilia/communitycounts/datareports/2022-03/table-5-platelet-disorders.html

CHES cares...

Comprehensive Health Education Services (CHES) since 2012, has provided programming and support services to members of the ultrarare coagulation disorder community when it hosted the very first F7 Retreat supported by an educational grant from Novo Nordisk in Highland, NY for families affected by FVII deficiency. In 2016 and 2019, CHES was honored to provide the Glanzmann’s Thrombasthenia Symposia, supported by an educational grant from Novo Nordisk. These two programs combined in 2020 to form One Drop: THE Rare Bleeding Disorder Consortium.

Over the last 10 years, we have listened to, learned from, and supported our program participants who have become like family to us.

In preparation for this article, we reached out to our families to ask them what they felt was the most important topics to be answered by healthcare professionals; information they wished to share with others new to an ultra-rare diagnosis, what they wished they had known, and what they still would like to learn.

A very special thank you to our program participants for their insightful questions, years of trust, and support! It is because of YOU that programs such as One Drop are so successful. And of course, they would not be possible without the generous support of Novo Nordisk.

Dr. Chitlur QA & with rare

Meera Chitlur, MD

Barnhart-Lusher Hemostasis Research Endowed Chair, Wayne State University Professor of Pediatrics, Central Michigan University Director, Hemophilia Treatment Center & Hemostasis Program & Special Coag. Lab Division of Hematology/Oncology, Children's Hospital Of Michigan

Q: What symptoms would lead you to suspect a rare coagulation disorder (different than F8, F9, and vWD)?

Dr. Chitlur: Petechiae or bruises from blood draws, or blood pressure checks. The symptoms are mostly similar, but not finding anything when you check for the common disorders should be the clue in itself.

Q: What lab testing should be considered if an ultra-rare bleeding disorder is suspected? How should patients advocate for testing beyond the typical F8, F9, and vWD panels?

Looking for other factor deficiencies (check other factor activities) and platelet dysfunction should be the norm when patients have symptoms.

Q: Do factor levels increase as people get older?

No significant differences occur as you get older.

Q: What advice would you give a newly diagnosed patient with an ultra-rare bleeding disorder?

1. Find a HTC with people who know about your disorder so that they can advocate for you.

2. Don’t hesitate to ask questions.

3. Have a plan for emergencies.

4. Have a plan for surgeries/dental work.

5. Wear your medical alert, it can save your life!

Q: What products are available to treat those with ultra-rare factor and platelet disorders?

Replacement of the missing protein may be required for major bleeds or prevention of bleeding.

• FI def: Cryoprecipitate or Riastap®

• FII: Prothrombin Complex Concentrates, FFP

• FV def: FFP

• FVII def: Novoseven® and Sevenfact®, FFP

• FX def: Coagadex®, FFP

• FXI def: FFP

• FXIII def: Corifact®, Tretten®, Cryoprecipitate, FFP

• Platelet disorders: Platelet transfusion, Novoseven if platelet antibodies are present

Minor bleeds and mucocutaneous bleeding (epistaxis and gum bleeding) may be treated with antifibrinolytics in many cases. Topical agents may also be considered.

Q: What considerations should someone with mild factor deficiency take while traveling or in choosing activities such as sports and recreation?

Choose wisely! Look at options that are associated with a low risk for head trauma or any major trauma. Individual activities such as swimming, golf, tennis, track may be better options for you. Even amongst these, swimming and golf may be the least likely to be associated with trauma and therefore the safest.

1. Also, must consider making sure you are ready for the activity. Make sure you stretch, warm up and help your muscles adjust to the activity rather than just jump into it. This will prevent muscle sprains and trauma related to poor conditioning.

2. Wear protective gear where necessary- always. Do not allow your friends to dictate what and how you do things.

Reproductive Health and Women with Rare Coagulation Disorders

Treating Menorrhagia

We know so many women with ultra-rare bleeding disorders who have had their periods permanently suppressed, or an early hysterectomy, due to heavy menstrual bleeding.

Q: What are your thoughts on managing menorrhagia throughout various stages of the menstrual cycle?

There is little guidance - mostly case reports. Given this fact these are my main thoughts:

1. Finding a GYN provider who is willing to work with you is paramount.

2. This person needs to understand that causes of menorrhagia are multifaceted and thus the approach needs to be multipronged. This person needs to be open to talking to hematology on a regular basis as well. The combination heme/GYN clinics have been so important in addressing the needs of women with rare disorders.

3. Often what works for some time stops working and needs to be changed and women need to be open to trying different combinations of therapy. It can be a frustrating situation as things change and women often feel alone.

There are a wealth of other estrogen-free options that are fantastic at managing heavy menses – such as the hormonal IUD.

It is important to remember that the progesterone only IUDs are excellent at controlling heavy bleeding, and very safe in all age women. They do not increase the risk of blood clots.

Judy Simms-Cendan, MD Ian Bishop, MD

Skye Peltier, MPH, PA-C

Prevalence of Heavy Menstrual Bleeding

George JN et al. Blood. 1990;75(7):1383-1395. Kadir RA et al. Haemophilia. 1999;5(1):4048. Kouides PA et al. Haemophilia. 2000;6(6):643-648

Napolitano M et al. Haemophilia. 2016;22(5):752-759 Peyvandi F et al. J Thromb Haemost. 2011;9(suppl 1):236-245.

Ragni MV et al. Haemophilia. 1999;5(5):313-317. Toogeh G et al. Am J Hematol. 2004;77(2):198-199.

Frequency of Bleeding Symptoms in Women with Bleeding Disorders

*Restricted 217 menstruating females

**Restricted to 119 females who had pregnancy

Byams VR et al. Haemophilia 2011; 17(suppl 1): 6-13.

Frequency of Bleeding Symptoms in Women with BD’s

Reproductive Health and Women with Rare Coagulation Disorders

Treating Menorrhagia (continued)

Q: What is your opinion on using the birth control pill after the age of 35? (Some gynecologists recommend going off oral contraceptives at that age.)

• If you recommend going off them, what do you recommend in their place?

• If you recommend staying on them, at what age do you recommend women stop taking them?

Judy Simms-Cendan, MD

There are NO contraindications to using combined oral contraceptives with estrogen after the age of 35, as long as the woman does not smoke, or have other reasons to increase her risk of venous thromboembolism (blood clots), such as obesity, hypertension or diabetes. For women with bleeding disorders, this risk is very low. In general, we know the risk of venous thromboembolism is proportional to the estrogen in the birth control pills. Higher estrogen leads to higher risk. After age 35 we try to use less estrogen because the older we get, the more likely we are to have atherosclerosis in our blood vessels which increases the risk of blood clots. In general, the pills we choose have 10-20 micrograms of ethinyl estradiol, the lowest dose pills.

Ian Bishop, MD

Age itself should not be a contraindication to combined hormonal contraception (pill, patch, ring). These methods can be continued until the age of menopause in healthy, non-smoking, normal weight women – and may be especially helpful for women using combined hormonal contraception to manage heavy or irregular menses. However, there may be other comorbidities (such as smoking, hypertension, etc) that might make us think about discontinuing earlier. To mitigate the risk of thrombosis for women over age 35 who desire combined hormonal contraception, I usually put them on a pill that has 20mcg estrogen – this would be considered a “low dose” pill.

Pregnancy

Q: Do you have any thoughts on successful pregnancies for F7 and GT patients?

Any insight on prophy during the pre-implantation period to prevent miscarriage? (We know of an instance when this was successful for a woman with FVII deficiency.)

Skye Peltier, MPH, PA-C

I am most familiar with the F7 literature and there is more information about delivery than preimplantation. I have not seen preventative therapy being used here unless the woman is having an implantation procedure.

The link and QR code below provides the article Skye refers to for delivery planning in F7 patients.

https://pubmed.ncbi.nlm.nih.gov/23607277/

Research & Future Treatments

FVII Deficiency

During the F7 Retreats from 2017-2019, Comprehensive Health Education Services coordinated with Dr. Diane Nugent, Chief Hematologist at Children’s Hospital of Orange County, CA, founder of the Center for Comprehensive Care and Diagnosis of Inherited Blood Disorders (CIBD) and the Hematology Advanced Diagnostics Laboratory (HADL) that provides access to specialty laboratory testing for the diagnosis of inherited blood disorders.

Due to the coordinated efforts between Dr. Nugent, the attendees at these family retreats and CHES, at our last live event, she reported the following information:

Among the 154 individuals sequenced... variants were identified in 55 patients and 67 relatives

Among all 154 individuals, 48 different variants were detected in the Factor VII gene.

NOT

Of those variants, 15 were listed with the NCBI databank or FVII Registry.

(Family studies proved highly informative in mapping the pathologic lesions to the functional regions of the FVII molecule.)

The variant types included:

4 stop gains

Of note:

*Only 3 patients had no Factor VII variants identified

*During the 2-day FVII Retreat, consent forms, bleeding questionnaires, and blood samples were obtained from those in attendance.

*Factor levels were obtained through each patient's primary physician at the time of diagnosis, and whole gene sequencing was performed at HADL, using their validated Next Generation Sequencing (NGS) clotting protein panel which contains FVII along with 21 additional clotting genes.

Once again, there was when the FVII level was >10-20%. However, for those patients with FVII levels, <5% of the bleeding scores were usually elevated, especially in women post menarche.

58 patients

A total of 154 individuals were evaluated comprising of:

48 families with index patients

41 related siblings or offspring

48 parents

4 grandparents

Diane Nugent, MD

Poster presented at World Federation of Hemophilia in 2018Glasgow, Scotland on F7 data.

Research (continued) & Future Treatments

Glanzmann’s Thrombasthenia

Bone Marrow Transplant

Q: What are your thoughts on Bone Marrow Transplant for Glanzmann’s Thrombasthenia?

Meera Chitlur, MD

This is the only curative option for this disease. We have now transplanted 2 sibs with GT and it is truly life changing for the patient and the family! I don’t know the success rate as such because the number of reported transplants is still in the double digits and potentially <50. I would recommend it especially if there is a sibling donor.

Q: What do you see on the treatment horizon for ultra-rare factor and platelet disorders?

Meera Chitlur, MD

Gene therapy is the only curative option but may not be in the foreseeable future as this is a challenging arena. We are anticipating treatment options like Fitusiran™ which alter the pro-and anticoagulant balance to make more of a presence.

Dr. David Wilcox and his team have tirelessly researched platelets for the past three decades to improve treatment for inherited bleeding disorders. These efforts have led to significant advances in our current understanding of platelets, and of specific proteins and their role in addressing several genetic disorders. Additionally, Dr. Wilcox has been investigating the potential for utilizing Human Stem Cells (HSC) as a target for gene therapy to synthesize therapeutic proteins within megakaryocytes, to correct disorders utilizing unique properties of platelets. As recipient of numerous grants and awards, both public and private, these efforts have led to three platforms for megakaryocyte-specific gene therapy, addressing diseases including Glanzmann’s Thrombasthenia, and Hemophilia A, as well as several types of cancer.

In May of 2022, he spoke at the American Society of Gene and Cell Therapy annual conference in Washington DC regarding this research with the hope of advancing to the first in human clinical gene therapy trial.

David Wilcox, PhD

Medical College of Wisconsin, Children’s Research Institute, Children’s Wisconsin; Versiti Blood Research Institute, Milwaukee, WI

What is it?

A Rare Coagulation Disorders Project leveraging the ATHNdataset to document the state of rare coagulation disorders in the United States

Principal Investigators:

Diane Nugent, MD

•

Executive Officer, Board President/Founder, President/Medical Director

• Center for Comprehensive Care and Diagnosis of Inherited Blood Disorders

A T H N

merican hrombosis and emostasis etwork

Amy Shapiro, MD

•

•

Chief Executive Officer, Co-Medical Director

Indiana Hemophilia & Thrombosis Center

ATHN sponsors research through partnerships with 135+ federally funded hemophilia treatment centers across the United States. ATHN 10’s goals are to:

• Enhance Rare Coagulation Disorders for phenotypic/genotypic data within the ATHNdataset

• Provide free genetic testing for those with Rare Coagulation Disorders and more

Requirements:

• Established diagnosis of a Rare Coagulation Disorder

• Currently receiving care at an ATHNaffiliated Hemophilia Treatment Center

• Must opt-in to the ATHNdataset

The initial stages of this project will collect data for those affected by the following Rare Coagulation Disorders:

• Bernard Soulier syndrome

• Glanzmann’s thrombasthenia

• Factor I (Afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, hypodysfibrinogenemia)

• FVIId (Factor 7 deficiency)

• FXIIId (Factor 13 deficiency)

Those that have participated in Dr. Nugent’s studies via the 2017-2019 F7 Retreats or the 2019 Glanzmann’s Thrombasthenia Symposia offered by Comprehensive Health Education Services will NOT automatically be enrolled in the ATHN 10 study. You MUST contact your HTC to be included in ATHN 10.

For a complete overview on ATHN 10: https://athn.org/what-we-do/national-projects/athn10.html or email ATHN at support@athn.org.

Do FEEL? Me makes That how Know YOU

by Dr. Gary McClain, PhD

Recognizing and Responding to Unconscious Bias

We are hearing a lot about unconscious bias these days. Along with many examples of what unconscious bias looks like, how it affects the ways in which we communicate, how it harms those who are the victims of unconscious bias. We hear about it in the media, of course. We see examples of it in our own lives, at school, at work, in our communities. And in the offices of healthcare providers.

What about you? Have you had unconscious bias directed toward you or your child? Or have you unintentionally directed it toward someone else?

So Just What is Unconscious Bias?

Basically, unconscious bias is made up of thoughts or feelings that you may not be aware of, but yet have an influence on judgments you make. Unconscious biases come from your preferences for or against something, and these preferences may lead you to have a positive or a negative bias.

For example, you may find yourself assuming that people from certain cities are naturally rude. You meet someone from that city, and you find yourself expecting them to behave in a rude manner, you haven’t actually taken the time to get to know them yet, you just “naturally” expect them to be rude.

Or consider this example: You are walking down the sidewalk at dusk and you see someone coming toward you, mumbling, and yelling with bags in their hands, asking you for money. Most likely, you don’t take the time to think about what they are doing and how they are feeling. Instead, you would pop into a store or a restaurant that is still open, or cross to the other side of the street.

We are constantly bombarded with information. Unconscious bias helps us to organize information and make shortcut decisions. As humans, we are hardwired for unconscious bias, for better or worse.

As humans, we are a collection of life experiences. These experiences, including the families and communities we grew up in, have a major impact on the unconscious bias we have incorporated.

Is unconscious bias a good thing or a bad thing? First it is human. It is automatic. That part of our brain that makes quick judgements in a potentially dangerous situation can also, unfortunately, cause us to make judgments that are unfair and potentially damaging to another person or, on the other hand, to have those unfair judgments directed toward us.

Unconscious Bias at Your Healthcare Provider’s Office

First, allow me to emphasize that my intention in the words that follow is not to accuse your healthcare provider of being biased toward you. As I hope I made clear in the previous paragraphs, unconscious bias is “hardwired” and often unintentional.

However, that doesn’t mean that even unintended unconscious bias is not harmful when it is directed toward you. I will give you some ideas about how to advocate for yourself when you are in this situation.

But first, here are some of the forms of unconscious bias:

Microaggression

Let’s start with an example. A mother who is Latinx has a conversation with her physician’s nurse about her 12-year-old daughter. Her daughter is refusing to eat dinner with the family and instead comes into the kitchen later in the evening and picks from the refrigerator.

The nurse informs her that “Kids at that age are very weight conscious. I am sure you’re a great cook. But maybe she is tired of all the fried food and rice and beans.”

Do you see what just happened here? The nurse made a comment that reflected an unconscious bias, based on her own limited exposure to members of the Latinx community and most likely her own experiences and assumptions, starting in childhood.

Her comment, however unintended, was experienced as a microaggression. This mother may have felt disrespected and devalued. If not embarrassed and ashamed, having been made to feel by someone she is seeking advice from that she is giving her children unhealthy food without having had the benefit of being asked what she serves her family.

Furthermore, by making this assumption, the healthcare provider has potentially stopped a conversation that might have led to an important discussion about what kind of help this 12-year-old needs.

Microinsult

Consider this: A wife accompanies her husband to an appointment with his physician. Her husband has been experiencing unrelenting pain. After exploring her husband’s symptoms, the physician announces: “Well, when a man finally complains about pain, I know the pain must be real.”

The woman in this situation most likely experienced this as a microinsult. The physician, by saying that men only complain when they have real pain, is also implying that women whine and complain about pain that isn’t worth taking the physician’s time. By encouraging her husband to talk about his pain, he insulted his wife.

Hopefully, this won’t discourage her from reaching out for help the next time she experiences pain. But it could.

Microinvalidation

Microinvalidation is a topic that I frequently discuss with my clients. Here are a couple of examples for you to consider:

Example 1: A parent meets with her daughter’s teacher to talk about the way in which the teacher treated her. Her daughter has a chronic condition. She had missed two days of school and was late with an assignment as a result of having struggled to complete it as soon as she could. The teacher had scolded her in front of other students for not completing it on time and emailing it to her.

When her mother tried to explain to the teacher that she had completed it as soon as she could, the teacher interrupted her. “Research has shown that kids with chronic conditions learn to play the victim and manipulate adults.”

devastated. She began bleeding elsewhere, especially in her kidneys, gastrointestinal system, and some joints. Her muscles continued to be swollen and sore. She was told she had fibromyalgia, was a klutz, and worst of all, one physician even accused her of “liking the attention” she received from going in and out of the hospital.

Microinvalidation is communicating in a way that excludes or discounts another person’s thoughts, feelings, or experiences. For example, implying that they are being dramatic, making symptoms up, or attempting to gain attention. To be honest, I hear versions of this story frequently, from adult patients and from parents of children with chronic conditions like bleeding disorders.

The physician may feel they are being straightforward, even showing “tough love,” but they are doing emotional damage to their patient, who is left feeling that she is not being heard by the individuals who are responsible for her health care. Leaving her at risk of not receiving the health care she needs.

And revisiting the first example, the teacher may think she is using a teachable moment to educate a parent, but the parent is being made to feel like she is all too often made to feel when attempting to advocate for her daughter - like her child is being viewed as an annoyance, as well as judged.

Microinvalidation impacts the emotional and potentially the physical wellness of the person to whom this is being directed. The result of microinvalidation? Feeling misunderstood. Disrespected. In a word: Invalidated.

Example 2: A patient reports, after 4 major abdominal surgeries in a 5-year span to remove large, blood-filled endometriomas and 5 pregnancies that never came to term, she had a total hysterectomy - removing all female reproductive organs. She was 29 years old and

Microinvalidation is communicating in a way that excludes or discounts another person’s thoughts, feelings, or experiences.

So What Do I Do When I am Experiencing Unconscious Bias

Again, unconscious bias is often unintended. But let’s face it, unconscious bias can also be expressed in a very direct and intentional way. We see examples of this in the media. You may experience this in your own life.

Here are some ideas to help you to cope, and advocate for yourself, when you experience unconscious bias:

First, feelings are not always reliable. The experience of unconscious bias can bring up really strong feelings –anger, sadness, frustration – depending on the situation and our own past experiences. Keep in mind that the first rush of emotions can lead to actions that you may later regret. So, take a deep breath. Evaluate the situation. Ask yourself if your emotions are in proportion to what just occurred.

Consider intentionality. Is it clear that the other person is intending to cause emotional harm to you or your child? Are they demonstrating their own ignorance? Or are they just in a hurry or stressed and not thinking about their words? I am not saying you should make excuses for other people and let them mistreat you. But I am saying that at times giving someone the benefit of a doubt can keep a situation from getting out of control.

Identify the unconscious bias. I live in New York City, and in our subway system, we have signs that say, “If you see something, say something.” By identifying situations where we feel unconscious bias, we can help educate the other person and potentially help protect other people from having the same experience. This begins with simply letting them know how you feel and why.

If you see something, say something.

FAMILY MATTERS

Here are a couple of examples to identify unconscious bias:

The point here is to clearly identify what was said that you experienced as unconscious bias and how you were emotionally impacted. Don’t accuse. Use I-statements. Focus on what was said and your reaction. Be a “patient” educator.

Keep your expectations realistic. I constantly remind myself, and my clients, that we can’t control how other people think, feel, and behave. On one hand, pointing out an unconscious bias may be an eye-opening teachable moment that results in an apology and needed change on their part. On the other hand, the other person may not see a problem on their side and toss it back on you (that’s called gaslighting). Be aware of your options in this situation, especially if this is a professional working with you or your child. If you see something, say something. Know when it’s also time to do something.

“I am not sure what your experience has been with members of my community, but we actually don’t ________________. We _______________.”

“I wish other people understood what it is like to live with this condition. When you said this about my child, I felt like _____________________.

How Do I Help My Child?

Here is one last example of unconscious bias. It is based on many conversations I have had with parents over the years:

A high school student living with a chronic condition reports to his father that his gym teacher told him to sit on the bench and observe other students doing rigorous calisthenics. The teacher told him, “I don’t know if you should be doing this and the school doesn’t need any liability issues.”

The young man felt deeply humiliated, and a few of the other students made fun of him later. His father is outraged; his son begs him not to make an issue of it for fear of retaliation.

“I don’t want to sound like I am attacking you or that I don’t respect you. But when you said __________, I felt marginalized/ overlooked/disrespected/invalidated. I don’t think you meant it that way, but I have to let you know how I feel.”

If you are a parent, you may have similar stories. Here are a few ideas for parents:

Help your children to develop a sense of their own self-worth. Set an example of open-mindedness and compassion. Insist that healthcare providers and education professionals, and anyone else who has contact with your children, treat them with respect. Identify unconscious bias when you see it. Advocate for your children.

Create a safe space for emotions. Make your home a place where it is okay to talk about feelings, “good” feelings and “bad” feelings. Set an example by talking about your own feelings. Ask questions: What’s going on with you today? How can I help? How can I help you feel safe?

Get involved in advocacy organizations. From a healthcare perspective, join an organization that supports families living with a chronic condition. Fortunately, the bleeding disorder community has amazing local and national advocacy resources. Get your children involved in meeting other children who share their condition and get to know other parents. But also get involved in your own communities, help your children be aware of other forms of unconscious bias, and be a family that embraces diversity in all forms.

Let’s Watch Out for Each Other

Look for signs of stress in your family members, your friends, your co-workers, and your neighbors. Keep in mind that stress can be the result of being exposed to unconscious bias. Reaching out is as simple as asking “How can I help?’ Helping begins with just providing a listening ear and listening with an open and non-judgmental mind. By offering another person the opportunity to talk about what they’re experiencing, they can better frame out the situation in their own minds in the process of helping us to understand.

And if you are experiencing unconscious bias, you might also want to consider speaking with a mental health professional. We can help you to gain perspective, to validate your emotional reactions, to identify your strengths, and share some coping techniques. We’re pretty good at being objective listeners. Don’t go through this alone!

As we’ve been saying a lot during the past two years, we’re all in this together.

Gary McClain, PhD is a therapist, patient advocate, and educator, specializing in helping clients deal with the emotional impact of chronic and life-threatening health conditions, 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 each other and healthcare professionals, and to listen to their own inner voice as they make decisions about the future. His website is JustGotDiagnosed.com

by Janet Brewer, M.Ed

Product Updates at a Glance

Hemophilia A News

Sanofi and Sobi announced on 3/9/22 that their Phase 3 XTEND-1 BIVV001, Efanesoctocog alfa, a once-weekly treatment for Hemophilia A met its primary endpoints to provide statistically significant and meaningful reduction in annualized bleed rates compared to standard FVIII prophylactic treatment.

Efanesoctocog alfa is designed to provide near normal factor activity levels for the majority of the week.

https://www.sanofi.com/en/media-room/press-releas es/2022/2022-03-09-06-00-00-2399557

Hemophilia Gene Therapy News

BioMarin announced positive results on 2/4/22 at the 15th annual Virtual Congress of the European Association for Haemophilia and Allies Disorders (EAHAD) from their 2-year analysis for their Phase 3 GENEr8-1 Roctavian (valoctocogene roxaparvovec) therapy.

They reported that nearly all participants did not require standard preventive therapies, and that 80% of the participants were bleed free at the end of the 2-year period.

https://www.prnewswire.com/news-releases/biomarin-announcesoral-presentation-of-2-year-analysis-of-largest-phase-3-genetherapy-study-in-adults-with-severe-hemophilia-a-at-15th-annual-congress-of-european-association-for-haemophilia-and-allieddisorders-eahad-2-4-febr-301475471.html

Hemophilia B Gene Therapy News

CSL Behring announced on 2/4/22 at the 15th annual Virtual Congress of the European Association for Haemophilia and Allies Disorders (EAHAD) from their Phase 3 HOPE-B study on EtranaDez (etranacogene dezaparvovec) formerly known as AMT061, demonstrates durable and sustained therapeutic effects

Participants yielded stable FIX activity 18-months post treatment with 98% of study participants able to discontinue use of prophylaxis.

https://www.prnewswire.com/news-releases/finalanalysis-of-pivotal-hope-b-study-demonstrates-durableand-sustained-therapeutic-effect-of-etranacogenedezaparvovec-gene-therapy-in-hemophilia-b--datapresented-at-eahad-2022-301475671.html

VonWillebrand News

The FDA granted approval on 1/31/22 for the first and only routine prophylactic treatment with VONVENDI for adult patients (18 and over) living with Severe Type 3 vonWillebrand Disease (vWD.) VONVENDI is approved for use for on-demand and perioperative bleed management for adults with vWD.

VonWillebrand Disease is considered to be the most common bleeding disorder with approximately 3 million (1 in every 100) individuals affected in the United States.

https://www.businesswire.com/news/home/20220131005214/en/ FDA-Approves-Prophylactic-Treatment-with-VONVENDI®-vonWillebrand-Factor-Recombinant-for-Adult-Patients-Living-withSevere-Type-3-von-Willebrand-Disease-VWD

Fueling for the S

by: Megan Allen, RD, LD, CDCES

Summer

The summer months bring about many enjoyable activities that get us outside and away from home more often. Backyard barbecues with friends, family vacations, and activities with kids create fun memories that last a lifetime. However, if your goal this summer is to fuel your body to keep up with all the activities of the season, you may want to still prioritize cooking meals at home. Fueling your body for a day of fun activities doesn’t have to be complicated if you have a plan. This will look different for every individual or family, but once you learn what works best for you, planning ahead for healthy meals becomes a simple process that can be easily modified depending on your plans for the week ahead.

MIND BODY CONNECTION

Take Inventory

Take a look at your pantry, refrigerator, and freezer for anything that needs to be used in the next few days. Start to build meals around these items first. For example, peppers and onions that are starting to look a little wrinkled, can be made into stir fry, fajitas, or taco soup! Be sure to look in your freezer for frozen leftovers, vegetables, or meat that can be used first to save money during your grocery trip.

If you’re new to meal planning, start by planning ahead for two meals and two or three healthy snacks for the week. As you start to learn how meal planning fits into your schedule, you can adjust things as needed. I like to build my grocery list on my phone using a standard notes app. This allows me to quickly add items as they pop into my brain or if I run out of something. If you utilize a grocery pickup option, you can use the app for the grocery you plan to purchase from instead. Just add items to your cart as you go, most apps will save your active cart until you submit the order.

Meal options

Lasagna

Tacos Chicken stir fry

Know your “Pain Points”

When life gets busy, we each have areas of our nutrition that fall to the wayside first. Spend the most time planning and prepping for the meals, snacks, and beverages that you struggle with the most. Ideally, most people should aim to have a meal or snack at least every four to five hours to fuel your body with enough energy for the day. Drink mostly plain water and if desired, sugar free or unsweetened beverages to help stay hydrated.

Be Flexible

This is where your personal schedule comes into play. If you want to prepare all your meals at the beginning of the week, then set aside the time to do this. Another strategy is to keep a list of meals that you have planned for the week and go to that list when you’re ready to start cooking. This gives you the flexibility to change your mind if one meal sounds better than another at that particular moment. You can also send this list to family members or place it on the counter or fridge in the morning and the family can vote on what sounds best that day! With any strategy, try to prepare fruits and vegetables at the beginning of the week for snacks. When fruits and vegetables are ready to eat you will be much more likely to eat them. The goal is to make healthy options the easy and convenient choice when you’re hungry.

MIND BODY CONNECTION

Take Shortcuts

Keep in mind, you don’t have to do all the work yourself! Feel free to use frozen, and canned fruits and vegetables to save time. You can even buy frozen whole grains such as brown rice and quinoa that can save an extra 30 minutes of cooking time. To save time at the grocery store, consider using pick-up and delivery services. Not only will you get an hour or more of your day back, but it also helps ensure you’re only purchasing what you actually need to save money and make healthier choices.

If you have other people living in your household, they should have an opinion too. Especially kids! Start by giving your kids a choice of two or three fruits and vegetables to choose from. They will be more likely to eat them if they have a say in what fruits and vegetables are served. If your kids are old enough to help you prepare the fruits and vegetables, this is even better. Every small task can help get kids excited about eating healthier such as washing fruits and vegetables, choosing their favorite from the freezer, or stirring together a colorful fruit salad!

Healthy On The Go

If this summer has you and your family traveling or just wanting to spend more time outdoors and out of the hot kitchen, it can be helpful to plan ahead for easy and healthy meals to go. To minimize restaurant meals while traveling, consider packing a “snack meal” in your cooler with a variety of healthy foods that don’t require utensils such as fresh fruit, raw vegetables with single serving dips or dressing packets, hard boiled eggs, cheeses, nuts, and whole grain crackers. Grilling can be a great way to stay outdoors with family and friends while getting a quick healthy meal on the table. Consider grilling your vegetables too so you can have the whole meal cooked on the grill. And lastly, don’t forget to hydrate with water and other unsweetened and sugar free beverages. Use sparkling water, sugar free water enhancers, and fresh fruit and herbs to add flavor to your water without added sugars.

There you have it! My ultimate guide to get you started with meal planning during busy seasons of life. Keep in mind your meal planning routine may change often depending on your family’s schedule. You don’t have to keep the same routine every day of the year. Remember, while it can be frustrating in the beginning, it is well worth your efforts. Meal planning is an investment in your health and form of self-care.

Megan Allen is a Registered Dietitian and Diabetes Care and Education Specialist who works one-onone with clients to help them set personalized nutrition goals to improve their health. She became a Dietitian because she believes food is a source of nourishment for disease prevention and treatment, but also enjoyment for your life as well. Food should make you feel happy and energetic, never guilty or stressed. When working with clients one-on-one she helps them set enjoyable, behavior-based goals to improve their health beyond hitting a certain target on the scale.

504 or IEPMaking Sense of It - All!

by Janet Brewer, M.Ed

There are two federally mandated plans that all school age students are entitled to. States also are mandated to provide special education services or related services and accommodations to children with a diagnosed disability

IDEA-Individuals with Disabilities Education Act1974 Amended 2004

IDEA requires states to provide a “free appropriate public education” to children with disabilities so they can be educated to the fullest extent possible with other children. If qualified, children are provided with special education AND related services under an Individualized Education Plan.

Disabilities Include:

• Physical, sensory, mental or emotional

• Emotional Disturbance

• Orthopedic Impairment

• Hearing-Vision Impairment

• Autism

• Traumatic Brain Injury

• Other Health Impairment

• Intellectual Disability

• Specific Learning Disability

• Multiple Disabilities

If your child is diagnosed with any of the above disabilities AND they require specialized instruction-they qualify for an INDIVIDUALIZED EDUCATION PLAN.

Specialized instruction is defined as adapted instruction designed to meet the unique need of the child with a disability. The content, methodology or delivery of instruction is changed to ensure the child’s access to the general curriculum.

Section 504 of the Rehabilitation Act of 1973

This Act’s focus is on non-discrimination. It maintains “no otherwise qualified individual with a disability will be excluded from participation in, be denied the benefits of, or be subjected to discrimination under any program or activity receiving Federal financial assistance”.

American with Disabilities Act 1990

ADA is almost like an extension of Section 504. It provides for the elimination of barriers related to accessibility for the disabled to buildings, transportation, and communication.

Both 504 and ADA provide related services and accommodations to qualified individuals with a disability through a 504 plan. Its intent is to provide access or remove barriers to participation. It provides students with the same rights and services as their “NON Disabled Peers”.

How a Bleeding Disorder Fits In: IDEA-Other Health Impairment

IDEA defines Other Health Impairments as due to chronic or acute health problems such as asthma, attention deficit disorder, crohn’s or hemophilia and adversely affects a child’s educational performance.

ADA/504-Medically Related Disability

ADA/504 defines a Medically Related Disability as a physical or mental impairment that substantially limits one or more major life activities or those basic activities that the average person in the general population can perform with little or no difficulty. The individual has a record of such impairment and is regarded as having such impairment.

To find more information on 504s, IEPs, accommodations and working with your child’s school, you may access more of Janet’s work through HFA’s School Toolkit at:

http://www.hemophiliafed.org/resource-library/settingthe-stage-for-school-success-august-2011-webinar

More on page 46 g

How Does My Child Qualify?

If there are NO academic, behavior or emotional concerns:

Make an appointment with your building principal/ADA coordinator, teacher (s), and school nurse and ask to develop a 504/ADA plan.

If there ARE academic, behavior or emotional concerns:

Write a letter to your child’s teacher asking for a formal evaluation process stating your concerns and submit it to the school. The school has an obligation to complete all assessments in the areas of suspected disability (ies) within 60 calendar days.

When assessments are complete:

1. A meeting will be scheduled

2. Reports will be shared with you regarding your child’s abilities

3. The Student Assist Team will then decide if your child:

a. Has a disability

b. Requires specialized instruction in order to make process

Remember:

Remember:

Disability + Specialized Instruction = Individualized Education Plan

Disability + Specialized Instruction = Individualized Education Plan

Disability + Related Services/Accommodations = ADA/504

Disability + Related Services/Accommodations = ADA/504

What are Related Services?

Related services may include:

• Speech/language therapy

• Audiology (hearing loss)

• Psychological

• Physical/Occupational therapies

• Counseling

• Rehabilitative counseling

• School health services

• Transportation

For example:

If you are comfortable with your school district’s physical therapist then PT could be included on your child’s ADA/504 plan and they could receive physical therapy during the school day. Providing the therapist with additional information on physical therapy with an individual with hemophilia will probably be necessary, as well as contact information from the ordering physician to be sure the therapist proceeds slowly.

What Accommodations Can Be Provided?

Reasonable accommodations are provided to remove barriers related to access. For example: If your child is on crutches, ambulating safely in

the hallway is a barrier. Allowing extended travel time around the building removes that barrier. An extra set of books to prevent a heavy backpack and subsequent shoulder bleeds. A locker that is accessible from a wheelchair at the end of the row is more accessible.

Maintaining open and honest communication with your school district enables your child to be more successful. Stating your needs in a detailed, positive manner yields better results. Most school districts are very responsive, but they are working with tight budgets and are often understaffed. Letting them know that you appreciate their efforts on behalf of your child goes a long way. Also remember, there may come a time that your child may not want these accommodations such as leaving early from class while on crutches or a wheelchair as it calls unwanted, sometimes intrusive attention. More than anything, our children just want to fit it. Let them make those decisions. Their social interactions with peers are just as important, if not more important than leaving class early. Or, there are times that it is ok to “play the hemophilia card” and invite a special friend or attractive classmate to push their wheelchair or carry their books!

Sources: Federal Regulations Part 300

WHAT’S the PLAN?

84 Beach Street

Middleboro, MA 02346

CHES Mission

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

Editors in Chief

Janet Brewer, M.Ed

Eric Lowe

Editor

Janet Brewer, M.Ed

Publication Designer

Eric Lowe

Contributing Writers

Megan Allen, RD, LD, CDCES

Ian Bishop, MD

Janet Brewer, M.Ed

Meera Chitlur, MD

Eric Lowe

Gary McClain, PhD

Skye Peltier, MPH, PA-C

Judy Simms-Cendan, MD

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