Laurie Kelley

5 things to know about hemophilia, factor activity, and exercise

Laurie Kelley February 5, 2023

Sponsored by Sanofi

With 2023 now under way, getting more exercise is probably on a lot of people’s minds. It’s a great time to make new plans to get moving! For people living with hemophilia, there are extra considerations to make sure you can safely participate in physical activities. Below are 5 things to know about exercising when you have hemophilia—and how factor activity levels play a role. Remember that you should always speak to your doctor before starting any new type of activity.

Exercise is important for people living with hemophilia. Engaging in physical activity is associated with both physical and mental health benefits¹—and that goes for people who have hemophilia, too. In fact, exercising can improve joint health and reduce joint bleeds.² Hemophilia severity, factor activity, and other personal health considerations may all affect what kinds of exercises you and your doctor decide are the best fit for you.
Factor activity levels determine hemophilia severity. Your factor activity level is how much clotting factor you have in your blood at a given time. These levels are used to help determine how severe your hemophilia is at the time of diagnosis.³ Higher factor levels in your body over time means better bleed protection. Factor activity levels and severity may affect the amount of risk that comes with certain forms of exercise.⁴
- Categories of hemophilia severity and how they affect lifestyle,
  - Normal (non-hemophilia range)^{5, 6, 7, 8, 9}
  - Factor activity: 50% to 150%*†
  - Lifestyle: normal
  - Activity: High impact activity possible with no pain
- Near normal (non-hemophilia range)
  - Factor activity: >40% to <50%*†
  - Factor activity levels between 40-50 percent are currently undefined by World Federation of Hemophilia guidelines
- - Factor activity: >5% to <40%*†
  - Lifestyle: minor adjustments
  - Activity: limited activity with some pain and risk of spontaneous or micro bleeds
  Moderate hemophilia^6,10
  - Factor activity: >1% to 5%*†
  - Lifestyle: significant adjustments
  - Activity: limited activity with some pain and risk of spontaneous or micro-bleeds
- Severe hemophilia^6,10
  - Factor activity: <1%*†
  - Lifestyle: vulnerable
  - Activity: high risk of spontaneous bleeds with low activity and pain with target joints
Keep your hemophilia severity in mind when choosing types and levels of exercise. There are all kinds of ways to get exercise, depending on your hemophilia severity, your preferences, and your doctor’s recommendations. For example, people with moderate hemophilia may be better off with lower risk activities such as swimming, walking, or stationary biking.¹¹ People with mild hemophilia may be able to participate in activities with more moderate risk, like weight lifting, hiking, rowing, or snorkeling.¹¹
There are also many ways to get exercise that aren’t the recreational activities that might immediately come to mind. Everyday activities like gardening or cooking are good ways to move your body and be productive at the same time.¹² While there are many benefits to exercise and many ways to get active, no activity is completely risk free. Work with your doctor to choose the best kind of exercise for you.
Treatment may affect factor activity and lifestyle. When your factor levels stay above 40% (in the near-normal to normal non-hemophilia range), you may be able to engage in activities longer.^7,8,9 This is why many people look for treatments that improve bleed protection to allow them to spend more time doing the activities they enjoy. You may want to talk to your doctor about what your treatment is doing for your factor activity levels and how that may impact your lifestyle.
Every person is unique. No two people living with hemophilia are identical. There are differences between hemophilia A and B, everybody’s factor activity levels are different, and everyone’s body reacts differently to exercise. Talk to your doctor to explore the options that are right for you. No matter what you decide together, it’s a great time to start the conversation.

To learn more, visit Levels Matter, a Sanofi website dedicated to helping people understand factor activity levels, how blood clots, how hemophilia is inherited, and more. The more you know, the better you will be equipped to make the right decisions for your lifestyle.

*Severity classifications may be different for women with hemophilia
†Factor levels may not reflect bleeding patterns

REFERENCES

Centers for Disease Control and Prevention. Benefits of physical activity.
https://www.cdc.gov/physicalactivity/basics/pa-health/index.htm.
Harris S, Boggio LN. Exercise may decrease further destruction in the adult haemophilic joint.
Haemophilia. 2006;12(3):237-240. doi:10.1111/j.1365-2516.2006.01214.x
Centers for Disease Control and Prevention. What is hemophilia
https://www.cdc.gov/ncbddd/hemophilia/facts.html.
World Federation of Hemophilia. WFH guidelines for the management of hemophilia.
https://www1.wfh.org/publications/files/pdf-1863.pdf.
National Hemophilia Foundation. Hemophilia A.
https://www.hemophilia.org/bleeding-disorders-a-z/types/hemophilia-a.
Martin AP, Burke T, Asghar S, Noone D, Pedra G, O’Hara J. Understanding minimum and ideal factor levels for participation in physical activities by people with haemophilia: An expert elicitation exercise.
Haemophilia. 2020;26(4):711-717. doi:10.1111/hae.13985
Iorio A, Iserman E, Blanchette V, et al. Target plasma factor levels for personalized treatment in haemophilia: a Delphi consensus statement. Haemophilia. 2017;23(3):e170-e179.doi:10.1111/hae.13215
Skinner MW, Nugent D, Wilton P, et al. Achieving the unimaginable: Health equity in haemophilia. Haemophilia. 2020;26(1):17-24. doi:10.1111/hae.13862
Weyand AC, Pipe SW. New therapies for hemophilia. Blood. 2019;133(5):389-398. doi:10.1182/blood-2018-08-872291
Centers for Disease Control and Prevention. Diagnosis of hemophilia.
https://www.cdc.gov/ncbddd/hemophilia/diagnosis.html.
National Hemophilia Foundation. Playing it safe.
https://stepsforliving.hemophilia.org/sites/default/files/playing-it-safe.pdf#overlay-context=resources/physical-activity.
World Federation of Hemophilia. Psychosocial Care for People with Hemophilia.
https://www1.wfh.org/publication/files/pdf-1198.pdf.

Bulletproof Your Target Joints

Laurie Kelley January 29, 2023

Dr. Michael Zolotnitsky, PT, DPT

Growing up with hemophilia, I endured frequent joint bleeds into my ankles and knees while playing basketball. I was told that those were my target joints because I continued to bleed into the same joints, and that ultimately, this would cause hemarthropathy. I was put in braces, and told to use crutches, ice, rest, and elevate. All good things, right?

Well, have you heard of Steph Curry, the all-pro basketball superstar? He continuously experiences ankle sprains that force him to miss games. In our community, we would say that Steph has an ankle target joint; but he doesn’t have a bleeding disorder. So why do some people with a bleeding disorder get target joints, and others who experience similar injuries do not?

If an athlete has a low-level ankle or knee sprain, it takes 4–6 weeks for a ligament injury to recover. Recovery includes exercise to strengthen the lengthened ligament and exercises to stabilize the joint. A more intense sprain or strain takes 8–12 weeks to heal, with additional stability training recommended. Someone with a bleeding disorder has different recommendations: usually rest, avoid weight bearing, and ice. If this is all we do, we develop scar tissue, lose range of motion, and lose muscle mass. This turns into a negative cascade of events, because now that particular joint has limited mobility, stability, and flexibility.

When a joint is in a weakened state, it’s more likely to be reinjured. After four to six injuries per year, we call this cascade a target joint. This is all preventable with the appropriate post-injury exercise regimen. We must “bulletproof” our target joints, but how?

Let’s look at different treatment stages following an injury. When the initial injury occurs, we feel pain, our joint swells, it feels warm and tingly, and we begin to limp. First, follow the treatment protocol that is recommended by your hematologist. After that, consider this four-step return-to-activity protocol that I recommend:

Step 1: The initial focus is to reduce swelling, so apply kinesiology taping specifically for edema reduction by using the fan strip.¹ (See “HemoDoc” videos on YouTube.)

Step 2: Active range of motion of the joint can improve blood flow in a non-weight-bearing position. For the ankle specifically, you can trace the alphabet with your foot, make ankle circles, and do gentle towel curls.² This will reduce scar tissue adhesion and reduce the loss of range of motion.

Step 3: Now begin to strengthen the joints and ligaments by employing gentle resistance. It takes three days to lose strength, but six weeks to regain it, so for a low-level ligament sprain, I recommend performing these exercises three to four times per week, for six to eight weeks.

Step 4: Gently and gradually return to activity.

In my first 13 years of life, I was continuously in and out of the hospital for insidious joint bleeds. I began to exercise and took control of my own life. This is what inspired me to become a physical therapist. And for the past 15 years, I have not experienced joint bleeds, and I can say that I don’t have target joints. My dedication and focus now are to help people affected by bleeding disorders.

We are just like normal people. We get hurt; our muscles and joints are weakened. If we don’t take the appropriate measures to rehabilitate them, then we’re more likely to reinjure them, just like NBA star Steph Curry. Joint injuries are preventable with appropriate workouts and mobility exercises. Allowing our muscles to regain their strength is possible, and will ensure improved overall joint health. Let’s all bulletproof those target joints!

Dr. Michael Zolotnitsky is director of neurological rehabilitation at New Jersey Spine and Wellness in Old Bridge, New Jersey. He also has severe hemophilia A. He can be reached at 732-952-2292 and michael.zolotnisky@spineandwellness.com.

A fan strip is a piece of tape that is cut in the shape of a fan. The head of the fan is placed above the area of the swelling, and four pieces are applied over the area without tension. This will allow a negative pressure gradient to occur to reduce swelling and improve circulation.
Towel curls strengthen the inner foot. Place a towel flat on the floor and use your toes to grab the towel and curl it toward you. Then use your toes to push it away from you. Increase resistance by placing a weight on the towel.

What a doll!

Laurie Kelley January 22, 2023

Our eminent researcher of all things hemophilia, Richard Atwood of North Carolina, has found a book with “paper” dolls related to hemophilia! I certainly had paper dolls growing up, and apparently, we still have them for kids these days.

Nicholas and Alexandra Paper Dolls, published in 1998, is an oversized book (9”x12”) on the family of the last Romanov Tsar, Nicholas II, whose son Alexei had hemophilia B. The book includes 11 dolls on 16 plates printed on lightweight cardboard. There are two pages of text with notes and instructions to provide historical insights and fashion commentary. Alexandra has two dolls, accompanied by six dresses, including her 1894 wedding dress and 1896 coronation dress, plus ball gowns. Nicholas has two dolls, accompanied by five uniforms. Alexei’s older sisters, Olga, Tatiana, Marie, and Anastasia all wear the same casual dresses, that may be covered by two sets of identical dresses. Alexei is dressed in a sailor suit that may be covered with a uniform. Other dolls are the mad monk Gregory Rasputin in a monk’s garb and Clementy Nagorny in a sailor suit. The book cover illustration could also be used as dolls for Nicholas, Alexandra and Alexei.

The life of Alexei is briefly summarized. Alexei was born in August of 1904. From his mother, he inherited hemophilia B that was soon evident to the family, who did not publicly reveal it. Hemophilia was a deadly disease then, with no known cure. The bleeding bouts caused pain. Gregory Rasputin is described as a healer who used hypnosis to relieve Alexei’s suffering. Rasputin is also described as a lecher, a drunkard, and a con man. Clementy Nagorny was one of two sailors who acted as Alexei’s nanny.

Alexei is correctly shown with a bent left leg, though the metal brace he wore for most of 1913 is hidden. Something not revealed in the text is that Alexei’s accident at Spalo in 1912 caused femoral neuropathy (or paralysis of femoral nerve) due of an iliacus hematoma (or an iliopsoas muscle bleed). This was the medical reason for his hip flexion and bent leg. The metal brace, not Rasputin, straightened Alexei’s left leg. The instructions printed in the book do not mention the need for a sharp cutting tool such as an X-Acto knife in order to remove the dolls and clothes from the book pages.

The author, Tom Tierney, who is an artist, has signed each of the 11 dolls. Tierny is known for his series of paper dolls for a variety of historical figures, including Queen Elizabeth II, Michelle Obama, Abraham Lincoln, Shakespeare characters, and more.

And, you can still buy this on Amazon.com!

From Mineola, NY: Dover Publications. 32 pages.

Name Game

Laurie Kelley January 15, 2023

Companies put a lot of money into “branding,” which is the process of creating a unique identity for a company or product. Branding involves using text, names, colors, and graphics to forge an emotional attachment to potential consumers. The goal is to get consumers to recognize a company or product, and have some positive association with it, to improve the chances of selling goods or services.

Pharmacy Times says “Naming a new prescription drug is a long and complex process, costing upwards of $2.25 million.”

Branding comes from the ancient rite of marking cattle, so that each cattle would be recognized as belonging to a herd and a particular owner. Each owner had their own symbol, scorched into the animal’s hide.

Read a great article about its origin here.

In the bleeding disorders community, it’s very important for different factor manufacturers to have strong brands, as so many products seem the same. And yet, they each need some recognizable name for their function. Our newest therapy is the gene therapy for hemophilia B called Hemgenix. That’s an easy name to decipher. Hem=blood. Gen=gene. IX=B (for hemophilia B).

Have you ever thought about what the other factor names mean?

Advate: “Advanced factor VIII (eight)”

BeneFIX: Bene means well, and FIX is factor IX.

NovoSeven: Novo means new, and Seven refers to factor VII.

Jivi: it’s not obvious it’s for factor VIII. Jivi is the Hindi word for “life.”

As for the others, I’m not sure. What are you guesses? Afstyla, Alprolix, Esperoct, Idelvion?

One suffix I encountered for company names and was curious about was “Rx.” We all know it’s used for prescriptions but why? Rx is used in some pharmacy company names like DiRx Pharmacy. I’m guessing is means direct treatment? And GoodRx is easy to figure out.

According to the book Who Put the Butter in Butterfly? “R” is the symbol of the Roman god Jupiter, the patron of medicines. Rx is also an abbreviation of “recipe,” from the Latin recipere, to receive. R appeared on top of all prescriptions, denoting “to take”: directions then followed. Even the English word recipe originally referred to medical prescriptions. Over time the word was also used for cooking–not unusual, as many of the same herbs and spices in cooking were being used in prescriptions at the time!

Flavored medicine has been around longer than we think!

I Love Ladybugs

Laurie Kelley January 8, 2023

I really do. I think they are the prettiest of bugs, and look so comical, with their bright red shells. In my household, we believe they bring good luck.

I also love LadyBugs, the national program designed for women with bleeding disorders. I remember years ago when it was just introduced, by such great women in our community who truly care. And nowadays it is operated by Comprehensive Health Education Services (CHES), and good friend Janet Brewer.

LadyBugs is beginning their Winter Webinars, starting with a fun opening session with our sponsors and a sneak peak at the SHEmophilia initiative. Topics will include:

January 26th, 7:30pm ET – Opening Night
February 23rd, 7:30pm ET – Bleeding Disorder Breakouts
March 23rd, 7:30pm ET – Reproductive Health
April 27th, 7:30pm ET – ReBalancing the Body with Diet with Medexus

Who’s invited to Register?

Any woman who has a bleeding disorder, carries a bleeding disorder or cares for a loved one with a bleeding disorder.

How to attend?

Join this session from your home as it will be hosted via Zoom. If you just want to watch, that’s okay. We love to see faces, but you can leave your camera off. Registration is necessary so we can plan accordingly.

You don’t want to miss these events! Register here.