Physical fitness

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.

  1. 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.
  2. 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
      • Mild hemophilia6,10
      • Factor activity: >5% to <40%*†
      • Lifestyle: minor adjustments
      • Activity: limited activity with some pain and risk of spontaneous or micro bleeds
      Moderate hemophilia6,10
      • Factor activity: >1% to 5%*†
      • Lifestyle: significant adjustments
      • Activity: limited activity with some pain and risk of spontaneous or micro-bleeds
    • Severe hemophilia6,10
      • Factor activity: <1%*†
      • Lifestyle: vulnerable
      • Activity: high risk of spontaneous bleeds with low activity and pain with target joints
  3. 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
  4. 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.
  5. 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.
  6. 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

  1. Centers for Disease Control and Prevention. Benefits of physical activity.
    https://www.cdc.gov/physicalactivity/basics/pa-health/index.htm.
  2. 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
  3. Centers for Disease Control and Prevention. What is hemophilia
    https://www.cdc.gov/ncbddd/hemophilia/facts.html.
  4. World Federation of Hemophilia. WFH guidelines for the management of hemophilia.
    https://www1.wfh.org/publications/files/pdf-1863.pdf.
  5. National Hemophilia Foundation. Hemophilia A.
    https://www.hemophilia.org/bleeding-disorders-a-z/types/hemophilia-a.
  6. 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
  7. 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
  8. 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
  9. Weyand AC, Pipe SW. New therapies for hemophilia. Blood. 2019;133(5):389-398. doi:10.1182/blood-2018-08-872291
  10. Centers for Disease Control and Prevention. Diagnosis of hemophilia.
    https://www.cdc.gov/ncbddd/hemophilia/diagnosis.html.
  11. National Hemophilia Foundation. Playing it safe.
    https://stepsforliving.hemophilia.org/sites/default/files/playing-it-safe.pdf#overlay-context=resources/physical-activity.
  12. 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

Originally published in PEN May 2020, ©LA Kelley Communications, Inc. www.kelleycom.com All rights reserved.

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.

  1. 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.
  2. 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.

Heart and Hemophilia

Laurie Kelley June 21, 2010


Standing on top of Chimney Rock, in North Carolina yesterday, I was a bit smug as I watched the skinny twenty-something-year-olds lying on their backs, catching their breaths. At 52, I think I felt pretty good after hauling up this 404 foot mountain in the 90 degree heat. But I took the back seat to friend Kerry Fatula, who forged strongly ahead of me. Cut me some slack: she’s 10 years younger, does running and karate, and has killer quads.

If you don’t think you ever have time to stay in shape, note that Kerry is the mom of four boys with hemophilia (three with inhibitors) and also is the executive director of the Western Penn Chapter of NHF. If anyone doesn’t have time it’s her, but she still makes the time to be in the kind of shape most of us dream of.

We were exuberant being outdoors, sweating like crazy, hiking, and taking in the stunning view of the Blue Ridge Mountains. Nothing like physical activity to clear your head, make your heart pound, blood race, and muscles ache. Coming down was even more challenging. Even the muscles in our toes ached! Kerry and I enjoyed this so much, we started making pledges out loud of all the things we would resume that we put on hold, because we had been “too busy”: rock climbing (me), karate (her), road biking (me), mountain biking (her), hiking (both).


And we both noticed as we strolled through the airports to get here, how incredibly overweight America is. As patients with hemophilia, being sedentary and being overweight are terrible paths to allow ourselves to take.

In “Heart Health and Hemophilia: A New Challenge,” an article found in Perspectives, a new program by Baxter, researchers once thought that the deficiency of factor VIII in hemophilia might actually protect people from cardiovascular disease. But this has never been proven, and instead hemophilia patients, like Americans in general, should seriously examine their activity levels and diet to ensure a healthy heart. As many of you know, about 67% of Americans are seriously overweight, and I wouldn’t even be so generous, especially as I spend time people watching at many airports. It seems to me that about 80% or more of Americans are overweight.

The article says, “For people with hemophilia who do suffer a cardiovascular event, invasive treatments can be more risky, so heart disease prevention is extremely important.” And while there are no specific guidelines for preventing cardiovascular disease in people with hemophilia, adults should approach prevention in the same way as people without it – by addressing modifiable risk factors through lifestyle changes, such as quitting smoking, eating healthy and controlling their weight. The American Heart Associations recommendation that cardiovascular screening begin at age 20. Great idea considering also how sedentary our youth may be living.

The article shares the story of 47-year-old Ron, who experienced an irregular heartbeat and unusually dangerous increases in his blood pressure: Upon arriving at his local hospital, Ron told the emergency room staff that he had hemophilia, yet they treated him like they would anyone else. “They even wanted to give me blood thinners,” Ron remembered. He started looking for a doctor with whom he’d be comfortable while keeping an eye on his blood pressure and heart rate.

Eventually Ron found a family physician that understands cardiac medicine and is eager to learn more about hemophilia. He was comfortable with his choice of a new doctor. “You should have your heart checked before you have symptoms,” Ron suggests. “It might not be as easy as you think to find a doctor who knows both cardiology and hemophilia when you need one.”

Ron continues to stay active. He loves spending time outdoors, including fishing and gardening.

Some people with hemophilia become sedentary and overweight because of their joint damage. Of course, obesity and a lack of physical activity are both major risk factors for heart disease. Get active! Walk, swim, do yoga… the more active you are, the better you feel, the less sugar you crave, the less you weigh and the longer you’ll live. See you at the summit!

(Parts of this blog are excerpted from Perspectives, an educational resource produced by Baxter, which provides insight into opportunities and challenges that are relevant to adults, featuring members of the community who share their personal stories. Reprinted with permission.)

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