If you or someone you love is living with severe hemophilia A or hemophilia B, you’re likely well aware of the risk of painful and debilitating joint bleeds. Bleeding episodes in the joints can be painful and cause problems at the time of the bleed, and they can do long-term damage too. It’s important to learn how to protect your joints from injury.

Symptoms of Joint Bleeds

Joint bleeds can be painful and make it hard to move around. One MyHemophiliaTeam member wrote, “I just had a bleed in both of my elbow joints. What could be worse? I can’t eat and am unable to do the usual things. Can’t lay down and get up, can’t brush my teeth.”

Some people realize right away when they have a joint bleed, though it may take a while if the joint is large and the bleed is small. Symptoms of a joint bleed include:

• A tingling feeling in the joint
• A warm feeling in or around the joint
• A difficult-to-explain feeling called an aura

Most people tend to have recurrent bleeds in the same joint. When a joint has more than three bleeds over a period of six months, doctors call it a “target joint.” Such joints become weaker with time and so require special care and attention.

Bleeds that don’t stop or repeated bleeds in the same joint can lead to:

• Chronic pain
• Muscle weakness
• Mobility problems
• Hemophilic arthropathy, a disabling joint disease

Tips for Protecting Your Joints

If you’re living with a severe bleeding disorder, there are several steps you can take to protect your joints and lower your risk of serious joint problems in the future.

1. Reach or Maintain a Healthy Weight

If you’re overweight, losing weight may help protect your joints. When you walk, your knees and ankles take approximately five times your body weight with every step. Since these joints are often affected by hemophilia bleeds, reducing the weight they bear by even a little bit could make a significant difference. Losing 10 pounds can reduce the weight these joints bear by around 50 pounds.

Losing weight with hemophilia can be a challenge, especially if the condition makes exercising difficult for you. However, working with your doctor to find the right combination of diet and exercise could make a big difference for your body. In general, a good place to start is eating more fruits, vegetables, and whole grains while reducing your intake of sugar, fat, and salt.

2. Exercise Within Your Limits

Though it may seem counterintuitive, exercise and physical activity are important for people living with hemophilia. When your muscles are stronger, they do a better job of protecting your joints and helping you recover after a joint bleed. One member of MyHemophiliaTeam said, “We need to exercise and walk to keep the joints operational. The moment you give in, you get lazy, and that’s the beginning of the end!”

Exercise can also reduce the number of joint bleeds you experience, which does even more to improve your joint health and help reduce joint pain. It’s also a great way to get your joints moving again after a bleed to help avoid stiffness and freezing and maintain your range of motion.

Aquatic exercises, including swimming and hydrotherapy, are scientifically proven to help improve muscle strength and physical abilities in people with hemophilia. Water naturally provides a safe environment where you can exercise without developing stress-related injuries.

Talk to your doctor before you start an exercise routine to make sure you are taking the right amount of clotting factor for exercise and that you are performing exercises correctly. They may recommend you work with a physical therapist to ensure your safety.

3. Treat Bleeds With Factor Fast

As soon as you realize you’re experiencing a joint bleed, take enough clotting factor to raise your levels to 40 percent, or to the level your doctor recommends. If you’re still bleeding after 24 hours (for hemophilia A) or 48 hours (for hemophilia B), health experts recommend administering more factor.

Note that your doctor may have a different schedule for you, and you should follow their advice if it differs. Administering factor as soon as possible can help limit the bleeding and thus the damage to the joint. You may want to consider carrying some in your hemophilia emergency kit.

4. Avoid Food or Drugs That Raise the Risk of Bleeding

Your doctor may have given you a list of medications, foods, and supplements that can increase your risk of bleeding. To give your joints the ultimate in protection, avoid foods, supplements, medications, and other items including:

• Black cohosh
• Bromelain
• Cat’s claw
• Feverfew
• Fish oil
• Flaxseed
• Garlic
• Ginger
• Ginkgo biloba
• Non-steroidal anti-inflammatory drugs (NSAIDs), like aspirin, ibuprofen, or naproxen
• Pau d’arco
• Vitamin E, especially in high doses

If you want or need to consume any of these things, talk to your doctor first. They can help you weigh potential benefits against the potential risk that they may cause bleeding.

5. Consider Joint Aspiration

Some severe joint bleeds may not respond to treatment with factor, or swelling may not go down even though the bleed has stopped. In these cases, talk to your doctor about joint aspiration. In this procedure, often performed at a hemophilia treatment center, blood is removed from your affected joint with a needle. This may help your joint recover faster and avoid long-term damage, while also alleviating pain and swelling.

Note that you may need to get an MRI scan before an aspiration so your doctor can see the joint clearly.

6. Keep Up With Joint Rehabilitation

If your doctor has recommended physical therapy or physiotherapy to help after a joint bleed or in order to prevent one, make sure you keep up with the exercises. These are specifically designed to help your body recover well and prevent long-term joint damage.

Physical therapy exercises can get tedious, especially if you’re doing them long term. Make the process more fun by watching a program you like while you do them, or by rewarding yourself for any week in which you do the entire regimen as outlined by your therapist. Once you see how the exercises help your joints recover and/or prevent future bleeds, you may be even more motivated to continue them.

7. Stick To Your Prophylactic Treatment

You and your doctor have likely talked about what you need to do to prevent as many bleeds as possible. This can involve:

• Taking various medications
• Using factor replacement therapy as a prophylaxis (taking it before you have a bleed) on a schedule
• Using other medications that can help stop acute bleeds

Some people who have received factor replacement therapy for a long time eventually develop “inhibitors,” substances in the blood that make you stop responding to factor therapy. In such cases, a doctor may prescribe a prophylactic treatment called emicizumab-kxwh (Hemlibra).

Whatever your doctor recommends, stick with it consistently to get the most benefit from your regimen. If you can't stick with part of it, or if you have questions, make sure you bring this up with your health care provider. They can help you find a program that works for you to keep your well-being and quality of life high.

New gene therapies valoctocogene roxaparvovec-rvox (Roctavian), for hemophilia A, and etranacogene dezaparvovec-drlb (Hemgenix), for hemophilia B) have shown promising results. In clinical trials, valoctocogene roxaparvovec-rvox reduced bleeds by at least 52 percent and seemed to reduce joint bleeds, too. Etranacogene dezaparvovec, in clinical trials, reduced joint bleeds by an average of 78 percent after 18 months.

Fewer joint bleeds mean a lower chance of experiencing joint damage over your lifetime. These therapies are both given as a one-time infusion. Talk to your hematology team about which treatment options may be most effective for you at reducing joint bleeds in the short and long term.

Read about the prognosis and life expectancy for people with hemophilia A.

Talk With Others Who Understand

On MyHemophiliaTeam, the social network for people with hemophilia and their loved ones, more than 6,100 members come together to ask questions, give advice, and share their stories with others who understand life with hemophilia.

What steps do you take to protect your joints with severe hemophilia? Share your tips in the comments below, or start a conversation by posting on your Activities page.