Is Hemophilia curable?
No, but symptoms of hemophilia can be controlled. Proper medical management of hemophilia can reduce the risk of bleeding as well as other co-morbidities including hemarthrosis and muscle bleeds.
When using factor products at home:
- Always follow the doctor’s treatment plan.
- Make sure to have enough factors on hand; do not allow your supply of factors to drop below a one-week supply.
- If you are traveling, be sure to bring enough supply to cover you for the entire duration of your trip.
- Always check the expiration date of your factor before infusing and use factors that will be expiring earliest. Do not use factor products that are already expired.
- Injections into a muscle should be avoided unless directed by your doctor.
- Factor products should be stored in the refrigerator and kept away from heat.
- Be sure to keep a factor log. Accurate documentations of when factors are used can help monitor how well hemophilia is being managed and prompt providers to change or alter treatment plans when necessary.
What are antibodies or inhibitors?
It is important to note that antibodies (also called inhibitors) may develop at any time but most commonly during the first 6 months of treatment. When antibodies attack infused clotting factors, the effectiveness of the clotting factor may be decreased or even eliminated, which can lead to uncontrolled bleeding. Presence of inhibitors should be suspected when bleeding continues despite factor infusions. Inhibitors are usually diagnosed via the Bethesda titer. Individuals with hemophilia who have developed antibodies to clotting factors may need to receive inhibitor therapy.
Should I be concerned about blood borne infections?
Advancements in blood screening and medical technology have drastically reduced your risk of becoming infected through receiving any factor products. The third generation recombinant factor products (e.g. Advate, Novoeight, Xyntha, Benefix, Ixinity, etc.) do not carry the risk of blood borne infections. Although plasma derived products do carry this risk, advancements in medical technology have demonstrated successful prevention of viral transmissions from using these products. Please contact your physician if you have concerns regarding your factor product.
What is hemarthrosis?
Hemarthrosis refers to bleeding into a joint. This will typically cause swelling of the joints and joint pain. Often times, osteoarthritis could be confused with hemarthrosis, leading to misuse of factor prodcuts.
Signature symptoms of hemarthrosis include:
- Warmth and/or tingling in the joint during the early stages of hemarthrosis. This is often called an aura. Treatment for the bleeding is imperative to prevent mild discomfort from progressing into severe pain.
- Inflammation and swelling at the joint area from repeated episodes of bleeding may lead to chronic pain and joint destruction.
- Infants and toddlers with hemophilia may exhibit a reluctance to move a leg or an arm because of bleeding into an affected joint. A bleeding episode of this nature generally occurs around the age of eighteen months or when first learning to walk.
Key Patient Information
- Hemophilia factor products may be administered at home by a designated health care profession or by patient/caregiver who has been properly trained. There is risk for infection regarding self-administered home infusion if poor techniques are employed. Proper patient/caregiver education regarding safety measures and injection technique is critical to avoid infections (especially with children and people who have developed inhibitors to the factor).
- Protective gear, including helmets or knee pads, are often used by children and teenagers with hemophilia to allow them to remain active while protecting them from injury in certain sports or other activities.
- Maintaining a healthy body weight helps limit the stress placed on your joints. When joints are stressed from being overweight, more bleeding episodes usually occur.
- Talk to your doctor about creating an exercise plan that is safe for you. Regular exercise strengthens the joints and muscles, which help prevent bleeding. (Exercise such as swimming that alleviates excess pressure on your joints is usually recommended.)
- Visit the dentist or hygienist for dental care every 6 to 12 months.
- Do not take non-prescription medications unless your doctor tells you to. Avoid non-steroidal-anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Motrin or Advil) and naproxen sodium (Aleve), which can affect the clotting of your blood.
- Recognize bleeding episodes early so if an injury or spontaneous bleed occurs, you can start treatment immediately. Common signs of bleeding include bruising, dark and tarry stool, redness in urine, significant pain and loss of range of motion in the joints.
- Always keep in close contact with your physician and or clinical coach to be sure you have the appropriate amount of factor and necessary supplies on hand should you need an infusion.
- If you live in close proximity to a nationally recognized Hemophilia Treatment Center (HTC), you may consider visiting the HTC to optimize your care. Please contact your clinical coach to help you find your local HTC.
Living with Hemophilia
Hemophilia can be challenging for both patients and their families. It is important to be able to recognize any signs of bleeding, start factor infusions as quickly as possible, and seek medical attention when necessary.
If you experience any of the follow, you should seek medical advice:
- Muscle pain
- Head or neck trauma
- Abdominal trauma
- Joint pain
- Continuous bleeding despite factor treatment
Depression is common in patients with chronic illness. Both adults and children may get depressed. People with a chronic illness like hemophilia have a 25-33% chance of developing depression.
Symptoms may include:
- Loss of interest in daily activities and/or feeling great sadness
- Loss of appetite or increased appetite
- Inability to experience pleasure
- Sleeping too much or having difficulty sleeping
- Headaches or general aches and pains
- Feeling guilty or worthless
- Difficulty completing tasks or concentrating
- Thoughts of death or suicide
- Anxiety attacks
- Tearfulness or being unable to cry
- Soft, slow speech
Depression can be successfully managed via medications and counseling. Patients who experience any of the symptoms above should seek medical help.
Overcoming Medication Challenges
It is important for you to know the benefits of your medications. Here at Magellan Rx, we understand your concerns and want to help you with any difficulties you may encounter. For additional information on how to overcome medication challenges, please click here: Overcoming Medication Challenges