FAQs: Adults
Adults with hemophilia are usually well-informed after having lived with the condition for many years—but the following frequently asked questions may offer some new insights. Just be sure to contact your doctor or Hemophilia Treatment Centre for any medical advice.
- What does someone with hemophilia need to consider in choosing a life partner?
- What do people with hemophilia and their partners need to know when considering starting a family?
- How can a pregnant woman who is a carrier of hemophilia prepare for the safe delivery of her child?
- Are exercise and sports safe for someone with hemophilia? Will they cause injury to the joints?
- How can joint disease be treated once it has developed in adult hemophilia patients?
- What precautions do physiotherapists need to know about when working on joint flexibility and other issues in a person with hemophilia?
- What is considered risky behaviour for a person with hemophilia, and why?
- What are the pros and cons of disclosing hemophilia to friends, classmates, co-workers, and others?
- What treatment or actions need to be taken for someone with mild hemophilia?
- What are the risks of using factor treatments? Can they transmit diseases?
- If someone with hemophilia needs a blood transfusion, do they need to worry about the safety of the blood supply?
Q. What does someone with hemophilia need to consider in choosing a life partner?
A. Hemophilia is a genetic disorder that can be passed on to children. So a spouse or life partner needs to be able to accept this situation and the risks, as well as talk openly about planning a family. Genetic counselors can help with these conversations. A partner of someone with hemophilia also needs to be prepared for the potential lifestyle implications that come with treatment of a bleeding disorder, from more frequent medical appointments to restrictions on certain risky activities.
Q. What do people with hemophilia and their partners need to know when considering starting a family?
A. Hemophilia is a genetic disease that can be passed on to children through the parents. So the decision whether or not to have children in a family with a history of hemophilia can be a difficult one. Unfortunately, there is no preventable treatment that can eliminate the risk of a baby being born with hemophilia. If it's the father that has hemophilia, the risks to the future children are lower—sons would be unaffected and daughters would be carriers. If the mother is a carrier, there is a potential for sons to have the disease.
There are many excellent resources available on the genetics of hemophilia as well as counselors who can work with couples to discuss all of their options. Ultimately, the choice is the couple's—other family members cannot make these decisions and must be respectful. But everyone should be aware that there are many families with hemophilia in their genetic make-up who decide to have children, and who live long and fulfilling lives. Discussion and preparation are the key to successful family planning.
Q. How can a pregnant woman who is a carrier of hemophilia prepare for the safe delivery of her child?
A. A pregnant carrier's physician must know that she is a carrier of hemophilia in order to plan for safety. Most women with bleeding disorders have few bleeding problems during pregnancy or during childbirth. But here are still many other issues to consider during pregnancy, such as the option of prenatal diagnosis, special labour and delivery plans, postpartum care, breastfeeding decisions, and care for the newborn if it has hemophilia. A multidisciplinary approach should be taken to managing pregnancy in carriers of hemophilia.
Ideally, the woman and her partner should meet with a hematologist, obstetrician, and genetic counselor to discuss options such as prenatal diagnosis and treatment during delivery, as well as post-natal care. There are many resources available with information specific to carriers, family and pregnancy—the Canadian Hemophilia Society can provide several, as can Hemophilia Treatment Centres (HTCs).
Q. Are exercise and sports safe for someone with hemophilia? Will they cause injury to the joints?
A. Exercise and physical activity are important for adults with hemophilia. Strong muscles protect and support the joints helping to reduce the risk of bleeding. Exercise develops strength, coordination, and flexibility. These are all necessary to help prevent injury. Exercise also helps to develop good concentration, maintain a strong heart and lungs, and build confidence. People with hemophilia should stick to recommended activities, avoid those considered risky (such as contact sports), and be sure to use any sport-specific protective equipment.
Q. How can joint disease be treated once it has developed in adult hemophilia patients?
A. Over time, serious or repeated bleeds to joints can cause complications such as pain or difficulty walking. Once a joint has been damaged, it is unlikely that it will ever be as good as new. However, if synovitis (inflammation of the joint's lining) or a target joint (a joint that bleeds repeatedly and doesn't heal well) have developed, there are several treatment options.
Prophylaxis may be recommended for a period of three to six months. This means that clotting factor infusions are given on a routine schedule, usually every second day, to prevent new bleeds and allow the joint to heal better. Sometimes an exercise program is also used to strengthen the surrounding muscles and protect the joint from further injury.
Splinting or bracing may also be used to protect the joint. Strengthening exercises are particularly important if a brace is worn because some braces restrict motion and may cause the muscles to weaken.
Steroid injections can be used to speed the healing, usually done by a joint expert such a rheumatologist or an orthopedic surgeon. Factor treatment is first administered, and then a small needle is used to inject the steroids directly into the joint to settle the inflammation.
A synovectomy is a procedure used to remove the synovium. This can dramatically reduce the number of bleeding episodes, allow some healing, reduce pain, and lessen the severity of damage to the joint surfaces. Over time, a new synovium will grow back. However, synovectomy cannot make the joint like new, nor restore motion that has been lost. There are three types of synovectomy: arthroscopic (or closed), surgical (or open) and radioactive synovectomy. In cases of severe joint destruction and loss of motion, joint replacement surgery may be an option. However, joint replacement cannot be done in young active individuals or children because the materials used to replace the joint wear out.
Q. What precautions do physiotherapists need to know about when working on joint flexibility and other issues in a person with hemophilia?
A. It's important that physiotherapists understand the medical background of someone with hemophilia, and how the condition can lead to the development of joint disease and pain. There are many helpful resources on the subject, both online (including this website) and in print. The patient should refer the physiotherapist to their Hemophilia Treatment Centre (HTC) for consultation with the HTC team prior to starting—this will ensure the best treatment plan and therapies for the child's particular condition.
Q. What is considered risky behaviour for a person with hemophilia, and why?
A. "Risky" behaviour for someone with hemophilia is anything that puts them at risk of injury. It's important to weigh the benefits and consequences of these behaviours—including the possibility of less likely, but more serious, injuries such as to the head and neck. If someone with hemophilia chooses a risky behaviour, they need to realize that choosing to take some actions now can prevent them from participating in something else enjoyable later on.
Q. What are the pros and cons of disclosing hemophilia to friends, classmates, co-workers, and others?
A. The reactions of people differ upon learning someone they know has hemophilia. People sometimes have a fear of the unknown; they may treat the person with hemophilia differently upon learning of their status. On the other hand, the open and honest communication can often help people to develop closer relationships.
Not disclosing hemophilia can cause tricky situations—for instance, what to say when invited to participate in contact sports or other risky behaviours? But ultimately, the decision to disclose is entirely the affected individual's, and it should not be made by anyone else, including university staff or a manager at work . A person with hemophilia does not put anyone else at risk by working or socializing with them, and therefore should not have to reveal his condition unless he wants to. Classmates, colleagues, and others who do know, however, should discuss privately with him what to do in case of injury and make sure they have access to a list of emergency contact details.
Q. What treatment or actions need to be taken for someone with mild hemophilia?
A. A person with mild hemophilia has some naturally occurring clotting factor in their blood, which is usually enough to prevent most bleeding episodes. If the person suffers an injury, they should call their nurse coordinator for advice, and if necessary, go to the hospital for an assessment.
Q. What are the risks of using factor treatments? Can they transmit diseases?
A. Recombinant factor concentrates were developed in the 1990s to treat both hemophilia A and hemophilia B, and the risks of being infected by these products are very very small. There are rigorous safeguards in place during the manufacture of these products in order to prevent the transmission of diseases. Factor concentrates first go through a purification process and then the final product undergoes a viral inactivation process. This step involves using different heat treatments and/or solvent detergents to kill any viruses, which might still be in the factor concentrate. These viral inactivation methods are very effective in killing HIV, hepatitis B, and hepatitis C. The concentrate is then further tested for the presence of bacteria or viruses, and to make sure it meets all manufacturing standards, including potency.
As a result, in Canada:
- Since 1988, there have been no HIV infections in people with hemophilia from factor concentrates
- Since 1990, there have been no hepatitis C infections in people with hemophilia from factor concentrates
Q. If someone with hemophilia needs a blood transfusion, do they need to worry about the safety of the blood supply?
A. The modern factor concentrates used in Canada today, whether plasma-derived or recombinant, have never been known to transmit diseases like HIV, hepatitis B, or hepatitis C. Hepatitis B infection occurs in only about 1 in a million blood transfusions. Those with hemophilia have a greater chance of needing a blood transfusion, but the risk of acquiring Hepatitis B need not be a concern since because they will have been vaccinated against the virus.
For people with hemophilia who received blood products in the 1970s and 1980s, hepatitis C (for which there is no vaccine available) was a serious concern. However, today donor screening methods and viral inactivation procedures have reduced the risk of being infected with hepatitis C to near zero. Fortunately, HIV infection through factor concentrates is also a thing of the past.
It is possible that a new unknown agent could infect the blood supply. But those responsible for hemophilia care and for the blood system, including the CHS, consumer groups, the blood transfusion service (Canadian Blood Services) and organizations around the world, are watchful of new dangers and take their responsibility for the safety of blood products very seriously.