How does blood clot?
A bleed occurs when blood vessels are torn. This can happen outside the body (like a cut), or inside the body (like in a joint or muscle). The tear in the vessel causes blood to leak out into the surrounding tissues – causing a bleed.
The body reacts quickly: recruiting tiny cells called platelets, which clump together to “plug” the leak. This platelet plug attracts clotting factors to the site of the leak, which form an even stronger plug called a fibrin clot. The clot quickly stops the bleeding. This entire process is complex but happens very quickly: this is why most bleeds are quickly managed in people without hemophilia.
What happens in people with hemophilia?
People with hemophilia do not produce any or enough clotting factor. So, platelets can’t clump together as well, resulting in a much weaker fibrin clot. Their blood will clot eventually, but it takes much longer than in someone with clotting factors.
Adapted from the National Hemophilia Foundation. Available at: http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=26&contentid=5&rptname=bleeding.
How & where bleeds happen
Bleeds can happen after a minor injury, such as a cut or fall. Bleeding can also happen during surgery, such as tooth extractions. In people with severe hemophilia, bleeding may be spontaneous, meaning it happens for no apparent reason. This can happen internally, or inside the body. Internal bleeds often occur within the joints (typically the knees, elbows and ankles) and muscles. The most serious bleeds are those in the vital organs. For joint or muscle bleeds, factor replacement therapy is recommended.
The most serious bleeding episodes, that can threaten a person’s life or functioning, occur in the head, neck/throat, abdomen, kidney or bladder, and the eyes. These require immediate medical attention – so visit your nearest HTC or emergency room if this happens.
Treatment with factor replacement
The most basic treatment for hemophilia is factor replacement therapy. This involves an infusion of the clotting factor a person is lacking to control bleeding. Factor replacement therapy is not the only treatment option for severe hemophilia. Your child’s doctor will determine which clotting factor they are lacking and advise if factor replacement therapy is appropriate for them; it may not be in some patients. Many patients, especially those with severe hemophilia, receive factor replacement therapy prophylactically to prevent a bleeding episode. Factor replacement is not permanent: clotting factors leave a person’s body within 2 or 3 days. So, regular treatment is required.
Every patient is different: the frequency of infusions will be a decision you make with your healthcare team. Two common approaches are:
- On-demand therapy. Infusions are given as soon as possible after a bleed happens to control bleeding. This is often how joint and muscle bleeds are treated. Sometimes, especially with prompt treatment, one infusion is enough. In other cases, treatment is given over several days. For more serious bleeding, a patient may need 2 to 3 infusions a day, for 10 to 14 days.
- Prophylaxis (preventative) therapy. Infusions are given once or more a week to help prevent bleeds. Often, children with hemophilia receive prophylaxis therapy to help prevent bleeds from happening at all. Making smart and safe choices about activities is also key to preventing bleeds.
Can factor infusions be done at home?
While factor infusions may be given at your HTC or doctor’s office, many patients and their families choose to infuse factor treatment on their own. This allows people to treat most joint and muscle bleeds, as well as mouth and nose bleeds, at home. Remember that serious bleeds – in the vital organs – require immediate medical attention. Ask your HTC team for the signs of serious bleeds that you need to watch out for. Monitor every serious bleed, and keep your HTC team informed, so that your treatment plan can be optimized. At-home infusions require proper training, so speak to your HTC nurse to learn more.
First Aid to help manage bleeds: RICE
In addition to factor treatment, knowing the basics of first aid is important to managing bleeds. First aid can help slow bleeding for minor bleeds into joints or muscles. A good way to remember how to give first aid is the abbreviation RICE, which stands for Rest, Ice, Compression, Elevation. Here are the basics:
- Rest – When a bleed happens, that area of the body should be rested. For instance, someone with an ankle or knee bleed should limit activities. It may be useful for people with hemophilia to keep a set of crutches at home. A temporary half-cast or splint can also be used to limit movement, which may help limit a child from overusing an injured limb.
- Ice – Applying ice helps control swelling and reduce pain. Crushed ice in a plastic bag or a bag of frozen vegetables, wrapped in a towel, works well. Be careful not to apply the ice for too long as this can cause muscle weakness. Ten to 15 minutes, every two hours, is sufficient.
- Compression – Apply pressure on the bleed area to help "pinch off" the blood vessels and slow down the bleeding. Using an elastic bandage (tensor), wrap the injured part in a figure-eight pattern. Watch carefully for coolness, numbness, or a change in color in fingers or toes—if these occur, remove the bandage and re-wrap it less tightly. An alternative is a Tubigrip® sleeve. If the injury is very sore, compression may increase the pain; if so, rewrap the injury less tightly or remove the bandage altogether.
- Elevation – Elevating the area of a bleed (to a position higher than the heart) helps decrease the pressure in the blood vessels and slow the bleeding. Try using cushions or pillows to make this more comfortable.
If you have any questions on how to perform RICE or when it’s appropriate to do so, ask your HTC team.
Other treatment options
Factor infusions are the most common treatment for hemophilia, but not the only treatment available. There are also other medications that may be used for some patients.
Desmopressin is a synthetic drug based on a natural hormone called vasopressin. Desmopressin may be the treatment of choice for people with mild or moderate hemophilia A because it avoids the expense and potential risks of using clotting factor infusions. It is also particularly useful in the treatment or prevention of bleeding in carriers of hemophilia. It is not used for hemophilia B. Each patient’s response to desmopressin can vary significantly, so your or your child’s response needs to be tested before treatment begins.
Tranexamic acid and aminocaproic acid are two medications that work by helping to promote the stability of a blood clot. In hemophilia, each of these medications is usually used in specific short-term situations (e.g., oral bleeding during dental surgery, nose bleeds, menstrual bleeding) in combination with regular hemophilia treatment.
How to identify a Joint Bleed: QUICK TIPS
- A tingling, bubbling sensation (but no real pain)
- Tightness and pain (but no visible signs)
- Swelling at the joint and a hot feeling when the skin is touched
- Pain when bending or extending the joint
- Worsening swelling, and difficulty moving
REMEMBER: The earlier you treat a joint bleed, the better your chances of preventing serious damage.
MYTH: People with hemophilia don’t have long lives.
FACT: Children born with hemophilia today can look forward to long, healthy, active lives. In fact, their lifespan is considered to be nearly normal.
Learning how to take care of yourself in the face of age-related issues is just as important to people with hemophilia as it is to the rest of the population.
Learn more about aging with hemophilia