Symptoms

Patients with hemophilia experience increased bleeding after injury or surgery. Sometimes, in more severe cases, patients may experience spontaneous bleeding. Severity is based on how deficient a patient is in clotting factor compared to a patient without hemophilia and the severity does not change over time.

Spontaneous bleeding often occurs in joints (elbows, knees, ankles), but can occur anywhere in the body. Spontaneous joint bleeding causes pain, tingling and tightness in the affected joint. Patients with hemophilia may also see an increase in bruising, bleeding of the mouth and gums, and frequent nosebleeds.

CFC Treatment

Clotting factors concentrates (CFCs) are the main treatment for hemophilia. CFCs provide the missing clotting factors and are intended to raise the level of clotting factor in blood closer to normal amounts. CFCs may be used on an as-needed basis whenever bleeding occurs, before and after surgeries or procedures, or more routinely to prevent bleeding.

A CFC dose is based on your weight and how it is being used. After bleeding episodes or surgeries, CFCs are usually given one to three times a day for a few days. If using for more routine prevention, the dosing may range from one to four times per week, based medication and your response to that specific therapy.

Administering CFCs

CFCs are given by an IV, directly into your blood stream. There are two ways to administer this medication: through a temporary IV line or a more long-term IV access device. Most patients can insert, access, and administer CFCs independently.

CFCs will come to you in the form of powder inside a glass container. A nurse will provide a teach on how to correctly and safely give yourself or a family member CFCs through an IV. There are a few steps that you will need to follow, including adding liquid to the powder, drawing up the medication into a syringe, and injecting it into your IV line over several minutes.

Side Effects

Side effects of CFCs include blood clots, allergic reactions, infection transmission, and inhibitor development. Infection transmission from CFCs is rare, but possible. Risk varies based on the specific product.

Side effects of emicizumab include headache, joint pain, and injection site reactions. Side effects of other therapies vary.

Medications to avoid

You should avoid medications that may further increase bleeding. Some of these medications include aspirin and most non-steroidal inflammatory drugs (NSAIDs) and blood thinners (enoxaparin, heparin, warfarin). You should ensure all healthcare providers are aware of your diagnosis before starting any new medications.

Lifestyle Changes

Nutrition

There are no dietary restrictions or requirements related to hemophilia. However, you should aim for a healthy, well-rounded diet based on the same recommendations for healthy patients of the same age group.

Physical activity

Regular physical activity is encouraged. Sports with high rate of contact (such as boxing, football, hockey, soccer, etc.) or fast-paced physical activity (motocross racing, skiing, etc.) should be avoided. Physical therapy may be a regular part of treatment if you have chronic damage or major injuries impacting physical movement.

Importance of Adherence

We want you to experience the best possible outcome while you’re on service with our specialty pharmacy. Taking the correct medication at the right time and following your doctor’s orders is very important to your hemophilia care at Chartwell. This is called adherence or adhering to your specific plan of treatment. Untreated bleeding or failure to provide treatment before surgery may result in life-threatening bleeding.

Chartwell Support

A Chartwell staff member will check in with you before your next refill is due to make sure you have all the supplies and medication you need. We also have a nurse and pharmacist on call 24 hours, 7 days a week in case of a clinical emergency.

Additional Treatment Options

There are a few other treatment options available to you at Chartwell in addition to the CFCs. Your doctor will prescribe what he or she thinks is the best treatment option based on your unique diagnosis.