Diplomat Specialty Pharmacy® and Diplomat Specialty Infusion Group® are joining the Optum® family.

To learn more about what this means for you, visit our FAQ pages:

Specialty Pharmacy FAQ  •  Infusion Pharmacy FAQ

Learn More About Hemophilia A


About 80 percent of people with hemophilia have Type A, meaning they have little or no clotting factor VIII in their blood. About 20 percent have Type B, where they’re missing or have low levels of clotting factor IX. However, types A and B both cause the same issues for patients.

About half of patients with hemophilia A and a third of patients with hemophilia B have the severe form. Those patients have less than 1 percent factor VIII activity in their blood. (For comparison, people without hemophilia have 50–100 percent factor VIII activity.)

Hemophilia affects about one in 7,500 males. Female hemophilia patients are rare.

Delaying hemophilia therapy can have significant consequences. Patients can develop inhibitors — antibodies that fight the treatments. They can also develop joint pain, swelling, and bleeding that can damage or destroy the joints.

Treatment for hemophilia patients has progressed a long way. With proper treatment, children diagnosed with hemophilia today can live as long as children without it.

Adapted from the National Heart, Lung, and Blood Institute and the National Center for Biotechnology Information



Hemophilia is caused by an issue with a gene on the X chromosome. Females rarely have hemophilia because both X chromosomes must have the gene. If a female has one X chromosome with the faulty gene, she will be a “carrier” who can pass the gene to her children.

A male usually has hemophilia because his mother was a carrier. But in up to 30 percent of hemophilia cases, a healthy gene mutates and passes from mother to son.

Signs & Symptoms

Signs & Symptoms

Hemophilia patients will bruise more easily than people without hemophilia. The signs and symptoms will vary by how severe the hemophilia is.

Severity of Hemophilia
  • Mild hemophilia: 5–40 percent of normal clotting factor

  • Moderate hemophilia: 1–<5 percent of normal clotting factor

  • Severe hemophilia: <1 percent of normal clotting factor

Hemophilia is classified by the amount of clotting factor in the blood, not by how severe the symptoms are. Symptoms might not match the indicated category.

Patients with hemophilia might notice these signs:

  • External bleeding, or bleeding outside the body. Nose and mouth bleeding are common. Bleeding is especially concerning if it has no obvious cause, seems abnormally heavy for the wound size, or starts again after it has stopped.

  • Internal bleeding, or bleeding inside the body that can’t necessarily be seen. External signs can include large bruises or blood in the urine or stool. Blood might also collect in joints. This will cause swelling and pain when the joint bends. If the bleeding isn’t treated, the joint can be permanently damaged.

Life-threatening bleeding can occur in the brain after a simple bump on the head. Symptoms include long and painful headaches, stiff neck, sleepiness, vomiting, double vision, convulsions, seizures, and problems moving the arms and legs.

Most people with severe hemophilia are diagnosed as infants. People with mild or moderate hemophilia might not be diagnosed until adulthood.

Source: National Heart, Lung, and Blood Institute


The main treatment for hemophilia is factor replacement therapy. This involves concentrates of the needed clotting factor being infused into a vein. This therapy can be conducted regularly (called preventive or prophylactic therapy), or only when needed to stop bleeding (called on-demand therapy).

Additional treatments might include:

  • Desmopressin, or DDAVP, is a hormone that causes the body to release stored clotting factor.

  • Antifibrinolytic medicines help keep blood clots from breaking down. They can be used for mild bleeding or before dental work.

  • Pain medicines or steroids might be used on a case-by-case basis for swollen joints. Physical therapy might be another option.

Treatments vary depending on each patient’s circumstances and type of hemophilia.

  • Mild hemophilia doesn’t usually require replacement therapy, although a DDAVP product called Stimate® might be given.

  • Moderate hemophilia might be treated with replacement therapy depending on physical activity of the patient. Certain activities or surgeries might call for treatment beforehand.

  • Severe hemophilia is treated with regular replacement therapy, often multiple times per week.

Many hemophilia patients choose to infuse themselves at home. Treatment at home works for as-needed and ongoing therapy. It has several advantages. Home infusion can mean quicker and more engaging treatment, less time at the doctor’s office, and lower cost.

Doctors’ offices are good resources to learn more about home treatment. Doctors can even implant devices in patients’ veins to make them easier to access for therapy. Infection is a risk with these devices.

Hemophilia treatment centers (HTCs) are a vital resource. There are more than 100 in the United States, focused on providing support, education, and treatment to hemophilia patients and their families. HTCs are staffed by doctors who specialize in blood disorders, nurse coordinators, dentists, physical therapists, social workers, and other specialists. For more information or to find an HTC, visit cdc.gov/ncbddd/hemophilia/HTC.html.

Clotting factor treatments can be expensive. They often require prior authorization, meaning the insurance company must approve their use after they are prescribed. It’s important for hemophilia patients to understand their insurance coverage. Diplomat consumer advocates can help patients check current insurance benefits and explore access and assistance programs.

Hemophilia patients must be careful with medications like aspirin, ibuprofen, naproxen, and other medicines that can cause bleeding. Doctors and pharmacists can help patients choose and use medications safely.


National Heart, Lung, and Blood Institute, Centers for Disease Control and Prevention

Managing Hemophilia

You can take these measures to manage your hemophilia:

  • Follow your doctor’s treatment plan.

  • See your doctor or other provider for checkups.

  • Make sure your healthcare providers know you have hemophilia. It’s also important to tell the medical staff at your workplace and any coaches or gym trainers.

  • See your dentist regularly and make sure they know you have hemophilia.

  • Keep a record of your treatments and take it with you to medical appointments.

  • Be alert to symptoms of bleeding, particularly in your joints. Call your doctor or go to the emergency room if you have heavy bleeding from a wound, bleeding you can’t stop, joint pain or swelling, or any signs of bleeding in the brain.

If Your Child Has Hemophilia

It can be difficult to hear your child has been diagnosed with hemophilia. Many resources exist to help and support you:

  • Talk with your doctors to learn basic information and emergency care procedures.

  • Talk with Diplomat staff for treatment options, education, and support. Consumer advocates at Diplomat can help with psychosocial issues, financial and transportation problems, and other concerns. Contact us at info@diplomat.is to connect with your advocate.

  • Look for the many available books and Internet materials about hemophilia, both for you and your child. Diplomat has many available resources for your family, including a set of books about hemophilia for young children.

  • Look into support groups and summer camps for children with hemophilia. Ask your Diplomat consumer advocate about these groups and camps.

  • Protect your young children with hemophilia from objects and activities that could cause bleeding. Use protective equipment when riding tricycles or bicycles, and safety belts or straps in car seats, highchairs, and strollers. Pad sharp corners in the home, and be alert for small, sharp objects.

  • Be alert for signs of bleeding and be prepared to treat bleeding if it occurs.

  • Make sure that all caregivers know your child has hemophilia. Your child should wear a medical ID bracelet or necklace. Consider a Diplomat StrapWrap® medical alert ID for backpacks or car seats.

  • Reassure your children. They might feel they caused their hemophilia. It’s important to explain their condition is not their fault and provide emotional support. Your Diplomat patient advocate is happy to help.

Physical Activity & Hemophilia

Although hemophilia patients can still be physically active, they might need to avoid certain sports or activities. Contact sports and other activities that could cause bleeding — such as football, hockey, and wrestling — should be avoided. Activities such as walking, swimming, biking, and golf are generally safe. Doctors, physical therapists, and hemophilia treatment center staff members can help hemophilia patients design safe exercise programs that work for them. Some doctors recommend treatment with clotting factor before exercising.

Adapted from the National Heart, Lung, and Blood Institute.


Up to 20 percent of patients with severe hemophilia A will develop antibodies that cause their bodies to reject the blood factors used in their treatment. Up to 5 percent of patients with hemophilia B will develop a similar response. These antibody responses are called “inhibitors.” They often appear during the first year of treatment and make hemophilia more difficult and costly to treat. Patients can develop multijoint arthropathy (joint damage from abnormal bleeding in one or more joints), which further increases the cost of treatment. This is why physical therapy is often a part of treatment for patients with inhibitors.

Diplomat’s REBUILD program offers physical therapy for people with hemophilia. It connects patients, their local therapists, and physical therapists who specialize in hemophilia and related bleeding disorders. To learn more, visit the REBUILD web page.

Source: Adapted from Centers for Disease Control and Prevention.

Costs of Hemophilia Inhibitors

Inhibitors drive up the cost of hemophilia treatment. According to a study in 2008, hemophilia patients with inhibitors paid almost five times as much for their treatment as patients without inhibitors. More than 75 percent of those costs were for clotting factor concentrate.

Diplomat consumer advocates can connect patients with help to reduce the costs of treatment. Contact us at info@diplomat.is to learn more.

Treatment of Hemophilia With Inhibitors

Hemophilia with inhibitors can be difficult to treat. A hemophilia treatment center (HTC) specializes in treating people with hemophilia and is recommended for inhibitor treatment.

Although many treatments exist for patients with inhibitors, the three standard types include:

  -Giving patients high doses of clotting factor concentrates to overcome low levels of inhibitors.

  -Using bypassing agents, which help form clots by “bypassing” inhibitors rather than attempting to replace missing factors. Because these agents can make blood clot too readily, monitoring is important.

  -Using immune tolerance induction (ITI) therapy, which treats patients by using high levels of clotting factor concentrates over a long period. The goal is to overwhelm the inhibitor reaction and train the body to accept treatment. This works in about 80 percent of cases, but treatment might continue for years even after the inhibitors go away.

Diplomat offers all clotting products and bypassing agents used in these inhibitor treatments. Diplomat can also play an important role in supporting patients who undergo a treatment regimen as intensive as ITI. Our IMPACT program provides treatment “coaches,” training aids, family and school educational programs, increased clinical interventions, and financial review or counseling. It also includes heightened reporting for and coordination with provider offices. Read more about the IMPACT program to see how we can help.


Centers for Disease Control and Prevention

Centers for Disease Control and Prevention and Georgetown University Medical Center. S. Guh et al., “Healthcare expenditures for males with haemophilia and employer-sponsored insurance in the United States, 2008,” Haemophilia, 2012

Other Inhibitor Treatments
FVIII/VWF Concentrates1

These concentrates, sometimes called “whole molecule” or “co-protein” products, contain both factor VIII and von Willebrand factor (VWF) proteins. VWF blocks inhibitors from attacking factor VIII.

FEIBA® NF Prophylaxis2,3,4

FEIBA NF is a bypassing agent to treat bleeding in people with inhibitors. Research shows that FEIBA NF can greatly reduce the frequency of bleeding for patients with severe hemophilia A. See more at feiba.com/hcp/prophylaxis.html.

NovoSeven® RT5

NovoSeven RT is a bypassing agent to treat bleeding in people with inhibitors. It contains factor VII and is recombinant (made without human blood or plasma). It can be readministered in as little as two hours after the initial dose. See more at novosevenrt.com.


1 F. Peyvandi et al., “A Randomized Trial of Factor VIII and Neutralizing Antibodies in Hemophilia A,” The New England Journal of Medicine, 2016;374:2054-64.

2 Valentino LA, “Assessing the benefits of FEIBA prophylaxis in haemophilia patients with inhibitors,” Haemophilia, 2010;16:263-271.

3 Perry D, Berntorp E, Tait C, et al., “FEIBA prophylaxis in haemophilia patients: a clinical update and treatment recommendations,” Haemophilia, 2010;16:80-89.

4 Valentino LA, Carcao M, Mathew P, et al., “The application of bypassing agent prophylaxis in haemophilia A patients with inhibitors: a meeting report,” Haemophilia, 2009;15:959-965.

5 Novo Nordisk, NovoSeven RT website, novosevenrt.com

The information contained herein may not be construed as medical advice. It is for educational purposes only. Diplomat Pharmacy Inc. takes no responsibility for the accuracy or validity of the information contained herein, nor the claims or statements of any manufacturer. Reference to a specific product, process, or service does not constitute an endorsement or recommendation by Diplomat Pharmacy Inc. Any such reference is provided for educational purposes only.