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Patient Education

Understanding Hemophilia

Hemophilia is a rare bleeding disorder characterized by a deficiency of specific clotting factors in the blood. Clotting factors are proteins required for blood clots to form after injury to a blood vessel. Hemophilia types A and B are inherited in an X-linked recessive pattern, so that they affect primarily males, who have only one X chromosome.¹

Treatment and Other Recommendations

Routine dental evaluations should be scheduled for Individuals with hemophilia beginning in infancy. Patients should be educated about proper oral hygiene, fluoridation, and overall dental care.²

Healthcare providers must teach patients and family members how to recognize the signs and symptoms of hemorrhage early so that proper replacement treatment to stop the bleeding can be obtained quickly. Replacement therapy helps to prevent life-threatening episodes of bleeding, the development of long-term complications, and a reduced quality of life.²

Healthcare providers also can instruct patients and their caregivers on how to perform factor replacement therapy at home. For individuals with severe hemophilia, this eliminates the need for transportation to the nearest emergency department during bleeds, and factor replacement can be initiated immediately. Parents can learn how to administer infusions to their children with hemophilia when they are as young as 2 years old. When the children reach 8 to 10 years of age, they can learn to infuse themselves.²

The National Hemophilia Foundation and the Centers for Disease Control and Prevention have developed the National Prevention Program (NPP), a public awareness campaign focused on people with bleeding disorders, their families, and their healthcare providers. The NPP suggests 5 major strategies for individuals with hemophilia to help them live longer and healthier lives³:

Undergoing an annual comprehensive examination at a hemophilia treatment center;
Being vaccinated for hepatitis A and B;
Catching and treating bleeds early and well;
Exercising and maintaining a healthy body weight to keep muscles strong and avoid becoming overweight, so as to protect the joints;
Undergoing regular blood testing for blood-borne infections.

Preventative measures around the home include eliminating furniture with sharp corners, checking play equipment and outdoor play areas for possible hazards, and locking away sharp objects and other things that can cause bleeding.⁴

Medications and Diet

Patients with hemophilia should avoid medications that affect blood clotting, including nonsteroidal anti-inflammatory drugs (NSAIDs) like aspirin, ibuprofen, and naproxen, as well as prescription blood thinners such as heparin, warfarin (Coumadin®), and others.⁴

Patients should be wary of taking herbal supplements, which may also affect clotting ability, and should consult with their healthcare provider or a pharmacist before use.⁴

Drinking plenty of water and fluids to ensure proper hydration is essential. It is recommended that individuals with hemophilia consume at least 8 glasses of water or clear, caffeine-free fluids daily to prevent dehydration.⁴

Although people with hemophilia do not have to adhere to specific dietary restrictions, it is nonetheless important to maintain a healthy weight by eating a nutritious, well-balanced diet that includes plenty of fruits, vegetables, and whole grains. It is recommended that the intake of alcohol, sugar, excessive salt, and saturated fat be avoided. Another recommendation for individuals with hemophilia is to eat smaller meals about every 3 to 4 hours throughout the day instead of 3 large meals daily.⁴

Exercise

Healthcare providers should encourage individuals with hemophilia to engage regularly in appropriate low-impact exercise. Participation in collision and contact sports, which may cause internal joint, organ, and muscle bleeding, should be discouraged.² Recommended exercise activities include swimming, cycling, and walking. Contact sports such as football, hockey, and wrestling should be avoided. Individuals with hemophilia should use helmets, safety belts, and knee and elbow pads to prevent head or joint injuries.⁴

Genetic Counseling

Ultimately, the purpose of genetic counseling is to interpret each patient’s family and medical history; educate patients on how hemophilia is inherited, diagnosed, managed, and prevented; offer information about resources; and help patients to make informed choices and manage their health as well as possible. Genetic counseling can inform carriers of the X-linked genetic mutation that causes hemophilia about how to undergo genetic and prenatal testing, and they can offer psychosocial support after the results of genetic testing have been obtained. Genetic counseling allows patients to maintain a better sense of control over their situation and mitigates the psychological and emotional distress that accompanies the diagnosis of a genetic disease.⁵

Patient Resources

Two organizations that offer support to patients with hemophilia and their caregivers are the National Hemophilia Foundation (https://www.hemophilia.org/) and the Hemophilia Federation of America (https://www.hemophiliafed.org/). These organizations are a source of educational materials, recent news, information on the latest research developments in the treatment of hemophilia, and connections to patient support groups. The National Hemophilia Foundation (NHF) has developed and expanded a resource and information center on bleeding disorders, known as HANDI, that provides access to a library of articles, textbooks, various educational publications, CDs, videos, and reprints to consumers, researchers, healthcare providers, and NHF chapters. An attempt to make this information available to everyone online is currently under way.³ The NHF also supports the “First Step” education program, which provides information on the diagnosis, management, and treatment of hemophilia, in addition to strategies for preventing secondary conditions, to individuals with newly diagnosed hemophilia.³

References

Hemophilia. MedlinePlus. Accessed August 3, 2021.
Drelich DA. What is included in patient education for hemophilia A? Medscape. Updated June 5, 2020. Accessed August 3, 2021.
Hemophilia health education and prevention funded projects. Centers for Disease Control and Prevention (CDC). Reviewed July 17, 2020. Accessed August 3, 2021.
Hemophilia condition guide. Prime Therapeutics: Specialty Pharmacy. Accessed August 3, 2021.
Alabek M, Mohan R, Raia MH. Genetic counselling for hemophilia. Treatment of Hemophilia. July 2015 No. 25. Accessed August 3, 2021.

Reviewed by Harshi Dhingra, MD, on 8/11/2021.

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.

Hemophilia