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Table of Contents > Conditions > Hemophilia
Hemophilia
Signs and Symptoms
What Causes It?
Who's Most At Risk?
What to Expect at Your Provider's Office
Treatment Options
Prevention
Treatment Plan
Drug Therapies
Surgical and Other Procedures
Complementary and Alternative Therapies
Prognosis/Possible Complications
Following Up
Supporting Research

Hemophilia is the most common inherited bleeding disorder. It involves a deficiency of one of two blood-clotting substances, known as factor VIII and factor IX, and leads to the inability of the blood to clot. There are two types of hemophiliac—type A and type B—but the symptoms are the same in both types. There are about 20,000 people in the United States who have hemophilia.


Signs and Symptoms

Hemophilia may be accompanied by the following signs and symptoms:

  • Joint pain and swelling, frequently in the knees and elbows
  • Heavy bleeding, or bleeding that lasts a long time, from an accident or other injury; the start of bleeding may be delayed
  • Large bruises
  • Swelling under the skin and between muscles, with fever, skin discoloration, and pain
  • Internal bleeding in the abdominal region, airway, or central nervous system
  • Bleeding of the mouth and gums; tooth loss
  • Blood in the urine
  • Digestive problems
  • Nosebleeding

What Causes It?

Hemophilia is an inherited disorder that almost exclusively affects men. Women are rarely affected but are the carriers who can pass the condition to their male children.


Who's Most At Risk?

Hemophilia affects people from all ethnic groups. The daughters of men with hemophilia are carriers. However, even if hemophilia runs in the family, only about half the sons of carriers will have hemophilia.


What to Expect at Your Provider's Office

Your healthcare provider will take a family history and do a physical examination, checking for deep bleeding, muscle spasms, limited joint motion, joints that are warm and enlarged, and bruises. Blood tests including tests of the blood's clotting abilities, computed tomography (CT) scans, and magnetic resonance imaging (MRI) for bleeding in the joints may be used.


Treatment Options
Prevention

Hemophilia cannot be prevented, but it is possible to test to determine whether an unborn baby will have the disorder. If a person has hemophilia, the following cautionary measures should be taken:

  • Avoid taking aspirin and nonsteroidal anti-inflammatory drugs (NSAIDs).
  • Vaccinate all patients, including infants, with the hepatitis B vaccine.
  • Self-administer factor VIII or IX to help delay arthritis and prevent joint disease.
  • Avoid circumcising male infants of women known to be carriers of hemophilia until the disorder in the infant has been ruled out.
  • Carry information at all times identifying the person as someone with hemophilia.

Treatment Plan

The primary treatment for hemophilia is factor replacement therapy, which involves replacing the blood's deficient clotting factor. This factor is produced from normal human or animal blood products or is genetically engineered. A healthcare provider may also prescribe pain relievers. Physical therapy may improve joint healing and function. Surgery may be performed for complications arising from hemophilia.


Drug Therapies

A healthcare provider may prescribe the following medications:

  • Factor VIII or IX replacement therapy
  • Pain relievers other than aspirin or NSAIDs, as they decrease the ability of the blood to clot
  • Topical medications to control bleeding
  • The drug desmopressin (DDAVP) may be used in people with mild hemophilia as an alternative to clotting factors.

Surgical and Other Procedures

Certain types of surgery may become necessary, including the following:

  • Synovectomy: removal or shrinking of the sac around a joint
  • Osteotomy: surgical cutting of a bone
  • Arthroplasty: surgical remodeling of a diseased joint
  • Removal of an uncontrollable, expanding hematoma (partially clotted blood under the skin that resembles a bruise)
  • Amputation of infected or useless extremities

Complementary and Alternative Therapies

Conventional exams, tests, treatment, and follow-up for hemophilia are very important. Never delay conventional care when you have bleeding or have joint pain or swelling. However, there are a few CAM therapies that may be helpful in conjunction with conventional care for certain symptoms and after effects of hemophilia. The degree of spontaneous bleeding has been linked to emotional and psychological stress. Some mind/body therapies may help relieve stress and anxiety. Studies even suggest that hypnosis may reduce the need for blood transfusions.

Self Hypnosis

Several preliminary studies suggest that those who have learned self-hypnosis need far fewer blood products over an extended period of time (namely two to three years) than those who have not learned the technique; one study included relaxation training along with hypnosis. Published and unpublished reports on individual patients also suggest that hypnosis may help blood clot better and may reduce the need for blood transfusions.


Nutrition

No studies have examined the link between nutrition and hemophilia. However, it would be wise to avoid vitamin E and fish oil if you have hemophilia, as they seem to increase bleeding time by keeping platelets from clumping. Vitamin K plays a role in normal clotting and may be useful either from dietary sources or in supplement form, but research is needed in this area.


Herbs

No studies have examined the value of herbs for hemophilia specifically. However, based on their own experience, herbalists may recommend herbs that strengthen blood vessels and act as astringents (causing contraction) to make bleeding less severe, such as the following:

  • Bilberry fruit (Vacciniummyrtillus)
  • Grape seed extract (Vitis vinifera)
  • Hawthorn berry (Crategus monogyna)
  • Horse chestnut (Aesculus hippocastanum)
  • Witch hazel (Hamamelis virginiana)
  • Yarrow (Achillea millefolium)

In the case of hemophilia, however, herbal remedies should not be used without the guidance of an appropriately trained health professional. In addition, people with hemophilia should avoid ginkgo (Ginkgobiloba), as it reduces platelet clumping.


Homeopathy

There have been few studies examining the effectiveness of specific homeopathic remedies. However, a review of several case reports found that the following remedies were helpful for individuals with hemophilia and even reduced their need for blood-clotting substances like factor VIII. Before prescribing a remedy, homeopaths take into account a person's constitutional type. In homeopathic terms, a person's constitution is his or her physical, emotional, and intellectual makeup. An experienced homeopath assesses all of these factors as well as any current symptoms when determining the most appropriate remedy for a particular person.

  • Arnica — for internal or external bleeding immediately following an injury; helpful for shock or trauma
  • Carbo vegetabilis — for people with pale skin and weakness who are extremely frail, even listless, but like cold and fresh air
  • Crotalus horridus — used when thereis bleeding into the muscles and when blood appears thin and dark; this remedy is most appropriate for individuals who are tall, thin, and pale, have diarrhea and an aversion to warm food and drink; may have fears of being alone and death
  • Hamamelis — for bleeding from a cut or wound, especially useful in nosebleeds, hemorrhoids, and broken blood vessels in the eye
  • Lachesis — for heavy bleeding that is dark in color, especially in red-headed individuals that are jealous and depressed
  • Millefolium — for internal or external wounds with significant bleeding and poor clotting
  • Phosphorus — for frequent, heavy bleeding; this remedy is most appropriate for individuals who experience cold sweats and have a desire for alcoholic beverages; the individual may also feel as though clothing aggravates the throat
  • Secale — for bleeding that is worsened by heat and lessened by cold

Acupuncture

According to a report of two cases in Australia, acupuncture may help relieve joint problems caused by hemophilia. It may also relieve pain when other treatments have failed and may be a useful substitute for medicines that can cause bleeding.

However, guidelines and precautions should be observed in using acupuncture for hemophilia. Bleeding into certain areas of the body is a danger in acupuncture and can be fatal for someone with hemophilia; acupuncture should be used only as a last resort. If you choose to try acupuncture, work with a trained, licensed acupuncturist who has experience dealing with hemophilia.


Physical Medicine

Physical therapy may play an important role in reducing joint problems caused by repeated bleeding in those areas. The following modalities may be recommended by the physical therapist:

  • Stretching
  • Movement exercises
  • Resistance training (such as weight lifting and other exercises that put tension on muscles)

A complete program may also include, when needed:

  • Splints
  • Ice
  • Heat
  • Ultrasound
  • Nerve stimulation
  • Hydrotherapy (water therapy)

Work with your healthcare provider to develop a program that is best for you. The routine should be used for at least 6 to 9 months to treat chronic joint inflammation and to prevent severe bleeding.


Prognosis/Possible Complications

Since 1983, improved replacement therapy techniques have reduced the risk of contracting infections such as AIDS or hepatitis from the blood supply, and this has lengthened lives. The life expectancy for people with hemophilia who do not have AIDS is 68 years. In people who do not receive factor replacement therapy, complications include the destruction of bones and joints, life-threatening cysts, bleeding in the brain, airway obstruction, gangrene, bleeding into muscles causing damage to nerves, chronic bruising, and anemia. Sudden bleeding can occur with emotional stress.


Following Up

Your healthcare provider will want to see you every 6 to 12 months. Your provider may also suggest centers where you can receive a range of treatments and learn more about how to manage your condition.


Supporting Research

Behrman RE, ed. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, Pa: W.B. Saunders Co; 1996.

Blumenthal M, Goldberg A, Brinckmann J, et al., eds. Herbal Medicine: Expanded Commission E Monographs. Newton, Mass: Integrative Medicine Communications; 2000.

Buzzard BM. Physiotherapy for prevention and treatment of chronic hemophilic synovitis. Clin Orthop. 1997;343:42-46.

Hoots WK. Hemophilia and related conditions. In: Rakel RE, ed. Conn's Current Therapy. 51st ed. Philadelphia, Pa: W.B. Saunders; 1999.

Hunton M. Homoeopathy in the treatment of haemophilia. Br Homeopath J. 1991;80:82-100.

Jonas WB, Jacobs J. Healing with Homeopathy: The Doctors' Guide. New York, NY: Warner Books; 1996: 256.

Koh TC. Acupuncture therapy in hemophilia. Am J Acupunct. 1981;9(3):269-270.

LaBaw W. The use of hypnosis with hemophilia. Psychiatr Med. 1992;10(4):89-98.

Lee GR, ed. Wintrobe's Clinical Hematology. 10th ed. Baltimore, Md: Williams & Wilkins, Inc.; 1999.

Lusher JM, Arkin S, Abildgaard CF, Schwartz RS. Recombinant factor VIII for the treatment of previously untreated patients with hemophilia A: safety, efficacy, and development of inhibitors. N Engl J Med. 1993;328(7):453-459.

Swirsky-Sacchetti T, Margolis CG. The effects of a comprehensive self-hypnosis training program on the use of Factor VIII in severe hemophilia. Int JClinExpHypn. 1986;34(2):71-83.

Ullman D. Homeopathic Medicine for Children and Infants. New York, NY: Penguin Putnam; 1992: 54.

Ullman D. The Consumer's Guide to Homeopathy. New York, NY: Penguin Putnam; 1995: 224, 325-326, 332, 336.


Review Date: December 2000
Reviewed By: Participants in the review process include: Jacqueline A. Hart, MD, Department of Internal Medicine, Newton-Wellesley Hospital, Harvard University and Senior Medical Editor Integrative Medicine, Boston, MA; Leonard Wisneski, MD, FACP, George Washington University, Rockville, MD.

Copyright © 2004 A.D.A.M., Inc

The publisher does not accept any responsibility for the accuracy of the information or the consequences arising from the application, use, or misuse of any of the information contained herein, including any injury and/or damage to any person or property as a matter of product liability, negligence, or otherwise. No warranty, expressed or implied, is made in regard to the contents of this material. No claims or endorsements are made for any drugs or compounds currently marketed or in investigative use. This material is not intended as a guide to self-medication. The reader is advised to discuss the information provided here with a doctor, pharmacist, nurse, or other authorized healthcare practitioner and to check product information (including package inserts) regarding dosage, precautions, warnings, interactions, and contraindications before administering any drug, herb, or supplement discussed herein.

 
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Acupuncture
Herbal Medicine
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