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
- 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
- Bleeding of the mouth and gums; tooth loss
- Blood in the urine
- Digestive problems
|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.
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
- Vaccinate all patients, including infants, with the hepatitis B
- Self-administer factor VIII or IX to help delay arthritis and prevent
- 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
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.
A healthcare provider may prescribe the following
- 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
|Surgical and Other Procedures|
Certain types of surgery may become necessary, including the
- 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
|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
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.
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.
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 (Vaccinium myrtillus)
- 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
(Ginkgo biloba), as it reduces platelet clumping.
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
- Crotalus horridus — used when
there is 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
- Lachesis — for heavy bleeding that is
dark in color, especially in red-headed individuals that are jealous and
- 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
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
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 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:
- Movement exercises
- Resistance training (such as weight lifting and other exercises that
put tension on muscles)
A complete program may also include, when needed:
- 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
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.
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.
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|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,
Copyright © 2004 A.D.A.M., Inc
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