MYTH: All forms of hemophilia involve a deficiency in clotting factor VIII.
FACT: About 80% of hemophilia patients have hemophilia A, which is caused by a deficiency in clotting factor VIII. The other 20% have hemophilia B, caused by a deficiency in factor IX.
MYTH: Patients with hemophilia always have a family history of the disease.
FACT: One-third of patients have no family history of the disease and the cause is attributed to spontaneous gene mutation. However, hemophilia is a sex-linked genetic disorder. Women who carry the defective allele on the X chromosome have a 50% chance of passing the defective allele to a son (causing the disease) and a 50% chance of passing it to a daughter (making her a carrier if the father doesn't have hemophilia).
MYTH: Girls never have hemophilia.
FACT: Although hemophilia is rare in girls, it can occur. If a girl's mother is a carrier and her father has the disease, she has a 50% chance of developing hemophilia; even if she doesn't have the disease, she'll be a carrier. Also, 10% of women who are carriers have clotting factor levels low enough to cause abnormal bleeding.
MYTH: Patients can bleed to death from a cut.
FACT: Although blood clotting takes longer, someone with hemophilia won't bleed any faster than someone who's unaffected. Ordinary first-aid measures, such as pressure, elevation, and ice, will stop bleeding from most superficial cuts. A greater threat to these patients is internal bleeding, particularly into joints, which is difficult to manage. Besides being extremely painful, chronic bleeding into joints can cause disability.
MYTH: Patients suspected of having hemophilia will have abnormal bleeding times and platelet counts.
FACT: Typically, a patient with hemophilia has a prolonged activated partial thromboplastin time (aPTT), but other lab results are normal. Factor VIII and factor IX deficiencies are clinically similar, so any male with a history of bleeding and an abnormal aPTT result should be checked for deficiencies in these clotting factors.
BY BEVERLY BRITTON, RN, MS
Before retiring, Beverly Britton was a nursing professor at North Harris Montgomery Community College in Houston, Tex. Selected references for this article are available on request.
Copyright Springhouse Corporation Dec 2003
Provided by ProQuest Information and Learning Company. All rights Reserved
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