| Hemophilia is a condition that involves problems with coagulation or clotting of blood, leading to easy bruising and bleeding. Parents are often worried that their children having a clotting disorder when they notice that they bruise easily or have frequent nosebleeds. However, if the bruising is in areas where your child commonly falls or hits himself, such as the front of the lower legs, then it is probably normal.
Children with occasional nosebleeds and easy bruising after trauma usually do not have a bleeding disorder. Prolonged or severe nosebleeds, especially if associated with easy bruising that produces very large bruises in multiple locations after very mild trauma or with bleeding in more than one location, such as nosebleeds and bleeding from his gums after brushing his teeth, are more likely to be associated with a bleeding disorder.
Children with clotting disorders bruise easily after minor trauma and in areas that you don't commonly see bruises or they may bleed for hours or days after a minor cut or surgical procedure. If your child has had a surgical procedure, including a tonsillectomy or adenoidectomy, and didn't have any problems with excessive bleeding, then he is unlikely to have a bleeding disorder. Women with bleeding disorders often have prolonged menstrual bleeding (menorrhagia).
Your child should be evaluated by his Pediatrician if you suspect that he has a bleeding disorder. Things that should make you suspect a bleeding disorder, include:
- a family history of a bleeding disorder
- easy bruising, especially if your child bruises spontaneously or after very minor trauma and in areas that children do not usually get bruises or if he gets large lumps under the bruise
- slow healing of minor cuts and bruises
- having a previous surgical procedure (including dental procedures) that resulted in prolonged bleeding
Although hemophilia is not very common, and occurs in only about 1:5000 males, other bleeding disorders are fairly common, including von Willebrand Disease, which is thought to occur in 1-2% of the population.
Screening tests that will probably be done if your child has a suspected bleeding disorder, include a platelet count, a Partial Thromboplastin Time (PTT), a Prothrobin Time (PT) and a bleeding time. Clotting factor assays for hemophilia may also be done.
In addition to hemophilia and von Willebrand Disease discussed below, other bleeding disorders include:
- thrombocytopenia or having a low platelet count, which can occur in children with immune thrombocytopenic purpura (ITP)
- Vitamin K deficiency, especially in newborns who didn't receive a Vitamin K shot
- severe liver or kidney disease
- serious illnesses or infections, usually associated with shock, which can lead to DIC (disseminated intravascular coagulation)
Hemophilia A is the most common type. It is also called Classic Hemophilia. It is caused by a defective gene on the X chromosome, which leads to decreased production of factor VIII, which is required to form blood clots, and this leads to easily bruising and bleeding. Since the gene for this blood disorder is on the X chromosome, it is usually inherited from a mother who is a carrier (meaning she has one normal and one abnormal gene). Male infants of a mother who is a carrier have a 50% chance of having hemophilia A. Children can have a mild, moderate or severe cases of hemophilia A, depending on the activity level of factor VIII, and this can be measured.
Symptoms of hemophilia A sometimes begin in the newborn period, and can include excessive bleeding after a circumcision or the develop of very large bruises after routine immunizations. Often, though, bleeding and bruising do not begin until children are older and they begin walking (and falling). Diagnosis is by a blood test that measures the activity level of factor VIII. Treatment is by preventing trauma as much as possible and replacing factor VIII when bleeding is severe.
Hemophilia B or Christmas Disease is another, less common type of bleeding disorder. It is caused by a deficiency of factor IX, which is also required to form blood clots. Symptoms are similar to Classic hemophiliacs, and treatment is by replacement of Factor IX.
Von Willebrand Disease is another type of clotting disorder that can occur in males and females. Symptoms usually include frequent nosebleeds, gum bleeding, and prolonged bleeding from minor cuts or after surgery. Testing for von Willebrand disease includes a prolonged bleeding time, and abnormal levels of von Willebrand protein, von Willebrand factor activity and factor VIII activity. Treatment includes replacement of von Willebrand factor for severe bleeding. Minor bleeding is usually treated with DDAVP.
Resource List for Bleeding Disorders:
- Kelly Communications: free books, newsletters, mailing lists and links to other resources from a parent of a hemophiliac.
- National Hemophilia Foundation: dedication to the cures of inherited bleeding disorders and prevention and treatment of their complications.
- World Federation of Hemophilia: an invaluable resource for the online global bleeding disorder community. Filled with articles, stories, ask the expert section and more.
- alt.support.hemophilia: Usenet newsgroup for the discussion of bleeding disorders. Lots of spam here though.
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