Hemophilia

By: Ava Foudeh

What is it?

There are two types of Hemophilia, A and B. Both are caused by deficiencies in the amount of clotting factor in the blood (VIII or IX). When the blood does not have enough of one of these or is missing one clotting factor, the bleeding may end very slowly or may not stop at all. The two types of Hemophilia are linked together by their similar clinical pictures and their similar inheritance patterns. The most dangerous part about having Hemophilia is internal bleeding. If internal bleeding is left untreated it can lead to deformity, disability or even death. In a Hemophiliac the bleeding continues until either it clots long enough for it to heal or the person will bleed to death.

History of Hemophilia

The earliest references to hemophilia can be found in second century Jewish writings. The first attempts to treat hemophilia was by replacing the clotting factory with blood plasma taken from pigs and cows. In the 1970s scientists found two approaches to the disease One that was called prophylaxis required injecting doses of the clotting factor on a regular basis The second was to inject the factor whenever the bleeding occurred

How do you get it?

Hemophilia is a genetic disease and is passed on by the X chromosome (the chromosome that carries the clotting factor). If a boy gets the X chromosome that carries the hemophilia gene he will become a hemophiliac. If a girl get the gene, she will become the carrier of the gene, not showing symptoms of the disease though she may have a long or heavy menstrual cycle. The carrier has a 50% chance of passing the gene on to her children every time she gets pregnant.

How do you get it ctd.

How do you know if you have it?

When a baby starts to crawl the parents may notice bruises on stomach, chest, buttock, and back. The baby may also be fussy, not wanting to walk or crawl Other symptoms include long nosebleeds, excessive bleeding from biting down on the lips or tongue, excessive bleeding following a tooth extraction, excessive bleeding following surgery and blood in the urine.

Testing for Hemophilia

There are two ways to test for Hemophilia before birth though both may cause a sight risk of a miscarriage One is to take a fragment of tissue from the placenta and look at the genetic structure. The DNA can be examined to see if the child has Hemophilia.

Another way is to insert a thin needle though the mothers abdominal wall. The needle has optical fibers and a system which enables the doctor to see the womb and fetus. With the image, the doctor can insert a needle into the umbilical cord and draw out a small amount of fetal blood. After the child is born, several blood tests may be carried out to determine if the child has it.

Daily cost of Having Hemophilia

Because Hemophilia is a life long disease if you have it you need life long treatment. It will cost any where from $75,000 to $125,000 a year to treat severe hemophilia. That is approximately 31 times an average nonhemophiliac pays for health care over the course of a year.

Average life span of people with Hemophilia

Year Average age at the time of death Treatments available at time

Before 1938
Before 1968

11
20

none
Plasma or Whole blood transfusions

1968
1983

Less than 40
64

Cryoprecipitate
Freeze dried clotting factors Same Factors produced by genetic engineering

1988 1999

40 ( impact of aids) Normal life span

Average life span ctd.

80 70 60 50 40 30 20 10 0 Avrage age Before 1938 before 1968 1968 1938 1988 1999

This is a diagram of the joints most commonly affected by Hemophilia. It most often occurs at the knees, hips, ankles, shoulders, and elbows

The most common muscles that bleed with Hemophilia are those in the the upper arm, upper leg (front and back), the calf and the front of the groin

Daily life with Hemophilia

Children can not play with toys with sharp or hard edges. Sports or other out-side activities may lead to injuries. The patients will most likely want to clean their teeth properly so diseases that can lead to bleeding in the gums will not occur.

Facts About Hemophilia

About 17,000 people in the USA have the disease. 400,000 people through out the world have it in 40 countries. Each case of Hemophilia is different. It Is called the royal disease because it was passed down from Queen Victoria and descended down the royal blood line.

Hemophilia may lead to arthritis because bleeding into the joints can inflame the joint lining and destroy cartilage. The Hemophiliac will not bleed any faster than a normal person, the bleeding just will not stop as quickly. Ordinary bruises are rarely a serious problem but cuts and abrasions may bleed for days (with out treatment).

Bibliography

Willet, Edward 1959. Enslow Publishers, Inc. Diseases and People: Hemophilia. Pgs 128. Hemophilia: Information provided by NIH & NHLBI http://www.medceu.com/tests/hemophilia.htm Hemophilia. Funded by Josiah Macy Jr. foundation http://www.ygyh.org/hemo/whatisit.htm "Hemophilia," Microsoft Encarta Online Encyclopedia 2004 http://encarta.msn.com/encnet/refpages/RefArticle.aspx?refid=76 1570021 Hemophilia A and B. Last updated June 23, 2004 Written by Hadi Sawaf http://www.emedicine.com/ped/topic962.htm Yahoo image search: http://burns.ee.cua.edu/~georgvis/classes/pennyb/xlink2.gif Yahoo image search http://www.drjerryherndon.com/images/teeth.jpg