Page 147 - 86 human physiology part-2

Chapter 18

adults who have recurrent pain episodes (at least 3 yearly), a cancer drug, hydroxyurea (marketed as
Droxia), has been approved to relieve symptoms. It appears to work by increasing the flexibility of
sickle cells.

Hemophilia

About two thirds of people who have Hemophilia have inherited it. For the other third, there is no
known cause for possessing the disorder. There are two types of hemophilia, Type A and Type B. Both
are caused by a low level or a complete absence of protein in the blood. Without this protein, blood is
not able to clot.

Some of the symptoms of Hemophilia are bleeding in the joints, knees, and ankles. Stiffness
without pain in the joints, stiffness with a lot of warmth,(most ability for movement is lost due to
swelling) blood in the urine or stool, excessive bleeding after surgery or loosing a tooth, excessive
bruising, abnormal menstrual bleeding, and nose bleeds that last for long periods of time.

Hemophiliacs blood does not coagulate like a normal persons. Coagulation controls bleeding, it
changes blood from a liquid to a solid. Within seconds of a cut or scrape, platelets, calcium and other
tissue factors start working together to form a clot. Over a short time the clot strengthens and then
dissolves as the injury heals. Hemophiliacs are missing the clotting factor, or it isn't working correctly
which causes them to bleed for a longer time. The most common myth is that a person with a bleeding
disorder will bleed to death from a minor wound or that their blood flows faster than somebody without
a bleeding disorder. Some of the risks hemophilia are: Scarring of the joints or joint disease, vision loss
from bleeding of the eyes, chronic anemia from blood loss, a neurological or psychiatric problem, death
which may occur from large amounts of blood loss or bleeding in the brain or other vital organs. Most
cases of hemophilia are caused from inherited disorders but sometimes people can get it from vitamin
K deficiency, liver disease, or treatments like prolonged use of antibiotics or anti coagulation drugs.
Hemophilia is the best known bleeding disorder and it has had the most research done on it, so
hemophiliacs have a slight advantage over people with other bleeding disorders.

Treatment for hemophilia

To treat Hemophilia, a Clotting Factor is needed. It is in the shape of powder kept in a small,
sterile glass bottle. It has to be kept in the fridge. When needed, The Clotting Factor is mixed with
sterile water, then one minute later it can be injected into a vein. It may also be mixed with a large
amount of water and injected through an IV.

There are over 140 centers that specialize in hemophilia. Most of these centers are
"Comprehensive Care Facilities". Comprehensive care facilities provide all the services needed by a
hemophiliac and their family. Services provided include: Primary physician, nurse coordinator,
physiotherapist, and dentist. Hemophiliacs require a special dentist because of the higher risk of
bleeding. It is recommended that hemophiliacs go to the treatment centers twice a year for a complete
check-up.

The basic and most common treatment for patients with hemophilia A and B is factor replacement
therapy. Factor replacement therapy is the IV injection of Factor VIII and IX concentrates which help
control bleeding. This concentrate comes from two sources: human plasma and genetically engineered

362 | Human Physiology