Hemophilia

AN INTRODUCTION TO HAEMOPHILIA

What Is Haemophilia?
Haemophilia is a disorder in which one or more of the plasma proteins
needed to form a clot is missing or reduced. The most common type of
haemophilia is factor VIII deficiency, or haemophilia A. The second most
common type is factor IX deficiency or haemophilia B. When a person with
haemophilia is injured, he (or, occasionally, she) does not bleed harder
or faster than normal, but will have prolonged bleeding because he
cannot make a firm clot. Small cuts on the skin are usually not a
problem, but bleeding in any deeper area can be prolonged. Some bleeding
episodes occur as a result of injury, but many occur seemingly without
cause.
About Clotting
There are two major processes involved in blood clotting.
The first part has to do with platelets. They are like little "sticky
shingles" which go to where a blood vessel has ruptured, and they stick
over the hole and make a plug. This is the first step of making a clot.
The plug is only temporary, and the platelets can easily fall off. The
platelets soon rupture and release chemicals that attract more platelets
and make them "sticky", too. The chemicals released by the rupturing
platelets also activate various clotting factors which are proteins in
the blood.
The next step is that fibres form from the activated proteins and mix
with the platelets. The fibres are like a net, or a weave of yarn, and
they make the clot stronger. The substance that makes the fibers is
called fibrinogen. There are twlele factors (one through twelve) which
work together to make the fibrinogen. People with haemophilia have a
problem with one or more of those factors. The most common of the twelve
factors to have a problem is factor VIII, which causes haemophilia A.
The second most common to have a problem is factor IX, and this causes
haemophilia B. Humans are not the only animals to have haemophilia,
other animals can have it, too. A lot of laboratory experiments about
haemophilia are carried out on other animals with haemophilia.
How Common Is Haemophilia?
Estimates indicate that approximately one in 10,000 males born in New
Zealand has haemophilia. NZ Maori have a higher incidence of haemophilia
compared to Pakeha.
How Does Someone Get Haemophilia?
Haemophilia is a sex-linked hereditary bleeding disorder transmitted on
a gene of the X chromosome. You can not catch haemophilia as you catch a
cold. It is something that some people are just born with. There is also
a very rare condition known as acquired haemophilia. This is a
spontaneous formation of an antibody against Factor 8. This autoimmune
system renders a person a haemophiliac with only slight differences from
congenital haemophilia. Among the genes and chromosomes we inherit from
our parents are two sex chromosomes, labeled X and Y. A woman inherits
two X chromosomes, one from her mother and one from her father. This
makes her female. A man inherits one X chromosome from his mother and a
Y chromosome from his father, instead of a second X. This makes him
male. If all of a person's X chromosomes have the haemophilia gene, then
that person will have haemophilia. The condition is much less common in
women than in men, since women have two X chromosomes, and it is quite
unlikely for both of them to have the haemophilia gene. In some cases
haemophilia is "hidden" for many generations if no affected male
children are born. The gene for haemophilia in such cases is carried
through several generations of females who, because they have a second X chromosome that is normal, do not suffer from the disease themselves.
Other cases may have no family history, meaning that the change in the X
chromosome is a new one. This would be a mutation of the gene. Most
mothers of children with haemophilia, however, have fathers,
grandfathers, brothers or other male relatives on the maternal side of
their families who were born with haemophilia. Very rarely, a female
with haemophilia is born if her mother is a carrier and her father has
haemophilia.

Medical Care in Russia
Hemophilia - disease not curable in the present time. In spite of the fact that the first messages on a hemophilia are dated time in 2000 back, specific treatment became possible only in 1940 when Mack Farlan with group of employees has realized, that transfusion of integral blood or plasma is a way of replacement of the missing factor responsible for coagulability of blood. Modern development of preparations coagulate factors from normal donor blood has provided means of treatment bleedings.
Treatment is necessary at inner joint and inner muscle bleedings and at all forms surgical, switching teeth, and also at hemorrhages in various bodies, after bruises and accidents.
In Russia, in industry, modern preparations of blood are not made. The probability of spreading through donor blood of such infections, as from here is great: AIDS and all kinds hepatitis.
The problem with purchase of preparations of the factor of blood it is solved only in two towns: is St. - Peterburg and Moscow. Other areas need to be content " with the Order of Ministry of Health about bringing in the factor VIII and IX in the list of vital and major medical products " , however money in regions are not transferred or Russian tradition settle in pockets of local officials, Department of public health Care.
The patient with a hemophilia, for a high-grade way of life, it is important to have a necessary stock of a preparation as we shall tell the patient with a diabetes that in an emergency situation or prophylactic purposes to stop a bleeding.
Formed practice when patient with a hemophilia is compelled to lose precious minutes, expecting arrival of the machine of first aid, and next hospitalization where the rendered help becomes not effective, is archaism and economically unreasonable.
In the advanced countries, "patients" with a hemophilia conduct an active way of life, go in for sports and even pass military service...
In our country, by 10 years the child is already the invalid, with the struck joints, in consequence of often hemorrhages and inadequate medical the help.