Transcription

Treatment of Haemophilia

Though there is no cure for haemophilia, it can be controlled with regular infusions of the deficient clotting factor, i.e. factor VIII in haemophilia A or factor IX in haemophilia B. Some haemophiliacs develop antibodies (inhibitors) against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII.

If a patient becomes refractory to replacement coagulation factor as a result of circulating inhibitors, this may be overcome with recombinant human factor VII (NovoSeven®), which is registered for this indication in many countries.

In western countries, common standards of care fall into one of two categories: prophylaxis or on-demand. Prophylaxis involves the infusion of clotting factor on a regular schedule in order to keep clotting levels sufficiently high to prevent spontaneous bleeding episodes. On-demand treatment involves treating bleeding episodes once they arise. In 2007, a clinical trial was published in the New England Journal of Medicine comparing on-demand treatment of boys (< 30 months) with Haemophilia A with prophylactic treatment (infusions of 25 IU/kg body weight of Factor VIII every other day) in respect to its effect on the prevention of joint-diseases. When the boys reached 6 years of age, 93% of those in the prophylaxis group and 55% of those in the episodic-therapy group had a normal index joint-structure on MRI. [6] Prophylactic treatment, however, resulted in average costs of $300,000 per year. The author of an editorial published in the same issue of the New England Journal of Medicine demands more clinical studies addressing the cost-effectiveness of prophylactic treatment. [7]

Armour and other pharmaceutical companies knowingly sold blood clotting products contaminated with HIV and hepatitis C to ten thousand haemophiliacs across America in the late 1970's and early mid 1980's. When the Federal Government banned their products from being sold in America, they promptly turned around and sold the HIV contaminated factor products overseas in Europe and Japan. [8]

Later criminal and civil lawsuits were brought against these manufacturers [8] and the Armour Pharmaceutical subsidiary of French chemical company Rhone-Poulenc. In Europe and Japan several high level executives received prison terms as a result of their knowingly selling HIV contaminated products to innocent haemophiliacs.

In America the pharmaceutical companies eventually paid out around 650 million dollars to compensate the haemophiliacs who were infected with HIV.

As a direct result of the contamination of the blood supply in the late 1970s and early/mid 1980s with viruses such as Hepatitis and HIV, new methods were developed in the production of clotting factor products. The initial response was to heat-treat (pasteurize) plasma-derived factor concentrate, followed by the development of monoclonal factor concentrates, which use a combination of heat treatment and affinity chromatography to inactivate any viral agents in the pooled plasma from which the factor concentrate is derived. The Lindsay Tribunal in Ireland investigated, among other things, the slow adoption of the new methods.

Since 1993 (Dr. Mary Nugent), recombinant factor products (which are typically cultured in Chinese hamster ovary (CHO) tissue culture cells and involve little, if any human plasma products) have become available and are widely used in wealthier western countries. While recombinant clotting factor products offer higher purity and safety, they are, like concentrate, extremely expensive, and not generally available in the developing world. In many cases, factor products of any sort are difficult to obtain in developing countries.

It was claimed that Rasputin was successful at treating the Tsarevich Alexei of Russia's haemophilia: however, to this day it is unclear how he accomplished this.

People affected with Hemophilia are recommended to do some specific exercises for elbow, knee, and ankles like stretching of calves, ankle circles, elbow flexion, and Quadriceps set etc. These exercises are recommended after an internal bleed occurs and on a daily basis to strengthen the muscles and joints to prevent new bleeding problems.

Causes of haemophilia

It is caused by a lack of clotting factors:

* Haemophilia A involves a lack of the clotting Factor VIII. (This represents 90% of haemophilia cases.[citation needed])
* Haemophilia B involves a lack of the clotting Factor IX.
* Haemophilia C involves a lack of the clotting Factor XI.
* Hypofibrinogenemia involves a lack of the clotting factor Factor I. Because it is so rare, about 1 or 2 cases per million births, it has no definite treatment approved by the FDA. It affects males and females equally. The blood of people with Hypofibrinogenemia neither clots nor contains sufficient amounts of Fibrinogen.

Haemophilia or hemophilia (from Greek haima "blood" and philia "to love"[1]) is the name of a family of hereditary genetic disorders that impair the body's ability to control blood clotting, or coagulation. In the most common form, haemophilia A, clotting factor VIII is absent. Hemophilia B, also known as factor IX deficiency, is the second most common type of hemophilia, but Hemophilia B is far less common than Hemophilia A. Occurring in about one in 25,000 male births.[2]

The effects of this sex-linked, X chromosome disorder are manifested almost entirely in males, although the gene for the disorder is inherited from the mother. In about 30% of cases of Hemopilia B, however, there is no family history of the disorder and the condition is the result of a spontaneous gene mutation. [3] A mother who is a carrier also has a 50% chance of giving the faulty X chromosome to her daughter. That does not give the daughter the hemophilia disease, but it does result in the daughter becoming a hemophilia carrier. Females are almost exclusively asymptomatic carriers of the disorder, and may have inherited it from either their mother or father.

These genetic deficiencies may lower blood plasma clotting factor levels of coagulation factors needed for a normal clotting process. When a blood vessel is injured, a temporary scab does form, but the missing coagulation factors prevent fibrin formation which is necessary to maintain the blood clot. Thus a haemophiliac does not bleed more intensely than a normal person, but for a much longer amount of time. In severe haemophiliacs even a minor injury could result in blood loss lasting days, weeks, or not ever healing completely. The critical risk here is with normally small bleeds which due to missing factor VIII take long times to heal. In areas such as the brain or inside joints this can be fatal or life debilitating.

The bleeding with external injury is normal, but incidence of late re-bleeding and internal bleeding is increased, especially into muscles, joints, or bleeding into closed spaces. Major complications include hemarthrosis, hemorrhage, gastrointestinal bleeding, and menorrhagia.

Malaria

Malaria is a serious disease caused by a parasite. Infected mosquitoes spread it. Malaria is a major cause of death worldwide, but it is almost wiped out in the United States. The disease is mostly a problem in developing countries with warm climates. If you travel to these countries, you are at risk. There are four different types of malaria caused by four related parasites. The most deadly type occurs in Africa south of the Sahara Desert.

Malaria symptoms include chills, flu-like symptoms, fever, vomiting, diarrhea and jaundice. The disease can be life-threatening. However, you can treat malaria with medicines. The type of medicine depends on which kind of malaria you have and where you were infected.

Malaria can be prevented. When traveling to malaria-prone regions

• See your doctor for medicines that protect you
• Wear insect repellent with DEET
• Cover up
• Sleep under mosquito netting

AIDS

Also called: HIV

AIDS stands for acquired immunodeficiency syndrome. It is the most advanced stages of infection with the human immunodeficiency virus (HIV). HIV is a virus that kills or damages cells of the body's immune system.

HIV most often spreads through unprotected sex with an infected person. AIDS may also spread by sharing drug needles or through contact with the blood of an infected person. Women can give it to their babies during pregnancy or childbirth.

The first signs of HIV infection may be swollen glands and flu-like symptoms. These may come and go a month or two after infection. Severe symptoms may not appear until months or years later.

A blood test can tell if you have HIV infection. Your health care provider can perform the test, or call the National AIDS hotline for a referral at (800) 342-AIDS (1-800-342-2437). There is no cure, but there are many medicines to fight both HIV infection and the infections and cancers that come with it. People can live with the disease for many years.

Lung Cancer

Also called: Bronchogenic carcinoma

Lung cancer is one of the most common cancers in the world. It is a leading cause of cancer death in men and women in the United States. Cigarette smoking causes most lung cancers. The more cigarettes you smoke per day and the earlier you started smoking, the greater your risk of lung cancer. High levels of pollution, radiation and asbestos exposure may also increase risk.

Common symptoms of lung cancer include

• A cough that doesn't go away and gets worse over time
• Constant chest pain
• Coughing up blood
• Shortness of breath, wheezing, or hoarseness
• Repeated problems with pneumonia or bronchitis
• Swelling of the neck and face
• Loss of appetite or weight loss
• Fatigue

There are many types of lung cancer. Each type of lung cancer grows and spreads in different ways and is treated differently. Treatment also depends on the stage, or how advanced it is. Treatment may include chemotherapy, radiation and surgery.

National Cancer Institute