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A, Hemophilia

About Disease:

Hemophilia is one of a group of inherited weeping complication which results in abnormal or farfetched oozing and meager blood clotting. People with this type of disease bleed longer than normal time period. This bleeding can usually range from mild to severe. In severe cases, people can bleed to death.

Hemophilia A and B are inherited in an X-linked passive hereditary pattern. Hence, males are generally afflicted whereas, females are usually shippers of this disease.

Clotting is the mechanism that thickens or congeals the blood. This averts a cut or other major injury from bleeding ceaselessly.

Hemophilia A is mainly caused by a lacking of clotting Factor VIII, while hemophilia B which is also called Christmas disease is caused due to deficiency of Factor IX.

Blood clotting comprises two sets of aspects. One set works with specific blood cells known as platelets. Platelets perform a vital role in process blood clotting. Another set of aspect includes coagulation structure. Coagulation is a coordinated array of chemical reactions which works by clotting and stopping the bleeding.

Types of Hemophilia:

  • Hemophilia A is one of the most common types of hemophilia diseases. People suffering with Hemophilia A do not retain abundant clotting factor VIII.
  • Hemophilia B can also be called as Christmas disease. The disease is caused due to deficiency in clotting factor IX. The disease can be classified into mild, moderate or severe.
  • Hemophilia C is also known as factor XI deficiency. It is rarely found in United States. Hemophilia C is resulted due to deficiency in clotting factor XI. Which is quite different than Hemophilia A or B.

 

Causes of Hemophilia:

The disease is caused due to a genetic metamorphosis. This mutation associates genes which usually code for proteins and are crucially important in process of blood clotting. The bleeding symptom emerges because blood clotting is flawed. Hemophilia A is induced by a metamorphosis present in the gene for factor VIII, which leads to further failure in clotting factor. The disease A is highly common than B. About 80% of the population with this disease has Type A. Type B occurs rare likely, in about 1 out of every 25,000 to 30,000 population. A subgroup of those with Type B inhibits Leyden phenotype, which is represents by a serious hemophilia in childhood which improves at puberty stage.


Signs & Symptoms of Hemophilia:

The disease can vary in its peak time, which is liable upon typical kind of mutations like genetic defect. The degree of symptoms of this disease depends on the level of afflicted clotting factor. People with this disease may bleed anywhere i.e. no particular place in the body. However, the bleeding seldom starts from minute cuts or puncture wounds. Rather, the affected people will bleed more than other people do from such minute injuries, trauma or huge surgery. How worst and often people bleed relates to how much clotting factor do they possess.

Serious disease can be defined as <1% factor activity, 1% to 5% factor activity is under moderate disease, and greater than 5% factor activity includes mild disease. The longer bleeding period dependent on the severity, the amount of factor activity and is equally similar for both Hemophilia A and B.

With a very serious Type (A or B) Disease, bleeding starts at very early age and may come into practice immediately. Symptoms are likely to evolve later in those patients inhibiting moderate or mild disease.

People with this disease may experience:

  • Bleeding into the joints - The joints are most common place for bleeding to materialize. Ankle, knee or elbow joints are most commonly affected joints for bleeding. The afflicted joint turns to be warm, painful and swollen. The person finds it too difficult to move joint as freely as before. This can happen post any injury or also without any cause.
  • Bleeding into muscle - may take place with hematoma formation which is compartment syndrome.
  • Bleeding into the esophagus, stomach or intestine can also occur.
  • Bleeding into the urine/urinary tract - can lead to blood in the urine which can be known  as hematuria.
  • Bleeding from the nose and gums - Bleeding after certain dental treatments is common, and oozing of blood from the gums may occur in young children at the time of growing teeth’s within for the very first time.
  • Heavy menstrual bleeding in women.
  • Intracranial hemorrhage likes, bleeding into the brain or skull can lead to symptoms such as nausea, vomiting, and/or lethargy.
  • Bleeding from the gastrointestinal tract can lead to blood in stool.

How is Hemophilia Diagnosed?

  • Severe reactions of the disease – people generally evolve bleeding issues within the first two years of life. Many infants are diagnosed when they practice long lasting bleeding circumcision. Others may come across in their childhood. They may increase bruising and bleeding into joints post some common childhood injuries.
  • Mild reactions – people suffering from the disease may not be diagnosed for decades. Diagnosis may only take place post experiencing abnormal bleeding after some kind of trauma, injury or surgery.
  • The diagnosis of Type A or B or factor XI deficiency can be looked into by performing examinations for specific clotting factors.

Expected Duration of Hemophilia:

This kind of disease is a lifelong illness. Symptoms usually can be controlled with appropriate medical treatment.

Treating Hemophilia:

Preventive Treatment – Clotting issues fixates help to regulate the missing clotting factor in the blood. This can help to cutback often and severity of you bleeding practice. Clotting factor settles given by injection into a vein. Recombinant hemophilia issues are developed in the test tube. They are not plucked from human blood products. Gently, it diminishes the risk of contracting transfusion related virus diseases, such as HIV and hepatitis viruses.

Treatment for excessive bleeding – at times enormous bleeding results despite the use ofrecombinant clotting factors. The situation may cause as an emergency case when it associates with the brain, neck, the central nervous system or a vital organ. The disease may lack blood transfusions to treat excessive blood loss.

Prognosis:

Many people with this disease can look ahead to a near-normal life span. Three main complications which may establish in people are:

  • Joint carnage – several chapters of bleeding into joints can lead in serious joint damage.
  • Blood-borne infection – Blood testing and purification techniques have enhanced considerably from years. It is now highly rare for hepatitis or HIV infections to be shipped with any blood products.

Enhancement of inhibitor antibodies – People who are looked upon with clotting factors can evolve inhibitor antibodies. These are the proteins which reduces effectiveness of clotting treatment. During this, treatment of the disease can be further more complicated and expensive.