Christmas Disease; Cause, Clinical Features and Management

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Christmas Disease also called as Haemophilia B; Factor 9 deficiency. Christmas disease is one of the bleeding disorders. In Christmas disease the ‘factor’ that is deficient is ‘factor 9’ which is necessary for the formation of blood clot. Factor 8 is deficient in Hemophilia A. The incidence is 1 in every 50,000 live male births all over world. Only boys are affected, due to the mode of inheritance.

CAUSE

 Christmas disease is inherited as an X-linked characteristic. The condition is passed down the female line, who show no effects, to 50% of their sons. Antenatal diagnosis can be made by fetoscopy. Measurement of the ‘factor 9’ coagulation level at birth in a male baby who has a family history of a bleeding disorder will also give a diagnosis.

christmas-disease

CHARACTERISTICS 

The people with this disease might show mild, moderate and severe characteristics depending upon the actual amount of “factor 9” present in the blood. Severely affected people will have less than 1% of normal activity of factor 9, while a mild sufferer can have as much as 20% of normally-acting factor 9.   

Boys with the severest manifestation of the condition will suffer bleeding into the joints following the slightest injury causing very obvious swelling and pain.

Unless adequate treatment is given, these repeated, inevitable knocks to the joints will cause damage to, and eventual destruction of, the joint.

Bleeding can also occur into the soft tissues of the body and bruising is frequently seen following only mild trauma. This easy bruising must be recognized and taken into account soon. 

Minor surgical procedures, such as tonsillectomy or dental extractions for example, can lead to severe bleeding unless prophylactic treatment is given.

Infection with hepatitis B is a very real threat to people with any of the bleeding disorders. This illness, characterized by general malaise, fever, lack of appetite and jaundice, can lead to serious and potentially fatal liver problems. Any person with one of the bleeding disorders should be immunized against hepatitis B as soon as the diagnosis is made. 

MANAGEMENT:

The severity of the patient’s condition will obviously have a bearing on his activities. Contact sports and activities where there is a high potential for injury should be avoided. 

Schooling should be possible for such children.Teachers should be informed of their pupil’s problems, and be aware of the action to be taken if an injury should be sustained.  

Care should be taken when giving pain relieving drugs to haemophiliac boys. Aspirin, and similar drugs which affect platelets function, should be avoided. 

The specific concentrate containing factor 9 must be used to treat haemorrhages as soon as possible after they have occurred. Parents can be given a supply to use promptly after their son has sustained an injury. This substance should also be given prophylactically before any surgical procedures are undertaken. Some boys may develop a type of immunity (‘inhibitor’) to their treatment. If this should happen, referral to a specialist haemophilia centre should be made. 

CONCLUSION:

With prompt treatment of haemorrhage, the possibility of long-term disability has vastly improved and a useful, fulfilling life can be lived whilst suffering from Christmas disease. Obviously care will have to be continued throughout life and certain careers and leisure opportunities will not be open to these boys. 

The only other possible problems connected with the disease are the possibilities of chronic hepatitis or infection with HIV. But with immunization against hepatitis B and heat-treated blood products, these problems are reduced to a minimum.

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