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Haemophilia is a rare inherited disorder in which the blood does not clot normally. It usually occurs in males, but can also occur in females. There is type A and B. Ascites refers to abdominal accumulation of fluid in the abdominal cavity. The most common cause of it is cirrhosis of the liver.
Symptoms:
Hemophilia – Prolonged bleeding, severe pain in the joints when affected, joint pains. When having intracranial bleeding, you could get severe headaches, stiff neck, mental condition, paralysis, loss of coordination.
Ascites – Feeling pressure and heaviness in the stomach, shortness of breath, protruding belly especially when lying on the back and a wavelike feeling when touched on the belly due to the fluid retention. Other signs include bruising, leg swelling, chronic fatigue, weight loss, increase in size of the male breast and vomiting blood.
Diagnosis:
Hemophilia - Generic test, chorionic villus sampling (involves a small sample of the placenta getting tested) and amniotic fluid testing (amniocentesis)
Ascites - Complete blood count exam, liver enzymes and coagulation diagnostic paracentesis. Other tests include microbiological culture, GemTone stain, cytopathology, CT scan and ultrasound.
Management:
Hemophilia A – Desmopressin, Octocog every two days, Nonacog to control clotting factor 9. Also avoid sports and any risk of cuts.
Hemophilia B – Nonacog AFA is used to manage clotting factor 9
Ascites – A salt restricted diet, diuretic therapy with close monitoring of serum potassium levels and renal function while on medication, ultra-filtration, aquapheresis and in acute cases, liver transplants.
Complications:
Hemophilia- Anaemia, joint deformity when you bleed around the joint, joint damage which leads to restricted movement. In case of intracranial haemorrhage; paralysis, loss of coordination, neck stiffness, and death could occur.
Ascites – Thrombosis, spontaneous bacterial peritonitis, and hepatorenal syndrome.
New Developments:
Hemophilia - New studies show the possibility of getting long acting drugs .Gene therapy is done in some developed countries.
Dr. Maurice Lusi of Rangel Park Memorial Hospital, Tassia Embakasi
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