Q: My brother has multiple joints pain since childhood. There is no swelling or morning stiffness. Occasionally, he gets low-grade fever and has been given Penidure injection on and off. There is no history of TB, diabetes, hypertension or renal failure. At the age of three and a half years he developed joint pains involving the large joints mainly, he had fever, epitaxis and jaundice too. The initial symptoms were severe abdominal pain following which he developed recurrent joint pains associated with swelling. My brother is youngest of three siblings. My eldest sister has a similar history, though the problem is not so severe. After investigation at several places his problem was diagnosed as sickle cell anaemia and was given: folic acid and hydroxyurea. He checks his Hb/TLC/DLC/platelets every thirty days. If the total WBC count is less than 4000 cu mm, he has to stop hydroxyurea and repeat the TLC after seven days. If TLC is more than 5000 cu mm he has to restart hydroxyurea. Incase there is no improvement in symptoms after three months he has to stop hydroxyurea. If there is reasonable improvement is symptoms in terms of frequency or severity, he needs to continue hydroxyurea beyond three months and report to the doctor. Incase of painful crises, he has been told to take detropropoxiphene (Darvocet) and vomeran if there is no significant relief, add Tidigesic. Also, he has to maintain adequate hydration. But my brother did not consume the hydroxyurea. Now he is 28 years and the problems are still there. The pain starts at any time without any indication basically in all the joints followed by fever.

A:Sickle cell disease can predispose to large joint problems particularly the hip and knee. As the viscosity of blood is high due to the abnormal shape of the red blood cells, blood is likely to stagnate and occlude small end arterioles in the bone. The head of the femur( thigh bone) is likely to suffer from a condition called avascular necrosis which means a death of the bone due to lack of blood supply. This may not be obvious on X-rays in the initials stages but only on MRI scans and a bone scan. As the problem is an inborn error, nothing can alter it. Sickle cell crises are likely to develop when there is dehydration, cold temperature( keep him warm at all times), and after less oxygen (hypoxia). If he has advanced joint destruction then hip replacements may be necessary.