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Question
Posted by: Maddy | 2006/04/16

Spferocytosis

My child was diagnosed with spferocytosis at the age of 3 Months.
He has received bloodtransfusion from time to time due to a severe
drop in his HB count. At three years they advised his milt will possibly be removed at later stage.He is now 7 years old, what is his change of outgrowing this condision or what can I do to postpone this operation as long as possible? He is leading a very active life and how will it effect is love for sport niw and in the future?

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Our expert says:
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Dear Maddy,

As the condition was diagnosed at such an early age, I must presume that it is the congenital form of spherocytosis, as opposed to the acquired form. In that case, he will have the condition for life, as his body is programmed to make abnormal red blood cells, and will continue to do so for the rest of his life. Simply put, these red cells have an abnormal shape, and are thus rapidly destroyed by the spleen. This results in anaemia. As part of this syndrome, he may therefore have chronic anaemia. Or he may have episodes of anaemia due to heamolysis, brought on by infections, especially viral ones. In rare cases, he may develop ulcers on his legs.

Removal of the spleen (milt) is usually advised to prevent the formation of gallstones, which occurs frequently in this condition. Splenectomy can also largely correct the anaemia, but does not correct the red cell abnormality. However, if this is done in children, there is an increased risk of severe infections. Splenectomy is thus postponed until adulthood if possible. Once this is done, there may be a significant improvement in the anaemia. Please note that before he has a spleen removal, you must discuss the matter of a Polyvalent Pneumococcal Vaccine with his paediatrician.

If your son has ongoing anaemia, he may feel tired all the time, and have very low levels of energy. One thing you can do is to give him Folate supplements, as this nutrient often becomes deficient in children with ongoing haemolysis. This is probably best administered as multivitamin.

As this is a complicated disorder, please consult your specialist paediatrician regularly. As the doctor who knows your son’s condition better than anyone else, s/he is the best person to advise you.

I hope your son’s condition stabilises.

HeartDoc

The information provided does not constitute a diagnosis of your condition. You should consult a medical practitioner or other appropriate health care professional for a physical exmanication, diagnosis and formal advice. Health24 and the expert accept no responsibility or liability for any damage or personal harm you may suffer resulting from making use of this content.

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