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Children's Health

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07/10/2010

Question:

my 7 year old son has sphereocytosis and has 8 blood transfusions this year is that a good thing or a bad thing? i have tyed for 3 years to get his doctor to take his spleen out and they tell me all the risk of it i have had my spleen out 12 years now and i am fine i need to know what the risk are of all the blood transfusions that he has had and no one will tell me please help me

Answer:

As you very likely know, spherocytosis is a hereditary disease that affects individuals across a wide range of severity. The mildest disease never requires transfusions or splenectomy and those more affected require both. Eight transfusions in one year seems like a more severe degree of the condition.

While surgeons and hematologists try their best to delay splenectomy until after six years of age as often as possible, some children will require it early. At seven years of age, your son has aged out of the highest risk age group for severe infection (sepsis), which may cause death. However, I don't know if your son has any other health issues that make the decision for a splenectomy a more difficult one, such as a weakened immune system or other chronic illness. Multiple blood transfusions do carry risks associated with possible viral contamination of the blood (despite a great blood banking system), fever, allergic reactions, graft-vs-host disease, and high iron levels requiring chelation therapy to protect body organs from damage.

It is very much worth a detailed conversation with your son's physicians to lay out your concerns and frustration and to hear their opinions and rationale for delaying splenectomy, something no one should take lightly in childhood. It is helpful to many parents to have a support person(s) with them for these difficult conversations both as a source of support but also as an additional memory to recall what was said and another mind to ask questions and process responses. 

If you are not satisfied, you always have the right to change physicians, which is not an easy decision to reach for many of us.

I wish you a timely resolution of your concerns and the best of health for your son.

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Response by:

Mary M Gottesman, PhD, RN, CPNP, FAAN Mary M Gottesman, PhD, RN, CPNP, FAAN
Professor of Clinical Nursing
College of Nursing
The Ohio State University