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Inherited Disorders and Birth Defects

Hereditary spherocytosis

04/09/2008

Question:

I am 32 years of age. When I was 9 I had a splenectomy as I was diagnosed with Hereditary spherocytosis. I am currently pregnant and I want to know if it is safe to have a natural birth or would I need to have a c-section? Are there any other precautions I should be taking during my pregnancy?

Answer:

Thank you for visiting NetWellness. This is a management problem for someone dealing with high risk pregnancies and a high risk OB would be the person to answer this question, and that expertise is currently not available on NetWellness.

A few general comments: My understanding is that, in general, the rate of complications during pregnancy and delivery for women with hereditary spherocytosis is not greatly increased above the general pregnant population, particularly if they have already had a splenectomy prior to pregnancy. It does not seem from the literature that C-sections are necessary based on the spherocytosis - would assume a C-section would be based on other criteria. Obviously, these patients should be carefully monitored for anemia, etc - however, I do not know what else is considered appropriate management. Again, a high risk OB is the specialist to answer your question.

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Response by:

Anne   Matthews, RN, PhD Anne Matthews, RN, PhD
Associate Professor of Genetics
School of Medicine
Case Western Reserve University