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Inherited Disorders and Birth Defects

Hereditary Spherocytosis

01/16/2008

Question:

My son is 6 years old. One day after he was born he was diagnosed with Hereditary Spherocytosis and AO incompatibility. He ad a partial Splenectomy on April 30,2007. The doctor took out 11 cms worth of spleen, leaving only 1 cm in. Six days post-op he had severe chest pain, after a few other episodes the doctor decided to remove his gall bladder. On June 5,2007 he went in to have that done, but prior to surgery he had an ultrasound done on the spleen. His spleen had grown to 6 cms. He then had a complete splenectomy AND cholecystectomy. He has had about 20 more episodes of chest pain, not quite as severe. He also complains of pain in both legs often, not in the muscle area but the bony areas. What other organs can be effected by Hereditary Spherocytosis? Could his leg pains be from HS? He had an x-ray done to see if he had chronic constipation but that was negative. I was told by a x-ray tech that a surgical clip was seen in the x-ray. Was this left there by accident or intentionaly? Could the clip be the reason for his chest pain?

Answer:

Its hard to answer specific questions like these without being directly involved in the care of your son. I can say, however, that Hereditary Spherocytosis should not cause pain like your son is experiencing.

Additionally, splenectomy is almost universally a cure for the disease. I suggest talking to your son's doctor about finding a different reason for his pains. The surgical clips are placed intentionally. They would not be the cause of his pain. I wish you well and hope you find a way to get to the bottom of this so your son can feel better.

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Response by:

Anthony D Villella, MD Anthony D Villella, MD
Formerly, Assistant Professor of Pediatrics
School of Medicine
Case Western Reserve University