Patient discussions
Patients and parents of a child with hereditary spherocytosis (HS) should be made aware of the potential complications of HS. They should know when to contact a healthcare professional for evaluation of acute crises. Increasing pallor, jaundice, or fatigue, especially when occurring at the time of a febrile or viral illness, may indicate a hyperhaemolytic crisis. Medical consultation should be sought.
Patients without prior parvovirus infection should seek medical attention with any febrile illness (especially if accompanied by a rash) associated with increasing pallor or fatigue, even in the absence of jaundice.
Post-splenectomy patients must be warned that for the remainder of their lives they should seek immediate medical attention for any fever >38°C (>100.4°F). At that time, a blood culture should be obtained with subsequent administration of a parenteral broad-spectrum antibiotic, such as a third-generation cephalosporin. Patients should be warned of the risk of infection from dog bites and babesiosis.[45] There is a suspicion of increased mortality due to malaria, and some evidence that malarial parasitaemia is higher in asplenic people. The importance of adequate malaria prophylaxis when travelling to endemic areas must be emphasised.
Patients should be made aware of the possible complication of pigment gallstones. They should seek medical attention for recurrent or severe right upper quadrant abdominal pain, especially when it occurs following a meal of fatty foods.
Due to the chronic nature of splenomegaly in children with HS, there is no evidence of increased risk of splenic rupture. Special precautions, such as avoidance of contact sport, are generally not warranted.[45][64] National Organization for Rare Disorders (NORD): anemia, hereditary spherocytic hemolytic Opens in new window
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