A 6-month-old baby is being tested for an inborn metabolic defect after experiencing escalating failure to thrive and milestone regression. Hypotonia, apathy, deafness, hepatosplenomegaly, and widespread lymphadenopathy are all apparent on examination. An inspection of the fundus reveals a cherry red patch. A liver biopsy reveals sphingomyelin-loaded foamy hepatocytes. The body of a zebra may be seen via electron microscopy. What is your prognosis? Please elaborate.

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