Werdnig-Hoffman is a form of spinal muscular atrophy.  This conditions are truly tragic and horrible.  The infant will lose neuromuscular function until he/she is unable to move.  The rate of progression varies with the specific form of the illness.  The baby can continue to breastfeed until the suck/swallow is affected to the point that the baby cannot feed adequately at breast.  When the diaphragm and respiratory musculature is affected, the parents will have to make some very difficult decisions as to whether or not to use a ventilator.  Once these kids are intubated, they never come off the ventilator.  The disease spares mentation, so they are fully aware of what is happening to them.

I am currently caring for a 5 month infant who has a particularly severe form of one of these SMAs.  In December she had muscle weakness but was comfortable and sociable and breastfeeding well.  Just 9 days ago she began to feed less well and develop difficulty breathing.  She is now on hospice status, as we (her parents & physicians) have elected to keep her comfortable and not intubate her.  This condition has progressed very rapidly; I suspect she has only a few days of life left at this point.  Sometimes, providing comfort is all we can do with certain horrible illnesses.

My sympathies with your patient; her parents will need all the support you can give them.

Linda L Shaw MD FAAP

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