NEHI Diagnosis

On April 28, 2011 I brought Emma to the emergency room because she wasn't feeling well and was getting pale. Within the hour Emma was put on oxygen and airlifted to DHMC. Even after 13 days of tests there, the doctors still didn't know what caused her respiratory distress. The picture to the left was taken during this hospitalization.

A lung biopsy was conducted in June 2011. The biopsy has been sent to at least two hospitals across the country for analysis. The initial results from Emma’s doctor indicated that Emma has “NEHI pathology”. When asked whether she has NEHI the answer is “she has NEHI pathology”. I think a simple yes/no answer is not given in the event that there are other yet to be known condition(s) unrelated to the NEHI that would complicate a prognosis for her.

NEHI is short for Neuroendocrine Hyperplasia of Infancy. I have no medical background and the following may not be 100% accurate, but the following is a brief explanation of NEHI as I understand it. Everyone is born with neuroendocrine cells. It is my understanding that kids with NEHI either have too many neuroendocrine cells in the lungs airway, the cells reproduce producing too many of these cells, or they were born with the appropriate number of cells and the cells never decrease.  Generally, the number of these cells decrease naturally. The cause of NEHI is unknown. In general, most kids with NEHI outgrow their need for oxygen at some point.  

There is little research about NEHI, its’ cause(s), prognosis, etc. The chILD Foundation was established to "to provide support, education and hope to families affected with a pediatric interstitial lung disease and to advocate and raise funds for scientific research." Click Here for a brochure they have prepared on NEHI.

There is not a lot of research on NEHI, prognoses, causes, etc. More important than knowing the cause of NEHI is the hope that one day Emma will be off oxygen. The hope that one day I can carry Emma upstairs to bed without holding Emma in one arm and the oxygen regulator in another, one day not having to hook up her pulse oximeter while she's sleeping, one day being able to take her for a trip without her oxygen tank, and one day being able to go for an outing without being asked "what's wrong with your baby". 

The photo to the right is the Emma we all know and love. She adores her big sister. She is sweet. Everyone always comments on her hair, which we all think is going to be red. She is weary of strangers. And,she is a happy baby who rarely fusses (except when she's tired, someone she doesn't know well tries to get too close to her, and at doctors visits).