Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia - DIPNECH. If you found your way to this blog, it's probably because either you or someone you love has this rare form of chronic obstructive lung disease.
The damage associated with proliferation of neuroendocrine cells in the lungs was first discussed in a paper by Aguayo et al. published in the New England Journal of Medicine in 1992. Some years later the World Health Organization gave this entity the name DIPNECH. I scan the medical literature monthly and find occasional reports of small numbers of patients, but never any discussion of treatment.
I am a 55 year old female. My symptoms (chronic non-productive cough and dyspnea) began almost 20 years ago, but the diagnosis was made following an open lung biopsy 7 years ago. The first 13 years were spent going from pulmonologist to pulmonologist who tried to treat asthma. The open lung biopsy was finally ordered by a pulmonologist who ordered a CT scan, became alarmed, and realized what I had always known - that it was not asthma.
The bad news is that my pulmonary function has declined quite a bit over those 20 years, from an FEV1 of 50% predicted at my very first visit to a pulmonologist down to a current 15% (<.5L). The good news is that I've still got that half liter, and have continued to avoid transplantation. But I'm finding every day life an increasing struggle.
When I was first diagnosed, we contacted Dr. Aguayo and he recommended we consider treatment with a somatostatin analogue used to treat carcinoids. At that time, octreotide had to be administered via subcutaneous injection three times daily; in recent years a long-acting monthly injection became available. I was reluctant to begin this regimen for many reasons, not the least of which was that I didn't expect any miraculous results. But after toying with the idea for over 5 years, about 1.5 years ago I began taking long-acting octreotide. The treatment has alleviated symptoms somewhat (coughing less frequently), but has not improved PFTs or functional ability.
I'm starting this blog in the hopes of identifying other patients and sharing experiences. Because our numbers are still so small, DIPNECH is not likely to be the subject of formal study at this time. But there may still be something that can be learned.
