My name is Jim Coryell. I happen to be a pediatrician. In March of 2010, my wife Marcia was undergoing a pre-operative evaluation for a planned hip replacement. We were stunned to find that she had a two inch tumor in her right lung. She had never smoked a cigarette in her entire life. However, she did have an oppressive cough that had defied diagnosis and treatment for thirty-two years. We also knew that she had several small nodules in her left lung for almost two decades, but these had not changed or grown during that time. Despite numerous consultations from various pulmonary specialists, we really didn't know what the problem was. We knew that the various inhalers and medicines that were prescribed didn't work, and we just came to call Marcia's cough her "asthma", hoping that this would satisfy the curiosity of friends and the all too frequent rude inquiries from strangers when Marcia had a coughing spell.
The needle biopsy showed that she had a carcinoid tumor, something that I had heard of but never encountered in my pediatric practice. Of course her oncologist assumed that this lung carcinoid might have metastasized from her intestine, and we were frightened and devastated. A PET scan revealed several other "hot" nodules in her lungs but nothing elsewhere in her body. I was determined to learn all that I could about carcinoids. I spent many hours on the internet gathering information. Marcia was really struggling with all the uncertainty and gloom of the situation. Being a physician allowed me to be able to analyze scholarly articles from arcane medical journals about pulmonary carcinoids.
And then I stumbled on the mention of a rare condition called diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. When I read the first case study, my heart wouldn't stop pounding. It was what Marcia had. Exactly. Several weeks later Marcia underwent a wedge resection of the tumor, which gave us a tissue biopsy. But I already knew without a doubt that she had DIPNECH. Even after that surgery it took quite a bit to convince her various doctors that she had DIPNECH. Quite frankly, none of them had ever heard of it.
It has been a year since the initial revelation. It seems more like a lifetime. Marcia is coping remarkably well. She had modest improvement in her pulmonary functions after her surgery, and has less coughing since starting octreotide. I am developing this website because information needs to get out there about DIPNECH. I believe that it is far more common than anyone realizes, because it is difficult to recognize and virtually unknown among physicians. It is the only illness that I have ever encountered that does not have its own website (until now).
I have several goals for this website. First and foremost, I want to be able to explain this disease to the patients and their families who have discovered that this is what has been happening to them all these years. Secondly, I want to educate physicians about this condition so that they will be better prepared to help their patients. Thirdly, I want to start gathering information about DIPNECH from the patients themselves. Knowledge is power. If we can learn more about this disease, then we can start working on treatments. I am hoping that an interested pulmonary specialist will come forward to champion this cause, and that if enough patients with DIPNECH can be followed by one medical center perhaps a "center of excellence" can be established. Finally I would like for fellow sufferers to have a forum for sharing their experiences. To that end I will have a blog site, and a page with biographies of patients who want to share their stories. I have also recently learned of an illness in infants called neuroendocrine cell hyperplasia of infancy (NEHI) that shares some similarities with DIPNECH. I have added a page on this entity to the website with all the same goals I have for DIPNECH.