DIPNECH the unknown lung disease

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH)

When people hear the words lung disease they think of COPD or black lung but there is another lung
disease called DIPNECH. DIPNECH stands for Diffuse Idiopathic Pulmonary Neuroendocrine cell
Hyperplasia. DIPNECH causes Neuroendocrine cells which are usually found in the intestines to
invade the lungs thus causing slow growing Carcinoid tumors to form in the bronchioles. However
these tumors differ from their counterparts since they are benign in nature.
Common symptoms are coughing, shortness of breath and sometimes wheezing. This disease mainly
presents itself in interesting diseases non smoking middle aged women who have been previously
diagnosed with Asthma. These women seek extra help when they realize regular asthma treatments
are not making them feel better.
The tests used to confirm this disease are a Cat Scan, pulmonary function test and a lung biopsy.
After it is a confirmed case of DIPNECH the proper treatment course can be implemented. However
as of right now there aren't many treatments that are helpful for patients with DIPNECH. It is
recommended that all patients diagnosed with DIPNECH be vaccinated for influenza, pneumonia
and whooping cough since these diseases would hinder breathing even more than it already is.

Unfortunately basic inhalers like albuterol will not give patients any relief since their bronchiole
obstruction can't be reversed like it can with asthma. Some patients do get relief form steroids
whether they are in pill form like prednisone or the inhaled form like Spiriva but it is only temporary
and certainly no cure.
There is another medicine that is called Sandostatin (the generic name is octreotide) and it is used
in patients with intestinal Carcinoid tumors but it can also help those with DIPNECH. It is believed
that the octreotide inhibits the pulmonary neuroendocrine cells (PNCs) enough to diminish the
cough therefore making patients feel a little better.
It is imperative that all patients have their oxygen levels checked while both awake and sleeping
since the PNC can cause more inflammation when they sense low oxygen in the blood stream.
Because of low oxygen in the blood stream many patients require the use of oxygen 24/7 or at least a
BIPAP machine while sleeping. The BIPAP machine "does two things - it helps push air into the lungs
and helps hold the lungs open to allow more oxygen to enter the lungs. Each time the patient
breathes, the BIPAP machine assists the patient by applying air pressure to the lungs while the
patient is breathing out (exhaling or expiration) in order to hold open the air sacs in the
lungs".(http://www.icu-usa.com/tour/equipment/bipap.htm)

When imaging shows Carcinoid tumors the best course of action is surgery. The purpose of surgery
is to remove the infected portion of the lung so the healthy lung tissue can expand better thus
improving the patients overall pulmonary functions. Lastly a lung transplant should be considered
for those patients whose disease has progressed into severe airway obstruction and they have lost
their ability to breathe on their own.
All information on DIPNECH can be found at
(http://dipnech.com/DIPNECH_Patient_information.html)
Personal story
With the medical explanations out of the way I can now explain how and why I know about this
disease. I had never heard of DIPNECH until my mom was diagnosed with it two years ago when she
finally went to National Jewish hospital here in Colorado. She started having asthma symptoms
about thirty five years ago and was put on albuterol for treatment. She was doing quite well with a
few colds etc here and there until 5 years ago when she was having a hard time catching her breath
so she went her primary doctor and he put her on oxygen. However he never sent her to a
pulmonologist partially because she didn't have insurance and because she didn't want to go to one.
I finally pushed for her to go to National Jewish Hospital hospital 3 years later when she needed
oxygen all the time but by then the disease had progressed to far and she was never able to go
without it ever again. The pulmonologist at National Jewish said that she had lost about 40% of her
breathing capacity and was very ill. He did put her on some steroid treatments but it only helped a
little. With an actual pulmonologist following her and the treatments she was able to at least
maintain what she had and not get any worse until she had to have surgery. My dad and I had to
take her to the Emergency Room because she was having tremendous amount of stomach pain and
that is when we learned she had a bowel obstruction and needed surgery. The surgery went well but
her breathing got dramatically worse and she had to be put on a ventilator. The doctors tried to take
her off the ventilator numerous times without any luck due to fact that her Carbon Dioxide levels
would get to high making her very ill. It finally got to the point where she needed the ventilator all
the time and became bed ridden. After 5 months in the hospital her body couldn't handle the stress
anymore and she had a massive heart attack which left her in a vegetative state. My Dad and I had
to make one of the hardest decisions of our lives and we chose to turn off the life support because
we knew my mom would never want to be that way. She died at the age 66 years old on May 21,
2010 at 9:45 pm leaving behind a daughter, husband and 3 grandchildren. However she touched so
many lives that many of my friends referred to her as Mom and their children called her Granny.