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 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 bedridden. 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.


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Comments 17 comments

Seeker7 profile image

Seeker7 5 years ago from Fife, Scotland

This hub is excellent.

I've vaguely heard about DIPNECH but really had no idea what the primary cause was or treatment. I found it very interesting and took note of a few points. In particular about asthmatic females in their forties. One of my sisters, is a non-smoker, asthmatic so this information is well worth knowing.

That is so sad about your Mum! I do know how the decision to switch off life support can affect you. We had to make the same decision for my Mum - she was in fact just about the same age as yours, my Mum was 64. Basically she would have been peg-fed for the rest of her life - she had a perforated ulcer and ended up having almost 3/4 of the stomach taken away. She also had two heart attacks due to the strain of numerous operations and so on. Like your Mum, mine would have been left in a vegetative state and no way did we want that for her. The pain is tremendous, but like yourselves, we definitely know that we made the right decision for Mum. For me life is only life if you have the quality, it shouldn't be about quantity.

Great hub + voted up!


Karen 5 years ago

Thanks for posting this info and personal story!!!!! I also miss her she was my twin sister.


Renae 4 years ago

I was have Dipnech .I have been treated for asthma and COPD for 18 years. I had a biopsy last month and Dipnech was the diagnois . They removed a tumor and I felt better for about a month .Now I am feeling worse than ever.I'm short of breath and in pain when I breath.It has left me feeling sadly fatalistic. My doctors are baffled as to what to do for me.So I make the best of every "good"day I have. I have asked to start the Sandostian . I will post my results later.To the few afflicted with awful ailment my heart goes out to you.Its a tough road ahead .Be strong.


bulldogrocks profile image

bulldogrocks 4 years ago from Colorado Author

Renae I am sorry to here that you are struggling with this disease. I pray that God will bless you and make your story a successful one. I will look for your results.


KAREN MALONEY 4 years ago

Just to let you know Renae i am on Sandostian Injections once a month and i found they helped me i have good days and bad days with my cough sometimes when i have a bad day with it i don't know what to do the pains in my chest and ribs is terrible.Hope everything goes well for you.


dessertforDIPNECH 4 years ago

My mom was recently diagnosed with dipnech. I have been emailing and trying to find out information on this disease. One of the things I have decided to do is start raising awareness about the diease. I talk about it every where I go. I have started a pastry selling buisness to raise money for dipnech awareness. So far, I am kind of in the hole, but people are learing about this cancer. Please check out dessertfordipnech.webs.com

I have a blog there that I haven't started yet, but I would love to get a patient forum so my mom has people to talk to about what is going on.


hopefordipnech 4 years ago

Hi everyone,

My mom was recently diagnosed with DIPNECH. This is the only website that I have come across that has an active blog. So I have put another one together called HopeforDIPNECH.org. My moms doctor (Dr.Wolin) at Cedars was telling me that there aren't enough patients out there to even do research on DIPNECH. So I am trying to put together a data base of patients with DIPNECH to take to him and grant writers and say Look at this, here they are! My goal is to find a doctor that would be interested in doing clinical research on this rare cancer. My mom has been excepted into a treatment center in German, as soon as I can get the blog working I am going to do a daily blog and video blog to highlight our experiences. So please check out my website, and hopefully we can get some experience physicians to answer questions, if not Dr. Wolin will just get annoyed with me asking so many questions.


Anne Simpson 3 years ago

My daughter Elaine who is 40yrs has been told she has Dipnech. She has also been told there is no cure, and she has 2 large tumours in her lungs.


Anne Simpson 3 years ago

hello


dawnisfree 3 years ago

Just been diagnosed dipnech, most interesting reading posts in here on this rare lung disease, hope to learn more and will pop back here very soon to keep myself informed. Wishing you all peace & happy days xx


Gwyn Redfern 2 years ago

Ive got DIPNECH told I had it 6 yrs ago no cure have scans every 3mths nothing a waste of time growing slowly don't worry havnt heard of any research update yet so come back in 6 mths so fustrating the not knowing


dawn 2 years ago

Hello Gwyn

hope you pop back in here would be good to talk/exchange any possible infor that may help both us and our Dr's as so little known about this lung disease. What part of the world are you in Gwyn ? What brought about your diagnosis?

Look fwd to hearing from you


cindy who 2 years ago from ny state

i have recently been diagnosed with this disease and am wondering what one does to maintain (?) my style of living. is there anything I should/can do to make my "life" better???


carmen 2 years ago

I have the DIPNECH disease. I was diagnosed in 2004. It also started with asthma symptoms and I took everything from steroids to inhalers until I asked my doctor for a lung xray which led to a biopsy which led to surgery. Today I am being monitored by an oncologist and a pulmonary doctor. I have multiple nodules and was told it is slow growing. I still show obstruction with my pulmonary function test. Somehow not knowing too much about this "Uncommon" disease can be very frustrating.............


Leah 20 months ago

I have just been told that I have dipnech. I am 57 and have been treated for asthma for 10 years or more. I was having very difficult time with breathing and pains in my chest. I was always exhausted and had pains in the ribs. I can not sleep at night in bed because I can not get comfortable or breath lying down. I am getting ready to have a full body scan. So scared. Please keep posting any information about this disease if anyone gets any relief from any treatment.


Maureen 7 months ago

Hi I was diagnosed with dip nech 7 months ago I had a lung biopsy I also had nodules,tumors and cystal glass in my lung, no ones knew why I was coughing, short of breath feeling weak my lower back hurts bad pain in chest ever thing u could thing of and this has been 2 yrs and just now 7 months ago they figure it out !! Now this is where i am on tons of Medes and nothing helping I'm starting to worry cause I'm in hospital more then I'm home !! So finally going for second opinion hopefully I get the answer I'm looking for . I will let u know and if any one has any advise please let me know


Sarah H. 3 weeks ago

I have had dipnech for almost 7 years, wasn't too bad to start with but I now cough all the time and it is very embarrassing and painful. !!!I have been on Sandostratin for 5 years and did good for awhile but now my drs. can seem to give me nothing that helps the cough????? If you know of anything that helps please let me know.

thank you.

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