Friday, October 12, 2007

DIPNECH

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.

55 comments:

lungdoc said...

RBR: I am a pulmonologist who has no particular expertise in this area. Indeed, I was looking at material because a colleague just diagnosed someone with DIPNECH, and I felt I should know something about the condition. I have a question for you. Since you have been on the long acting octreotide analogue and your cough has improved, have your PFT's stabilized even if they have not improved? Although not clinically proven to be of benefit, DIPNECH seems to have a lot of EFGR which suggests that an EFGR blocker (or perhaps a HER-1 blocker) may affect the process. Of course, if scarring has occurred, stopping the process may only stop progression and not reverse damage already done. Best of luck.

RBR said...

Dear LungDoc,
Sorry for the long-delayed response. To be honest, after the first month or so with no "hits" I forgot I had set this up. I'm not sure what made me check today, but I found your note 2 1/2 months after it was sent.
To answer your question, my PFTs have been more or less stable over the past two years, but truthfully that isn't saying much because if they were much worse I probably would not be here writing.
There was a recent paper out of Germany abou EGFR expression in DIPNECH. So far I haven't been able to interest my doctors in this. Your final sentence is precisely on the mark, and the main challenge in any discussion of potential therapies: because scarring has occurred, we can at best stop or slow progression. But how do we know if we have?
Thanks for your interest.

Joyce Stokes said...

I am also a sufferer of DIPNECH in the UK, I was diagnosed about 4 years ago after being treated for asthma for 5 years. I have two carcinoid tumours picked up on the last CT scan 9 months ago. It is frustrating not having contact with others with this disease. I am lucky to still be working full time as a teacher, my difficult time is January/February when I tend to pick up chest infections. Once the weather improves so does my health. I was also diagnosed by open lung biopsy.
Joyce Stokes

RBR said...

Dear Joyce,
I hadn't checked this in some time and I'm not sure what made me do it today, but here I find your note. So please forgive my not responding sooner.
Are you receiving any treatment?
I, too, continue to work full time, but everyday life has become an increasing challenge. I'm now using portable oxygen on airplanes and when walking, because I desaturate very quickly.
Please do keep in touch and let me know how you're doing.
- RBR

Joyce Stokes said...

Dear RBR
It is good to hear from a fellow sufferer. I am still working and coping reasonably well. I am on seretide and mucodyne which have improved my peak flow slightly. What is EFGR?
My oxygen SATs are about 97% when I am resting which is good. My biggest problem was accepting that I needed to slow down. Walking slowly means that I don't get out of breath so much.
Keep in touch.

joyce

winjenray said...

I have just been diag. with DIPNECH and it's a little overwhelming that it's so rare. For years they thought I was a servere asthmatic! I had a VATS lung biopsy for them to discover what was wrong. Keep up the blog and any information you can pass on.
Thank you,
Winnie

helen.schrader said...

I also have dipnech. found out about 18 months ago. before that I was being treated for COPD. Finally went to Denver ,National Jewish Hosp. for lung diseases. After the tests I was sent back to Oregon to Lung specialist and had the biopsy and found out what I really had. First person they found in the state who had it. After some meetings the doctors decided to try a cancer med. So far it hasn't gotten worse. I get a shot every month. getting ready to go off the med for a few months and see what happens. Hoping for good news but I don't seem to be doing better, just about the same. HS

RBR said...

There are 4 DIPNECH patients on this blog now - Rebecca, Joyce, Winnie, and Helen. I'd bet that all of you are "middle age." This is the typical profile of DIPNECH, such as it's known, middle aged, non-smoking females.

Now I'm going to ask a question. If you have children, or were pregnant, were your pregnancies complicated, and if so, by what?

JMM said...

Here is a shocker - my HUSBAND - 46 year old - never smoked - was just diagnosed with DIPNECH. We went to Fox Chase Cancer Center on the 8/25 for his 6 month cat scan check up and our doctor there is convinced that it is not sarcoidosis but DIPNECH. For some history, every year my husband gets a chest cold mid October - bad, constant very, very productive cough, it usually goes away in a week or so, but last year, it didn't go away - it got worse. After many x-rays, cat scans, pet scans, etc. 2 masses were foud in his right lung, one upper, one lower. In January, he had 2 biopsies done and once he was opened up, they did 2 wedge resections to remove both masses. They ened up being carcinoid tumors and also they suspected sarcoidosis from the pathology of the tissue. After the diagnosis, we went to Fox Chase Cancer Center since our regular pulomnolgist had no experience dealing with carcinoid. My husband has been weaned off all but 4mg of Prednisone and is also on Albuterol and Pulmicort nebulizer treatments 2 times a day - which we were told happens to be the same treatment for sarcoidosis and DIPNECH. If he tries to go lower than 4 MG, he cough coems back - at this dose, there is rarely a cough. The only strange thing is this - he has a VERY, VERY productive cough, not dry as others experience. We go see my husband's regular pulomonoglist the end of Sept - he is the one treating him for the sarcoidosis so we are unsure how he is going to take this new diagnosis from the other doctor. This condition is very rare and I am no doctor but to me, if this is endocrine related and effects mostly middle age women (menopausal) then there HAS to be some sort of hormone level factor that causes this endocrine malfunction. Any other thoughts?

jill said...

.... add Jill to the names on this blog that have been diagnosed with DIPNECH.

My story has similar elements to most of yours: non-smoking, middle-aged female, medically unresponsive severe asthma-like symptoms for about ten years, a trip in 2006 to National Jewish Hospital in Denver, endotracheal biopsy, and then the diagnosis.

Since then I have had monthly injections of octreotide and have done quite well. Overall, on a day-to-day basis I feel very good --- my symptoms have diminished significantly, I am very active and able to exercise without difficulty.

My PFTs aren't great, but have not really worsened since my diagnosis (from the outset I was told not to expect improvement). As for my CT scan, I have three small tumors which have remained stable since
they were first identified.

To JMM: regarding your question about hormones, I know there is a correlation although I can't explain the science. My experience is anecdotal but interesting. Some time after starting treatment I noticed the my hot flashes had stopped. I did not connect the change to my disease/treatment until an attempt was made to lower my dose of octreotide and the hot flashes returned. Once the dose was readjusted back to the higher level the hot flashes dissipated. This tells me that there is a hormonal component to the disease. My MD's are intrigued by this occurrence and are attempting to following-up.

For those of you on the same treatment, have any of you experience peripheral numbness and tingling? This is a symptom that I have been dealing with recently and have seen a neurologist for an explanation. When I posed the question to him about the symptoms being possible side effects of octreotide he decided to investigate, I am now awaiting his feedback.

I am extremely grateful that I happened upon this blog and hoping that our communications will continue and be a source of support and information. I hope all of you are doing well.

jill

jill said...

.... add Jill to the names on this blog that have been diagnosed with DIPNECH.

My story has similar elements to most of yours: non-smoking, middle-aged female, medically unresponsive severe asthma-like symptoms for about ten years, a trip in 2006 to National Jewish Hospital in Denver, endotracheal biopsy, and then the diagnosis.

Since then I have had monthly injections of octreotide and have done quite well. Overall, on a day-to-day basis I feel very good --- my symptoms have diminished significantly, I am very active and able to exercise without difficulty.

My PFTs aren't great, but have not really worsened since my diagnosis (from the outset I was told not to expect improvement). As for my CT scan, I have three small tumors which have remained stable since
they were first identified.

To JMM: regarding your question about hormones, I know there is a correlation although I can't explain the science. My experience is anecdotal but interesting. Some time after starting treatment I noticed the my hot flashes had stopped. I did not connect the change to my disease/treatment until an attempt was made to lower my dose of octreotide and the hot flashes returned. Once the dose was readjusted back to the higher level the hot flashes dissipated. This tells me that there is a hormonal component to the disease. My MD's are intrigued by this occurrence and are attempting to following-up.

For those of you on the same treatment, have any of you experience peripheral numbness and tingling? This is a symptom that I have been dealing with recently and have seen a neurologist for an explanation. When I posed the question to him about the symptoms being possible side effects of octreotide he decided to investigate, I am now awaiting his feedback.

I am extremely grateful that I happened upon this blog and hoping that our communications will continue and be a source of support and information. I hope all of you are doing well.

jill

winjenray said...

RBL: You are right I'm 54 and a non-smoker, typically for DIPNECH. I taught in a sick classroom for 5 years which caused most of my asthma problems which I never had growing up! I did have complications in pregency such as High blood pressure, and 2 c sections. Not sure if that is the info you need or not.
The doctors at National jewish in Denver this summer suggested i have a lung biopsy which confirmed the diagnosis of DIPNECH. My allergy doctor from my hometown suggested I go out there to find out why all the steriods didn't help my asthma. I' glad I went and had the lung biopsy. I'm on monthly injections of Sandostatin LAR and my PFT's are stable, from June until now. I'm still able to teach full time, just exhausted all the time, some swelling of ankles, etc. But glad to wake up every morning to a new day! I think it makes you appreciate life and all the changes more! Good luck and keep up the good web site!
Winnie

laura said...

my little sister (45) has been diagnosed with DIPNECH back in june of 09. she had a tumor removed in June 09, and went to the Cleveland Clinic in October 09 to do tests for a possible double lung transplant. Cleveland told her on the last day of testing that she would not be eligible for the transplant unless she cancer free for 5 yrs. to date, she has not received any treatment. I see alot of you on here have lived 3+ yrs, but i am not sure my sister fits into this catagory. Does anyone have any insight as to how/why you have been able to beat the odds?? what treatment have you received?? Please, any insight to your case is most welcome. She is on ox 24/7, and lung capacity now is at 38%. We as a family are desprately seeking any information. thank you.
Laura

bulldog said...

Hi my name is Jennifer and my mom Kathy who is also a non smoker age 65 got diagnosed with DIPNECH in June. But it took them 3 yrs to figure it out and now she needs oxygen all the time because without it her O2 level drops in the 60s. Right now she is having a problem with her blood gases be to abnormal giving her to much CO2. Come to think of it she has been treated for asthma for 28 years so I wonder if it could have been caught sooner. Plus I noticed her breathing symptoms get worse after shes stopped taking her hormone therapy meds 4 years ago. My biggest fear is what if it is heredity me and my three children also have asthma. One question I have is what to you all do for exercise since my mom cant walk what else can she do so she doesn't start losing her muscle tone? Well I hope you all are having a good time for the holidays. Take care and God Bless.

AnitaRN said...

I too can be added to the list of DIPNECH. I was diagnosed in June of 2009 after experiencing right flank pain that my MD thought might be a kidney stone. "Pulmonary nodules too numerous to count" found on Cat Scan, PET scan was negative but ended up having a pulmoary thoracentesis with 2 wedges removed. I still have one in my right lung (where the others were) that is almost 10 cm. I see my MD next Friday and am afraid she may want to have it removed which means another 8 weeks off of work. My biggest problems are exhaustion and pain in both of my lungs in my back. I also am having personal battle with this diagnosis as I am a Hospice nurse and work with patients with carcinoid tumors and watch their rapid progression after "turning the corner". I have looked for clinical trials but have not found any for DIPNECH and do not know if they would be beneficial. Curiously, how do you know when it is time to start the Sandostatin. Octreotide or my doctor has even mentioned chemotherapy (which I have read does no good for DIPNECH) Sorry for the scatterbrained questions but just glad to find others like me.

Lynn55 said...

I am so happy I found this blog! I can't believe your story, this is my story! I have been fighting this cough for over 20 years..with multiple pulmoniary doctors, allergists, multiple surgeries, reflux and sinus, etc.. I finally went to National Jewish this week and they diagnosed me in one week. I too have Dipnech. I am very depressed right now. My volume level is only 36% and that is down from 60% in 1995. I have had this cough for so long and everyone told me it was asthma only I kept telling everyone the normal astham medicimes have no effect on mfe what so ever. I will be 55 this year also. I still have an uncontrolled cough but I was just diagnosed today. There is one more test they need to do on my biopsy they took today from my lungs then they will know for sure. I have to tell you I am very depressed right now. I am too young to be on oxygen but that may have to be my life from now on. I really suffered this week in Denver with the higher elevation. If anyone has any tips for me, I would welcome them. I just can't believe this. I am glad to see that I am not alone. I have never had children. I used baby powder/talcom power for years and am wondering if I breathed it in too much and it caused it.. anyone ever heard of that? I never smoked.. I am glad to have found you.. I am book marking this page..

Julia said...

Dear RBR,
My mom was just diagnosed with dipnech today. She is a 59 year old female who never smoked. She has suffered from a severe cough since she was in her twenties and, like many of you, was treated for asthma to no avail. Since this is such a rare disease, I have been unable to find much information on it. I'm scared because I have no idea what to expect. The doctors are saying that surgery would be the best thing for my mom, but her pulmonary functions are at 20% of what they should be and they are still running tests to see if they feel they can safely operate on her. Judging by your own experiences, do you have any suggestions of what I should expect? Or what I can do to best support her through such a difficult time?
Thank you

bulldog said...

Hey all I hope all is well. I am unfortunately very sad since my mom died from complications due to her illness. Her breathing became so bad that she had to go on a ventilator 5 months ago when she went in for surgery and couldn't get her body to recover so she could come off. She kept getting infections and was restricted to bed being feed through a tube. But she kept fighting she wanted to see her grand kids but sadly she died May 21, 2010. She was only 66 yrs old and left a Husband, Twin sister, Two daughters and four grand children. She will be greatly missed

Lynn55 said...

Hi Jennifer, I am so sorry for your loss. It is so sad. I am hoping they will come up with better therapies or at least better prognosis for lung transplants before I actually need one. I am down to 36% lung capacity so far.

bulldog said...

Lynn hopefully your lung capacity doesnt get any worse I will keep you and every one else in my prayers.

RBR said...

Jennifer, I am so sorry to hear about your Mom. It sounds like she really struggled at the end, but I'm sure your love and support meant a lot to her.

For the others who are reading this, the relatively good news is that Jennifer's mom is a bit unusual, in that most of the cases written up in the literature at this point have not rapidly progressedn the way Jennifer's mom did. However, my pulmonologist worries a good deal about infections. I've managed to dodge the bullet so far, and am hoping to continue to do so. But I also recognize that it may just be one nasty bug between me now and the end of me.

I agree with Lynn - I'm hoping for ongoing improvements in lung transplantation before I'm forced to go that route. I've completed the full work-up and all the other general health requirements.

My FEV1 is < 15% of predicted. But with the supplemental oxygen I'm still working and getting around. I just hope to continue to do that.

Jim said...

Jim: I am a pediatrician. My wife Marcia was recently diagnosed with DIPNECH. My daughter Julia posted to this blog about a month ago. Marcia has lived the life that all of you have so eloquently written about. She is currently 57and began to cough almost 30 years ago. Twenty years ago a pulmonary nodule was noted on CXR but serial CT scans showed no progression so we elected not to have it resected. She has been to many pulmonologists including Stanford and Sacramento, with multiple diagnoses including asthma, brochiectasis, bronchiolitis obliterans, etc., but no treatments were helpful. Recently, she was to have a hip surgery but her pre-op chest x-ray revealed a 5 cm mass and multiple small nodules. A needle biopsy showed that the large mass was a carcinoid. As you can imagine, I hit the internet bigtime and stumbled upon the entity DIPNECH in my research. I knew instantly that this was what Marcia had. She then underwent an open thoracotomy two weeks ago wherein the large tumor was removed by wedge resection and another nodule was also removed. The specimens confirmed carcinoid tumors and the presence of diffuse bronchiolar infiltration by neuroendocrine cells (DIPNECH).
Marcia is of course devastated even though she has been dealing with the disease without a diagnosis for almost thirty years. Because of her cough she has had to avoid many activities that she loves. She has had to endure negative comments from strangers becuase of her cough. However, she has persevered and until recently was working full time as a special education teacher. We have three lovely daughters but no grandchildren. She tires easily but is not on oxygen. Her most recent PFTs show an FEV1 at 29% of predicted.
I have included all this information intentionally. As a physician I believe it is extremely important that all of us who are affected by DIPNECH should be communicating regularly. This is important for mutual support, and it is possible that we may be able to find some answers by comparing our experiences. I will be looking into the EGFR idea. Unfortunately, DIPNECH is so rare that I doubt any research is being done on it. Therefore, we need to be our own advocates. Marcia is still too fragile at this point, following her surgery and all the "bad" news, to be blogging, but I know she will want to be communicating with all of you when she feels better.
I have a question. Two years ago, Marcia was diagnosed with rheumatoid arthritis. This was based largely on a sky high rheumatoid factor blood test. Oddly enough, she doesn't really have any significiant small joint disease as would be expected with someone who had a Rheumatoid Factor of 1500. I believe it is related to DIPNECH. Do any of you have RA or elevated rheumatoid factors? Also Marcia had a terrible time with pain after her thoracotomy and she is wondering if anyone else had thoracotomies that were difficult.
I am very happy to have found this blog. I will be checking in regularly and hope to hear from as many of you as possible.

Lynn55 said...

Hi Jim, my heart goes out to you and your wife and family. I completely understand your wife's depression since I have been there myself. Just yesterday, I got the results from my sleep test. I need to go on oxygen and a CPAP machine at night My oxygen levels drop to 79 while sleeping and my breathing stopped 97 times. I have been in denial at this point. I need to have the larger biopsy done but I think I will wait until next year since I have had so much done this year already. I can think back that I have had this cough for over 30 years it has just gotten worse. I also still work full time since I can't really afford to retire yet but thank God i work at home which is a huge help. I do not have Rheumatoid arthritis but sometimes my hips hurt. My lungs hurt also frequently but not bad enough where I need to take anything for it. I just get exhausted from coughing. The only thing that seems to help is Tussinex but I only take a small amount when I am desperate. My pulmonary specialist here had never heard of DIPNECH either and suggests I get the larger biopsy done for the firm diagnosis also. I think I am in denial. Even though the Dr.s in Nathional Jewish are pretty sure I have this disease, if I get the full biopsy, I will know for sure and will be more depressed. I know it doesn't make sense. I am sure I won't be able to go on octreotide or the other medicine until I get the firm diagnosis. I did want to let everyone know that from the research I have done, there is about a 50% chance that this disease will turn into lung cancer. I found a clinical trial on Inositol in Preventing Lung Cancer in Current or Former Smokers With Bronchial Dysplasia. I know this is a wild hare idea but I started taking insoitol daily as a supplement in the event it might slow down or stop the development of lung cancer. Here is the website: http://clinicaltrials.gov/ct2/show/NCT00783705

Since there is no research being done that I have found, we need to try to help ourselves. I bet this is a way underdiagnosed disease and there are a lot more people walking around coughing and do not know what they have.

RBR said...

Jim, thank you so much for sharing your wife's experience. DIPNECH is often an incidental finding to carcinoids or other carcinomas. But there does seem to be a basic belief in some camps that it is a precursor to those, and that makes sense when the patient has had symptoms for so long. I hope she continues to recover from the surgery. Because she actually had full-blown carcinoids, she would certainly seem to be a good candidate for trying ocreotide (Sandostatin). My impression is that there are some patients who have responded quite well, even with improvements in lung function. I did not have such a dramatic improvement, but at least I'm still here, with no significant growth of the so-called tumorlets, so since I'm tolerating it I've stayed on.

Lynn, that particular trial really doesn't relate to DIPNECH. The whole purpose of the trial is to try to figure out whether that supplement might in fact help, but still they are looking at either former smokers (which most of us are not) or patients who've been treated for lung cancer (which most of us are not). So whatever the findings, they may well not be relevant to patients with DIPNECH. That's not to say you shouldn't take this supplement, but just to make sure your eyes are open.

Jim, please do keep us informed of Marcia's progress.

Maybe it really is time to start collecting more detailed information from our little community of DIPNECH patients. Let me think about putting together a form to gather data.

helen said...

I have been reading all of the information that has been sent during the last few years. It seems to me that each one of us has a different story about this Disnech disease. My thoughts are that Dipnech is not just one disease but a group a diseases that can't be put in one pot, so if not sure call it Dipnech. To many different stories. I have had this disease for a number of years now. I went through the Denver Hospital also, and then back to Oregon, where I live. The lung biopsy was done in Portland and again the findings were that I have dipnech. I was put on Sandosatin for a year and a half. Then I was taken off the meds to see how my body would re-act. My lungs have stayed about the same. I have been off the Sandosatin for almost a year now. My breathing is not so great, but I can live with it. No more long walks or even short walks. I would like to wish everyone well, and keep in touch. Helen

JMM said...

All, while my heart goes out to all of you, please know there is hope and I want to share our experience. I wrote a while back -JMM with my husband's story. His doctors are still battling it out as to what exactly he has but I can tell you the treatment they HAVE agreed on is working, prednisone and nebulizer treatments. PLEASE have your doctors consider SARCOIDOSIS if you have Carcinoid tumors that have clinical findings somewhere between typcical and atypical. Unfortunatly, carcinoid tumors and the presence of diffuse bronchiolar infiltration by neuroendocrine cells can also be SARCOIDOSIS! I am GLADLY reporting that my husband is off all of his meds and is down to 1 nebulizer treatment a day with pulmacourt and albuterol. PLEASE look into this for yourselves if you have been diagnosed with Carcinoid tumors - there IS hope for preventing decreased lung function if it is caught before the tissues is scarred! My husband has coughed for the 13 years I have known him and it is a BLESSING not to hear him cough anymore! Best of luck to all of you!

Jim said...

Hi all
This is Jim. Thank you for your thoughtful reponses. I am encouraged that we can share our experiences.

RBR (?Rebecca) - I have been doing research on EGFR (HER1 receptors). I found the same article in PATHOLOGE that I beleive you were refering to in a prior blog. It certainly presents intriging possibilities. I was unable to find any reference to clinical trials with regard to DIPNECH. However, I plan to call the pharmaceutical compnay directly regarding their clinical trials with HER1 receptor blockers in cancers and see what I can find out. I am also pursuing some other leads about resources for DIPNECH and will keep you informed.

I noticed that at least three of you have been to National Jewish Hospital in Denver. This would lead me to believe that they have as much experience as anybody. There is a physician there who is the lead author for the only review article I could find on DIPNECH. His name is Jeffrey Swigris. Have any of you met him? Marcia and I would like to travel there, but we want to see a doctor who has at least some experience with DIPNECH.

I am still wondering if Marcia's elevated rheumatoid factor is from DIPNECH, not rheumatoid arthritis. If any of you are getting lab tests, could you ask your doctor to order a RF (rheumatoid factor)?

I have learned that neuroendocrine cells are oxygen sensitive. Therefore I wonder if low levels of oxygen might cause them to proliferate leading to more small airway obstruction. If any of you have low oxygen levels, even if it is just while asleep, it makes sense to me to go on oxygen. Probably everyone should get a sleep study even if you feel well (I am now thinking of Anita, Winnie, Jill, and Joyce whose comments suggest that this aspect of DIPNECH has not been addressed).

DIPNECH is the first subject that I have ever encountered which does not have its own website. Do any of you have experience in website design? We need a larger forum.

Finally if any of you would like to communicate with me directly, my email address is jdc735@gmail.com . If any of you are feeling especially hopeless about all this, the article I mentioned above states in its summary that the long term prognosis of DIPNECH is generally good. The article was published in a prestigious medical journal called BRONCHOLOGY but costs $35 to get copies of an article. I would be happy to send any of you a copy if you like. Since medical articles are full of large, fearful sounding words, I could just send the summary.
Lets stay in touch. Marcia is still too apprehensive to blog about DIPNECH but she is doing better this week and may want to log on soon.

Debie said...

I am recovering from the surgery that gave me the diagnosis of dipnech. I am 57, never smoked and I don't have a cough. I had thyroid cancer about 4 years ago and I am a DES daughter. I haven't been back to my follow up appointment yet. So far I have an appointment with a lung doctor and am waiting for results of specimens sent to another doctor. I have no idea what to expect. It would be nice to hear from someone else that knows what I am going through.

Thanks,
Debie

Lynn55 said...

Hi Debi, my heart goes out to you. I also had the bigger lung biopsies in October and they came back positive for DIPNECH also. I haven't done anything else since. You certainly fit the profile. I do to. I am 55 and never smoked. But I have a cough all the time. I really need to go back to the National Jewish for a follow-up appointment but I have been so busy at work and taking care of my Father, I just have not had the time.

laura said...

my sister, 46, was diagnosed with DIPNECH last year in may of 09, and had her lower lobe removed in june of 09. her biggest complaint was either pneomia and/or broncitis. it is not dec 31, 2010 and we just now found out that they are going to stop the clinical trial she is on......since jan 2010. we don't know what to do at this point, and she is an out patient at Roswell Park Cancer Institute. we don't know what lies ahead for us now, and there is no cure, and almoost no hope at this point. what is one to do??? what are we supposed to hang onto?? there is not a trial out there now that will accept her, and we have to hope and pray that we can get into another trial. this whole thing sucks, and we wont' give up.

laura said...

I posted many times on different blogs, but i still want to know of anyone who is doing clinical trials for dipnech. my sister was diagnosed in in 2009 and had a lower lobe taken out. since then, we are on a clinical trial at roswell park cancer institute, but the side affects are getting worse. the tumorlets have not grown since the trial started, but as i said, the side effects are terrible. anyone else out there?? she was diagnosed at the age of 45 and a non smoker. go figure. it is a long hard road and a tough pill to swallow, but we will not give up.

Lynn55 said...

Hi Laura, I don' tknow of any clinical trials.. but as far as I know my nodules are not growing either so maybe the trial has nothing to do with that in her sister. Why was the lower lobe removed? Did she have cancer? I am in the same boat.. 55 and never smoked.. Tell your sister to hang in there. What was the clinical trial? What is the medication she is taking and where is it being conducted? I think that would be of interest to the others on this blog.

helen said...

from Helen
What I know about Dipnech, I found out 3 yrs ago, that I have Dipnech. I went to Denver Research hospital at that time and when they ruled out other diseases, I was sent home to work with a Doctor in Portland Or. who kept in touch with the doctors in Denver. My Doctor in Denver, was Dr TATE. I had the bi-op in Portland where they confirmed the Dipnech disease. After much discussion(because, they had no guidelines on how to treat me the doctors finally decided to prescribe Sandosatin Shots(very expensive). I was on that for 1 and 1/2 years and the results are good at this point. I have been off the meds for over a year now and still going in every 6 months to check that all is going well. There is always help, and we just have to hope for the best. God Bless each of you that is fighting this diease.
Know that you are not alone in this

Lynn55 said...

Hi Helen, I would love to talk to you if you don't mind.. my email address is gwiede1@tgti.net

RBR said...

Amazing coincidence - Laura posted earlier today, and I didn't know that when I started a new post this evening about a paper published last month. Please read that post and send e-mails to the author. Let's encourage them to start up something!
Laura, there must be something else going on with your sister because there are no trials specifically for DIPNECH. The only thing I can figure is that they are treating it as a neuroendocrine tumor, and there are many drugs in trials to look at those. The trouble is that it's not exactly that, so we have no good way of knowing whether that would help.

laura said...

Hi again, I will get the name of the chemo pills my sister mona is taking for the clinical trial. i think she does have more than the dipnech, and the tumor was removed from her lung. i forgot to say that she also had a cough, for almost 15-18 years that i can remember. i always told her to get a second opinion, but it took this long for her to get diag. she goes to roswell every other week. she takes these pills for 28 days on, 2 weeks off. ct scans every 6 weeks. we thought they were going to take her off this trial, but the ct scans were stable so they kept her on this. i know its not fda approved, but as i said, i will get the name of this drug. she also has tingleing in her fingers and arms. she has also gained 120lbs since being on this drug. she was also diag with RA three months ago, and has since had injections in her knees. i just hate seeing her so exhausted all the time. there has to be something else out there that has a better quality of life!!! it will be two years in june since she was diag, so we are happy about that.

JMM said...

There is a not a whole lot of info known about this disease. My husband's doctors are still fighting whether it is carcinoid cancer (which is usually in the gut, not lungs) or DIPNECH. He had a wedge removed where the nodules where and there were tumorlettes attached which led one doctor to confirm that it was carcinoid and the doc at Fox Chase Cancer Center diganosing it as DIPNECH. To further complicate matters, he also has sarcoidosis and after a year of up and down on prednisone and daily treatments with Pulmacort and Alburterol in a nebulizer, he has almost full lung capacity again and is off all of his meds.
Don't take what the doctors tell you at face value. Carciniod cancer looks JUST LIKE DIPNECH and is one of the few cancers that does not spread and can be cured by removing the nodules. It is very slow to grow so it does not appear on PET scans.
My husband's pulmonologist and onocologist can debate it all they want. all I know is the treatment seemed to work and whether his condition is Carcinoid/Sarcodosis or DIPNECH doesn't matter as long as he is recovering!
I wish you all luck as we go through this together!

laura said...

Hi its me again, yes, my sister mona has more than DIPNECH, she also has the neuroendocrine tumors and is taking the unapproved fda drug called Cep11981. if she only had the dipnech, i think she would be feeling better than she is, but due to the other problems, she is just not well. the pain meds she is on includes opana 40mg 2x day, and oxycodone 15 mg 4 times a day. she has a perm pain pump in her back due to the fact that when they inserted the chest tube, it scraped against something in her chest and has caused severe constant pain. since the pump, that particular pain is now under control. alot of her pain now comes from the excessive weight gain and the strain on her hips, knees, legs, back etc. we try to get her to eat so she does loose weight, but with the chemo pills, she feels sick to often. i have her drinking the boost extra, yogurt and fruit and she seems to like that. well, i hope this explains a little more for you all. i will be going with her to roswell to ask more questions next week.

Jim said...

Hello fellow DIPNECHERS. I have logged on to this site several times, became overwhelmend, and logged off. However, I am feeling cheerful and hopeful today, so I wanted to say "Hi" to all of you. Ten months ago I had surgery to remove a large carcinoid tumor of the lung, and was diagnosed with DIPNECH. The only reason I got this diagnosis was because my husband spend hours researching my symptoms and found your blog site. (Thank you!) All my physicians had never heard of DIPNECH. I want to let you all know that after a long recovery, I am feeling pretty darn good most days. I am a little short of breath, and seem to get a little more tired than others, but its definitely doable. I even went back to work as a Special Education Teacher half time.
Anyway, the only comment I want to add is that I believe this disease can be very slow for many. I now think I have had DIPNECH more than 30 years. I started occtreotide injections and my cough improved greatly.
Last comment, my husband, who is a pediatrician, is trying to set up a web site to help us all gather more info.
Good breathing to all! Marcia Coryell in California

Jim said...

Comment to Joyce,
I teach also, and the last cold I caught lasted 6 weeks and gave me many problems with coughing and shortness of breath. It made me wonder if teaching is a good place for people with DIPNECH? Marcia

Joyce said...

Dear Marcia
I am still teaching full time and have only lost one teaching day sick since the start of term in September. This is the first winter I haven't been ill. This is due to Azithromycin an antibiotic I have been taking for the past year. I have had colds but not the awful coughs I was getting after them. I have been diagnosed about 5 years but have had problems with my breathing for about 15 years. I have a couple of tumorlets but they don't seem to be causing any problems. Good luck to all of you.
Joyce

bjunette said...

I was just diagnosed with DIPNECH this week. My symptoms started almost 1 1/2 yrs ago with a dry cough. I am a 49 yr old female non-smoker. I too was told I had asthma or sarcodosis and finally hypersensitive pneumenitis. Inhalers never worked and neb treatments dind't help. I finally decided to seek 2nd opinion & was referred for lung biopsy. It showed a carcinoid tumor and multiple tumorlets. I was referred to M.D. Anderson cancer hospitl in Houston, TX. The endocrinologist has started me on octreotide and its seems to have helped the cough some. It's too early to tell if this will be a help since I have only had 1 shot. I am on oxygen at night and that helps to feel stronger during the day. I have 49 to 53% lung function and O2 stats of 96 to 98 during day. I am still working fulltime although I am very tired by the end of the day. I am happy to have found this blog as it is very frustrating how little info is available on DIPNECH.

laura said...

Hello all, its Laura again. Its been a little bit since i last posted, and bj, i am sorry to hear of your diagnosis. if have researched what little is out there, dipnech is a very slow growing cancer if that gives any hope. I found out my sister has in addition to dipnec, pulminary carcinoid tumors. the trial she has been on for that, not the dipnech has only made the side effects worse. she is the only one left on this trial for the tumors but they took her off for an additional two weeks because of a sore that just appeared on her left breast. what this means, is she is off the chemo pills for a total of four weeks, (longest allowed by the protocal) to see if the pills are causing the sores to start now. we have only 6 days left to go and back to roswell to see if they are going to "let" her continue on this exper. I hope to god they take her off of it, as the some of the side effects are probably irreverseable at this point. arms/hands shaking, can't hold on to anything, numbness in arms and fingers, still the massive weight gain (130+ lbs now). i have contacted the cancer instit of america and they gave me a number to call for possible local trials for the carcinoid tumors, but once again, it is up to her to make that choice. we keep hanging in there, but nothing at this point is getting better. we just celebrated her 47th bday in april!!!

Lynn55 said...

Hi bjunette - I am sorry you are having to go through this... It sounds like you are not that bad though at this point. Your lung function is reasonable it sounds. Mine is down to 31% an I still work full time also. I also get tired. I was using oxygen at night also but I did not see how it was helping.

Lynn55 said...

Laura: I thought this was NOT cancer but it could possibly turn into cancer if the tumors get larger.

laura said...

You are right Lynn, DIPNECH is not cancer, its a very rare lung disease. I stand corrected.

helen said...

I have written on this blog a number of times over the last 3 years. I have dipnech and went on the med Sanddostatin for 18 months and have been off of the meds for almost 2 years. The disease is still there but has not gotten any worse. It will never go away but if it doesn't get worse, I feel like I have won the race. I inquired about testing and was told that not enough of us have it so there is no money for research. Wow doesn't that put us on the bottom of the list.

Emily said...

I am a 41 year old mother of four, and two years ago finally had a CT scan after living with "mystery asthma" all of my life, and coming down with pneumonia/bronchitis every winter, including H1N1 in 2009. I just knew it was more than asthma. That cough never went away! So, the CT revealed a large tumor in my right bronchi, and I had an open thoracotomy and a bi-lobectomy and they found it was a carcinoid tumor after the surgery. I have a carcinoid specialist here in Portland, OR, and a pulmonologist but have not yet been officially diagnosed with DIPNECH - I am buried in medical debt and can't afford to try sandostatin or get a lung biopsy. I am still saving up for my yearly CT scan. I have had the cough/asthma for so long, and yet I've been pretty healthy until the last 10 years. My pregnancies were easy - my babies were born at home, and I eat well and use natural medicine whenever possible. I am glad they took out the tumor but I have had a difficult time recovering from the thoracotomy (the pain continues) and finding energy to clean my house and raise my kids (I did have to quit my job as a preschool teacher) is a daily challenge. I have been looking for a trial or study to be part of to help pay for DIPNECH diagnostics, and am interested in going to Jewish National in Co. if it won't cost too much, but not even sure an official dx will do me any good at this point.

Massimo Milan said...

buongiorno,
sono una signora di 60 anni ed anche a me è stata diagnosticata la DIPNECH dopo essere stata trattata per asma per lunghi anni. Sono in cura presso l'ospedale di Forlì in Italia. Ho visto che ci sono molti pazienti che insegnavano (o ex insegnanti). Può secondo voi può esserci un collegamento tra la polvere di gesso e la malattia?
Grazie

hello,
am a lady of 60 years and I too was diagnosed with DIPNECH after being treated for asthma for many years. They are being treated at the hospital in Forli in Italy. I have seen that there are many patients who were teaching (or former teachers). Can you think there may be a link between the chalk dust and disease?
thanks

Gail said...

So glad to find this blog! I don't use FaceBook so I hope the DIPNECH blog is still being checked from time to time.

I'm not diagnosed yet, hope to go to Jewish National soon. All the typical symptoms (also a non-smoker) have been with me for years. I was diagnosed with bronchiectasis in '06 for lack of what to call all the gunk found on CT. I also have elevated ACTH, elevated Cortisol, and elevated Chromogranin A. Plus diabetes, high blood pressure, facial flushing, hypothyroid, diarrhea that lasts for months. It's looking like I might have some ACTH secreting cells which is causing ACTH dependent Cushing's Syndrome, and that I might also have Carcinoid Syndrome. I've gotten the run around from doctors pretty much all my life.

I'm also hoping to get in touch with Helen (from Portland) who has posted several times here on this blog. Helen could you share with me who is your doctor there in Portland? I live in Mexico but I fly to Portland about twice a year to visit my daughter and I'd like to see if I could get my foot in the door with whoever is treating you there. First I'll get myself to Jewish National and hopefully they will nail down a diagnosis or two, but if I need follow up care or a biopsy, it's easier (and far more fun) for me to stay with my daughter in Portland, than alone in a hotel room in Denver, so I'm hoping to hear back from you Helen, and I'm also hoping to hear that you are doing well. ~Gail

Gail said...

I forgot to mention that we've been living at 8000 feet since Jan 2006 and I do think it's had an adverse affect on the lungs and the health in general, though I've had respiratory problems since childhood--repeated bouts of "croup," bronchitis, pneumonia, strep throat, etc. I've been on bipap since 2008 for sleep apnea. I noticed an improvement in my O2 sats when I started taking Cytomel (a T3 only thyroid medication) for hypothyroidism. Financially, at this point in time, it's not an option for us to move down to a lower altitude, although I wonder how much longer I can hold out up here.

Robert Cobb said...

I'm a 50 yom who for the last 5 years has had DIPNECH.and as with everyone else the 12 years prior was a hit or miss asthma treatment ,but after an open lung biop I got on the right track ,I use c-pap at night and have lost 48 % of my lungs. the inhalers have slowed the progression down but it hasn't stoped .I use advair & spriva but due to insurance cuts I now have to switch to symbacort,my cough is dry and non-productive and is ALWAYS there hot cold dry or wet weather .my O2 sats always run around 89% unless I'm doing something physcial then I go down to around 82% .I still work my full time job in the ER but I have noticed tasks are getting harder every year,wish som breakthru would happen soon.Thanks and god bless you all.

RDC@utmb

Jennifer Curry said...

I'm Jennifer & 36. Jan 2014 I was diagnosed with DIPNECH at the University of Washington Medical Center. I was born 3 mo premature and have bronchial pulmonary dysplasia (formerly, chronic lung disease of infancy) & required long term ventilator use in the NICU. I had an uneventful non-sickly childhood & young adulthood. My symptoms are mainly chronic fatigue (which my doctor believes is an autoimmune issue, such as lupus or RA). I have shortness of breath with exertion. My oxygen levels can drop to mid 80's just walking and am on oxygen for a large part of the day. I also have been found to have bronchiolitis Obliterates (sp?) and multiple lung nodules less than 4 mm. I have wheezing often. I just received my requested records and was shocked. As for hormones and women-specific mysteries, my first pregnancy (at age 19) I developed varicose veins and DVT after delivery and my only son is now 17 years old. My second pregnancy in early 2010 ended in miscarriage at about 5 weeks; My recent pregnancy in Feb 2013 had to be terminated due to severe breathing difficulties (thought to be pregnancy hormone-caused) and vascular phlebitis; during this pregnancy I was diagnosed with severe emphysema, COPD, and evidence of pulmonary fibrosis. I asked for a second opinion since my premature birth raised many questions for me and glad I did. I also have increasingly significant gastro issues, such as chronic constipation. I have had hyperhidrosis and acne, both controlled but are regulated by hormones. I first had breathing problems in my 20's in college; SOB and wheezing. At age 27 I ended up in the ER. I was told by my then-PCP "nobody gets lung diseases in their 20's" and diagnosed with anxiety, despite doing horrible on my breathing tests. I was skeptical. My psychologist refused to refer me for further lung function testing upon my concerns for emphysema even after explaining I was a former smoker and preemie. After my DIPNECH diagnosis, I was relieved I was not crazy. I worked for two and a half years as an office manager before my career was cut short due to increasing medical mysteries and I had to resign. I now do not work nor volunteer, mainly due to exhaustion. Supplemental oxygen is a task; not tripping and carrying such heavy equipment is a chore. I have a chronic cough. Also GERD, although I do not have symptoms. My doctor is pretty sure this has a lot to do with me being born premature as well as an autoimmune component such as RA or Lupus. Thanks for this blog. I wanted to introduce myself, join the small crowd, and share/confirm that DIPNECH in my experience has characteristics that all have been mentioned. I may be the exception and a variation to the norm; a former (short-term) smoker, I am under 40, my disease is not stable-my oxygen levels decreased (desaturated) over the past year to the point where I need oxygen just walking, and I was born pre-term. However, share the qualities such as cough, female, possible hormone related, lung nodules, and this is very rare and hard to find info on. I can only hope my disease course stabilizes at some point. This disease(s)really has taken my life away.I start pulmonary rehab next week. Take care and sorry this is a dissertation. Good luck to all.

Lynn55 said...

Hi Everyone, I haven't heard from anyone in a long time. I guess we are all just busy and dealing with life. Has anyone heard of any updates for this disease? I am still working full time and dealing with other issues now.

laura said...

Hello Lynn55 and everyone else. I have not posted in a while due to the fact that my little sister who has DIPNECH, has lived past her 3year diag. My sisters situation is much different than you and others due to the fact that she had a wedge removed from her lung, which brought on a whole differnt situation. I wish i could offer more info, but after her taking "trial medication" she got much worse. I do believe that most of all DIPNECH diag. people can live a somewhat productive life, but she can't be compared to most of you. She is going on year #5, and is stuborn as heck. i know this doesn't help, but in the realm of things and much research on my part since 2009, if one had to choose a disease/cancer..........this would be the one i would want to have because it is such a slow growing disease. I hate to say such a thing, but after living through this as a caregiver and a soulmate, its not the disease that is killing her, it was the clinical trial that is. Hope all is well, and any new things/ideas are always welcome........we still have hope for something....