@mcohan
Most doctors, even radiologists, pulmonologists and oncologists haven't even heard of DIPNECH. I recently went to a cancer talk and asked the lead thoracic surgeon at the hospital if he was familiar with lung NETs. He said yes. I asked about if he's heard of DIPNECH and he said no. I gave him and overview and asked him to spread the word.

DIPNECH is rare with only a couple hundred diagnosed cases, but they think it's underdiagnosed. Mine went undiagnosed for 12 years after a CT spotted 50+ tumors. At least 20 specialists had incorrect guesses. We monitored with CT scans for awhile and all the tumors were very slow growing. I finally stopped that because of radiation exposure, but then when I was diagnosed with recurrent breast cancer, they really pushed for the lung biopsy. I hadn't let them biopsy for 12 years because I wasn't going to let them cut my chest wide open when a lot of specialists were guessing it was something benign. But after 12 years, they had grown enough that they could do a needle biopsy. After lung NETs was diagnosed from the biopsy, I started reading up on that and couldn't figure out why people usually had one tumor yet I had so many. Through my own research, I came across a Mayo Clinic retro study on DIPNECH patients -- aha! I was sure that was it. My breast oncologist had never heard of it. I asked for a referral to the UCLA NETs multidisciplinary team and they were familiar and confirmed that's what I have in addition to lung NETs.

DIPNECH can also cause hypoxia (low blood oxygen levels). My resting oxygen level based on my pulse oximeter is 92-93%. I only started monitoring when the pandemic hit so it may have been low for years and years. For two months straight recently it was 90-91% with dips to 88-89%, but then went back to 92-93%. Mystery. It's lowest when I'm in the recliner (even just slightly reclined) or in bed. I lied down flat at the pulmonologist's office to show him and yep, 93% just like I said. If I'm sitting in a desk chair, it's 96%. It will go higher if I'm up moving around. So weird and not good for my body or brain, but he said insurance only covers oxygen if lower than 88% even though 95-99% is considered the normal range.

I have CT scans every 6 months to monitor my lung NETs, DIPNECH and advanced breast cancer. I do it all at once to minimize radiation. DIPNECH is very slow growing, but often very symptomatic. DIPNECH is typically diagnosed in middle-aged non-smoking women in their 50s-60s who have a long history of coughing and shortness of breath that has been misdiagnosed as asthma, COPD, etc. Note that I also have had asthma and allergies from the day I was born, but it turns out DIPNECH really added insult to injury. I now know that due to the improvement with the octreotide treatments.

@meryls12
Since Mayo Clinic was the first to do a big study on DIPNECH, that should be a good place to go. Florida is large so I'm not sure how close you are to Mayo. Robert Ramirez DO at Vanderbilt University in Nashville has done more talks on DIPNECH than any other NETs specialist I'm familiar with. If I ever feel I need a second opinion on DIPNECH, he's the one I plan to contact. Right now, I feel like my UCLA NETs team is on top of it. In fact, my IR said he's seen 14 patients with DIPNECH. That's a lot considering how rare it is.

You could probably do a video consult with Dr. Ramirez if you send him your records. The first link is the latest talk on DIPNECH by Robert Ramirez 11 months ago. The second link is was his talk 3 years ago. Very informative. Most NETs talks just mention DIPNECH briefly or not at all.

Note, not all patients with DIPNECH will progress to lung NETs, but it can be a precursor. The rule of thumb for DIPNECH is anything under 5 mm is considered a tumorlet and anything over 5 mm is considered a carcinoid. Most lung NETs patients do not have DIPNECH. Not only do DIPNECH patients have more tumors, but my IR told me it's not the tumors we can see on the scan that cause my respiratory symptoms, but it's the hundreds or thousands of very tiny ones too small to see on a CT scan that line my airways that causes the symptoms. He told me I've likely had it for decades. I had terrible respiratory symptoms for 30 years before octreotide so I know I've had it since my mid-30s or before. My tumors were first discovered at 49 when being treated for bronchitis and diagnosed at 61. Although DIPNECH alone is not cancer, the mere volume of the tumors causes healthy tissue displacement and airway blockage so that's a problem even when it doesn't become cancerous.

You'll hear in the talk that some people can eventually need a lung transplant from blocked airways, but from what I've read, it seems that is not typical. Again, I think it's just starting to get more attention and data because they believe it's underdiagnosed. My IR told me there just isn't enough data on it yet to make good predictions for me, but he expects we'll be long term partners in this. The talks are worth watching if you have or think you have DIPNECH. I hope this helps. There is a new study going on right now though.