Written by patient and advocate John Torrence

The Infectious Disease doctor told me that this is an extremely rare condition, yet all of his big words sounded strangely familiar. My mother has had a long history lung issues, so I called her and asked her to describe her symptoms and medications. The more she relays to me, the more I remember about her condition. I remember being at her hospital bedside a few years earlier. At 76, my mom was diagnosed with breast cancer and had to undergo a mastectomy. I stayed with her the whole weekend, and what I remember most was the continual stream of doctors coming into her room changing her prescriptions. The Rheumatoid Arthritis doctor put her on steroids. The Infectious Disease doctor took her off, due to her lung issues... Back and forth.

Mom told me she has MAC, which stands for Mycobacterium Avium Complex, and is the most common form of Nontuberculous Mycobacterium (NTM). I remember her joking about it being closely related to Leprosy! Now that's something that should get your attention. It turns out, the sturcture of the bacteria NTM, TB and Leprosy all have the same triple wall around the cell that protect them and make them very difficult to treat. So, structurally the three bacteria are similar, but their effects on the body are different. After we finished joking about my mom having Leprosy, she also mentioned that she has something called Bronchiectasis. In three years of going to doctors, I have not heard my doctors use that term, but I planned to find out what it was.

At my next Pulmonology appointment, I reported to the doctor everything my mom had told me. He seemed unimpressed that my closest living relative has the same rare disease that I have. I mentioned Bronchiectasis, and I asked if I have that as well, and if NTM and bronchiectasis were connected, but I didn’t get much helpful information from him. I did notice, however, that my after-visit report from him indicated that I had "Acute Exacerbations of Bronchiectasis.” I am not sure if he indicated this on the report because I had mentioned bronchiectasis or because I was indeed experiencing an exacerbation of bronchiectasis. I wasn’t sure what an acute exacerbation was, but it seemed like if I was experiencing one, he should have noticed long before I brought it to his attention. I started to become concerned about the doctor’s knowledge of my condition(s).

The Infectious Disease (ID) doctor has an entirely different bedside manner than the pulmonologist. Rather than bury his head in the computer through my entire appointment, the ID doc wass Mr. Personality, took no notes during my appointment, and could rattle off rare diseases like they were his favorite pastime. He ggave me my first explanation of Bronchiectasis, and how it relates to NTM. My head was spinning by this point.

During this process, we were living in Maui, which as you may know, is a very small Hawaiian island in the middle of a very large (Pacific) ocean. Maui has a hospital and several clinics, but it does not have all those big city services that are needed to manage a very rare disease. So often times, I found myself having to travel by plane (to Oahu) to get some of the more technical services, such as the PET scan. This meant that appointments that would normally take an hour or so, actually required a full day by the time I got the the airport, flew over, took a shuttle, and reversed the process to get back home. Luckily, my health insurance covered all these transportation costs and made all of the arrangments.

Now on to the fun stuff. The ID doc set up a "cocktail" of antibiotics to fight the Mycobacterium abscessus. Two of the three antibiotics (Tigecyclene and Amikacin) were intravenous (IV), and one (Azythromycin) was oral. The idea for this regimen was to hit the bacteria so hard and so long that it cannot survive. The bacteria is known to morph around antibiotics, so delivering three drugs at the same time keeps the bacteria guessing. To administer the IV drugs, I had to go back to Oahu and get a Peripherally Inserted Central Catheter, also known as a ‘PICC line’. The PICC line is a tube that is positioned on my arm and is connected to a large vein that carries blood into my heart. The plan was to drip hard core drugs directly into my bloodstream, which will deliver the medicine to my lungs. I got my very own (loaner) IV machine to take home, and I had to learn how to operate it, and how to dose the IV medicine. The ID doctor told me that Abscessus is a particularly difficult strain of bacteria to fight, and that it would take 18 to 24 months of this drug ‘cocktail’… and that I may also need surgery.

SURGERY?!?!?! What did I do to deserve that comment? Apparently, Bronchiectasis is a permanent dilation/widening of some of the airways in the lungs. The widened airways do not clear out mucus, so they become great places for the bacteria to grow and hide. While the drugs may kill the existing infection, I was likely to get the same infection again without surgery. All of this because of the Bronchiectasis, which, as you may remember, I had to convince the Pulmonologist that I had!

The ID Doctor said that while he is confident of what I have, he was not confident on how best to proceed. He said that he had only seen this infection three times in his career (he appears to be in his 50s). He recommended that I immediately start working on getting a referral to National Jewish Health in Denver, CO, as they are the experts and will know exactly what to do...

John will continue sharing his journey with us. The next part of his story will discuss being referred to National Jewish Health. A Weeklong diagnostic routine at NJH reveals he needs surgery at the University of Colorado Hospital…

The COPD Foundation advises that before you make any changes to your medication or therapies that you first consult with your doctor. The COPD Foundation recognizes that everyone’s experience is unique, and this is John’s experience.