CureTBM. All rights reserved. 

Looking for support? Have questions? Join us on Facebook

  • Facebook Social Icon
Search
  • Cure TBM

Maryalice's Story

Updated: Aug 21, 2019



TBP via robotic surgery on 10/19/18 at NYPresbyterian/Weill Cornell Campus NY, NY


Eugene Shostak, Interventional Pulmonologist and Benjamin Lee, Thoracic Surgeon


Until I was in my mid 40’s, I felt I was relatively healthy, working full time as an RN and generally not paying that close attention to my own health as I was busy living life, working, raising a family. As a child I frequently had ear infections, sinus infections and colds that often became bronchitis, but I was never diagnosed with asthma or any other serious respiratory issues. I do recall being the child who, if I had a cold, it was sure to become bronchitis and then pneumonia.


I began to have frequent and serious sinus, ear and respiratory infections, including pneumonia when in my 40s. On one occasion, I developed a common swimmers’s ear while on a beach vacation. Within hours, I became septic. This prompted me to look further into why I was becoming very ill so often. I had sinus infections that seemed to last for months. I found and consulted a “sinus guru” whose specialty is sinus treatment and is board certified in Allergy/Immunology. He also referred me for sinus surgery, actually a total of 3 surgeries. Due to the type of cultures that they found, they had strong suspicions that my immune system was involved because the type of infections I was having are opportunistic, or more common in patients who are on chemo, have cystic fibrosis or are otherwise immune compromised.


This led to a series of studies that confirmed a Primary Immune Deficiency. This means that I do not make antibodies to fight off bacterial or fungal infections. I do not hold a response to most vaccines, because I do not make the necessary antibodies. As a result, I began treatment with IV Immunoglobulin(IVIG) , every 3 weeks, which is essentially antibody-rich plasma collected from donations. Other people’s immunity is transferred to me. The treatment is very effective, but I am still prone to respiratory infections as well as opportunistic infections that donors have not developed antibodies against.

Somewhere along the way I was told that I have asthma as well, so was started on oral and inhaled meds as well as prednisone when needed. I was fairly stable on this regime until around my 60thbirthday when I developed a pneumonia that did not seem to respond to antibiotics. It was a difficult recovery and I never really felt like I was over it. I was fatigued, wheezing, had a terrible cough and shortness of breath with very little exertion. Red Flag #1. The chest x-ray and cultures were coming back negative, or free of infection, but I was still wheezing, coughing and generally not breathing well.


I was then referred to a local pulmonologist who tried yet another round of steroids and antibiotics to no avail. I was finally referred to the Pulmonary Division of a regional Medical College. On the first visit I was seen by a team including an Attending, a third year Resident and a second year Fellow in Pulmonolgy. They all told me that my history was unusual in that asthma does not usually first present in adulthood. It will sometimes return after childhood asthma but they all agreed that it didn’t seem likely that I had asthma. This was Red Flag #2. I then had my first bronchoscopy and was told that it appeared that I had severe infection, however all of the cultures taken during that procedure came back negative. The bronch report stated “significant dynamic airway compression was noted.” Red Flag #3


At that time, I did not realize that this was significant, and the Pulmonary Team never mentioned it either. Chest x-rays and CT scans were unremarkable. Pulmonary Function Tests consistently showed restrictive airway problems. Red Flag #4. I remained under their care for almost two years. During that time I became sicker, wheezing was constant and the frequent very productive cough was alarming. Worse, I began to have episodes of nearly passing out during coughing, waking at night choking and gurgling and feeling as though I was suffocating. The amount of sputum I was creating and coughing out was unbelievable, I had to travel around with a roll of paper towels and a trash bag. As a result, I became more and more homebound. I honestly felt like I was circling the drain.


I was seen in the Emergency Department several times for acute shortness of breath and chest pain and had numerous tests, xrays, CT scans, MRIs, an angiogram and even a cardiac catheterization which was negative.


Finally, after 2 years of constant medication changes, increased steroids and trials including a new injectable biologic, with no improvement, the Pulmonary Team at that Medical College sat with my husband and I and said that at this point, they had nothing left to try. They said I was the most difficult to treat patient on their service because they have thrown everything they could at me but nothing seemed to make a difference. The only thing they could suggest was to try a procedure called Bronchial Thermoplasty, which they do not do there nor did they even know who to refer me to. They were unable to explain why it might be helpful, other than to say that nothing else has worked and I was getting worse. I had very little endurance and had begun to experience more frequent episodes of being unable to breathe and feeling like I was suffocating to death.


It took about a week of internet searches and phone calls to finally find an Interventional Pulmonologist who had the capacity to do Bronchial Thermoplasty. I explained to the office staff what was happening and I received a call back the same day to come down the next day to be evaluated by Dr. Eugene Shostak, an Interventional Pulmonologist on the Cardiothoracic Surgery Team of NY Presbyterian/Weill Cornell Campus in New York City. Dr. Shostak listened to my story, reviewed my records and said he had an idea about what could be happening so immediately scheduled a bronchoscopy within the week. He explained that this would be a little different than other bronchoscopies in that I would have to be somewhat awake in order to follow directions. I was so desperate at this point that I would have agreed to stand on my head while being scoped.


After the procedure, Dr. Shostak came in and told my husband, daughter and I that the news was good and bad. He explained that he found what he had suspected when first reviewing my case, a condition called Tracheobronchial Malacia, a severe collapse of my trachea and my main stem bronchi. He said surprisingly, my lungs themselves looked pretty good. The good news was that it is surgically repairable. I had actually never heard of this, but was strangely relieved and even happy that something was actually found to explain what I was going through. Dr. Shostak scheduled another bronch for a few weeks later for what he described as a stent trial; he wanted to place temporary stents to hold my airway open and if they were helpful, then it is likely that surgery will help. In between appointments, of course I had to Google TBM and found some great information on CureTBM.org. The surgery, as described by BIDMC, seemed very frightening, but I tried to take one day at a time. When I returned to NY for the stent trial, I shared with Dr.Shostak some of the information that I had read and he told me that he had done his advanced training (a Fellowship in Interventional Pulmonology) in Boston with the team at BIDMC, and he knew exactly what I had read on the site, so I felt a little better. I had the stents placed and stayed over one night on the Cardio-Thoracic step down unit, which is the only unit this team admits to, besides the Pulmonary ICU. The nursing care was outstanding and gave me confidence that if I did need surgery, I trusted them here. The stent trial was amazing! I immediately stopped wheezing and gurgling and coughing. I walked without nearly passing out, I raked the yard and felt like a miracle had happened. I honestly did not want to go back and have them removed, but he was adamant that they were only temporary and I am too high risk for infections and complications. When I returned a week later to have the stents removed, Dr. Shostak told me that one of his colleagues on his team does robotic surgery and they had discussed my case and felt I would be a good candidate. Although I had not heard of this surgery being done robotically, it sounded so much less invasive to me that I immediately said sign me up! Dr. Benjamin Lee came to see me that day and explained that although this was not always the procedure for TBP, there has been success using the DaVinci robot. He explained that he and the other surgeons on his team also do the traditional open thoracotomy, and I would have to sign consent for that in case they felt that was necessary during the procedure, but he seemed quite confident that he could do this repair robotically.


Within six weeks of my first consult with this team, I was scheduled for the surgery. I first had several tests to rule out GERD and vocal cord involvement and I started using CPAP at night to keep my airways open.


The surgery itself took about 5 hours and I woke up with a chest tube, a foley catheter, an epidural for pain control, an arterial line, oxygen and several IVs. The nursing care was top-notch, I was out of bed to a chair the next morning and I was able to be rid of the epidural, art line, the chest tube and the bladder cath later that afternoon. I actually had very little pain; most of the discomfort was around the chest tube site when I moved around. I was able to be “free range”, walking around the unit as soon as all the tubes were gone. The original plan was to be discharged on day 3, but I began wheezing again on the second day and began coughing more frequently as well, so Dr. Shostak decided to do another bronchoscopy on the evening of day 3, he said he washed out “a lot of debris” and after that my breathing immediately cleared up. I was discharged on day 4 post-op.


Now it is less than one year out and I am breathing freely, have been off most of the asthma meds and have totally tapered off the dreaded prednisone. I have had two episodes of pneumonia since the surgery, but that is not unusual for me given the Primary Immune Deficiency. Despite the pneumonias, which both were cleared with oral antibiotics and did not require hospitalization, I did not have a recurrence of the severe breathing difficulty and I did not become steroid dependant once again. I am back to my previous level of activity and very grateful that I have been treated. I really do not think I would still be alive if I continued on that downward spiral.


#patientstory #adultpatient

124 views