The first memories I have of cystic fibrosis were of my mom telling me, “pack some books for tomorrow, we have to go to Charleston.” We would get up early in the morning, and I would sleep on the way, and would wake up as we were pulling into the parking garage, the only one I had ever seen. Slowly, I realized that we were at a hospital.
Those first pediatricians who saw me are long gone, and probably came with the bricks when they built the medical university. But, they were very passionate and caring physicians. Bedside manner seemed to be a top priority. True CF science was more science fiction at that point. Pancreatic enzymes were really all there was in terms of drugs specifically for CF patients. Everything else available was trying to treat symptoms and palliative care, for lack of a better term.
The last pediatrician that followed me was middle-aged and had a truly warm disposition. He was more gastrointestinally oriented, which worked out well for me since he was the one who discovered my liver disease during my adolescence and got it under control. It is still managed with the medication he recommended more than 20 years ago. He was old school: long white coat, comfortable shoes, khaki pants, and a tie. Imagine Detective Columbo with a white coat. One of the purest people I have ever encountered in the medical field.
One day in the pediatric clinic during the early 90s, he told me that the new adult CF doctor was coming to meet some of the older pediatric patients who would be transitioning soon to adult care. In walks my new CF doctor, fresh on the scene. I immediately noticed the sharp contrast in appearance and projection to my pediatrician. He was a young, spiffy sort, with dark hair and a flair for really nice clothes and loafers. I already didn't like him. Sometimes you don't recognize greatness right away.
Before getting into this any further, I must explain that when it came to daily lung treatments, I was totally noncompliant. My lung function was close to 100 percent, I never had bronchitis or pneumonia, and I was staring down both barrels of a liver transplant, so to me it was a complete waste of time. That being said, there wasn't much available at that time for airway clearance. The Flutter™ had literally just come out, and it did nothing for me. Even the active cycle breathing technique, which was as cutting edge as Orkambi® is today, did not help me. Had my lungs been more involved at the time, I'm sure my parents would have been doing manual chest percussion on me and making me do the other lung treatments.
My new doctor sat down with me and asked how I was doing. I told him fine, to which he replied, “Are you telling the truth?” Then, after a conversation where he actually explained to me -- for the first time -- how the mucus in CF affects the lungs and covers the cilia, etc., he dropped the hammer on me.
“You're doing okay right now, but I am here to tell you that -- eventually -- you WILL end up in the hospital due to lung infection, and you WILL be on IV antibiotics.” This was not the bedside manner to which I was accustomed. It was also the first time I had ever been subjected to the harsh realities of CF.
He continued to visit me on the pediatric side for a quick couple of years, and time came for me to transition to the adult clinic when I was 18 years old. He emphasized that I was an adult now, and asked that my parents remain in the waiting room instead of going into the exam room with me. As harsh as this may sound, I think it is a good practice to draw a line and make patients take ownership of their care because -- past a certain age -- no one can make someone compliant. My mom was very reluctant about this, but she understood. What finally made me compliant was coughing up some mucus with traces of blood in it. Since then, I have been 100 percent compliant with every single treatment recommendation.
I don't know exactly when or how it happened, but after a couple of years, my doctor and I grew used to each other, and he and his nurse coordinator (who has now been with him more than 20 years) became the north star of my health. I have to see other specialists for other things like my liver, but it is all coordinated through my CF care center.
I think my doctor grew to appreciate my compliance with his suggestions, and saw that when he gave me an inch, I would not take a mile. I began to understand his vision and what he was building, and that he was not just a doctor, but an internationally known scientist who took an interest in the patient, not just the illness.
Through the years we have grown to know each other as people, and that has caused our doctor-patient relationship to get stronger. I know that when I email him, I will get a thorough and thoughtful response. I hear of patients who don't have that kind of relationship with their providers, or have to relocate and change clinics, and I feel for them. Worse yet, I know there are some CF patients who don't even go to a CF Foundation-accredited care center, even though they are able.
Change is hard. Growing up is harder. CF is hardest. Sometimes the combination of the three is overwhelming, and it isn't very smooth. But, don't lose hope. Sometimes what may start off as a shock -- or clash of personalities -- will eventually sync. The dunking of ice water that once shook us can also mean victory.
