In April 2017, I was hospitalized after having severe shortness of breath and fatigue. The doctors told me I had viral pneumonia (RSV). Since it was viral, there was no treatment and I just needed to “wait it out”. After a month, I was released from the hospital albeit with an oxygen tank following me like a dog. Results from the CT scan showed that I had interstitial lung disease, which comprises of various lung conditions including pulmonary fibrosis. I even joined a pulmonary fibrosis support group online, which was more depressing than supportive. Currently, there is no cure for pulmonary fibrosis, and the two drugs available may only slow down disease progression. My doctor prescribed me one of these medications and I took 9 pills religiously every day for almost 6 months, but my need for oxygen increased and my health was rapidly declining.
I was beginning to lose hope. After getting a 2nd and 3rd opinion, I still did not have a definite diagnosis. My doctors told me that my only hope for survival was a lung transplant so I was transferred to UCLA and admitted to the ICU…That’s when serendipitous things began to happen. First, the doctor on call who happened to be a specialist in pulmonary fibrosis took one look at my CT scan and confidently declared that I did NOT have pulmonary fibrosis. That meant I didn’t need a lung transplant after all!!! My jaw dropped. I couldn’t believe what I was hearing. In fact, the doctor thought the CT scan was more indicative of PAP or pulmonary alveolar proteinosis, a rare lung disease I had never heard of. Then, the second miraculous thing happened....
Dr. Tisha Wang, one of the PAP experts in the nation was also on call that night in the hospital. What were the chances? After a series of tests, she confirmed that I had secondary PAP, due to a medication I was taking, sirolimus. I was happy to finally have a diagnosis and treatment available. That being said, I have been a “professional” patient for nearly half of my life. I was diagnosed with leukemia when I was a sophomore at UCLA when I was 19. I have undergone countless cycles of chemotherapy, total body radiation and a bone marrow transplant, but nothing was as intense as my time in the ICU. The first lavage Dr. Wang performed lasted 5 hours under general anesthesia and I was intubated for 8 days. During this time, I was in a state of emotional turmoil, drifting in and out of consciousness and having vivid hallucinations. Later I would learn that these hallucinations were the side effects of Propofol (AKA the Michael Jackson drug). After the first lavage, I did not know if I could endure another one both physically and emotionally. I would plea with the doctors to spare me the misery and put me out of my suffering. While I convinced most of the doctors to put me on “comfort care”, Dr. Wang never gave up on me. What was most frustrating was the inability to communicate or eat for days. I couldn’t talk obviously because of the breathing tube and felt trapped in my own body with thoughts I couldn’t express. Physically, I was also becoming weaker and weaker, due to muscle atrophy and bedrest. At one point, I remember my hands were so weak they couldn’t hold the weight of my cell phone. One night I remember calling for my mom to help me since my leg had gone “overboard”. My leg had fallen out of the bed, but I was not strong enough to pull it back into bed…Finally, after the 5th lavage, my lungs started showing signs of improvement. The doctors were amazed by my recovery, but I think Dr. Wang always knew in her heart that the lavages and GM-CSF would work. She even brought the doctor who suggested comfort care to visit me to prove that I made it through.
During my hospital stay, the PAP Foundation held its annual Patient Education Day. Since I unable to attend, I sent my dad to record the lecture. Dr. Wang even mentioned me and my PAP story in her talk. In addition, my dad met with other PAP patients and their caregivers. He said that most of the patients no longer required oxygen. One of these patients surprised me with a visit to my hospital room with her mom and sister. Her visit really gave me hope that recovery was possible. We still keep in touch via email and I even saw her once at Dr. Wang’s office during a follow-up visit. It was then I was realized the importance of banding together as a community to support each other since we were all dealing with similar issues.
Eventually I was transferred from the ICU to a regular room, and then was discharged to a rehab hospital, where I continued my recovery: 3 hours of intense physical and occupational therapy every day of the week. I had arrived at rehab at 117 pounds (usually I weigh 140 pounds), just skin and bones, but after a month I had gained some of my muscle back. I was discharged home with those pesky oxygen tanks and GM-CSF.
I am getting stronger every day. In August, I took my first steps unassisted in over 5 months. My need for oxygen has decreased substantially and I hope that one day I will be weaned off of oxygen entirely. Even though I had some very dark days, I consider myself very lucky. Despite fighting tooth and nail every time I needed another lavage, Dr. Wang never gave up on me. Later, she told me that I was one of the sickest patients she had ever treated, but she always had hope I would recover. So I am deeply indebted to Dr. Wang for her tenacity and the risks she took as a doctor to save my life. I am grateful for her research team and the Foundation in their endeavors to find novel treatments for PAP. I wish I could have been there in Kentucky today, but here is my story to remind everyone to not give up, ever!
Leila, 18-year cancer survivor, 10-year bone marrow transplant survivor, and 6-month PAP survivor😊