Hey guys, I'm a 24 year old from Australia, was diagnosed with Pulmonary Alveolar Proteinosis (autoimmune) at the end of last year, been experiencing the symptoms for about a year. I've already gone through the biopsy and my first complete lung lavage of both sides. Because of the illness I've left my job due to stress and fatigue and now need to find a new career path that's more suiting. Been searching around the internet for a while now to see if there's a place where others in my shoes are having an open discussion and talking about dealing with everyday life, and due to how rare this is, it seems like there's almost no one out there. Wanting to know what success others have had with ongoing lung lavages, oxygen therapy, and GM-CSF spray, and even physiotherapy or finding a right diet (anything that makes a little difference) has it made life easier? At the moment I'm not the clinical trial yet and not on oxygen therapy, although I always feel great on oxygen/just coming out of hospital after the lavage. Wanting to hear from anyone with how they've coped and if there's any other forums/communities where people regularly talk about this.
Ingrid S.
Jun 4, 2019
Discussion
Around June and July of 2017, I started to feel shortness of breath. So, I decided to go to my primary Dr. in August and told her about my symptoms. For some reason I thought that maybe I got pneumonia again. I suffered the same symptoms in 2015 and I was diagnosed with pneumonia. They prescribed me antibiotics and when I was done with the medication, my symptoms went away. This time the x-rays showed pneumonia once again. I was prescribed antibiotics and when I finished, my symptoms were still there. My primary Dr. referred me to see a pulmonary specialist. During this time September 2017, I was in a process of changing insurance through my work, so I couldn’t go as soon as I wanted too. I could finally see a pulmonary Dr. on October 16th 2017 with my new insurance. I explained what I was going through. My oxygen levels were not good at the time of my appointment. At rest I was on the low 80’s. He then told me I couldn’t continue working under those conditions, so he prescribed me supplemental oxygen and I was put off work right away. He then ordered x-rays and a CT-scan to be done. When he saw the results, he told me he suspected I had PAP. On the CT-scan it showed the crazy paving related with PAP. After this I had a lung biopsy on November 9th 2017 and I was finally diagnosed with PAP. Then I had my first set of whole lung lavage on December 11th of 2017 and January 5th of 2018. After the whole lung lavage, I didn’t feel any improvement. The Dr. then decided to send my blood for testing and send it to Cincinnati Children’s Hospital to find out what type of PAP I had, which was primary PAP. He also decided to prescribed me the Leukine for inhalation. During this time of February and March of 2018, I was having issues with which pharmacy was going to provide me the medication. While waiting for the medication to be delivered, my condition got worse. I lost my appetite and suffered weight loss. I didn’t have the energy to do something simple as getting off bed, going to the restroom, brushing my teeth, or walking around the house was an everyday struggle. Getting a bath or getting dressed by myself was something I couldn’t do anymore. I needed my mom’s assistance for almost everything. The constant cough got worse and exhausting. I came to the point of not being able to sleep laying down. I was doing 5 liters during this time, but I felt I needed more than that. Then the medication finally came on the March 27th 2018. During early morning of March 28th 2018, my every day cough kept me awake and while trying to get some sleep, I suddenly felt chest pain and was having trouble breathing. I went to emergency and was treated for a pneumothorax or lung collapse. I stayed for a week under high flow oxygen and the lung collapse was not getting any better. For some reason I got my appetite back and I was craving for food again. I also started the Leukine inhalation at the hospital too. The Dr. said that my only treatment option for my PAP was a lung transplant. When I was told this, a lot of stuff went through my mind, I just couldn’t believe it. Since I was diagnosed with PAP, I was still trying cope with the fact that the disease is a rare one and that the medication might help. Not so sure myself I agreed and the Dr. transferred me to UCLA hospital on April 4th 2018. During my stay at UCLA, I had the opportunity to meet Dr. Tisha Wang. Still under high flow oxygen, she guided me through my recuperation and another whole lung lavage was done. Little by little my lung collapse healed and also my need of oxygen was reduced to regular flow. I was finally discharged from UCLA hospital on April 19th 2018. I was happy to finally go home, still weak and under oxygen, but with the ability to breath much better than before was something I was very thankful. When I was at the hospital I was invited to the PAP Education day at UCLA. I decided to go with my mom and my sister. It was a nice experience to meet people with the same problem as me. We were able to hear interesting stories and struggles of what involves PAP. I felt more hope when I saw the PAP patients without supplemental oxygen. I was the only one with supplemental oxygen. The information that was provided regarding PAP was also helpful, it gave me another perspective of this disease. Also, during the meeting, I met a parent of PAP patient. We shared our stories and it so happened that her daughter was still at the UCLA hospital. We asked him if could go and visit her daughter and he agreed. So, after the meeting we went and we met each other. She was still weak, but she was willing to listen and ask some questions, so we shared some our struggles. Her parents were also happy and thankful to see that I was able to visit them. I was also thankful to have the opportunity to meet her. So far, we’ve in contact through e-mail. As a PAP patient, I had another set of whole lung lavage on May 25th and September 4th 2018 and still inhaling the medication. For my oxygen intake I do 2 to 4 liters. I can tolerate more movement than before, but the shortness of breath still there. I know this is going to be a long journey, but I will trust in God. I am glad that due to the circumstances I was able to meet Dr. Tisha Wang and that there are more people studying this disease and trying to find more treatment options. I know that someday I will be off oxygen. When will that be, I still don’t know. But I have faith that God will guide me until we reach that goal. Thank you very much for giving me the opportunity to share my PAP story.
hi sorry to hear about your disease. i'm a PAP doctor in los angeles and trying to connect patients more. we have had good success with both lung lavages and GM-CSF medication and i encourage you to enroll into a clinical trial if possible. in australia rob stirling seems to be a PAP expert so perhaps try and see him. let us know if we can help.
sorry to hear you have been diagnosed with PAP. i too jave been dianoaed after an open lung biopsy in 2014. im hear in south louisiaba where drs have never had a patient with this. after many stays in the hospital, on stersteriods, antibiotics, nothing was working. my pylmonsry dr reached out to several drs from all around to see how to treat this rsre disease. i had a lung wash on my left aide only for it to be filled back up within minutes. i was an obese 36 yr old fighting for me life . i was on a ventilator and o2 therapy 24 hours a day. i did 90 daya of gmcaf injections faithly at the hoshospital everyday! i started vest therapy and i currently use a cpap at night to keep my chest wall opened. .y lung funcruon has improved but i still have spots that are concerning. i feel great as i have lost weight aftrr going through gastric bypass surgery 3 yrs tomorrow but i still get the symptoms of pap. im waiting on a ct scan to see what will be the next step as far as treatment. having this rare disease, i too had to leave my job and i limit myself around people who have colds, illness etc... ill wear a mask to keep myself protected. by no means has this been easy for myself or my family but my dr has been an angel snd saved my life. she continues to work on getting me in remissions and clearer lungs. never give up!!!
Glad to hear you've had some success even though still suffering with it. Here where I live, its also completely unheard of, and my only surgeon with knowledge and experience with doing lavages has decided he doesn't want to do the procedure anymore, for reasons I'm completely unsure of. Now there's no one else in South Australia with the experience to help me and I've been rejected by the pharmaceutical companies for the GM-CS F treatment due to them deciding that the lung lavages aren't 'treatment failure' for me and other treatment called Rituximab has even less prove of helping me even though I've heard a lot of success with it. So at the moment I'm denied all pharmaceutical treatment and have to find a new person elsewhere to do my lavages, seems like I wont get any support until I can no longer breathe. But mentally I'm doing well and still quite positive about it all, strangely enough.
As frustrating as it can be having this rare disease, i feel good. I hope in the near future you can get the care you need to help you heal. Healthcare all around can be frustraing especialy with denials and no help. Noone knows what its like not to be able to take a breath unless they too suffer with PAP. I wiah you well. Keep posting as i will do the same. I have a ct scan next week and waiting on approval for gmcsf inhaled therapy.