Hello,
I've had about 12 lavages. My first set was done in January 2015. I had a little over a year’s remission before the PAP came back with a vengeance. After each lavage my remission varies. Sometimes I get three weeks. Other times I've gotten about 12 weeks. I started inhaled GM-CSF in February 2017 250MCG once a day. After reading some of the post on here I asked my doctor to increase me to twice a day about two weeks ago. I had a another whole lung lavage November 9th 2017. They can normally do both my lungs in one sitting. This time was pretty rough. Some how my lung was punctured during the procedure. I normally go home the same day. I ended up with a chest tube and staying through the weekend. Over the summer the doctors added IV infusions of rituximab and plasma apheresis. Both things helped, but it just wasn't enough to get the PAP under control. Tomorrow I go back down to Baltimore and they are adding Cybor-D to my treatment. My PAP seems to be pretty aggressive and the doctors are worried about potential scarring. I had to stop working a year ago and now I am on disability. For those of you using GM-CSF twice a day how fast did you notice it working? How do you manage the constant coughing? I can’t even lay down to sleep. Everything makes me cough or feel short of breath. What are your triggers and what changes have you made to avoid them? I feel like i'm fighting a losing battle.
Adrian,
I'm so sorry about your difficulties. I know a another patient who might be able to share some useful information. I'll pass this on to her. Hang in there.
Best,
Judith
(mom to daughter dx at 15 yrs old, 4 lavages, on 250mg gmcsf x 3 years - no side effects, lungs mostly clear, stable disease)
hi im looking for help i live in canada and not many doctors here know about Pap. i been dealing with this since 2006 was my first lung biopsy. they couldnt figure out what lung disease i had. but found protein build up in my lungs. so thry thought i was aspirating my food into my lungs. i finally found an amazing respirologist who took one look at my xrays and said uou have pap. sent me to one of the biggest hospitals in toronto where i had my first lung lavage. was black and blue and was in hospital for 4 days. revovery was brutal. managed a ct scan whivh they said improved so we decided to do the other lung. when i went in 2014 i was not aware that the pap came back with a vengenfe. they preformed the lung lavage managed to get 13.74 letets and manged to break two ribs. was in hospital for three months with other complications. so no more lavages ffor me. i then was in touch with childrens hospital in concinatti who confirmed through a blood test that i tested abnormally high for pap. i have declined in health i have been intubated over 23 times last august i almost lost my life due to a intubation gone wrong they had to do an emergency trecheatomy. which i struggle every day to breath as my pap has gotten worse. i have tried to infussions of rebuxitumb with little help. i dont know what else i can do. looking for help.
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Dear Maria and Adrian,
I am so sorry to hear about your battles with PAP. I hope I can share a few things with you to buoy your spirits and give you some hope. I consider myself a PAP survivor, even though my antibody test shows my PAP is still active, it is held in check with daily 500mcg inhaled dose of Leukine. However, from 2000 to 2006 I was pretty sick. I had 44 lung lavages, sometimes only 4 weeks apart. (Some patients have had hundreds of lavages.) Even after the lavages my O2 SATs were still bad. I had to go on oxygen. I was on continuous oxygen 24/7 for over 4 years. My left lung collapsed after a 3rd exploratory bronchoscopy and spent a week in the hospital. With all the protein in my lungs, I also had multiple infections of pneumonia, tuberculosis, and Norcardiosis.
Those were very dark years. It was difficult to keep going. But, I had (have) a beautiful supporting wife and 2 kids. I couldn't go where the darkness was wanting to take me.
The one upside was I was in the best place in the world to be treated; National Jewish Hospital here in Denver, Colorado by Dr. James Ellis. On the first day of my lavage he would flush out my right lung. I would spend the night in the hospital, and then the next morning he would flush out my left lung. I would go home the evening of the second day with two cleaned out lungs and no problems or complications. In my opinion, Dr. Ellis is the best in the world at performing lavages. He has performed more than any other doctor by far. Dr. Ellis is now with the University of Colorado Medical Center. Contact info for Dr. Ellis, and many other PAP doctors, can be found under the PAP Map link on the PAP Foundation web page.
Back in 2000 they really were not sure how to treat PAP. They started me on daily 250 mcg injections of Leukine. I took them for 8 months with no improvement. After that it was just years of lavages with no improvement. However, after a visit to the Cleveland Clinic and Dr. Mani Kavuru, we decided to restart Leukine, but with daily high dose injections of 1300 mcg. Within months I was seeing marked improvement. The lungs were clearing. However, I developed dangerously high hypertension. So, we stopped the high dose injections and went to lower inhaled doses of Leukine. My hypertension is in check with a daily pill of Lisinopril. The lower dose inhaled Leukine cleared the rest of the protein. (A recent study by Savara Pharmaceuticals showed that healthy, non-PAP, people could inhale up to 600mcg Leukine daily with no adverse side effects.)
Not all PAP patients respond to the same treatment. Some respond to Leukine, some to Cellcept, some to rituximab and plasmapheresis. Some go into remission with no treatment. There are also new drugs and treatment being developed right now.
I am 60 years old now and am living life to the fullest. The PAP does not stop me from doing anything. I only share my story to let you know that there is hope. Don't let the darkness take over! There is Light at the End of the Tunnel!.
Here are some things I would suggest:
If your 02 levels are low (<90), get on supplemental oxygen. Use liquid oxygen portables if you can, as they give you much more portability. I played golf and hunted big game with a liquid O2 portable. This will make you feel better and keep your body from further deterioration. It will also help you stay active.
If you are suffering collapsed lungs, bruising, and cracked ribs from lavages, then you are not seeing the right doctor. Find a PAP doctor that knows how to safely and efficiently do a lavage.
Please visit the PAP Info for Patients link and signup for the clinical trials and PAP registry. And please sign up as a PAP Foundation member so you can get the latest information. Also check out all the PAP doctors and clinics out there.
Most important, know that you are not alone. The PAP Foundation, I, and others can help you. Sometimes just talking to someone who understands can be a big boost. Please feel free to contact me at my private email clanpark@comcast.net where we can exchange phone numbers. I speak with patients all over the country. I, and the other PAP board members and advisors are in contact with some of the best PAP doctors and clinics in the country.
Don't give up! Keep fighting!
Steve Park - PAP Survivor and Vice President for Development, PAP Foundation.