Pulmonary Fibrosis and Why We Need Prayer

A Snapshot of the Disease

• The disease affects 200,000 Americans; alarmingly prevalence has increased as much as 150% percent since 2001
• The median survival rate is only 2 to 3 years, and more than 2/3 of patients will die within 5 years
• There is no FDA-approved treatment or cure for IPF
• An estimated 40,000 people die each year from IPF – one every 13 minutes (the same number that die annually from breast cancer)
• An estimated 48,000 new cases are diagnosed each year
• IPF can affect anyone, but the disease tends to affect men more than women; the mean age at the time of diagnosis is 60-70 but can occur at any age
• IPF is 5 to 6 times more common than cystic fibrosis, yet it remains virtually unknown to the public, many policymakers, and even some physicians

Those are the facts. It carries a 100% chance of fatality with the exception of those few who are able to get a lung transplant - which is the only known cure. Even after a lung transplant, the average life expectancy is only 5 years. Not a whole lot of hope.

That's the factual side. There is a whole other personal side as well. I live with this, day in and day out. I cannot turn it off or walk away from it. Each day I see it progressively robbing more and more of my life - more and more of me. It started a few years back when I noticed that I seemed to be getting winded much quicker than I ever had before. At first I just chalked it up to being overweight and getting a little older. I mentioned it to my doctor but he didn't seem too concerned, so neither was I. It kept getting worse and somewhere along the way, I picked up a little cough. It wasn't producing anything and it wasn't all the time, but it was there. My doctor showed a little more concern at that time and ordered a chest x-ray. Not particularly liking what he saw, he referred me to a pulmonologist who did a complete work up. My PFT's (pulmonary function tests) were down but nothing too alarming. He instructed me to lose weight and exercise more. So that's what I tried to do. I did lose a significant amount of weight (50 lbs) in a year but still my breathing was rough, so my primary sent me back to the pulmonologist. He again did the tests and they were down a bit more. My logic was that if it was due to weight, shouldn't they have improved or at the very least stayed the same? He did a CT scan but still attributed it to my weight - and not very nicely. Pretty much telling me my breathing problems were all in my head. Needless to say, I left there in tears vowing never to go back.

In the fall of 2008, I was scheduled to have carpal tunnel surgery and was going through the normal pre-op work up when something caught the attention of my doc on the EKG. He sent me for a complete cardio-pulmonary work up before he would sign off on okaying the surgery. Cardio came through fine but we ran into problems with the pulmonary. Ironically enough I had to go back to the office, that I vowed never to go in again, due to time constraints. I didn't have enough time before surgery to find a new pulmonologist. Mercifully I didn't have to see the same doc, but his partner. This time when they did the PFT's they were significantly reduced and everything else was stopped so that they could pursue that. The next few months led me through more CT scans, a bronchoscopy, alveolar lavage, and an open lung biopsy a week before Christmas. It was from that biopsy being sent to Mayo Clinic that I got my diagnosis and was delivered the news of a death sentence.

A month after the biopsy, I was able to finally join my husband in Tennessee and my records were transferred to Vanderbilt. While there is normally an eight to twelve month wait to get in to see them, I was able to see them within two months. For the last two years, they have tried different drugs to see if they could come up with some combination that would stall or slow the progression of the disease down. I have had bad reactions to each and every one and none of them helped with slowing it down. Since they weren't helping and were making me so sick, I've been taken off all but a maintenance dose of prednisone, partly in hopes of getting me ready for a lung transplant.

For the last 6 months, I've been going through testings and appointments to see about qualifying for a lung transplant. Things were moving along pretty well until last month when I lost my insurance because my COBRA ran out. I have automatically been disqualified from pursuing a lung transplant. I'm ok with that though, because I really wasn't sure that was the route I wanted to go anyway. Lung transplantation opens up a whole other can of worms and hardships - that will be another blog itself.

I'm struggling day by day. It's getting harder to breathe and I am having to use oxygen pretty much most of the time. I sleep with it all night, I cannot leave the house without it and whenever I do anything around the house, I have to have it on. If I'm just sitting here quietly on the computer, I try not to put it on, but even then there are times I must. It's hard on Oliver to watch his once very vibrant, active wife, just sit there sometimes gasping for breath. It's hard for me not to panic during those times, knowing that the day will come when I won't be able to recover. It's not so much the dying that scares me, but the suffocating and choking to death that does.

The lack of oxygen flowing through my blood is affecting other parts of my body as well. I have become quite forgetful and that is worrisome to me. I literally can watch something on tv and an hour later not remember watching it. I forget when I've talked to people or when I go into another room, what I went in there for. I feel like that email that's been circulating for years about going to do one thing, getting distracted, etc. until you're right back where you started. That's not so funny to me anymore. My heart is stressed and it's beats are numbered. Sometimes it even hurts. Pain is magnified because the oxygen is not getting around the way it needs to. I sometimes fall asleep mid-sentence in a conversation. Falling asleep in church has become pretty standard. On my chair, daily. Oliver doesn't even try to wake me up anymore, he just turns off the light and covers me up. On the flip side, I have trouble sleeping at night. I can only get a couple of hours in at a time and it's fitful at best. Sometimes I'm just afraid to go to sleep, wondering if I'll wake up. I'm watching my life slip away and there is nothing I can do to stop it. I fight as hard as I can but I'm losing ground.

That's where the prayers come in. Somehow in the midst of all this struggle, I'm able to find peace and that's due to the prayers of friends and family. I know it's too late for a cure to be found in my lifetime. I'm a realist. But I do have a son and grandson that I care deeply for that I would hate to worry about them having to deal with this. I watched my own mom die a slow, painful death of what I think was the same thing I have - our symptoms are the same, but they didn't really know too much about PF back in 1974. It's hard enough to get the diagnosis today. We need God to step in. We need the funding for research so that treatments and ultimately a cure can be found. This deadly disease affects far more than you can imagine - both directly and indirectly. As you pray for me and my family, I too am praying that it does not touch yours but if it does, I pray a cure can be found swiftly so that you don't have to walk in the shoes that I've had to walk in and the ones that so many before me have. If you don't think your prayer can make a difference, think about this... a snowflake all by itself doesn't amount to much of anything, but look what happens when a bunch of them stick together. : ) Thanks and God Bless.