Is This Progress?

ImageIs this progression or an infection?  A reaction to the weather or some other episode? These are the questions I ask every time my cough gets worse or I have more shortness of breath, or my sats just won’t stay up.  They’re the same questions everyone with PF asks. Because this is a ninja disease.  You may be stable for months, years even, and then BAM!  Without warning, your condition worsens almost overnight.

That’s what I’m wrestling with right now.  Is this progression, or just another infection?  I was in the hospital with bacterial pneumonia in early July — 6 days, including 2 in ICU.  But I responded quickly to treatment and came home almost at baseline.  Almost, but not quite.  I had been resting on 6 LPM and found I was more comfortable at 7 LPM.  And before the infection, I’d been moving on 10 LPM, but after I definitely needed 15 LPM.  My doc told me not to worry; it takes a long time to fully recover from pneumonia, and since this was my third bout, it would take longer still.

I settled in for a quiet summer of resting and steering clear of potential infection sources (i.e. crowds!)  But I still felt like I just wasn’t getting better.  We played with my prednisone but even that didn’t help.  Then, two weeks ago, I was having trouble making it down the hallway without getting short of breath. My sats weren’t holding, either, so I kicked up my oxygen another notch — 8 LPM.  When I woke up with a productive cough (my cough is ALWAYS dry), I called my doc.  He put me on oral antibiotics and bumped up my prednisone again.  After a week of antiobiotics, I wasn’t coughing up gunk anymore, but I still felt lousy.  And my sats were falling again.

As it was, I had maxed out the oxygen system I had available at home.  My 10 liter concentrator only yields 10 LPM if I use a 7 ft. hose — that’s the same length you use with a portable tank.  A 25 ft. hose — which allows me to move around my bedroom and to the bathroom — yields 8 LPM.  My portable liquid tank goes to 15 LPM, which means that anytime I want to move more than a few steps, I have to use my portable.  I talked to my doc and we added a second 10 liter concentrator.  They are Y’d together and with a 25 ft. hose, I get 16 LPM.  That’s perfect right now — sitting on 16, I can keep  my sats right around 90%.  I still need to use my portables if I want to go anywhere else in the house.  I’ve Y’d two of them together to give me 20 LPM so I can make it out to the kitchen to eat dinner with the family.  The problem is, at these high flow rates, even liquid portables don’t last long — maybe half an hour at 15 LPM.  I can probably get 45 minutes to an hour when I Y two together (10 LPM each for a total of 20).  That means excursions beyond home are impossible.  I can do doctor visits, but lunch with friends is off the table.



One of the big side effects of pumping such high O2 levels is nose bleeds, lots and lots of nose bleeds.  Even with a bubbler on the concentrator, my sinuses are parched.  I use Ayr gel and Ayr saline spray throughout the day, which help.  But the assault on my mucous membranes is vicious.  My doctor prescribed a new machine called the MyAirvo to help with this.  I was on one in the hospital (where it’s called OptiFlow).  It humidifies and heats the oxygen, so it is much easier to breathe.  It’s also quieter because you don’t get the “rushing air” sound through the cannula.  On the downside, however, the tubing is only 3 ft. long, so I have to switch off to a portable to do anything.  And I will need to add a third concentrator to get enough oxygen flow to keep my sats up.  Right now, when I use the MyAirvo, I have to Y in a 3 LPM from my liquid to keep me sats even close to 90%.  And then there’s the machine rental itself.  It’s an expensive piece of equipment, and it’s so new that most insurance companies (including mine) don’t cover it yet.  So I’m deciding whether this is right for me just yet.

When I look at my oxygen numbers, they just seem ridiculous.  From what I’ve seen, most people don’t use oxygen at this level until the bitter end.  But I’ve always seemed to require higher O2, and my body still feels like it’s raring to go (okay, not raring, but eager).  I’ve had a couple of crazy instances where my liquid ran out and walked 20 feet or so without O2 — I crumpled on the spot like a marionette without strings.  I was still conscious and talking, and as soon as I got oxygen and brought my sats up, I was up walking again like nothing happened.  At least for now, I’m only limited by the Os I can get up my nose.  I pray the body hangs in there with me.


How It All Started

  Over the past few weeks, I’ve met several wonderful people who’ve shared their IPF stories with me.  I thought I’d add my own here, in the hopes that it might help someone to seek answers earlier than I did — or at least with a lot more gusto!

To really understand my story, we have to go back to 1996 when we first moved to St. Louis.  That year I had five bouts of bronchitis and my first experience with asthma.  St. Louis is notoriously bad for allergy sufferers, and like so many others, I quickly succumbed.  I found an allergist, had a full battery of tests and was sent on my merry way with two inhalers — albuterol and Flovent.  Since my asthma only flared during rigorous activity, I was able to control it pretty easily.

Then in 1999, I once again developed a series of respiratory infections, one after the other.  My regular doctor’s medical secretary decided it was her job to keep nuisance patients at bay, so she refused to give me an appointment.  As I would start to describe my symptoms over the phone, she’d interrupt me and say “Upper respiratory infection, I’ll call in some amoxycillan.”  And so it went for three courses of secretary-prescribed antibiotics over a period of four or five months.

My fourth infection that year was much worse. It hurt to breathe, my chest felt heavy with a rattly productive cough.  I got winded walking up the stairs or talking.  I called one last time to see if I could get an appointment and once again I was stone-walled.  So I went my allergist instead.  He immediately diagnosed me with pneumonia and put me on a short course of prednisone and Zithromax.  I wonder now if this series of infections was the “triggering event” that started the slow and steady progression of my IPF.

From that point on, I settled into a pattern of once-a-year infections, which we knocked out with a course of antibiotics and prednisone.  I used my albuterol inhaler when I exercised, pretty much forgot about the Flovent, and seemed to get along fine for several years.

Then menopause hit and I started to gain weight.  My fitness level declined as well and everything seemed to get harder. In 2008, we took a trip to Denver and I struggled from the moment our plane touched down. The mile-high altitude left me dizzy just walking from the car to our hotel lobby.  When I tried to hike with the family, I ended up sitting on a boulder by the trail, gasping for breath.  I knew I was out of shape, but this just didn’t feel right.

I described what had happened to my doctor during my physical. She told me it was just asthma and I needed to get serious about using my Flovent inhaler.  My asthma  wasn’t that bad, so I pressed her.  She said not to worry, just be diligent with my inhalers.

In 2009, I developed an intermittent dry cough and began buying Halls cough drops in bulk.  I started getting winded walking up even just a single flight of stairs.  Again, I chalked it up to being overweight and out of shape. Over the next year, the cough worsened, becoming my constant nagging companion.  Both my allergist and primary care doctors said my lungs sounded clear with the exception of my annual respiratory infection.  Neither seemed particularly concerned with my shortness of breath.

In February 2011, while on business in Atlanta, I headed out to lunch with one of my longer-legged coworkers.  I found I couldn’t maintain a conversation and keep up with him at the same time.  We climbed a long flight of concrete steps and by the time I got to the top, I was dizzy and gasping.  I lagged several feet behind my him, trying desperately to catch my breath. Finally, I just stopped and doubled over, my chest heaving.  I recovered after a couple of minutes and vowed to avoid stairs from then on.

Between February and July, the dry cough became more insistent and I decided I had to do something about it. I went to my allergist who suspected the cough was a side effect of my blood pressure medicine. He changed my meds and I went back two weeks later, still coughing.  Since my lungs sounded clear and my meds weren’t the problem, he figured acid reflux must be the culprit and prescribed an acid-controller and a new inhaler — Dulera.  As an afterthought, he decided to send me for a chest x-ray. (CXR). Divine Intervention?  I think so.

I went for my x-ray and the doctor called two days later.  It’s never good news when the doc calls. He said it looked like I had interstitial lung disease and waved off my questions, saying he was out of his depth and I needed to see a pulmonologist.

A quick web search showed that interstitial lung disease encompassed some 200 different diseases, ranging from curable to terminal.  I stopped at that point, realizing I needed more answers. I did my due diligence and selected Dr. Daniel Potts as my pulmo.  Divine Providence again — Dr. Potts belongs to the same practice as Dr. Neil Ettinger who is one of the nation’s key researchers in IPF.

Dr. Potts ordered a CT Scan and a massive array of blood tests — 10 tubes!  They checked for everything from Lupus and Rhumatoid Arthritis to fungal infections and a bunch of diseases I’ve never heard of and can’t remember.  The CT showed I have infiltrates throughout both lungs, and the blood tests all came back negative.  The only thing left was a biopsy to confirm what he already suspected.

So now we know.  And I hope anyone who has similar symptoms doesn’t wait as long as I did to find out what’s going on.  The biggest lessons I learned?

1.  You are your best advocate.  I didn’t push hard enough when I felt in my gut something was wrong.  You know your body better than anyone else.  Speak up!

2.  Don’t accept crappy service. You deserve better.  I waited through 4 increasingly severe infections before I sought help from another doctor.  Really?  I wouldn’t have accepted that kind of abuse from a restaurant.  Why did I accept it from my doctor’s staff?

3.  Get your head out of the sand!  My docs didn’t seem worried, so I figured that meant my own nagging concerns were unfounded.  See #1 — if you think something’s wrong, insist they check it out!  Dr. Potts told me about a study that showed the attention span of the average doctor is 17 seconds.  They see dozens of patients every day, so chances are you really don’t have their undivided attention for the 10 or 15 minutes of your appointment.  Keep asking questions until you get the answers you need.

What’s your story?  I’d really like to know.