Category Archives: Living With IPF

At Home After the Diagnosis — Learning About IPF

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Dr. Smith, my pulmonologist, diagnosed my condition as idiopathic pulmonary fibrosis (IPF). She told me that it is an incurable, progressive disease. A word she did not use was ‘terminal’ but the words she did use, combined with her attitude and sympathy, told me that IPF is terminal, i.e. life ending. Generally a person is not considered as ‘terminal’ unless the life expectancy is less than six months. So I suppose I should say that I have a chronic, life-ending illness but I (apparently) am not yet terminally ill. But that doesn’t give me much comfort.

I hadn’t heard of IPF before Dr. Smith named it as my probable diagnosis. It was easy to get a basic understanding of the disease because it’s name tells what it is. Some diseases have names which offer no clue as to the nature of the illness, e.g. Hansen’s Disease or anthrax. Other disease names give you some indication of the symptoms of the disease, e.g. scarlet fever. But IPF is in that group of diseases where the name provides a good definition:

  • idiopathic: of unknown origin or cause
  • pulmonary: of or pertaining to the lungs
  • fibrosis: buildup of fibrous connective (i.e. scar) tissue

So there we have it: a disease of unknown origin in which excess (unneeded) scar tissue builds up in the lungs. That’s all I know when I leave Dr. Smith’s office so when I return home I hit the computer and ask my friend Google to find me more information.

I skim a few sites which repeat what I already know: unknown origin, no cure, progressive. Then I learn some new things. IPF is included in a disease group called ILD or interstitial lung disease; there are more than 200 different disorders classified as ILDs. IPF is considered a rare disease. “Currently, more than 80,000 adults in the United States have IPF, and more than 30,000 new cases are diagnosed each year.”1 80,000 adults is not enough to populate even a small city. It is only about 3 ten-thousandths (or .03 percent) of the US population (more than 300 million).

The body builds scar (fibrous) tissue as part of the healing process. Fibrous tissue is different from the normal tissue — that’s why scars on the skin are so visible. The fibrous tissue looks different and has a different structure and different properties. In IPF fibrous tissue is applied to the the lung tissue even where it isn’t needed. It’s as though the body has been fooled into thinking that the whole lung has been damaged and needs to be repaired with scar tissue. The fibrous tissue has two effects in the lungs. First, because it isn’t normal lung tissue, it hinders the movement of oxygen from air into the blood stream. Second, because it is less flexible than normal lung tissue, it causes the lungs to be stiffer so they don’t expand to the same extent as healthy lungs. The stiff fibrous tissue causes the lungs to make a characteristic sound like Velcro being pulled apart. One can listen to the lung sounds at the Insights in IPF web site.

Several of the items I find just repeat the above information but then I encounter a journal article which stops me cold when I read the second sentence of the opening summary: “The median survival of patients with IPF is only 2 to 3 years” from diagnosis.2 Two to three years? Two to three years! That is not nearly enough time to finish living. The fibrosis was first noted on a CT scan in April 2015 and the radiologist suggested the diagnosis at that time. That was a little over a year ago, so if that counts as my time of diagnosis then my expectancy is a bit less than two years. I sit stunned and stare at the computer screen. I read and re-read the sentence which is not just a grammatical construction but is also a sentence — my life sentence. The article does note that some patients live “much longer”. However, since the statistic is the median, that also means that as many patients don’t live nearly that long. I may not be terminally ill yet, but the odds are that I soon will be.

This is difficult news. It is harsh. A cold hand has grasped my vital organs and is squeezing. I’m in a state of mild shock for some time. I’m numb to my surroundings as I try to grasp this information and assimilate it. It is all I think about for a couple of hours, and then is the main thing I think of until going to bed. My mind turns to something else for a while but is brought back to the statistic — two to three years from diagnosis. I have clear memories of two to three years ago — it wasn’t that far in the past — and two to three years in the future seems far too brief. And the time may be less; it could be more but it may be less. In bed the statistic fills my thoughts again, and I toss and turn until I finally succumb to a fitful asleep. There is no rest or comfort here.

Footnotes

1. Idiopathic Pulmonary Fibrosis, Cleveland Clinic.
2. Brett Ley, Harold R. Collard and Talmadge E. King Jr., Clinical Course and Prediction of Survival in Idiopathic Pulmonary Fibrosis, American Journal of Respiratory and Critical Care Medicine, Vol. 183, No. 4 (Feb 15, 2011).

2016.04.11: Starting Supplemental O2

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Today the durable medical equipment (DME) company delivered an oxygen concentrator, several small tanks, and a large emergency tank. Today I begin using supplemental oxygen.

At my last clinic visit I told Dr. Smith that when resting I feel okay but when I go up stairs or walk to the park at the end of the block I start breathing very heavily. She called the nurse who administered a standard six minute walk test. This is a simple test. The nurse puts a pulse oximeter on my finger, looks at her watch, and says, “Let’s start walking.” We walk at a brisk pace through the halls to the exterior wall of the building, turn around and head back. Then we do it again without stopping. Every twenty to thirty seconds she tells me to check the reading on the pulse oximeter.

pulse-ox-1936588_640The pulse oximeter is an interesting little device. I’ve seen them before as they are widely used in hospitals and doctor offices but I’m still amazed by this bit of technology. It is simple and complex. It clips on the finger. A light shines through the fingertip and a sensor on the other side of the finger measures the light. The device indirectly measures the oxygen saturation of hemoglobin in the blood. A microprocessor determines the percentage of hemoglobin molecules which have bound oxygen. And it does this by measuring the small change in a beam of light. Amazing. And much less painful than having a needle suck into an artery to obtain a blood sample — the other (and more accurate) way to determine oxygen saturation.

The pulse oximeter reads 96 when we begin walking — an at-rest reading for a healthy person would be 98 or 99 so I begin below the norm. We walk and the reading drops: 94, 92, 91. We pass by Dr. Smith who asks, “How’s it going?” “He’s going to be on oxygen,” the nurse replies, “The reading is going down quickly.” It is dropping more slowly but continues to decline: 90, 89, 88. I’m breathing heavily when the nurse says to stop. It’s not yet six minutes and I’ve hit the point which indicates that supplemental oxygen is needed. I want to sit down but there’s no chair there. “Just breathe deeply and slowly, and you’ll recover soon,” the nurse tells me. She’s right and before long I feel like I can walk back to the exam room.

Dr. Smith is waiting. “Was it hard?” she asks. “Harder than I expected,” I tell her. She’s written a prescription for oxygen at two liters per minute (O2 at 2 lpm). She says I probably won’t need it when I’m sitting reading or watching TV but to make sure I use it when walking or engaging in other activity. She also tells me to check my O2 saturation from time to time, especially when active, to make sure I’m getting the oxygen my body needs. “Try to keep it above 95,” she instructs.

Philips_Respironics_Oxygen_Concentrator._Photo_taken_by_me_on_April_24th,_2012The supplemental oxygen order was processed and today the DME company arrives. The primary piece of equipment is a home oxygen concentrator. This device draws in air from the room, extracts most of the nitrogen (the earth’s atmosphere is about 78% nitrogen and 21% oxygen) leaving “air” with a high concentration (90 to 95%) of oxygen for breathing. The mostly-oxygen air is sent through a tube which connects to a nasal cannula. I have forty-five feet of tubing which is sufficient to allow me go to any place in my house or out on the front or back porch. The cannula has two prongs which are inserted into the nostrils. It feels odd when I first put the cannula in place. “You’ll get used to it,” the technician tells me. He shows me how to adjust the flow on the concentrator and explains the function of the switch and indicator lights. The oxygen passes through a bottle of water to add humidity before going out through the tubing. Water (distilled — no other kind) has to be added to the bottle about every other day. Otherwise there’s not much for the user to do: turn the machine on and clean the exterior from time to time is pretty much it.

The technician shows me a large tank of oxygen for emergency use if the concentrator fails or if the electricity goes off (thus stopping the concentrator). He shows me the small portable tanks and takes me through hooking up the regulator, opening the valve on the tank, and setting the regulator. A carrying bag for the portable tank is included. The tank and regulator weigh about five pounds — not heavy but noticeable.

I now have a tether at home, fifty-five feet of plastic tubing tying me to a machine which provides what I need to function (almost) normally. When outside of the house I have a constant companion — a small green cylinder of vital gas. I am grateful that the supplemental oxygen should make me feel better and will reduce the shortness of breath. But I don’t like this very visible announcement of my disease, this evidence that my body is failing.