I’m sitting in the exam room, waiting. Waiting is one of the most pervasive of human experiences. We wait, and we wait for something, so waiting has an air of expectancy. We wait for many things, and what one is waiting for colors the experience. A father-to-be waits for the delivery of a baby; a student waits for the results of an exam; a driver waits for a traffic light to change from red to green. The father-to-be is joyously expectant and probably nervous. The student may dread seeing the test grade. The driver may be bored or antsy depending upon the nature of the trip.

I’m waiting for the physician to enter the exam room. I expect the physician but the physician is just a messenger; what I’m really waiting for is a diagnosis.

I am nervous and feeling some dread because the diagnosis could be bad; but I’m also hopeful that it won’t be — that it will be something easily treated and that I’ll experience a return to health and normal activity.

Dr. Smith is a pulmonologist. I was referred to her by my primary care provider (PCP) and first saw her on February 19 of this year. (This visit is chronicled in an earlier post.) She examined me, ordered a high resolution CT scan (HRCT) and pulmonary function test (PFT), and referred me to a rheumatologist. I have the results from the rheumatologist (no disease or condition identified) and today Dr. Smith will tell me the results of the HRCT and PFT. These test results, along with the information she gathered previously, may lead to a diagnosis. I sincerely hope so; it’s been two years since I first sought medical attention for for my problems. It’s been a long road with many physicians, exams, blood tests, x-rays, and scans.

Dr. Smith enters the room after a shorter wait than I expected.
“Hello, Mr. Franklin. How are you today?”
“I’m nervous.” [slight grin] “How are you?”
“I’m doing well.”

She gives a slight pause with a slightly embarrassed look, as though she should not be feeling well.

She continues. “I have the results of the HRCT and PFT. The PFT shows that you have a definite decline in lung function. The HRCT has more information because it shows the lung tissue.”

Another short pause but no embarrassed look. Is this pause because she doesn’t want to continue? Is she considering what to say?

“The HRCT shows scarring in the lungs, as was reported in the CT scan last year. The HRCT shows more detail, and the scarring is clear.”

A little pause. The pauses are getting to me. I’m waiting, expecting a diagnosis, and the pauses draw out my waiting time and increase my anxiety.

She continues. “I’m sorry, but the HRCT clearly shows idiopathic pulmonary fibrosis (IPF).”

The pause this time is welcome. I need to get hold of this and try to get it solidly in my head before I can listen to whatever else Dr. Smith has to say.

And what she says needs to be heard but is hard to hear. “It’s a progressive disease. There’s no cure, and there’s very little treatment available.”

Another welcome pause. I probably have a “deer in the headlights look.” Or maybe a deer in the sights of a rifle, if the deer could see the rifle.

I look at her. “Last time you mentioned doing a lung biopsy. Should we …”

She shakes her head. “I don’t think a lung biopsy will give us any additional useful information. Let me show you the HRCT scan.” She turns her laptop around and clicks a few times. “Here’s the view of your lungs from the bottom up. See these lines? They are scar tissue. This pattern is called ‘honeycombing’. It is indicative of IPF and is definitive for a diagnosis. Your scan is textbook so I don’t think we need to do more tests; I think the diagnosis is certain. I’m sorry.”

She writes prescriptions for two medications which help my breathing, but she emphasizes that they do not treat IPF; they just help the remaining healthy lung tissue to function well. She says that later we will talk about two drugs which are available to treat IPF. “You need to think things over,” she says.

Yes, I do.

She listens to me. “You are definitely ill,” she says. I am relieved when she says this. For two years I’ve been searching; physicians haven’t found the cause, and sometimes it seems they think I’m malingering. Sometimes so do I. I don’t feel good, but I lack specific symptoms — it’s an overall feeling of sickness with great fatigue. Many times I’ve thought that perhaps I don’t have an illness, that I’m just run down. I’ve had mild depression and I’m not sure whether it’s a result or cause of my fatigue and sick feeling.

My primary care physician (PCP) has referred me to a pulmonologist because of an abnormal finding on a CT scan. This is my first meeting with Dr. Smith, the pulmonologist, and I’m very happy that she is courteous and is taking me seriously.

She listens to me, and I am grateful. Some physicians do not listen, or at least do not listen well. “Tell me everything which is wrong,” she says.

I tell her that I’m unsure which of the various complaints I have are relevant to whatever disease is causing this. She tells me to tell her everything, not to worry about whether it is important. She says that she will sort through my symptoms and the test results to determine what is relevant.

So I tell her everything. It actually isn’t a long list. My chief complaint is fatigue. I am tired most of the time. I sleep a lot and I’m still tired. When I’m not tired I do things — some computer work (how I earn money) or clean house or run errands. But I lack stamina and I tire quickly. I haven’t been able to work an eight-hour day in two to two-and-a-half years. I’m self-employed and work from home so I work for a while, rest or sleep, work for a while, rest or sleep. But even with (sometimes long) breaks I can’t work eight hours in a day. The fatigue is so overwhelming that at times I fall asleep while working at the computer. Watching TV or a movie is impossible because I fall asleep; I’m just too tired to stay awake.

I also feel sick; just a general overall feeling of not being well. I have a persistent dry cough. And I have pain my toes and fingers; it’s a nearly constant burning ache and feels as though it’s in the bones. Sometimes there are very sharp shooting pains in the bones. These don’t last long but they’re quite intense. I have a few other things which I mention but they are secondary to the fatigue, feeling ill, and bone pain.

“Do you get short of breath?” she asks. “No,” I answer. “I breathe hard when engaging in physical activity but after all, I’m 61 years old so that’s to be expected. I’ve never been short of breathe and gasping for air.” I will learn later that I am mistaken: What I have experienced is in fact shortness of breath and it isn’t just because of my age.

Another change which I don’t recognize at the time as a symptom is that my shoes are too tight. They weren’t too tight when I purchased them — in fact they were quite comfortable — but a year later they are tight and there isn’t enough room for my toes. It turns out that my toes are clubbing which causes them to take up more room and make the shoes tight. I had no idea of this at the time and didn’t mention it as a symptom to Dr. Smith. A month later I will learn that this symptom is significant.

Dr. Smith listens carefully, takes notes, and sometimes asks for clarification or additional information. Then she opens the lab results tab on her notebook computer. She starts telling me significant results from my blood tests over the past year. I’m aware of most of these. I worked in a hospital clinical laboratory while in graduate school so I’m familiar with the tests and I’ve reviewed the results as they were posted in my online medical record.

“Your white cell count has been elevated for some time but it hasn’t changed. The pattern doesn’t look like an infection and it isn’t high enough to indicate cancer. Your sed rate is quite elevated as is the C-reactive protein. Most other results are normal or nearly normal so there isn’t a clear indication of any specific disease. Your thyroid hormones and liver enzymes are normal so that rules out thyroid or liver dysfunction.” She changes to the radiology results tab. “The only clear indicator is the abnormal CT scan.”

Months earlier, in fact nearly eighteen months earlier, I experienced bouts of abdominal pain. One Friday night the pain was so bad that I went to the emergency room. The ER physician ordered a cat scan of the abdomen and the scan showed that I had gall stones although my symptoms were not typical for gall stone disease. The scan caught the lower portion of my lungs and the radiologist noted the presence of scarring in the lungs. This is what eventually got me to the pulmonologist. (I’ll give a fuller version of this story in a later post.)

Dr. Smith continues. “The scarring is indicative of pulmonary fibrosis, which is scar tissue in the lungs. It can be caused by many things and I can’t tell you much more until we run additional tests and do further studies. Right now I would state that you pulmonary fibrosis, probably idiopathic pulmonary fibrosis, but we need to investigate further. It may be something else. The underlying cause may be a disease which I don’t treat but I’ll set up referrals and order tests, and we’ll find out what it is.”

“Thank you,” I say. “Thank you.” And it is heartfelt. I’ve been trying to find out what’s wrong with me for two years and I’m not only physically tired from illness, I’m emotionally and mentally drained from pursuing and not getting an answer to the question of what’s wrong.

It is obvious how relieved I am and Dr. Smith says that she will keep me in her care until the cause of my illness is determined. “You have indicators of a long-term inflammatory disease and your illness is probably systemic. Most of the likely candidate illnesses would be treated by a rheumatologist and I’ll set up a referral for you. There are some tests which I know the rheumatologist will want so I’ll order those today and the results will be ready at the time of your appointment. I’m also going to order a high resolution cat scan (HRCT) of your lungs to see what that discloses and a pulmonary function test (PFT) to determine the extent of the damage to your lungs. Does that sound okay?”

Okay? It sounds great to me as this seems to be a plan which will produce results. I simply tell her that it is fine. Now I’ll get some blood drawn, get the HRCT and PFT, and wait for my visit to the rheumatologist.