The Backstory

If I told you my story, you would hear hope that wouldn't let go

And if I told you my story you would hear love that never gave up

And if I told you my story you would hear life, but it wasn't mine

~from My Story by Big Daddy Weave

I started out my day in a familiar place.  My appointment was at 9:45.  I got there at 9:00 and the respiratory therapist called my back about 9:05.  He asked me how long I had to drive to get there, because "this test literally takes five minutes."  He was right.  I was in my car at 9:18.

The tests were simple- I exhaled against pressure and inhaled against pressure. The results of this test will reveal the strength of my respiratory muscles.

When I started writing the blog, I said that I would tell you about my disease.  So for those of you who want the details, here is a simplified explanation- the "backstory," as my grand daughter would say.  Sorry for the diagrams, but I did not want to plagiarize or use copywrighted material- setting a good example for my students, right?

The disease I have is called constrictive bronchiolitis, bronchiolitis obliterans, or obstructive bronchiolitis.  It is an obstructive disease- I can get air in, but not out.  There is a similarly named disease- BOOP (or COP) which is NOT the same at all.  It is a different disease.

When you inhale, air goes down your trachea, or windpipe, into the bronchus, and then to the bronchi of each lung.  These passages have rings of cartilage which keep them open.  From there, air travels through tiny passages known as bronchioles, to the alveoli, where oxygen and carbon dioxide are exchanged.

Each lung has about 30,000 bronchioles, and 300 million alveoli.  Bronchioles are 1 mm in size -a little smaller than the thickness of a dime.  Alveoli are 0.5 mm to 1 mm in diameter.  These structures are tiny, but we have A LOT of them!  The bronchioles do not have rings of cartilage, but are instead lined with smooth muscle and connective tissue.  Therefore, they can contract and expand.  The contraction of bronchioles causes wheezing- bronchodilators such as albuterol are designed to open the bronchioles during times when they are constricted (smoke exposure, asthma attack, etc.).  

I have lupus and Sjogren's, which are autoimmune diseases.  The human body is constantly trying to keep out foreign invaders, such as viruses, cancer cells, and bacteria.  My immune system is confused, and recognizes my own DNA as foreign.  In an effort to attack the DNA, my immune system caused inflammation in the bronchioles.  Autoimmune diseases are also connective tissue diseases and can attack anywhere there is connective tissue- such as the joints, which is common in Rheumatoid arthritis.

In BO, inflammation occurs around the bronchioles, constricting them and making them narrow.  Over time, the inflammation turns to fibrotic scar tissue and this change is irreversible - it is permanent.  

I can get air in, but it is difficult to get air out.  Therefore "old air" is trapped in my lungs, which decreases the space available for new air.  Eventually the exchange of oxygen and carbon dioxide is affected, and the carbon dioxide starts to build up.

I also have NSIP (non specific interstitial pneumonia) but I won't go into that here.

Enough of the boring stuff.  Let me tell you the real story.  In December of 2003, I noticed I was having trouble breathing.  I blamed it on being out of shape, but it progressed.  I had an appointment with my rheumatologist for a checkup on my lupus, and when she listened to my lungs she was concerned.  She told me this was not due to being "out of shape" and that I could have lymphoma.  She ordered breathing tests, a CT, and referred me to a pulmonologist.  The breathing tests showed a severe obstructive deficit.  I had a bronchoscopy, but there was no diagnosis.  For the next nine months I declined and even lost 30 pounds.  I was told I had "idiopathic COPD."  In September I developed a pneumomediastinum (air leak in the center of the chest).  I was hospitalized for a few days, and came to the conclusion that I would die if I did not do something.  

Before I became sick, I had begun a doctoral program.  I had no idea why- I am one of those people who actually likes school and likes a challenge.  The program I was in was very competitive, and there were only about 15 of us in the cohort.  I had access to the medical library at UT and began to research the abnormalities on my CT-"mosaic attenuation" and "ground glass opacities."  All of the radiology articles pointed to autoimmune disease as a common cause of these findings.  The specialists did not believe my lupus was causing this, because the pleura of the lungs was not affected.  I referred myself to the Mayo clinic.

The doctors immediately said "we think you have bronchiolitis obliterans."  They ran some tests and scheduled a lung biopsy.  I had no idea what this disease was, but was relieved that there was an answer.  I did some research and my relief turned to anxiety.  I saw the terms "rare disease" and "often fatal."  It is so aggressive that it is on the compassionate allowance list for disability. There was not much in the literature at that time, and I believe I read every article that was published.

The doctors told me I would probably live 3 years, and guessed at some treatment to suppress my immune system and halt the progression of the disease.  Every time I took that medicine I prayed over it.

On a cold day in January of 2004, I was sitting in a hospital bed at General struggling to breathe more than I ever had before.   It was about two months after my diagnosis; my right lung had collapsed and I had a chest tube.  For the first day or two things were better.  That particular night, my breathing had gotten worse, my saturation was low even on oxygen, but the respiratory therapists and nurses did not seem too worried.  I am not an alarmist, so I was pretty calm, I thought that this must just be expected……..that the three years would turn into fewer and I would die sooner rather than later.  I have never asked "why me?", but that night I asked "why now?".  I was halfway through a highly competitive doctoral program and had about half the data for my dissertation collected.  I was working two twelves, two eights, every third Saturday, and going to Memphis each Wednesday to work with a pediatric endocrinologist for my fellowship.   I asked God why he would put me in that program and waste a spot that would go to someone else if I were not going to be around to use it? It would be much later before God revealed the answer.  As it turned out, an x-ray the next morning showed that my lung had re-collapsed and I now had a tension pneumothorax. I was whisked immediately to the interventional radiologist who inserted a larger chest tube, which took care of the problem.   I kept the chest tube for 21 days, even returning to work with it.

Well, obviously I did not die and now I know the answer to the question I asked God that night.  There are many reasons I was in that doctoral program.  First of all, I am goal oriented and generally do not start something if I will not finish it.  School kept me distracted from how I was feeling.  More importantly, if I did not have that doctorate I would have never tried teaching and found out I loved it so much.   I would have never come to Union, and never gone on the Dominican Republic trip.  I also had access to the UT Medical library and was able to do some research on my own to “help” those who were trying to take care of me. 

Over the years, it seems that my testimony has been how I have coped with the disease.  I have tried to keep a positive attitude and to move forward.  There is only one reason for this- God.  I know that every breath I take is in his hands, and I will breathe exactly as long as he needs me to in order to accomplish his purpose for my life.

Many times people say to me "You don't look sick."  I think they mean it as a compliment.  I certainly do not want to "look sick," whatever that means.  If I could ask you a favor, please do not say that to anyone- it often makes me think that people really mean that I am malingering.  Once a doctor documented these words in my chart "deceptively well appearing."  That sounds a little better.  There are many "invisible diseases."

Here is a picture of me from the UT yearbook in the fall of 2003, before the lung issues that began that December.

Here is a picture of me exactly 12 months later, 9 months into lung disease and 30 pounds lighter.

For 13 years I have been able to do many things- travel, teach, go on mission trips, etc.  I have spent some time in the hospital, and I have had to use oxygen.  I have been blessed to have a rich, fulfilling life with chronic lung disease.  

Less than a year ago the values of my pulmonary function tests began to decline, and there has been a slow downward trend ever since.  

Here is a copy of what the results of pulmonary function tests look like.  This is the report from two weeks ago. Notice all the "As" in the far right column- those do not mean "excellent," as in grades, they stand for "abnormal."

The first value, FVC, stands for Forced Vital Capacity.  That is the amount of air you can exhale after a deep breath.  I should be able to exhale 3.06 liters, but you can see the value is 1.44 liters, or 47%.

The next value, the FEV1, is what many people call "lung function." For me, it corresponds well with how I feel.  This tests measures the amount of air you can blow out forcefully in the first second after a deep breath.  That value should be 2.4 liters for me, but it was 0.58 liters, or 24%.  This is down from 33% a year ago. 

Further down the page is RV, or residual volume.  This is how much air is left in your lungs after exhaling as much as you can.  Mine is 203%.  You can appreciate that there is little room for fresh, oxygenated air in my lungs.  

At the bottom of the page is a DLCO, which tells how well the alveoli are exchanging oxygen for carbon dioxide.  My diffusion capacity is  46%.

When my sister graduated from high school in 1976, one of the graduates sang the Carole King song “Tapestry.”  I remember being fascinated by the concept of life as a tapestry.  I believe that just now is God really giving me an understanding of that concept.  I can look back and see the tapestry that He is weaving together that is my life.  The mission trip that I love so much is represented by threads of “rich and royal hue.”  But even the most beautiful threads in my tapestry would not shine nearly as bright without the dark ones running right alongside them.  I would not change a thing if I could; for it would change the design of the tapestry.  Hallelujah! The really amazing thing is that the tapestry is not yet complete! 

And that is my story- so far!