My diagnosis finally came in October 1995 following an open lung biopsy, though signs of illness began a few years previously with shortness of breath and a lot of fatigue. Since I was very athletic, this needed to be looked into. An evaluation at a sleep disorder clinic showed I had obstructive sleep apnea. This occurs when the body temporarily stops breathing on its own. A CPAP machine was prescribed to correct the condition and. although I began waking more alert, my energy never returned to former levels, something I thought odd.
Around that same time a series of upper respiratory infections began. By spring of 1995 my breathing problems became more frequent, accompanied by wheezing. Diagnosis: Asthma. 'Hmm' -- I thought my childhood asthma was returning (there's a long family history of it). The prescribed inhalers seemed to help, so I felt this was a family legacy, never questioning the diagnosis.
Pneumonia was the next condition to happen along. I ended up in the hospital where I quickly responded to standard treatment and was discharged a few days later. For the next few months "asthma" attacks became more frequent until August '95 when I 'crashed' with a second bout of pneumonia. That time I didn't respond nearly as quick, so a lung specialist was finally consulted. He immediately suspected something wasn't right because of the multiple URIs and 'pneumonia' twice in such a short time. He said he didn't know what it was, but he felt sure it wasn't the run-of-the-mill pneumonia. A bronch and bronchial 'washings' (aka lavage) were performed. Although some abnormal cells showed up, the biopsy was OK, so no further tests were performed.
But a month later I was back in the hospital !!! -- this time much worse than ever before ('what was happening??) Another inconclusive bronch!! This time the specialist suggested an open lung biopsy. Because all prior tests were 'inconclusive' he felt OLB was the best method to learn exactly what condition I have so that we would know better how to treat it. Off to surgery I went.
When it was over, the doctor came to tell me the biopsy wasn't good. It showed I had Usual Interstitial Pneumonitis (UIP). He explained this is an autoimmune disease and the body starts to get the wrong messages (for some uncertain reason). Some of cells of the body think the lungs are a foreign object, and those cells begin to attack the lungs. That causes inflammation which in turn scars the lungs. As the lungs become scarred, they harden and lose their ability to stretch as I breathe. UIP is sometimes thought of as an advanced stage of IPF -- Idiopathic Pulmonary Fibrosis. "Idiopathic" means its source is unknown.
Prednisone is the first line of treatment for UIP. The specialist's concern was that statistically a person who has used the 'dreaded pred' off and on (which I did for my "asthma") doesn't usually respond as well to it as someone who had started and continued with it. But, initially, it worked quite well in controlling progression of the disease. Of course what wouldn't work well at 80 to 100 mgs a day!!!
I ended up with just about every possible side effect from prednisone use. Along with a hefty weight gain, (photo below) I developed insulin-dependent diabetes; both muscular atrophy and myopathy; constant bruising and skin tears that wouldn't heal, collapsed veins, severe fluid retention, even a herniated disk in my neck.
During a one year period I was hospitalized on nineteen different occasions. My antibiotic use was so frequent I developed allergies to them. Other medications prescribed along with prednisone were Imuran (up to 200 mgs), Trimethaprine (essentially Bactrim without the sulfa), Diflucan for the fungal infection I developed (even in the lungs). All the while I continued to use lasix and Zaroxlyn (a heavy duty fluid pill) to get rid of severe edema; then 240 mgs of potassium a day to replace what lasix washed away! When I developed a deep vein blood clot during an ICU stay, Coumadin (a blood thinner) was added to my meds; finally even morphine became part of my "pharmacy" to dull the pain from the herniated disk in my neck.
Physicians, friends and family believed I was dying. All my time was spent in a hospital bed using oxygen around the clock. Some days I couldn't walk the few steps to the bathroom to void, -- couldn't bathe -- couldn't exist if not for those who became my caretakers. There was talk of Hospice referral, and whispers about 'six months to live' There were days I felt I was dying too, and I was only 33!!
Then Dr B started me on Colchicine. This old 'gout' medication was being used at Mayo Clinic with great results. It began turning things around for me, too. After many adjustments with meds, a stay in a rehab hospital learning to walk again and care for myself, life began looking up. Finally I was able to go below 20 mgs of prednisone/day, and eventually came off them completely. Imuran was lowered to 50 mgs a day; after six months on Coumadin, doctors wanted me off it completely. My friend (a nurse) and I questioned the doctors on that. We felt since I had done so well in the previous six month on the combination of Imuran, Colchicine and Coumadin, why change something that was working so well? The doctors agreed.
Once prednisone use stopped, I was able to lose almost 100 pounds gained while using that drug. I'm thankful for each day I continue to be alive because, after all, I am not cured of UIP. It's just not as active as it once was. The side effects from the drug are permanent. The herniated disk in my neck makes it difficult to move easily or quickly without a lot of pain. There are good days and bad ones. That is something everyone has in common if you have lung disease.
To anyone who has just been diagnosed with any lung disease -- you need to find a really good doctor to work with you. I went to other states for second and third opinions! Positive attitude, faith and hope also make a difference in how we live and deal with a diagnosis that is incurable.