I have been very fortunate as regards my health. Over nearly 65 years I’ve spent one night in a hospital and usually saw a doctor once a year for a physical, if that often.
However, in 2015 I noticed something was going on. When I walked up a flight of stairs I had to catch my breath before I could talk to my wife. I was supposed to be training for my eighth Ironman race and my training was flatlining when it should be peaking! What was this all about? I had an aerobic engine better than guys half my age. I used to run flights of stairs as a workout.
What happened was diffuse scleroderma, an autoimmune disease that hardens your skin and can affect any number of internal organs. It was considered incurable and the options going forward are not very good. I was diagnosed on December 23, 1015, only a few months after noting that something was wrong. In that respect, I was again fortunate. The average time to diagnosis is 3 to 4 years after the first signs of the disease.
What follows here is a rather detailed log of what has transpired over the past year and a half to provide a permanent record for reference. I’m not out of the woods by any measure, but at least I have an opportunity to improve my outcome. The history may help.
- April-May 2015 My triathlon training program began flattening out at a time when it should be improving significantly.
- August 2015 I noted that I was fatigued and really did not have the energy I usually had.
- September 2015 Walking up one flight of stairs left me out of breath to the point that I could not talk to my wife without waiting to first catch my breath.
- October 8, 2015 I woke up to find my hands swollen so much that I could not close them.
- October 14, 2014 I went into U. of M. Emergency for suspected heart trouble (fatigue, swollen hands, shortness of breath). I ended up being admitted for three days while undergoing a battery of tests (X-rays, CT scan, Echocardiogram, etc.). The diagnosis was pneumonitis which was reasonable except that my hands were still swollen.
- October 22, 2015 First pulmonary function test.
- October 24-28, 2015 In bed and ill with severe cough and very tired.
- November 9, 2015 Saw Dr. Kiningham (PCP) with a sore throat and cold; coughing, chills, fever, and hands that are still swollen. Basically confirmed that it was pneumonitis and ignored the swollen hands.
- November 16, 2015 CT scan showed slightly swollen lymph nodes in my lungs.
- December 14, 2015 Bronchoscopy with lavage and biopsy.
- December 23, 2015 Dr. Khanna diagnosed me with diffuse scleroderma based upon swollen hands, blood tests, and bronchoscopy. Started Prilosec for reflux.
- December 30, 2015 Lung biopsy confirmed diagnosis of interstitial lung disease.
- January 7, 2015 Right heart catheterization showed no sign of heart involvement and a normal PAH (i.e. none).
- January 8 to March 16, 2016 Began Cellcept orally at 3,000 mg per day. No positive effect. Lung function is still declining.
- April 20 to September 7, 2016 Six doses of Cytoxan (1,430 mL per dose).
- August 8 & 22, 2016 Two doses of Rituximab. Cytoxan was not looking effective so Rituximab was added.
- October 11, 2016 Appointments with Dr. Khanna and Dr. Flaherty. Dr. Khanna he is somewhat encouraged that Rituximab is having a positive effect. The problem is that my PFT today shows a continued decline in my DLCO (27%) and I am running out of numbers on the low end. My meeting with Dr. Flaherty was to discuss a lung transplant. Because it takes months to go through the approval process it was decided to begin testing to determine if I am a candidate for lung transplant. Also discussed stem cell transplant with Dr. Khanna. He said I am not a candidate because my lung function is so low. All in all not a good day today.