Amyloidosis - My Personal Story by Paul Langlois
By late 2010, I had trouble walking uphill without quickly becoming breathless. I had nearly given up bike riding in disgust, for how weak I had become. I noticed fluid forming in my legs and ankles. I began to have episodes of nearly fainting when I stood up from a chair. I noticed I could not sleep at night with just one pillow, as I would choke and wake up feeling like I was drowning. My annual visit to my doctor in late 2010 had me pushing him very hard toward looking further into my problems, and he sent me out for some special lab work, as he noticed some extremely elevated protein in my urine test.
Before waiting for those lab results to come back, I checked into my local Emergency Room, the day after Christmas 2010. I was lucky, in that the duty Cardiologist did a thorough history review with me, and after performing an EKG, Chest X-ray and an Echo-cardiogram, reported that I had severe cardiomyopathy (Congestive Heart Failure). I couldn’t believe the news I had just been given, and immediately had to quit working as a pilot, surrendering my aviation medical certificate to the FAA, and calling my employer with the very surprising and grim news.
My Cardiologist called me into his clinic two days after the ER visit, and told me that due to the 
high protein levels in my urine, he suspected Amyloidosis, of which I knew nothing. He believed this rare blood disease may have caused my heart function to deteriorate, due to the infiltration of amyloid deposits. My anxiety reached a new level. He ordered a heart biopsy, for which he advised was the only way to confirm this disease, using the standard Congo Red stain test. The biopsy test came back positive a week later, and my new life with Amyloidosis began in earnest. (I didn’t realize it at the time, but my Cardiologist had saved my life by making the correct diagnosis so quickly).
Naturally, I was devastated, like everyone else who gets such news about acquiring this rare disease. I immediately had a bone marrow biopsy with a local hematologist/oncologist, and he suggested I seek an appointment ASAP to Stanford, the Mayo Clinic, or Boston University, all of which had Amyloidosis treatment clinical expertise. I opted for Stanford since the facility was only two hours (100 miles) from my home, and it sounded like treatment plans may require me to live away from home for long periods. Fortunately, I had health insurance which would cover most of the expenses. In early February 2011, I was initially seen at Stanford by Dr.Witteles (Cardiology) and Dr. Schrier (Hematology), who are the Co-Directors of their Amyloidosis Center.
I was advised by Dr. Witteles that my heart was in such dire condition that I would need surgery immediately for an Implantable Cardioverter Defibrillator (ICD), to hopefully prevent any ventricular fibrillation, which could be life threatening. He also told me that I would need to consider heart transplant if I wanted to survive much longer (i.e. months). I was told that I would need to arrange for a full time Caregiver tending to my future needs during the heart transplant recovery period, or I would not be eligible. Of course, my wife Linda, and my son Nathan, both agreed to assist me.
The next day, Dr. Schrier confirmed that I definitely had Primary Amyloidosis, and that the urgent initial concern was restoring my heart function, in which he had to defer to the Cardiology Dept. Through various tests, it appeared my other organs were not in a dysfunctional state, and he immediately placed me on a regimen of chemotherapy and steroids to hopefully reduce the spread of the Amyloids. Discussion of a stem cell transplant was mentioned, but not recommended from the onset, as my failing heart would likely not tolerate the stress of such a procedure. My chemo regimen consisted of three weeks of Revlimid/Dexamethasone, followed by one week off. The weekly dosages of Rev were increased each month from 5, 10, 15mg, with Dex staying steady at 40mg. I learned that my critical blood chemistry value of lambda free light chain (Amyloid) was initially measured at a concentration of 28, and that we ultimately wanted to see this number drop to less than 2.
I agreed to the ICD surgery (March 2011), and simultaneously commenced a vigorous six weeks of pre-screening tests for heart transplant qualification, made more restrictive due to my Amyloidosis condition. If the pre-screening tests had demonstrated that I had any other organ dysfunction, the Stanford heart transplant committee would most likely not have approved my transplant. There were two invasive tests done, including a right heart catheterization, and an upper/lower GI exam. With my great fortune, the committee approved my transplant for Priority Level 1B, and I surprisingly only had to wait eight days until I “got the call” that a donor’s heart had become available. I had a successful heart transplant on Easter Sunday (24 April 2011) under the skillful hands of Dr. Oyer and his expert team at Stanford.
After surgery recovery in two levels of Intensive Care Units, I was discharged from the hospital in only eight days, but was required to live nearby Stanford for many weeks, due to frequent check-ups and to quickly mitigate any emergency complications, for which I had few. Stanford protocol dictates patients remain close by for up to three months post-transplant. I was required to take daily immuno-supressants, various medicines to prevent infections, and supplements typically recommended for heart transplant patients, which continues to this day and beyond. The dosages and medications are managed very closely by the post-heart transplant team through routine blood draws.
Two weeks after heart transplant, I was placed on a new chemo regimen of Velcade and Dex, as the earlier Revlimid was considered a poor drug choice for heart transplant patients. Dr. Schrier advised that I would need to wait about six months before the possibility of having a Stem Cell Transplant -- all the risks and side effects (including possible death) were presented for my full awareness by Dr. Arai in the Blood and Marrow Transplant Center (BMT). With hope, the Velcade treatment would begin to lower my light chain levels, such that I may not even need the stem cell transplant. Only time would tell the next step in my treatment plan.
Soon after heart transplant, I tried to get into a daily routine of walking with Linda, which was made a bit more difficult due to having to wear a protective mask for many weeks. The mask serves to restrict inhaling any particulates that could cause infection. Much to my surprise, only six weeks after transplant, I was able to walk for 5 miles on a hilly trail around the popular Stanford Radio-telescope park. I was amazed. I soon realized that frequent exercise and maintaining good circulation was very helpful for recovery from surgery and treatment. As the weeks went by and I was finally able to return back home, I was happy that I could once again ride my bike long distances, and get back into the gym for strength training to reverse the muscle atrophy. I also started doing some Yoga classes, along with the walking. Although I was recovering very nicely from the heart transplant, I could not help but think that the heart transplant was only Step 1 in my journey, and knew the unwanted Amyloid proteins were still in my blood -- I definitely did not want them attacking my new heart. I wrote a letter to the family of the donor at six months after the transplant, seeking to thank them for their precious gift of life, but have not heard back -- organ donation is such a private and personal matter.
In August 2011, at a monthly appointment with Hematology, Dr. Schrier advised that it was time to
make a decision on whether I wanted to move forward with the Stem Cell Transplant. As it turned out, the past four months of Velcade/Dex chemo treatment had lowered my lambda free light chain count to a lowest point of 8, but had then risen back up to the 13-15 range, indicating the chemo had been somewhat useful, but not fully effective for my needs. We all agreed that moving forward with a stem cell transplant was in my best interest, and that my body and new heart had finally become strong enough to endure this difficult treatment. I knew the risks, and I knew the recovery would not be easy.
In early October 2011, I began the long process of Autologous Stem Cell Transplant at Stanford. Although I was treated superbly, it was a very difficult process with almost three weeks inpatient, followed by a few weeks living nearby the hospital. The recovery period was quite unpleasant, and might have even been more difficult than the heart transplant recovery, but as each week passed, I became stronger and slowly started to feel better. I got back into my exercise routine as soon as possible when I got back home, despite some days of extreme fatigue, nausea, pain, including frequent migraine headaches. I was told that it would take about three months for the transplant results to become apparent.
In January 2012, after anxiously awaiting some positive news on the outcome, I learned that my lambda free light chain numbers were still at the mid-teen level, versus a desired reduction to less than 2. My conclusion was that the transplant did not work for me. How disheartened I was. Unfortunately, the procedure is not effective for everyone. Despite this psychological setback, I have been feeling better each week, and always keep a positive mindset. I continue to trust that my Stanford doctors, nurses and the entire hospital team are very knowledgeable and doing the right thing for me – simply put, they have all been fabulous.
As I conclude this testimonial, I am currently awaiting recommendations from my Stanford doctors on what the next recommended treatment plan may be for me, most likely resuming a chemo regimen with Velcade. Quite obviously, I have not been able to resume employment as a pilot, and simply tell people I am retired, and otherwise on disability. Not being an active member of the working community has been difficult for me, but I have adapted. I like to think of my future as being a long term survivor, yet at the same time very lucky to have made it to the one year point after the Amyloidosis diagnosis. Linda and I plan to book a vacation to Hawaii in a few months, as we had to cancel the trip last year for our 30th anniversary due to my heart transplant.