My Journey to Wellness
If you are reading my story it is likely that you, or someone you love, have just been given the diagnosis of amyloidosis. As you have already discovered, this is a very rare and difficult to diagnose disease, so we are the fortunate to have an explanation for the symptoms we have suffered and an opportunity to treat the cause. We are the lucky ones.
The symptoms of this disease came many months, perhaps years before I received my diagnosis of primary amyloidosis, AL. The normal activities of my life had become more difficult. I was tired and had given up things that I loved, like skiing and hiking. My work as a dentist had become so tiring that I would just collapse in my chair at home at the end of my day. Months before my diagnosis, I noticed it was becoming increasingly difficult to walk any incline, and even at level I was becoming short of breath and dizzy. My sense of taste had changed and nothing tasted good. I had lost 25 lbs over the last year. I suffered from dysphagia, difficulty swallowing, yet none of the tests showed any cause. My vision had deteriorated and I was seeing double. The optometrist gave me a new prescription but nothing helped. I searched out a new general physician who listened to my symptoms ran tests and suspected I had had a heart attack as I showed an abnormal rhythm and an increased BNP (Brain neuro Peptide). I was sent to a cardiologist who did an echocardiogram, missed the enlarged heart and reported pristine vessels. Finally in March, 2009, I ended up in the emergency room with severe abdominal pain. The initial search for gallstones came back negative and they sent me home on pain medication only to have me return a day later with increased epigastric pain. I was admitted to St. Vincent's hospital where a team of gastroenterologists, hospitalists and neurologists ran tests exhaustively yet came up with nothing and planned to send me home. The night before discharge my troponin (cardiac) level increased and I was sent to the cardiac care unit for monitoring. Around midnight a brilliant young cardiologist, Dr. Sohn, came in to see me saying he had reviewed my chart (now extensive) and suggested I might have a rare disease, amyloidosis that had caused a buildup in my heart of amyloid proteins that had made my heart thick, conducting electricity poorly. I would need a heart biopsy for diagnosis. It was decided that I would be sent to Oregon Health Science University (OHSU) for this procedure.
Fortunately, I had an advocate at OHSU. Brian Druker, who was one of my dental patients was the Director of Oncology Services for the university. He set me up with a wonderful oncologist, Gabby Meyers, the heart failure team, and the gastroenterologist service. I was admitted to the cardiac care unit and testing began right away with a heart biopsy, fat skin biopsy, rectal biopsy, endoscopy, urine collections, blood collections, etc. All the biopsies came back negative for the congo stain for amyloidosis and the physicians were again at a loss. This disease had eluded testing. Finally on the last day, a team of 6 physicians from the GI and general medicine services came in to say they could not determine the cause of my symptoms and they would be sending me home without a diagnosis, in abdominal pain, suggesting perhaps there was a psychological component to my pain. Moments later, as my friend Brian Druker came in to visit, the results of the blood test and second heart biopsy came back with a diagnosis of primary AL amyloidosis. After 31 days in the hospital I had a diagnosis and a plan for treatment. I went home to prepare.
Autologous Stem Cell Transplant
The decision was made to treat the amyloidosis with high dose Melphalan followed by a rescue with a stem cell transplant from my own stem cells harvested before treatment. My Lambda light chains were 282.5 prior to transplantation. I was admitted on June 15, 2009 to the OHSU bone marrow unit. My transplant was uncomplicated. The treatment had obliterated my immune system and my white blood cells were gone. I was fortunate that I had no fevers or infections and I came home on day 30. Unfortunately, my light chain levels increased to 345 in September following the transplant and it was determined I had failed the treatment with Melphalan and the autologous stem cell transplant.
Velcade and Prednisone
On November 3, 2009 I began my treatment with Velcade and Prednisone. This outpatient treatment was delivered in cycles of weekly IV bolus doses over two weeks with a week off in between. This further altered my sense of taste and increased my fatigue but it was tolerable. We watched the light chains come down monthly over the 7 cycles decreasing from 345 to 50 in March of 2010. Unfortunately, during these months I began to exhibit increasing signs of cardiac failure with significant swelling of my legs, abdomen and scrotum. Lasix did nothing for this edema but we discovered Torsemide did offer some help getting the fluid off. I was very dizzy and began passing out when I got up after sitting for any length of time. My oncologist, Gabby Meyers, was very worried that I was in severe heart failure. On March 17, 2010 I was admitted to the ICU after an evaluative angiogram, by the extremely skilled heart failure physician, Dr. Tony Kim. My cardiac index was 1.68. The doctors said I was in severe heart failure from the amyloidosis and needed a heart transplant. The conundrum was they had never done a heart transplant on a patient with amyloidosis. As tactfully as they could communicate the situation they explained that there are large numbers of people with heart failure waiting for a transplant and not enough hearts to serve those in need. If they gave a heart to me and it became affected by the amyloid deposition they would not be helping anyone. There was much discussion with the transplant team at OHSU. Right around this time we were sent a blog about a patient with amyloidosis who had received a heart transplant at Stanford. A physician himself,
Kevin Anderson, had been the first patient with amyloidosis to receive a heart transplant at Stanford. His testimonial in the Stanford webpage ended in 2008. Concerned he may not have survived we tracked him down at his urology practice in Sunnyside, California where he had just returned to his medical practice. Happy news indeed! He generously shared his story and his incredible journey of hope with us. We shared this story with our team of oncologists and heart failure cardiologists. On June 2, 2010 I was placed on the list for a heart transplant at OHSU.
My wise and caring oncologist, Dr. Meyers decided to hold off on the Velcade and Prednisone regime and allow my body to recover. I was on the list as a heart transplant as a level 2. Level 1-A is the level of priority for patients who are acutely ill, on life support in the hospital. Level 1-B is the level of priority for patients with a ventricular assistance device VAD or who are on multiple medications. Restrictive cardiac myopathy due to amyloidosis would not respond to any of these modalities, so my level of priority was at a reduced priority. At level 2 we expected the wait could be months if not years….. so we prepared for the wait. I decided to sell my dental practice. I had been fortunate to have excellent dentists covering for me but with the expectation that I would likely not be able to return to my practice in the near future selling the practice seemed the best decision for my patients whose welfare was critical to me.
On July 23, 2010, only 12 days after selling the practice, I went in for my first appointment with the heart failure physicians since being placed on the waiting list for a heart transplant. Dr. Eric Adler, a very earnest and brilliant young cardiologist who had advocated for me having the transplant came in very excited. "Are you ready for your transplant" he said. "Sure", I responded thinking he was speaking in general terms. Then he said excitedly that they had never had it happen before that they had just received a call that there may be a match for me as I came in for my routine appointment . He came back in a couple moments later saying I was second in line. My wife and I left the office not expecting a surgery that day but encouraged that I was closer on the list. A couple hours later I got the call to come in the hospital and by the next morning, July 24th my surgeon, Dr. Song, had delivered to me a new heart.
It has been 15 weeks since I received my new heart. These 110 days are a gift. In celebration of the transplant my Lambda Light Chains dropped to the normal levels, 17.4 for the first time and remain ~25. I no longer pass out. I have no more edema or swelling and I am attending cardiac rehab, growing stronger each day.
My main continued challenge is the abdominal pain that has not left me since diagnosis. The doctors suspect this is the neurologic involvement of the amyloid proteins in the nerves of my gastrointestinal system. I have transitioned from IV pain meds to patches but hope that someday as the light chain levels remain reduced these proteins will be released from the nerves allowing me to get off the pain medication.
I am ever grateful to my excellent physicians who kept me well and advocated for me, to my friends and patients who sustained me with their love and humor and who put me on many prayer lists working the power of prayer, to all my family especially my wife, Brenda, and three sons who have been there for me every step of the way and lastly to the donor and his/her family who generously donated the most cherished gift of life. I am filled with gratitude for the opportunity to look forward with hope.
If you are newly diagnosed and beginning your journey we welcome you to contact us. I encourage you to become informed. Get to an institution where the physicians are experienced in dealing with patients with amyloidosis. Partner with them along this path by being informed and being your own advocate. Be kind to yourself, getting rest and nourishment and surround yourself with supportive, positive people. Most importantly….. Be Hopeful.
-- Vince and Brenda Liguore