Amyloidosis – My Story Thus Far
Kevin R. Anderson, M.D.
November 17, 2008
Chapter 1 - The descent into illness
People say I have led a charmed life. For the most part, that is true. Things have always worked out well for me. I do not consider my diagnosis of Amyloidosis and resultant need for a heart transplant to have changed that view in anyway. Given the possibilities, things continue to work out as well as they could.
I was born in Glendale, CA the 4th of 9 children to wonderful parents. I was raised in Marin County, CA. I chose to be a physician when I was ten and worked toward that goal. I left for college at age 16 and graduated from BYU in1981 with a degree in Zoology. As a Mormon, I spent two years in Buenos Aires, Argentina serving as a missionary. I married Barbie Dison in 1982 and we have 4 wonderful children, three of whom were married this year.
I finished medical school at University of California, San Diego in 1986 and went onto a residency in Urology at UC Davis in Sacramento, CA. After a fellowship in minimally invasive surgery and laparoscopy at Washington University in St Louis, MO, I joined the faculty at Yale University as an Associate Professor of Surgery and served as director of minimally invasive Urology and Chief of VA Connecticut for 11 years.
I returned to Roseville, California in 2004 and currently am the Chief of Urology for Kaiser Permanente in Sacramento.
In 2006, I began to notice some physical changes in my health. They began quite subtly. I often would ride my bike the 12 miles to work from Lincoln to Roseville, but began to have difficulty with the endurance to ride up hills. I was also in the habit of walking up to two miles at lunch to whatever nearby eating establishment fancied me that day. I would notice that after lunch, fatigue would set in on the walk back to work and I would have an ache in the pit of my stomach. But, even more significant would be the 2 year battle that would begin with the flight of stairs from the hospital lobby to the operating room. This would measure daily my descent into heart failure.
The hallmark of amyloidosis is its subtlety and how it mimics other common benign conditions. That is why it is difficult to diagnose. I would climb the stairs fine, but at the top of the landing, I would suddenly feel ‘out of breath’. Technically, I knew that it was not a lung issue, but I would be breathing harder than expected for the work that I did. This made it hard to describe to my doctors, because they always wanted to exclude pulmonary issues. I knew it was not my lungs, but there was no better way to describe the sensation. What I wanted to say is that ‘I ran out of energy’. Within seconds, I was fine and on my way to the OR. However, over the ensuing two years, the degree of ‘shortness of breath’ and the recovery time increased. Eventually, I could not continue walking after the climb and would have to stand and wait for a minute to regain my strength. Otherwise, standing, walking or sitting, I felt fine. This made it easy to hide my limitation from others. As a surgeon and chief of my department, I knew that if people knew I was sick, they would view me differently, affecting my ability to interact with patients and colleagues in an unbiased manner. So I kept it to myself, and in a way, from myself, because I didn’t know why it was happening either. Until I had a diagnosis, and a plan to either fix it or live with it, I felt uneasy sharing it.
Other symptoms were associated:
Hoarseness, jaw pain, difficulty swallowing, (dysphagia) abdominal bloating, early satiety (fullness with eating) periorbital petecchiae, (red spots on my eyelids), chest tightness, nasal congestion, palpitations, sensation of a fast and/or irregular heartbeat, sleep apnea (noticed by my wife as short periods of breathing cessation at night) a pounding heart beat lying down at night on my left side and I would get dizzy getting out of a car after sitting for a while.
An internet search (after having learned of my diagnosis) listing symptoms of amyloidosis gave this result:
· Weight loss
· Shortness of breath
· Numbness or tingling in the hands or feet
· Severe fatigue
· An enlarged tongue
· Feeling full after eating smaller amounts of food than usual
· Dizziness upon standing
· Swelling of the ankles or legs
- Difficulty chewing or swallowing
- An irregular heartbeat
In August of 2006, I called my doctor to report on these changes, we decided to keep an eye on it. By October, things had not improved, I called my doctor and his partner ordered an ECG. This showed subtle changes, but a possible T-wave inversion, (possible ischemic changes) so a cardiac work-up was done with an exercise tolerance test and nuclear medicine perfusion study, both of which were normal and showed no ischemic changes. Interestingly, the ECG technicians always had trouble with getting a good ECG because I would have low-voltage tracings (classic for amyloidosis and restrictive cardiomyopathy). This was attributed to my hairy chest and poor lead placement.
By April of 2007, nothing had improved and I finally saw my primary care physician. The following is a portion of his note:
5 months ago, noted running up stairs he felt some increased tachypnea running up stairs. Occasionally heartrate seems increased, feels lightheaded or dizzy. Doesn't have the exercise tolerance he used to have. Sometimes getting out of car, feels lightheaded. Had extensive cardiac w/u a few months ago; nl nuclear med stress test.Has also had hoarseness; seems to be worse as the day goes on. This has been going on for the last month. He has been having some allergy sxs over the past few weeks as well with some nasal congestion.
Occasional petechial hemorrhages in the upper eyelids. This seems to occur only when he misses the blood pressure medication for a couple of days.
Has some difficulty swallowing which has been getting worse recently. Has had severe GERD in the past. Had endoscopy 2 years ago which was normal.
Wife has noted some apneic episodes recently. This seems to correspond with a worsening of allergy sxs.
Severe intermittent back pain in the low back; that is improving. Has had an MRI which did not show any problems.
HYPERTENSION (primary encounter diagnosis)
Note: good control. There is some concern that some of his sxs could be the result of hypotension; pt would like to remain on the HCTZ for its ability to inhibit renal stone formation.
Plan: D/C lisinopril/HCT
Continue HCTZ 12.5 mg daily.
Monitor BP and call if it seems to be going up significantly.
Note: suspect this, along with the hoarseness and dysphagia, may be secondary to GERD. May be triggering some bronchospasm at times, especially as sxs seem to be worse after eating a large fatty meal.
Plan: trial of Prilosec daily
If no improvement then discuss with cardiology regarding need for echochardiogram vs. PFT>
APNEA OR APNEIC EVENT
Note: may be sleep apnea vs. Allergy sxs
Plan: pt started on Nasarel; if continues to have apnea as reported by his wife consider sleep study.
Note: suspect secondary to GERD
Plan: HNS evaluation if no improvement with Prilosec or Nasarel
Note: again, this would be explained by GERD, esophageal spasm. Pt with recent UGI endoscopy which was unremarkable. Further evaluation if sxs do not improve; would discuss with GI
LOW BACK PAIN
Note: improving at this time
Plan: Nasarel as above. Recheck if no improvement
Lipids, ALT, CBC, lytes, fasting glucose, TSH.
I don’t blame my doctors for missing this. We have a saying in medicine, “Common things are common.” However, the contra positive to that statement is equally true, “Rare things are rare.” The last time that most physicians have heard of amyloidosis was in medical school, myself included.
Then things got worse.
In May, I was on a hike with my family to some waterfalls and on the 2 mile hike out I got severely fatigued to the point that I almost fainted and had some vision loss. I made it to the car but this scared my wife. A month later we took a family trip to Italy. I knew I would have trouble, but did not want to cancel the trip. I walked slowly and despite some dizzy episodes, I survived and the trip was wonderful.
At this point, I decided to see a cardiologist. Dr Khurana began an extensive workup. ECG, Holter monitor, Echocardiogram and cardiac catheterization. Various supraventricular non-specific arrhythmias were found, but nothing to point to a specific etiology. The cardiac catheterization showed clean coronary arteries, but the machine that assesses cardiac output was not working that day. The echocardiogram showed only mild changes with some borderline thickening of the intraventricular septum, but not sufficient to diagnose restrictive cardiomyopathy. The term right heart dysfunction or diastolic dysfunction was used, but to me had no particular frame of reference. I knew that my right heart pressures had to be high because every night the tri-phasic bounding venous pulse would beat this into my brain as I fell asleep. In desperation, I ordered an abdominal CT on myself to ‘rule/out’ anything else. Of note was the finding of perihepatic vein edema. I tried to get someone to comment on this, but to no avail. However, this finding always worried me. Ultimately, this was an early sign of my high venous pressures from right heart failure. My gut was under pressure.
In addition, I had a blood test called a BNP which measures congestive heart failure. Mine was in the mid 200 range, elevated, but not enough to make the diagnosis.
The focus became the arrhythmias and an attempt at beta-blockers was tried with disastrous results. Beta-blockers made my abdominal pain severe. This was thought to be a drug reaction but other beta blockers had the same results. Calcium channel blockers were tried, and worked well. My arrhythmias stabilized and I felt better. I did note that when I did exercise, I could never get my heart rate to go above 110, however, at rest, it never would drop below 85. I seemed to have a very narrow range of rate where my heart wanted to live. I would later understand why.
By January of 2008, I was worsening; I was dizzier all of the time. Dr Khurana told me I had diastolic dysfunction, etiology unknown. I asked her if this was congestion heart failure and she said it was like it. I asked her if there was anything I could do to improve it or stabilize the condition and was told maybe. This was depressing. I thought that I would never run again. I gave my bikes away. I would see older people running, I would see obese people climbing stairs and think, “this is not fair, why can’t I do that”. But I continued to keep my peace, not complain and stay silent.
Dr Khurana, however, did two things that helped quite a bit. She started me on a low dose beta-blocker that I could handle, Coreg, and a low sodium diet. Within 1 week I lost 10 lbs and felt much improved. The low sodium diet then became my mission. How I felt daily was directly related to my weight and salt intake. People began asking, “You look great, how are you losing the weight?” I said I was on a low sodium diet for high blood pressure. What they didn’t notice was that I was always sitting or leaning against a wall as I chatted with them. And what I didn’t realize was that I was in a state of malnourishment from abdominal edema from my right heart failure. My stable weight was the zero sum of increased fluid retention minus fat and muscle loss. Over the next 4 months I lost 30 lbs of fat and muscle without realizing it. I finally figured something was wrong when sitting became painful due to a lack of ‘padding’ on my gluteus maximus. In March, I had a brief episode of right field vision loss. I went to the ER and was admitted. The vision loss was intermittent with standing a lasted 6 hours. I was in the hospital for two days and an MRI showed a possible ischemic area in the left occipital part of the brain. But this was not conclusive and the neurologist called it an atypical migraine and put me on aspirin.
I always knew that when walking on flat terrain caused fatigue, I was in trouble. This happened in May of 2008. A third echocardiogram was performed which confirmed the diagnosis of restrictive cardiomyopathy, had the classic ‘speckled’ appearance with a thick intraventricular septum, and I heard the word amyloidosis for the first time. Then everything changed.
Chapter 2 - Diagnosis
Mid June the Echocardiogram was reviewed and it was clear that the heart had worsened. The question was why. It could be idiopathic, amyloidosis, multiple myeloma or some other rare infiltrative process. It was time to break the silence. Barbie sent out an email to a few friends in California and Connecticut and we told our family. On July 1st, I informed my partners in our monthly meeting that I had restrictive cardiomyopathy and was waiting for a biopsy to determine if it was amyloidosis. I said I would continue to work. The news spread like wildfire. The response was overwhelming and somewhat difficult for me to adjust to. I was not accustom to being the individual in need; my role was always the opposite, to support others in their suffering. I did not want people to worry about me. This would begin a huge personal transformation in how I saw myself and the need to always be in control. This would be a very important life lesson for me.
I was scheduled for a fat biopsy and referred to the regional transplant cardiology group at Kaiser in Santa Clara. A heart biopsy was done on July 2, 2008. At the time of the biopsy my cardiac index was 1.7. It should have been double that amount. This meant that my heart was only pumping half of the volume per beat than it should have because it was so stiff from the amyloid deposits. My heart didn’t have time to fill up between beats to have an adequate volume for the next beat. This is why I would get worse if my heart beat too fast or too slow. A fast heart rate would further decrease the time to fill and a slower heart rate would not pump enough per minute to satisfy my needs, my heart rate had to stay around 85 beats per minute. The amyloid deposits also interfered with nerve conduction in the heart, causing arrhythmias and decreased voltage on the ECG. Beta blockers slowed the heart too much causing further heart failure, I could only tolerate a low dosage or Coreg.
At the end of the heart biopsy and right heart flow studies, Dr Weisshaar, the head of the cardiology group there and the one that did the biopsy shocked me by suggesting that I be admitted to the hospital that day to begin dopamine to improve my heart function. To a surgeon, dopamine is what you give a dying patient in the ICU that has a blood pressure that cannot sustain life. I was still working. In fact I had to cancel a nephrectomy to drive to Santa Clara for the biopsy and rescheduled it for the following week. Still thinking like a surgeon and not a patient, I reassured her that I felt fine and if we could continue the work-up as an outpatient. She agreed. I asked if I would need a heart transplant and she said I would, but might, not be a candidate if the disease was involving other parts of my body such as the kidney, liver, gut and nervous system. When she left the room, I realized for the first time the true reality that I could die in the not too distant future from this disease, it became real to me, and I silently wept there on the fluoroscopy table.
That week the fat biopsy was done and was negative for amyloid. A bone marrow biopsy was also done. At this point the work-up shifted into high gear and a multitude of blood tests and radiologic exams were done. A skeletal survey revealed that the bones were uninvolved. But the chest x-ray revealed a large right pleural effusion; water was collapsing my right lung. Was this from amyloidosis or congestive heart failure? There were abnormal levels of protein in my urine and a spike on immunoelectrophoresis indicating production of an abnormal protein. Then, the following Wednesday, the news came.
I was sitting in my office between surgeries doing some administrative work; I still had a major stone case and the laparoscopic nephrectomy to do that afternoon. The phone rang and Dr Weisshaar informed me that the biopsy revealed that I did, indeed, have amyloidosis and it had invaded my heart. She then said that I should stop working immediately. I hung up the phone and wept again uncontrollably, it felt as though my heart was ripped out in that moment. My life had always been defined by my responsibilities to other people, that was who I was and a large part of that ‘me’ evaporated in that moment. I immediately called in my assistant and my head nurse and informed them that I would be shutting down my practice effective the end of the week, it was one of the hardest things that I have ever done. But, I still had two major surgeries that day, I prayed that this news would not affect my abilities, that is was of the amazing things about doing surgery, you are so focused during the case, all other outside issues are temporarily suspended. The kidney removal went perfectly, but as I spoke with the family after the case, I could not help but think that as I had just cured this man of his cancer, he would now probably live longer than I would. Somehow, that was reassuring to me that I could still help others even in the state that I was in. This became my goal, no matter what might lie ahead for me; I would always do whatever I could to help others. He was home in less than two days. I then became a fulltime patient.
The bone marrow biopsy showed no evidence of multiple myeloma, but confirmed the diagnosis of a plasma cell dyscrasia and AL (systemic) Amyloidosis. An attempt at thoracentesis was done to remove the fluid that was collapsing my right lung. As the tube was draining my chest, I began to feel a coldness sweep up from my feet and asked the doctor if this procedure ever caused a vasovagal reflex. This is something we see sometimes when body organs are stimulated during procedures. He said yes it could. I could feel that I was becoming light headed. A blood pressure was taken and I was surprised to see that it was 37/16, but even more surprised that I was still conscious to see it. Since I was sitting, they then aborted the procedure and lied me down. The doctor wanted to put an IV in, but I said, “It’s just a vasovagal reaction, I’ll be fine in 15 minutes.” I was.
The following Monday, I was seen again in Santa Clara and it was decided that I did need a heart transplant, and as far as they could tell, my other organs were minimally involved with amyloid. Normally, Kaiser has their heart transplants done at Stanford, but Stanford had abandon the practice of doing heart transplants on systemic amyloidosis decades before because they did so poorly afterward as the amyloidosis destroyed the new heart as well. For that reason, I was to be sent to the Mayo Clinic since they had the largest experience with heart transplant in amyloidosis. Calls were made and two days later Barbie and I were on a plane to Rochester, MN. We just left our two daughters at home and Barbie’s mom flew from Utah to stay with them while we were gone, for how long, we did not know. We started a website to inform friends and family of what was going on and this became a lifeline for us. From this point a real time documentation of what happened was recorded. It can be found at www.kevinandbarbie.com .
The Mayo Clinic is an amazing place. The facilities are grand, the artwork is serene and adds to the well being of the patients visiting there. The people were so kind there. The doctors were very supportive and thorough. The daily schedule, however, was quite grueling. I would begin every day the first week there fasting for some study or test. At this point I was very fatigued and not eating well, so this took a toll on me. I believed that every day, after my tests were done, that a committee there would invent new test just to do to me. You name it, I had it done. Everything that was done at Kaiser was redone at the Mayo Clinic. But it was worth it because, in the end, they determined that the amyloidosis was mostly confined to my heart. I do have systemic involvement, in my tongue minimally, and in my GI tract. But the gut, kidneys and liver are not significantly involved and there was no neurologic involvement. Thus, after review by Dr Lacy in oncology and Dr Edwards in Transplant cardiology, it was felt that I should be presented to their transplant committee to determine if I was a candidate for a heart. The following Monday, Dr Edwards informed us that I had been accepted. We were so excited. But then he said something that I had not expected to hear. Four years prior, they had seen another patient from Kaiser in Northern California that they had referred back to Stanford for a heart transplant and he had done well with his, granted, he did not have AL amyloidosis, but rather the familial type AA. However, Dr Edwards felt it was worth exploring whether I might be able to be done at Stanford. He got on the phone and spoke with Dr Witteles, the transplant cardiologist there. Fortune was smiling upon us that day as Dr Witteles has a special interest in amyloidosis and was supportive of exploring the possibility of having my transplant done at Stanford. This was greater news than we possibly could have expected. We were mentally prepared to spend the winter in Rochester. We were going home after only two weeks there. As soon as we left Dr Edwards office, my phone rang, and to my surprise, it was Dr Witteles, he had some further questions for me and I remember trying to convince him that I would be a good candidate and good patient for a heart transplant. The next day we flew home.
We were home three days and on Friday my case was presented at the Stanford selection committee, and after what was described as a ‘lively discussion’ I was accepted to their program as a class 2 (priority for hearts is by health status: 1a – on a ventricular assist device VAD, 1b – inpatient status on inotropes, such as dopamine and 2 – outpatient) But when Dr Weisshaar called to inform me of this news, she asked how I was doing. As I reported all of my heart failure symptoms and how they had worsened over the previous two weeks, she told me to drive immediately to Santa Clara to be admitted and started on dopamine. I immediately was moved to 1b status.
To facilitate the administration of dopamine, a long-term IV was placed, called a PIC line. This is placed in an arm vein, but is long and the tip is in the superior vena cava. The dopamine gave me more strength. I began to walk around the nurses’ station to try to increase my endurance and general health. Because I was being monitored on telemetry, I could not leave the cardiac unit. I would walk in circles. I counted my steps and five laps equaled a half mile; which I did 3 times a day. In addition I was on IV lasix, and began to lose water-weight. In 5 days I lost 20 lbs. Once I got to 175 lbs, I began to feel weak and realized that this was my dry weight, (what was left of me when the water overload was gone). I started eating as much as I could to increase my protein intake. I wanted to be as healthy as possible for the heart transplant.
You never know when a heart might become available, so you learn to be patient. Barbie was there with me every step of the way; always at my side. She slept on a roll-away bed in my room. Our roles reversed and she had to take care of everything, driving, logistics, bills etc… I had originally thought that maybe I could be on dopamine as an outpatient so I could fly to San Diego for my son’s wedding on August 16th, but Dr Weisshaar said that would be unwise, saying, “What if the heart came that day? Your life is more important. “I thought the odds were slim, but resolved that I would miss the wedding. The day came that Barbie had to leave to head to San Diego and we both felt devastated at separating. That night, I got a call on my cell phone from the Mayo Clinic informing me that they had a heart and wanted me to fly to Rochester for a transplant. They thought was a good heart, but still had to do some tests. I called Dr Weisshaar and she began making arrangements for me to go by air ambulance. I told Barbie and this was beyond our ability to handle. This would mean that I would be alone for the transplant and then remain in Minnesota for at least 3 – 5 months. I would miss my daughter’s wedding as well, but duty and reason told me that I had to go. My heart said otherwise.
This was one time in my life when I felt paralyzed in making a decision; very much unlike me. Dr Weisshaar arrived at the hospital and came and asked me how I felt about going to the Mayo Clinic and what I wanted to do. I expressed my concerns and she said she would take care of it. After an eternity, she returned and said, “Get some sleep. We passed on the heart.” I was so relieved. Even though I realized that it might be months before another heart became available, it was a risk I was willing to take. I only had to wait 3 days; the heart came on the day of my son’s wedding.
Chapter 3 – A New Heart
Friday afternoon, August 15th, I was practicing tying a bow tie for the internet video conference that we had set up so that I could interact with people at the wedding. Dr Weisshaar came in my room and said, “You won’t need that bowtie, Stanford has a heart for you.” I was ecstatic. I immediately called Barbie. She was incredulous. I booked a flight for her to come back after the wedding and reception were over the following day. As I was taken by ambulance for the 20 minute ride to Stanford, the sun felt so good as I hadn’t been outside in two weeks. I had no thought about the surgery, recovery, pain or potential complications; I was just thrilled to know that it was going to happen.
By 10:00 PM I was in the OR as the anesthesiologist was explaining what would be happening. The mask was put on my face, and after a few breaths, 12 hours of memory were extricated from my life. During anesthesia, time does not exist. You don’t dream. What felt like a moment passed and I heard someone speaking about the ventilator. They were ‘weaning’ off the ventilator and I was fighting it. I made motioned with my hand to ‘pull the tube’. They stopped the machine so I was breathing on my own and soon thereafter extubated me. I saw my brother, Daren and his wife, Rachelle sitting at the end of my bed smiling at me. It was hard to imagine that it was over. I felt no pain.
My first thought was to call Barbie to tell her I was OK. I said, “I love you with my whole new heart.” I was in the ICU for three days and move to a regular room on my 49th birthday. Barbie asked what I wanted for my birthday, I told her I already got it. The recovery was painless. I never took a shot or pill for my surgical incision. I felt wonderful. By day 4 I was walking outside. The right lung chest tube kept me in the hospital longer, because of persistent drainage of my pleural effusion, but soon I was on my way to the Residence Inn in Sunnyvale where Barbie and I would stay for a month while I recovered.
My follow-up care was back at Kaiser in Santa Clara where I had weekly heart biopsies and lab tests to assess my heart and monitor for rejection. Every day I felt stronger and would go for walks and try to eat what I could. Initially, food had a strange taste. This was due to the high dose of steroids. At week two I suddenly became very ill and felt horrible, but within a week, I improved greatly and from then on felt great. I hadn’t felt this strong or had this much energy for years. My new heart was simply amazing. My body was finally getting the blood that it needed. Most of all of my previous symptoms disappeared. My pleural effusion resolved. I realized that could actually live a normal life again and do those activities that I thought had forever been banned from my life; bicycling, backpacking and boogey-boarding.
After four weeks my 4th biopsy showed no rejection and I was sent home to Lincoln after being away for two months. What a reunion that was to be home with our two daughters for good, not to have to leave again. I was back.
Chapter 4 – Amyloidosis
My attention turned to getting healthy and addressing my underlying disease, amyloidosis. I knew I would probably need to wait 3 months before beginning any treatment such as chemotherapy. I Saw Dr Schrier at Stanford University, an expert in amyloidosis, in late September and began Revlimid 10 mg a day and Decadron 40 mg a week beginning the first week of October. Revlimid is difficult to get because of the birth defects that it can cause, and very expensive. It is taken for 21 days with one week off. My kappa light chains prior to starting Revlimid were around 40, (normal is less that 19). After one week on Revlimid, (and the 3rd day after Decadron) I began to feel very week and dizzy. This continued for 4 days. October 14 I had a biopsy that showed moderate rejection, even in the face of supra-therapeutic levels of cyclosporine. I was therefore changed to Prograf and started on high-dose prednisone 100 mg a day. Another biopsy was performed a week later, it was worse, but still 2A/3R (range NED, 1A, 2A and 3A). This concerned my doctors greatly. After much discussion it was determined that either the anti-rejection drugs were being blocked in their efficacy, the levels were erroneous, I had an occult infection or the Revlimid was somehow involved, the latter proved to be the most cogent theory. It turns out that Revlimid might cause T-cell activation. I stopped the Revlimid on Day 19 and started Solumedrol 1000 mg IV on September 23th, one day after my Decadron dose and two days prior to my daughter’s wedding, which I refused to miss.
Solumedrol is a nasty drug, by the 3rd day; I could barely get up from a chair. The wedding was beautiful. My daughter was radiant and her joy carried me through the day. We had catered the wedding with magnificent hors d oeuvres, but everything tasted horrible to me, even the water was salty. But my dance with my daughter was spellbinding as I sent her off on her honeymoon and her new life. Then I crashed.
I went home to give myself my last infusion of Solumedrol in the I.V. left in my arm by the infusion center nurse. For the next three days I felt like a zombie, Frankenstein and a statue combined. It took great effort to convince myself to get out of a chair or even to speak. I could not eat. I lost 10 lbs in four days. I knew this was the steroids, but could only wait for them to get out of my system. They did, however, help, and my biopsy on October 28th was improved to 1A. I slowly improved, but the dizziness persisted. The plan was to wait a month for my heart to improve and then to continue the treatment for my amyloidosis, possibly with Velcade. In the meantime, I continue to take 40 mg of Decadron every Wednesday, It doesn’t really treat the amyloidosis, but it may help suppress it. My kappa light chain level did not change significantly after the first cycle, but it often doesn’t. Two weeks later, on November 11th, my re-biopsy of my heart again showed 2A/3R moderate rejection. This was a surprise, as symptomatically, I was feeling quite normal (except for the transient dizziness that I still get when I get up after sitting for more than 30 minutes.) This news was devastating. Normally I can connect a complication or setback to some fixable or temporary change, but this time I couldn’t which for the first time scared me. The effect on Barbie was much worse. She was very scared, then sad. We have been hopeful for so long, this was quite overwhelming. I started back on high dose prednisone and will have a follow-up biopsy tomorrow. I haven’t given up hope. I expect it to be improved. I walk two miles a day, I lift weights, my muscle tone is returning. I continue to do everything in my power to improve, both physically and emotionally. I remain the eternal optimist and will start Velcade when I am told I am ready and am prepared to receive a stem-cell transplant at the appropriate time. I will return to work and continue to care for patients as long as I am able to give back a small portion of the great gift that I have received. And as such, I will be a better doctor for having been such a cared for patient. Words cannot express the gratitude I feel toward my doctors and their staff. But overall, I will be eternally grateful for the love of my supportive family, my God, and above all, my constant companion in this journey, Barbie, my beloved wife.
Chapter 5 – The Future
I am no verbosaphobe (is that what you called it? I'll have to go back to the top and look :))I read every single word.
Hi Doctor Anderson!
Our family was thinking about you again today and we just wanted to send a lil note your way let you know you’re in our thoughts and our prayers. You are such kind man who has impacted our family in so many ways. Without you being there treating our Boydie we would have been terrified. You have always made our son feel special and you have been so caring and showed him so much kindness; we will never ever forget that and never forget how grateful we are to have you as his doctor. You’re an amazing person and Physician.
Stay strong and God bless you!
Have a wonderful Thanksgiving,
Love The Sheets Gang!
my name is Felipe Ulloa, I am from Chile. My Mom has just been diagnosed with amyloidosys. Surfing on the web I found your story. I think we have some things in common. We are members of the LDS Church, and I served my mission in Buenos aires, argentina.
We are just learning about this desease, and as you may guess, here in chile is hard to find a doctor with the required experience to treat this desease in a proper/effective way.
So I was hoping that you could give us a hand.
I look forward to hearing from you.
My email address is email@example.com
Dr. Anderson, I too had the subtle decline. It first affected my GI tract (lost 20 pounds and diarrhea) then the subtle increase in fatigue and light headedness. My cardiologist suspected amyloidosis after 6 months of GI tests showed nothing. I went to the Mayo Clinic and was diagnosed a month ago with amyloidosis. Mine is ATTR (not AL) and the TTR mutation is THR-060-ala. I am a candidate for liver transplant since there is no other option. I think I have identified my onset relatively early and hope for a good outcome. My heart remains healthy enough for a transplant. I guess the jury is out on whether the heart will improve, stay the same or get worse after a liver transplant. However, material I have read indicates the GI function will likely get better. Thanks for your essay.
Your story is amazing and so much like the symptoms showed by my son. He is only 40 years old and was diagnosed in Dec 2012 with amyloidosis and multiple myeloma Like you he has severe cardiac involvement that is scaring me to death like you his cardiologist has put him on coreg Twice now he has passed out and has fallen on his his face Once while walking our dog on our steet,ending up in the hospital The other night it happened again He also has kidney and GI tract involvement They say he is not a candidate as yet for SCT because of his poor cardiac condition At present he is receiving treatment at a wonderfull cancer center in Tampa FL Moffitt He is on velcade.cytoxin/dex I look forward to reading your book
Dear Aunt Ngaire, I have been looking for you on Facebook, but never had much luck, I did however find you on Twitter, I read Kevin and Barbie-Our Journey of Hope, I am so very sorry about Charlie, I dont know what to say, How is he doing, and also How are you? I can imagine how difficult this must be for you, please keep in touch when you can, I moved just recently and cant find what my Mom did with our phone books, All my Prayers and thoughts are with you and Charlie. God Bless you both. ~Debby
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