Wednesday, December 31, 2008
The Velcade and Dexamethasone have side-effects. Mostly causing fatigue and bloating on day 2 and 3 after the dose. Those days I am functional, but not fun to be around. I have not had significant neuropathy from the drugs, just the usual numbness and tingling in my hands, feet and lips. What annoys me most is that my teeth are super-sensitive to cold, but at least food tastes good, too good, I have gained 15 lbs. in 4 weeks. I wish that I could say it is all muscle, but, alas, dexamethasone, besides causing a 'moon' face, also causes truncal obesity. So if I appear fat and in a bad mood, you can blame the steroids.
Today, however, I feel great. I am off the chemo for two weeks and hope to enjoy every day of my break. Tomorrow we go to Tahoe to go snowmobiling.
My hopes for 2009 are:
1. See my amyloidosis go into remission
2. Get a Stem Cell Transplant
3. Return to work in February
4. Help Barbie achieve her goals
5. See my children progress on their own paths.
6. Ride the Foxy's Fall Metric Century (100 Km bike ride out of Davis, CA)
7. Get to the point where my health is not the major part of the conversation.
8. Start giving back again.
2009 is going to be a great year.
Monday, December 22, 2008
It is impossible to describe the cumulative effect on our lives of the combined faith of so many wonderful people. We have been so very blessed. I can witness that in every step we have taken, miracles have occurred. But the greatest miracle to me is to realize the magnitude of the love that I have felt from so many. We go through our lives and don't always realize how many people we have touched, how many remember and care about us. I am just one person, and yet, my life has been enriched in having known or met thousands of other individuals over my 49 years. Each person I have known is unique and has added to the person that I have become. I will never feel alone.
Barbie and I also recognize the impact of faith and prayer as it as blessed our family. Our children our at peace as they know that we will continue to do well and remain together as a family. We have felt this power carry us through each step in our journey.
People often ask how I am doing. Barbie will attest that I do have some difficult days, often related to side effects from the medications, but, overall, I am doing great. I feel no pain. Blood tests, procedures and even surgery have not really hurt, because each one has either increased our understanding of my diagnosis, or served to reverse its effects on my health. I feel I can handle anything if it helps to improve my condition. Also, I would rather have a diagnosis of a potentially fatal disease than to remain in darkness as to my perpetual failing health. This light of discovery has additionally lit the path that confirms I am in God's care.
I have learned much from this experience. I have completely put my trust in God. I may not live as long as I expected to, but I will live as long as I need to. Also, given the faith of so many on our behalf, I expect to live longer than my diagnosis normally allows. But, what I have really learned is that I would rather live just one day feeling loved than 1000 days feeling alone.
This is the greatest gift I could have ever received which makes this the most wonderful Christmas ever.
To all who have shared their thoughts and prayers on our behalf, I thank you from the depths of my heart and wish you a very joyous Christmas.
Wednesday, December 17, 2008
1: "I don't want my body mutilated." I find this very interesting given the fact that, in California, if you suddenly die, not under the care of a physician (i.e. any accident), you are required to have an autopsy. Only the coroner can waive that. Prior to medical school, I was a Denier. In French that translates to 'keeper of the morgue'. It was after participating in my first autopsy that I determined I would forever be an organ donor. Transplant surgeons would be somewhat more delicate in harvesting my organs than would the coroner. When the autopsy was over, I put everything in a bag and put it back in the cavity and sewed up the body. I figured that organ donation would be less disruptive.
2: "For religious reasons, everything has to stay together for the resurrection." To that I always ask, "Which matter will be used in your resurrection, your 35 year old matter or your 73 year old matter?" Everything we eat, the air we breath changes the composition of our body every day. Physically, a large portion of the molecules in me today weren't there 20 years ago. They were earth, oceans, clouds and other stuff. The matter doesn't matter. My guess is that God uses the 'blueprint' and rebuilds us with perfect materials. When we die, we decompose back to the earth from which we were created. There is nothing particularly sacred about those elements other than the memory of what they represented. All major religions both support and encourage organ donation.
3. "I don't want the doctor to let me die too soon, just to get my organs." This will never happen. As physicians, are primary responsibility is to save our patients. There is zero incentive for us to have transplant harvesting as any motivation to alter our care for any patient. This misconception can arise from a misunderstanding of 'when we die' and is often exacerbated by how this process is depicted on TV medical dramas. We die when our brain dies, not when our heart stops. The heart does not need the brain to beat. Case in point, there are no nerves connecting my heart to my brain. It began beating spontaneously when my surgeon placed it in my chest and supplied it with oxygenated blood.
When the brain is damaged so severely that is is no longer viable, we die. However, if there is still a blood pressure and oxygen getting to the heart, it will continue beating, for a time.
Of course, this is an extremely emotional time for the family; underlying the importance of having made the choice to be a donor prior to the moment of loss. I have 100% confidence in this process.
The true reason that we should be donors is because of how much good is generated by such an action. It not only saves the individual receiving the organ, but also affects thousands of lives of those who will helped by their continued existence. Like a ripple in a pond, the gift continues to expand. Heart disease, kidney failure, diabetes, liver failure, lung disease and blindness can all be corrected. A friend of mine, a urologist from Connecticut, told me that both his kidneys were healthy and had served him well for 70 years. He felt he could live just fine on just one kidney. He became an anonymous live kidney donor. What fulfillment he must have felt. Being a designated organ donor can provide the same satisfaction, but without the pain.
It is simple to become a donor. Most DMV's allow you to designate your decision on your license. In California you can also sign-up online at the California Transplant Donor Network (CTDN). Once you have made this choice, you should let your family know your wishes to avoid later confusion.
Give Life, Be a Donor.
Saturday, December 6, 2008
It seems that you can use humor to apply to the group to which you belong. Hence, cancer patients often will use humor to lighten the gravity of their condition.
Yesterday, I was sitting in the Roseville Oncology infusion center. My magnesium was being replaced as my Prograf strips me of my magnesium and I can't keep up with pills alone. I was also getting my 1st dose of Velcade to treat the Amyloidosis, since I can no longer take Revlimid. The woman sitting to my left looked like she had cancer, which is a fair assumption in the cancer center. She was bald and her head was covered with the scarf often used by women in this condition. She was talking with her friend (the chairs are very close together) and she mentioned that she would be attending a new ward. I got this big grin. Members of the Church of Jesus Christ (LDS) refer to congregations as wards. She saw my smile and said. Are you LDS? The conversation ensued, which it always does, to search for common acquaintances. In our church we are connected not my 6 degrees of separation, but rather, more like 3 degrees of separation.
She told me that she had metastatic ovarian cancer that had metastasized to he peritoneal cavity and had caused bowel obstruction. She was supposed to die already, but the GynOncology surgeons removed the obstructing tumors and the chemotherapy shrunk the rest. She feels very blest.
I made the comment that Kaiser is the best place in the country to get medical care; (yes I am biased, but it is why I work here.) Now it was the turn for the woman on my right to interject. Her husband was in the chair next to me receiving therapy and she recounted his story and how she felt my statement wasn't just hyperbole. However, once she found out I was a Urologist, the consult began. The man was paralyzed from an infection of the spine and his bladder didn't work, so he was taught to catheterize himself 3 times a day. She asked if he should be seen by a Urologist. To which I responded, "You're seeing one now." I asked the usual questions, got his medical record number and told her that I would arrange follow up for him to have bladder pressure studies in our clinic and to see me in February.
It amazing what you can experience when you are a captive audience in a transfusion clinic.
So why is this funny? The woman to my left with cancer put a video on YouTube of her dancing in her glorious baldness, like the guy from the Six-Flags commercial entitled "Fun with Chemo"
Check it out. I find that humor is essential to coping with these types of situations, although timing and appropriateness are key. Being able to laugh at ourselves is a better pain reliever than any pill and with fewer side effects. Just don't make it too funny, or you'll get the response, "You're killing me" or "I just bust a gut" or "That was dead on" or "I just died laughing" or, you get the point.
Thursday, December 4, 2008
A letter arrived the other day
From the parents of my donor
The pain in its penning twice mentioned.
Their mourning continues
At the loss of their son
So also their love remains strong.
With mortal existence cut short
A vital part of him lives on
His name was Shane.
Even in their pain his parents wish my happiness
A gift doubled in their charity
Such sacrifice inexpressible
My duty to them, to him
In each act of kindness I commit
May I shorten their mourning
Pain replaced by comfort
In time that they will again see
The beauty of the trees
The majesty of the clouds
And feel joy in the light of a new day.
Monday, November 24, 2008
When we got to the venue I realized that if our seats were one more row back, we would be free-falling outside the wall of the building. But, at least, it put my new heart to the test as I climbed 6 flights of stairs without collapsing.
The concert was amazing. To hear all of my favorites from Clocks to I'll Fix You as Chris Martin leaped around the stage with with his signature side-ways glances at the audience was just totally cool. It was great fun to be there with Jeremy, Alexandria, Samuel, Michelle, Caitlin and Barbie.
Then began the pulsating beat of strings, guitars, drums and a 'Jerusalem Bell' resonating at the tempo of a heartbeat. What a contrast from four months ago as we then also listened to the title song of the album while driving from Santa Clara on the day of my first biopsy. The tears we felt then were now replaced by the joy of hearing the same song together as a family. The three words of the title with, now, with a new meaning for me: Viva la Vida or Long Live Life.
Enjoy life today.
Thursday, November 20, 2008
Tuesday, November 18, 2008
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
Saturday, November 15, 2008
Wednesday, November 12, 2008
Songs are different. An unexpected song comes on the radio or Ipod in random mode, and you are immediately taken to a specific place and time; and the emotion of that moment washes over you uncontrollably. This is only amplified if you are on steroids. Steroids bring your emotions to just under the surface so that, as I tell Barbie, they make me cry at toilet paper commercials. That 'quilty' softness gets me every time. So on days like today, when I take 40 mg of Decadron, I feel it.
There are five songs that make me cry. (This qualifies me for a "High Fidelity" top five list.) The first, (in chronological order) is "100 years" by Five for Fighting. That was the day that I had made the decision to leave Yale and Connecticut to move to back to California. The second is "Viva la Vida" by Coldplay. Barbie and were driving home from Santa Clara having just been diagnosed with Amyloidosis and told that I needed a heart transplant. The third is "Let Love In" by The Goo Goo Dolls. That day I was alone in the hospital, as Barbie had to leave me to go to Sam and Michelles' wedding and we had to pass on the heart from the Mayo Clinic. The fourth was when Caitlin came to Stanford, just after my transplant, and sang the song "Waiting" that she wrote for my birthday. The fifth was tonight, while taking Caitlin to piano lessons and hearing Imogen Heap sing "Hide and Seek" knowing that tomorrow I have to go back on high dose steroids because my heart is once again in moderate 2R/3A rejection. And we don't know why.
Generally, with every side effect I get, I try to connect it with something that is temporary, reversible or fixable; be it drug reactions, low magnesium or diet and exertion. But this time, I am out of connections. I can only trust in my doctors and keep my faith in God and be patient. I go back on 100 mg of Prednisone tomorrow for 3 days with a rapid taper. Beyond that, I cannot predict. But don't pray for me, pray for Barbie, she's the one who has to live with me.
We had planned on going to Utah to see Coldplay in concert and stay for Thanksgiving, It might end up being Boston Market in Roseville. (I actually love their turkey dinner, it's all the salt.) Seriously, though, I am actually feeling great, both physically and emotionally. I have the best doctors and the best family. I know everything will work out well. it always does. And there are so many songs yet to be written.
Tuesday, November 11, 2008
Sunday, November 9, 2008
Last week when I went to see my cardiologist, Dr Weisshaar, in Santa Clara, the pressing question was what to do next with me. Dr Weisshaar called and spoke with Dr Lacy, the oncologist that saw me at the Mayo Clinic and she did confirm that Revlimid could lead to heart rejection. She also added that waiting untreated for the Stem Cell Transplant sometimes might allow the amyloid to progress as amyloid is quite unpredictable. As I heard this, they were like more pieces of a puzzle that began to reveal the underlying picture, which in this case was a plan to proceed. There are 3 drugs that are commonly used to treat amyloidosis, Revlimid, (which I probably won't take again) Velcade and Melphalan. The latter can suppress stem cells and is therefore not a great option if you plan on proceeding to transplant. This leaves Velcade. Initially, I was hoping just to be treated with medication and avoid a stem cell transplant, but I feel that a transplant is probably likely and, actually, I would accept that if it means a more permanent cure or control of the disease without having to continue to take steroids. I really don't like the idea of being on steroids for a long time. My doctors met on Friday to arrive at a plan which I will learn of on Tuesday. I am prepared to do what they recommend, but I hope it includes interim chemotherapy until my heart is healthy enough to have the stem cell transplant. I will continue to take the weekly high-dose steroids (Decadron) to at least remind my misbehaving plasma cells that I am aware of their shenanigans.
Otherwise, I feel great. My strength is returning; I walked two miles today. Food tastes wonderful and I am not such an impatient 'toad' to Barbie. They warned me that the steroids would do this, but I thought that I could control it. Well, I was wrong.
I love the process of discovery, even if I am the one being used to reveal the right path. Like I tell, Debbie, "I'd rather it be me than you." If what we learn from me makes it easier for her, it's worth it. Nothing makes you feel more alive than taking risks.
Can't wait to see what's next.
Wednesday, November 5, 2008
I am feeling great. I believe that I have mostly recovered from the high-dose steroids. I have also had some severe dizzy spells and muscle weakness; then I saw that my magnesium levels were very low and this was likely the cause. Prograf causes you to waste magnesium and even though I was taking 3200 mg a day, it was not enough. I take more now. Also my doctor felt it was likely exacerbated by an inner ear issue and started Claritin and Nasarel and this has also helped. Today, I forgot that I was ill, I love those days.
A young man, a patient of mine, was recently admitted to the hospital, his grandmother commented to one of the nurses there on 3 South, named Windy (she is a great nurse, but, of course, so are also the nurses that I have met at Kaiser), how it was sad that Dr Anderson left his practice and had abandoned her grandson. Windy reassured her and showed her the web-site (Its mere existence being my own personal HIPPA waiver. In my case, personal knowledge of my medical condition actually has been helpful to others and continues keep alive my desire to teach.) She was both moved and relieved that my absence was not of my own choice.
While getting labs, I dropped by to visit this young man and reassured him that I would always be his doctor. I am very concerned about him. Then it occurred to me, there must be other patients who feel the same way. If anyone knows any of these patients, please reassure them for me that I am still here, my goal is to return to work and I will remain their doctor as long as I can. I also wish to express my deep thanks to my partners for their continued care of my patients.
After visiting this young man, I stopped by operating room to see Susan, a friend there, that had a gift for me. It was base-relief sculpture of the 'tree of life'. What a beautiful gift. This actually reflects a deep personal symbolism in my life that I alluded to previously, but is unknown to her. I thank her for it.
I looked at the board where all the cases were listed and had this desire to put on some scrubs and get back to work, but alas, I am an obedient and compliant patient and will abide by my doctor's orders and wait until she give me the 'green light' to return to work. Right now she is saying February. The most difficult day ever was when she told me to shut down my practice, it felt like my heart was torn out that day. Caring for others is who I am, I felt like I lost a piece of me that day. My next happiest day will be when I find myself with a ureteroscope in one hand and a 200 micron Holmium laser fiber in the other while blasting an elusive ureteral stone, what joy. Not as Bruce Willis in Armageddon, but like Michelangelo (I always tell my residents, don't drill the stone, sculpt it.)
To all of my patients, I will return and remain your physician as long as I can. This is my goal, this is my hope.
This gift from Drs' Stapp, Nanigian and Takahashi included a donation to plant 30 trees around the world.
Thursday, October 30, 2008
Mostly dead is what happens to you after 5 days on 100 mg of Prednisone, followed by one day of 40 mg of Decadron (200 mg prednisone equivalent) followed by 3 days of 1000 mg of IV solumedrol (Prednisone equivalent to an "uberdose") I felt wonderful at the wedding and reception, though, as I was lifted by my daughter's beauty, exuberance and infectious smile. But once she an Corey were gone, so was my strength. I gave myself the last dose of solumedrol, at home, in the IV left in me by the nurse.
The next three days, I couldn't move. It required huge mental effort just to get out of a chair. Speaking was a chore, and eating was impossible since everything tasted horrific. Note to self: Avoid high dose steroids in the future.
But then, with a finger wiggle and a head giggle, life began to return. Now, lets inventory our assets.
This all began two weeks ago with a biopsy that showed moderate rejection, the following week it was worse. This concerned my doctors greatly because my anti-rejection drugs were supra therapeutic. Phone calls flew around the country to try to figure this out, meanwhile the only treatment option was to go 'medieval' on the steroids. The Revlimid was stopped on the chance it might be interfering. The jury is still out on that question.
But me, being me, I always expect things to improve and they have. The heart biopsy last week showed no evidence Amyloid deposits, and the heart biopsy this week shows marked improvement with the rejection now at a 1a. I will take a month off of chemotherapy to let my heart get better, and then, who knows? I am still on bolus Decadron weekly on the chance it may keep the amyloidosis at bay. My Kappa Light Chains (amyloid marker) remain low.
Many questions remain, however. Was it really the Revlimid that caused my strange rejection? Was it an occult infection, masked by steroids? Was it the economy or election season hysteria? I am sure my doctors must see me as two people since I am a paradox. But then, I have always been unique. But I don't worry; if not plan B, then plan C and eventually on to plan 401K.
In the end, I am always aware of the sincere support of so many on my behalf, this makes the bad days bearable and the good days glorious. I haven't written recently because, well, I was mostly dead, Miracle Max did offer me a cure, but it took a while to swallow, as the chocolate was still quite unpalatable. Today, chocolate tastes wonderful, and that says a mouthful.
Glad to be among the living.
If you haven't seen The Princess Bride, it is worth watching.
Wednesday, October 29, 2008
Life is measured in events. Albeit, these events are rare compared with our day to day activities. But these are what we remember, mark and measure our life. These events can be seen as islands on the horizon in the ocean of time on which we spend most of our days. We float along daily in the glistening ripples and waves of daily activity, the forward movement almost imperceptible as we sleep, eat breakfast, answer emails discuss the calendar and talk and listen and do 95% of what our lives really are. These moments are critical, but seem mundane. However, the big events are both behind us and ahead, a reminder of what we have accomplished or hope to do. The islands in our ocean fade into past memory and appear anew on our horizons: Birth, death, prom night, 1st football game, a major illness, a family vacation, a promotion, new job, new house. As a parent, they are an inevitability. Someday, your children grow up and leave you. You prepare them for this their entire life, but it is always bittersweet.
As a father, you hope and pray that your daughter falls in love with a good man, a man better that you are, one who will respect and care for her, but as she dates, you give up hope. Then one day, she comes home with a sparkle in her eye. This is different. This young man is not like the rest. You meet him and he is was she needs and more. Suddenly, on the horizon, a distant peak of an island becomes visible for the very first time and you feel the currents pushing you toward that inevitable port. However, almost simultaneously, storm clouds begin to gather obscuring the view and the way.
On June 13, 2008 Corey proposed to Rebecca. Corey included me in his plan to surprise her and it worked beautifully. She accepted and the date was set for October 25th. Ironically, that same morning, I finally found out my diagnosis of what had kept me ill for two years. I had restrictive cardiomyopathy. A week later it was determined that this might be due to Amyloidosis. Further investigation was disheartening as the prognosis was bad and the course of action unpredictable. The storm clouds descended and the waves begin to carry us off course. With two of our children getting married in the near future, it was difficult to know what to do. Samuel and Michelle's wedding was only 2 months away, while Corey and Rebecca's was in four months. With a open-ended work-up in Rochester MN, inpatient status in Santa Clara and waiting for the elusive new heart, could we make a course correction in time to share these cherished events. To Sam I said, "I'll do my best to be there." It was not to be. But to Rebecca I promised, "I will dance with you at your wedding." I meant to do everything in my power to keep that promise.
We arrived at the appointed date last Saturday. The beauty of the morning was only exceeded by the beauty of Rebecca. Her smile and unabashed joy kept us all afloat. The wedding was a dream come true. We all arrived, family and friends at the reception where she floated around the room in her white dress with such grace and poise. Then the moment came. I danced with my daughter.
I took her in my arms, as I have done for 20 years, and carried her around the floor. She told me not to cry, but the emotion was overwhelming. We reminisced how she used to stand on my feet when she was two as we danced around the family room. She loves to dance. I told her how much I loved her. I always will. But now begins her time to dance with another. And I am so happy that she has Corey. A fathers' greatest desire, to give his daughter to a man worthy of her love who will care for her with all of the loving attention of her Dad.
The dance with my daughter was done, the event now fades into past memories, the promise was kept. As I look back, this island paradise will remain in view for a very long time. The ocean seems a little bit more calm today and my vision seems to extend a few more miles than usual.
Congratulations to Rebecca and Corey
Friday, October 24, 2008
I feel this way right now. since Stanford hasn't treated an amyloid patient after a transplant in many years, it is unknown what the chemotherapy does to the new heart. Just as well, my doctors in Santa Clara are experts at protecting the transplant, but have not taken care of an amyloid patient either after transplant. They were all very concerned that my rejection did not resolve after two weeks of intensive intervention. Yesterday everyone was talking from Santa Clara Kaiser, Standford and the Mayo Clinic. The questions involved drugs levels, drug interactions and ultimately one pharmacist suggested that Revlimid might increase T-Cell activity and IL-2 levels. This would have a direct toxic effect on my heart despite high levels of anti-rejection drug activity. This was unexpected as the usual literature doesn't mention this. This trail blaze was missing.
As a result, I have stopped the Revlimid for now and am again on high dose steroids in the form of an IV infusion of Solumedrol for 3 days. I imagine that within a week this will all resolve and I will be back on the correct path. I guess someone has to blaze the new trail. It will be more clear for the next person who follows.