Dear Friends and Family,
I woke up this morning earlier than normal and realized what a lucky man I have been. Today is the fourth anniversary of my heart transplant. Easter Sunday on 24 April 2011 is etched in my memory as the start of a new life. The past four years seem to have flown by, although largely filled up with visits to hospitals and clinics for treatments and surgeries. I have accepted this new lifestyle as the new norm. I was really hoping that this fourth year would be relatively quiet on the health care front, but that did not prove to be true.
Looking back to my last Summer update, I'd like to bring you up to speed on the latest. I had mentioned that I required a revision knee surgery following the total knee replacement, and that happened last July, with four days Stanford inpatient and no complications. Extensive physical therapy continued afterwards, and really has become a daily requirement to keep the leg strong and artificial knee as mobile as possible.
I was beginning to get quite active and attempting to get back into shape when I got a case of the shingles last October. That landed me back in Stanford inpatient for four days. My cardiologist team tends to get rather nervous and protective of me whenever I come down with any fever, virus, bug or whatever. They watched me closely until the shingles began to vanish. It was an unusual case, in that the rash covered my whole body, versus just a typical band or isolated strip. Looked more like chicken pox to me, but they said it was definitely shingles. For those of you over 60, you may want to consider getting the shingles vaccine. Try to avoid the unpleasantness at all costs.
By Christmas, I was finally starting to get back into shape, moving my knee a bit better, able to start riding my bike fairly regularly, and hitting the gym to try and get some overall strength back. I figured my bad luck had run out and the New Year was going to be smooth sailing. I was wrong.
In mid-January, I came down with a week of fevers, which landed me back in Stanford inpatient, when they quickly discovered I had pneumonia. They kept me inpatient for two weeks, administering IV antibiotics and a number of other tests and studies, but were not able to positively determine what strain of virus I had. It felt like a jail break, when they finally let me go home, with two more weeks of oral antibiotics to follow. The low grade fevers and night sweats continued for yet another month, and finally resolved in late March, while Linda and I were half way through a nice 10 day vacation in Oahu. I figured the Island climate must have finally broken the superbug.
Arriving back home, free of pneumonia, I once again started to try and regain some form of fitness, after two months of little activity due to constant fatigue and low energy. I planned to be back in great shape by May/June. Guess what...I was wrong again.
After returning from my monthly clinical trial at Stanford on April Fool's Day, (yes, I'm still on the NEOD001 trial), I woke up the next morning with an unusually sharp stomach pain on my left side. I dismissed it at first, as simple poor digestion, and let it go for a day. The next day, the pain had not diminished and I noticed my abdomen had become quite bloated, along with losing my appetite. By late in the day, I asked Linda to take me to the nearest ER, which is a good thing, as they had diagnosed a ruptured spleen within one hour of my arrival. I was told to expect emergency surgery within the next few hours. After 7 units of transfused blood before and during surgery, I had a successful splenectomy on Easter morning. That was followed by a week in the ICU. Turns out I had been bleeding profusely internally, and the surgeon estimated I may only have survived a few more hours if I had not checked into the ER. Is this Easter surgery thing becoming a trend?
So what caused the spleen to rupture? Normally, this happens during a car accident or some type of blunt force trauma to the stomach area, but that wasn't the case for me. After surgery, the pathology report indicated there was amyloid involvement in the spleen. Although not confirmed as an absolute, my guess is that amyloid tissue infiltrated the spleen, weakened the organ, and over time caused it to ultimately rupture. I hope to get further confirmation on my theory in the near term. Meanwhile, my recovery is a long process which requires little activity and lots of rest. I have become quite good a daytime naps. My healing progress is on track, and the 36 staples were removed last week. I now have an attractive scar from my throat, straight down the middle of my torso almost to the groin area, where the heart transplant incision connects neatly with the latest opening...sort of like a long zipper in front.
So, as the story goes, in a few weeks, after my surgeon gives me the green light, I will start working out again, regain some fitness and get on with my life, not expecting any more of these surprise health setbacks. As I always say...never give up.
Aside from all this "noise" in the background, my four-year heart is doing quite well, as measured by cardiology checks/tests about every four months. Good strong ejection fraction of 65% at my last echocardiogram. I remain so lucky to have received such a precious gift from my donor.
As mentioned earlier, I have been on the Prothena clinical trail with their antibody drug coded NEOD001 for nearly one year. My 12th cycle is scheduled for next week at Stanford. I actually entered on the tail end of the Phase 1 study, and so I am assured of actually getting the drug, whereas the Phase 2/3 started last fall, and 50% of the cohorts were given a placebo. I have experienced no side effects, and my kidney function has remained stable. With amyloidosis, "stable" is a very good word, as many patients experience steady deterioration in affected organ function. To date, there remains no known cure for systemic AL. However, there is yet another new promising FDA approved drug that my Hematologist is looking at shifting me over to later in the year, but no confirmation yet on whether I will be a good candidate. We will stay the course with NEOD001 in the mean time. My lambda light chains have remained relatively stable, too, oscillating in the 4-8 range.
That's it for now. I am very lucky to have such outstanding physicians and health care providers, but I am convinced that the power of prayers and your constant support and friendship has been equally important in my survival over the past four years. Linda and Nathan remain totally committed to my care and needs here at home, and they are my anchor.
I really hope that at my fifth year anniversary, I have nothing but great news to report. Until that time, best wishes and health to you and your families, and have a great spring.
p.s. feel free to forward to those who may be interested. (I am not on Facebook).
Patient Testimonial for Stanford's Amyloidosis Website
Prepared by: Paul Langlois
As I write this in February 2012, I am 57 years old, and have been a survivor of Primary AL Cardiac Amyloidosis for one year since diagnosis. The point of my testimonial is to offer hope, information, and encouragement to anyone who is faced with this disease, whether they are the patient, family member, or Caregiver. I was lucky, in that I was likely diagnosed earlier than most, which can be a real lifesaver.
I would like to give a little background about myself before I was diagnosed. I was a very healthy and fit endurance athlete (cycling, triathlon, marathon runner, oarsman), and competed in my age category my entire life, even winning several national titles. I never smoked. I always ate right. My body weight throughout adult life was the same as when I competed in college. I have no family history of heart disease. I spent over 30 years on active duty in the Coast Guard, mostly as a rescue helicopter pilot, Aero Engineer, and retired at the rank of Captain in 2006. I then resumed flying and worked for REACH Air Medical Services in Santa Rosa, California, as an Emergency Medical Services (EMS) helo pilot for five years. As an aviator, I was required to have routine annual physicals and medical checks to ensure I was healthy. I would never have believed I was soon going to be in a battle to stay alive.
In late 2009, I noticed I was having trouble climbing uphill or even keeping a moderate pace on cycling training rides with my team. But when I was at rest or at work, I exhibited no symptoms. At that time, I went to my family doctor for routine annual checks and labs, but pushed him into seeking further tests. In early 2010, I was given a cardio treadmill stress test and a pulmonary function test, with both results showing I was normal (Note: Endurance athletes may not test similar to the rest of the aging population which is typically sedentary). I had also thought I might be developing some form of asthma or allergy, since my heart and lungs were otherwise "tested" to be working normally -- 2010 was a very frustrating year, as my cycling performance deteriorated significantly, and I could not train hard enough to race anymore. Something was very wrong with me, yet my doctors told me I was in top physical condition, and I was simply "getting older".
By late 2010, I had trouble walking uphill without quickly becoming breathless. I had nearly given up bike riding in disgust, for how weak I had become. I noticed fluid forming in my legs and ankles. I began to have episodes of nearly fainting when I stood up from a chair. I noticed I could not sleep at night with just one pillow, as I would choke and wake up feeling like I was drowning. My annual visit to my doctor in late 2010 had me pushing him very hard toward looking further into my problems, and he sent me out for some special lab work, as he noticed some extremely elevated protein in my urine test.
Before waiting for those lab results to come back, I checked into my local Emergency Room, the day after Christmas 2010. I was lucky, in that the duty Cardiologist did a thorough history review with me, and after performing an EKG, Chest X-ray and an Echo-cardiogram, reported that I had severe cardiomyopathy (Congestive Heart Failure). I couldn't believe the news I had just been given, and immediately had to quit working as a pilot, surrendering my aviation medical certificate to the FAA, and calling my employer with the very surprising and grim news.
My Cardiologist called me into his clinic two days after the ER visit, and told me that due to the high protein levels in my urine, he suspected Amyloidosis, of which I knew nothing. He believed this rare blood disease may have caused my heart function to deteriorate, due to the infiltration of amyloid deposits. My anxiety reached a new level. He ordered a heart biopsy, for which he advised was the only way to confirm this disease, using the standard Congo Red stain test. The biopsy test came back positive a week later, and my new life with Amyloidosis began in earnest. (I didn't realize it at the time, but my Cardiologist had saved my life by making the correct diagnosis so quickly).
Naturally, I was devastated, like everyone else who gets such news about acquiring this rare disease. I immediately had a bone marrow biopsy with a local hematologist/oncologist, and he suggested I seek an appointment ASAP to Stanford, the Mayo Clinic, or Boston University, all of which had Amyloidosis treatment clinical expertise. I opted for Stanford since the facility was only two hours (100 miles)
from my home, and it sounded like treatment plans may require me to live away from home for long periods. Fortunately, I had health insurance which would cover most of the expenses. In early February 2011, I was initially seen at Stanford by Dr.Witteles (Cardiology) and Dr. Schrier (Hematology), who are the Co-Directors of their Amyloidosis Center.
I was advised by Dr. Witteles that my heart was in such dire condition that I would need surgery immediately for an Implantable Cardioverter Defibrillator (ICD), to hopefully prevent any ventricular fibrillation, which could be life threatening. He also told me that I would need to consider heart transplant if I wanted to survive much longer (i.e. months). I was told that I would need to arrange for a full time Caregiver tending to my future needs during the heart transplant recovery period, or I would not be eligible. Of course, my wife Linda, and my son Nathan, both agreed to assist me.
The next day, Dr. Schrier confirmed that I definitely had Primary Amyloidosis, and that the urgent initial concern was restoring my heart function, in which he had to defer to the Cardiology Dept. Through various tests, it appeared my other organs were not in a dysfunctional state, and he immediately placed me on a regimen of chemotherapy and steroids to hopefully reduce the spread of the Amyloids. Discussion of a stem cell transplant was mentioned, but not recommended from the onset, as my failing heart would likely not tolerate the stress of such a procedure. My chemo regimen consisted of three weeks of Revlimid/Dexamethasone, followed by one week off. The weekly dosages of Rev were increased each month from 5, 10, 15mg, with Dex staying steady at 40mg. I learned that my critical blood chemistry value of lambda free light chain (Amyloid) was initially measured at a concentration of 28, and that we ultimately wanted to see this number drop to less than 2.
I agreed to the ICD surgery (March 2011), and simultaneously commenced a vigorous six weeks of pre-screening tests for heart transplant qualification, made more restrictive due to my Amyloidosis condition. If the pre-screening tests had demonstrated that I had any other organ dysfunction, the Stanford heart transplant committee would most likely not have approved my transplant. There were two invasive tests done, including a right heart catheterization, and an upper/lower GI exam. With my great fortune, the committee approved my transplant for Priority Level 1B, and I surprisingly only had to wait eight days until I "got the call" that a donor's heart had become available. I had a successful heart transplant on Easter Sunday (24 April 2011) under the skillful hands of Dr. Oyer and his expert team at Stanford.
After surgery recovery in two levels of Intensive Care Units, I was discharged from the hospital in only eight days, but was required to live nearby Stanford for many weeks, due to frequent check-ups and to quickly mitigate any emergency complications, for which I had few. Stanford protocol dictates patients remain close by for up to three months post-transplant. I was required to take daily immuno-supressants, various medicines to prevent infections, and supplements typically recommended for heart transplant patients, which continues to this day and beyond. The dosages and medications are managed very closely by the post-heart transplant team through routine blood draws.
Two weeks after heart transplant, I was placed on a new chemo regimen of Velcade and Dex, as the earlier Revlimid was considered a poor drug choice for heart transplant patients. Dr. Schrier advised that I would need to wait about six months before the possibility of having a Stem Cell Transplant -- all the risks and side effects (including possible death) were presented for my full awareness by Dr. Arai in the Blood and Marrow Transplant Center (BMT). With hope, the Velcade treatment would begin to lower my light chain levels, such that I may not even need the stem cell transplant. Only time would tell the next step in my treatment plan.
Soon after heart transplant, I tried to get into a daily routine of walking with Linda, which was made a bit more difficult due to having to wear a protective mask for many weeks. The mask serves to restrict inhaling any particulates that could cause infection. Much to my surprise, only six weeks after transplant, I was able to walk for 5 miles on a hilly trail around the popular Stanford Radio-telescope park. I was amazed. I soon realized that frequent exercise and maintaining good circulation was very helpful for recovery from surgery and treatment. As the weeks went by and I was finally able to return back home, I was happy that I could once again ride my bike long distances, and get back into the gym for strength training to reverse the muscle atrophy. I also started doing some Yoga classes, along with the walking. Although I was recovering very nicely from the heart transplant, I could not help but think that the heart transplant was only Step 1 in my journey, and knew the unwanted Amyloid proteins were still in my blood -- I definitely did not want them attacking my new heart. I wrote a letter to the family of the donor at six months after the transplant, seeking to thank them for their precious gift of life, but have not heard back -- organ donation is such a private and personal matter.
In August 2011, at a monthly appointment with Hematology, Dr. Schrier advised that it was time to
make a decision on whether I wanted to move forward with the Stem Cell Transplant. As it turned out, the past four months of Velcade/Dex chemo treatment had lowered my lambda free light chain count to a lowest point of 8, but had then risen back up to the 13-15 range, indicating the chemo had been somewhat useful, but not fully effective for my needs. We all agreed that moving forward with a stem cell transplant was in my best interest, and that my body and new heart had finally become strong enough to endure this difficult treatment. I knew the risks, and I knew the recovery would not be easy.
In early October 2011, I began the long process of Autologous Stem Cell Transplant at Stanford. Although I was treated superbly, it was a very difficult process with almost three weeks inpatient, followed by a few weeks living nearby the hospital. The recovery period was quite unpleasant, and might have even been more difficult than the heart transplant recovery, but as each week passed, I became stronger and slowly started to feel better. I got back into my exercise routine as soon as possible when I got back home, despite some days of extreme fatigue, nausea, pain, including frequent migraine headaches. I was told that it would take about three months for the transplant results to become apparent.
In January 2012, after anxiously awaiting some positive news on the outcome, I learned that my lambda free light chain numbers were still at the mid-teen level, versus a desired reduction to less than 2. My conclusion was that the transplant did not work for me. How disheartened I was. Unfortunately, the procedure is not effective for everyone. Despite this psychological setback, I have been feeling better each week, and always keep a positive mindset. I continue to trust that my Stanford doctors, nurses and the entire hospital team are very knowledgeable and doing the right thing for me - simply put, they have all been fabulous.
As I conclude this testimonial, I am currently awaiting recommendations from my Stanford doctors on what the next recommended treatment plan may be for me, most likely resuming a chemo regimen with Velcade. Quite obviously, I have not been able to resume employment as a pilot, and simply tell people I am retired, and otherwise on disability. Not being an active member of the working community has been difficult for me, but I have adapted. I like to think of my future as being a long term survivor, yet at the same time very lucky to have made it to the one year point after the Amyloidosis diagnosis. Linda and I plan to book a vacation to Hawaii in a few months, as we had to cancel the trip last year for our 30th anniversary due to my heart transplant.
I would be willing to discuss my situation with anyone who has questions or comments. As my journey with Amyloidosis continues, I hope to provide an update to this testimonial. You can reach me at firstname.lastname@example.org.
UPDATE DECEMBER 2012
As the Year 2012 comes to a close, I would like to offer my first update. It goes without saying -- I have beaten the odds - I am still alive after two years since my diagnosis of primary systemic cardiac Amyloidosis. I ended my initial testimonial at the stage where my stem cell transplant results had proven to not be effective. However, m y second year with Amyloidosis has been much easier than the first year, although the journey remains quite challenging. My clinical appointments are now far less frequent, and my ability to cope with many medications and their side effects have become somewhat of a normal routine in my life. All of my doctors, nurses, caregivers, family and friends have kept me in positive spirits through their own unique way of watching over me. I am most grateful to be surrounded by so many special people.
I was very active this past year. Linda, Nathan and I took a 10-day vacation in April to Oahu and Kauai. During one of those days, I surprised myself and was able to hike with Linda down the entire Waikiki Beach strand, and then all the way to the top of the Diamond Head crater, and then back! We had a great time on the beautiful islands. Later in May, the three of us also took a 4-day vacation to Yosemite National Park, and I amazed myself once again by completing several very difficult, steep day-hikes up to Yosemite, Vernal and Nevada Falls - we didn't quite have the time, energy (or ambition) to trek all the way to Half Dome. Later in the summer, Linda and I trained on our road bikes extensively, and we entered a fairly grueling, hilly charity ride in Sonoma County near our home, which had us on the bikes over 5 hours and nearly 70 miles, with 7,500 other riders from around the world. Although my strength, energy level, and power output is far reduced from when I was healthy, I really believe frequent exercise at a reasonable pace can be quite beneficial in controlling this disease. If nothing else, the resulting peace of mind and positive physical aspects have been priceless in helping me to cope.
The status on my heart transplant has been fantastic. With approximately 20 heart biopsies completed in the past two years, I have had no signs of any heart rejection, and I'm told the first year is the critical year if any complications were to show. I continue to take most of the same post-transplant drugs as reported earlier, including immuno-suppressants, anti-virals/bacteria, vitamins, supplements, and other meds to control blood pressure, cholesterol, and bone disintegration. Currently, I travel for scheduled clinical checks and a heart biopsy at Stanford every three months this year, shifting to every four months next year.
Last May, after waiting six months for a response from the letter I sent to the family of my heart donor, I received a letter indicating how grateful they were that I had contacted them. I arranged for a personal meeting at their home to thank them for their teenage son's generosity, who was accidentally killed by a passing motorist while he was walking home from high school with his friends, as they all crossed a city street. It was a very emotional day with their family, and Linda and Nathan accompanied me for the drive to the distant California city. They have become my second family.
And now for the update on my various chemotherapy regimens, with the goal of trying to lessen my free light chains. This past March, after a 3-month reprieve from any therapy following the stem cell transplant recovery, I resumed what would become many 4-week cycles of various combinations of chemotherapy, always accompanied by the steroid Dexamethasone. Dr. Liedtke (Stanford) has become my principal hematologist monitoring my case. Since March, I have been on almost continuous weekly subcutaneous injections of Velcade (3 weeks on/1 week off). Fortunately, I don't have to make the long trip to Stanford to have my weekly injections, but have them administered at a Cancer Clinic close to my home in Santa Rosa. All other chemo drugs prescribed have been administered by me, orally at home.
For just two months this past summer, Cyclophosphamide (Cytoxin) was added to the Velcade, a chemo combination known as CyBorD, which I tolerated quite well. During this past year, my lambda free light chain lab results have oscillated between a high of 16, and hit a low point of 6 at the end of August, after two months on CyBorD. At that point, despite some positive progress with controlling the light chains, I asked Dr. Liedtke for a "vacation" from chemo, as I was simply getting so worn out and fatigued from the constant treatment, that I felt I needed a break. She agreed to one month off. During my month away from treatment, my light chains almost tripled in value, which suggested that I had better stay on some form of chemo to keep the amyloids in check. This is the big dilemma for Amyloidosis patients - while I felt really good during the month off chemo, without having to deal with all the difficult side effects of the medications, the downside is the likely probability of a rise in the concentration of the light chains in the blood, which can exacerbate organ damage.
In October, I began a new, and more aggressive regimen, and was placed on Revlimid (25mg per day for three weeks), along with the standard weekly Velcade and Dex. After two weeks on the Revlimid, I could not tolerate it anymore, due to extreme cramping in my hands, feet, arms, and legs, which rendered me nearly dysfunctional for hours at a time. We agreed in the next cycle to reduce the Revlimid dosage to 15mg per day, to determine if I could tolerate it. The cramping seemed to subside on the lower dose to a more controllable level, but the daily administration of the drug has left me with other side effects, constant fatigue, along with noticeable neuropathy in my hands. But the good news is that this new RVD regimen which I am currently on has reduced my light chain concentration to 5.4, after two complete cycles. This is the lowest light chain value I have seen in two years!! I start the third RVD cycle in early December. Dr. Liedtke would like to see my light chains drop to less than 2. I am not sure if that would mean I would then be in remission, and the chemo could stop for awhile, but I am hoping for this outcome. I have learned that all patients respond differently to these drugs, and that there is no guaranteed outcome on the various methods - I sometimes feel like my body is being used as a chemistry lab experiment. But, I continue to rely on the expertise of all my Stanford doctors.
In my case, perhaps even more troubling than the various chemo drug side effects, was how I reacted to the steroid Dexamethasone. After well over one year on 40mg/week, I asked for a reduction in dosage, and am now on 20mg/week. On the day of my weekly dosage, Dex typically makes me feel somewhat energized, emotionally uplifted, and a bit aggressive, where I can accomplish surprisingly high amounts of exercise or activity. The problem is that when it wears off after a couple of days, I experience deep fatigue, depression, and leg/feet swelling which often put 10 pounds of fluid into my system. This constant weekly cycle of ups and downs had become very difficult to maintain any sense of stability, and it often interfered with my ability to commit to social engagements or travel plans. However, the reduced dosage to 20mg has proven to be much easier to tolerate. The take-away lesson for me in the past year is to positively engage with your doctors and let them know in clear terms how you are being affected by the various drugs - and don't be afraid to ask for a change in protocol if you can't tolerate the current prescription.
I am disappointed to report that over the past year, my renal function has deteriorated significantly to a moderate level (Stage 3) of chronic kidney failure, most likely due to amyloid involvement, but perhaps enhanced by some of the prescribed post-transplant medications, particularly cyclosporine. I also have been experiencing constant anemia throughout the past year. After consulting with Dr. Lafayette (Stanford Nephrology), the only treatment plan recommended for my kidneys would be to simply continue with the chemotherapy already in place. Trying to push the light chains to the lowest possible level is the goal, to lessen (and even reverse?) any further damage. Apparently, the kidneys have a much greater tolerance for organ damage than my original heart. I continue to feel lucky with my overall situation, in that had I been experiencing the kidney dysfunction alongside the original heart failure of two years ago, I would likely not have been qualified for the heart transplant. There has not yet been any discussion that I might need to be placed on dialysis anytime soon, and I sure hope to be able to avoid that requirement. Likewise, the topic of kidney transplant has not yet been a focus at this early stage, but I am told that I may not be eligible anyway, if that day should ever come.
I find it difficult to publically discuss my health situation, but my purpose is not to put myself in the spotlight, or seek any form of sympathy. So many people are sick with various diseases, and many family members and friends unselfishly give of their precious time through the daily chore of caring for the sick. It is part of life. My purpose is to share information with Amyloidosis patients, caregivers, and anyone wanting to learn more about this dreadful disease. Fortunately, Amyloidosis appears to be getting more attention and awareness among the local primary care physicians, along with some potentially exciting new clinical trials among the global medical and pharmaceutical research community. There certainly is hope for the future. By leaving my contact email email@example.com in my first testimonial, I estimate that I have made direct contact with over 50 people from around the world, through email dialogue and follow-on phone calls. Sharing accurate info among our small community with Amyloidosis is the key. If I can help even one more person, this testimonial will be so worthwhile. I plan to report back after my third year of surviving Amyloidosis at this time next year. Cherish every day. Always stay positive. Never give up.
Health Update - Langlois - Summer 2014
Dear Friends and Family,
It's been over 7 months since I sent out an email blast on my latest dealings with battling Amyloidosis. Good news. I am winning. No turning back for me. Against all odds, I feel I am going to beat this thing. All of you are largely responsible for helping me to keep a positive attitude through this turmoil. You have my deepest thanks.
Last fall I told you that I was going in for surgery for a total knee replacement (TKR). I also was going to start a new clinical trial. That's where I will pick up this latest update.
Before my TKR last October, I was required to get off chemo and steroids prior to the surgery. The knee surgery and hospitalization went fine, but I really jinxed myself in my last update when I said that this surgery was going to be a piece of cake, at least compared to my past transplants. After many months of diligent physical therapy and rehabilitation, my right knee has remained far from desired range of motion. I have developed a "stiff knee" syndrome, where the joint has filled up with an very large amount of thick scar tissue. It prevents me from straightening and flexing the knee to expected movement parameters. So....the only solution is to have another open surgery to cut out this scar tissue, and start rehab again. I am scheduled for this revision surgery on 08 July at Stanford. What's this got to do with Amyloidosis? Well, nothing really, but it has become a major distraction in my health challenges over the past half year. I will overcome this sideshow with time. Dr. Maloney is the Orthopedic Dept Head at Stanford, and he will perform my surgery next month.
Only a few short weeks after my return home from knee surgery last November, I began a voluntary clinical trial at Stanford with a new chemo drug being tested for certain Amyloidosis patients, for which I qualified. The drug is known as Carfilzomib (Krypolis), It is a newer generation of Bortezomib (Velcade), for which I had been receiving weekly for almost three years. The downside of the clinical trial is that I had to travel and overnight at Stanford for two days per week for the treatment and lab checks, vice getting my regular weekly chemo treatments locally. I stayed on this therapy through the Holidays and after three months I had to opt out of the trial, due to terrible side effects and illness which lasted a few days after each treatment. The good news is that this drug lowered my light chains to the lowest value since diagnosis, down to a level of 5. As mentioned earlier, my original diagnosis had me at level 28, and Dr. Liedtke (my Stanford Hematologist) tells me if I get down to level 4, they consider me in remission. After stopping the clinical trial, which required a high dosage level and the twice/weekly infusion, my doctor was able to authorize me to get this same drug locally, but at a much lower dose, and only once/week. I stayed on that therapy for two more months, but once again could not tolerate the horrible side effects and had to quit. My light chains have remained nearly the same, at level 5, since that time. This is extremely good news for me.
Last April, after I told Dr. Liedtke that I could no longer tolerate the Carfilzomib, she discussed with me another clinical trial opportunity which I may be qualified to start, once the Phase 2 study began. She is one of the few doctors in the USA allowed to administer this novel antibody treatment coded as NEOD001, developed by the Irish drug company Prothena After many tests and pre-qualification matters, I began this trial last week. It should be much easier than the past trial, as I only need IV treatment once per 28 days, along with a few lab checks along the way. This antibody has been specifically developed to target Amyloid proteins in effected organs, and help the body to naturally rid the protein and tissue from the organs. In my case, while I am stable at Stage 3 Chronic Kidney Disease, we will measure my kidney function over time to determine if this treatment is having any positive effect. Of course, there is the ongoing concern of also watching my light chain value. If my light chains rise over 10, I may have to stop the trial, and resort to some form of chemo treatment again. I sure hope I can avoid more chemo, and that this new trial will work for me. I feel very lucky to be able to participate in this clinical trial, and I'm told that I am among the first small group of cohorts in the world to start the Phase 2 study. Thus far, I feel no side effects. So, the human experiment with my body continues. I chuckle after recently reading a few scholarly Stanford articles written about patients with my disease, which anonymously include myself.
What about my heart? Well, the good news continues. All the tests over the past three years continue to show my transplanted heart is in great shape, and the fact that I can tolerate moderate exercise on a nearly daily basis demonstrates that fact. In the past six weeks, I have been off chemo, and my energy level and strength has started to rebound noticeably. I reflect back and realize that I had been on every form of chemo drug used for Amyloidosis over the past three years, without much break. Many people I have spoken to cannot believe that I have been on non-stop chemo for over three years, and still look so good and remain so active.
Last February, Linda and I took a 10 day vacation to Oahu, and Nathan joined us for the last 7 days. We had a great time of course, despite my limping around. I had given up the walker and cane by that time, following the knee surgery, so my mobility was fairly decent. My biggest challenge was taking a dip up to my waist at Waikiki Beach without stumbling and embarrassing myself...I picked a day when the surf was almost flat.
Otherwise, I am enjoying the retirement lifestyle and staying as active as possible. The constant drill of frequent trips to Stanford, local labwork and physical therapy has become second nature for me. As mentioned earlier, I really feel that I am going to win this battle against Amyloidosis, as I am teetering on remission right now. I have all of you to thank for your concerns, thoughtfulness, prayers and good wishes.
I wish you all a very nice Summer.
Enjoy every day!
Health Update - Langlois - Spring 2016
Dear Friends and Family,
It's been nearly one year since I sent anything out your way, and this weekend is an opportune time. It's Good Friday as I type. Good in many ways. I haven't been inpatient in the past year!!
One year ago, I wound up in the local ER, and had emergency surgery to have my ruptured spleen removed, which turned out to be loaded with Amyloid. It had been a strange twist of fate that my life was saved on two Easter Sundays.....the previous event five years ago when I had the heart transplant. In those first four years since I was diagnosed with AL Amyloidosis, it seemed like my trips to the hospital for surgery or treatments were never going to end. Well....my situation has vastly improved in the past year. It has been a very Good Year for me.
In my last update, while recovering from the splenectomy, I let you know that I was simultaneously having routine treatment in a clinical trial, coded by Prothena as NEOD001, This trial required me to travel to Stanford for two days every month. I wound up staying in the trial for 18 months, until last November. The treatment was an infused antibody that targeted amyloid cells in any affected organs, which in my case were my kidneys. The good news on this treatment was that I experienced few side effects, and only had to have an infusion once per month. It also served to keep my free light chains (lambda) from rising too much, although the drug was not designed to curtail the concentration in my blood stream. Unfortunately, I never saw any improvement in my kidney function, and my light chains slowly started creeping up after many months. My Hematologist thought it was time for a change, as some new promising drugs were starting to become available.
While I avoided inpatient during the past year, I had outpatient surgery last summer to install a Port-Catheter under the skin in my right chest, to allow easier infusions, as all the veins in my arms had been damaged by several years of constant IV and needle sticks. The port requires no maintenance on my part, and supposedly can remain installed for the rest of my life, if necessary. Just prior to the port installation, I wound up back in the ER with severe torso pains - after a Cat Scan and Nuclear scan, it was determined my gall bladder was a bit bothered, but luckily not to the point of needing surgical removal. I was sent home, and eventually the pain went away. Whew, dodged another bullet. This past January, I started on a recently approved drug called Darzalex, which my Hematologist thought would be worth trying, as it gained fast-track approval by the FDA with Multiple Myeloma (MM) clinical trial patients. It is not uncommon for Amyloidosis patients to receive the same drugs administered for MM. Darzalex (daratumumab) is the first monoclonal antibody approved for treating this disease, but is only offered to patients (like me), who have failed at least three other forms of treatment. I easily qualified, as I had been on four different chemo drugs, and two clinical trials. When I tell people that I have been on nearly non-stop chemo for five years, they just stare in disbelief, as I appear to be in better shape than most people my age.
Treatment with Darzalex requires infusion every week for the first eight weeks, and then the frequency extends to every other week for another four months, and ultimately just once per
month. Each treatment takes about six hours. Recently, Stanford authorized me to have treatment done at my local cancer clinic, saving me and Linda a long trip to Stanford. After just one treatment, and much to my amazement, my lambda free light chain value plummeted from almost 10, down to a normal level of 2. And it has stayed at that level through the course of Week 10. Finally!!! It seemed like a miracle cure. After five years, we have found a drug that works for me. And I have not had any side effects. Now, the waiting game begins, as we hope to see if my kidney function might show signs of improving. I have been in Stage 3 chronic kidney failure for almost four years now, fortunately staying ahead of Stage 4, where discussions of dialysis and kidney transplant might begin. So, now all my mental focus is on trying to convince my kidneys to get better. Yes, the power of positive thinking is simply amazing. Not to mention all your support, prayers, great doctors, and the marvels of modern medicine. If any of you need a refresher on AL Amyloidosis, here is a useful website of info...http://www.amyloidosis.org/facts/al/
Although traveling is difficult for me, we took a road trip down the coast to San Diego last fall, and very recently had a fantastic 10 day vacation to Maui. We just love the Hawaiian Islands.
I keep riding my bike quite a bit, and faithfully do my gym routine a few times per week. I manage to ride up to three hours, which might be more than my doctors want me to do, but I usually keep it under two. After the total knee replacement revision, I spent many months in physical rehab working with professional physical therapists on a weekly basis, along with doing my daily exercises. I have secret desires of trying to get back on the ski slopes again, but my orthopedic doc sternly warns me not to take that chance. OK....maybe I will try to pick up my golf game again instead. Last fall I interacted with a cardiologist who is also a triathlete, and put together this blog which you might find interesting...http://www.athletesheart.org/2015/12/a-conversation-with-cyclistand-heart-transplant-recipient-paul-langlois/
Last summer, I was asked by my Hematologist to speak to an auditorium full of Stanford physicians, at one of their monthly education forums. Dr. Liedtke first presented my entire case study to them as an example of what an Amyloidosis patient might have to endure. She wanted to share the good news that someone diagnosed with this rare incurable disease, can survive, and even thrive, despite all the treatments.
After her presentation, I reflected upon my experience over the past years, from a patient perspective. It was rather intimidating talking to about 300 physicians, but many of them told me they gained much insight from my talk. I guess the word got out on my aspiring lecture circuit, as a few weeks later I received a call from the American Heart Association, asking me if I would speak to hundreds of people at the start of Santa Rosa's annual Heart Walk, up on a stage with a microphone. I managed to put together an appropriate pep talk for the crowd, and then joined them for a five mile walk through the local Park. I don't tell my "Survivor Story" to gain any sympathy from people, but rather to educate and hopefully inspire others who may face similar challenges -- and not to give up hope. I can't thank you all enough for your constant support and prayers over the past five years.
Back in early 2011, when my cardiologist told me I may not survive more than two months, I never believed that I would be happily sending out this type of note five years later.
So, yes....it is a Good Friday. May you all have a Blessed Easter weekend.
Health Update - Langlois - Spring 2017
I was using Darzalex last year with fantastic success, and went into remission. But last September, I got hit with a blood infection, sepsis prevailed, but I finally recovered, but had to have four amputations. Just when I thought I was looking at a bright future, this major setback hit. Home now, after 4.5 months inpatient, with my lovely wife taking care of my many needs. On new leg prostheses, and expect the same for my arms next month. Otherwise in a wheelchair, with many months of therapy to follow. Staying positive.
Dear Friends and Family,
Happy New Year!
I wanted to send out a quick update on my situation. My last update was this past Easter, on the fifth Anniversary of my heart transplant. At that time I was feeling great, and my new Amyloidosis drug treatment with Darzalex had essentially put me in remission. This past year Linda and I had a nice 10 day vacation to Maui, and did lots of hiking and cycling together. I had even started to play golf again, and it really felt like I was getting my life back together. In August I was invited to attend the annual Coast Guard Festival in Grand Haven, Michigan, where Linda and I were granted VIP treatment, while they focused on celebrating the Centennial of Coast Guard Aviation. We had a blast.
This past September, while cycling with Linda through Wine Country near our home, I suddenly became very weak and cold, and had to stop riding. Linda was very worried, and we slowly pedaled back to our car and drove home. I knew something was very wrong with how I felt. My vibrant health was about to change for the worse.
Over the next few hours I became somewhat delirious and non responsive. At that point, I have no memory of what took place for the next two weeks. Linda called 911, and an ambulance brought me to local Santa Rosa Memorial hospital. I went into septic shock and delirium due to a blood infection. My blood pressure plummeted, and Linda was told that I would likely not survive. My right knee was opened up to clean out any infection that may have settled in my earlier total knee replacement hardware. I was also given pressors to elevate the blood pressure to my vital organs. Unfortunately, pressors have the terrible side effect of eliminating blood flow to the extremities.
After a day or two at Memorial, I was medevaced by Stanford Lifefight helicopter to Stanford. (I wish I was conscious so I could keep an eye on the pilot). I went into critical ICU at Stanford. My lung collapsed, but was regained. I have memories of extreme pain and horrible nightmares while in delirium. After two weeks, I awoke back to reality, and the first people I saw were Linda and Nathan standing over my bed with some doctors. I had survived!
I was so happy to come out of the deep sleep and see my family with me. There were tubes down my throat and I couldn't talk, but everything was explained to me, going back to my bike ride with Linda.
I quickly realized my hands, feet and nose were all blackened and very painful. The tissue was dead and hardened, due to the pressors. As a result, my hands and lower legs had to be amputated. Over the next two months, I had seven surgeries, but fortunately retained the leg hardware from the previous knee replacement. I was put on kidney dialysis for many weeks, but no longer require it. I had a large skin graft taken from my left leg to place over my wrist area, following hand amputation. They used existing tissue in my lower leg, after they made equal length cuts in my lower legs, midway between my ankles and knees.
My emotional state was obviously put to the test, even more than the past. But I was given constant encouragement by my doctors, therapists, and all of you who offer support through your prayers and support. Linda visits me every day, and has also been tested to the brink with all her current and future Caregiver requirements. Nathan and my brother Richard are always there as her backup. I am a lucky man to have Linda.
As almost three months passed at Stanford, and my wounds were satisfactorily healing, I was transferred to a Long Term Care Facility at Kentfield in Marin County, about one hour from home. This was much easier on Linda, as she could now live at home, instead of with her brother Kevin, who has a home in Pacifica, 40 minutes from Stanford. I began some daily Occupational and Physical Therapy. I made contact with a popular prosthetist who fabricated leg prostheses...and I took my first steps on Christmas Eve. My therapists have all been amazed how strong I am so early in the rehab process. I guess my earlier active lifestyle is paying off now.
After three weeks at Kentfield, I was transferred to Santa Rosa
Memorial again, into their Acute Rehab Unit. Three hours of therapy a day is the game plan, to get me ready to go home in a few weeks. Unfortunately, after just one day here, they detected what appears to be a bone infection in my right wrist, where the wound had not fully healed. Now Stanford wants to look at it, so I am currently awaiting ambulance transport back to Stanford. Hopefully, this will just be a little speed bump, and I will return back to Santa Rosa Rehab soon. My doctors, therapists, and prosthetist, all expect me to get back on a bike this coming year, with a lot of hard work. Never giving up!
I wish my New Year greeting could have been more uplifting, but the fact that I am still alive, and surrounded by loved ones, great friends, and a multitude of prayers coming from so many around the nation, leaves me in my normal state with a positive attitude, and so grateful for the joys of my past life, along with high expectations for the future.
I wish all of you and your families the very best in 2017. God Bless you all.
p.s. Feel free to pass this on to anyone who might be interested.
UPDATE: JULY 1, 2017
Dear Friends and Family,
Well, it has been six months since I sent out my last update to all of you on New Year's Day. Many of you have been asking for the latest, so I figure it's about time to send this out; and it's a lot easier for me to type this out en masse, since I still don't have any hands, artificial or otherwise.
As I ended my last update, I was awaiting ambulance transport back to Stanford, from the third consecutive hospital where I was inpatient, at Santa Rosa Memorial. The reason was for determination of a suspected bone infection in my right wrist, at the amputation site. After spending two days inpatient at Stanford through the Holiday, they sent me back to Memorial, determining the suspected infection was nothing to worry about. More on that infection later.
In late January, I finally returned home, after nearly five months inpatient at three hospitals, one helicopter transport, and five ambulance trips.
As mentioned earlier, I was fitted with dual leg prostheses on Christmas Eve. These have worked out fairly well, but require periodic adjustments, and so we changed our prosthetist provider much closer to home, to save on frequent distant travel. I can now walk short distances without the aid of special (hands free) crutches, but I always use them outside for safety.
So, what about getting hand prostheses? Well, to be honest, I am a bit disappointed that progress has not gone faster. It is a long process for skin grafts to heal, and take firm hold upon their new location. My left wrist has finally healed up since the Thanksgiving surgery, and my prosthetist has just begun fabricating an arm socket to support a body-powered hook device. I might be wearing it next week.
But my right wrist is still not fully healed at the amputation site, and to make matters worse, that same infection from New Year's suddenly showed up again a few weeks ago. Since late April, I have been seen every few weeks at the Advanced Wound Care Center at Stanford. After being started on a different antibiotic last week, I was advised that if this new drug doesn't clear the bone infection within a month, I would likely need another surgery on the wrist. I pray that another surgery will not be needed.
Most of my days are spent at home, and my departures are typically for follow-up clinics, physical therapy sessions, or for prosthetic adjustments. Linda and I have managed to get out to a restaurant three or four times. I can't tell you how much she has given to me in the way of constant support and care through this new ordeal. Let me tell you, being a full time Caregiver is very demanding. I always say I am a very lucky man - and that's because I met Linda 38 years ago.
My son Nathan and brother Richard are also very helpful, and I see them often. I have had quite a few friends stop by for a visit, many of them my Coast Guard buddies from all parts of the country. My local bike team threw a big Welcome Home party for me in March, which really lifted my spirits...it was the first time I dared walking without my walker for support. I have yet to trip and fall.
I now have three wheelchairs, one a fancy motorized rig provided by the VA, but I am making an honest effort to not become wheelchair dependent. So, I tend to wear my leg prosthetics nearly all day, despite the pain and discomfort which comes with constant use. I figure it will toughen me up for the future.
I started going to our local athletic club with Linda twice per week. I have managed to pedal a recumbent elliptical machine for 30 minutes non-stop, and then do leg weight machines and abdominal work for another 45 minutes. But my arms and chest are still pretty frail, since I can't grasp anything. That will change soon when I get my new hands. Check out the website "Touch Bionics", for an example of the new cutting-edge hand prosthetics that I expect to receive eventually. I will become the Bionic Man.
I didn't mention much about my underlying disease, for a good reason...last summer I went into remission with Amyloidosis, and it has not shown any indication of returning, despite no treatments since. But the damage is done. My kidney status is stable since leaving the hospital in late January, but now remains at Stage 4...pray that I will not need dialysis downstream. My new heart is fine!
I really feel, and appreciate, all your prayers, good wishes, and gifts. I am so lucky to have met so many amazing friends in my lifetime.
Please feel free to share this with anyone.
Have a great summer and a Happy Fourth!