Amyloidosis: I wondered whether I would die
David Birchenough had been experiencing suspicious symptoms for months before he was eventually diagnosed with AL amyloidosis.
I am a 54-year-old automotive spray painter who has worked in and around a solvent saturated environment most of my life.
In November 2009, after two years of intermittent breathlessness, pains in my chest on exertion and many tests including an echocardiogram and cardiac angiogram, which appeared normal, I was diagnosed with AL amyloidosis. In the months before diagnosis my symptoms worsened with bloating, night sweats, clamminess, nausea and episodes of feeling very faint. On two occasions I was found to have protein in my urine that perhaps should have rung alarm bells but didn’t.
After collapsing on the golf course, a further echocardiogram showed nothing. I then collapsed at home and a heart monitor in the accident and emergency department identified a period of excessive heartbeat and the ECG recorded an episode of tachycardia. I was told I wasn’t going home until a reason was found.
A test finally reveals amyloidosis
Further tests and a renal biopsy finally identified AL amyloidosis affecting my heart and kidneys, which I quickly learnt was a rare and serious condition with dire consequences if left untreated and I would need an implantable cardioverter-defibrillator implanted followed by chemotherapy and a stem cell transplant.
As we knew little about the disease we felt we needed a second opinion, but from where? This was not because I didn’t trust my doctors but given that it was such a rare condition, I wondered how many people with this disease had these doctors actually treated.
My wife found The National Amyloidosis Centre in London on the internet. We emailed them with the proposed treatment and received an immediate answer assuring us that if my diagnosis was definitely AL amyloidosis, the suggested treatment was the best option for me, emphasising that time was of the essence.
In November 2009 I received my first lot of chemotherapy followed by stem cell stimulating injections.
In December I received high dose Melphalan before being given an infusion of my own stem cells. I quickly learnt that nothing goes entirely to plan and instead of the three weeks I thought I would spend in hospital I was actually there for seven weeks, three of these in intensive care on dialysis after developing sepsis and going into kidney failure. There were periods when I wondered whether I would die and I remember feeling at one stage that it would be easier to just slip away. I might have done so if it wasn’t for the support and love of my wife and children. I was eventually released to the outside world in January.
I took some time to begin to recover but the good news was that my free light chains were dropping which indicated the treatment was working and stopping the production of the amyloid proteins. I am pleased to say that I am doing well and my light chains are within the normal range.
I decided this year with the backing of my renal physician, to visit the National Amyloidosis Centre in London for a Serum amyloid P (SAP) scan which is unavailable in Australia. There appears to be different opinions as to how helpful this test would be and that it would not alter the outcome, however I decided to go ahead.
The SAP scan only showed a small loading of amyloid in my spleen that I had suspected due to tenderness in that area. The doctor said that it often does not show up in kidneys that have had chronic kidney disease for a prolonged time as in my case. He went through my story from the beginning and said that my symptoms were very typical including various symptoms I was unaware were associated with amyloidosis. He spent about an hour consulting with me and said I was in good shape considering I was at the top of the ladder as far as prognosis was concerned. He also said that if and when my clonal response to the treatment deteriorated, my body would be in a much better state to withstand a second round of treatment than it was when originally diagnosed. I couldn’t have hoped for a better outcome and even though it doesn’t change anything, I feel incredibly encouraged by the results and feel a lot better psychologicaly. For me it was worth the 3363 British pound cost.
What I’ve learned
As I reflect on my journey over the last two and a half years, I’d like to share what questions I still have and what I have learned.
I now understand the importance of early diagnosis in the treatment of amyloidosis and I still wonder why my original echocardiograms didn’t pick up the amyloid in my heart sooner. I also wonder whether there have recently been developments in the way cardiac amyloidosis is diagnosed.
I question, as I am sure many patients do, whether there is any relationship between a person’s working environment and amyloidosis and whether any research has been done in this area.
It is so important to understand why your doctor makes particular decisions about your treatment before agreeing to go ahead with it. Be fully informed. Asking appropriate questions at the appropriate time is vital to this understanding.
Once I had made the decision to accept the suggested treatment, my amyloidosis became almost secondary to surviving the ever-changing side effects of that treatment and my world became very narrow. My wife was left to worry about the wider picture.
It is not just the physical aspect of treatment that affects you but also your psychological state is vitally important. Although I did see a psychologist when I was in hospital it was only when I began to feel better physically that found I was suddenly experiencing very emotional moods, often bursting into tears for no reason. Normally this left me feeling better afterwards. I am sure many of us experience anxiety before our follow up appointments and the results of our free light chain assays.
Quite some time after my stem cell transplant my GP arranged a care plan, a government paid initiative which included a pharmacist visiting me at home to explain my medications, an exercise and nutrition program and sessions with a psychologist. Every aspect of this package has been extremely helpful and I would recommend it to everyone. I firmly believe that exercise is the best treatment for fatigue.
It was some months after my treatment finished that my hematologist began to talk about prognosis and how well I had done compared with others with the disease affecting their heart. He talked about guilt from surviving and encouraged me to join a support group. I am a member of the Australian Amyloidosis Society and also join the Leukaemia Foundation’s amyloidosis telephone forums. This has given me the opportunity to communicate with other patients which I found very helpful.
Share your story
If you would like to share your own personal story of living with your diagnosis and have it published here, get in touch.Last updated on June 19th, 2019
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