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AL amyloidosis week: a patient’s experience

During this week we are raising awareness of AL amyloidosis. In 2019, patient Linda Wolfe shared with us her experience after she was diagnosed with AL amyloidosis in 2014, and how she has lived with the effects of the condition.

Patient stories // 22nd February 2021

When my husband Alan and I retired from our respective teaching posts in 2007, we had exciting plans to follow the recommendations of our two well-travelled sons.  

All went well for a few years, with treks in New Zealand, Nepal and elsewhere as well as lots of mountains at home in Scotland. In 2011 I began to experience some very well-known heart symptoms: a heavy sensation in my chest, with a pain travelling down my left arm. My GP diagnosed angina, which was confirmed with a treadmill test and an angiogram.  

Although we continued to lead an active life with lots of travelling, walking, swimming and the arrival of two grandsons, my health slowly deteriorated. My GP and the cardiologist changed the drugs, increased dosages, added in diuretics when my ankles started to swell up and stipulated “you should be feeling better than this”. I was alarmed by how I struggled with just a few stairs, or a moderate incline.  

One evening, at the end of a book group meeting, a doctor friend said “These don’t look like your legs, Linda. How much extra weight are you carrying?” I indicated about 5 kilos to which she replied, “This is serious!” Alan and I decided to privately seek a second opinion. The cardiologist I saw concluded that my main problem lay in my kidneys. He asked for a 24-hour urine sample (a Bence Jones Protein Test). Two days later I had a call from my GP asking me to report to the Haematology Department at Ninewells Hospital in Dundee.

 

The key objective was to “switch off the tap” to halt the over-production of the amyloid protein. 

 

Upon arrival there in October 2014, the consultant sat us down and said, “We have a diagnosis for you. You have amyloidosis.” He gave us some information about this rare blood disorder, which originates in the bone marrow. A referral would be made to the NAC (National Amyloidosis Centre) at the Royal Free Hospital in London for tests and treatment advice. The key objective was to “switch off the tap” to halt the over-production of the amyloid protein.  

When we returned home, still somewhat shocked, I phoned my friend from book group and told her my news. “That’s rare,” she said “but treatable.” That was so reassuring! That evening I followed internet links from the NAC website to find out more.  

My first appointment at the NAC was in November 2014. On that first visit I struggled to walk the length of the station platform to find a taxi. However, once in the NAC, it is a very professional and thorough procedure – an interesting mix of the hi-tech (SAP scan) and the lowtech (6-minute walking test)! It was confirmed that I had AL amyloidosis with amyloid deposits in my heart, kidneys and spleen. A CVD regime (cyclophosphamide, Velcade [bortezomib], dexamethasone) was agreed.  

By December 2014, I had started this treatment. I was admitted to hospital for the first dose of chemo just in case there was an adverse reaction. Luckily, I tolerated it all then and, by and large, continued to do so. Before being discharged, the pharmacist came to the ward with an enormous quantity of drugs. She explained the purpose of each: several drugs to help protect me from infections as well as the core treatment drugs, a drug to help deal with nausea and injections to help avoid blood clots.  

For four Tuesdays in each five week cycle, I attended the Haematology Day Care centre – “the Tuesday Club”, we called it. I was given the Velcade injection while other patients were on drips, infusions etc. Most were being treated for myeloma. A doctor or nurse gave me a thorough check each week and advised on dealing with the inevitable side effects. My skin became very fragile, my hair grew thin, I oscillated between diarrhoea and constipation, and I lost weight. Steroid-induced wakefulness had me unable to sleep for long periods at night. The darkest thoughts seemed to occur around 3am.  

 

After five cycles of treatment, the doctors decided I could stop. 

 

Quite early during my period of treatment I suggested to my husband that I set up ‘camp’ in our guest room so that he could have a good night’s sleep. This worked well because I could pass those wakeful hours watching some of the many box sets of costume dramas lent by well-wishers or purchased by Alan on our laptop. I found that I could not concentrate on reading, but films were ideal.  

Fatigue was an issue, but on good days I was happy to have visitors or be taken out for a drive. Alan provided an excellent meal service depending on my appetite and many of the visitors brought homecooked meals or soup. On good days, we could prepare meals together.  

After five cycles of treatment, the doctors decided I could stop. The lamba blood test measure had gone from 138.0 to 9.4 – a complete clonal response. That was in June 2015. Within a few months my hair started to grow back in, I returned to my normal weight and my energy levels gradually improved. I have now been in remission for four years.  

In November 2015, I attended an Infoday for patients with AL amyloidosis and their carers. It was an excellent event which I would thoroughly recommend to others. Hearing the staff involved in the various aspects of research speak was interesting, as was meeting other patients. Delay in diagnosis was a very common problem. I resolved to use every opportunity to alert nurses and doctors with whom I came into contact to the symptoms and gravity of amyloidosis.  

Sending off monthly (now bi-monthly) blood samples, four-monthly consultations in Haematology Outpatients and the annual visit to NAC all make me feel that I am lucky to be so well cared for. The kindness and professionalism of all involved in my care are remarkable. In January 2019, the doctor at the NAC drew our attention to the lamba measure which had nudged up from 9 to 45 – “a partial response”. Were it to continue to climb beyond 50 they might want to intervene with some more chemo in tablet form. But even that is reassuring – there is a plan and I continue to enjoy a full life.

This experience was originally published in AL amyloidosis Matters in 2019. Read Linda’s full story here.