Gordon Hoosonâs story of his fight with Amyloidosis.
Gordon was a strong and healthy man, full of life and fun, adored by all who knew him.. We married at 21, bought a house and had our first child when we were 25. We were to have 2 more children before our family was complete. I loved him deeply and we were very happy.
Gordon was never ill, the only problem he did have was a tingling in his fingers whilst driving on long trips. He was diagnosed with bilateral carpal tunnel (the reason for the tingling in his fingers) in 1999. He had operations in 2001/2002 to relieve this condition but even though amyloid was identified, no action was taken to treat him.
Gordon had been to his GP over several months in early 2004, with a persistent cough, which was put down to asthma, after x-rays revealed little wrong with his chest.Â We went to our GP and I told the doctor of my concern that he was not responding to the medication for asthma. He was sent for an x-ray and blood tests. We were told the x-ray showed an enlarged heart and called a cardiologist who would see him the next day.
An ECG was done in the surgery followed by an ultrasound of the heart the next day and an angiogram the following week. The end result was a diagnosis of Hypertrophic Cardiomyopathy, to be treated with heart medication and fluid tablets as Gordon was carrying fluid in his abdomen (ascites).
During the next few months Gordon became weaker, walked less often and struggled to play golf. We had been back to the cardiologist every 4-5 weeks with problems of fluid retention (swollen ankles and a feeling of fullness in his stomach). When he first started on the fluid tablets he weighed 84 kg. After 2 weeks he was down to 76kg. He then slowly would regain the fluid and have to increase the dose to control the fluid.
He had also developed a hoarse voice and saw two respiratory specialists who both said he had an over developed cough reflex and sent him to speech therapy to learn exercises to control the cough. This was to turn out to be helpful later on but for a different reason.
Over time I noticed that he had started to lose weight in the face and shoulders. Over the next few months he lost all muscle tone and became thin and frail, but still had a very large stomach, which was very uncomfortable. He also had that cough and was often breathless, these days, after very little exertion eg having a shower!
By March we were all very concerned about his health and asked for a referral to a specialist in Sydney for a second opinion. We of course went to see a cardiologist specialising in Hypertrophic Cardiomyopathy. He did a second ultrasound and came out and told us that Gordon did not have Hypertrophic cardiomyopathy but Restrictive cardiomyopathy. We had by then been referred to St Vincentâs Hospital for assessment for a heart transplant. We had to wait 6 weeks to be seen and by then Gordon was very ill.
We went to St Vincentâs with our hopes high, knowing that a heart transplant would give him back his life. He had an initial consultation and was sent straight up to have a heart biopsy. This biopsy was sent to Brisbane where a pathology lab using a Congo Red stain and identified the presence of amyloid, which our very clever Sydney cardiologist had suspected immediately.
Gordon was admitted to St Vincentâs Private Hospital the following week for 5 days of tests. This turned into 10 days, as it was discovered his persistent cough was caused by a pleural effusion, affecting 40% of his right lung. One and a half litres of fluid was drained from his lung.Â Â This was the start of the investigation, which, 10 days later, led to a correct diagnosis of Primary Systemic Amyloidosis with cardiac involvement.
A telephone call to the hand surgeon who treated the carpal tunnel in 2001revealed that the pathology showed âsmall traces of amyloid present in the tissueâ. Back in 2001!Â That proved conclusively that the amyloid was systemic. It also showed that it was there in February 2001. A bone marrow biopsy showed amyloid producing cells. Blood tests confirmed an increased level of creatinine, which meant the kidneys were failing.
The treatment was medium dose melphalan and dexamethasone, once every month for 3 months to see if it stopped the bone marrow from producing amyloid. The idea was to then allow the body to slowly rid itself of the amyloid. This would take 11months or more.Â Gordon asked the Haematologist what sort of life expectancy he had. The doctor replied âthis would buy you more timeâŠ. perhaps a few yearsâ. We had no choice. This was the first time I realised that I was going to lose him.
I travelled home that night, a 2 hour drive, to tell my 3 beautiful children who were 23,21 and 17, that their father was going to die from a disease none of us knew anything about. Our eldest daughter announced that by the end of the weekend she would be engaged and they had decided to marry at the end of Dadâs first round of chemo. âHe has always said it would be one of the proudest days in his life, and he is not going to die before he does it!â Sure enough they were engaged on Friday night and the date was set for the 2nd July, 5 weeks away.
We girls went dress shopping on the Saturday and by Sunday afternoon we had the wedding dress, two bridesmaid dresses, suits for the men, a venue, a church tentatively booked and the photographer all lined up! They even had the honeymoon organised! Who said you need 12 months to organise a wedding! We did it in 5 days!
Gordon was admitted to St Vincentâs Hospital in Sydney the following week on 2nd June 2005. His case was unique as he had to have a cardiac care bed so his heart could be monitored during the chemo treatment. Once again, his stay was much longer than anticipated. He was there for 3 weeks, and he kept reminding the doctors that he had to be out of there by the 1st of July. He was discharged 3 days before the wedding, after several false starts (including a cardiac arrest a few days after the chemo, and increased levels of creatinine in his blood as well as low potassium and very swollen ankles!) Â I am sure they only let him go because he pestered them so much! We sent the doctors a photo after the wedding!
On the day of the wedding he was up and walking around, very slowly, and was the happiest and proudest dad ever. He walked his daughter down the aisle, and his children and I were ecstatic. We could only be proud that he had triumphed over this disease and made it to the wedding!
The next day his son travelled to Canberra to play in the NSW Combined High School hockey team with dear friends of ours. Gordon wanted to go too and I knew he might never get another chance to do this, so after he farewelled his parents, brother, sister and niece and nephewsÂ (which would be the for the last time), we headed off to Canberra. We returned a few days later to be admitted to the Newcastle Mater Hospital Cardiac Care Unit and start his second round of chemo, closer to home and his adored children. Two days later his heart went into an irregular ventricular rhythm and I was told not to expect him to last more than another day.
We called his family and the children. The children made it to his bedside and he joked and chatted as normal, our friends came and saw him and his parents were able to get there that day as well. I stayed with him overnight and his heart settled about 2 am back into normal sinus rhythm.
At 6am I thought I would go home and have a shower. I arrived back at the hospital with his parents, brother, sister, and families to be told he had had a cardiac arrest and they were trying to resuscitate him. They succeeded after 20 minutes, and he was taken to Intensive Care but had another cardiac arrest at 12.30pm and left us.
His courage was an inspiration to us all, and his love remains with us. People were amazed to know that he knew his time was short. He didnât mope about it, he just enjoyed what time he had, trying to believe he could fight this disease. He did not complain about his illness much, except that he was thirsty and felt full all the time. He loved having his legs massaged and convinced all of his visitors to go down to the hospital shop at St Vincentâs and buy him a water icy pole. They were sold out on many occasions whilst he was there!
Can you imagine how hard it is to know that his disease was evident in 2001 and the doctors knew but did nothing? I can only assume the hand surgeon did not know the full implications of amyloid deposition and his GP did not bother to look up amyloid in the dictionary. If he had had treatment back then he may have still been with us to see our other daughter and son married, to hold his grandchildren in his arms and care for them as he did our own beautiful children, to be my life partner and for us to grow old together as we were meant to. Instead, we grieve silently and pretend we are ok.
We all treat grief and loss in different ways. My way is to continue the fight to raise awareness of this terrible disease, to celebrate other amyloid patients success stories, and grieve with those so like my own.
I have learned that this disease has been known of for 300 years, that there is no cure, but it can be treated successfully if diagnosed early. I have spoken to some wonderful people including his doctors at St Vincents, who are sharing my experiences and also the frustration of knowing that Australia is a long way behind the rest of the world in both diagnosis and research of Amyloidosis.
The statistics are that 8 per million people are diagnosed with this illness in its varying forms each year in USA. Here in Australia that equates to 160 Australia wide. I would like to know that every one of them has support and access to the best treatment available worldwide, but I fear this is a silent killer, and that many older people with the disease either die of heart or kidney disease undiagnosed, or misdiagnosed, or are simply not treated because the doctor thinks there is no treatment available.
My children have decided to raise funds for the Australian Amyloidosis Awareness organization, to help âspread the wordâ. Our specialist doctors need a reminder, our pathologists need to look further than simply diagnosing amyloid, to type that amyloid so that treating doctors can give appropriate treatment and search the internet for world leading specialists and the latest research. Our GPs need to ask âcould it be amyloid?â
New drugs and treatments are being trialled all the time in this area, and we need to be on top of it. If you are reading this article you probably have a relative or friend who has this disease. I would encourage you to spread the word âamyloidosis awarenessâŠ.the first step towards a cureâ.
Vickie Hooson, Kristen and Dean Birkett, Kimberly Hooson and Matthew Hooson.
If you would like to support the Reid family in their quest to raise awareness, and purchase a Wristband or Calendar Magnet from us, email us on:
All funds raised by the sale of these bands will go to the Amyloidosis Australia organization to raise awareness and support families and patients with amyloidosis.