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Ken Farr – Age 73 – Retired maintenance engineer
Box Hill, Victoria.
History of Amyloidosis
Update: August 2009:
It is 18 months since diagnosis of Al Amyloidosis and Ken has been on haemodialysis for 9 months.
He had a badly bruised face from a fall and 3 weeks later suffered stroke like systems – a blood clot (linked to the fall) was found on the brain. It was removed and another fall in hospital a week later made a second operation necessary. This was quite a hurdle to overcome and the little driving he was doing was curtailed for 3 months. Much rehabilitation with physiotherapy followed and he is much improved. This was in late ’08.
However on-going low blood pressure after dialysis has been a concern and recently an extremely low pulse rate has yet another specialist involved – Dr George Proimos, Cardiologist. It has been suggested than Ken consider peritoneal dialysis which would be kinder to his heart as the fluid is taken from the body slower as it is daily procedure. Many aspects of this method were investigated but for the time dialysis is left in original state.
Ken sustained another fall in May’09, resulting in 5 fractures to pelvic area. The following hospital stay was extended due to contracting shingles which forced a stint of isolation. Osteoporosis was discovered in a scan as the amyloids had filtrated into the bones. Rehab. followed and then with much anxiety and after 7 weeks, a very frail person returned home. Slowly he has regained strength and balance but much of his/our life is taken up with medical appointments and resting/sleeping. Despite all this, he has mostly remained in good spirits.
Jan
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Diagnosed in January, 6 months ago. Now, in July, 2008, Ken has been very ill, but his health has stabilized and he is “hanging in there”.
Unfortunately, as in many cases, the early vague symptoms evaded our G.P. From September ’07, I went with Ken to his many medical appointments to be another pair of ears. A “significant unexplained weight loss” was an issue, as was lack of energy, poor interest in food and an extremely high cholesterol level which did not respond to several medications at a high dosage. He had many return doctor visits, blood tests, a hospital stay due to low blood pressure and dark coloured stools, with a colonoscopy and a gastro scope finding nothing. He was given saline and blood because of a low blood count and returned home, still weak and tired and losing weight. This was mid October ’07.
Again, nothing was found from an Ultrasound or a C.T. in November.
This situation of general ill health continued until his G.P. had more detailed tests done in late January which showed an extremely high reading of Creatinine, Urea and an estimated kidney function of 11%.
Ken was then referred to Nephrologist, Fiona Chow, who was puzzled as to the cause of his drop in what had been a very healthy person. She needed a biopsy to help form a diagnosis so he was to be admitted to Epworth Hospital the following day. Urgent blood tests were redone, resulting in a worsening reading of Creatinine, Urea and now with a kidney function of 9%. This was within a week.
So Ken was admitted to hospital that night so that a kidney biopsy could be performed as soon as possible. This of course gave the diagnosis of this dreadful disease and we were alerted to the fact that kidney dialysis likely to happen in the very near future.
We were both calm and accepting as we ploughed through the printed material prepared for us. It took a little time for us to absorb the enormity of lifestyle changes that we would have to make, - travelling, that was such a big part of our retired life would not be possible, maybe to relocate our home, as we have a large suburban property which needs much maintenance, friends and family to inform.
Within a week, I had contacted Amyloidosis Australia and had a response from Ellen Reid, who in fact came to visit me and give me newly made CDs to distribute. (From time to time I look up more information on the internet as other issues crop up, but have been busy trying to be N O R M A L).
A bone marrow lumbar test was needed to identify which type of Amyloidosis Ken had – it was AL Primary.
The next procedure was for a fistula to be formed in his wrist for future dialysis and for a permacath near his collar bone for emergency access/
Dr Rohan Doig, Haematologist/Oncologist was to oversee the chemotherapy plan. V.A.D. – combination of Vincristine, Adriamycin as infusion and Dexamethasone in tablet form. Chemotherapy was started one week from initial admission, of a 96 hr. drip as an inpatient. He was only home for a week, when extremely low blood pressure caused him to be admitted for saline as treatment for dehydration (in spite of drinking large amounts of fluids).
Ken returned to the day ward three times for the VAD course to be hooked up to wear as a body pack and then 4 days later to return to be disconnected. Unfortunately about a month after the permacath was inserted he developed shivers and shakes with a high temperature. An ambulance was needed to return him to hospital and Staphyococcus aureus (Golden Staph) was found to be the cause and 14 days of antibiotic drips were administered. He was extremely ill at this time and losing weight. The hospital dietitian was most helpful in this stage and indeed, I consult him as the situation changes. Low protein food was what we had to keep in mind and anything that would take his fancy, to keep stimulating the appetite during the chemotherapy stage.
Medication has been continually altered and we now are working on Plan 11 which consists of 38 + tablets daily and a weekly self administered injection to stimulate haemoglobin as low kidney function brings on anaemia. A high phosphate reading means that Ken is to chew 5 calcium tablets in the middle of each meal to bind the phosphate and help it pass through the body.
The aim of the treatment is to reduce or eliminate the bone marrow disorder, that is, the plasma cells which are responsible for producing the amyloid. A review of serum light chains (abnormal proteins), 2 and 3 months from commencement of treatment showed that it had been effective.
As well as causing great damage to the kidneys, Ken’s gastrointestinal area has been affected. The bowel is severely irritated with Ken having urgent, frequent movements, although not actually diarrhoea, both day and night. Malabsorpsion has prevented weight gain.
Recently several drastic bowel bleeds led to another short stay in hospital – for a colonoscopy which revealed haemorrhoids and a very wrinkled lining of the bowel. The accompanying bowel biopsy showed that the amyloids were also in this area of his body. Diet is now an issue to limit unsoluable fibre and many fats.
The medication Cotazym – in capsule form had been introduced to stimulate the pancreatic enzymes and to be taken with food to absorb fats.
A scan of the liver showed it to be enlarged.
Ken is always chilled, needing many layers of clothing, and the home heating is always on. Poor muscle tone and general weakness are issues requiring assistance.
The social worker at the hospital is responsible of the following 3 aspects of action:
i. A Disabled Parking sticker – which has been most useful.
ii. Ken attends twice weekly physiotherapy and occupational therapy sessions.
iii. I applied for a Carer’s Allowance from Centrelink – as I knew my caring for him would be on-going. If single, he would have needed to be in some type of hostel, respite setting. This claim was rejected, however I appealed and it was accepted.
A battery of tests were done 4 months from the start of this ordeal. Dialysis has not been undertaken, as Ken now presents with a low blood pressure which is not desirable and the failing kidney tests had shown stabilization.(although now at 7%). Liver function has deteriorated but he has no physical awareness of this. Kidney transplant was never an issue as he was not within the age limitation.
We are both impressed with the two specialists who are overseeing Ken’s treatment, Dr Fiona Chow and Dr. Rohan Doig, as well as the wonderful staff at the Epworth Hospital. So at this stage he is being reviewed at wider intervals, but it’s up to us to go about living as normally as possible. The future of course, is shaky.
Jan Farr – Wife – July, 2008
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