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Ken
Farr – Age 73 – Retired maintenance engineer
Box
Hill, Victoria.
History
of Amyloidosis
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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