Vintage

EARLY  DETECTION  -  THE  KEY  TO  SUCCESSFUL  TREATMENT  ? 

Sharon Page, Sydney, Australia 

Today, as I update my story (September 2005) I am 56 years of age.

I have spent most of my life in the inner city suburbs of Sydney Australia, usually within a very short distance of our beautiful harbour. I am not married. I have no children. I am very lucky to be able to share a large part of my life with a younger sister. She is my support person in times of stress or need. This is reciprocal.

I was a healthy child. I had NO significant medical problems during my childhood, not even mumps or chicken pox, although I did have measles. In my mid twenties I had my very first hospital admission. I had to have my gall bladder removed. Also I am allergy prone, but nothing too bad, mainly some food and pollen/dust problems. I have an extremely sensitive sense of smell and I suffer from lots of headaches, occasional nosebleeds, frequent indigestion and now a bit of arthritis. These are minor problems and are only included as “background”.

Before my diagnosis with AL Amyloidosis, my immune system had always been extremely good. I seemed to fight off most things (colds, etc) while others were “dropping” around me. Fighting off infections in third world countries, where most of my overseas traveling has been done, did not seem to be a problem.

So  ……… what is my Amyloidosis story?

Coming up to Christmas 2001, I was a healthy 52 year old female. I had been working in health related areas all of my life and was very aware that it is necessary to have regular health checks. You know the kind, PAP smears, breast checks and (being an Australian) skin cancer checks.

At the time I was also seeing, on a six monthly basis, a very well respected Endocrinologist (Phillip Clifton-Bligh). I was seeing Phillip at his rooms at the North Shore Private Hospital. This small private hospital is located in the grounds of one of Sydney’s biggest public teaching hospitals (Royal North Shore Hospital – RNSH). Medical Specialists working at Public Hospitals in Australia often have a “right to private practice” and I was seeing Phillip about “flushing” problems that I had been experiencing since my mid twenties. I believe that the fact that Phillip continued to “track” this condition (along with the medication he had prescribed) is what saved my life. This along with his highly developed diagnostic skills and knowledge (as well as those of his colleagues at RNSH).

Before each appointment with Phillip it was usual for me to have a series of basic blood tests. However, approximately every two years he ordered more extensive blood tests. In December 2001 I had the more detailed tests. Everything was OK except for my blood Serum Creatinine levels which were far too high (0.17). An earlier reading that I had in my records for the mid 1990s was within normal limits (0.08). This was something to worry about.

I had a “kidney problem” indicator, but I had no “kidney problem” symptoms. Phillip immediately referred me to the RNSH Renal Unit for kidney function tests. In doing this he had transferred me from the private health care system to our public health care system for “full on” diagnostic evaluation.

In Australia we are very fortunate because we have access to a universal health care system (Medicare). This gives us free treatment in a Public Hospital (including our major teaching hospitals). The only thing is that, if you are admitted as a public patient (ie. you do not have or have chosen not to use your private medical insurance in a particular situation) you do not have “choice of doctor”. However, the policy is (and it has been my experience three times now) that which doctor looks after you is based on medical need. This gives really ill patients access to “the best” medical care in our big public hospitals, even if they have no medical insurance.

In December 2001, Phillip ordered a CT scan of my kidneys which showed NO abnormalities. In very late January 2002 I had my kidney function tests. These showed that I only had around 50% kidney function. This did NOT look good AND there was NO explanation for it. All sorts of things were discussed including that I did NOT have diabetes.

I was advised by my new Renal Specialist (Dr Robyn Caterson) that, even though there were some significant dangers in having a kidney biopsy, I really needed to have one. This was despite the fact that I had no obvious symptoms of kidney disease (other than, as I discovered later, frothy urine and mildly swollen ankles after I stopped taking my fluid tablets). Arrangements were made for the biopsy to be carried out “under CT scan”. Five weeks later (in March 2002 – my preferred date) the biopsy was done at RNSH.

The day after the biopsy I was extremely surprised to receive a phone call from the RNSH Renal Registrar who had supervised the biopsy. She asked me to come back to the hospital the next day for an appointment and more tests. When I arrived I was even more surprised to see that my Renal Specialist (Robyn Caterson) was also present and that both doctors were looking very grim. This is when I was given the bad news. The biopsy had shown that I had Primary AL Amyloidosis. After some genuinely shocking (but kindly delivered) explanations about the condition I was sent off to the hospital’s Cardiology Department to have a heart echoscope (to quote): “even though we have no real suspicion that your heart is affected”. Thank goodness, all indications to this day still exclude heart involvement, including the results of a later Thallium (?) scan.

When the heart echoscope results came back as “normal”, the doctors discussed possible “treatment” options with me. These were discussed at a fairly preliminary level as I was still “in shock”. I do remember being told that an autologous bone marrow stem cell transplant was the best option providing that I was “suitable”. (To make sure that I was staying in the real world I was told that there was only around a 40-50% chance that I would be suitable for such a transplant.)

Cost of diagnosis in dollars …… Nothing …….  (except for the original Endocrinology consultation cost). This information is provided to contrast costs often quoted for patients in the USA.

My next appointment was with Dr Christopher Arthur (haematologist) also a RNSH specialist (and researcher). He was very happy with my overall general health and recommended that we should proceed with the bone marrow transplant. He explained that the known success rate at that time (mid 2002) for autologous bone marrow stem cell transplants in the treatment of Amyloidosis was only about 50/50. Even so, what choice did I have …… this one or none. I was lucky to still be well enough to have the transplant. (NB: Dr Arthur also explained, at the time, that there was a chance - around 5% - of dying from the procedure itself. I believe that today, this percentage is higher ?? This is probably because some of the patients undergoing the procedure now are more ill when they have their transplant than was the case in 2002 when I had mine ????).

Everything was moving very quickly now and coming to terms with it all emotionally was very hard. As mentioned above, I am not married and my closest support person is my sister. My colleagues at work were wonderful except that they kept looking up “stuff” on the internet from the medical journals (rather than the USA based www.amyloidosis.org support site which was really the only patient support site available at that time). Everyone was looking so glum all the time. I told them “I don’t want to know, just let me take it at my own pace”. I did this and was able, without scaring myself to death, to glean enough knowledge from the NET to ask sensible questions of the doctors so that I could have some control and be involved in planning what was happening to me.

Next step ….. A bone marrow biopsy to rule out Multiple Myeloma (Oouch!). I also had a series of lung function tests. Results of both …… OK.

Then I was given lots of written material and an introduction to the Nurse Practitioner who was the RNSH Transplant Coordinator. I was definitely in the system now, but “oh what a system”. Although there are only very low numbers of Amyloidosis patients, RNSH does lots of donor bone marrow transplants (as well as kidney transplants) for other reasons, on what was to become “my ward”. I just knew that these people were GOOD and that they knew what they were doing.

Next I had to self inject G-CSF (Granuloyte Colony Stimulating Factor) over a period of 5 days to stimulate my bone marrow to produce lots of Peripheral Blood Stem Cells (PBSC). My blood was then checked to make sure that this procedure had worked. It had, so I was booked into hospital to have the stem cells harvested. This process took over 5 hours. Basically, my blood was taken out of one arm, fed through a large “filtering” machine to “harvest” the stem cells and then put back in the other arm. The stem cells were then taken off in their “transfusion” bags and frozen for when I returned to hospital to start the transplant at the time of my choosing.

As I said in my heading, I was diagnosed early and therefore had much more control over things than would normally be the case. I calculated (working with the hospital, my employer and my family) that going into hospital on Tuesday 30/7/02 would be best for everyone. So on 30/7/02 I booked into hospital. That afternoon I was sent to surgery to have a Central Line inserted in my neck. On Wednesday 31/7/02 I was given one massive dose of Melphalan (Alkeran) through the Central Line and then, because of my damaged kidneys I was given massive amounts of Saline through the Line for the rest of Wednesday and all of Thursday.

Friday 2nd August 2002 became my Day Zero as they call it. This was the day when my own stem cells were defrosted and given back to me, once again through the Central Line. These first few days were psychologically really challenging but otherwise very low key. The ward staff, including the dedicated Clinical Nurse Practitioner assigned to transplant patients (a different one to the Transplant Co-ordinator who organised all the pre transplant stuff) were tremendously supportive. There was NO turning back now.

As mentioned previously I had been admitted as a public patient to a Public Teaching Hospital. This meant that there were NO medical costs for me to worry about. Because I was so ill I also had all the best doctors (Specialist Consultants and Haematology Registrars) looking after me as well as my Renal Specialist visiting regularly. I also had lots of medical visitors asking about my condition and experiences, including visiting/seconded (from overseas) students and registrars. So what happened to me after Day Zero?

I got swapped backwards and forwards from an isolation bed to a shared 4 bed ward through out the rest of my stay in hospital which was close to 4 weeks in total. The ward I was on depended on what stage I was up to regarding my immune system’s vulnerability.

Over the time I was in hospital I had: major rashes; mouth ulcers; a couple of low level infections for which I was given antibiotics; black colored diarrhea as the mucous membrane was stripped from my insides; huge amounts of saline fed through my system; transfusions of red cells and platelets (for blood support); medication for retching and nausea; etc. What was the worse thing about all of this? THE NAUSEA. I will never forget how bad it was and continued to be for several months. AND oh course, I lost all of my hair and had been sterilized (but at my age this was not a major issue).

The most painful thing for me was stuff to do with my bad (poorly accessible) veins. First the insertion of the Central Line and later the constant daily blood tests gave me many bruises and a cringing attitude every time I saw a Pathology Lab person approach (and this was daily).

BUT HEY ……… HERE I AM ……….. Three years beyond Day Zero. All my medical indicators are back to pre transplant readings, including my blood Serum Creatinine levels (returned to and stable at 0.17). All of my doctors are happy that I appear to continue “in remission”. My immune system seems to be back to being very good at fighting off most things but my renal specialist will not let me travel to third world countries the way I used to (because of my continuing reduced kidney function). The stem cell transplant seems to have stopped the progress of the Amyloidosis at this stage but of course it has not given me new kidneys.

I returned to work (after 9 months on sick leave) working for 15 hours per week. This increased rapidly up to 22 hours per week. Then, after turning 55 in March of 2004 I decided that there are lots of other things in life (other than work) so I retired early.

I am back to leading my normal life except for the need to continue protecting my kidneys. For example: I must beware of things like having dyes as contrast agents during CT scans; I must be careful about traveling in countries where renal infections may be an issue; and I must avoid anti-inflammatory drugs as much as possible. I continue to see my GREAT doctors (Endocrinologist – Phillip Clifton Bligh and Renal Specialist – Robyn Caterson) at RNSH every six months for check ups (after doing a 24 hour urine test and having copious blood tests done).

BUT, I must finish on one “sour” note. I recently entered a different hospital for a day procedure. This was a routine colonoscopy (checking for bowel polyps). I had had three colonoscopies in the twelve years prior to my Bone Marrow Transplant done by the same gastroenterologist at the same hospital. However, this time during the procedure my bowel perforated and I had to undergo emergency surgery. The five hour emergency surgery left me with a colostomy bag to be removed at a later date once "normal" bowel function could be shown to have been restored.

What does this have to do with my Amyloidosis? Well the colorectal surgeon who did the emergency work reported to me that the ascending part of my colon was extremely “spongy”. Histology results showed traces of Amyloid in the vein tissue around the junction of the small bowel and the colon. I am convinced that this damage/weakness was there prior to my transplant (and so is my Renal Specialist who is my “Managing Medico” for the Amyloidosis) but this can not be proved.

In May of this year (2005) I returned to hospital for the “repair” bowel surgery.

I’m sorry that my story is so long, but so far (three and a half years after diagnosis and three years after treatment) it is a very positive story. I feel fully recovered (except for the recent bowel “mishap”) and have great confidence that I have beaten this thing. I tell my self that I am going to live until I’m 90. Considering that I’m only 56 now this seems to be a pretty good goal.

Hang in there everyone ……… and be positive. Always push for early diagnosis for everything, no matter what … be AWARE.

I have found that learning as much as I can about what to expect at each step along the way and then staying positive while taking part in the decisions about my treatment has helped me more than anything else. My doctors have assisted me on this path. No question is ever too small or too silly. Also the Australian Universal Health System (Medicare) eliminated all financial worry for me and my family. This was a major factor in keeping my stress levels as low as possible over the last three years (and ongoing). Thank goodness I did not have to sell my house like some people in other parts of the world.

Finally, the original American support web site was exactly that, A GREAT SUPPORT, giving me hope, not just negative medical jargon. NOW we have our own Australian version ……………..  thanks, thanks, thanks to those who have got it up and running, especially the Reid family members.

Sharon Page

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