Goodpastures Syndrome

Too close for comfort - a near-fatal encounter, told by the patient

16. Goodpasture's Syndrome - Thoughts on Survival

(This is a true account of my experience with Goodpastures Syndrome, but a few people’s names have been changed, indicated by *.  My aim in writing this is threefold; first, that victims and families of people suffering from Goodpastures Syndrome can have some knowledge of what to expect in a serious event but also to show that Goodpastures is survivable, even in a case like mine; second, it would do no harm for physicians treating Goodpastures Syndrome or other devastating diseases, not to mention GP’s prescribing medication to patients, to read this as there are lessons here for some of them; lastly I am trying to exorcise the psychological after-effects of my experience with Goodpastures Syndrome).

How did I survive such a devastating illness? In essence, because the treatment works. In that case, why did the disease have a 90% mortality rate? Because the treatment takes time and the disease is so severe that most victims’ hearts succumb before the treatment can take effect. In my case on the debit side were a number of factors:

  1. Late diagnosis. Dr Smit* later told me that he had never even heard of Goodpasture’s Syndrome and, considering how rare it is (the previous case in the Eastern Cape was ten years before mine), I would think that this is generally the case among G.P.s. Only following my renal failure was the correct diagnosis arrived at;
  2. Severity. Goodpasture’s typically presents with pneumonia and renal failure. There are, however, degrees in renal failure. The worst, as in my case, is aneuric failure, i.e. with no urine produced at all. In such a case, not only do the toxins circulate in the blood but the body accumulates fluid until the victim drowns in it, as so nearly happened to me;
  3. The biopsy. A renal biopsy is of its nature a risky procedure with something like a 50:50 chance of destroying the kidney. Needless to say with my luck the odds fell the wrong way;
  4. Complications. Vasculitis, hypertension and the bleeding consequent upon the failed biopsy and subsequent nephrectomy;
  5. Plasma pharesis. The twelve days I went without this treatment, and I am in no position to comment on what medication I was or was not receiving, represent twelve days lost in my treatment that took me within a whisker of death.

On the plus side I had two things in my favour:

  1. I was in good condition for my age, never having been overweight, a drinker or smoker, walking a good deal and two or three times a week swimming one kilometre non-stop. As a result my heart was very strong and, while I have never been physically powerful beyond my size, I have always had plenty of stamina. This, I believe enabled me to last out long enough for the treatment to take effect;
  2. That I fell ill in Port Elizabeth. I did not know it at the time but the Renal Unit of the Provincial Hospital here was considered to be the best in the country. In fact, on one occasion I saw an outpatient being dialysed who seemed familiar to me and it turned out to be a friend of the Oosthuizens in Pietermaritzburg whom I had met once. She had been a renal patient for some time but, despite there being a major hospital there, they had decided to move lock, stock and barrel to Port Elizabeth in order for her to receive treatment here. The two Senior Sisters of the unit, MacKenzie and de Kock, must take most if not all of the credit for this as they had infused all their staff with their own efficiency, enthusiasm and positive attitude. The morale of the unit’s staff was excellent and this transferred itself to their patients. As I have already mentioned, I believe it to have been their intervention which caused the plasma pharesis to be resumed, ultimately saving my life, and it was certainly their attitude and encouragement which prevented my despairing earlier than I did.

Subsequent to my discharge I visited the hospital’s medical library and read up what I could about Goodpasture’s. There I read that the lucky survivors had been known to live “up to twelve years”. I do not know what the statistics are to-day but, as I write this, I have managed twenty years. Do I have any thoughts on how I have survived for so long?

Yes I do; in early 1991, some months after leaving hospital, Dr van Tonder* further diagnosed me, after a series of tests, as having Polycythemia Vera. Going back to the library I discovered that this is caused by a mutation in the stem cells of the bone marrow. Naturally I wondered what might have caused such a mutation and for a long while I assumed it must have been all the X-rays I had. A year or so later, a bit slow on the uptake, I wondered about my medication. At the time I left hospital I had been on substantial doses of Cyclophosphamide, an immuno-suppressive drug, as well as a considerable cocktail of other pills. My hair had started to fall out in handfuls and when I moaned about this I was switched to Azathioprine.

Now, the next time I collected my medication, I asked for an information leaflet on the Azathioprine and discovered it to be both mutagenic and teratogenic (monster-forming, like Thalidomide). Only then did it dawn on me that these powerful drugs, life-saving though they were at the time, were also highly toxic in their own right and I immediately decided to work myself off them which I did over an extended period of small reductions. When Dr Peter Crafford, the psychiatrist I was seeing at the time, heard of this he was sufficiently concerned to contact Professor van Zyl Smit about it. The Professor gave his opinion that I should be all right provided I took care to avoid contact with or inhalation of all hydrocarbons and smoke. I suspect that if I had continued with this medication more unpleasant consequences would have resulted in the long term and my survival would have been shortened.

In all fairness to Dr van Tonder* I was obviously still at considerable risk at the time of my discharge and needed these drugs. Later in 1991, however, I no longer saw him at the clinic and in his place saw a succession of young doctors who mechanically repeated the original prescription he had issued.



Goodpasture's Syndrome - Thoughts on Survival             copyright 2011 Richard Binstead Goodpasture's Syndrome

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