Goodpastures Syndrome

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

8. Goodpastures Syndrome - Prognosis Terminal

(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).

On 23 June I was transferred to a single room in M1. I was told that this was because it would be quieter for me than Mosenthal but I realised that I was now considered to be terminally ill. I knew this unofficially at the time because, firstly, it was commonly known among the patients that single rooms could not be had for love or money but were reserved for the dying and patients who for one reason or another could not be kept in a general ward and, secondly, because a few other patients had been moved out of Mosenthal to single rooms and then a few days later we heard through the grapevine that they had died.

This perception was confirmed after I left hospital and found a difference between the one hospital account and their reminder for the same period. I went to the hospital’s accounts office to try and clear this up and a very helpful lady pulled my administration file (as opposed to the medical file which is kept by the patient's bed). Paging through the file the words “Terminally Ill” in red diagonally across a whole page came into view.

M1 was the cardiac ward and would have been a very jolly ward had I not been in my condition and immobile because the patients generally were not in pain and spirits were high. I would often of an evening hear something resembling a party going on outside.

Time seemed to last for ever; I would lie counting the seconds, as it seemed for hours, and when a nurse came in to check the drip I would ask her the time. More hours would drag by and when the nurse came in for the next check I would again ask the time and discover to my misery that a mere 20 minutes had passed.

At this time I was visited one evening by Mrs Alberts, a lady who worked in the Legal Process sub-division of the traffic department and with whom up to that time I had therefore had nothing to do. Needless to say, I was rather surprised but later learned her to be a warm-hearted and caring person. She talked to me for a while and then looked around the room and said “You know, this is the very same room my father died in”. This remark did not exactly serve as a pick-me-up.

On 25 June Dr van Tonder* told me he had decided to perform a kidney biopsy to try and confirm the diagnosis of Goodpasture’s. Later, when I was able to reflect on it, I thought he had begun to doubt his diagnosis because I had failed to die as anticipated. It is only fair to point out that prior to the illness I had always been very healthy and fit above the average for my age (43), never overweight, non-smoker and almost a non-drinker, although not teetotal, but the doctor had only met me for the first time when I was seriously ill and grossly swollen with fluid due to renal failure.

Only a few years later there was to become available a blood test (Basement Membrane Antibody) to confirm Goodpasture’s but in 1990 the only options available were lung or kidney biopsies.

At 10:00 on 26 June Dr van Tonder* performed the biopsy under a local anaesthetic. It was agonising and left me very shaken. From this point on my recollection of the day’s events is confused and what follows is gleaned from my file.

By 18:45 I was complaining of pain and feeling very weak and dizzy. “Patient is very pale, pulse weak… abdomen slightly distended… patient appears to be bleeding. Dr van Tonder* notified.” One side effect of Goodpastures is bleeding due to a reduced platelet count. At 19:40 I was given one unit of packed cells and one of plasma, at 20:00 10 mg of Vitamin K and at 21:30 100 mg of Valoran for pain.

At 22:15 my blood pressure was 80/60 and my haemoglobin 8.5. “Dr van Tonder* notified”. At 22:40 I was given more packed cells and the circumference of my abdomen was 92 cm.

At 01:20 on 27 June the file records my saying “The pain is becoming unbearable”. Staff comments were “Patient moaning, movements restricted, pain is severe and shock appears present, packed cells… 50 mg Valoran.”

At 02:30 “Patient still moaning in pain, blood pressure 60/40, pulse 80 and very thready, Dr van Tonder* notified and instructs 20 mg morphine in 100 ml saline.”

“Breathing is more shallow, put in Fowler’s position, abdomen appears to be more distended, bladder is very distended and hard, patient does not pass any urine, says he feels faint.”

03:15 “Pasiënt se toestand onbevredigend, Dr van Tonder* getelefoneer en gevra om pasiënt te kom sien en het ingewillig”. (Patient’s condition unsatisfactory, Dr van Tonder* phoned and asked to visit and agreed). 03:35 “Seen by Dr van Tonder*, 20 mg morphine, Dr Scott notified for surgery”.

At 05:15 I was trundled off to an operating theatre, at 05:25 anaesthetic was administered and from 05:40 to 06:45 Dr Guillum-Scott removed my right kidney assisted by Dr Maasdorp.

In a report back on 2 July Dr Guillum-Scott wrote “Ek het… ‘n explorasie van sy regter nier gedoen en ‘n enorme groot haematoom verwyder. Daar was 5 klein punksie gaatjies in die onderpool van die regter (nier) sigbaar en uit een van hulle het arteriele bloed gespuit. Ek het geen ander opsie gehad nie as om ‘n Nefrektomie te doen. Na-operatief sterk hy goed aan.” (I performed an exploration of his right kidney and removed an enormous haematoma. There were 5 small punctures visible in the lower portion of the right (kidney), from one of which arterial blood was spouting. I had no other option but to perform a nephrectomy. His post-operative recovery has been good.”

Such was my ignorance at the time that I had pictured internal bleeding resulting in blood sloshing about all the abdominal organs and intestines. Apparently the body has its own mechanism of containing such bleeding, forming a sort of sac around it – a haematoma.

Many months later I was to drop in to Dr Guillum-Scott’s chambers to thank him for his good work and to show him the extent of my recovery. During our conversation he remarked that he had never seen so much blood come out of a patient on the operating table and the patient survive, but I do not know whether this might not have been an exaggeration to flatter me.



Goodpastures Syndrome - Prognosis Terminal             copyright 2011 Richard Binstead Goodpasture's Syndrome

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