Goodpastures Syndrome

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

9. Goodpastures Syndrome - Post-Nephrectomy

(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).

Immediately following the nephrectomy I was admitted to the Respiratory I.C.U. at P2 and then, at 10:30, transferred to the Cardiac I.C.U. also at P2. My only recollection of that morning is a very vague, almost hallucinatory, recollection of having become partly conscious, seeing a box marked “Poison” on the wall, feeling an undefined need to get out of bed and go for a swim(!), a sudden rush of people around me, a voice asking “Do you know where you are?” followed by unconsciousness.

The following morning, 28 June, I was transferred back to my single room in M1. I discovered a huge dressing on my right side which felt as stiff as a board. Amazingly I felt no pain from the wound, due I presume to morphine in the saline drip, only some stiffness if I tried to move. The nursing staff enjoined me to lie as still as possible so as not to disturb the wound. This was obviously good sense but the lack of movement also exacerbated my growing physical weakness.

The wound was bleeding internally as a side effect of the Goodpastures and, despite a drain, a fresh haematoma was forming in my lower right abdomen which in due course was to attain the size of a rugby ball and crush my right femoral nerve.

I was soon visited by one of the nurses from Mosenthal who had heard about what had happened. She asked me various questions which I tried to answer but every attempt at speech provoked a gale of coughing which left me weaker. Then she left and another one entered: same questions, same result. Then a third one came in and I realised that the wretched girls were in a queue outside waiting their turn. Their intentions were the best but they were literally killing me with their kindness and I had to ring my bell and ask the ward staff to stop any more of them visiting me.

On 30 June I was taken to P3 High Care for my first plasma pharesis as it was necessary to be on a cardiac monitor while this process was undergone.

Plasma pharesis involves pumping out a quantity of blood, separating the plasma (which carries the antibodies) in a centrifuge and then replacing the plasma with fresh uninfected plasma. This obviously only dilutes the number of antibodies in circulation and so a continuing course of pharesis is needed in combination with powerful immuno-suppressive drugs to crush the immune system and prevent it producing more antibodies and cortico-steroids to reduce the vasculitis (inflammation of the arteries, involving some failure of arterial walls and internal bleeding).

Two sisters from the Blood Transfusion Service had brought the pharesis machine from their building next door to the hospital. From the start, it alarmed me as it appeared as something that that been hammered together in someone’s garage out of odd pieces of metal and bits of kitchen junk. Its engine was also very noisy. After this experience I was taken back to M1.

I can not say why there should have been such a long delay between diagnosis and commencement of curative procedures unless it was perhaps doubt about the diagnosis. Examination of both the biopsy specimens and the kidney itself at the Institute for Medical Research in Johannesburg confirmed the diagnosis of Goodpastures. Dialysis had, however, been continuing on a regular basis.

On 1 July I vomitted up 1000 ml of gastric fluid. 20:30, “’Voel ongemaklik’. Abdomen kom gedistendeerd voor. Dr Krige geskakel.” (‘Feel uncomfortable’. Abdomen appears distended. Dr Krige contacted). A nasal gastric drain was now inserted which caused me great discomfort.

On 2 July there was further decline. 05:00 “Kom bleek voor. Tandvleis bloeding kom ook voor. Skrotum en penis kom voor of daar onderhuidse bloeding is, donker pers blou in kleur. Pasiënt kla nie van ongemak maar kom baie pap en lusteloos voor.” (Appears pallid. Bleeding gums also apparent. Scrotum and penis have appearance of subcutaneous bleeding, dark purple-blue in colour. Patient does not complain of discomfort but appears weak and apathetic).

That same day I was visited by a friend from my days in the Durban City Police (1978/79). Johann and Barbara von Bargen had become very good friends of mine and continued so after I left the D.C.P. Johann had also later left the City Police to take up advanced driving instruction. He has since become South Africa’s leading instructor in defensive driving and anti-hijacking techniques as well as being a popular personality as the Traffic Guy for Natal's East Coast Radio but in 1990 he was really struggling financially to get his business up and going, For him to buy an air fare to Port Elizabeth and then take a taxi to the hospital, having no idea of its location (the same situation as in Durban would have cost many times as much) was a considerable risk.

He had been in touch with Sister de Kock of the Renal Unit so I knew he was coming but did not know when to expect him. While being dialysed I heard a knock on the ward’s door and then a short conversation between Sister de Kock and someone outside. I thought I recognised Johann’s voice but then the Sister left the ward. I was frustrated, thinking “Time’s a’wasting” and then my bed was wheeled back to my single room. Only then did Johann appear and spend the rest of the day with me until he had to catch his flight back to Durban.

Months after leaving hospital, when I was well enough to travel, I went to Durban and stayed with them a few days. It was only then that Johann told me what had happened. On arriving at the hospital he had been informed that I was in M1. When he found that ward he was told that I was up in M2. When he arrived at the Renal Unit Sister de Kock took him aside to the nurses’ tea room and talked to him about my condition. As Johann put it, “She told me, ‘Before you see your friend there’s something you should understand: everything has stopped working except his heart and he’s going to die, but not while he’s in my charge.’”


Johann von Bargen, the "Traffic Guy" of East Coast Radio


Goodpastures Syndrome - Post-Nephrectomy             copyright 2011 Richard Binstead Goodpasture's Syndrome

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