LONGER TERM CONSEQUENCES

Goodpastures Syndrome

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


19. Longer Term Consequences

(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).

The consequences of my illness have been considerable with a variety of knock-on effects, both medically and in my career.

The haematoma in my lower abdomen had over the course of several months crushed my right femoral nerve leaving my right leg more atrophied and weaker than the other. This left me lame in that leg and it was several years before I was able to walk without a stick.

This was compounded when visiting someone in Cape Town who had a dog. This very friendly and obedient creature loved chasing balls and, unlike many of its kind, was very good about bringing the ball back and dropping it at your feet without a fight. Without thinking, I once kicked the ball sideways with my right foot and I could clearly hear something snap inside my knee which then swelled up like a football. Upon returning to Port Elizabeth I showed it to my then doctor who merely glanced at it and said that the swelling would go down and that would be that. But the knee is still very weak and easily put out.

My musculature obviously suffered extremely and, not long after leaving hospital, I lost much of the use of my right arm. Dr Smit* sent me initially for a CAT scan in case I had suffered a stroke and, when that came up negative, to neurologists who performed tests involving small electric shocks and measurement of reaction times which confirmed a quantifiable degradation in nerve responses which they tentatively attributed to the vertebrae in the neck where the nerve from the arm connects.

In March 1991 I became the second patient to have an MRI scan in Port Elizabeth. This clearly showed that the nerve was being pinched by one of the vertebrae which had shifted position. It was then proposed to send me to a neuro-surgeon to operate but there was an Assistant Superintendent at the Port Elizabeth Traffic Department who had had such an operation. They had cut his throat to provide access to the spinal column. At this point I chickened out being deeply afraid of operations. Over the course of some months the problem with my arm seemed to cure itself so I presume that as my neck muscles grew back they pushed the vertebra in question back into position. This problem has not since recurred.

A worse problem occurred later when using a weed-eater. I hurt my back and stopped what I was doing but by the next day I was immobilised with pain. I then spent two weeks in bed in immense pain, unable to sleep, until eventually I found just one position in which I could lie without pain. If I moved either foot one inch either way, however, I was in pain again.

Eventually friends came and hauled me off to Dr J.N.L. Oosthuizen at the nearby Westering medical centre where I was X-rayed and given a Voltaren injection which removed the pain. A few days proper rest and things were back to normal. Dr Oosthuizen informed me that I probably had a herniated disc which might from time to time move and pinch a nerve. This has, in fact, proved to be correct and I have had several bouts of lumbago varying from the severe, requiring Voltaren and Valium injections, to the mild where I simply need the assistance of a stick to move around. A complication here is that when I have to try and avoid bending as much as possible, I have to use my knees to substitute for the bending, resulting in added strain on them and the risk of damaging either or both of them again.

My reduced kidney function has also left me prone to occasional gout which is currently kept in check by taking prophylactic medication to reduce the purine in the body.

As mentioned earlier, in 1991 I developed Polycythemia Vera. This mutation of the stem cells in the bone marrow results in an overproduction of red blood cells which then thicken the blood until thrombosis occurs. There are several ways of treating this but it was decided that bleeding would be the safest and least intrusive for me. This is essentially like a donation of blood except that the blood is subsequently incinerated (after all, one would not want to transfuse another patient with my Goodpastures Syndrome antibodies!). The plasma is replaced faster than the red cells and so the haematocrit (the proportion of solids in the blood) reduces, for a time.

In the first few years I was being bled every two weeks when the books I consulted in the hospital library suggested three to four times a year as being normal. Eventually one of the sisters at the Blood Transfusion Service commented on how frequently I was there to be bled and asked me what medication I was on. I told her the list and she observed that iron and folic acid were two of the constituents of red blood cells. In other words, my medication was fuelling the disease – another case of the young doctors at the clinic simply repeating medication without considering the consequences. I was then able to tell these doctors to omit the two offending pills.

A side effect of the Polycythemia has been hepato-splenomegaly. One of the functions of the spleen is to destroy red blood cells at the end of their life. To cope with the increased quantity it swells in size as do the portal vein and the liver, which are all interconnected. Dr Oosthuizen had pointed out to me the dangers of this: a blow in the stomach area, whether deliberate or accidental would burst one of these organs which would result in death by exsanguination within minutes.

In 2009 he decided to send me for an abdominal ultra-sound scan to determine the size of the spleen etc for future tracking of changes in dimensions. The report came back that my spleen was “massively” enlarged and the portal vein and liver also over-sized. The report also revealed that I had “rolling stones” in the gall bladder. Dr. Oosthuizen then referred me to a surgeon, Mr. Russell, with a view to considering removal of the spleen. Mr. Russell, it turned out, was not worried by the spleen saying that a spleen can grow huge with almost no chance of rupturing by itself, although he qualified that nothing can be certain in medicine.

He was more concerned about the gallstones as, if these migrate from the gall bladder, they can cause serious problems. Normally, he said, he would opt for immediate removal of the gall bladder as the operation is quick and simple but (there always seems to be a “but” where I’m concerned) that system being in my case under hypertension would make the operation quite hazardous and he would prefer not to operate unless it becomes unavoidable. He observed that this might or might not happen. I was grateful that he had not turned out to be one of those surgeons who jump at the chance to demonstrate their dexterity with the knife, as there are rumoured to be. Unsurprisingly, as I have heard from others since then, Mr. Russell has an excellent reputation both for his skill as a surgeon and for his reluctance to operate unless necessary.

 

 

Longer Term Consequences             copyright 2011 Richard Binstead Goodpasture's Syndrome

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