My Cousin's Death

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My Cousin's Death

The year was 1996, and I had traveled to England to spend the summer with my cousin at his farm in northern Lincolnshire. I arrived in late May, and was warmly greeted by my host. He suggested we travel in to the town and go for a pint at the local pub. I agreed and we traveled the few miles from his somewhat isolated residence to the nearby village of Barton upon Humber, a quaint historical village with a population of only a few hundred people.

However when we arrived at the Red Lion, as the local public house was called, there was an eerie air of sobriety. Everyone was watching the television behind the bar, which was tuned to the evening news. The story was that there had been an outbreak of bovine spongiform encephalopathy (BSE), also known as "Mad Cow Disease" at a farm in another region of Lincolnshire. This was obviously disastrous news, since virtually all of those present were, like my cousin, livestock farmers and such an outbreak had the potential to destroy whole herds of animals. Even those not yet infected might be destroyed or at least quarantined in order to eliminate the risk of spread of the disease.

I was given to understand, from my cousin on the return trip to his farm, that the disease originated from use of cow and sheep meat in animal feed, or offal, but that it had not been identified until 10 years before and the regulations banning protein supplements containing sheep and cattle offal had not been rigidly enforced until 1992. Little more was known about the disease, except that it did have a variant which could potentially kill humans who ate infected meat. However such cases were extremely rare relative to the huge numbers of infected animals.1

Over the following weeks the scale of the epidemic increased. Government officials from the Department of Health came round to everyone's farms to perform tests and in some cases the animals were ordered to be slaughtered and their remains burned. The whole thing seemed to me wholly medieval, in the sense both of the lack of any cure and the destructive solutions.

My cousin these days was acting somewhat strangely. He would forget to lock up the animals and perform other tasks around the farm.

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When the inspector came he grew very angry and refused to let him near the animals. Ultimately he had to be restrained. At the time, however, I attributed such outbursts and distractedness to the stress that the epidemic was having on all farmers. Also I had not seen my cousin in some time and so was not sure wholly what to expect. Thus I didn't think more about the issue.

Yet by July my cousin had grown worse. He limped and was hunched over and when I asked him if he had been lifting anything heavy or anything else that might have caused these back and leg problems he said he could not remember. He then shouted at me for always getting in his way, but his speech was somewhat slurred such that I thought he must have been drinking, yet I could smell no alcohol on him and was almost certain he had not had access to any. I let him calm down before I suggested he go to a doctor. He was strongly against it, which again was strange because when I knew him previously he had been most cautious with his health.2

I humored him for a few more days but his gait and limp were no better and he had begun to spasm [with] random moments such that he could barely hold his utensil at meals and was unable to do the work of the farm. I was able to get him to bed despite his protestations and called the local doctor. He came, but was unable to give a diagnosis. He suggested there was possibly a genetic mental defect but since the family had no history of mental illness this was unlikely. He referred me to a doctor in the city of Middlesbrough, which we went to visit the next day.

The doctor in Middlesbrough turned out to be a very competent neurologist, and he ran several preliminary tests on my cousin including blood testing and simply asking him questions. He told me he was concerned with his findings and decided to check my cousin in to the local hospital. This scared me, and I began to think there might be something very seriously wrong with my cousin. I checked in to a local hotel and went to visit my cousin regularly over the following days. It was now August and each day he seemed worse. He struggled to speak and when he did he seemed very disoriented and confused, almost like my late great uncle who suffered from Alzheimer's disease.

Finally in mid-August the doctor's conclusively diagnosed that my cousin was suffering from Variant Creutzfeldt-Jakob Disease (vCJD), the human form of BSE and likely had been since March. He had ingested some abnormal proteins, known as prions, which were now corrupting the normal proteins in his brain and slowly causing it to waste away. I was shocked, and asked what could be done. Sadly it turned out that as with the animal form there was no cure. I was told that prions have no RNA or DNA so they are far different from bacteria and other biological agents, thus combating them was almost impossible.

I returned to my cousin's side, but his stupor only grew worse. I delayed returning to school but was assured by the doctors they would do all they could. My other relatives had come down when I told them about my cousin entering hospital and they agreed to keep me informed. The next few months seemed to last forever, but despite all efforts my cousin ultimately died the following April, a little over a year after he was first believed to have ingested the diseased meat. I now understood the harsh measures taken to stop this disease since although rare it is truly heartbreaking to see.

1 Sean Henahan. Mad Cow Disease: The BSE Epidemic in Great Britain. 1996.

2 Adviware Pty Ltd. Medical Dictionary: VCJD. 2005.


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