Joostlike! One Too Many

Tuesday 31 May 2011: 4 years 8 months on … DEUCE

Some three weeks ago the name Joost van der Westhuizen was splashed across the media yet again.

This time is was not because of his rugby deftness or because he had been the Springbok rugby captain, nor his being South Africa’s version (together with his ex-wife Amore Vittone) of Posh and Becks, nor his SuperSport appearances, nor his new range of clothing, nor his trysts and bedroom indiscretions.

Unfortunately, Joost has been diagnosed with, what the media reports as, motor neurone disease (MND). Whilst it is sad that anyone is diagnosed with such an illness, I am pleased that the diagnosis in such a high profile person has suddenly raised so much awareness of these neurological problems.

I have had numerous queries regarding this disease and how it relates to the corticalbasal degeneration (CBD) with which I have been diagnosed.

Whilst I have no medical training, I will try my best to explain with the help of the reading that I have done since I became ill.

Motor neuron(e) diseases are a group of neurological diseases that selectively affect motor neurones, the cells that control voluntary muscle activity including walking, speaking, breathing, swallowing and general movement of the body. 

Forms of MND include amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS), progressive muscular atrophy (PMA) and Bulbar – but do not include spinobulbar muscular atrophy, spinal muscular atrophy, Charcot-Marie-Tooth disease (and many others).

What makes it confusing is that theses diseases are often referred to by different names in different parts of the world and by different medical personnel and agencies. They also follow different courses in different patients.

In the USA, MND is more commonly called amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, after the baseball player.

Readers may be familiar with Morris Schwartz (of the book, movie and play “Tuesdays with Morrie” fame), diagnosed with ALS, and Professor Stephen Hawking, sometimes referred to as having ALS and sometimes MND. My disease, CBD, is also sometimes referred to (correctly or incorrectly) as being a motor neurone disease.

Schwartz was diagnosed in his late sixties and told he had three to five years left to live – he lived for two more. Hawking was diagnosed in his early twenties – also given some few years, but is now 69 years old!

What has become a talking point is “WHAT” causes these diseases and why they appear to be on the increase! Everyone seems to have expert opinion in this regard.

To add fuel to the fire and to the skinder (gossip), this weekend’s press has reported on the use of steroids in South African high schools and rugby in particular. The dramatic headline reads “Steroid scourge rages in school rugby”.

And a further comment on this story by a reader on a newspaper website read as follows:

 “Here is why you must test for steroids:  Ruben Kruger, Andre Venter, Joost van der Westhuizen, Wium Basson, All Springboks in the same team, all seriously ill or dead from nerve related illness. SARU! If it is caused by steroids you must act. If it is caused by the game it must be banned (however much it is part of my soul)

The inference here is that nerve related illness may be caused by rugby and/or the use of steroids. This comment quickly did the rounds on Facebook and was added to by many of the social network’s   “expert” doctors. I also threw in my money’s worth – I have the “nerve thing” but was never a rugby player or a steroids user. If the inference is correct then I have lost out yet again: I could have had the “nerve thing” and big muscles! (Ironically, just on Monday it was suggested to me to request my doctor to prescribe testosterone/steroid injections in order to build up my atrophying muscles brought on by the CBD!)

My curiosity led me to read up on the above-mentioned players, or in modern-day parlance, I “googled” these names, and found the following amongst the players of the 1995 SA Rugby World Cup champions:

Ruben Kruger had a brain tumour, Andre Venter transverse myelitis (a disease of the spinal cord possibly brought on by disorders of the spinal blood vessels), Joost van der Westhuizen MND, Wium Basson liver cancer and Otto Krynauw brain haemorrhage.

Googling steroids, I found that the use of steroids may lead to many health issues, inter alia, cardiovascular and liver problems. So there may be a link … or is there?

 According to my further reading on neurological illnesses, about 90% of cases of MND are “sporadic”, meaning that the patient has no family history of ALS and the case appears to have occurred with no known cause. Genetic factors are suspected to be important in determining an individual’s susceptibility to disease, and there is some weak evidence to suggest that onset may be “triggered” by as yet unknown environmental factors.

The bottom line is that cause of illness is as yet unknown and there is currently no cure. Most cases of MND progress quite quickly and MND is typically fatal within two to five years (although we have seen that Hawking has lived for more than 40 years with the disease).

And this seems to be the pattern with all neurological illnesses – as yet, no known cause and no known cure.

So maybe a call for more research/investigation into the use of steroids is warranted. At the same time, maybe an investigation into school sport is warranted. One of the topics discussed at our recent reunion was the comparison between the size of players 25 years ago and their size today.

I am of the opinion – controversially so – that, whilst there may be no direct correlation between the “nerve thing” and sport, we have definitely lost our minds when it comes to the position that sport and especially rugby takes in our educational system today.

Steroids are used because too many hopes are placed on playing in first teams and on the need for them to win. There are too many teachers who coach and don’t teach, too many hours of school time used to organise, prepare and play, too many kilometres  travelled on tours, too many “social” gatherings, too many nights in hotels, too many meals in restaurants, too many unaccounted for and hard-earned rands spent by sponsors and expected to be paid by parents, too many rands in “retainers”  paid to school boys by franchises and unions – all in the name of rugby, of winning and of the supposed educational spin-offs that all these activities bring.

There is, in my opinion, too much money and time spent on what has become our international professional sporting enterprise. That’s why we have to pay R600 for a ticket to watch a game of rugby! And the process starts in our school system – in a country that can least afford such luxury and has one of the weakest educational systems in the world.

Where does it all lead to? As I write this, the popular unhappiness of dictatorships in Tunisa, Egypt and Libya (and elsewhere) has spread to that fiefdom of FIFA and its absolute monarch, Sepp Blatter. (And to think, it was just a year ago that we all in SA were shouting “Hail Caeser!” – it makes one wonder!)

In many (all?) cases, it’s the economic underpinning of the system that creates the root unhappiness.

Yes, please, investigate steroids, and all the rest. It would also be interesting to investigate just how much money is spent on the research of neurological illnesses vis-a-vis that which is spent on rugby!  

In the meantime, each additional case of neurological illness, is too many – one too many!

CIRCUIT BREAKER (version 2)

Tuesday 1 February 2011: 4 years 5 months on …

There are a number of neuro-degenerative diseases. Most people have never heard of them or have needed to pay much attention to them.

At the end of 1999, shortly after returning from a two-week study tour to the University of Disney World in Orlando, Florida, I had undergone surgery to rectify a herniated disc in my neck. A piece of my right hip bone was used to fuse two vertebrae in my neck!

In September 2006, just before my 50th birthday, I became aware that my left hand and arm were not functioning properly. As these were similar symptoms to those that had appeared previously, I thought that it was merely a recurrence of the problems of 1999. However, that was not to be, and it was after six months of medical tests that I was advised, on Thursday 8 February 2007 that I, most probably, had, what was referred to as an extra-pyramidal Parkinsonian disorder called corticalbasal degeneration. I was informed that I would become severely incapacitated within three years, BUT I was also told that there was a possibility that after five years I would possibly be able to return to sue the specialist for having made an incorrect diagnosis.

I have subsequently searched the internet and read many articles on CBD and various other neurological (and other) disorders. Most people have heard of Parkinson ’s disease (PD), but even then, more often than not, joke about the condition as the one where people rattle and roll and shake tea-cups and drop their cutlery. In fact, there are many differing types of PD, and no two people may have exactly the same symptoms. Some people with PD do not even have the shakes!

There are numerous neurological illnesses, diseases or syndromes or whatever you want to call the affliction. In my reading, I have encountered many, such as corticalbasal degeneration (CBD) – sometimes referred to as corticobasal degeneration, or corticalbasal ganglionic degeneration (CBGD). Then there is Amyotrophic Lateral Sclerosis (ALS) – in the USA often referred to as Lou Gehrig Disease, Motor-neurone disease (MND), Progressive Supranuclear Palsy (PSP), etc etc. (Often Muscular Dystrophy [MD] is also thought of as a neurological illness, but is, in fact, a hereditary muscle disease that leads to paralysis.)

It would appear that even two people diagnosed with the same illness do not necessarily experience all the same symptoms.

Two well known people with the same diagnosis: Morrie Swartz – of  the book / movie / stageshow “Tuesday’s with Morrie” fame had ALS, diagnosed in August 1994 and given two years – he died slightly more than one year later. Professor Stephen Hawking was diagnosed with ALS at the age of 21 in 1963 and given 2 – 3 years to live. He is still alive today at the age of 69 – completely paralyzed and speechless.

There are a whole lot of boxes each with a different label depicting a different condition. The problem is that it is extremely difficult to put a specific condition into a specific box. The reason being that these conditions all seem to overlap and symptoms from one condition or one patient may or may not appear in another condition or in another patient. And all they have to go on are the symptoms that they are able to observe.

To complicate matters more, it is almost impossible to conclusively put a condition into a specific box (or give it a specific label) unless one delves into the deep recesses of the human brain. And that, of course, is impossible until the person has died and the medical fundi’s are then able to scratch around in the brain and decipher just what went wrong! In more than 50% of cases of neurological disorders, it has been shown in death that the diagnosis in life has been incorrect! 

I say it’s like taking your car to the garage – telling them that there’s a problem in the engine and asking them to please tell you what it is, and to fix it, but not allowing them to open the bonnet of the car to check on anything. The most they can do is to listen to the sounds and watch the problems! Now that’s quite a tall order!

 There also appears to be quite a bit of confusion between the various disorders and their causes, so recently on the radio I attempted to explain it as best I could in the following manner:

Most people are familiar with an electrical circuit: there is the battery that provides the current that runs through the electrical wire and that illuminates the electric bulb in the circuit. In order for the bulb to light up, all the parts in the circuit must be in working order.

If any one part does not work, the bulb does not light up. If the battery is flat, or the bulb is fused or the wire is severed, short-circuited or not connected properly, then the bulb will not work!

If one compares this simple illustration to the human body, then the battery is the brain, the wire flex, the nerves and the bulb the muscles. In order for the muscles to work, the whole circuit must be in working order.

In the case of my CBD, the problem lies not with the muscles (the light bulb) or with the nerves (the flex), but with the battery itself (the brain). The brain cells that manufacture dopamine (a chemical required to convey the messages (the current) from the brain to the muscles have malfunctioned and no-one knows why. So the dopamine goes into short supply, the battery goes flat, it can’t be replaced or recharged, and, therefore,  the messages from the brain do not get to the muscles and they stop working! In fact, the muscles start contracting and that’s why my limbs, fingers, toes and shoulders have started curling inwards.

With other neurological illnesses, the problems lie with other brain cells, the muscles themselves or with the nerves, also resulting in paralysis, but resulting from a different cause.

Unfortunately, my battery this week appears to have gone into an ESKOM load-shedding mode, and I have not been able to finish off this blog. Instead, I have spent time with the medical professionals and popped some more pills!

PLEASE BE PATIENT – WE HAVE A PROBLEM!

 THE TECHNICIANS ARE WORKING ON IT AND WE WILL BE BACK AS SOON AS THE PROBLEM IS RECTIFIED.

Sunday 6 February 2011 …

 Circuit Breaker (continued) …

In MS (Multiple Sclerosis), for example, the problem lies with the flex – the nerves themselves. Nerve cells communicate by sending electrical signals down the flex – long fibres called axons which are wrapped in an insulating substance called myelin. In MS, the body’s own immune system attacks and damages the myelin. When myelin is lost, the axons can no longer effectively conduct signals, in the same way as when the plastic covering the elctrical wire in the flex is damaged and a short circuit is created. The earth leakage blows and the bulb does not glow. In the human body, various neurological symptoms appear, including physical and cognitive disabilities.

In the case of muscular dystrophy (MD), the paralysis is caused by hereditary muscle diseases that weaken the muscles themselves. MD is charcterised by progressive skeletal muscle weakness, defects in muscle proteins and the death of muscle cells and tissue.  

Motor neurone diseases is the term sometimes used to cover, almost as a group, all neurological illnesses that SELECTIVELY affect motor neurones, the brain cells that control voluntary muscle activity including speaking, walking, breathing, swallowing and general movement of the body. I have highlighted the word SELECTIVELY because it is obvious (from examples above and from case studies that I have read) that it is highly unlikely and unusual for any two people, even those diagnosed with the same disease, to have the same symptons.

What is frightening though, is the number of people who have these diseases and yet how little is still known about cause, treatment and prognosis. Unfortunately, I have not been able to source figures for South Africa, but the following are estimates for the USA (which has a population of some 450 million people – 10 times more than RSA):

  • Total number of people with a form of degenerative neurological condition – more than 6 million
  • Parkinson Disease – 1,5 million
  • Alzheimer’s Disease – 4,5 million
  • PSP – 20 000
  • MS – 400 000
  • ALS – 30 000
  • CBD – 10 000

 It would be interesting to research just how much money is spent globally on researching these illnesses. When I attempted to raise money for research work into CBD in South Africa, I was told that no such research work was being done in this country. Much research is done on the brains of those people who live and die with neurological illnesses and donate their brains to medical science.

Last week, I saw the medical people.

This week I have to see the legal people.

I won’t be leaving much material wealth behind. Anyone want my brain?