Wednesday, April 4, 2012

Rainwater's Disease

So Richard Rainwater has PSP. At least that was the story on a CNBC panel interview this morning.  Squawk Box is aired so early in the morning that such a topic is all the more disturbing. Somehow early AM bad news is disquieting in an empty stomach sort of manner.

This rare disease is one of the curses of living on the way to death. PSP is always fatal as a result of the complications of the disorder (most commonly pneumonia). Progressive Supranuclear Palsy is often confused with Parkinson's disease, especially if there is an associated tremor. But PSP is not Parkinson's, especially when it comes to a response to levodopa. PSP patients do not fare well with this drug. Moreover, the disease acts more like Alzheimer's disease than Parkinson's.

PSP is characterized by blurred vision, abnormal eye movements, dizziness and frequent falls. The tumbles often lead to fractures and other injuries. Difficulty swallowing is common, resulting in aspiration and pneumonia. Often feeding tubes are placed into the affected as the disease progresses. Worse yet, there are often mental features of this disease including apathy, emotional lability, confusion and loss of mental acuity, which are particularly vexsome. Loss of dignity and integrity are poignantly hurtful. The average lifespan after diagnosis is 4.5 years...

The cause is unknown. Unknown is always a difficult disease etiology. Treatment, then, is at best supportive and palliative. There is no cure! Of course, theories abound... slow viruses, abnormal proteins (tau), free radicals run amok and genetics. Well, isn't everything genetics, one way or another? Diagnosis is mostly clinical, usually delayed and often difficult. Misdiagnosis is common.

Rainwater is a well known investor, having make a fortune in oil investments, early Disney and a myriad of other bold and creative ventures. He is worth $2.3 billion, give or take. His disease was diagnosed in 2009. Initially he worked for the Bass family of Texas and of note is the fact that he lost the first $5 million he had to invest as a young businessman. Later he and the Bass group made a lot of money together. Initial failures are often not predictive of future successes.

For sure, research has been ramped up. It is hoped a revelation, a discovery or a miracle will become evident to help all of the people afflicted with PSP. When high profile individuals come down with medical maladies, aggressive action is prompted. Richard has already attacked the problem much like he would handle any difficult issue. He has gathered experts in research to tackle the PSP mystery in forming the Tau Consortium. If anybody can beat it, yup, it would be Richard Rainwater.

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