Book Review: One Man's Struggle over Chiari Syndrome

Author and Chiari Syndrome sufferer, Rahael D'Alonzo, has written a fascinating personal account and investigation of this obscure, chameleon and pervasive syndrome, which often requires neurosurgery and may be life-threatening. His style is direct, his observations detailed and scientific, his accounts of human mismanagement of his diagnosis and treatment revealing but fair. His perspective is both useful and unusual because of his own background as a pharmaceutical research scientist and his strong personality and physical stamina. He has a sophisticated understanding of the United States health insurance industry.

Book Review:Raphael D'Alonzo, Contents under pressure: One man's struggle over Chiari Syndrome (Second Edition), Lulu Publishing, 2008

Chiari Malformation Syndrome

Author and Chiari Syndrome sufferer, Rahael D'Alonzo, has written a fascinating personal account and investigation of this obscure, chameleon and pervasive syndrome.

His style is direct, his observations detailed and scientific, his accounts of human mismanagement of his diagnosis and treatment revealing but fair.

Sudden overwhelming obsession with death

Shortly into D'Alonzo's book we find that the author puts his confidence in a priest after his life is suddenly rocked by shocking pain - out of the blue, but lasting only hours - and an obsession with death, and a craving for asparagus, which last for several months. It is 1980 and he is 27 years old. He thinks these may have been the first signs of Chiari Syndrome. Since D'Alonzo is a medical-pharmaceutical research scientist, his reliance on religious faith may seem rather odd to those of us unfamiliar with the pervasive force of religion in the US. One might perhaps have expected it more towards the middle of the book, perhaps nearly 20 years later, just before his brain surgery, when D'Alonzo was literally on his last legs - hardly able to walk, hardly sleeping, with little remaining muscular force. However, by that time D'Alonzo has placed himself at the mercy of medical practitioners and the United States "Health" system, which predictably took forever to diagnose him. There is an irony there, about the skills of priests and the skills of psychiatrists and medical doctors when dealing with the unusual.

If you look for them, you will find other Chiari voices in different countries attempting to find understanding and to educate. Click on the picture above for a video about the disease by SzChristie, who is a Hungarian sufferer from Chiari Malformation Syndrome. She first became aware of the symptoms of the disease when she was 19 years old. Her channel on youtube seeks to help people suffering from other rare diseases as well as Chiari.

Marathon

D'Alonzo was and is a marathon runner, but, in the book, I could not help but imagine that the paperwork and time-wasting required to extract money from the health insurers, must have been the biggest, longest marathon. D'Alonzo is a highly restrained but nonetheless astute critic of the US profit-obsessed health system and cogently articulates the tragic farces it orchestrates. For instance, he is sent home after major neurosurgery, with a fever, urinary retention, and a bottle to urinate in, when he can hardly even look down to aim into the bottle, and absolutely no aftercare. That’s barbaric, and stupid as well, for many treatable, but potentially fatal complications happen shortly after operations. Recovery from operations on the neck, spine, brain and skull are complicated (to say the least) and patients need advice and rehabilitation.

”Many people with Chiari have significant mental and/or physical deficits following decompression surgery. One problem right off the bat that nearly all decompressed Chiarians share is the need to regain full range of motion in their necks. I am sure that many have had automobile accidents as a result of not regaining full range of motion in their necks. There are, of course, many other problems such as balance or gait problems, pain, vision problems, weakness, and the inability to concentrate or recall words to name just a few.” (p.144)

“My arms were a different matter. They were both weak and painful. The surgeon told me that my arms would feel better in about a year but provided no explanation as to why they were sore in the first place or instructions to aid in their rehabilitation.”(p.145)

D’Alonzo also criticizes the New Zealand health system, through the experiences of one Chiari Syndrome sufferer there who also had to wait years for a necessary operation, which was delayed four times. The New Zealand and the Australian health systems are purportedly free, but delays in the public system and hidden costs for unavoidable procedures and hospitalization in the private hospital benefits schemes mean that these antipodean systems are not all they are cracked up to be.

Misdiagnosis and Pharmacy in Chiari treatments

To deal with some bizarre, incapacitating symptoms, D'Alonzo is prescribed some major psychotropic drugs over the years. He is refreshingly sophisticated in his understanding of psychotropic and other drugs. He observes his own reactions, checks with the literature and draws logical conclusions.

It is thus educational to read of his experiences – well after his cervical decompression operation - coming off a 7.5mg daily dose of Zyprexa (Olanzapine) and then later, considering his height and weight (he is 6 feet 5 inches tall) what amounted to a tiny dose of 2.5mg daily.[1] He had problems with withdrawals and remarks on his experience ceasing the 2.5mg:

”It’s difficult to find any official or valid information on withdrawal side effects of Zyprexa. Some anecdotal accounts on the Internet can be found but many believe that there are no withdrawal side effects, just the return of symptoms from the underlying disease. This was not my experience. First, I had no ‘underlying disease’. As I indicated, I was not schizophrenic or depressed. Over the next two days, I experienced considerable GI distress, twitching, chills and sweats. My mood was down as would anyone’s be who was not feeling well but was not clinically depressed. I could still experience happiness and laugh at ordinary jokes and humor. Finally at about 8 days after I discontinued taking Zyprexa, I began to feel well again. All of the withdrawal symptoms faded away, my mood was excellent and I could actually sleep.”

A pharmaceutical reseacher (former Associate Director of Research and Development at Proctor and Gamble Pharamceuticals) his observations on the efficacy of medications, his ability to separate side-effects from the effects of Chiari Syndrome, including secondary effects, such as depression, and to admit when he doesn't know, is most instructive. He applies similar gifts of observation and analysis to the various health professionals who attempt to treat him.

We see most of these people as if they were dealing with him quickly, at arms length, in their offices, after he has waited weeks or months to see them. We recognise the humility and desperate self-control of the patient who simply must find medical help for a range of bizarre and refractory ailments.

Depression

If you experience difficult symptoms in the absence of known cause which stop you from leading a normal life, you are likely to become depressed. D'Alonzo, who has been for some time part of a Chiari Syndrome self-help group, finds that more Chiari Syndrome people are treated by psychiatrists - and for depression - than are treated by neurologists. (Then again there is the problem in neurology of higher status being accorded to brain surgeons than to clinicians who imaginatively support, assist, listen to and educate people away from the operating table.) The Chiari Syndrome often remains undiagnosed or untreated even if diagnosed, with the symptoms as physical consequences downplayed in favour of being reinterpreted as a depressed or obsessed reaction with something apparently minor to the apparently objective view.

"CMI is usually misdiagnosed as multiple sclerosis (MS), fibromyalgia (FM), chronic fatigue syndrome (CFS) or clinical depression. Recent research by Dr. Dan S. Heffez suggests that as many as 20 to 25% of the 4 to 5 million patients in America diagnosed with FM/CFS have been misdiagnosed and actually have CMI."

D'Alonzo seems to waste literally years - and presumably enormous amounts of medical insurance and energy on psychiatrists. He is referred to them by ordinary doctors whom he initially consults for a strange sore throat which can finally probably be traced to pressure on the lower cranial nerves. One of his dominant problems becomes insomnia. These psychiatrists prescribe him anti-depressants and major tranquillizers in an effort to assist him to get sleep. And they try to find psychological causes. During one period he goes for eight days without sleeping, although this does not sound like mania.

Surgery

Surgery was put off for years even after a correct diagnosis was made. The reason was apparently confusion as to the importance of the amount of prolapse into the spinal canal of the brain. D'Alonzo's prolapse was judged not to be sufficiently profound. The basis of this judgement seems to have been a medical fashion, or poorly tested science. D'Alonzo tells us about this in detail.

Amazingly, after his successful surgical treatment one antidepressant appears to work on some severe recalcitrant symptoms. After his long-awaited decompression surgery, his insomnia and his preoccupation with it and the resurfacing of ancient long-forgotten resentments, were driving him closer and closer to suicide. Reluctantly he checked into a psychiatric facility which also had a sleep clinic. (It sounds like an unusually good facility).