Parkinson's and DBS: the skinny

WE: our Plight, our Fight!

This is the last in this series regarding Parkinson’s and Public Awareness. So I want to thank you for patience. Well done if you've stayed the course…you’re amazing! My sincere apologies for this coming so late – I do fear I've lost my audience – it has to do with the two inevitabilities of life and it isn't about taxes. There; hope that helps to get you to read!

Instead of going on about the horrors of Parkinson’s (did I mention the tortuous pain of rigidities, spasms, and torticollis? These aren’t like a Charlie-horse, which is small and can be relieved quickly. They’re involuntary sustained muscle contractions which in themselves cause twisting, abnormal postures – horrible to witness and impossible to help – the body won’t be stopped! To add insult to injury, they’re often caused by trying to initiate movement), I thought I’d turn a moment to Dystonia, a Neurological Movement Disorder. Dystonia is simply awful and most often occurs in childhood. Although it can get worse with age, it is not neurodegenerative.

Scary picture time – promise no more after these!

These photos (deliberately old) demonstrate the motions associated with Dystonia in extremis; but they are also included in the plethora of more advanced PD symptoms. Alas, I could not find a photo that in any way demonstrated the sight of my husband’s torticollis just prior to his DBS op in 2012. I may be a sort of medical brat, but nothing could prepare me for that – his neck and face were pulled so tight that each attachment to every tendon was visible; straining at the skin – I thought his neck would break. His face was like Clint Eastwood’s Dirty Harry expressions times a thousand fold.

Still. I won’t go on about toe-curling, tongue-thrusting, choking etc. Let’s explore some PD fun facts!

I recently read in a Neurological Journal that oddly enough, people who smoked for 20 years have about half the chance of developing Parkinson’s that those who never smoked. We’d read something similar back in 2002, and that’s why my husband slapped on the highest mg. patch of Nicotine before a heinously busy lunch shift at our restaurant. Upshot: Remember what happened to Pinocchio when he drew deeply on that cigar? Same thing.

The patient’s ‘handedness’ correlates with the dominant Parkinson side. So if you’re right-handed for instance, it’s likely that’s the side that’ll first show. It’ll move to affect the other side eventually.

Atypical parkinsonisms are usually associated with little or no tremor, relatively early speech and balance difficulty, and little or no response to dopaminergic drugs, like Levadopa. This is what I referred to when I mentioned my husband was given the good news as he was Tremor-based. The other kind, akinesia and rigidity (AR-type), is much harder to diagnose. I’ve met some who waited 10 – 20 years before a diagnosis was possible. So, his ‘good news’ came after one measly year!
Remember I told you Parkinson’s used to be a 20-year life-sentence? Well, this was when they were ‘messing’ with Levadopa and other drugs. Then I showed you two ‘staging’ scales used today in rating to progression of Parkinson’s? Back in 1967, when Hoehn and Yahr first published their scale, their seminal study stated ‘the average disease duration for patients with Parkinson’s as 9.4 years’.

That was pre-Levadopa and other dopaminergic antiparkinsonism agents. Some in vitro studies have suggested that levodopa is toxic to dopaminergic neurons. This has led to the hypothesis that levodopa might hasten progression of dopaminergic neuron death as well as morbidity and mortality in patients with Parkinson’s (PD). There’s data that indicates survival was no better or worse in patients starting levodopa within two years of symptom onset versus greater than two years after symptom onset. However, the effect of levodopa on PD progression and mortality remains controversial despite its obvious symptomatic benefits for PD patients. Interesting…..back to morbidity, or life-span. And no, it’s not the PD that kills you (much like it’s not the gun, but the bullet thing), it’s the overwhelming symptoms and sheer bloody exhaustion that will finally lead to that last event; and it’ll be welcomed.

Comparing several published Medical papers (a spate published in the ‘90s’, so could well be updated), this is what you can calculate the likely reduction of life-span in Parkinson’s. OD = onset of disease, LE = life expectancy:

OD between 25 – 40 years: LE reduced from a mean of 49 years to 38. 11 years difference.
OD between 40 – 65 years: LE reduced from mean of 31 to 21 years. 10 years difference.
OD at 65 years plus: LE reduced from mean of 9 to 5 years. 4 years difference.
OD minus 40 years (Juvenile) mean age of death: under 60 years. 20 years difference. This figure has since been revised since to indicate a longer LE, but as Juvenile-onset Parkinson’s is so recent, it’ll take some time to get an accurate picture.

Reports of Juvenile Parkinsonism have now appeared in medical literature frequently enough to provide a picture of a disease which has an age onset of 3 to 30 years.

Just to belabour that point, the youngest reported case of PD is that of a 10-year-old girl from Oklahoma, who showed her first symptoms at TWO years of age. It took 7 years for doctors to diagnose PD. A case was reported in 1971 of two brothers who developed the disease at 10 and 19 respectively. They showed signs of stooped posture, shuffling gait, mask-like facial expression, resting tremor and rigidity. Both improved significantly with Levodopa therapy, but soon developed response fluctuations and abnormal involuntary movements. This peculiar phenomenon appeared in a matter of days and is the first mention in the literature of a characteristic pattern of treatment response in early-onset Parkinsonism.

Putting all that aside, I’ll tell you what really gets my goat: snake-oil salesmen. When you’ve first been diagnosed; and subsequently garnered as much information as your overloaded brain can stand, most PD sufferers will be very, very tempted to seek holistic or complimentary or radical therapies. This is the sitting-duck time for those without the science or compunction to prey on the vulnerable…and they do – all the time.

As a caveat to that, there’s no question that exercise and a healthy lifestyle can really help relieve symptoms as your PD progresses, as can Chiropractic, Physical Therapy etc. in management of symptoms. And I’m all for clinical trials. Not only do they offer up potential remedies (from Pharmaceuticals to Med-Tech to natural compounds), but they are highly regulated…bringing a product to market is one seriously expensive deal, so complete accountability under full scrutiny is a good thing!

So what CAN be done for PD sufferers? You may be getting the idea that I’m not too fond of Levadopa. I prefer to say I am ‘fearful’ of it, knowing its back-bite. This particularly, and other dopaminergic antiparkinsonism agents do have serious, unalterable toxicity problems. But it’s impossible, once a person has started on this course (the relief is tremendous for a time), to deny them that. Of course, there’s a bunch of Pharmaceuticals out there looking for an alternative – we’ll see.

Other potentials lie in Stem-cell and Gene therapies. The big problem is delivery. Crossing the blood/brain barrier is nigh-on impossible. In fact, one of the few things that can cross that border is virus, so piggy-backing a compound onto a harmless virus is under intense investigation and trial. The difficulty with virus is how to stop them doing what they do – replicating!

That just leaves DBS (Deep Brain Stimulation). This has my backing, for what it’s worth! Anyone who’s read my stuff will know that I’m pro Boston Scientific’s ‘Vercise’ device. That’s for two reasons; my husband has it and it’s the only med-tech device out there with a 25-year battery, unlike most with 3 to 5 year battery-replacement surgeries (and risk thereof) involved.

The thing with DBS is that it allows the patient’s intake of drugs to be (as much as) halved – less toxicity. Most PD sufferers wait until the point of no return and drug-efficacy has maxed out. The trouble with that is, by that time, they may well have developed something else (depression for example – common in PD) that will negate the chance of receiving this opportunity. More patients are now opting for earlier DBS. Personally, I’d like to see this choice made as early on in PD as they can, as it’s reversible and might well extend the life (with significantly less toxicity) of Levadopa. Absolutely everyone – regardless of which device – has said they've not regretted their decision to have DBS.

Right – I’m outty. Just leave you with a couple of video clips, if you will.

http://youtu.be/UGaPeIe0kkU 25 year old Brandon…still un-diagnosed – scared but hopeful!
http://youtu.be/pCONDfjm25s watch the whole thing. Martin is left-handed – left side affected. His Parkinson’s is Dystonia-based – as you see - no tremor. Notice the facial droop on the left side of face, toes curling and painful left arm spasms (rigidity). One brave dude!