How did it start?

Parkinson's disease develops slowly, we don't know what causes it, and it can't be stopped. But for most patients, the key word is slowly.

Apparently a common early symptom is a weakened sense of smells. I had that since my teenage years. I just thought it was because I was allergic a lot.

In hindsight, the last year I still played football, the accuracy of my passes started to suffer. I was always proud of my long and accurate passes and it was weird to miss the target by 2 meters. Interestingly, the Parkinson is more advanced on right foot but the problem was mostly affecting passes with the left foot. This was because you're then standing on the right foot and it no longer was able to provide a stable support.

I started getting neck pains and back pains. Bought a better chair, went to the gym. These actually helped quite a lot. But they don't stop the progress. Parkinson's is a "progressive" disease, which makes me smile, because I'd hate to have a conservative disease! Badabim.

Soon the right foot started "flapping" when walking. I could no longer open a champagne bottle because my hand was too weak. And my spoon would drop all the cereals - the hand shaking was at its worst in the morning. I had to get a classic keyboard with big keys with long "travel", so that I could type with my shaking hand. At some point I realized a glass of whisky helps against the shaking. I figured I could probably drink quite a few glasses and still write above average code, but also I realized this line of thinking had some problems...

It turns out at a certain level of seniority producing lines of code is quite a small part of your work and you spend more time reading, reviewing, analyzing benchmark results... And maybe 20% or so writing code. Famously, I also spent more time deleting code than writing new code. So it was fine but a bit worrying.

The diagnosis

There also isn't some simple test you can take to confirm or deny that you have Parkinson. Like a blood test or x-ray. The doctor will make you do various exercises and subjectively "scores" your performance. Like walk, run, clap your hands... If you get enough points, you are diagnosed with Parkinson.

To emphasize how vague all this is, the definitive way to confirm Parkinson's is when they do the autopsy. About 25% of patients actually turn out to have been misdiagnosed. Especially for elderly people there are all kinds of reasons why they may have tremors. In some cases the same medicines help, which further muddies the waters. One of the focus areas of ongoing research is to find some marker molecule that could be used as a reliable indicator that the patient really has Parkinson.

My first visit I didn't get the diagnosis and was sent home. I looked at the doctor in disbelief, so he said he'll schedule a follow-up a year later. I used Mongodb's generous healthcare stipend to buy a lot of physiotherapy that year. It does help.

Eventually the next year I was confirmed with Parkinson.

The math

My first doctor wasn't particularly chatty. He just gave me the prescription I needed, pretty much. Later there was a separate session with a nurse. She gave me the brochures from the Finnish Parkinson association I had already read online. Other than this, the treatment is great. I was given an account in an online portal and can chat with a nurse who responds in 24 hours. I can increase dosage through this system without having to actually drive to the hospital each time.

After getting the diagnosis I went online to read about Parkinson. Of course starting with Wikipedia. I learned that the average life expectancy is 5-15 years. But the next sentence says that Parkinson is an old people's disease and the average age to get a diagnosis is 65 years. Ok, so does that means I'm dead in 5 years or when I'm 80? I'm grateful for my university level statistics education that helped me understand it's probably the latter. But it was a bit worrying for a while. I'm not afraid of dying, but I am the sole provider with 2 relatively young kids, so just the logistics of dying sooner than 10 years were a bit problematic... A close friend immediately pointed out Michael J Fox (the Back to the Future guy) got Parkinson in his 30s and is still alive. This helped, acutally. Anecdotes can be more useful than averages!

Only about 10% of Parkinson patients are "young", meaning not yet retired. There was a separate resource for patients still working age. The top two advice are: 1) Ask your employer if you can work more from home, and 2) if you can be allowed to sleep longer in the mornings. I already worked from home for over a decade, and sleep until 10 or more, because I work with Americans who are 7-10 timezones away from me. So not much that could be improved here. My boss laughed when I "asked" for permission to do the above.

Part of the challenge in trying to understand numbers like average life expectancy is that there were a lot of new meds introduced in the last decades. And since Parkinson progresses relatively slowly already - especially with the already existing treatments there to help you - it seems that we simply don't yet have enough data available to know how much the new drugs will impact life expectancy. What I do know is that there's still a lot of "runway" in terms of additional treatments I can be given in addition to what I have now. So eventually I stopped fixating on average life expectancy, accepting the thought that it's not an exactly knowable figure at the moment. (Or rather, it's only exact as a historical number.) Instead I'm coping by setting intermediate milestones for myself. My immediate goal is to try to stay working until the mortgage on the house is paid and both of my kids have grown up and moved out. This is about 10 years. At that point, I can re-evaluate and set myself a new milestone. Note that there's also a lot of space between early retirement and kicking the bucket. So there's room for more milestones I'm sure.

The treatment

Once diagnosed, I got prescribed 2 drugs: Levodopa and Pramipexole. Both are relatively strong and can cause nausea and other side effects. The way around it is you start with small doses and then double every month. Much like the liver of an alcoholic will get used to greater doses of alcohol, my body got used to the drugs surprisingly easily.

Parkinson is caused by brain cells not producing enough dopamine. Dopamine is used as part of the signalling of the nervous system. When there is insufficient dopamine, the signaling gets noisy, which causes your muscles to either get stiff, or shaking or both. I have both and sometimes the two symptoms seem to cancel each other out in interesting ways.

So the basic idea of the treatment is you just add more dopamine to compensate! Just one small problem: Brain cells are so dense, that a large molecule like dopamine won't get to the center of your skull where it's needed. Levodopa is a finer grained molecule, which can penetrate your brain tissue, and acts as raw material to form dopamine inside your brain. About 5% of the Levodopa I eat actually gets into the brain, the rest circulates around elsewhere in the body and eventually gets into the kidneys. When Levodopa was first used in the 80s, a problem was that it would destroy the patient's kidneys in 5 years. So whether or not you eat your meds, you would die one way or another! Today Levodopa is administered together with an additional chemical that breaks it down. It's a tricky balance to try to get some of it into the brain and still break it down before it gets to your kidneys.

Side effect of Levodopa is that I wake up after 6 hours of sleep because I need to go to the bathroom. Sometimes I can catch sleep again, sometimes not.

Pramipexole is one of several recent meds that take a different approach: Instead of adding more dopamine to the signaling mechanism, it reinforces the signal on the receiving side. Brilliant!

Side effects of Pramipexole are interesting. Shopping, gambling and sex addiction. Ok, so if I get that, how can we tell the difference? Seriously though, we moved savings into a bank account I don't have access to. Just in case. Also you might hear voices or schizophrenia. It turns out side effects for many depression and mental health meds are symptoms similar to Parkinson. It's kind of a balance you need to hit, and the motoric problems and mental problems are just opposite ends of the same spectrum of symptoms.

The Pramipexole I take in the morning once per day. The Levodopa is more delicate: I take it 3x per day, with 5 hours in between. This gives a period of 15 hours that I can move and work fairly normally. After this the effect of the drug disappears (but I still have the Pramipexole) and the remaining 9 hours I'm expected to sleep. This makes me kind of a Cinderella: I can go to parties, but I need to go to bed around midnight, lest I turn into some kind of hunchback of Notre Dame. That said, on occassion I have worked past midnight with some American customers, and sitting still in front of the computer I can do just fine. At DataStax my role is more supervisory and advisory, so often I just listen and try to make wise comments, while others do the typing.

My doctor tells me I'm already maxed out on the Levodopa dosage. Probably can double or so the Pramipexole, which should buy 2-3 more years. (I haven't quite figured out whether the progress is linear or hyperlinear.) After that, the next step of treatments tend to be surgical. For example, a container can be inserted into the head that administers the same drugs directly into the brain, thus avoiding side effects in the rest of the body. Also a powerful treatment is simply to insert a device that electrically stimulates the remaining brain cells to produce more Dopamine. Finally, there were recently successful results with growing more brain cells through stem cell transplants. With these treatments it's perfectly likely to live until old age, at least until retirement.

The science

The combination of a relatively slowly progressing disease and a relatively active field of research into new treatments is interesting. It means that it's realistic that some of the currently researched treatments can arrive in time to help me, not just other future Parkinson patients. Since I like science, it's been interesting to read about ongoing research on a topic where I have a personal stake. As I already mentioned, Michael J Fox has managed to stay alive much longer than would have been expected in the 90s when he was first diagnosed. His foundation has literally donated 1 billion to Parkinson research. I benefit from this research myself already, as I have access to several drugs that weren't available in the last century.

The most interesting research of course is that with potential to cure or at least stop the progress of Parkinson, rather than just treating symptoms as they get worse.

It turns out some of this research is happening where I live! Just before I was diagnosed Swedish Karolinska Institutet and Helsinki University Hospital (HUS) finished experiments on a new protein called CDNF. Again there was the issue that you can't just swallow this protein and expect it to find its way to the center of your brain tissue. Rather, the patients had to have a small pipe surgically inserted to their brains, which was used to once a month spray the experimental protein into the center of your brain! But, there were some non-conclusive signs this helped restore brain cells that would produce more dopamine.

A really exciting development is a new drug called ISRIB. Details about it were published late last year from University of California. So far only trials on mice have been published, and not a single human clinical trial was attempted yet. The premise of this drug is that the whole family of neurological diseases like Alzheimer, MS, ALS, Down, and... yes... Parkinson!  ...are due to the same root cause. Rather than the dopamine producing brain cells dying, they are just blocked, or "inhibited" from doing their work due to some kind of inflammation or stress. Rather than trying to generate new cells, the ISRIB molecule simply helps get these existing cells back to a normal so they can continue producing their daily dose of dopamine.

What is particularly exciting about this drug is that you only need the treatment for a couple weeks, after which the brain cells are healed. This is very different from the current need to eat a lifelong cocktail of drugs.

While ISRIB has yet to be tested on human subjects, it is a simple chemical substance that can be ordered online from Ukraine. I have no intention of doing that just yet, but I do follow with interest the bold individuals who tried it and shared their experiences on Reddit!

A similar study was published in Helsinki University just last month, based on an entzyme called PREP. Both of these chemicals attempt to break down so called alpha-synucleins, which are believed to cause the death, or alternatively inhibit, of the relevant brain cells.

Why am I writing about this?

We're getting close to getting our second vaccine shots in EU and therefore hopefully getting back to regular business travel. I look forward to meeting my DataStax colleagues f2f for the first time and other friends at conferences. I can't really hide my limping right foot or shaking right arm, so I might as well be open about it. One of the symptoms is a relatively bad reflux, which means I can literally throw up a bit after a nice meal. Apologies in advance. (Most often I manage to keep my mouth shut and just go like, yummy, ice cream...)

Even during the past year I've made it a point to tell every engineer I managed about this, because it was important to make the point that even though I might respond to a Slack thread at 2 am, and then again at 6 am, I don't expect anyone else to work like that, rather a mild side effect of my medication is that I do wake up at nights. And I do go back to sleep after 6 am and often sleep several hours more!

It's regrettable I had to give up football, but to put this in perspective, most of my friends this age had to give up on it long time ago anyway because their knees and ankles couldn't handle it. I'm grateful for the 8 years I was able to play in the senior team before it came to an end. Apart from restrictions on some sports I live a fairly normal life. I'm lucky that my work is very interesting and something I'm passionate about, and I can also do a lot of gardening, sailing and family stuff. In other words live a normal and happy middle aged life.

One word to summarize how I feel is simply: Older. I continue living fairly normally, but can't do some sports, wake up at night, walk slower, and go to the bathroom more often. So far, these are relatively small things.