Still Miles to Go

When it rains, it pours.

It often seems like all the bad stuff gets clumped together in one big barrage of awfulness. This hospital stay is surely no exception.

First, we’ve dealt with a few episodes of dystonic storms. These are often triggered for me by exhaustion, physical pain, and exertion. When these three things are combined, the storms are worse. Dystonia is a movement disorder that causes involuntary movement and posturing of different muscle groups. I’ve suffered from dystonia in my left side for about 9 years. Recently, the storms have caused me to have bilateral leg involvement. It’s painful and the treatment often given cannot be used on me due to the underlying mitochondrial disorder. In short, it sucks.

Secondly, I’ve developed an upper respiratory infection – likely from the multitude of children in the LGH Emergency Room – and that has caused a ton of coughing and congestion. Luckily, it’s been minor and has been treatable with some Robitussin with Coedine.

Next, we found out that my colon is dilated (stretched) to over 11 cm. We were told that medical professionals start to worry and think about surgery when colon dilation reaches 10 cm. This, understandably so, has us a bit worried. Although this is usually concerning, they’re trying to keep me out of the OR because it’s well-known how I react to physical trauma and anesthesia. So for now, we’re exhausting all non-surgical medical interventions we can think of. And we are surely getting creative!

This morning, for instance, we finally tried the Neostigmine (the drug given to patients with myasthenia gravis) with very little to no success. The positive is that we were able to stay on the floor. Instead of moving to the ICU, I had a team (including an internist, a nurse, and a nurse specialist) dedicated just to me and they monitored me closely with an EKG. Although my abnormally high heart rate kept my heart rate in a safe range, we had a few other highly undesirable consequences to the drug. All of my muscles (including my tongue) began twitching and jerking while I became overwhelmingly sweaty. My eyes wouldn’t focus and I was in intense pain from all the jerking so we discontinued the use of the Neostigmine after just two doses (I could have received up to 10). The literature shows that Ogilvie syndrome usually responds after one or two doses so it’s quite possible that additional medication wouldn’t have helped anyway. So we’re back to the drawing boards.

Finally – and I wanted to put this off until I had more time to process it – both my pancreas and liver have been showing signs of chronic inflammation and damage. Now, this is expected with TPN. Unfortunately, this pattern began long before the TPN began. It means that, more than ever, I need to get off the TPN since it’s obviously not helping the damage to these two organs. Additionally, because I’m essentially vegan and can’t tolerate high-saturated-fat foods, the damage was not likely to be caused by diet. That leaves the mito as the last logical culprit. The damage to these two organs is what – we hypothesize – is causing my wild blood glucose swings. The liver damage is also causing incredibly high cholesterol and triglycerides and there’s nothing we can do about it because most cholesterol-lowering agents also cause liver damage. It’s scary because there’s no real solution, but I’m confident I’ll pull through this latest hiccup just as I have all the previous.

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April showers bring may flowers

When you’re in the middle of the storm, you sometimes forget what good can come out of it. For instance, my episodes in Dec/Jan brought me to doctors that have been able to help me gain weight and get proper nutrition. Without walking through that storm (strapped to a lightening rod), I wouldn’t be nearly as healthy as I am now.

My hope is that this hospitalization will serve as a wake-up call to my doctors and we’ll be able to figure out some solutions to my horrible dysmotility/chronic pseudo-obstruction. Because I cycle through these episodes roughly every 2 weeks, it’s important to prevent them from happening – something my current home regimen isn’t doing.

I know it’s hard to see how the flowers will come from all these ugly storms, but I am confident the daffodils and tulips will bloom again.

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Always remember to play after every storm.
– Matty Stepanek, 1990-2004, who also had mitochondrial myopathy

We’re already plotting how to enjoy my escape.  A Sox game?  Perhaps a trip to New York City?  If you have any ideas of fun, local trips or activities, let me know because I’m gonna need to play hard to make up for this nasty storm!