Still Miles to Go

I’ve been home for nearly two weeks now. Recovery has been slow and has sapped me of most of my excess energy so many apologies. Because there’s so much to cover – and I’m having difficulties organizing my thoughts – we’re going to go about this bullet-style.

• I’m home. I was discharged with a four-week course of IV Vancomycin (a very powerful antibiotic) at a very high dose. Because we never got those sensitivities back (i.e. what bug we’re fighting and what antibiotic is best to fight it) and my reaction to the infection scared them (though I assure you, it did not scare nor surprise me or Keith), the doctors are playing it safe. I guess that’s what they do best. I was also on Cipro (another antibiotic), but I have finished that course.

Although the blood cultures (above) never revealed what bug we were fighting, we battled back aggressively.

• While still in-patient, my stomach suddenly (over the course of an hour or so) blew up to look as if I were ready to deliver a full term baby. This is due to what is called an ileus. This is when the gastrointestinal tract stops moving entirely. My GI has very little movement daily, but whenever I am sick, it stops completely. This is part of the response of my autonomic nervous system to stress. While it isn’t too uncommon for either me or someone with sepsis (a blood infection) to experience an ileus, it is always an issue that my doctors take very seriously. Why? Because when my colon in particular stops working, it dilates to dangerous levels and can possibly rupture. This itself can cause sepsis and a whole host of other issues. Luckily, we caught it early and were able to address it properly.

• We were reminded repeatedly how important it is for me to have central line access. Not only was I blowing lines (that were already hard to start) every few hours, but they couldn’t even find access to draw labs on numerous occasions. Additionally, my potassium (an essential electrolyte) drops rapidly and to scary levels when I’m not on daily TPN (IV nutrition that requires a central line). Luckily, my awesome nurses were able to effectively communicate how dangerous this was to my physicians (images of my bruised and battered arms and legs were an effective tool as well, I’m sure) and they went against protocol and inserted a new line when I was still on tylenol to control my fevers.

A central line is essential to deliver all the various infusions I rely on to keep me happy and healthy. It's not just an IV; it's my life line.

• We made our choice for central line access: a double-lumen PowerPort. What does that gibberish mean? Well, first it’s an implanted port. That means a small plastic piece was inserted under the skin in my chest. It means if there’s ever a time that I don’t have to be hooked up to continuous infusions, then I will not have visible tubes coming out of my chest.”Double-lumen” means the single line is split into two, allowing for two infusions concurrently (i.e. I can run TPN and IV antibiotics at the same time). And finally, it’s a “PowerPort,” which means it can handle infusions at high rates, such as the infusion of contrast for CT scans. Because I have frequent CT scans (unfortunate, but a reality), we knew this was a must. Otherwise, we would have to insert a peripheral IV each time I needed at CT scan and we’d encounter all of the above difficulties. Finally, it’s made of a non-electromagnetic material so it’s safe for MRI’s as well. Phew!

The Bard "PowerPort duo M.R.I. Implantable Port" - i.e. It's safe for CT scans and MRIs and allows me to run two infusions at once.

• Although the decision to go with the above Port was easy (more on that below), the insertion was, well, not-so-easy. The moment I reached the vascular surgeon, his looked at me nervously. Apparently, they weren’t used to implanting ports of this caliber into patients of my age and size. How do I know this? Well, throughout the 3-and-a-half-hour-long procedure (no, it doesn’t usually take that long), he alternated between muttering “you’re too young” and “you’re too little” under his breath every few minutes. Because it’s implanted under the skin, stretchier skin (as seen in older people) and adequate fat (as seen on people not me) make the implantation easier. Add in the fact that I needed a double-lumen and CT scan compatibility and it wasn’t an easy go for the poor man. But I happily chatted away with the very pleasant resident while the surgeon dug away at my chest during the procedure. Because I can’t have Versed (a muscle relaxant usually given during minor surgery), I was completely coherent. Luckily, I couldn’t feel much with the massive amounts of Lidocaine (a local anesthetic) the surgeon had pumped into my chest. The only discomfort I felt was in my neck where he had to guide a wire around an awkward bend and into my subclavian vein.

The port is implanted under the skin. Accessing it is simple with a special needle. Inserting the port, however, is not as simple.

• So, why did we decide to go with the implanted port? Why not go with a simple tunneled catheter (e.g. a “Hickman” or “Broviac”)? The answer is quite simple: quality of life. I don’t really care about body image (and it’s debatable which one would affect that more) and I have an incredibly high pain tolerance so the threat of weekly access using “big” needles didn’t scare me a bit. There was massive debate between all of my team as to whether the port or tunneled catheter would be better as far as infection risk; neither side was particularly more influential than the other. So what did it come down to? Swimming. The reality of mitochondrial disease is that I’ve had to give up a ton: hiking, most sports, running, and even walking. All those physical pleasures are (essentially) gone. Sure, there are adaptive sports that we do pursue, but I can swim unfettered. I can feel free in the pool where gravity doesn’t have as big a say. Yes, a Hickman would’ve been easier. But I’m not ready to give up on the pool. And that’s why we went with the port.

A summer preview

• Finally, the last few weeks have been pretty absorbed by planning efforts for this September’s Show of Hope. Keith and I joined Stef, Ralph, and Liz at a recent Air Traffic Controller concert to meet the band. Now we’re even more excited about the event. And I didn’t think that was possible! In case you don’t remember, Air Traffic Controller (along with the always lovely Liz Longley) will be headlining the Show of Hope, a pet project dreamed up between me and my best friend. The concert will benefit the United Mitochondrial Disease Foundation.

I think that just about covers the last couple of weeks. We’re really looking forward to having my cousin in town this weekend. She’s one of my favorite people in the world and she and I always have great fun when we get together. So until next time, all my wishes for health and happiness!