To Breathe or Not to Breathe

My highly accurate illustration of what my lungs look like

Here are ssome of the things that are hard to do with a paralyzed diaphragm:

Sleep, go up stairs, pick up something off the ground, most yoga positions, walk the dog, walk farther than 1/4 of a block, swim, hike, get ready for the day, dance, keep up with children and puppies, stand up from a chair, hurry, carry things, go shopping, stand for more than a few minutes.

Instead of swimming, I kick around in the pool. I don’t even try basketball or tennis. Sometimes, when I’m someplace like Costco or Walmart, I forget I can’t breathe and set off at my pre-surgery pace. Somewhere around the clothes section at Costco or kitchen goods at Walmart, I realize my mistake as I run out of breath and get lightheaded. The fatigue is deep and all-encompassing. It’s unnerving to go from going about your day to feeling like you might collapse on the spot. I stop going places alone because I am never sure I can get there and back safely. 

One day I might be able to sustain a near-normal level of activity, but the next it will take all I have to go from the bed to the chair. I almost always have a headache and a stiff neck. The chiropractor tells me it’s because of all the work my body is doing to breathe. The weird thing is that I don’t notice the effort it takes to breathe until I am already out of breath. At the end of the day, my Apple Watch tells me I did 30 minutes of hard exercise; all I actually did was walk from my car to my office and back. Does it count as exercise if my watch thinks it was?

*****

The one thing that was abundantly clear from all of the Mayo appointments is that the loss of a functioning diaphragm in my left lung is having a sizable impact on my life and health. Everything else going on is manageable, but the diaphragm is an issue. When I did a “sniff test” (perhaps the easiest and shortest medical test there is), I saw a video of what my lungs do when I breathe in. The functioning diaphragm dutifully pulls downward, increasing lung capacity. The paralyzed one that is already crowding out my lung pushes up, lessening the capacity even more. Talk about useless!

It may seem obvious that losing half of a lung would make it hard to breathe, but, apparently, there are people who live perfectly happy lives without a phrenic nerve. They reach a point where they experience no significant difference between their pre- and post-surgery activity level. At my surgeon's urging, I’ve spent the months since surgery trying to push myself back to my normal activity levels. I can't even get close. 

I have two options. I can adjust to the limited activity level that is my new normal, or I can have surgery to try to improve things. The surgery* is major (with a 3-4-month recovery time), and the success rate is lower than I’d like. Also, at best, I can only expect things to get 50% better than they are now. I have no idea what that means in practice. Before the mass was removed, my lung function had been reduced to 80%. After the surgery,  sans phrenic nerve, it decreased by another 20-30%. Does that mean my current capacity is 50%, and the surgery could potentially get it back to 75%? No one can tell me for certain. 

I tend to err on the side of not doing surgery. I had carpal tunnel for 17 years before finally breaking down and having it fixed. During those 17 years, I couldn’t hold a phone to my ear for more than a few minutes, my hands tingled whenever I drove, I couldn't carry bags without losing feeling in my fingers, and I woke most mornings to hands on fire. It wasn’t until I could no longer function that I had the procedure. Ditto my tennis elbow. 

I don’t like playing the odds, and I’m not eager to have a big surgery that might not help all that much. But I know that if things are this bad now, they will only get worse as I age. For the pulmonologist, the decision is a no-brainer. Someone my age shouldn't be this limited in terms of lung function. Still, I really don't want to have robot arms forced through my ribcage again. 

This is not how I thought I would be spending my sabbatical semester, but when I am being rational, I can admit that I’m too young to be done with tennis and hiking and walking the dog. I’m too young not to be able to take the stairs. Here’s hoping it helps!

*  The surgery involves folding the diaphragm down like an accordion and stitching it in the folded position. It won’t fix the diaphragm, but it will give the lung a bit more room. 

In Which I Talk about My Health (Part 2)

In April, the mass is removed. The surgeon expected it to be the size of a lime. It is actually a small grapefruit. The surgeon is pretty proud of the fact she was able to get the mass out through my ribs, going so far as to reenact how she yanked it out. You know those cartoons where one of the characters has to put both feet on the wall and pull with his whole body weight to get his friend unstuck from a random hole? Her reenactment looks a lot like that.

I am happy to wake from surgery to find my sternum still intact, but having a grapefruit yanked through your ribs hurts.

With the grapefruit goes one of my phrenic nerves. The phrenic nerve controls the diaphragm which controls breathing. It turns out, not having a phrenic nerve is a drag, but I don’t learn that until later. During my four days in the hospital, I’m just happy to see my pulse ox finally get to 90%.

A text from Falcon/Pink on the day of my surgery. Lol.

When we receive the pathology report a few weeks later, we expect to learn about the stage and type of thymoma I have. Instead, we hear the words Castleman Disease (CD) for the first time. When they tell me I have Castleman Disease they do it with a combination of awe and happiness. The surgeon tells me she can’t believe my luck—the chances of my mediastinal mass being CD is infinitesimally small. She tells me when they come up with cases to try to stump med students, they don’t even consider using a case like mine because no one would ever be able to diagnose it correctly. It’s House-level rare.

I don’t know whether to be annoyed or thrilled that I have achieved this level of rarity.

*****

I read about Castleman Disease, and it’s not great. Flares land people in the hospital for months at a time. Treatment protocols require regular infusions of meds used in chemo. There is no cure. So little is known about it that newly-diagnosed patients are encouraged to share their tissue samples and test results to help build a database for future research. There’s a website, a Facebook group, and an annual summit where doctors and researchers update patients on new treatment protocols. After years of spine issues for Ren and a host of other health challenges with the kids, I feel infinitely tired of dealing with complicated health stuff. I feel like this is a battle I cannot fight.

When I see the hematologist, I learn I have Unicentric Castleman Disease (UCD) and not Multicentric Castleman Disease (MCD). I have UCD; I am lucky, but I still feel like sh*t, and no one knows why. The surgeon and the hematologist tell me it can’t be the UCD, but I wonder how they can be so sure since it seems I’m only one of a handful of people to ever have Castleman Disease of the thymus.

Although I recover from the surgery itself, I feel unwell all of the time. I wonder how I could go from a healthy, fairly active person to one who can’t do much of anything and takes 13 medications every day. If it isn’t the CD, then what is it? No one can tell me, so I get a referral to Mayo Clinic.

The Mississippi at the Wisconsin-Minnesota border

*****

Over the next few months, I spend a total of seven days getting evaluated in five different departments at the Mayo Clinic in Rochester, MN. I don’t live close to Rochester, MN, so this is not an easy feat.

Days at Mayo are long. The system is efficient, and everyone is incredibly nice, but appointments seem to beget appointments. Often you start the day thinking you have 2 or 3 appointments but end it having had 4 or 5. The ability of the Mayo staff to schedule and get people in and out of appointments on time is Olympic-level. Prior authorizations and ok from insurance companies happen almost instantaneously.

Mayo is a strange universe unto itself (Rochester, too, honestly). Hotels have Mayo rates and free shuttles to and from the hospital campuses. People chat about their illnesses over their free continental breakfasts or while waiting for the shuttle. They stay at the same hotel each visit and eventually make friends. At the clinic, everyone—from the doctors to the technicians to the people at information and check-in to the other patients—is unwaveringly positive and kind. Given that this is the place where people come when the other places have failed them, it’s both weird and totally understandable why people seem so glad to be here.

My seven days of appointments are enlightening in what they rule out as much as in what they discover. They help me put the pieces together. I learn more about how the Castleman Disease mass may have triggered many of the problems and get a better idea of which problems will and won’t resolve over time. I meet with a Doctor of Pharmacy to comb through my list of meds and come up with a plan to better manage side effects. I figure out which follow-up procedures I can avoid and which ones I need to do. In the process, I eventually stop coughing, and my voice edges closer to normal; my memory issues and brain fog are a lot less noticeable, too. I even start to sing again, albeit badly.

Statue along the Mississippi River

What’s the takeaway?

It’s complicated.

It’s possible that with more diligence by the doctors in 2023 and 2024, most of this could have been avoided. It’s also possible that the rarity of the thing was too much for them to grasp. It’s possible that the esophagus issues and the GERD and the brain fog and the crazy BP and the exhaustion weren’t related to the CD at all, but it’s also possible that they were.

The only thing that is abundantly clear is that I HAVE to do something about the paralyzed diaphragm because I miss being able to breathe. That surgery happens later this month.