New Skill Set

I'm proud of my mom; she's someone who has, for the larger part of her life, not had any medical issues. I think she has done amazingly well in mastering the skill of how to navigate the medical system under challenging circumstances.

My parents are part of the generation which saw their own parents live out their lives only visiting a doctor if there was a dire issue: Broken bones, surgeries or a catastrophic illness. Most things either resolved on their own or the person died, simple as that. No routine screenings for colon cancer, no yearly pap smears or mammograms. It's no wonder the medical system can be difficult to navigate- many illnesses, tests and reasons to visit the doctor have come into being only in the past 40-50 years. My parents' generation has had no modeling for this aspect of their lives and are now having to set precedence for the rest of us.

Just knowing when to call the doctor is a challenge. What symptoms are concerning? What is normal, what's not? How does one find the best care for their particular situation? What makes a good doctor and how does a patient find one? None of this is taught to us and, unfortunately, it isn't something that comes naturally to most people.

Medicine is 75% science and 25% art, in my opinion. It's more than just looking at lab values, numbers, test results. It takes some creativity to weave all the information together with the information gleaned from the patient: how they are feeling, how their illness affects them, treatments that are going to fit in with their life. It's not a one-size-fits-all kind of thing. Two people may have the exact same diagnosis, but with very different presentations and treatment plans.

Over the past few years my mom has faced an increasing number of medical issues, which she has handled with grace and good humor. She's a medical challenge requiring numerous experts and specialists, all of whom really need to work together since one change in medication can cause disastrous results. The learning curve has been steep- it's not easy to go from a healthy, active person to someone who needs to see a number of doctors, sometimes on a weekly basis.

I'm so happy she has a good care team in place, with most of her doctors at one facility, which both my parents are truly impressed with. Cooperation seems to be the key there, and an effort is made to accommodate the patient. For example, if tests are ordered they are scheduled at the time of other appointments, saving the patient a trip back in.

It's never easy navigating a change in circumstances, especially in terms of health, but I feel confident that my mom is in very good hands and can add that she is a top-notch patient. Definitely one of the more challenging aspects of life to master!

Unchartered Territory

You're never too old to feel left out apparently- it's not just for awkward middle school moments! As a rule I don't usually feel like an outsider when amongst my friends, but lately I'm more aware of the rift. I'm not one to bring up medical issues unless asked, assuming that unless there is some invitation to share that people would rather not hear about it. That is just my style and I'm not about to change things at this stage in the game. Anyone who knows me well understands this, I figure.

I truly understand that it isn't always about me. Just because I have serious medical issues that nearly always trump whatever others have going on, doesn't mean that my problems are worse, per se. I'm empathetic (I hope!) and try never to say "oh yeah, you think that's bad? listen to this!" In all honesty, I'd much rather listen to friends' problems which I consider much more interesting my own. That being said, I am somewhat sensitive to the fact that there is frequently no inquiry into my health, what is going on for me even a "how are you feeling today?"

I've reached a point in my life where I'm not able to easily participate in many of the activities that others in my social circle find fun. It's more difficult to get around with the O2. I can't be as active and my energy is lower. I need to avoid smoke. I'm ready to go to sleep when, by most people's standards, the evening is just getting interesting.

So what's the answer? I don't want friends to have to alter plans in order to include me. Why should their social lives and fun be penalized because I can't keep up? So do I politely smile and say "no thanks" when invited, or would I rather that they didn't mention activities I can't be involved in at all? Not sure. It's easy for me to see that isolation and pulling away is the path of least resistance, being around others who can understand first hand what this feels like is much easier and more comfortable. I know many people who wrestle with this issue and have yet to come across a good answer to the question.

As with most things, a work in progress. Here ends my mental dump and sort.

Cardio Workout

Today was my appointment with the cardiologist for a second opinion. What a long, strange trip this has been- starting last April when I failed a 6 minute walk test and was started on oxygen. The first cardiologist, Dr. P, interpreted the results of the level 3 Baird test completely differently than my pulmonologist. In fact, I preferred his interpretation: you're fine, just out of shape, that's why you are so short of breath. Try taking a baby aspirin each day and come back to see me in a couple years.

Whoa! Very different than what I was told by my CF doc: you have exercise-induced pulmonary artery hypertension as well as left ventricular failure. Easy to see why Dr P's diagnosis was preferable, no?

After hours of online research and asking questions of basically every medical professional I came across, I had the name of another cardiologist: Dr S. who runs the heart failure and transplant clinic at MGH. He conducted a study involving the level 3 Baird and cystic fibrosis patients several years ago which was published in the medical journal Chest. The down side is that he is extremely busy and difficult to get an appointment with. Needless to say, his secretary and I are now on a first name basis; I called at least once a week to try to get into a cancelled appointment slot. My persistence paid off and I snatched up today's opening.

Basically, he said he didn't have a clear picture of what is going on with me. I've stumped the best of the best! He was concerned about my blood pressure, especially when I exercise, when it topped out at 210/112, much higher than a normal person's. The pressure in my pulmonary capillaries is much higher than normal as well, 50, when it should be around 12. As if this wasn't enough, my left ventricle doesn't pump or relax properly and this in turn affects the right ventricle. Cardiomyopathy or heart failure. Oh, and a small PFO.

However, despite all the cardiac abnormalities, he doesn't think my shortness of breath is heart related; if it was, I wouldn't feel better using the oxygen. Not only do I feel better, but my O2 saturation is much higher when I use the oxygen. He's going to need to confer with my pulmonologist in order to discuss this and come up with a plan.

As frustrating as it is not to have a definite cause for what is going on at least I feel that I have the very best minds working together to figure it out. I suppose in the end it doesn't really matter whether the root cause is cardiac or pulmonary; my personal theory is that it is a combination of the two. In the meantime, I'll continue to use the oxygen for activity; I'll keep up with exercise, and (this is going to the the tough part!) cut down on my salt intake. I'll wait to hear back about anti-hypertensives and just keep on keepin' on.

I Need A Spoon-full of Sugar

Although I hate to dwell on medical issues they are an inescapable part of my life, so I will include an update here. Just the facts, please, ma'am.

Let's see, where was I? Oh yes, the echocardiogram results of a PFO back in June. After consulting with a cardiac surgeon last week (the head of the department, I might add) it was determined that I could skip the lengthy and involved approval process for PFO closure because of my CF and hypoxia (low oxygen levels). Skip to the head of the class. The only drawback being that I would need one or possibly two additional medical procedures prior to being able to have the PFO repaired.

Medical test number one: TEE or trans-esophageal echocardiogram. Basically the same test I had back in June, but a bit more detailed than the previous echocardiogram. For this one they need to do the ultrasound from within your esophagus, which gives a much better picture of the heart structures. Sedation is used as most people tend to not enjoy having an ultrasound scope inserted down their throat, and because of my history of CF I'd need to have general anesthesia in case of the need for a quick intubation. They'd give me light anesthesia so I'd still be breathing on my own, but were ready to step in should there be any difficulties. Ready, set, probe!

The test was actually quite simple, I had a nice sleep and woke up feeling not too much worse for the wear, only a slight sore throat and fuzzy feeling in my head. I actually found it easier to recover from than the sedation they usually use for such procedures. The results came in yesterday and indeed, it is a PFO with a trace amount of blood shunting from one atrium to the other. Next stop: cardiac cath/pulmonary lab to verify how much of a shunt there is on exercise to see if that is the cause for my hypoxia. If it is, then the PFO will be closed. If not, they won't fix it and we'll need to do a bit more medical detective work.

Apparently the Level III Baird test is so popular there is a 3 month wait time to have it done. A date should be confirmed today and I'll be put on the waiting list in case there are any cancellations. Part of me wants to have it over with and the other part would like to put this off as long as possible.

Cardiac catheterization is a procedure where a small incision is made either in the groin or above another large vein and a catheter introduced up and into the right side of the heart. During the level III baird test, not only is there a cardiac catheterization, but also arterial monitoring of the blood to detect oxygen levels, usually done in the radial (wrist) artery. Neither of these aspects of the test sound like much fun to me, but the icing on the cake is that the patient is required to exercise while all this is going on. I would like to wish the physician and other staff the best of luck in advance for this one.

After the results are in from that I'll have a much better idea of the plan. If there is significant shunting from the right to left atrium causing less blood to pass through the lungs then they will go for the repair. There's a part of me that is wondering if all this is worth it. Using the oxygen isn't that bad... sure, it's inconvenient but I'm much more used to the odd looks people give me. Is it really worth going through all these unpleasant tests without a guarantee that this is the cause of my low oxygen saturation? I suppose it is, but I can't pretend I won't be disappointed if the level III baird doesn't yield some helpful information. Being poked, prodded and probed has to have some benefit, doesn't it?