Heart Fillings

The ritual filling of the purple pill box occurs every 7-8 days. I had this morning off and I’d taken my “T” meds yesterday morning so the filling happened this morning, “W”. I’m back to cutting the 5 mg prednisone pills into quarters. I think to myself that it might be the same amount of medicine if I just licked them a few times. The little pieces of devil are tiny. At my rheumatologist’s appointment last week, he said I’d gotten too eager with my self-designed prednisone taper and wanted me to take 8 mg instead of the 7.5 mg. But I’m trying to set up mail order pharmacy so I can get three months supply at a time and they are taking a while to get the 1 mg tablets mailed to me. So I slice and dice with my super sharp pill cutter. I try to convince myself it is cool to have a pill cutter and a trendy purple pill box. It’s not cool.

I also started taking bid hydroxychloroquine—“bis in die”—latin for twice day. I suppose it is fortunate that I already had to remember my evening dose of vitamin K to balance out the coumadin, but the twice daily dosing still makes it harder to remember and plan ahead for taking meds along on long call shifts. Ah, the life!

One of the attendings in pediatric cardiology clinic talked yesterday about how much he likes one of the rheumatologists. “It almost makes me with I had a rhematologic disease,” I overheard him say to the fellow. That still stings. I forget about it for a while, but comments like that and I remember. At its worst it’s like waking up the morning after someone you love has died. In those first hazy moments, you don’t remember, but then the awful reality comes rushing back and you just want to fall back asleep until the pain abates—or maybe forever.

“You don’t want a rhematologic diagnosis,” I look up from the heavy cardiology text book on my lap that I read between patient visits. I haven’t told them about my lupus. I get tired of talking about it. “I said ‘almost,’” he replied with a light-hearted grin and turns back to the fellow. “Which one would you have?” the fellow asks him, “Arthritis? Psoriasis? You should have psoriasis on your legs. You always wear pants so it wouldn’t matter that much.” He retorts that he does wear shorts when he’s not at work; he surfs and wears trunks then.

I’ve been wearing skirts to work more because my pants are still tight despite running and working out a couple times a day and fretting over my prednisone-increased appetite and weight. It’s good I don’t have psoriasis on my legs—the swollen arthritic knees are easier to conceal.

These past few days I have enjoyed cardiology clinic; I am learning a lot. I can hear murmurs that I could not have heard before. Another of the attendings reviews a thorough cardiac exam with me and tells me that the med-peds residents typically have better accusatory skills than the categorical pediatric residents. I hear them more and more accurately, and work on translating those noises into diagnoses and treatment plans.

We talk a lot about ventricular septal defects throughout the day. It is a hole between the two bottom chambers of the heart and the most common congenital heart abnormality. In one clinic day I see it in many of its stages. We have a baby come in with a classic murmur of a small VSD—holosystolic, harsh, best heard at the left mid to lower sternal border. A four year old Hispanic boy comes in with a history of VSD and a loud murmur, “solpo,” but he also has a fever which makes the sound louder than when he feels well. We will see him back in a month when he’s no longer sick. I listen to an older child who has the blowing sound only through part of the filling cycle of her heart beat instead of the whole cycle--nonholosystolic. The muscular part of the septum between her ventricles closes off the hole for part of the beat which is why I don’t hear the murmur throughout systole. We look at another child’s ultrasound of the heart (echocardiogram) that shows one of the valves covering the hole for part of the cycle; this will sound nonholosystolic as well.

One eleven year old girl who comes in pouring over her book report on “Where the Red Fern Grows” and in a hurry to get to her soccer and then her cotillion classes has no murmur any more. I listen carefully, making sure I don’t hear it. Her heart sounds normal to me. Lub dub. Lub dub. No lub-whoosh-dub. Not even a soft one. I ask her if she wants to listen. She looks at me with disbelief, “Really?”
“Sure,” and I put my stethoscope in her ears pointing the right direction for her to listen. When I do this, kids get a sort of trance-like look on their faces—lulled by the beating of their own hearts. Intrigued and soothed at once. I think it might be a reminder of their time in the womb when they could hear their mother’s heart and a simultaneous realization of their developing autonomy. This young woman loved it. I took off my stethoscope and she had forgotten about the book report and plans, “That was so cool! I’ve always wanted to do that! Wow.” Her mother smiled and told me I’d just made another doctor.

I went to present her to one attending, but Dr. Seltzer caught me first—the one who “almost” wishes had had a rheumatologic diagnosis. He had an interesting patient for me to see with him. I remind myself that it usually is not good to be the “interesting” patient. And in this case, it does not take one to know one. Though maybe it does take one to more fully empathize.

I quickly present the eleven year old to the first attending, who has been teaching me about VSDs all day, “Eleven year old female, VSD diagnosed as an infant, no cardiac symptoms, playing basketball, soccer goalie, no fatigue, on exam, no cyanosis, equal femoral and radial pulses, murmur has resolved.” It would be a confirmatory test for me to see if he doesn’t hear the murmur either. He asks me if I want to go with him to see her, but I choose to head in with Dr. Seltzer and his “interesting” patient instead. I would find out later that he agreed with my exam. “Diagnosis: closed VSD,” he scribbled on the billing sheet before dictating the visit.

I come in part way through the history. The baby is largely covered in the stroller but looks about two weeks old. The baby’s mother is overweight beyond the normal post-partum physique and I compare my body habitus to hers. Many people struggle with their weight; I’m not alone in that; I’m not even alone in having prednisone make it harder. Sometimes those thought bring comfort, sometimes not. When I went for a mid-day post 30 hour work shift jog on the warm Sunday beach day everywhere I looked I saw thin couples. The arthritis in my knees started hurting despite running on the soft sand and I walked home hanging my head and suppressing frustrated tears. Frustrated with this disease and frustrated that I cared so much about how I looked and frustrated that it made me feel like this longest non-relationship period of my adult life may still stretch far into the future.

Back to cardiology clinic, though. The baby’s father, a thin bearded red-headed kind-looking man was there, too, adding to the history and listening attentively. When Dr. Seltzer later told them their baby’s heart had problems, he would cradle her gently and repeatedly stroke her bundled small left foot while acting brave and trying to ask the right questions.

Their little girl had three heart defects: supravalvular aortic stenosis, pulmonary stenosis, and coarctation of the aorta. The first of these and sometimes the combination of especially the first two make pediatricians suspicious that she may have William’s Syndrome, a genetic disorder of chromosome 7 causing people to have variable expressions of Elvin facial features with “cocktail personalities,” shorter stature, high calcium, mild to moderate mental retardation and an unusually well-developed sense of pitch. Dr. Seltzer drew the cardiac defects on a diagram of the heart and told them that she would probably need heart surgery. He then explained that the pattern of her heart defects might mean that she has William’s Syndrome. He told them gently and reassured them that his William’s Syndrome patients were some of his favorites; he told them it could be lucky that she has this chromosome disorder passed on from one of them; that William’s syndrome children are very special. We referred them to a geneticist to see if his suspicions were real. Their beautiful baby girl with the full head of hair was not perfect—or perhaps she became a different definition of perfect.

Just now I listened to an NPR story about a teenager with William’s Syndrome. He feels very lucky to have it because it defines him; he seems to feel he would not be himself without it. But there in the exam room with Dr. Seltzer, these two parents with their first child, could not be other than mourning. The mother would start crying discretely as soon as we stepped out to get them a handout. The father looked paler than usual beneath his freckles; his eyes were wide and veiled and protective. The repercussions of this 30 minute doctor visit would spill far beyond that day. Perhaps they would spread only over the next months as she had her heart repaired and the genetic test came back negative. Or perhaps the diagnosis would fill the years of her life, defining and shaping her future in a way different than either of young parents dreamed.

Dr. Seltzer had been right, though. She is an “interesting” patient. Maybe one days she will be interesting enough to fill her own purple pill box, too.