Pediatrics II

One other patient I saw was a 20 year old girl who had gone through more than most people do in a lifetime. Born with a fairly subtle congenital heart defect, she caught an infection of a heart valve that sent her to surgery after surgery. The literature in antibiotic prophylaxis for dental work for those with congenital heart defects is mixed. Previously, we would give antibiotics to patients who had abnormal valves before they saw a dentist. Mucking around with teeth, it turns out, is a risk factor for seeding the blood with bacteria. More recently, guidelines have changed, suggesting that it is not necessary to give pre-dental antibiotics. Large studies suggest it's not cost-effective. But take this patient in particular. She did not receive antibiotics, and got a valve infection that could not be cleared with antibiotics. She had a valve replacement, but it was complicated by an aneursym of the aortic root. When she got the aortic root repaired, she had heart block and required a pacemaker placed in her teenage years. Then she does well for a few years until her mechanical heart valve gets infected; mechanical heart valves are so prone to infection that in someone her age, it was inevitable that some time, she'd probably get endocarditis. She had her aortic valve replaced again which caused more trauma to her aorta until the surgeons deemed her inoperable. Finally, she gets a heart transplant. Although we think of heart transplants as new slates, they unfortunately aren't; she works hard to stick with her transplant medications, more medicines than most people take until they are senior citizens. She tries to live a normal life - make friends, go to college, find a job. But finally she comes in to the hospital because she starts to have neurologic signs - vision cuts, vertigo. It's a little worrisome. On exam, I find that she has bulky lymph nodes. Initially, I was concerned about meningitis (since patients are immunosuppressed with their transplant medications) but now I was putting together a picture for a disease I knew only a little about - post-transplant lymphoproliferative disorder.

PTLD is a lymphoma-like picture seen in the post-transplant patient population. Unfortunately, it meant we had to cut back on the patient's immunosuppressants, risking a higher chance that she'd reject her new heart. My post-call morning was a-flurry. Oncology felt that the neurologic involvement made this a near-emergency; they wanted a stat biopsy by interventional radiology, neurosurgery, or ENT as well as an emergent consultation by radiation oncology. This was all complicated by the fact that she concomitantly had an acute surgical emergency, but surgery wanted to hold off on operating because it would set her chemoradiation back. I realized how complicated it was juggling recommendations from differing services. In the end, we managed to treat the surgical emergency with aggressive medical care, got a stat biopsy, and began chemotherapy to decrease the disease burden of PTLD.

Throughout the course, I was awed by this patient's self-sufficiency. She went through hardship after hardship with nothing but perseverance and the hope to achieve a quasi-normal life. It really is a privilege for me to meet and work with and treat these patients, and I have a lot to learn from them.