Broken Brains

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A state school, in my state at least, is a sanctuary of sorts for folks with broken brains. Aged 16 to near 100 years chronologically, the patients live with mental retardation, multiple congenital anomalies, uncontrollable seizures, schizophrenia resistant to most medications, and/or behaviors involving injury to self or others. Some are referred from the courts because of illegal actions absent personal accountability.

This particular state school is situated in a rural field outside a modest city. The 160-acre “campus” consists of 30 tired buildings, scruffy grass, some nice pecan trees, and chain link—lots of chain link and locks.

“Why do you work here?” I often ask the staff. The most common response is, “Because I really like it.” Remarkable people work here. They show very little fear in what is frequently a dangerous enterprise; have a warm and loving attitude; and eschew higher-paying nursing assignments in town. They are exceptionally calm around physical agitation, non-sensical verbalization, hyperkinesis, spittle, vomitus, enuresis, bowel incontinence, and flailing arms and legs.

The common feature of the patients here is that they all have broken brains. By genetic fluke, early injury, or some mistake in the brain's chemical soup or wiring, their brains do not function at all well. A curious common denominator is a gait aberration. They bounce, they shuffle, they crab walk, they flail, they festinate, they circumduct, they list, they stagger, they swing their arms or don't swing their arms, and they fall. They fall a lot. Walking must be one of our most complicated enterprises.

Some diseases are uncommon here. I have yet to take care of a patient with diabetic ketoacidosis, acute pulmonary edema, unheralded myocardial infarction, significant pressure ulcers, or alcoholic cirrhosis. Here a hemoglobin A1C over 7.0 is cause for a unit conference. Lipids are checked annually, thyroid profiles are reviewed frequently, tuberculosis skin tests are placed annually, and all vaccinations are current. Medications are given consistently and on time and diets are precise. My work consists of treatment of minor lacerations; annual physical exams and Pap smears; status epilepticus; bowel and bladder mischief; in-service education; and the institution of management plans suggested by a nice community of consultants.

How important is the correct diagnosis? For the traditional internist the correct diagnosis is critical. What if it doesn't make any difference in the treatment plan? A recent patient I examined carried the diagnosis of Down's syndrome. This wasn't quite right. Perhaps he has Waardenberg's Syndrome. Does it matter? How many of our undiagnosed neuromuscular syndromes stem from mitochondrial DNA mischief? I really would like to know but does it make any difference? Could we add to the medical literature, describe a new syndrome, help some confused parents understand, or get better at what we do? The allocation of scarce state resources suggests that we are better off if we ensure that everyone gets hepatitis B and influenza vaccinations rather than spend money on esoteric and costly laboratory testing.

I was at the school Thanksgiving week. I was reminded to be thankful. This is the gift the patients give to all who work here. I relate this experience because I want you to know of this cul-de-sac of medicine and to the special patients being cared for by some very special people.