Sex, Drugs, and Public Health

Five year-old Jhon was carried into our exam room for clearance by anesthesia. The nurse reached for him, and the anesthesiologist jumped up.

“Be careful with him! Don’t even take a blood pressure – you could break his arm.”

The boy has a severe case of Osteogenesis Imperfecta – a genetic defect in bone metabolism that results in a delicate, easily fractured and deformed skeleton.

In his X-ray you can see that the “long” bones of the legs are neither long nor straight. Moreover, if you compare the density of his bones with those of Jordy (case # 1), you’ll see how fragile they appear.

The surgeons believed they could help him by straightening his femurs and tibias. The anesthesiologists cleared him in spite of a distortion of his chest cage which made it impossible for his lungs to work fully.

You can see how they achieved this on his right femur and tibia.

In spite of his severe deformity and a childhood of pain from fractured bones, this is a pretty easy kid to like.

Everyone involved hopes for a success with Jhon.

Insights:

“Zebra” in medical jargon, refers to a rare disease. “When you hear hoofbeats” – the saying refers to symptoms – “think of horses, not zebras.” Med Students are, like the general public, fascinated by rare conditions. But the Physician’s responsibility is to diagnose accurately. So the admonition is to think of and rule out the most common diseases first.

O I occurs in only one of every 20,000 births. There are eight different types of its manifestation, so some kids are only mildly affected. The mother of another kid in Cuenca told me that she knew of six other kids in the city with the disease.