COTW: Hematocrit 87

(From Alyssa)
After Faders and I worked for a week at Kibuye Hospital in Burundi last May, I reported a "Case of the Week" with two ladies with severe anemia - hemoglobin 1.1 and 1.7 (which translates to hematocrits of 3-5). Today our case of the week is a 7 year old boy I saw in Cuzco, Peru on Thursday with the opposite scenario - a hematocrit of 87 (normal for his age is 40).

Jorge lives at 4000 meters (13,100 feet) in a small village four hours from Cuzco. He's the seventh child in the family and was born at home. His family noticed from a young age that he seemed blue, but most of the children in their area end up with darkened cheeks from the altitude, so they didn't think much of it. They live very far from any clinic and Jorge only received one set of immunizations as an infant. At age five, they noticed Jorge was unable to walk to school because he became very short of breath, so he stayed home. Given their poverty and distance from any medical care, they still did not bring Jorge to the doctor. But his cyanosis and shortness of breath gradually worsened.

For two weeks prior to coming to the hospital, Jorge began spiking high fevers and complaining of headaches. He was treated with herbal medications in his village. Then he had a partial seizure. On evaluation in the emergency room, he was noted to be extremely blue but with almost red conjunctiva. He was breathing very fast and his heart rate was high. His oxygen saturation was 68% (normal is above 92%). His liver and spleen were enlarged and his fingernails were cyanotic and severely clubbed (from chronic low oxygen levels). His blood count (hematocrit) was 87 (more than twice the normal value) and the reason was explained by an echocardiogram (ultrasound of the heart). Jorge was diagnosed with Tetrology of Fallot - a congenital heart condition that is relatively easy to repair in a newborn, but in Jorge's case at age 7, his blood is now shunting the wrong direction and the pressure in his lungs as a result is too high for heart surgery to be an option. And really he has the worst case scenario, because not only is his oxygen level low because of his heart condition, but also because of the very high altitude where he lives above Cuzco. That's why his hematocrit is so high - his body is trying to increase his oxygen carrying capacity in any way possible which means making more blood to carry more oxygen.

And the cause of his fever and seizure?

Several large brain abscesses - a known complication of untreated cyanotic congenital heart disease. Jorge is now on IV antibiotics to treat the infection in his brain, and he has not had any more seizures. Because of the severity of his heart condition, though, he likely will not live long. I wish I could have spoken with him, but he only spoke Quechua (not Spanish). He was impressively cooperative with many doctors examining him - if only he had been examined long before age 7 and had been born in a less remote place with more resources and medical care, his outcome likely would have been very different. Pray for peace for him and his family.