by Dr. Gregory Bartha
November 2, 2021
Joel, age 10, came to the Cross Clinic in the spring of this year. He complained of weakness and shortness of breath. He was small for his age and had a loud heart murmur. His lips and fingernails had a bluish color. The ends of his fingers were clubbed, meaning they were enlarged with downward sloping of the nails, like an upside down spoon. Also, he was frequently found squatting when he was short of breath.
Tests were done on his lungs and heart. He was diagnosed with Tetralogy of Fallot, one of the most common types of congenital heart disease. It consists of a large defect in the muscle separating the right and left ventricles of the heart and obstruction of flow of blood to the lungs through the pulmonary artery. This results in the flow of deoxygenated blood in the right side of the heart to the left where blood is carrying oxygen. This mixing of oxygenated and deoxygenated blood gives rise to the bluish discoloration called cyanosis. The body does not get enough oxygen, and the person is weak and short of breath. Surgery is done to repair the defect between the right and left ventricles and to relieve obstruction to the flow of blood to the lungs. This surgery is very successful with over a 95% survival rate. The patients need to have medical followup. Sometimes additional surgery is needed to improve blood flow to the lungs. Persons should not be involved in contact sports or do heavy isometric exercise.
Some of you may remember Billy Pomeroy who was a fellow student at Sam Houston and Bowie elementary schools in the early 1950’s. I remember seeing him and noticing that he had a bluish color and that the ends of his fingers had an unusual shape. I think that I saw him squatting once in a while. (Squatting tends to reduce blood flow to the right side of the heart and to raise pressure on the left so there is less mixing.) It seems clear that Billy had Tetralogy of Fallot. At that time heart surgery was just beginning to be done on children. Billy’s parents took him to Boston, one of the few places where this surgery was being done. Unfortunately Billy died.
I remember how sad it was at the time. People put a black wreath on the door of his home. At the funeral his mother was crying uncontrollably when she came into the church.
Joel also faces an early death unless he has surgery. In Uganda the cost of the surgery is around $5000. I hope that the necessary funds can be raised.
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