A second opinion

We had the privilege of talking with Dr. Desai today at the Lucile Packard Children's Hospital annual Valentine's Day party. This is not Riley's regular cardiologist, but he knows a bit about Riley's case because he talked with us extensively while Riley was on ECMO at UCSF following his Fontan surgery last March.

We gave him a brief update on Riley's health over the past year after being discharged from UCSF: The ups and downs with diuretics, the week-long hospitalization in September that revealed fluid under Riley's lungs, the disappointment that the fluid was actually accumulating instead of disappearing when we had a follow-up in January. We also talked about the current plan that includes a cardiac MRI, probably a heart catheterization, and numerous blood tests.

Dr. Desai's reaction affirms some of our fears. His said that there is no reason a Fontan patient should still be draining a year after surgery unless there is a problem. He estimated that diuretics will not fix the problem. Rather, the increase in diuretics is likely just a band-aid covering up a bigger problem. He gave three possible reasons for the continued fluid accumulation: 1) some kind of blockage within the Fontan system, 2) a serious condition called protein-losing enteropathy or PLE, 3) some kind of lymphatic problem. Because Riley is not his patient, he said many times during our conversation that he cannot tell without all the information what is the true cause of the continued fluid accumulation. He also said that Riley looked great, and as Dr. Tarnoff often says, they way a patient looks is sometimes more important than any specific test result.

This doctor said that some questions need to be answered soon. For example, what is Riley's albumen level? Albumen is the protein in the blood. If the albumen level is low, that would be an indicator that Riley is developing PLE. This can be answered with a blood test. Riley is scheduled for a series of blood tests before his next appointment in March.

Dr. Desai also wondered about Riley's Fontan pressures--the pressures in the different chambers of the heart and lungs. This could be determined with a heart catheterization. If the pressures are not balanced properly, then that could be an indication that the Fontan is not working properly.

Fixes for both of those problems could happen by re-doing the Fontan.

The third possibility is that there could still be damage in his lymphatic system. That would mean that the fluid under his lung is lymph fluid. However, Dr. Tarnoff felt it was not lymph fluid, although Ken and I cannot remember his explanation. Still, if it was lymph fluid, Dr. Desai said that there could be some surgical repair for the lymph system, if and only if the source could be identified, which is very difficult, he said.

Still, we will hold out hope that if there is some blockage, that it can be repaired in the cath lab and NOT the OR.