Albumen low, PLE possible

Riley had some blood work done yesterday. We had hoped to eliminate some of our fears, but for now, we have more questions. Dr. Tarnoff called this morning to let me know that Riley's electrolytes are within the normal range. However, his albumen levels are slightly lower than normal. Albumen is the protein in the blood. Low albumen levels can be a sign of a serious condition called Protein-Losing Enteropathy or PLE.

Dr. Tarnoff said it is unusual to have slightly-lower levels of albumen in the blood. It is not clear if lower levels mean that he is just started to develop the disorder or if it is something else. So more testing is needed. First, Riley will have a stool sample. If they find protein in his stool, he is losing protein from his blood, and that would confirm PLE. He will also have an abdominal ultrasound to look for pockets of fluid in his abdomen and also at his liver and the hepatic veins that were moved during Riley's Fontan. A cardiac MRI is still in the plan so that they can look for obstructions in the Fontan circulation.

If this is PLE, Dr. Tarnoff said that it is a very rare form of it and there is no established treatment. We have tried diet (with the two rounds of non-fat diets). We have tried lots of diuretics, which have not worked. Other options include a round of steroids. Intravenous globulins are also an option, which might make his chemistry better for a short amount of time. Heparin injections might also be a possibility. "Everything is a two-edged sword," Tarnoff said.

Mostly, it seems that we're heading back to surgery (in case you lost count, that would be his 4th heart surgery). If the stool sample and abdominal ultrasound confirm PLE, it seemed that Fenestration would be the viable option. Recall that Fenestration was a hot topic when Riley's heart was in severe distress following his Fontan. Fenestration would create a hole between his inferior vena cava and his atrium. If he got better as a result of the lower pressures, they could then close the Fenestration in the cath lab several months or years later. The downside, of course, is more heart surgery. In addition, Riley would end up with lower oxygen saturation levels. As a result, he would likely be bluer in color and have less energy.

Clearly, this is not the road we want to head down. It is frustrating that his albumen levels were not tested in September when Riley was in the hospital for a week and the pockets of fluid under his lungs were discovered. We have also been asking about PLE and his puffy hands and feet. Why haven't we looked for pockets of fluid elsewhere before now? Why haven't we looked at his liver to see if it's enlarged before now?