Pulmonary Vascular Hypoplasia

Some bad news. After a 1+ hour echocardiogram today to try to figure out why Eli's oxygenation levels are so low, they diagnosed him with a rare condition known as pulmonary vascular hypoplasia. Basically, this means that his pulmonary (lung) arteries, where they branch out in their furthermost parts, are severely underdevloped. This is a congenital defect. His lungs themselves are full size, it is the vessels in them that are not properly developed. This condition is extremely rare. The lead neonatologist of the NICU at UK said that she has never seen this. She said that as far as she knows no one ever has. The pediatric surgeon agreed with her. Eli's underdeveloped pulmonary arteries explain why in spite of "every known intervention" Eli is still not oxygenating his blood properly. The relief of finding out what was wrong was soon overshadowed by the knowledge that there is essentially no knowledge about this condition - no treatment, no prior cases - nothing to go off of. Every question we asked was answered by "we don't know - no one does." I can only speculate on Eli's prognosis because "no one knows." This is of course hard to take. The current plan is for them to try to get the interventions and medicines at the best minimal balance and then "wait and see." The hope is of course that with more time, the distal parts of his pulmonary arteries will develop more and expand thereby decreasing the pressure in his lungs and raising oxygenation. There are no guarantees or statistics - only speculation and guesswork. On the bright side, despite this bad news, Eli has done surprisingly well today. His oxygen levels have been increasing all day. Last we heard they were 101 (the doctors would be happy with having them in the 40s or 50s since he has a lung problem). His oxygen levels have remained high even after an hour long echocardiogram, changing out all of his medications (which means he was off of his meds for a couple of minutes while they were changed), and putting in another pic line (an IV that leads directly to his heart). We as Eli's parents are hoping for the best. We do know (and I reemphasize know) that Eli is in the Lord's hands. God has blessed us with two wonderful, beautiful boys and we pray that he will let us raise Eli. We pray that he will be ok. We ask others to pray with us for we believe that there is power in numbers. We know that families are eternal.