Cath Results

Results today confirmed most of what the physicians expected, she does have quite elevated pulmonary pressures. Blood flows down a pressure gradient, from high pressures to low pressures. The blood in her pulmonary artery (blood flowing to the lungs) was at pressures in the 80's while her systemic pressure (blood flowing to the rest of the body) was in the 60's. So naturally blood shunts, or bypasses, the lungs. Her pulmonary vascular resistance (PVR), a measure of how clamped down her pulmonary arteries are, and "resisting" flow of blood, was also high. Normally, babies run around 3 or 4, and her PVR is in the mid teens. This makes it very difficult for blood to flow through the lungs. They tested her response to the inhaled nitric oxide and her pulmonary pressure dropped by about half. That's great if she could be easily managed or if it were safe to use inhaled nitric long term. The NO also predicts her response to Viagra, but she is already at a near max dose of that. She did not respond to the NO in a manner that would lead the cardiology team to believe that she would respond to calcium-channel blockers, another medicine that has been used in pulmonary hypertension. The cardiology team will now meet to form a plan for exactly what and how much to add for medicines. This will likely include two other medicines that work outside that chemical pathways addressed by the Viagra. One will be bosentan (Tracleer) a endothelin antagonist, that is an orally-available medication. The second will be Flolan (epoprostenol), a medicine that she has been on before in the inhaled form. This time around she will be on the IV form, as the inhaled form is continuous and not practical for long-term usage. Unfortunately the IV form is not as specific for the pulmonary vasculature as the inhaled form. Whatever medicines they add to the sildenafil, there is not likely to be a vast improvement in her condition. They cardiologist noted that there are some chronic changes already occurring in her pulmonary vasculature as a result of her disease. If her pulmonary hypertension does not improve well, she may become a lung transplant candidate. The good side of that is that St. Louis Children's is number one in the nation for the amount of transplants they perform. The bad side of that is, well, lots. That is still down the road quite a bit. I'm not much of an optimist these days, and plainly stated, we are in a really shitty position with Elise. It makes me even more comfortable about my leave of absence from KCUMB. Elise may possibly be extubated later today, she is very comfortable with sedation and pain meds right now. Feeds will likely begin later as well. I don't know what else to say to make this sound any better, this is just a very difficult time for us. Love you all,

Jason