8/25 Update
After starting Patrick back on milrinone last week, he had a great week. His mood, energy, and temperament all improved. He spent a great deal of time in his Jumperoo, smiling at everyone who walked by. He also took trips around the unit in a wagon. With one person pulling the wagon, one in charge of the IV pole, one rolling the oxygen tank, and one person taking the pics, the 5 of us made a little Patrick parade. He waved and smiled at all the nurses and had a ball.
He was NPO starting at 2 a.m. today for his heart cath this morning, so he was understandably fussy and hungry when we saw him. Unfortunately, his PICC line had clotted and they had had to give him a peripheral IV in the foot so that they would have access for the cath. Then, they needed blood work and they couldn't get the IV to give them a good draw, so they had to stick him elsewhere for that. Needless to say, he was pretty unhappy by the time he was wheeled away for the cath around 11.
Originally, they anticipated him being under for about two hours. Patrick had other ideas. His heart rate kept dropping, so they had to give him multiple bolus doses of epinephrine. They also put him on nitric again. His pulmonary vasculature (a fancy name for all the lung arteries) is quite complicated. It's all very small and little branches shoot off in so many directions that placing any more stents in his distal PAs would effectively shut off flow to some areas. As a result, they ended up doing more balloon angioplasty to try to open as much up as they could. They also opened up the hole (fenestration) in his VSD patch to allow the right ventricle (RV) to shunt. The RV pressure is still systemic, causing the RV to remain severely dilated, resulting in poor function.
The PAH (pulmonary arterial hypertension) specialist, who also treats Mira, consulted with the doctor in the cath lab. They are contemplating putting Patrick on Flolan--a prostacyclin that is "the hammer" in terms of vasodilation drugs. Unfortunately, it's a very "fussy" med. It is continuous IV, so he would need a central venous port. It has a short half-life (3 minutes), and has to be mixed daily and refrigerated constantly--including kept on ice while being administered. However, in speaking with Dr. R, he wasn't certain it would work very well on Patrick because his vasculature is just small and the drugs simply can't do much to counteract their smallness. He was going to consult with some other people and see if they had any success with this type of treatment where the pressures were more size-related.
The cath ultimately took about 5 hours. When Patrick came back to his (new) room--he got moved to a single room again, thank goodness, as his new roommate this morning was coughing up a lung and didn't sound too great--he was still intubated, still on nitric, still on epinephrine, and pretty worn out. They were working to get his PICC line working again and putting him in restraints so that he doesn't hurt himself as the sedation wears off.
The plan is to present the results at the cath conference tomorrow to get everyone's eyes on him and talk about different options to come up with a plan. That will also allow his surgeon to take part in the discussion, since he wasn't there today.
Given what we learned about Patrick's small vasculature and the possibility that PAH meds may not work for him, we decided it was time to get a second-opinion about his condition and the possible need for a heart/lung transplant. We have made contact with a physician with whom we have a personal connection who works at one of the best places in the country for them and will speak with the team here today or tomorrow about signing the necessary consents and getting the information sent over to the other physician. We are still pleased with Patrick's care here and hope it doesn't become necessary for a transplant that will require us to be out of state, but we would rather prime the pump while things are relatively stable than have to rush the process.
Thanks for keeping us all in your hearts and prayers.
