A Lot of Hope

In 1999 Margret got tired of being tired. She decided she wanted to get her heart repaired, at long last, but the pediatric cardiac surgeon suggested by our family doc said she was not a candidate for surgery.

Upset that he would not explain further, I hunted the internet for a doctor specializing in adults with unrepaired CHDs, and found one at Jefferson. We got an appointment and he told Margret that she had Eisenmenger's Syndrome. Finally, in January 2000, we had a diagnosis! He said the reason she was not a candidate for surgery to repair her heart is because changes in the lungs caused by the heart defect become permanent, and are not compatible with a repair. In other words, if she got her heart fixed, she would die. He encouraged us to consult with the tranplant doctors at Temple. It was a year of catastrophes, so we did not get with the transplant people until October. Her evaluation was scheduled for just after Thanksgiving. My Mom died Halloween morning, and we buried her with Dad on November 6th.

Margret had been put on oxygen in June, because I noticed her feet turned from their usual purplish color to a dirty white when she walked around. No one had thought to check her oxygen saturations before May, because she seemed to be doing well. I had no idea. Her saturations were in the low 80s, and 6 liters of oxygen only brought them up to the high 80s.

The transplant folks originally thought an 'en bloc' of heart and lungs would be a good idea. They finally settled on a plan of giving her new lungs, and borrowing a pediatric cardiac surgeon from the children's hospital next door to repair her own heart. Our insurance said no. Given a choice of fighting the insurance or going to a different hospital, we went.

The doctor there said there was medication that helped with the PH. I'd asked the transplant guys about medications, and they tossed it off with, "Oh, it's experimental, we only give it to our people at the very end to help them hold on so they can get new lungs." The new doctor explained there were THREE medications she found useful, and how she did a challenge with each medication while measuring the pressures on both sides of the heart through catheters to decide which medication was best, and what dosage would be most effective. I explained it again, in little words, to make sure Margret and I both understood. Then Margret decided she would try medication. It was a week in the hospital, the first day in the cath lab, that night in CICU, and the rest of the week in the cardiac stepdown unit while the doctor adjusted the doses of the other medications she would be taking with the main one.

When we went home, Margret trotted right up the stairs to her room, not stopping once! I had tears in my eyes, because she used to stop 3 times on the way up the stairs to rest, finally clinging to the rail at the top while deciding if she had to use the bathroom, or if she could just go plop on her bed.

A Little Logic

Margret had a few episodes of fainting, five that I know of, over a period of several years. Here's how I reasoned one day in the late 1980s that she had pulmonary hypertension:

Margret had just completed a riding lesson, and had done well. She was now standing, holding the horse's head, while her teacher picked out his feet.* Her lips became very blue, and she fell over. Fortunately she still had on her hard hat to protect her head. She was limp, her skin was bluish, and she didn't respond to my voice nor her teacher's nor to being shaken.

While this was going on, some analytical part of my mind said, "She was just riding, so there was a greater demand for blood in her body, and her capillaries are now dilated. That lessens resistance to blood flow out through the aorta. With the hole in her heart, lessened left side resistance encourages the blood to go that way, through the hole from right to left, and not off to her lungs to pick up needed oxygen. So. Cyanosis. And a faint. That means the resistance to blood flow in her lungs is high, needing higher pressures to push blood through, hence pulmonary hypertension."

She revived on her own after a very short time. She appeared quite normal after, but was excruciatingly embarrassed that she'd wet herself. We went home, mentioned the incident to the doctor, saw the cardiologist, and life went on.

When I explained my reasoning to her cardiologist, he nodded absently and moved on to something else. I wasn't sure if he blew me off, or if he simply didn't think this fainting business was important, but that's as far as it went. We did not get a proper diagnosis for years.

Wish I knew then what I know now. He would have got an earful, and we might have changed specialists. I would have done a number of things differently. But I didn't know. I don't think there were many people at that time, medical or lay, who knew.

Hindsight. Isn't it wonderful? and terrible? and generally useless in the now except to make you feel bad.

I did the best I could with the information I had. I just didn't have enough information to do the things that might have slowed her PH down. Back then, the phrase 'pulmonary hypertension' was just a description of something I had worked out with logic, not a diagnosis, not worthy of capital P and H.

*Students usually picked out the horse's feet on their own, but Margret had knee problems. The amount of difficulty she experienced, and fear of an accident, exempted her from doing feet, but she had to participate by holding the horse still.