I am thankful, very thankful, to have had as many years with my daughter present in my life as I did.
In spite of the pediatrician at the hospital where she was born, who told me she would die by age two, or if she reached her second birthday she would die before age ten, she lived to be an adult.
Her health declined and she wanted to have her heart repaired. The surgeon, after his office played phone tag with me all summer in 1999, left a message stating simply that she was not a candidate for surgery. His office and I did the phone tag thing because I was back and forth to Florida to deal with my mother's affairs, and especially her house, which needed to be cleared out so it could be sold. I spent a total of eight weeks there, in one and two week blocks. After my return, I could not elicit a reason from his office staff as to why she was not a candidate. We did find out the reason when, in January, we saw the doctor who gave her her diagnosis of Eisenmenger's syndrome. He explained that for many years surgeons would repair the heart of an older child, only to have the child die after successful surgery. As soon as they figured out the surgery was causing these deaths, they stopped. After that, only children young enough not to have the permanent changes in their lungs caused by the heart defects were considered for repair. He's also the one who said she should see the tranplant people, for completeness sake.
Margret was prescribed supplementary oxygen in June of 2000. At that time her 'normal' saturations were in the 80s. The low saturations went a long way toward explaining why she had difficulty following directions, and considering whether she should act on an impulse or not. She lost one of her volunteer postings earlier the same year for not following directions. I am sure it would have helped if she had been given oxygen earlier, but who knew? I certainly didn't. She managed well enough with the day to day minutiae of her life that no red flags went up for me or for her doctors. There were no obvious symptoms as her saturations dropped. A cardiologist opined that the drop was slow enough for her to learn to compensate, so he, and others, did not pick up on it. She lost another volunteer posting after starting on the oxygen. The director did not want a volunteer on oxygen to be in, or anywhere near, their kitchen.
With the other things happening that year, it was October when she first saw the transplant doctor to see if she ought to be considered. He thought she might be eligible for the transplant program, so he scheduled her to see the people at TUH for an evaluation the week after Thanksgiving. While she was off having a test where they didn't need Mom trailing along, one of the doctors stopped by her room looking for her. He found me instead. Realizing how uncomfortable I was asking about her death with her in the room, I took that moment, "What do you think of her life expectancy without a transplant?"
He thought about it for a very long moment and replied, "At her current rate of decline, I give her two years, maybe a little less."
She didn't have the lung transplant. Insurance didn't want to pay for a procedure done so seldom, borrow a pediatric cardiac surgeon from the children's hospital next door (had they ever done that before? I don't think so.) to repair her heart, and give her new lungs.
I can almost hear some of you wondering, "Why a pediatric cardiac surgeon?"
Answer: Unless you are a pediatric cardiac surgeon, you have never had to repair the sort of heart defect Margret was born with. Most folks with that type of defect have either had it repaired as an infant, or died before reaching adulthood, so a typical cardiac surgeon (for adults) has never done this repair.
Along with that, when Margret had an echocardiogram done, the techs at the adult hospital didn't know how to interpret what they were seeing, so the docs sent her next door to the children's hospital to have one done there. The chief of pediatric cardiology himself did her echocardiogram. He also offered us an apology, on behalf of his entire profession, that surgeons around the time of Margret's birth did not offer to repair a heart defect if the heart in question belonged to a child with Down syndrome.
What I wanted for her was the best quality of life with the least invasive treatments, so I was not at all unhappy when the doctor at the next hospital said, "She doesn't need a transplant. We have drugs to manage pulmonary hypertension."
The medications she started on there made her quality of life much better. See also A Lot of Hope The main medication was nifedipine, a calcium channel blocker, which relaxes smooth muscle so it widens arteries and veins. For Margret especially, with her pulmonary hypertension, it allowed more blood to flow through her lungs.
That specialist also believed in treating for CHF (congestive heart failure) before it became obvious. She said her patients were more comfortable, for longer, with treatment. Margret said, "Comfortable is good!" She treated Margret with a diuretic (furosemide) to prevent the fluid build up that is CHF.
Her low oxygen saturations encouraged her body to make extra red cells for oxygen transport. If you move to a higher altitude, your body will do something similar to compensate for the lower partial pressure of oxygen there. It's called the High Altitude Adaptation, and it's what allows people to live anywhere from sea level to high in the Andes. (and other mountain ranges, of course) The reaction by Margret's body was extreme, so her platelets were vastly outnumbered, as is true of many people with PH. On the other hand, the edges of the holes in her heart are rough, and encourage platelets to stick, and start the clotting process. So she is at risk both for random clots and uncontrollable bleeding. The clotting being more dangerous, the doctor prescribed a blood thinner.
This specialist was also hopeful about life expectancy. She told Margret that as long as she responded well to her medications, and nothing unforseen happened, she could live a long time. How long? we asked. Well into her forties, she said.
Some years later, when she was again doing less well, another specialist put her on Revatio to help control the PH. The difference it made was remarkable. From being quiet, she returned to chatty self, and from an "Anything will do," attitude, she recovered her ability to be choosy, and sometimes even to argue with me. You would not believe how happy that first argument made me. We argued about whether we would have ice cream for dessert. Yes. She won.
All these medications, and others, improved her quality of life, and increased the time we had together.
I knew she was likely to die before me, but I wanted her to grow old and let me die first. No parent should have to cope with the death of their child. There is a part of me (selfish, selfish!) that still insists we should have had more years together, more birthdays, more holidays, more road trips. More everything, more time.
We did the best we could, she and I. I sometimes wonder if my best was good enough, but her best was absolutely perfect. She enjoyed her family, and they enjoyed her. She enjoyed being around people and chatting with them, and her volunteering allowed her to give of herself. Giving was important to her, and making the world a better place. She did that.