Blyth’s daughter, Cameron, was diagnosed with TaySachs disease, a fatal, genetic condition that results in progressive disability, seizures and death, usually in early childhood. Blyth and her husband had no illusions about the prognosis, and in fact, were familiar with TaySachs because their nephew had been diagnosed with the same condition a year before Cameron. Blyth was able to work closely with Cameron’s pediatrician, who served as the primary care provider and coordinated her care. Together they made decisions about Cameron’s care including the most difficult ones related to when to stop actively treating her.
Shared decision making and palliative care from the beginning of the process were critical in helping Blyth and her husband cope with this illness, its progression, and Cameron’s eventual death. “We always knew we were focused on quality of life. I mean, honestly, with her diagnosis, there was no treatment and no cure, so it was only about quality of life. We had to decide what quality of life means to us, what we think it means to her, and does quality of life equate with length of life.
There were choices we made that were not just about comfort care, that were about duration of life. “The most important component (of the relationship with the doctor) was one of trust. Just absolute and total trust in him and his respect for us. We trusted him to guide us and listen to us. He understands disease and symptom management in a way we do not. At the same time, we were the ones who were in control of what defines quality of life for our daughter.
“Anticipatory guidance is really helpful. We knew all the bad things that were coming. We weren’t living in fear of the other shoe dropping because we knew that eventually seizures were going to happen. We knew that she would get pneumonia, that we would have to decide about putting a feeding tube in. It’s unpleasant to wait for the seizures to start, but at least you know what to expect and I think that was very helpful. “Through my creation of Courageous Parents Network (www.courageousparentsnetwork.org) I have become an advocate for parents of seriously ill children. I tell them they want to have a doctor or care team that they trust, that listens to them and communicates honestly. I tell them they want to find a community of others who can help them feel less alone, which is typically other parents. And, I recommend that early in their child’s illness, they get a good palliative care team in place to provide an extra layer of support as a lifeline to quality of life.”