Caregivers Debate Treatment Options for Dying Down’s Syndrome Patient

ethics-debate-3The prognosis for 8-year-old Angela Downing is bleak. Born with trisomy 21, her severe heart disease has run the gamut with an atrioventricular canal defect, moderate mitral insufficiency, and a failing left ventricle. Seven days after her valve replacement, she failed to wean from extracorporeal membrane oxygenation (ECMO) due to her poor left ventricular function. She underwent re-exploration for bleeding, difficulty gaining adequate heparinization, and thromboemboli in several fingers and toes.

Her situation is tenuous, and her parents want “everything done.” Her surgeon must meet with the parents about whether to embark on a ventricular assist device (VAD)-transplant pathway or remove the child from life-sustaining therapy.

Minoo N. Kavarana, MD

Minoo N. Kavarana, MD and Jessica M. Turnbull, MD will take opposing views during the Ethics Debate: When a Child’s Heart Is Failing.

Dr. Turnbull, an ethicist and pediatric intensivist at Vanderbilt University in Nashville, will advocate for withdrawing ECMO, and Dr. Kavarana, a pediatric cardiac surgeon at the Medical University of South Carolina in Charleston, will argue to replace ECMO with a VAD as a bridge to heart transplantation.

“This is a horribly unfortunate situation,” Dr. Turnbull said. “Complicating the whole matter is that there is still equipoise in the transplant community about whether transplanting kids with chromosomal abnormalities is the right thing to do, given their limited life expectancies. They will be dependent on caregivers for probably the entirety of their lives.”

Dr. Turnbull speculated that the child would be left with a poor quality of life post-transplant.

Jessica M. Turnbull, MD

“We sometimes don’t have time to consider when a kid’s not doing well, so we hedge our bets, and we put them on ECMO,” Dr. Turnbull said. “We try to do the right things for these kids, but that leads to a lot of harm without the potential of appreciable benefits. Instead of going to a VAD and a transplant, her course should be transitioning to one of comfort and then likely withdrawal of life-sustaining therapy.”

Dr. Kavarana noted that children with Down’s syndrome live to be 50 to 60 years old and often have good support from family at home.

“You have to decide if you withhold the same therapy that you would offer a non-Down’s syndrome 8-year-old,” said Dr. Kavarana, noting that the child’s size merits the use of an implantable intracorporeal VAD. “Until we determine futility, we would not withhold a heart transplant. We clearly have not demonstrated futility. She’s just 7 days from her surgery on ECMO, which would be a perfect time for a VAD transition.”

However, it is difficult for Angela to be compliant. She often removes the CPAP mask she was prescribed at age 4 to ameliorate her obstructive sleep apnea.

“I know her caregivers are doing the best they can, but when we’re talking about a VAD and a heart transplant, we’re asking our families to take on increasingly complicated levels of care at home,” Dr. Turnbull said. “I think it does a disservice to our kids and our families when we offer therapies that are arduous to carry out at home and then will not yield the best outcome possible. It’s a heart-breaking situation to have a patient like this.

“Despite the fact that she has lovely, amazing parents who love her very much, and despite the best efforts of the medical team, we’ve unfortunately run out of options. I think withdrawing life-sustaining therapy isn’t killing. I think it’s transitioning to comfort. Her path was set when she had a mitral valve that couldn’t sustain her for as long as we needed it to sustain her. Unfortunately, that’s the process that’s killing her, not our withdrawal of life-sustaining therapy.”

For Dr. Kavarana, doing nothing would be wrong.

“I think you can improve this child’s heart failure symptoms and ease her suffering. It would be withholding care for a child who potentially could be resuscitated or weaned off ECMO,” Dr. Kavarana said. “Clearly, there is an ethical dilemma when it comes to solid organ transplantation and patients with disabilities. There is no doubt about that.”

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