When is Palliative Care Right for Families of Children With Single Ventricle Congenital Heart Defects?

Single ventricle congenital heart defects (SV) disrupt critical blood flow and require a series of complex operations in order for a child to survive. As few as 80% of children born with SV live to age 1, 70% to age 20.

Because of the high risk of death and the chronic and complex nature of SV, involving a palliative care team only after a serious complication may be too late to help families making hard decisions, Nationwide Children’s Hospital practitioners suggest.

Instead, engaging a palliative care team with families immediately after diagnosis should be the standard of care, the cardiac and palliative care practitioners conclude in a study in Pediatric Cardiology.

These providers helped change the standard at Nationwide Children’s through a quality improvement project. Now the palliative care team, called the Advanced Illness Management (AIM) team, begins regularly meeting with every family of a child with SV starting when the child is admitted for the first palliative surgery. The AIM team supports families in being advocates for their child and in making decisions for their child’s health care through at least the second stage of surgery.

Earlier involvement has led to increased family support and decreased the average time to the first palliative consultation from months to 3 days and the average age of the child at consult from 100 to 3 days. It has also improved communication among the cardiac and AIM teams, the practitioners found.

“Palliative care is now integrated with the medical team early on, helping families understand the diagnosis and the chronicity of SV,” says Karen Texter, MD, a cardiologist and medical director of the Single Ventricle Program in The Heart Center at Nationwide Children’s and study co-author.

The early involvement builds trust the AIM team needs to support families when they must make significant medical decisions about complications or end of life, says Dr. Texter, who is also a clinical assistant professor of Pediatrics at The Ohio State University College of Medicine.

The providers began examining their practice largely due to a single case, says Jo Ann Davis, CPNP-AC, a nurse practitioner in the Single Ventricle Program and study co-author. Due to a fast decline in a child’s condition, by the time palliative care was introduced, “it was really too late to provide the family the level of support they needed.”

A review of SV medical records from the two years prior showed the AIM team was involved with about one-third of the families whose child passed away, Davis says. “That was pivotal. We knew we wanted to try and provide better support.”

Alice Bass, CPNP-PC, an AIM team member and study co-author, says meeting with every family of a child born with the diagnosis can take time, but building that relationship is invaluable.

“We meet with these families early on to introduce palliative care, follow along in the family’s journey, learn their values, and build rapport,” Bass says. “We find this incredibly valuable, to both parents and to us, in the event that their child’s condition worsens and we need to provide support to families regarding goals of care.”

The practitioners believe their approach with SV patients can be applied to other chronic illnesses. They are currently investigating how to measure the effects of the new standard of care on families for a further study, and whether the AIM team should become involved even earlier, when a child is diagnosed prenatally.

Reference:

Davis JAM, Bass A, Humphey L, Texter K, Garee A. Early integration of palliative care in families with single ventricle congenital heart defects: a quality improvement project to enhance family support. Pediatric Cardiology. 2019 Nov 1. [Epub ahead of print]