293 - Can't be Fixed, but Still Alive: Planning a Rich Life for the Child with Life Threatening Heart Disease

Abstract

Introduction: Despite advances in pediatric cardiac care, some children will have limited treatment options and a shortened life expectancy. Treatment goals for these patients then become how to extend their lives where possible, and how to improve their quality of life by minimizing both physical and non-physical causes of suffering. To achieve these goals at CHLA, a combined Cardiology and Pediatric Palliative Care (PPC) clinic was conceived and implemented. This “Total Life Cardiac” (TLC) clinic meets monthly and is housed in the patients' cardiology medical home. Patients are identified and referred from within cardiology. Core team members include the primary cardiologist, PPC physician, social worker, psychologist, care coordinator, and nutritionist. Multiple clinic rooms are available so that patients can see various team members without moving between rooms, in a flow based on a pre-visit discussion. A detailed post-visit meeting generates a comprehensive care plan. Results: From July 2013 to April 2017 the TLC program served 40 patients, with 99 total patient visits. Patient age at referral ranged from 2 weeks to 24 years (median 10.9 yr). Diagnoses varied, with 23 (57%) congenital structural heart disease, 12 (30%) cardiomyopathy, 4 (10%) secondary pulmonary hypertension and 1 (2.5%) primary arrhythmia. Significant co-morbidity from other organ systems, including orthopedic, genetic, pulmonary, metabolic and renal, existed in 13 (33%). Total visits per patient ranged from 1–10 (mode = 1, median = 2). There was on average a 10% yearly attrition due to death, (total 10 in 4 years, 2 with primary cardiomyopathy, 8 with structural heart disease). Of the 14 (35%) patients that came only one time, 10 (20%) had their needs adequately addressed at that first consultation, and 4 (10%) died before their next visit. Of the 10 (25%) patients referred for cardiac transplant evaluation, 3 had successful transplants, 2 single ventricle patients died while awaiting transplant, and 5 were not eligible for transplant. A total of 5 patients (13%) were medically improved since their first TLC visit after a medical procedure or with medical treatment. Significant logistical barriers regarding cost, resources, and administrative buy-in were overcome, and over time we have clarified the program's mission and values, decreasing some of the common misconceptions surrounding palliative care in these patients. We discuss the principal benefits of the TLC program to the patients, to the divisions of cardiology and PPC, to the primary cardiologist, and to the medical system. Conclusion: Our model demonstrates the feasibility and some of the potential benefits of an integrated PPC and pediatric cardiology clinic for patients with life limiting illness, including those who may not be candidates for further palliation with heart transplant.