Polysymptomatology and Polypharmacy at End of Life in Patients with Duchenne Muscular Dystrophy.

OBJECTIVE: To characterize symptom frequency and symptom-directed treatment approaches in patients who died with advanced Duchenne muscular dystrophy (DMD). STUDY DESIGN: This was a retrospective cohort study of patients in a multidisciplinary DMD program who died between January 1, 2013, and June 30, 2021. Inclusion criteria were patients who died with advanced DMD in the time period studied; exclusion criteria were low exposure to palliative care (<2 encounters). Demographic, symptom, and end-of-life data, as well as medications used for symptom management, were abstracted from the electronic medical record. RESULTS: In total, 15 patients were eligible for analysis. The median age of death was 23 years (range 15-30 years). One (6.7%) experienced a full code at death, 8 (53.3%) had do-not-resuscitate orders, and 4 (26.7%) had limited do-not-resuscitate orders. Mean palliative care exposure was 1280 days. All 15 (100%) had pain and dyspnea; 14 (93.3%) anorexia, constipation, and sleep difficulty; 13 (86.7%) wounds; and 12 (80%) anxiety and nausea/vomiting. Multiple medications and drug classes were used to target symptoms. CONCLUSIONS: We found significant polysymptomatology and polypharmacy in patients who died with advanced DMD. Clinicians who care for patients with advanced DMD should clarify goals of care and document advance care planning. Given the complexity of multisystem disease progression, palliative care should provide subspecialty pain management and assist with psychosocial burdens.

Fetal outcomes and continuity in perinatal palliative care patients at a quaternary care pediatric hospital.

OBJECTIVE: Perinatal palliative care (PPC) is the coordinated application of palliative care principles to the care of families, fetuses and newborns with suspected life-limiting conditions. This approach relies on continuity of care that spans pregnancy, birth and beyond. The goal of this retrospective cohort study was to evaluate outcomes and PPC continuity in infants born to families who received PPC at a quaternary care pediatric hospital, and to identify targets to improve care continuity. STUDY DESIGN: PPC patients seen between July 2018 and June 2021 were identified via local PPC registry. Demographic, outcome, and continuity data were gathered from the electronic medical record. Descriptive statistics were used to calculate the rate of postnatal palliative consult and infant mortality rates. RESULTS: 181 mother-infant dyads were identified as having a PPC consultation and had available data following birth. Overall perinatal mortality was 65%; 59.6% of all liveborn infants died prior to discharge. Only 47.6 % of liveborn infants, who did not die in the perinatal period, received postnatal palliative care. Location of birth (primary versus non-network hospital) was significantly associated with postnatal PPC consult rate (p = 0.007). CONCLUSION: Continuation of palliative care after birth in families who received perinatal palliative care is inconsistently achieved. Creating reliable systems for PPC continuity will depend on location of care.

Burnout in pediatric hematology/oncology-time to address the elephant by name.

The last decade has brought increasing recognition that the wellness of health care providers has an impact on the quality of care, patient satisfaction, and health care economics. This review will describe models of burnout, discuss the impact of burnout on medicine with a focus on pediatric hematologists/oncologists, and present interventions that may help ameliorate physician burnout.