P3.I176: Role of Palliative Care in Pediatric Patients with Leukodystrophy

Background: Children with leukodystrophy often experience progressive physical and cognitive decline, which can cause considerable suffering. Literature on the role of palliative care for children with severe neurological impairment emphasizes that palliative care helps with multimodal symptom management, building trust with medical teams, and managing advanced care planning and care coordination. Currently, there is limited research specifically evaluating the involvement and the impact of palliative care within the pediatric leukodystrophy population.

Methods: A single-center retrospective chart review was conducted on pediatric patients (0-18 years) with leukodystrophy, followed at the leukodystrophy clinic at Children’s Healthcare of Atlanta (CHOA) from 2016-2023.

Results: There were 206 patient charts that were reviewed and included in this study. All patients had a leukodystrophy diagnosis, based on neuroimaging, genetic studies, and clinical features of disease.

Of all patients with leukodystrophy, 26% (53/206) had a palliative care consultation placed during their disease course. Palliative care referrals were placed during an inpatient admission and were only completed in an inpatient setting. There was an average interval of 2.4 years from date of diagnosis to initial palliative care consult. The hospitalist team (38%, 20/53) and ICU team (27%, 14/53) placed most referrals, and disease-related/progression of disease (96%) was the most common referral reason. 100% of palliative consults listed goals of care/advanced care planning as a referral reason, and 42% listed symptom management in their reason for referral.

Of the whole cohort, 20% (41/206) of the patients had died. Of these, 66% (28/41) had a palliative care consult. The average length of time from palliative consult to death was 2 years, and the average age of death was 5.9 years. More than half, 54% (22/41) of the deceased cohort was enrolled in hospice. 27% (11/41) of deceased children had full code status, 39% (16/41) were DNR/DNI, and 27% (11/41) were DNR. For place of death, 49% (20/41) of children died in a hospital and 37% (15/41) died at home/hospice house.

Conclusion: Palliative care medicine can be another layer of support for children with leukodystrophy. Pediatric patients and caregivers can benefit from goals of care conversations and advanced care planning while navigating the neurodegenerative disease process. Concurrently, the medical team also benefits from palliative care involvement in a clinical setting, as seen with care coordination, symptom assistance, hospice enrollment, and discharge planning. There are unique psychosocial and medical needs in this patient population, and there are many opportunities to improve neuro-palliative integration early in the disease course for children with leukodystrophy.