Case Report: Treatment of a Medically Complex Adult Patient with Down's Syndrome in a Children's Hospital

Background: Adults with intellectual disabilities admitted to children’s hospitals for medical care face unique vulnerabilities. Modifications to treatment algorithms, delirium interventions, and approaches to decision-making capacity may be necessary to ensure effective care of adults in pediatric settings. These patients may benefit from a case-review approach from hybrid teams comprised of both pediatric and adult consultation liaison psychiatrists (CLP). We present an illustrative case of an adult patient with a six-month hospitalization in a tertiary academic children’s hospital complicated by delirium secondary to methotrexate toxicity, episodic catatonia, depression, and neurocognitive problems who derived benefit from a lifespan-based treatment approach.

Case: A 35-year-old male with Down syndrome, Hirschsprung's disease, and OSA was admitted to a children’s hospital for treatment of B-cell Acute Lymphoblastic Leukemia with intrathecal methotrexate. Psychiatry was consulted for acute onset of episodic disorganized behaviors, irritability/agitation, and disorientation. Differential diagnosis included delirium from methotrexate toxicity, early onset dementia/Alzheimer’s Disease associated with Down Syndrome, and Down Syndrome Disintegrative Disorder.

Antipsychotics were initially helpful, but subsequently precipitated catatonia. Treatment with lorazepam improved catatonia1, but higher doses worsened delirium. Depakote was added for agitation with some improvement. In consultation with CLP at the affiliated adult medical center, decision was made to add memantine due to benefit being shown in methotrexate toxicity, catatonia, and dementia2. Patient stabilized and tolerated discontinuation of Depakote and lorazepam. Throughout hospitalization patient had episodic delirium due to various etiologies including metabolic derangements, infections, and other issues. He also had episodic periods of catatonia due to metoclopramide use and lorazepam was reinitiated. As length of stay progressed beyond 3 months, patient developed depressive symptoms potentially further exacerbating catatonia. Low-dose escitalopram was added with improvement. Throughout hospitalization, communication with adult CLP colleagues was maintained and frequent care conferences were held with patient’s family and representatives of full team at pediatric center. Patient maintained his own guardianship during admission despite concern for dementia and delirium. At time of discharge, patient was doing well on memantine, escitalopram and lorazepam.

Discussion:

Patient’s diagnosis with B-cell ALL at age 35 raised the question regarding if treatment would be better suited in an adult hospital by oncologists unfamiliar with ALL vs. in a pediatric center with psychiatrists, neurologists, and other staff unfamiliar with assessing/managing adult patients with Down syndrome and/or possible dementia. More globally this case raised questions about adult patients with intellectual and/or developmental disabilities who are their own guardians.

Conclusion/Implications: Patients with developmental disabilities have unique treatment requirements that cannot always be met entirely within a single, even highly-resourced, pediatric or adult medical system. Development and maintenance of collaboration partners across the lifespan can improve patient care and create educational opportunities for trainees and staff psychiatrists in both pediatric and adult hospitals.

References: 1. Miles JH, et al. Catatonia in Down syndrome: systematic approach to diagnosis, treatment and outcome... Neuropsychiatr Dis Treat. 2019 Sep 20; 15:2723-2741

2. Shodeinde AC, et al Methotrexate encephalopathy: Two cases in adult cancer patients, who recovered with pathophysiologically based therapy…..SAGE Journals, 2017 Jan