Hospitalizations of Children with Huntington's Disease in the United States

Abstract

Background Juvenile Huntington's disease (JHD) is a childhood‐onset neurodegenerative disorder. Although it is caused by the same pathologic expansion of CGA repeats as adult‐onset Huntington's disease, JHD has distinct clinical features. Most clinical research in HD focuses in the adult‐onset disease; therefore, little is known about acute care outcomes for patients with JHD. Methods The Kids' Inpatient Database (KID) was used to examine hospitalizations of children with JHD and to determine the diagnoses and procedures associated with inpatient care for JHD. Regression models were built to examine acute care outcomes, including death, length of stay, and disposition at discharge in patients with JHD compared with patients in the general KID data. Results The proportion of JHD cases among hospitalized children was 1.23 per 100,000 KID inpatient stays. Seizures/convulsions (58.5%) and psychiatric conditions (26.1%) were the most common primary or secondary diagnoses among JHD patient hospitalizations. The most common procedure was percutaneous endoscopic gastrostomy tube placement (8.6%). Compared with hospitalizations of the general population, hospitalizations of patients with JHD had a lower odds of discharge to home (adjusted odds ratio [AOR], 0.23; 95% confidence interval [CI], 0.14–0.37) and an increased likelihood of death (AOR, 8.03; 95% CI, 2.98–21.60) or discharge to a short‐term care facility (AOR, 4.44; 95% CI, 2.59–7.61). A diagnosis of JHD was associated with increased length of stay (7.04 vs. 3.75 days; P < 0.01). Conclusions Children with JHD have unique acute care patterns. Future studies are needed to determine the extent to which coordinated care may impact inpatient and disposition needs.

Palliative care in advanced Huntington’s disease: a scoping review

“…Two case reports [ 37 , 52 ], one cross-sectional study [ 53 ], and a narrative review [ 24 ] discuss palliative care in pediatric HD patients (onset < 18 y/o) who have a different phenotype compared to adult HD patients, which is characterized by physical symptoms, including dysarthria, epilepsy, myoclonus, dystonia, spasticity, and ataxia, and psychological symptoms, such as early cognitive impairment, behavioral symptoms, hallucinations and delusions [ 24 , 37 , 52 ]. For family caregivers it is often difficult to find adequate respite care due to unfamiliarity with pediatric HD [ 37 ].…”

“…For family caregivers it is often difficult to find adequate respite care due to unfamiliarity with pediatric HD [ 37 ]. Reasons for referral of pediatric HD patients to hospitals are epilepsy, seizures and convulsions, followed by psychiatric diagnosis and respiratory infection/aspiration; the most common procedure was percutaneous endoscopic gastrostomy and endotracheal intubation [ 37 , 53 ]. A case study described the value of extensive EOL conversations on hospice care, and of hospice care in avoiding hospital readmission [ 52 ], as children with pediatric HD were 8 times more likely to die during their hospitalization than other hospitalized children [ 53 ].…”

Hospital Admissions of Huntington's Disease Patients in a Huntington's Disease Centre Between 2011 and 2016: A Retrospective Analysis

“…Another more recent study assessed reasons for hospital admissions in juvenile HD patients (here defined as people with HD aged ≤20 years) and found epilepsy, seizures, and convulsions to be most frequent (58.5%), followed by psychiatric symptoms (26.1%), and respiratory infections (15.9%) 5 . There were only two patients (3.8%) with pediatric HD (motor onset <18 years) 24,25 in our cohort (CAG repeats on the longer allele: 70 and 72).…”

“…Falls, weight loss, and aspiration pneumonia are well known consequences that may require attention of a specialist in an emergency setting 3 . However, only very few studies have systematically assessed hospital utilization among the HD population 4–6 …”

“…3 However, only very few studies have systematically assessed hospital utilization among the HD population. [4][5][6] In this retrospective analysis, we aimed to identify acute reasons for hospital admissions of adult HD patients treated at the Medical University of Innsbruck and its associated clinics as well as their baseline and clinical characteristics.…”