Healthcare utilization, complexity higher in PWS hospitalizations
Claims database analysis finds longer stays, more deaths in PWS patients
Hospitalizations among people with Prader-Willi syndrome (PWS) are characterized by higher healthcare utilization and complexity compared with non-PWS patients, according to a large-scale analysis of a U.S.-based administrative claims database.
In addition to high rates of obesity, PWS patients had longer hospital stays, higher associated costs, and more in-hospital deaths.
Among the youngest PWS children, the disease itself and respiratory failure drove most hospitalizations. Scoliosis, a sideways curvature of the spine, was the most common reason patients ages 5-18 were hospitalized. In adult PWS patients, hospitalization was mainly due to septicemia, a life-threatening infection of the bloodstream, with respiratory failure particularly prevalent among young adults.
“These insights highlight the potential value of tailored strategies to improve the management and outcomes of PWS hospitalisations,” the researchers wrote.
The study, “Inpatient hospitalisations for patients with Prader–Willi syndrome: a 2019–2021 National Inpatient Sample analysis,” was published in the Journal of Intellectual Disability Research.
Claims data provide broad sample
PWS is a rare genetic disorder marked initially by symptoms such as low muscle tone (hypotonia), feeding difficulties, and developmental delays. At about age 4, patients develop hyperphagia, an overwhelming and constant hunger, which increases the risk of obesity.
PWS symptoms may also include cognitive disabilities, anxiety, compulsive behaviors, and respiratory problems, along with respiratory infections.
“Large-scale longitudinal studies have helped characterise the clinical presentation of PWS across the lifespan, but many such studies rely on active participation of patients and their families, meaning that findings may not generalise to the entire population of individuals with PWS,” the researchers wrote.
Administrative claims data, derived from medical records gathered during routine clinical care, provide an alternative data source not tied to active participation. These datasets are large, amassed from multiple nationwide centers, and up to date.
Researchers at the Massachusetts General Hospital, in collaboration with the Foundation for Prader-Willi Research, examined data from the National Inpatient Sample (NIS), the largest publicly available, all-payer, inpatient care database in the U.S. It contains detailed inpatient information on demographics, clinical presentation, inpatient utilization, costs, other medical diagnoses, and outcomes.
According to NIS records, from October 2019 to December 2021, there were 4,400 hospitalizations for people with the PWS diagnostic code. Patients’ median age was 24, and 53.5% were male.
Researchers matched each PWS hospitalization to five non-PWS hospitalizations (controls) based on the person’s age and sex, as well as year of hospitalization and hospital characteristics.
PWS hospitalizations showed a greater proportion of white patients, higher use of Medicare, lower use of commercial insurance, and more patients living in high-income ZIP codes than non-PWS hospitalizations.
“It is unclear whether prevalence rates are identical in all races or whether the racial differences observed here instead reflect failure to diagnose or code for PWS among non-White patients,” the researchers wrote.
PWS patients spent more time in the hospital than non-PWS patients (median 5 days vs. 3 days), with higher associated hospital costs (median $46,463 vs. $31,031). PWS patients were also twice as likely to be discharged to other facilities (17.8% vs. 8%) and to die (2.2% vs. 1.3%).
Infections, respiratory problems drive PWS hospital stays
The primary reasons for PWS hospitalizations were infections (19%) and respiratory problems (16.2%). PWS hospitalizations were more than twice as likely to be related to septicemia than non-PWS hospitalizations. Among in-hospital deaths, septicemia was the cause of death in a higher proportion of PWS patients (47.4% vs. 32.1%).
Mental health conditions, including schizophrenia and depressive disorders, were less frequent in PWS patients than controls, but the two groups had similar rates of bipolar disorder.
Among the youngest patients, ages 0-4, the most common reason for PWS hospitalization was PWS itself, followed by respiratory failure, bronchitis, and asthma, all at rates higher than for control hospitalizations. Scoliosis was the most common reason for hospitalizations in PWS patients aged 5-18, and at a rate higher than for those without PWS.
For older PWS patients, septicemia was the most common discharge diagnosis, which occurred three times more often for PWS individuals ages 19-30 than for controls. Respiratory failure was also more common in PWS patients across all age ranges, particularly among 31- to 45-year-olds, who were 13 times more likely to experience respiratory failure than non-PWS patients (7.1% vs. 0.5%).
More than twice as many hospitalized PWS patients were overweight or obese compared with hospitalized controls (38.1% vs. 15%). Among hospitalizations for PWS patients older than 4, nearly half (45.6%) had overweight/obesity codes, and Hispanic ethnicity was significantly associated with 73% higher odds of being overweight or obese.
“This study elucidates the distinct hospitalisation patterns and healthcare challenges faced by individuals with PWS in the United States,” the researchers wrote. The data suggest “that hospitalisations for PWS patients are more complex and resource intensive, likely due to the multifaceted medical needs and potential complications associated with the syndrome,” they concluded.
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