Care for Rare Pediatric Skin Conditions Inconsistent & Costly

Although some medications can sometimes mean the difference between life and death, they can sometimes cause complications, resulting in rare diseases and reactions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). These diseases can cause symptoms like severe rashes, blisters, burns, and fevers that can be life-threatening when present in severe degrees.

With such severe adverse reactions and symptoms, lengthy hospitalizations are not uncommon. However, according to a study published by the Journal of Allergy and Clinical Immunology: In Practice, these stays are often expensive, and treatment is often inconsistent due to varied treatment strategies among physicians.

In an effort to help close the treatment disparity experienced by SJS and TEN pediatric patients, lead author, James Antoon, MD, PhD, assistant professor of pediatrics in the UIC College of Medicine, and his colleagues used the Pediatric Health Information System to conduct a retrospective analysis of treatments and outcomes of hospitalized children diagnosed with SJS or TEN between 2008 and 2015.

From the sample, Dr Antoon and his colleagues reviewed treatments, cost of hospital care, length of hospital stays, mortality, recurrence, hospital readmission, and complications related to SJS or TEN in addition to demographic information, such as age, race, gender, insurance type, and geographic location.

“These are conditions that doctors fear because the disease can be extremely severe and there is a lack of research to guide treatment decisions,” said lead author, Dr Antoon in a recent statement. “We know that children with SJS and TEN can have life-long physical and psychiatric effects, but because cases are rare and unpredictable, rigorous studies to evaluate treatments have been difficult to design and implement.”

In the final analysis of pulled data, which included 900 patients with a mean age of 10 years, Dr Antoon and his colleagues found that only 0.56% of the children in the sampled population died from SJS or TEN compared with adults who have been reported in previous studies to have a mortality rate of 23% .

However, the study also found that nearly 1 in 4 pediatric patients was admitted to an intensive care unit, with the stay lasting for a median length of 8 days. Additionally, readmissions were found to be common, with 1 in 5 patients being admitted back to the hospital within 6 months.

"Our finding of low mortality differs from prior, smaller studies performed in the pediatric population," Dr Antoon said. "This is helpful information for physicians counseling families. This means that when we talk with parents, we have evidence to help inform families on expectations and overall prognosis."

Unsurprisingly, expensive costs pertaining to the length of stay were also recorded. The average range of costs fell between $7,000 to $34,000, and the median cost of adjusted hospital charges racked up a bill of about $16,000 per patient.

“There was an expected amount of inconsistency in the treatment of SJS and TEN; however, our analysis showed that no single agent or combination treatment was best at treating SJS and TEN,” Dr Antoon explained.

The study found intravenous immunoglobulin (IVIG) to be the treatment most commonly used in 25% of patients, followed by steroids in 18%, and a combination of both IVIG and steroids in 17% of children. However, regardless of what was used for treatment, little difference was found in patient outcomes. In fact, despite the fact that patient outcomes were similar across all different therapeutic interventions, certain approaches, such as using antibody treatments, were found to be significantly costlier compared with the others.

Furthermore, the study found that cost of care varied depending on the geographic region in which patients were receiving their care. For example, those being treated in the Middle Atlantic and East North Central regions were found to have an increased likelihood (26% and 24%, respectively) of receiving combination therapies that were more expensive compared with those receiving treatment in the West North Central region (8%).

“This is the first large-scale evidence to suggest we can potentially reduce the cost of care for families by selecting lower-cost treatments to achieve the same outcomes,” Dr Antoon stressed. “Overall, these findings suggest we can be cautiously optimistic about SJS and TEN outcomes in a pediatric population,” Antoon said, “but that we need more research to dig deeper into strategies that reduce complications, hospital stays and even the cost of care.”