Factors related to survival discharge in trisomy 18: A retrospective multicenter study

Abstract

Infants with trisomy 18 (T18) previously had a poor prognosis; however, the intensive care of these patients has markedly diversified the prognosis. We investigated the current situation of patients with T18, clarified factors for survival discharge, and surveyed actual home healthcare. A total of 117 patients with T18 admitted to nine institutions between 2000 and 2015 were retrospectively investigated. After excluding four patients whose outcomes were unclear, we divided 113 patients into two groups—the survival discharge group (n = 52) and the death discharge group (n = 61)—and compared maternal factors, perinatal factors, neonatal factors, and therapeutic factors between the groups. In addition, home healthcare, readmission, utilization of respite care and home nursing, and cause of death among the survival group were surveyed. Fifty‐two (44%) patients with T18 survived at discharge and their 1‐year survival rate was 29%. The survival group had a longer gestation period, larger physique, and longer survival time, compared to the death group. Independent factors associated with survival discharge were the absence of an extremely low birthweight infant (ELBWI), the absence of esophageal atresia and patent ductus arteriosus, and cardiovascular surgery. All surviving patients required some home healthcare. The most frequent cause of death was a respiratory disorder. We recommend discussing the treatment strategy with families in the presence of neonatologists or pediatric surgeons, who can explain differences in prognosis, based on the gestation period, birthweight, severity of cardiovascular disease, and cardiovascular surgery.

A validated model for prediction of survival to 6 months in patients with trisomy 13 and 18

“…Using a parental survey, Kosho and colleagues reported that intensive neonatal care including gastrostomy tube placement, mechanical ventilation, gastrointestinal surgical intervention, and antibiotic administration resulted in an improved 1 year survival (29%) and median survival (107 days) in T18 (Kosho, Kuniba, Tanikawa, Hashimoto, & Sakurai, 2013). Kato et al reported similar 1 year survival in a Japanese cohort of T18 patients, the majority of whom received intervention (Kato et al, 2019). The high degree of variability among healthcare providers, in the face of the literature, suggests many providers may be relying on personal experiences and their personal quality of life judgments to guide their decision‐making.…”

“…Ishitsuka et al reported one‐third of patients admitted ≥7 days received nutritional support via gastrostomy feeds (Ishitsuka et al, 2015). In a multi‐institutional Japanese study, all T18 patients who survived to discharge received tube feeding, which included gastrostomy feeding (Kato et al, 2019). In a retrospective, statewide study in Florida, Nguyen et al reported higher one‐year survival—18% in T13 and 17% in T13—than previously anticipated in patients who underwent gastrointestinal surgery (Nguyen et al, 2018).…”

Building trust and improving communication with parents of children with Trisomy 13 and 18: A mixed-methods study

“…Recently, more infants are receiving medical interventions, and survival for more than a year is not uncommon, leading to a recommendation to avoid calling these conditions “lethal.” 1 We have previously reported that parents who lived with children with T13-18 experienced significant emotional and financial hardships, but that they also reported many positive experiences. 2–4…”

“…1 We have previously reported that parents who lived with children with T13-18 experienced significant emotional and financial hardships, but that they also reported many positive experiences. [2][3][4] T13-18 are heterogeneous conditions: some children will die within weeks, despite attempts at life-saving interventions, while others may survive for several months or years, even with comfort care. Medical or surgical interventions, which may sometimes be beneficial, can also at times be a burden.…”

A visual tool inclusive of fetal ultrasound and autopsy findings to reach a balanced approach to counselling on trisomy 18 in early second trimester

“…Additional information available in the literature regarding the use of a patient-centered care approach and the impact of surgical interventions on the long-term outcomes were also communicated [2,3,6,8,14,[25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42][43][44]. Following a painful decision-making process, a final request for therapeutic termination of pregnancy was made.…”