238 I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 3. Results The literature search identified 2933 discrete citations. After screening the corresponding titles and abstracts, 35 studies seemed to be eligible for inclusion according to our criteria. The full texts of these studies were then reviewed in-depth. Thirty studies had to be excluded: 6 studies that included no, or too few, children with SDDs; 13 studies that did not focus on EoLDM; 5 studies that did not focus on the parents’ perspective; and 6 studies that appeared to be case reports. An additional 4 studies were subsequently found by searching the reference lists of suitable studies. In the end, nine studies were eligible for inclusion in our review. The flow of information for our review is summarized in Fig. 1. In Table 1, we present the characteristics of the 9 included studies. The studies were inconsistent in the terminology used to describe EoLDs. We encountered, for example, ‘limit or withdraw life support’ (Sharman, Meert, & Sarnaik, 2005), ‘decision-making in the PICU’ (Madrigal et al., 2012), ‘choosing plan of care’ (Guon, Wilfond, Farlow, Brazg, & Janvier, 2014) and ‘critical decisions’ (October, Fisher, Feudtner, & Hinds, 2014). All but two studies had a qualitative design, although in some studies questionnaires were used (Guon et al., 2014; October et al., 2014). The remaining two studies had a quantitative design (Friedman, 2006; Madrigal et al., 2012). Three of the nine studies prospectively followed parents throughout the decision-making process (Madrigal et al., 2012; October et al., 2014; Sharman et al., 2005). The other six studies retrospectively investigated parents’ experiences and preferences. In these studies, the period of time that had elapsed between the EoL decision and study participation varied considerably, from several months up to nineteen years (Brotherton & Abbott, 2012; Friedman, 2006; Guon et al., 2014; Morrow, Quine, Loughlin, & Craig, 2008; Rapoport, Shaheed, Newman, Rugg, & Steele, 2013; Reilly, Huws, Hastings, & Vaughan, 2010). All studies were conducted in the United Kingdom, United States, Australia or Canada. In Table 2, we present regulations of parental involvement and conflict resolution in these countries according to the professional guidelines. One study also included a small percentage of parents from other countries (Guon et al., 2014). [(Fig._1)TD$FIG] End-of-life decision making Mental retardation Parental or patient participation Children 0-21 710 MEDLINE 2043 EMBASE 458 PsycINFO 424 CINAHL 3635 Hits Deduplication 2933 Articles identified by screening on title 35 Potentially relevant studies retrieved for review in full 5 Relevant articles 30 Articles were excluded: • 6: No or too few children with an SDD • 13: Not related to EoLDM • 5: No report of parents • 6: Case reports 4Articles • Articles identified from a reference list search 9Articles in analysis Fig. 1. Flow diagram for study selection.
Table 1 Characteristics of included studies. Source Study participants, n Nationality Patients, n Age Sex Underlying diagnosis of patients, (n) Decision Factors described d Timing of data collection Study design (data collection; analysis) Relevance/ Credibility —Qualitative Health Research Friedman (2006)— Journal of the American Medical Directors Association Guon et al. (2014)— American Journal of Medical Genetics Madrigal et al. (2012)— Critical Care Medicine Morrow et al. (2008)— Archives of Disease in Childhood October et al. (2014)— Pediatric Critical Care Medicine Rapoport et al. (2013)— Pediatrics Mothers, n =20 UK b n =20 Age: 1–15 y Sex: 11 girls and 9 boys Parents and/or guardians, n =60 Mothers, n =98 Fathers, n =30 US c n =60 Age: 2–32 y Sex: 31 girls and 29 boys 83% US, 5% Canada, 5% UK, 7% from 12 other countries n = 107 Age and sex not specified Parents, n =87 US n =75 Age: 0–19 y Sex not clearly specified Mothers, n =17 Fathers, n =2 Foster mother, n =1, Sibling, n =1 Mothers, n =25 Fathers, n =18 Mothers, n =6 Fathers, n =5 Australia n =21 Age: 4–19 y Sex not specified US n =29 Age: 1–10 y Sex: 16 girls and 13 boys Canada n =7 All children died Age and sex not specified Cystic fibrosis, global Gastrostomy developmental delay, placement faltering growth, cerebral palsy, congenital malformations, neurodegenerative disorders (numbers not specified) Severe developmental disabilities and complex medical problems Trisomy 13, Trisomy 18 E-I P-I G-F Retrospective (2 mo–7 y 8 mo after gastrostomy placement) Qualitative study— (in-depth interviews; discourse analysis) Resuscitation G-F Before and after Quantitative study— provision of written (structured interviews by information phone and review of records; statistical analysis) Continuance of pregnancy Choice of plan of care (full interventions, comfort care, combination of full interventions and comfort care) Cardiovascular, Medical decisions respiratory, congenital or in PICU a setting genetic, neuromuscular, metabolic, malignancy (numbers not specified) Quadriplegic cerebral palsy with feeding difficulties Haematologic/oncologic (10), respiratory (6), neurologic (4), trauma (4), gastrointestinal (2), metabolic/genetic (2), sepsis/shock (1) Genetic or suspected genetic conditions Interventions for feeding difficulties Critical treatment decision (decision to initiate, escalate, withdraw lifesustaining therapies) Forgoing artificial nutrition and hydration (FANH) G-F Retrospective Mixed methods study— (a computer assisted self-completion questionnaire with 10 open-ended questions and 12 demographic questions; statistical analysis and thematic qualitative content analysis) P-I Prospective Quantitative study— (interviews and standardized instruments; statistical analysis) E-I G-F Prospective Qualitative study— (focus groups; constant comparative method) G-F Prospective Mixed methods study— (structured interviews by means of Good Parent Tool and Good Parent Ranking Exercise; thematic qualitative content analysis) P-I G-F Retrospective (1.5–5 y after the death of the child) Qualitative study— (in-depth interviews; interpretive description approach) High/High High/low High/high High/ moderate High/high Low/low High/high I.H. Zaal-Schuller et al. / Research in Developmental Disabilities 49–50 (2016) 235–246 239