ABSTRACT
OBJECTIVE: To compare phenylketonuria (PKU) management by a centralized, expert team in the Province of Nova Scotia (NS) with the decentralized approach in New Brunswick (NB). STUDY DESIGN: Retrospective chart review documented frequency of outpatient visits, phenylalanine (Phe) concentration, and medical formula use. Structured telephone interviews with the 8 regional NB dietitians (NB-D) documented their knowledge and support in PKU management. Patients with PKU (n=108; age, birth to 42 years) reside in NB (n=69) and NS (n=39). More were lost to contact in NB than in NS (9/69 vs 1/39) and more were completely off diet in NB than in NS (24/60 vs 1/38, P=.05). All 15 children <2 years old followed by a PKU team in either NS or Saint John, NB had optimal Phe levels. Children 2 to 12 years of age in NS had better Phe control and more medical visits than in NB (P <.01). Older patients had more episodes of elevated Phe levels (P=.01). Formula was dispensed in appropriate yearly amounts to 52% in NB and >95% in NS. Mental handicap or borderline intelligence was common in both NB (44%) and NS (42%). All NB-D wished additional specialized medical, nursing, or social work assistance. CONCLUSIONS: PKU management appears to be more effective with an expert, coordinated team approach.
Subject(s)
Models, Organizational , Outcome and Process Assessment, Health Care , Patient Care Team , Phenylketonurias/diet therapy , Primary Health Care/organization & administration , Adolescent , Adult , Age Factors , Canada/epidemiology , Child , Child, Preschool , Clinical Competence , Dietetics , Guideline Adherence , Humans , Infant , Infant, Newborn , Intellectual Disability/epidemiology , Intelligence Tests , Office Visits/statistics & numerical data , Patient Compliance/statistics & numerical data , Phenylalanine Hydroxylase/blood , Primary Health Care/statistics & numerical data , Retrospective StudiesABSTRACT
OBJECTIVES: To explore whether self-injurious behavior (SIB) alters pain expression in children with severe cognitive impairments and the relation between SIB and chronic pain. STUDY DESIGN: Caregivers of 101 nonverbal children 3 to 18 years of age (55% boys) completed the Non-Communicating Children's Pain Checklist-Revised (NCCPC-R) retrospectively and for an observed pain episode. Caregivers of children with SIB (n = 44) completed the Behavior Problems Inventory, the Self-Injury Grid, and the Self-Injury and Self-Restraint Checklist. RESULTS: Multivariate analysis of variance indicated that NCCPC-R scores did not differ between children with and those without SIB. However, t tests indicated that children with chronic pain (n = 13) self-injured less body surface (P =.01) and fewer body sites (P =.04) than did children without (n = 31). Multiple Correspondence Analysis generated 2 dimensions (49% variance), suggesting a distinction between two SIB forms: (1) high frequency of SIB to the head/hand and absence of chronic pain and (2) less frequent SIB near the site of pain. CONCLUSIONS: Children with severe cognitive impairments who display SIB do not have reduced pain expression, and chronic pain may influence the frequency and location of SIB. Further research should examine the usefulness of these findings for management of SIB and pain.