Optimal management of phenylketonuria: a centralized expert team is more successful than a decentralized model of care.
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).


METHODS
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.
Camfield
Carol S
CS
Department of Pediatrics, Dalhousie University, the IWK Health Centre, Halifax, Nova Scotia, Canada. Camfield@dal.ca
Joseph
Marissa
M
Hurley
Teresa
T
Campbell
Karen
K
Sanderson
Susan
S
Camfield
Peter R
PR
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002170:Canada / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D002983:Clinical Competence; D004046:Dietetics; D019983:Guideline Adherence; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000453:epidemiology; D007361:Intelligence Tests; D017146:Models, Organizational; D009819:Office Visits / Q000706:statistics & numerical data; D010043:Outcome and Process Assessment, Health Care; D010348:Patient Care Team; D010349:Patient Compliance / Q000706:statistics & numerical data; D010651:Phenylalanine Hydroxylase / Q000097:blood; D010661:Phenylketonurias / Q000178:diet therapy*; D011320:Primary Health Care / Q000458:organization & administration* / Q000706:statistics & numerical data; D012189:Retrospective Studies
D010651:Phenylalanine Hydroxylase