title: The case for a national service for primary immune deficiency disorders in New Zealand.
abstract: Primary immune deficiency disorders (PIDs) are rare conditions for which effective treatment is available. It is critical these patients are identified at an early stage to prevent unnecessary morbidity and mortality. Treatment of these disorders is expensive and expert evaluation and ongoing management by a clinical immunologist is essential. Until recently there has been a major shortage of clinical immunologists in New Zealand. While the numbers of trained immunologists have increased in recent years, most are located in Auckland. The majority of symptomatic PID patients require life-long immunoglobulin replacement. Currently there is a shortage of subcutaneous and intravenous immunoglobulin (SCIG/IVIG) in New Zealand. A recent audit by the New Zealand Blood Service (NZBS) showed that compliance with indications for SCIG/IVIG treatment was poor in District Health Boards (DHBs) without an immunology service. The NZBS audit has shown that approximately 20% of annual prescriptions for SCIG/IVIG, costing $6M, do not comply with UK or Australian guidelines. Inappropriate use may have contributed to the present shortage of SCIG/IVIG necessitating importation of the product. This is likely to have resulted in a major unnecessary financial burden to each DHB. Here we present the case for a national service responsible for the tertiary care of PID patients and oversight for immunoglobulin use for primary and non-haematological secondary immunodeficiencies. We propose that other PIDs, including hereditary angioedema, are integrated into a national PID service. Ancillary services, including the customised genetic testing service, and research are also an essential component of an integrated national PID service and are described in this review. As we show here, a hub-and-spoke model for a national service for PIDs would result in major cost savings, as well as improved patient care. It would also allow seamless transition from paediatric to adult services.
authors.affiliation: Departments of Clinical Immunology, Virology and immunology, Auckland City Hospital, Auckland, New Zealand. rohana@adhb.govt.nz.
authors.forename: Rohan
authors.initials: R
authors.lastname: Ameratunga
authors.forename: Richard
authors.initials: R
authors.lastname: Steele
authors.forename: Anthony
authors.initials: A
authors.lastname: Jordan
authors.forename: Kahn
authors.initials: K
authors.lastname: Preece
authors.forename: Russell
authors.initials: R
authors.lastname: Barker
authors.forename: Maia
authors.initials: M
authors.lastname: Brewerton
authors.forename: Karen
authors.initials: K
authors.lastname: Lindsay
authors.forename: Jan
authors.initials: J
authors.lastname: Sinclair
authors.forename: Peter
authors.initials: P
authors.lastname: Storey
authors.forename: See-Tarn
authors.initials: ST
authors.lastname: Woon
mesh_terms: D000328:Adult; D000486:Allergy and Immunology / Q000458:organization & administration*; D002648:Child; D017074:Common Variable Immunodeficiency / Q000191:economics / Q000628:therapy; D003695:Delivery of Health Care / Q000191:economics / Q000458:organization & administration*; D019468:Disease Management; D017048:Health Care Costs; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000191:economics / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000191:economics / Q000628:therapy*; D007155:Immunologic Factors / Q000191:economics / Q000627:therapeutic use; D009520:New Zealand; D011787:Quality of Health Care
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors