Chronic paediatric conditions: Cystic Fibrosis

This code list is part of a set of harmonised code lists to identify chronic paediatricconditions across primary and secondary care datasets across the UK.

Published ICD-10 and Read v2 code lists were combined with SNOMED codes derived usingCALIBER as below:

• Read v2 cystic fibrosis code list: https://phenotypes.healthdatagateway.org/phenotypes/PH14/version/28/detail/
• ICD-10 cystic fibrosis diagnoses as per Hardelid 2014 mapped through CALIBER to produce SNOMED code list.

Methods

Chronic paediatric conditions were defined as health problems requiring medical follow-upfor more than 12 months in 50% or more of cases. Code lists were produced by mappingexisting ICD-10 codes for chronic paediatric conditions to SNOMED and Read v2 codesusing the CALIBER R package, with review by paediatric sub-specialists.

An established ICD-10 code list of chronic paediatric conditions (Hardelid 2014)was used as the basis for the new lists.1 Chronic paediatric conditions were definedas any health problem requiring medical follow-up (hospital admission, outpatientfollow up or use of support services such as physiotherapy) for more than 12 monthsin 50% or more of cases.[1] A Chronic Paediatric Conditions Committee of paediatricsub-specialists was established. The original ICD-10 list was screened by two paediatriciansand acute conditions removed, with disagreements resolved by a third paediatrician.Conditions which were likely acute and would not fulfil our chronic disease definitionwere excluded, including skeletal injuries and self-harm. Drug-induced conditionswere excluded as chronicity was not known. Infections other than human immunodeficiencyvirus (HIV), hepatitis B or C, or congenital infections (syphilis / toxoplasmosis/ rubella / herpes simplex virus / cytomegalovirus) were excluded. Chronic sequelaeof infections were retained.

Published code lists were then identified for Read v2 and SNOMED codes for well-characterisedconditions including asthma, diabetes, cystic fibrosis and cancer. The remainingICD-10 codes were mapped to SNOMED and Read v2 codes using the CALIBER R packageand Technology Reference Update Distribution NHS Data Migration tables [2] to producenew code lists suitable for primary care data. These resulting code lists were screenedby two sub-specialists to ensure conditions were chronic and in the correct subgroupwith disagreements resolved by a third clinician.

References:

1. Hardelid P, Dattani N, Gilbert R. Estimating the prevalence of chronic conditions in children who die in England, Scotland and Wales: a data linkage cohort study. BMJ Open 2014; 4: e005331.
2. CALIBER codelists-package: Generate ICD, Read and OPCS codelists in CALIBER codelists: Generate ICD10, Read and OPCS codelists, https://rdrr.io/rforge/CALIBERcodelists/man/CALIBERcodelists-package.html (accessed 19 October 2022).

We have considered conditions to be active if they had been coded for in the precedingfive years. In addition, conditions from the following subgroups were consideredto be permanent, therefore active if coded for at any time: metabolic, diabetes,cystic fibrosis, transplant, receiving palliative care, other congenital multisystemsyndromes and chromosomal abnormalities, immunological and cerebral palsy / paralysis(a subgroup of "Other neurology" code list).