Cystic Fibrosis in CRMS/CFSPID: Infection Prevention and Nutritional Management

Clinical Scenario

This protocol addresses individuals with Cystic Fibrosis-related metabolic syndrome (CRMS) or CF screen positive, inconclusive diagnosis (CFSPID) — those who have screened positive for CF but have an inconclusive diagnostic workup.

The clinical focus is on two areas: preventing cross-infection in healthcare and high-contact settings, and supporting nutritional status in those with adequate growth.

Management Approach (Overview)

The structured approach involves implementation of standard CF infection prevention and control (IPC) measures in healthcare settings — particularly where close contact with multiple individuals who have CF or CRMS/CFSPID is likely. Alongside this, nutritional management is addressed for individuals with adequate growth.

The complete regimen — including specific IPC protocols, the role of the primary care provider, and full nutritional guidance — is detailed in the structured evidence-based protocol.

Instant Access to Structured Evidence-Based Regimens

References

The CFF recommends, for people with CRMS/CFSPID, the implementation of standard CF infection prevention and control (IPC) guidelines in health care settings and situations in which there is a high likelihood of being in close contact with multiple pwCF or people with CRMS/CFSPID (Grade B).

The CFF recommends, for people with CRMS/CFSPID with adequate growth, that nutritional management be provided under the direction of the PCP (Grade B).

DOI: 10.1542/peds.2023-064657

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