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Severe combined immunodeficiency (SCID):
initial diagnosis and management

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To download the full Stardard of Care documents, please click on the either the DOC (Word document) or PDF buttons above. To email a comment, click on the comment button. Key Standards of Care are listed below.

SCID: Key standards of care

1. Where a diagnosis of SCID is suspected the following diagnostic tests must be undertaken

  • Lymphocyte count
  • T and B cell numbers
  • T, B and NK cell numbers and %.  
  • Immunoglobulin IgG, A and M

2. If a diagnosis of SCID is suspected the following actions must be taken

  • Contact the local immunologist
  • Discuss with one of the SCID specialist centres (Newcastle or GOSH)

3. A child with probable SCID must be cared for as follows:

  • When in hospital they must be cared for in a cubicle (HEPA filtered/Laminar flow where available)
  • Protective isolation measures must be used to protect them from infections.
  • If a cubicle is not available and child requires inpatient care, they must be transferred to another hospital with a cubical available whilst awaiting transfer to the SCID centre.  
  • If the child is well and does not need to be in hospital, they can be cared for at home by parents.

4. The following treatments must be instigated in discussion with the local/ supra-regional immunology/SCID team:

  • If a possibility of active infection:
    • i. Seek and treat promptly considering empirical CMV/PCP or other atypical organism therapy
    • ii. If BCG has been given, start isoniazid and rifampicin (or other suitable drugs).

     

  • Avoid further infections
    • i. Nurse child in a cubicle with protective isolation measures
    • ii. Give prophylactic co-trimoxazole (Septrin)
    • iii. Give prophylactic fluconazole (or other anti-fungal)
    • iv. Start replacement immunoglobulin: (even if IgG normal) consider a loading dose of 1g/kg (See SOC on administration of Ig)

5. Avoid complications

  • No vaccinations should be given
  • All blood products must be irradiated and CMV negative

 

 


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