22q11.2 Hypocalcemia Screening/Treatment Clinical Pathway — Inpatient, Outpatient Specialty Care and PICU

Clinical Pathways Home Emergency ICU Inpatient Outpatient Specialty Care Primary Care

Goals and Metrics

Patient Education

Related Pathway

Thyroidectomy Calcium Management, Inpatient and Outpatient Specialty Care

Peri-Operative Management of Hypocalcemia in Children with 22q11.2 Deletion/Duplication

Preoperative Care

Scheduled Elective Procedures

Anesthesia APP Provider
Endocrine Calcium Emergency Letter in Epic
- Review need for Stress Dose Calcium given evening prior to procedure
Current Ca treatment
Recent Ca level (within 3 mos)
Notifies outpatient Endocrinologist of planned procedure
Place blood drawing IV for repeating post-op Ca levels
Pre-Procedural Endocrine and Genetics Evaluations
- Scheduling with Endocrine, urgent appointments, children without known history or hypocalcemia
Endocrine
Orders Ca, albumin if no results within 3 mos
Documents peri-operative recommendations in Epic

Inpatient Procedures

Hospital Provider Reviews:
- Endocrine Calcium Emergency Letter in Epic
- Review need for Stress Dose Calcium given evening prior to procedure
Current Ca Treatment
Recent Ca level (within 3 mos)
Orders Ca, albumin if no results within 3 mos
Consults Endocrine

Postoperative Hypocalcemia Treatment and Monitoring Recommendations Signs and Symptoms of Hypocalcemia
	Monitoring	Treatment for Ca < 8.5 mg/dL	Escalation
High Risk Patient Factors History of hypocalcemia with procedures/illness or baseline requirement for calcium and/or calcitriol supplementation Procedure Factors Longer/more invasive procedures Delayed transition to PO/usual enteral intake	All Post-op Ca within 1-4 hrs post procedure if procedure lasts ≥ 2 hrs Inpatient Continue Ca monitoring every 4-6 hrs Discontinue if 2 Ca ≥ 8.5 mg/dL more than 8 hrs apart Outpatient Review child’s post-discharge Ca monitoring plan Typically, if Ca normal, check 2-3 days post-discharge Labs ordered by primary endocrinologist	Consult Endocrine Give IV Calcitriol in the OR/recovery room Followed by Oral/IV Calcitriol and Oral Calcium as soon as able to tolerate PO Goal: Start within 4 hrs of surgery Asymptomatic Start at lower end of suggested dose ranges Signs/Symptoms of Hypocalcemia Consider escalation Titration Titrate the calcium and/or calcitriol with support of Endocrine consult if there is no improvement in Ca level	Signs/Symptoms of Hypocalcemia Consider Oral/IV Calcitriol IV Calcium Transfer to ICU
Intermediate Risk Patient Factors History of mild hypocalcemia with procedures/illness Procedure Factors Longer/more invasive procedures Delayed transition to PO/usual enteral intake	All No routine post-procedure Ca monitoring Obtain post-procedure Ca if child is not back to baseline (sedated, not taking PO well) within 4 hrs Inpatient Continue Ca monitoring every 4-6 hrs Discontinue if 2 Ca ≥ 8.5 mg/dL more than 8 hrs apart Outpatient Review child’s post-discharge Ca monitoring plan Typically, if Ca normal, check 2-3 days post-discharge Labs ordered by primary endocrinologist	Consult Endocrine Give Oral/IV Calcitriol and Oral Calcium Goal: Start within 4 hrs of surgery Asymptomatic Start at lower end of suggested dose ranges Signs/Symptoms of Hypocalcemia Consider escalation	Signs/Symptoms of Hypocalcemia Consider Oral/IV Calcitriol IV Calcium Transfer to ICU
Low Risk Patient Factors No known history of hypocalcemia Procedure Factors Short procedure with quick post-procedure transition to home feeding regimen	No routine post-op Ca monitoring required Review Symptoms of Hypocalcemia with family prior to discharge

Discharge Recommendations

Posted: May 2021
Revised: May 2024, Revisions

Authors:

Editors: Clinical Pathways Team

Evidence

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.

PICU, Inpatient and Outpatient Specialty Care Clinical Pathway for Peri-Operative Management of Hypocalcemia in Children with 22q11.2 Deletion/Duplication

Evidence