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SICKLE CELL DISEASE IN CHILDREN AND ADOLESCENTS:

DIAGNOSIS, GUIDELINES FOR COMPREHENSIVE CARE, AND CARE PATHS AND PROTOCOLS FOR MANAGEMENT OF ACUTE AND CHRONIC COMPLICATIONS*

Peter A. Lane, George R. Buchanan, John J. Hutter, Robert F. Austin, Howard A. Britton, Zora R. Rogers, James R. Eckman, Michael R. DeBaun, Winfred C. Wang, Prasad Mathew, Sarah Iden, Michael Recht, Jesse D. Cohen, Ernest Frugé, Leanne Embry, Lewis Hsu, Brigitta U. Mueller, Robert Goldsby, Charles T. Quinn, Marie Mann, and Michele A. Lloyd-Puryear for the Sickle Cell Disease Care Consortium

*Revised at the Annual Meeting of the Sickle Cell Disease Care Consortium, Sedona, AZ, November 10-12, 2001 ( representing Arizona, Colorado, Georgia, Missouri, New Mexico, Tennessee, Texas, and Utah)

Supported in part by the Mountain States Genetics Network, by the Texas Genetics Network and Texas Newborn Screening Hemoglobinopathy Grant (Texas Department of Health), and by Project #5H46 MC00132 and a contract from the Maternal and Child Health Bureau (Title V Social Security Act), Health Resources and Services Administration, Department of Health and Human Services.

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TABLE OF CONTENTS

INTRODUCTION 

PRINCIPLES OF CARE FOR CHILDREN AND ADOLESCENTS WITH SICKLE CELL DISEASE 

DIAGNOSTIC TESTING FOR THE COMMON SICKLE CELL SYNDROMES 

NEWBORN SCREENING FOLLOW-UP GUIDELINES

Follow-up of Infants with Probable Hemoglobin Disease 

Follow-up of Infants with Probable Hemoglobin Trait 

Follow-up Procedures for Infants with Hemoglobin Bart's 

Unidentified Hemoglobin Variants

Genetic Counseling 

SICKLE CELL DISEASE -COMPREHENSIVE CARE 

Routine comprehensive evaluations 

Immunizations and prophylactic medications 

Sickle cell patient & family needs assessment 

ACUTE ILLNESS IN SICKLE CELL DISEASE: 

Illness Requiring Urgent Medical Care 

Transfusion therapy for acute complications 

CLINICAL CARE PATHS

OUTPATIENT EVALUATION AND MANAGEMENT OF FEBRILE ILLNESS 

INPATIENT MANAGEMENT OF FEVER 

OUTPATIENT EVALUATION AND MANAGEMENT OF PAIN 

INPATIENT MANAGEMENT OF VASO-OCCLUSIVE PAIN 

ACUTE CHEST SYNDROME 

ACUTE SPLENIC SEQUESTRATION 

APLASTIC CRISIS 

ACUTE STROKE OR NEUROLOGIC EVENT 

OUTPATIENT MANAGEMENT OF PROLONGED PRIAPISM 

INPATIENT MANAGEMENT OF PROLONGED PRIAPISM 

GENERAL ANESTHESIA AND SURGERY 

CHRONIC TRANSFUSION PROTOCOL 

HYDROXYUREA PROTOCOL 

HEMATOPOIETIC STEM CELL TRANSPLANTATION 

TRANSCRANIAL DOPPLER ULTRASONOGRAPHY 

REFERENCES 

A P P E N D I X

Sickle cell disease care consortium and  Other Contributors 

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Last modified: January 07, 2003