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Table 4 Schedule of HSCT Center tasks for infants with IKD requiring HSCT. These are the steps to be taken after: 1) KD-NBS and confirmatory testing established a diagnosis of IKD, 2) diagnosis and care options were discussed with the family

From: Consensus guidelines for newborn screening, diagnosis and treatment of infantile Krabbe disease

1. Refer to transplant center ASAP (DOL 5-6) (Fig. 1)
2. HSCT Center helps to arrange insurance coverage, lodging, admission for work up
3.Baby admitted to HSCT Center (DOL 7-8):
 a. Blood drawn for stat HLA typing (high resolution Class I ABC, Class II DRB1), and studies, including, blood type, and psychosine
 b. Maternal blood for donor screening tests
 c. CSF for protein, cell count
 d. Neuroimaging tests: MRI brain with DTI
 e. Neurophysiological tests: EEG, BAER, VEP, nerve conduction tests
 f. Neurology and neurodevelopmental consult
 g. Hearing and vision evaluations
 h. Echocardiogram to check for PFO or PDA. If present, filter IV lines to prevent air emboli
 i. Physical therapy consultation
 j. When HLA typing is available, search for an unrelated cord blood unit donor, select units (> 4/6 match and > 5×10e7 cells/kg for HLA-confirmatory typing and GAL-C enzyme levels) to be used for final unit selection
 k. Proceed with insurance/third party payer authorization for transplantation
 l. Place central line and consider G-Tube placement for supplemental feeding
 m. Administer chemotherapy (currently 9 days)
 n. Make final cord blood unit selection during chemotherapy
 o. Administer transplant (DOL 21+)