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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+) |
