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+) |