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+) |