[CIS PIDD] [cis-pidd] CGD transplant question

[David Buchbinder]

Dear Colleagues,
 
I have a new 3 year old young man (previously healthy) that came in with pneumonia.  His chest CT demonstrated bilateral airspace disease and consolidation. He also has bilateral cystic changes in both changes which were felt to be related to his necrotizing pneumonia.  His bronchoalveolar lavage grew out aspergillus fumigatus and b. cepacia.  A DHR assay confirmed a diagnosis of CGD.  His genetics are pending given the family history of consanguinity.    
 
He is clinically improving on a variety of antimicrobial agents (levaquin, meropenem, and voriconazole).  He is now afebrile with inflammatory markers that are normalizing.  He came in with respiratory distress and need for supplemental oxygen and these issues have also improved remarkably.  We are also working on his nutrition since this was also a prominent issue.  
 
He does have a matched sibling.  I was considering transplant for this kid in the future.  Any words of wisdom given the burden of lung disease?   I was considering Dr. Grungor's approach, but the busulfan use worried me.  Other reduced toxicity approaches may also be possible (Campath and low dose TBI as recently published by Dr. Kapoor's group).  Would a entirely different approach be better (? gene therapy). 
 
Thanks as always...
 
Dave

CHOCLX-MGSM made the following annotations
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Sat Jun 27 2015 22:22:47 

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