[CIS PIDD] BMT for newborn with CGD

[Jane Peake]

Thanks Mort and John. 
We had both previous boys on antifungals, Bactrim proph and IFN gamma and provided extensive education. There were social issues which contributed a bit but to be honest both boys spent most of their lives in hopsital on intensive treatement for the various organisms we managed to culture from their lungs and only went home for very short intervals. I am not sure there was much more we could have done. I think they had a very severe phenotype and also contracted early some bad fungal organisims.
Jane
________________________________________
From: John Ziegler [j.ziegler at unsw.edu.au]
Sent: Tuesday, 28 August 2012 2:53 PM
To: pagid at list.clinimmsoc.org; Jane Peake
Subject: RE: BMT for newborn with CGD

Jane

I agree with Mort. Prophylaxis with antifungals, co-trimoxazole one would think might make a big difference to the outcome, also education about CGD for the family and their carers. + IFN=g if possible.

John

-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Cowan, Mort
Sent: Tuesday, 28 August 2012 2:18 PM
To: pagid at list.clinimmsoc.org
Cc: Long-Boyle, Janel
Subject: Re: [CIS PIDD] BMT for newborn with CGD


Were either of the other kids on any prophylaxis and could that be why they deteriorated so quickly? It would be best if you could buy a little more time but if you have to move forward with a transplant I've cc'd Janel Long-Boyle, PhD, our pharmacist at UCSF who has a special interest in busulfan and fludarabine dosing in children under 6 months of age. She can give you advice on how to dose both drugs. In particular, fludarabine dosing needs to be reduced significantly due to the poor renal clearance in infants.

Mort

Morton J. Cowan, M.D.
Professor of Pediatrics
Chief, Allergy, Immunology, and Blood and Marrow Transplant Division
UCSF Children's Hospital, Room M659
505 Parnassus Ave
San Francisco, CA 94143-1278

Phone: 415-476-2188
FAX: 415-502-4867

**Confidentiality Notice** This email communication and any attachments may contain confidential and privileged information for the use of the designated recipients named above. Distribution, reproduction or any other use of this transmission by any party other than the intended recipient is prohibited.


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Jane Peake
Sent: Monday, August 27, 2012 7:39 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS PIDD] BMT for newborn with CGD



Hi

I was wondering if I could canvas opinions. We have a  baby boy who is now 7 weeks old with confirmed  AR (p67) CGD that has been born to a family that has had 2 previous infants die with CGD. They come from a remote part of Australia and on presentation the other children had a large number of fungal organisms in their lungs and a lot of lung damage. One died  very suddenly with a febrile illness when they presented to their local hospital and  he could not be resuscitated. The family refused a postmortem. The other young boy underwent a matched sibling donot BMT buit died of respiratory failure.
I belive that this infant whilst he is still well should undergo BMT as we have a matched sibling donor sooner rather than later but I have some concerns regading the conditioning regieme in a baby so young both from the increased toxicity from full dose busulfan in neonatal population and long term neurocognitive outcomes.

I would be very interested in others thoughts.
Kind regards
Jane
The CIS-PIDD listserv is supported by:
Clinical Immunology Society - The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

The CIS-PIDD listserv is supported by:
Clinical Immunology Society - The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!