[CIS PIDD] [cis-pidd] chronic rhinosinusitis in ADA SCID patient

Thu May 26 12:38:26 EDT 2016

Endoscopically directed cultures accurately reproduce sinus puncture.  See my review in JACI 2008;121:917-27.

He has continuing infection with H. influenza.  Assuming that it is at moderate or heavy growth, there is concern that it is not being eradicated,  either because of his immunological state or because of other factors: osteitis, biofilm (causing misleading sensitivity information), inadequate penetration of Augmentin into the sinuses, reintroduction of the same organism because of irrigation container contamination.  He has already developed resistance to SxT as a result of chronic treatment and is likely to develop resistance to Augmentin with continued use.

Have his cultures been done off antibiotics?  There may be partial suppression of other organisms if done on SxT.    What bacteria showed up on gram stain?

I would be happy to discuss this further by phone if you would like as he is clearly a complicated patient..

Sincerely,

Wellington S. Tichenor, M. D.
Associate Clinical Professor of Medicine
New York Medical College
642 Park Avenue
New York, New York 10065
212 517-6611
wtichenor at sinuses.com<https://register.concentric.com/home/apps/mail/mbox_compose.cgi?pTo=wtichenor@sinuses.com>

________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, May 26, 2016 9:45 AM
To: CIS-PIDD
Subject: RE:[cis-pidd] chronic rhinosinusitis in ADA SCID patient

I’ve had my best luck with sinusitis in ADA-SCID using Levaquin. I had some concern using it in kids, but did it anyway b/c I found the ADA deficient patients often dropped their white counts to beta-lactam antibiotics. I am aware of the recent warning by my own agency that the risks of fluoroquinolones outweigh the benefits in sinusitis, but this may not be the case in SCID, as such patients have frequent health-care contacts and are therefore exposed to a different range of pathogens. Also, if the family and child are known to have excellent compliance and a high level of medical sophistication, the risks of tendonitis might be attenuated by their attention to early signs. As for the hematologic toxicities, I would be more concerned about myelosuppression by beta lactams than by quinolones in ADA deficiency (see Blood 118: 2688, 2011).

I would consider repeat sinus aspirate, with the ENT surgeon understanding to send cultures for everything, including mycobacteria and fungus. Would send PCRs for respiratory viruses, although positive results would have to be interpreted in the context of what sounds more like a bacterial infection.

Is this patient still on Adagen? If not, I would consider re-evaluating adenosine metabolites with Mike Hershfield and rechecking T-cell numbers and function. If he is on Adagen, I would do this also, as he may need dose adjustment. In my older patients, I have used high-dose adagen, and there is a case report suggesting a benefit of achieving higher plasma levels than are typically obtained in older patients on standard doses (Tartibi HM, Hershfield MS, Bahna SL. Pediatrics. 2016 Jan;137(1). doi: 10.1542/peds.2015-2169. Epub 2015 Dec 18.) If adenosine metabolism is not optimal, would consider increasing dose. If adenosine metabolism is OK, but T cell function or numbers have deteriorated, would consider increasing the dose anyway, depending on trough plasma ADA activity. Another consideration would be decreasing the ADA dose to favor expansion of the maternal T-cells. In the past, I have assessed maternal T-cell chimerism in such a child using X-Y FISH on peripheral blood.

Am happy to discuss if you would like to do so.

--
Rob Sokolic, MD
Medical Officer
Office of Cellular, Tissue and Gene Therapies
Center for Biologics Evaluation and Research
Food and Drug Administration
White Oak Building 71, Room 5261
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Silver Spring, MD 20993-0002
Robert.Sokolic at fda.hhs.gov<mailto:Robert.Sokolic at fda.hhs.gov>
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FAX: (301) 595-1305

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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, May 25, 2016 5:16 PM
To: CIS-PIDD
Subject: [cis-pidd] chronic rhinosinusitis in ADA SCID patient

Dear colleagues,

I am seeking input on a teenage ADA SCID patient (s/p non-conditioned haplo BMT with persistent T cell lymphopenia) with chronic rhinosinusitis.  He has been on multiple rounds of culture-directed oral antibiotics with fair response.  His sinus CT demonstrates mild mucosal thickening.  Scoping by ENT demonstrates persistent purulence with cultures growing H. influenzae type A beta-lactamase negative and sensitive to all testing antibiotics except TMP/SMX.  He has been treated repeatedly with augmentin and is a highly adherent patient.  His monthly IVIG dose has been increased with most recent IgG trough 944 mg/dl.  Intranasal steroids have been tried (OMCs are patent). He does nasal lavage with sterile normal saline 2x/daily.

He has fair response to oral antibiotics and has a history of severe C. Diff colitis.  His quality of life is not bad, but he has frequent sore throat. occasional cough, and intermittent serous OM.

Are any of you aware of new approaches to this common problem in our patient population or can share anecdotal successes?

Thank you

Christine M. Seroogy MD,  FAAAAI
Associate Professor
University of Wisconsin School of Medicine and Public Health
Department of Pediatrics
Division of Allergy, Immunology & Rheumatology
1111 Highland Avenue
4139 WIMR
Madison, WI  53705-2275
phone: 608-263-2652
fax: 608-265-0164

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