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

Thu May 26 09:58:38 EDT 2016

I have had success using Cipro and Levaquin 500mg in 500ml of saline for
nasal irrigation for chronic suppression. I do not treat active infection
this way but have found add the antibiotic to the nasal irrigation solution
to decrease the frequency of symptomatic infection. I don't know what the
system absorption is in the situation but I suspect it is pretty low. This
approach has been especially helpful in patients with IBD who tolerate
antibiotics poorly so I suspect that not much gets swallowed and absorbed.
Richard Wasserman
Dallas

On Thu, May 26, 2016 at 8:45 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> 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
>
> 10903 New Hampshire Ave
>
> Silver Spring, MD 20993-0002
>
> Robert.Sokolic at fda.hhs.gov
>
> (240) 402-5564
>
> FAX: (301) 595-1305
>
>
>
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> This communication does not constitute a written advisory opinion under 21
> CFR 10.85, but rather is an informal communication under 21 CFR 10.85(k)
> which represents my best judgment at this time, but does not necessarily
> represent the formal position of FDA, and does not bind or otherwise
> obligate or commit the agency to the views expressed.
>
>
>
> *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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-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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