[CIS PIDD] [cis-pidd] challenging patient for IVIG/SCIG

[Maite de la Morena]

I would be curious to ask how many people have seen incapacitating neurologic disorders temporally associated with IgG products

I have recently begun caring for a patient who carries the diagnosis of autoimmune encephalomyelitis-Stiff Person Syndrome with evidence of anti-GAD antibodies.

Mother feels everything started after an unusual reaction to an IVIG product (was changed from Octagam to Gamunex). The reaction occurred at the end of the infusion characterized by tachycardia , then fainting episodes that kept her admitted for two weeks.
Thank you
Maite

Maite de la Morena, MD
Associate Professor of Pediatrics
Division of Allergy and Immunology
University of Texas Southwestern Medical Center in Dallas
5323 Harry HInes Blvd
Dallas, Texas 75390-9063
Phone 214 456-5161
Fax: 214 456-8317
Email: maite.delamorena at utsouthwestern.edu





From: Javier Chinen [mailto:chinej20 at hotmail.com]
Sent: Tuesday, November 05, 2013 8:19 PM
To: CIS-PIDD
Subject: RE: [cis-pidd] challenging patient for IVIG/SCIG

hi Elena,

I am managing a CVID patient who did not tolerate several IV and all 3 SC Ig products, though not with an anaphylaxis-like reaction. She always had problems but then she developed an incapacitating neuromuscular syndrome after doses.
Reasoning this had to be managed following drug allergy guidelines, we designed a 'desensitization' protocol, with premeds:

- Advance from 0.1 cc to 1 cc SQ Hizentra on day 1. Increases every 15 min
- Stay in 1 cc daily for 2 weeks= 15cc = 3g
- Advance 1 cc weekly- next two weeks = 35 cc= 7g -->> 10g first month
And so forth, now she tolerates 20cc (4 g) every 3 days
Symptoms reappeared when she reached 10cc/dose. Switched to Gammagard and Gamunex with no much difference. She is still with significant pain and weakness after doses, but it is not as incapacitating. Her levels are now in the 800s. While finding the right approach her levels went down to 200s and recurrent infections occurred, stressing the need of replacement. Logistically, with this approach there is an issue with waste of product that needs to be anticipated.

Hope it is helpful

Javier Chinen, MD, PhD



> From: e.perez13 at med.miami.edu<mailto:e.perez13 at med.miami.edu>

> To: cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>

> Date: Tue, 5 Nov 2013 20:22:00 -0500

> Subject: RE:[cis-pidd] challenging patient for IVIG/SCIG

>

> Thanks colleagues for your replies...

>

> The IgG is for replacement in her case.

>

> She has apparently tolerated hizentra SC for "a while" but then also eventually developed reactions.

>

> Per mom, it seemed that the reactions would worsen gradually after some time on different individual products. Per mom, these reactions have happened at the beginning of infusions. Premeds have been adjusted and also given during infusions but "nothing seems to help"...

>

> Per mom & patient, anxiety does not seem to be a factor.

>

> I'm pretty sure that gammagard low IgA was already tried, but will confirm with her previous immunologist, and perhaps try to revisit this.

>

> Maybe another subcutaneous product would work.

>

> I haven't seen someone react to every single Ig product tried before including subcutaneous.

>

> I'm going by history here, as I haven't yet managed this patient's replacement therapy...

>

> Will keep you all posted, and curious about other's experiences/approach.

>

> E

> ________________________________________

> From: John Ziegler [j.ziegler at unsw.edu.au]

> Sent: Tuesday, November 05, 2013 6:17 PM

> To: CIS-PIDD

> Subject: RE:[cis-pidd] challenging patient for IVIG/SCIG

>

> Dear Elena

>

> Presumably this is a replacement indication. Subcut sounds to be the way to go.

>

> John

>

>

>

> Professor John B. Ziegler

> School of Women's & Children's Health, University of NSW

> C/o Department of Immunology & Infectious Diseases

> Sydney Children's Hospital

> High St., Randwick NSW 2031

> Australia

> T: (02) 93821515

> F: + 61 + 2 93821580

> E: j.ziegler at unsw.edu.au<mailto:j.ziegler at unsw.edu.au>

>

>

>

> -----Original Message-----

> From: Perez, Elena [mailto:e.perez13 at med.miami.edu]<mailto:[mailto:e.perez13 at med.miami.edu]>

> Sent: Wednesday, 6 November 2013 4:09 AM

> To: CIS-PIDD

> Subject: [cis-pidd] challenging patient for IVIG/SCIG

>

> Dear All,

>

> Anyone have strategies beyond the normal premedication and the usual routine, etc for patients who fail multiple IVIG products for signs/sx of anaphylaxis?

>

> The patient is a cardiac transplant patient who is almost 13yo, chronic immunosuppression, very low T cells, normal B cells. Reactions reportedly happen within minutes of infusion, and involve increased heart rate, swelling of face, "glassy eyed" pale, and itchy throat, and feels that she will pass out.

>

> I am seeing her now for the first time, after she has failed at least 5 therapies (adjusting hydrocortisone, solumedrol, antihistamines before and during infusion etc.) under the care of outside immunologist.

>

>

>

> She often tolerated 4-5x then with subsequent infusions becomes sick and pale, HR increased,

>

> SC Hizentra -- tolerated for a while then started with signs of allergy.

>

> Gammagard and Hizentra: glazed look, pale, itchy throat, swelling of eye within one two minutes. VSS HR went up a little. She had been on hizentra for a while and reactions kept mounting. Despite benadryl and hct up dosing.

>

> Privigen--pale, lower back pain. Ran over 36hours.

>

>

>

> I was thinking to try Octagam (no volume issues), in house OR try small doses of subcutaneous daily.

>

>

>

> Appreciate others experience with difficult infusion reactions or patients with allergies to multiple products. In my experience this is very rare.... and usually solved by switching products or usual premeds. She definitely needs the therapy it is just a question of how to give it.

>

>

>

> Thanks!

>

> Elena

>

> ---

> The CIS-PIDD listserv is supported by the Clinical Immunology Society

> The science & practice of human immunology

>

> P: +1.414.224.8095

> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

>

> Not a member of CIS? Please visit www.clinimmsoc.org<http://www.clinimmsoc.org> to join!

>

> You are currently subscribed to cis-pidd as: j.ziegler at unsw.edu.au<mailto:j.ziegler at unsw.edu.au>.

> To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824805.15fcb3e73d1784a002f4bfafdad280c5&n=T&l=cis-pidd&o=44075864

> or send a blank email to leave-44075864-183824805.15fcb3e73d1784a002f4bfafdad280c5 at lists.clinimmsoc.org<mailto:leave-44075864-183824805.15fcb3e73d1784a002f4bfafdad280c5 at lists.clinimmsoc.org>

>

> ---

> The CIS-PIDD listserv is supported by the Clinical Immunology Society

> The science & practice of human immunology

>

> P: +1.414.224.8095

> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

>

> Not a member of CIS? Please visit www.clinimmsoc.org<http://www.clinimmsoc.org> to join!

>

> You are currently subscribed to cis-pidd as: e.perez13 at med.miami.edu<mailto:e.perez13 at med.miami.edu>.

> To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824713.e16dcd8ee7e2256466cc7c866f3622a7&n=T&l=cis-pidd&o=44078332

> or send a blank email to leave-44078332-183824713.e16dcd8ee7e2256466cc7c866f3622a7 at lists.clinimmsoc.org<mailto:leave-44078332-183824713.e16dcd8ee7e2256466cc7c866f3622a7 at lists.clinimmsoc.org>

>

> ---

> The CIS-PIDD listserv is supported by the Clinical Immunology Society

> The science & practice of human immunology

>

> P: +1.414.224.8095

> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

>

> Not a member of CIS? Please visit www.clinimmsoc.org<http://www.clinimmsoc.org> to join!

>

> You are currently subscribed to cis-pidd as: chinej20 at hotmail.com<mailto:chinej20 at hotmail.com>.

> To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824420.44a1efe34c59bc95780b9f7b51291cb5&n=T&l=cis-pidd&o=44078804

> or send a blank email to leave-44078804-183824420.44a1efe34c59bc95780b9f7b51291cb5 at lists.clinimmsoc.org<mailto:leave-44078804-183824420.44a1efe34c59bc95780b9f7b51291cb5 at lists.clinimmsoc.org>

---
The CIS-PIDD listserv is supported by:
[http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>

Not a member of CIS? Please visit www.clinimmsoc.org<https://cis.execinc.com/edibo/Signup> to join!
You are currently subscribed to cis-pidd as: maite.delamorena at utsouthwestern.edu<mailto:maite.delamorena at utsouthwestern.edu>.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183824444.7bb99a9770361b164a9b42c33af3da7f&n=T&l=cis-pidd&o=44079011

________________________________

UT Southwestern Medical Center
The future of medicine, today.

---
The CIS-PIDD listserv is supported by the 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!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=44084042
or send a blank email to leave-44084042-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/pipermail/pagid/attachments/20131107/186357cf/attachment.htm>