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

[Perez, Elena]

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



-----Original Message-----
From: Perez, Elena [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

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