[CIS PIDD] [cis-pidd] VS: Patient question

[Seppänen Mikko]

In my clinic in Finland, I see both alternatives in CVID (PAGID/ESID possible/probable) patients, even SADs. 
A) they are the heathiest of the family once on good troughs, mostly on SCIg
B) once they get small children, they start an endless journey of especially sinus problems (I cannot recall any pneumonias/systemic infections if no problems in reaching decent troughs). IgG does not reach airways that much (nFcR:IgG1+3,not IgG2 and no sIgM, IgA?), some of them already have chronically damaged epithelium, might have structural problems, maybe even other genetic polymorphisms.Plus baby may not sleep during the night, chronically sleep-deprived, more lymphopenic etc.

Bs are among the least satisfied, As the most, but how to predict what happens?

And anyway most of them (whether As, Bs or in betweens) tend to get their noros, enteros as more-prolonged symptomatic infections, which is understandable. 

I tell them that CVID is no reason not to get kids, if they want them, and afterwards bad memories of sinus problems disappear, but good memories of their childrens' childhood linger.. and that is what exactly happens

IgAs with robust responses, now that is a clinically mixed lot...

mikko


________________________________________
Lähettäjä: Mel.Berger at cslbehring.com [Mel.Berger at cslbehring.com]
Lähetetty: 5. maaliskuuta 2013 7:18
Vastaanottaja: CIS-PIDD
Aihe: RE:[cis-pidd] Patient question

You may want to increase his dose/IgG level if his incidence of infectious starts increasing if/when the kids are in day care. His risk may be decreased if the kidsare kept at home as long as possible, bui all of that depends on his baseline exposure to infections prevalent in the commnity witheach season.

Mel

________________________________
From: Church, Joseph [JChurch at chla.usc.edu]
Sent: Monday, March 04, 2013 9:57 PM
To: CIS-PIDD
Subject: [cis-pidd] Patient question

Colleagues:

I saw a young adult with CVID today.  He has just gotten engaged and asked the following:  when he has children how much of an infectious risk do they present to him?

I guess they present an increased risk for viral respiratory infections, but between appropriate immunizations of the kids and his excellent adherance with SCIG infusions he should be at no increased risk for life-threatening infections.

Any other thoughts?

Joe Church
Children's Hospital Los Angeles

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