Awful labwork. but clinically ok?

[Guest guest]

Hi everyone! I mostly lurk for the good info I get here so most of you may not

know who we are. I have 3 boys who have immunodeficiency secondary to their

Mitochondrial disease. My middle son was on IVIG for almost a year and we had

to trial him off as my dh lost his job and we could only afford to pay for the

COBRA, but didn’t have any money left to actually use it. He has done

surprisingly well since trialing off and continues to hold his own illness-wise.

My oldest son has recently had issues with his titers as well and has become a

non-responder to his pneumo vaxes like his younger brother. My youngest has had

low Ig levels, but has managed to hold on to enough of his titers to where we

were not overly concerned. The immuno has been pushing us to start sub-q for

the older 2 boys due to their pneumo titers, but we have held off because they

have clinically been doing well enough…no life-threatening illnesses and they

bounce back in a normal time frame and they have not had illness upon illness

upon illness either. They have had a handful of whatever is going around each

sick season...not the healthiest kids in their class, but certainly not the

sickest either. The other factor that has been holding us back is the cost. We

don’t have Medicaid and since the older boys already have feeding tubes and O2

at night and everything else, the 20% OOP after their deductibles are killing us

financially.

That brings me to today. We finally got our immunology labs back from their

appt in Dec. They had the normal labs drawn…the Ig levels were pretty run of

the mill for them. Their pneumo titers were surprising. My middle son (who was

on IVIG before) had only 1 over 2.0 and considered protective…that is normal

for him. But, SEVEN were measured at <0.3, which leads me to believe those were

undetectable. My oldest was only protective to one of 14 (down from 4 of 14 at

the previous draw) and my youngest, who has responded to vaxes fairly well up

until now only has protective levels to 4 of 14 (down from 8 of 14 at the

previous draw). The older 2 used to respond well enough, but they eventually

lost the ability to mount a response to the Pneumovax and are considered

non-responders now. In seeing these results, I am feeling as though my youngest

is following his brothers with their immunological decline.

My question is, with the labs they have now, would you continue to see how they

do since they are doing ok clinically? Or would you start them on infusions

given the labwork? Quite frankly, I was totally ok with letting them be until I

saw how low my middle son’s are. Half are not even able to be accurately

measured because they are so low and that scares me. Just looking for some

feedback as to what to do now…before we talk to the immuno and see what she

thinks. I know she is all for them going on treatments, but I am not sure we

have a leg left to stand on to put her off any longer…and I am not sure at

this point that we should. Thanks for any insight you can share with me.

Melody, mom to 3 little boys with immune deficiency secondary to Mito