I've never heard of a
Primary Immunodeficiency Disease Awareness Month but we got
our first celebration underway at the hospital while mom was receiving an
intravenous immunoglobulin treatment (IVIG).
Let's back up and start from the beginning.
Mom has been diagnosed with a
primary immunodeficiency disease known as
Hypogammaglobulinemia (also known as
Common Variable Immunodeficiency, or CVI). She has
very low levels of
immunoglobulins (or antibodies) in her blood. These antibodies are what is supposed to fight off infections. Pretty much -
my mom has been living the last two years without an immune system.
No wonder she has been so sick!
CVI can cause
major health problems. When left undetected, it can cause major
organ damage through the stress of chronic infections and cause some patients to need
organ transplants. Immunodeficiency diseases can also be
fatal. We are lucky that the doctors caught this so
"early" (2 years doesn't seem
early to me but apparently, in the immunodeficiency world,
it is).
They
don't know what causes it. Some think it is
genetic. Others think it is
acquired.
(I'm pulling for acquired.) In all the research I've done the last two days I haven't been able to find a consensus. It doesn't really matter at this point how she got it. What matters is
how we are going to fix it.
Well, there is
no cure for CVI. She will
always have very low natural levels of antibodies. So to boost her immune system (or really
to give her one), she will have to receive monthly
IVIG treatments. The treatment is given as a
plasma protein replacement therapy (like she is getting blood but it is clear and full of lots of these anitbody things). This will help her maintain a normal immunoglobulin level and be able to
fight off infections like you and I do
everyday without even knowing about it. They have to administer treatments in the hospital and it takes about a 20-hour or so visit to receive the therapy and be monitored to make sure she doesn't have a reaction to the plasma.
Because the IVIG only temporarily maintains her immunoglobulin levels, she will have to receive these treatments monthly
for the rest of her life. That may seem like a hardship for some but we are
ecstatic that only one day in the hospital a month will keep my mom feeling good.
My mom returned home from the hospital a few hours ago. It takes the antibodies
24 to 48 hours to completely begin their work. Then, she should start to get better. The antibodies will start fighting away at the
bronchitis and
sinus infection and
my mom will feel good for the first time in two years! Fingers crossed!While this is very rare
(1 in 50,000, I believe) I do want to take this
tiny platform and let you know about this disease. If you or someone you know is
constantly diagnosed with
sinus infections,
bronchial infections,
gastrointestinal problems, and/or is diagnosed with
adult onset asthma -
please tell them about the possibility of a
primary immunodeficiency disease.
Too many people go years without knowing what is wrong with them and
spend lots of money on medical care that just addresses the
symptoms.
Thank you all for your warm thoughts. They are much appreciated!
Keep them up as my mother's recovery continues. Hopefully we will have her back out on the tennis court in no time.
She is already clambering about playing tennis in the Fall leagues.And a big thank you to
Dr. Spera.
I still haven't met him but he is our angel!
