Common Variable Immune Deficiency

Sam is our middle child and fits many of the middle child stereotypes.  

• Family clown
• Master Negotiator
• Independent
• Risk Taker

He is our family's mover and shaker.  If things get boring, count on Sam to stir up some fun, aka trouble and mischief.

He also is constantly on the go.  This boy never stops.  He loves soccer and has great natural talent. He loves the outdoors, science projects, building things, Legos, air soft guns, and anything that allows him to be on the go.  

So a few years ago we were shocked when he told us he was too tired to go to soccer practice.  A few days later he asked to be pulled from a game because he was tired.  Then came the day when he slept, all day.  I mean all day.  No breakfast.  He slept through lunch.  He woke long enough to have a snack and he returned to bed.  Not normal for Sam.  Not normal for any healthy child.

Sam asleep during the day.  A sight we never saw when he was younger.

Off the the doctor's office we went.  We ran a series of blood tests which told us he was recovering from mono. That eased my worry and we allowed him lots of rest and good food.

Sam recovered for a while.  But the cycle started again. During the next 2 years, Sam dropped out of soccer, quit playing with friends after school, had mono twice, and began having migraines.  

We are blessed to have a fabulous pediatrician (who adopted special needs children as well).  He trusted my instincts that something was wrong and continued to test Sam.  The tests came back that Sam's IGG levels were a little low. So our doctor sent us to an immunologist.

After more testing, Sam was diagnosed with Common Variable Immune Deficiency.  CVID is a disorder that impairs the immune system which means Sam is highly susceptible to infections.

Treatments include a medication called Hizentra.  IGG levels had to drop pretty low for the insurance company to approve the treatment.  And the medication is expensive enough (thousands a month), that we needed insurance.  

So we waited 1 year for his levels to drop and I worried about his health.

Finally, we started infusions of Hizentra about 6 months ago.  I get to administer them to Sam at home which includes placing 4 small needles in his body so the medication can slowly be absorbed. Treatments usually take 3-4 hours, including 1 hour of numbing cream before placing the needles. The treatments are painful and sometimes cause severe headaches.

All the medication, supplies and equipment can be overwhelming.

Preparing for the injections with the numbing cream.  A must!!!!

Sam prefers leg sites, although we prep other areas as well.  Just in case!

Injection sites leave large bumps for a day or two. 

Our first infusion required six sites so the body could get used to the medication.

Last week I had placed 2 needles, both of which were painful when Sam refused to continue.

Sam-Take them out!  I'm done. (Tears streaming down his face)

Me-I can't Sam.  I have already drawn the medication.  

Sam-Nope, I'm done!  Get them OUT.  I am going to rip them out.

After about 10 minutes of this I persuaded him to put Lidocaine back on the spots we were planning on using.  20 minutes later I was able to place the needles and continue with the injection.

Although we were able to continue, I had to leave the room afterward and go have a quiet cry in my room.

Sam doesn't like the injections.  He hates the headaches he gets.  He feels like he has enough problems from being exposed to meth during pregnancy.  It's not fair.  And I have to agree that's its not fair.  

I reached out to a friend who's daughter has similar issues.  She was able to give some good advice and told us about a conference for families dealing with this issue.  I want to go.  I want Sam to see he isn't the only one. That others understand what he is experiencing.

Hope is difficult but not impossible right now.  I am grateful for new medications that are working to save his life.  We are blessed! I would love to hear what those of you with a chronically ill child do to help buoy them up.  

We have hope.