what is a treatment like?

As I give her today's treatment I'll try to describe it for you. An hour before her treatment we put on some numbing cream and cover it with a clear plastic bandage, like what they cover an IV with. Then I try to distract her for an hour while the cream works. Right now she is poking the cream and saying, "gushy, squishy, gushy."

While I'm trying to distract her I get the supplies ready for her infusion. We use a plastic mat, pump, bag, neck cord, 20ml syringe, y tubing, transfer spike, journal, gauze pads, alcohol prep pads, paper tape, clear bandages, coban wrap, and hizentra.

The first thing I do is to lay down the mat and wipe it with an alcohol pad. Then I pop the tops of the hizentra bottles and wipe the tops of the bottles. Next I stick the transfer spikes into the bottles, which are covered with a flexible membrane. One at a time I draw the liquid into the syringe making sure to get as much liquid as possible. After that I attach the y tube to the syringe and prime the tubing by pushing the liquid almost to the end of the tubes. Then I wipe the cream off and clean the site with an alcohol pad. The ends of the y tube have a small needle on them, and a tab on each, side to hold onto. I stick the needles in, taping each one down with a clear bandage and pull back on the syringe to make sure no blood comes out. If no blood comes out I unscrew the plunger from the syringe and attach the pump. Then I start the pump. I also have to keep a journal of her reactions, lot numbers for the hizentra, and start and end times. Sometimes we cover the infusion sites with coban, which is a self adhering elastic wrap, so she doesn't touch the sites. Each infusion takes an hour. We try to keep her sitting so the needles don't move. When the infusion is over the pump beeps, I take out the needles and put pressure on the sites so they don't leak. I cut the needles off the tubing, put the needles in our sharps container, and throw everything else out.

We think she is developing an allergy to the clear bandages. We found some new tape that is better than paper tape and more gentle on her skin, but we need the clear bandages so we can see her reactions and to keep the numbing cream in place.

Her usual reaction to the hizentra is mild, think of an immunization, but with a teaspoon and a half instead of a small amount. She gets really big goose eggs. As her dose increases we will add more sites to spread out the liquid. Her current pump is electronic and will only allow for one increase in dose, then we move to a spring driven pump.

Today was the first treatment I did without another adult to hold her arms. She did really well. I think she is getting used to the idea, but is still nervous when I stick her the first time. She doesn't really feel it, so the second stick is easier. We started her infusion at 11:50, and now, half an hour later, she is up and playing.

low grade fever

Fiona has had a low grade fever for a few weeks now. I'm starting to wonder if it's a side effect of her antibiotic. We've checked to make sure she doesn't have any bacterial or viral infections, and so far everything is negative.

Tomorrow is her treatment day. Treatment days are exhausting. I think I'll try to do her treatment while the girls are at school. I'm glad our community is postponing trick or treating. I think it would be too much for her.

The next few weeks are going to be busy with doctor's appointments. Next week Fiona will see her doctors here and in Cincinnati., and the next week is her two year checkup. I'm going to push for more testing, hopefully the genetic testing, before the end of the year. Since we've met our out of pocket I want to get some of the more expensive tests done while our insurance is covering 100%.

I'm really looking forward to seeing what her igg level is next week. Hizentra has introduced a formula that would let us do treatments every two weeks. I'm going to ask the doctor if that is an option for Fiona. I'm also hoping that she weighs more than 30 pounds. If she does she can get an epipen instead of the glass vial and syringe of epinephrine we have now.

my hands

This is more of a whiny post about myself than anything about Fiona, so feel free to ignore it. The specialists have told us that the best way to keep her healthy is to wash our hands often. I think I wash my hands at least 20 times a day. I also use a lot of hand sanitizer when I run errands. My hands feel like sandpaper. At the end of the day it's hard to straighten my fingers because my skin is so dried out. If any of you have great advice about dry hands please comment. Thanks!

treatment cost

We were able to look up a claim from our insurance company for Fiona's medicine. For a four week supply it's about $1,600. The price will change as her dose increases. It looks like it will be billed as a prescription, but it will go towards our deductible and out of pocket. I think our insurance company is adding a specialty tier, but I'm not sure what that will mean for us. It would be nice if the insurance company gave us a rep like our medical supply company does. Being able to talk to someone who really knows our situation and can give us specific details would make a huge difference in our stress levels.

eating like a horse

In the two days after treatments Fiona gets hungry. Really, really hungry. After her first IVIG she came home and ate almost as much as Christian and me. After a particularly memorable treatment she shoved handfuls of chicken into her mouth as fast as I could cut bite sized pieces. She seems to love protein and chocolate lately. Before she was diagnosed she loved fruits and vegetables, now it's hard to get her to eat them. I wonder if this is just a normal toddler phase or if this is her new way of eating?

Settling down

Really not much to update, which is great! Fiona has had some mild fevers, but they don't last more than a few hours. She goes back to Cincinnati in two weeks.

Christian gave Fiona her infusion yesterday. He did a great job. I'm trying to figure out a routine for infusion days. So far I haven't been successful. It's an exhausting process, and by the time it's over my house looks like a tornado passed through. I hope it will get better once the doctors let us change her treatment day. It would be nice to give her infusion when the older kids are at school, but I'm not sure if I can get the lines started without someone to hold her hands.

I had a great conversation with another mom last week. We talked about how as moms we can be judgemental of each other. I am embarrassed to admit that before James and Fiona I judged other moms. I used to wonder why some moms couldn't control their kids. I've learned that motherhood isn't about control, it's about working with what you're blessed with.

Fiona's energy level and attitude the day after her treatment are at best wild. She was in Tasmanian devil mode at our church trunk or treat tonight. It's hard to watch her energy come and go so quickly. I find myself wondering if her tantrums are because of headaches and joint pain that are side effects of her treatments. Because she looks so healthy and normal I forget how hard these treatments are on her body.

Pricing and pharmaceutical assistance programs

I still don't know how much Fiona's treatments cost. I've heard wild estimates, but I'm still waiting to hear back from our supplier. Not being able to plan for the cost is stressful. The lowest estimate that I've heard is $1,200 a month, and the highest is $60,000.

Most people that we talk to about the expense of Hizentra say that we should check with the manufacturer, Behring to see if they offer an assistance program. They do, but only for people who are uninsured, or whose insurance companies don't cover the drug. I found a nonprofit that helps with copays, but their primary immune deficiency program is temporarily full.

I know this will be difficult. I know it will be a struggle to pay for treatments. I know that we will always have to fight for Fiona, but she's worth it! How many parents get to raise a unicorn?