new tests

Tomorrow we should get the package for the next round of blood tests for Fiona. After we get the results of these tests, if they are normal, we are discussing sequencing her entire genome. In order to do that they have to sequence my genes and Christian's genes. Genome sequencing costs about $15,000, and about 40% of insurance companies will pay for it. I was surprised to learn that even if we test all of her genes there are some genetic problems that won't show up.

I learned something funny today. There is a rating scale called the Karnofsky/Lansky scale. 100% means that you are totally healthy. 0% means that you are dead (do they actually rate you when you are dead?). I'm not sure why I thought it was so funny. Fiona is 100%!

Today I'm feeling a little discouraged that we haven't made any progress in diagnosis, but hopeful that we are starting some new testing. Waiting is hard.

Domed hizentra lid

I like to think there are two purposes to this blog, keeping friends and family informed, and being a resource to other moms of immune deficient kids. This post is for resource purposes. When I started Fiona's treatment on Sunday I popped all of the caps off the hizentra bottles and started to wipe them with alcohol. Then I noticed that one of them looked different. I called the nurse hotline and they told me not to use the bottle, send it back, and they will send us a new one. I wanted to keep pictures just in case, then I thought I should share them with you. This is about $130 worth of hizentra. I'm glad they're replacing it!

The bottom of the lid should be smooth, not rippled like this.

The lines you can see in the metal part of the lid are from the cap that covers the lid. The metal part pushed into the cap so hard that it embossed these lines. The lid should be smooth.

Great treatment

Yesterday's treatment was so easy for me. We are back to our small pump, and simple set up. Fiona did great. She said ouch a few times, but didn't really complain. I think she's developing a reaction to the new clear bandages, IV3000, we use over her needles. I've started to notice that when we use her belly she has a more dramatic swelling reaction, as well as a reaction to lidocaine and tape. It also seems like the weeks we user her belly are the weeks that she complains of pain. I think I'm going to premedicate with acetaminophen those weeks. I'm going to try switching back to benadryl instead of her anti-anxiety medicine. I would rather have her sleepy instead of falling down and grouchy later.

I've noticed an increase in her abundance of energy right after her treatment and the next day, which is today. So far today she's had breakfast, asked for more food an hour later, reluctantly took her antibiotic, chased the dog, dropped James off at preschool with me, watched 2 episodes of Diego, washed her hands 4 times, pumped soap all over my bathroom floor, played a preschool game on the kindle 5 times, pretended to rescue a giraffe, asked for more Diego, asked for help with her game, walked away from Diego and her game, took the mango I was eating, put a spoon in it and walked away from it, and asked to be put in pajamas. It's only 10:30. I'm not sure I can keep up with her today! I'm glad she feels good though.

After weeks of eagerly taking her antibiotic she's decided that she doesn't like it any more. Now I have to fight and hold her down, and keep her from spitting it out. The doctor said he wants her to stay on it until the end of April. Then she'll only take her weekend antibiotic.

she knows

Yesterday Fiona brought me the blue case that holds her pump. I told her that her treatment is on Sunday. She told me that she wanted her treatment now, followed by, "I said so!". I'm amazed that even though her treatment is painful she still asks for it because she knows it makes her feel better. She has really dark circles under her eyes, I wonder if she's fighting something.

What no one told me

When you have a sick child no one tells you how hard it will be. No one tells you how hard you will have to fight to get things for your child,things that she needs, but things that no parent ever wants. They don't tell you how hard it is to do what's best for your baby, even when it hurts her. There is nothing that can prepare you for the overwhelming sadness that hits at inconvenient times, like when you almost burst into tears at the grocery store. I'm amazed at how tired I am some days. I'm surprised by the frustration of trying so hard convince people, doctors, nurses, pharmacists, and strangers that they need to help her. I've become a germaphobe, not because I am afraid of illness, but because of what I'm afraid of what it will do to Fiona. Sometimes I wish for an easy solution. I'm sad and discouraged when tests come back normal. I don't want her to be sick, I just want to find an answer, a solution, anything. I've become an advocate. I ask millions of questions, and research everything. I can't imagine life without her. She's worth it all.

Insurance update

We found a way to go back to Biorx as our specialty pharmacy. After a long drawn out fight I feel like we won. Thanks to Melissa at Biorx, and my husband's employer's HR department we finally figured it out. It will be such a relief to go back to our normal treatments and routines.

Fiona continues to be healthy. We feel very blessed that she isn't sick. Finding other moms of immune deficient kids has shown me what a miracle her health is. I never understood what the doctors meant when they told us that she should be very sick, until I read about other kids like her.

changes

We are trying to get the right pump for Fiona this week. Accredo called a few days ago and I went over all of the options with a pharmacist. After going through everything he told me that they didn't have any other options, and that none of them would be good for Fiona. Yesterday the manager of the pharmacists called and told me that she thought they could get the small pump for Fiona, but she had to look into it. She called back and said that they have it and will be sending it to us. I'm not holding my breath. A member of a support group for immune deficiencies told me that Accredo was supposed to send her the same pump, but once the head of nursing for her area found out the order was canceled along with the order for her medicine. They don't offer training for the pump we want, which is fine, because we used it for a few months.

We are in touch with someone from HR, she is trying to work everything out. Our three main complaints about Accredo are the pump, the separate copays for bottle sizes, and their lack of payment plans. One of their billing reps told me that until an account is paid in full they won't send more medicine. We are fortunate that we could pay for the first month of Fiona's medicine, which was $2,700. We hit our deductible last month, so until we reach our out of pocket we have a $150 copay. The bottle size issue will also be resolved soon. Accredo charges a copay for each bottle size. Right now we are getting 20, 1 gram bottles for $150, which means I have to draw from 5 bottles each week. We used to get a 1 gram, and a 4 gram bottle for each week, and I only had to draw 2 bottles each week. If we wanted 4, 1 gram bottles and 4, 4 gram bottles we would have to pay $300 per month. Ridiculous!

Fiona is still healthy. We saw our local doctor yesterday. We will be getting the stuff to draw labs for the next set of tests this week. Because they are unusual tests they need to go to research labs, which will be a challenge. First Cincinnati has to figure out where to send them, we have to have them drawn and sent to the labs, then Cincinnati has to figure out how to get the results.