Wednesday, August 20, 2014


Fiona's antibiotic is still backordered with intermittent supply. We were able to get a bottle last week. It's a different formula than we've had before and she hates it. She got used to the thin grape liquid, now it's a thick cherry liquid. I mix it with an equal amount of water before I give it to her or she won't drink it.

She seems to be doing better this week. We reduced her Hizentra dose by a gram. I'm worried about what that will do to her IGG level, but I'm glad she feels better. She's sleeping better too, which helps us both.

I'm starting to really feel like now is the time to figure out if she would benefit from a bone marrow transplant. If the problem is in her bone marrow then it would fix her problem. If the problem is in her thymus then a bone marrow transplant won't help. Her thymus appeared normal on a CT scan. I'm going to ask for a thymus biopsy to rule out a problem with her thymus. It's so frustrating to know something is wrong and not have any answer or solution.

Wednesday, August 13, 2014

no antibiotics

Our pharmacy wasn't able to get Fiona's antibiotic. According to the FDA it was supposed to be available after the 1st week of August. We don't know when it will be available again.

My kindle is working again! Fiona couldn't be more thrilled. I had to restore it to the factory settings, so I lost everything saved on it, but I'm happy that it's working in time for her next treatment. It's a great distraction tool.

We are giving Fiona ibuprofen to see if it helps calm the fi-beast. She seems to be doing better this morning. We're also dropping her dose of hizentra by 1 gram. Lowering her dose is scary, but it will let us see if she gets sick. She just fell asleep in my lap, so I think she's feeling a little better.

Monday, August 11, 2014

Fi Beast

Every few months Fiona has a treatment or set of treatments that don't agree with her. The past two treatments haven't agreed with her. She is really wild, restless and cranky, which we attribute to a bad headache. We are giving her tylenol and benadryl, but they aren't really helping. Anything that doesn't go her way causes an unpleasant screaming episode. Parenting a sick kid is hard, and primary immune deficiencies suck, especially for the patient. In the past we've discussed the possibility of aseptic meningitis, or serum sickness.

Today during treatment my kindle, which is a great distraction tool for Fiona stopped working properly. I called Amazon, and they suggested resetting it to factory defaults. It had to charge in order to reset, so I'm keeping my fingers crossed that it works. It's past the warranty so they offered me a discount to replace it. We can't afford to replace it, even with the discount.

antibiotic update

The company that manufactures Fiona's antibiotic now has an intermittent supply, which is much better than no supply. Our pharmacy is ordering it for us. She hates the compounded liquid that our pharmacy made for her. I'm sure it isn't yummy. Hopefully we will be able to get some for next weekend.

This afternoon she'll have her weekly treatment. We read a story last night about what her treatment does for her. She's been exploring her medical supplies. She loves bandaids in every form. I caught her putting a big clear IV patch on her tummy last night. She said it made her feel better.

For anyone who wants to do something to help us and can't donate to the fundraiser, please donate blood or plasma. They'll pay you to donate plasma. We are always concerned when they talk about shortages at blood banks, because eventually they will affect us.

Lots of people are concerned about the outbreak of Ebola reaching our country. I read an article last week that mentioned that the Americans who returned with Ebola were given an experimental treatment involving antibodies from mice exposed to parts of the Ebola virus. The reason this makes sense as a treatment is that once antibodies are exposed to a virus, they remember how to fight the virus. This is called adaptive immunity. Adaptive immunity is the reason that vaccines work. Fiona's body doesn't produce enough antibodies to fight infections. It makes me wonder if they took the igg from a patient who recovered from Ebola if it would be a better treatment?

Thursday, August 7, 2014

new needles

Before Fiona's treatment on Monday she had her igg tested. The doctor wanted to see what her low number, which they call a trough was. Her trough igg is 714, which is good, but much lower than I thought it would be. When they drew labs in Cincinnati her igg was 924, two days after her treatment. Giving her subcutaneous treatments is supposed to keep her levels more even. A fall of 310 seems uneven to me.

We got Fiona's new needles and I used them for the first time on Monday. I'll take some pictures, they are pretty amazing. The metal needle pulls out and leaves a flexible tube. She was much more comfortable, and was able to move with no pain. I hit a blood vessel on one of her sites. It didn't bleed when I pulled back on the syringe, but later there was blood in the tube. She has a bruise now.

This week our older kids went back to school. This week has been crazy, but is starting to settle down. Fiona has been a ball of energy every day. I think she is reacting to the batch of hizentra she got on Monday. She's being super naughty. I've started making sure she's in the same room I am so I can keep an eye on her.

Friday, August 1, 2014

going to a doctor?

Every time we walk into an office building Fiona asks if we are going to a doctor. Today we walked into a building with big glass doors and she asked if it was a hospital.

Her t-cell function tests are back. They aren't horrible, but they aren't great. Her resistance to candida (yeast) is pretty low again. Her resistance to tetanus is low too. Her t-cell stimulation tests are normal. I think I'm seeing a pattern of low t-cells and high stimulation, and improved, but still low t-cells and low stimulation. I'm making a graph to see if I'm right.

We got our package from BioRx today, and it has the needles I want to try. I'm a little nervous to try them the first time, so I've been watching youtube videos. It looks pretty easy.