Fiona's cape

A few months ago I requested a cape for Fiona from Capes4Heroes. Her cape arrived a few weeks ago, but with all of our crazy I'm just getting pictures posted. Fiona loves her cape. It's beautiful green satin, with flannel inside. A huge thanks to Capes4Heroes, and a generous sponsor!

sick girl

Poor sick girl. We went to the doctor on Thursday, and the ER on Friday. She's had a steroid and an antibiotic. They did labs and a chest xray. One of the fun things about an immune deficiency is that they do a viral test when she gets a cold. It's not all that helpful to know what the virus is, but it's interesting to find out.

I got some of her labs back today. Her white cells are normal, which is high for her. Her lymphocyte count is 1.0 (some labs will list it as 1,000), but the result came from a different lab than we usually use, so I'm not sure how to interpret it. Her IGG is down 350 from September's labs. With replacement she's at a normal level still, but I think we want to see it higher.

We should know more about how she's recovering tomorrow when the steroid has worn off. Today she's congested, has a fever, is cranky, and sleepy. She's loosing her voice, and probably has a sore throat. This is what we dread. There's no way of knowing if a minor illness will hit her hard, or if her replacement antibodies will help her fight it.

Being on an antibiotic has 2 disadvantages. They didn't see a clear source of bacterial infection, so the antibiotic may not help. It may increase her risk of developing an antibiotic resistant infection. It also puts her at risk of getting thrush, or yeast. Because of her low resistance the yeast is more likely to become systemic, which means a few months of diflucan.

Halloween

Fiona is trying to decide what to be for Halloween. She was going to be a blue princess, then a green robot, then a blue robot. We'll see.

This has been a crazy week. I had surgery to take out some scar tissue and adhesions. Fiona has been a trooper. We hang out and watch cartoons, take naps and read books.

Her treatments keep getting better. Last week we used 3 sites, and she still did really well. She has a little rash around her nose that I'm keeping an eye on. Other than the rash she's still healthy. At her checkup on Monday the doctor said she looks great. She's growing really well.

another great treatment

Today's treatment was great. She was watching Dora on the kindle and didn't even notice when I poked her. She was really interested in seeing what the tubes that are left behind after we pull out the needles looked like. She said, "They're soft."

We still don't have all of our results back from Cincinnati yet. Usually we see them all within 2 weeks. I'm going to call this week to get the rest.

Fiona is doing really well. She's not sick, which is a huge blessing. We are isolating again. With the weather turning colder, cold and flu season will be starting. It's been almost a year since she last in the hospital.

We are applying for the Hizentra copay program, which will pay for up to $4,000 of Fiona's treatments. It will be amazing if it works out. That's half of our out of pocket for 2015!

today's treatment

Today's treatment went really well. I premedicated with Benadryl and Tylenol, and used her numbing cream. I was in a hurry afterwards to get to a meeting so I just left her quick set needles in. When I took them out an hour later they didn't leak at all, usually a little bit of Hizentra leaks out. Fiona is doing really well, I think she's over her cold, and it was pretty mild.

Cincinnati, labs, and genetic testing

When I get Fiona's labs back I feel like I'm opening a gift. Sometimes it's awesome, sometimes it's lame. I'm pretty happy with her labs from yesterday so far. Her lymphocyte counts are all a little higher, not normal, but higher than two months ago. She tested positive for rhinovirus, but negative for the major viruses, cytomegalovirus (CMV), Epstein Barr (EBV), and adenovirus. They brought in the ultrasound IV team to draw her blood. It was unpleasant, but quick, and they only had to stick her once.

They drew blood for genetic testing yesterday. We were able to talk to a geneticist, and she was great about answering our questions. She said that it's likely that Fiona has multiple genetic mutations working together to cause the problem. We know that her deficiency has a genetic cause, we just don't know what it is. Only 25% of people tested find a result, but as they learn what specific genes do, they may figure it out. The results will be back in 9-12 months. I wish we had know how long the wait was. I would have pushed to start testing a year ago. They are waiting to start the testing until our insurance preauthorizes it.

We had a chance to talk to the doctor and ask lots of questions. We're frustrated that we are moving so slowly. It would be nice if there was a test that would tell us where Fiona's t-cell problem originates. It could come from her bone marrow, or her thymus. If it comes from her thymus a bone marrow transplant wouldn't help. We know that her thymus looks good on a CT scan. We have tested for thymus related conditions, and they were all normal.

Today we are all exhausted. Yesterday was a long day. I think the virus is finally catching up to Fiona, she's taking it easy this morning. Hopefully she'll take a nap this afternoon.

Genetic testing

Our insurance company says they will pay for Fiona's genetic testing as long as we get precertification. Because of some recent issues with our insurance I'm nervous, but excited. I was expecting to have to fight to get testing covered. It seems a little too easy.

We go to Cincinnati next Wednesday. We'll be drawing blood for the genetic tests. Christian and I will also be tested. It was mentioned at our last visit that we need to be prepared that we may find things we aren't looking for. Today I think that may be a good thing, but if they do find something we don't expect I may change my mind about that.

Fiona has diarrhea again. A bacteria called Shigella is going around our middle school. It causes bloody diarrhea and a fever. So far she doesn't have a fever (except for a low grade fever she's had for weeks), or blood.

Her treatments are going really well. I think it helps her that I can show her that I pull the metal needle out. When I say, "the needle is gone" she seems relieved.

We are worried about EV 68. As of yesterday there are confirmed cases in Northern Indiana. In normal kids it manifests as a cold, but in kids with asthma or immune deficiencies it's worse. We are starting to isolate Fiona. Because this isn't a new virus, the donated blood that her treatment is made of may have antibodies against it, but it was pretty rare before 2008.