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.

making a plan

This weekend Christian and I are fasting and praying. I'm tired of our no diagnosis limbo. Everything I read says that transplanting early is better than waiting. So far she doesn't have any major illness, but how long will it be before she does. I don't want to risk her going into a transplant with a virus. On Monday I'm going to call Cincinnati to set up an appointment to present our plan. I'm going to ask for a thymus biopsy so we can see if that is where the problem is. If her thymus looks normal I'm going to push for a transplant. According to the Immune Deficiency Foundation, "Fortunately, the success rate of stem cell transplantation, particularly for patients without an HLA-matched sibling donor, has improved substantially over the past few years so that the risk of this treatment has become much more acceptable for less severely affected individuals." Fiona has an HLA matched sibling, so the risk is even lower.

Yesterday the middle school sent home a letter saying that they have had a case of Shigella at the school. Shigella is a bacteria that causes a fever and diarrhea. It's not really serious, but it is resistant to the antibiotic that Fiona takes.

updates

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.

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.

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.