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.

pump comparison

I just sent all of this to Christian to show his HR department, so I thought as long as I was working on it, I would share it with you. Hopefully this will be enough to get someone's attention and let us go back to what was comfortable and worked for us.

This is our current pump, infusion bag, cassette tubing, and needleset. The coiled tubing is 96 inches long and holds 5 mls of fluid. This is the second pump Accredo sent to us.

This is the pump, syringe, and needleset from Biorx. The blue cord hangs around her neck like a necklace. This set up uses the same needleset as the first picture, but no bag or cassette tubing. Inside the syringe is a plunger that is pushed up with a spring driven pusher.

This is a side by side of all three pumps. The grey pump is the first one Accredo sent. It's big and hard to manage, but very lightweight.

This is the cassette tubing over the three needleset tubes. I know it's hard to see, but the cassette tubing is almost as big as all of the needleset tubing combined.

For reference, our current pump next to a deck of cards. It's big, and very heavy.

Another reference, this is the Biorx pump, it weighs about the same as the deck of cards.

Research

I love research. Someday when life has settled down I would love to go back to school and work in a lab. Maybe in 5 years I'll get there. For all of you who love research too, I have some terms for you that describe Fiona's condition. If you have no interest in research skip to the last paragraph.:) Here they are: inverted cd3 to cd4 ratio, ideopathic cd4 lymphocytopenia, hypogammaglobulinemia, and combined immune deficiency.

Some things don't fit exactly: it's not just her cd4 cells that are low, it's all of her t-cells, she makes b-cells, there isn't really a combined immune deficiency, an inverted cd3 to cd4 ratio is the hallmark of AIDS, but she doesn't have AIDS, none of her deficiencies are due to viral suppression, she has a thymus and it seems to be normal, we've ruled out most genetic kinds of SCID, and there is no possibility that she has transient hypogammaglobulinemia of infancy as her low igg level was 109. Let me know if you come up with something or have any questions.

As much as trying to find a diagnosis is hard on me, it's much harder on Christian. He is really struggling. In an effort to pay for Fiona's medical expenses he is teaching a class at IUPUI. He works a full day and then comes home to work another 4 hours, and all weekend. Please keep him in your thoughts and prayers. We are watching our baby suffer things we never imagined possible.

Fiona's fundraiser

more tests

Cincinnati is ordering more tests. They're pretty unusually, but so is Fiona. Our coordinater has to research where to send some of them, because he hasn't seen them before. We can draw the labs here, which will save us a 6 hour round trip. As more labs come back normal I'm starting to wonder if she really is the only one to have her condition, or at least the first.

More labs have come back. Fiona's protein level is normal, but her albumin level is still low. Fiona's mitogen testing was normal. Mitogen testing means they add something to Fiona's t-cells and watch for a response. The higher the number the better the response. A good response means that her t-cells are working like they should. Her t-cells are a little higher. As with most lab tests the numbers will naturally fluctuate. Abbie and James both match Fiona for the important viruses, so either one could donate if we ever get to the point of a bone marrow transplant.

Fiona is still on tamiflu to prevent the flu, and two antibiotics to cover all of the bacterial bases. She's still healthy, she still has dark circles under her eyes. Once flu season is over we are thinking about slowly exposing her to the world again.

What's next?

We don't know how to proceed from a diagnostic standpoint. At our last appointment we discussed the possibility of Fiona having a missing or underdeveloped thymus. That would explain her low T-cell counts. I don't think that's the problem, but you never know, right? I would like to come up with a plan for lab tests. I'm going to ask Fiona's doctor for a list of what we've ruled out, and what we need to test for in the future. I feel like we've lost control and understanding of the diagnostic process.

Genetic test

The genetic test we've been waiting for since November came back today. It was normal. Most parents hope for normal test results. I'm so disappointed. Not knowing what is wrong, or how to fix it is so hard. We were so hopeful that this test would show something. If it did we could cure it. Now we're back to total uncertainty about what is causing her deficiencies. I'm taking my 15 minutes to cry now.

Great treatment for Fi!

Yesterday's treatment was the best so far for Fiona. I think we've found the right combination of medicines to give her before her treatment. She wasn't scared, and she didn't shake. It was really good for her. She was a mess yesterday afternoon, and she still is this morning, but I'll deal with it if it means a better treatment for her.

The new pump and system are very complicated and time consuming. The ridiculous thing about this pump is that the tubing that comes with it holds 5 mls of hizentra. That's one fifth of her treatment. I worked really hard to make sure that there was extra air in the infusion bag to push the fluid through the tube up to her needle set. We're going to let our insurance company know that this will not work for us. Hopefully they will let us go back to Biorx, and their tiny, wonderful pump!

Everything else is the same. Fiona is healthy and isolated. Her treatments are working. We are careful not to expose her to anything. I wash my hands a million times a day. We keep hoping that the genetic test will come back soon, and that it will give us some answers.

Fiona's fundraiser