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.

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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.

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distraction

Today I was thinking about who I used to be. Fiona's illness has changed so much of our lives. I can't remember what I did before I found out she was sick. I did crafty things, I went grocery shopping during the day, and I read, but beyond that I can't remember. There is a constant distraction in my head, thinking about the next treatment, the next lab result, the next appointment. This is what happens when the future is so uncertain. I have to rethink everything over and over, but it never leads to a conclusion. There is no final plan. I'm a planner, I need a plan. We live in limbo, between two difficult paths, never being allowed to choose either one. Six months of this has taken a toll on us.

I know it will get better. I'm still fairly certain that she needs a transplant. Eventually we'll get there. I have to hope that when we look back in a few years we'll be glad for the lessons learned during this time.

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 Fiona's Fundraiser

I came home from Cincinnati with a stomach bug, now Fiona has it too. I hope it's short lived.

The Ronald McDonald house was amazing! The rooms are comfortable, the common areas are nice, and they have a huge kitchen. They have 2 meals each day provided by community groups. There are fun activities, and lots of toys for the kids. We loved it. It was a good first trip, and we learned a lot about the house. I realized on this trip that we have a child with a chronic illness. I knew that she was sick, but I've never thought of it in that way before.

Our appointment with the gastroenterologist was great. He answered a lot of our questions, and I feel like I really know what's going on with Fiona's belly. He commented that her belly was really distended, but we've been dealing with that since she was born. I don't think they will do any scopes unless they are prep for the transplant.

Our appointment with hematology was confusing. Last time it seemed like there were no other tests beyond the genetic SCID panel for diagnosis. Now it looks like there are some other things. They need to make sure that her thymus exists, and that it is functioning correctly. Once the genetic test comes back they will look at other genetic problems. One that was mentioned was CHARGE syndrome. After I googled it I'm sure she doesn't have it. The doctor asked if I had gestational diabetes during my pregnancy with Fiona. I did. He said that can cause problems with a child's thymus.

We are frustrated with the up and down. One appointment we feel like we are going to transplant, and the next we are sure that they won't. Not being able to diagnose her condition beyond symptoms and abnormal labs is frustrating. We can't plan our future, because we don't know what it will be.

The labs that have come back so far look about the same as before. Some of her T-cells have come up a little, but her helper T-cells are still low. Her IGG is at the high end of the normal range. She has more energy than we've seen for a long time. They also drew labs for James and Abbie, our two sibling match donors, to make sure they are positive or negative for the same viruses as Fiona.

In summary, we want a diagnosis, we want to move forward with the appropriate treatment, and we want to be able to plan our future. We're tired of making plans that don't happen. It would be nice to have a treatment that would fix her condition. Someday I would like to walk into an appointment and have them say "we found it!".

anxiety again

Today Fiona was visibly shaking when it was time to stick her. The nurse who was here to teach me how to use the new pump saw it too. I really don't like seeing Fiona so scared and anxious. I premedicated and used numbing cream. Once the needles were in she was fine for the entire infusion.

We go to Cincinnati tomorrow. I'm looking forward to seeing the Ronald McDonald house. I'm anxious to find out what they have decided about transplant. Waiting for someone else to decide the future of Fiona's health has been difficult. It would be nice to have a decision so we can plan our lives. I'm very sure we will eventually end up there, so my feeling is let's get it done.

Tomorrow James and Abbie have to get labs drawn. In addition to the genetic match there are viruses that the doctors want to see matching viruses also. If Fiona is negative (or positive) for those viruses they want her donor to be negative (or positive) too. Knowing that they aren't going to like having blood drawn has brought out some nervous feelings for me. One of them is going to endure some pain to save Fiona. How do you explain that to a child. I think Abbie is more emotionally prepared for it. If I got to pick my trials, these would not be the ones I picked.

Fiona likes to stick her arm inside my sleeve so she can touch my arm for comfort. I think it started when she was in the NICU and they suggested skin to skin contact. On treatment days she needs a lot of comfort. Having a small arm stuck inside the cuff of my sleeve means that I don't get much done on Mondays. I can't wait for short sleeve weather!

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Looking back

I was reminded today how difficult it was to start Fiona's care, diagnosis, and treatment. Looking back things are easier now that we know which parts of her immune system are involved, what keeps her healthy, and who to call when something goes wrong. It's amazing how time and perspective have made this seem like a normal life. There are still things that go wrong, but it's not as overwhelming as it was in the beginning.

We have to keep Fiona on Tamiflu, which tastes horrible and bitter. It's always a struggle to get her to take it. More often than not she ends up spitting it out or throwing up once she has it down. I thought I had a great idea last night, I mixed it with a teaspoon of presweetened kool-aid powder, which I don't normally let my kids have. Huge mistake! When she threw it up it was a red mess. Today I just went back to fighting to get it down. Two tricks to getting a toddler to take yucky medicine, I put one hand under her chin to make her close her mouth once the medicine is in, and I blow in her face to make her swallow. I don't like forcing her to take her medicine. If you have any better ideas, please comment below.

new pump

Next Monday a nurse is coming to our house to train me how to use the new electronic pump. I though I was going to have to fight to get it. A nurse called me today to set up a time for a nurse to come watch Fiona's next infusion. After a few minutes I think she understood that I knew what I was doing and set up a shipment for a different pump.

After next week we are going to switch Fiona's treatment day to Sunday. Cincinnati thinks it may help to have everyone here to distract her during treatments. Hopefully her next treatment will be much better.

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today

Today Fiona had an appointment with our local doctor. He said that 3 hours is too long for her infusion. He told us that Fiona shouldn't be going anywhere, especially the children's hospital, unless she is taking Tamiflu. He would like to see her go back to an electronic pump. She still looks good. I think that isolation has kept her healthy.

Accredo called to set up the order for her infusions for next month. It was a long call because they are trying to figure out how to reduce the length of her infusions. The plan is to have a nurse come watch me give her an infusion. They are sending a new pump just in case this one is malfunctioning. If I'm giving the infusion correctly, and the new pump doesn't reduce the time they will try different rate tubing. Rate tubing controls how fast the liquid comes out of the pump. The tubing we are using should make the infusion about an hour and a half.

I'm going to work on having our doctor's office write an order for an electronic pump to Accredo. Hopefully we can go back to the same pump we used to use.

Fiona's fundraiser