Antibiotics for the winter

I've started trying to post once a day, but I talked to Cincinnati after I posted. Another test came back normal. Fiona is having some sleeping trouble, sometimes too little, sometimes too much. She also has a low grade fever. They've checked for lots of viruses, but nothing has come back positive. In an effort to add an extra layer of protection she's starting amoxicillin for the winter. Hopefully it will prevent any bacterial infections. Now all we have to worry about are viruses. The IGG we give her comes from a huge donor pool and temporarily transfers their immunity to her, so it will help with viruses. The last part of her protection comes from an antibiotic they give to people with t-cell deficiencies called bactrim. Bactrim should protect her from opportunistic infections. So to recap, she takes IGG to replace what her b cells aren't making, bactrim to protect her because of her low t-cell counts, and amoxicillin to cover all of the bases.

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little bird

Christian and I were talking a few days ago about how fitting we think the bird in the cage at the top of Fiona's blog is. When my dear friend designed it for us I loved the bird, and as I've looked at it for a few months I've grown to love it more. There are a few reasons we feel it fits our situation so well. First, we think it represents Fiona and the layer of protection we try to wrap around her. Second, we feel caged by not finding the real problem. Having a better diagnosis would be so freeing. Third, Fiona is like a tiny trapped bird. She can't go out, she can't see other kids her age. She is so limited by her immune condition. She used to run for the door when it opened to try to get out, but now she's forgetting to try to escape. That makes me sad.

Yesterday our wall hand sanitizer dispenser came in the mail. I never thought I would love having hospital equipment stuck on the wall of my front hall. It doesn't look as bad as I thought it would. It is a novelty for the kids, especially James, who has the cleanest hands he's ever had! I'm starting to wonder how long each package of hand sanitizer will last?

treatment changes

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Last week's treatment was pretty painful for Fiona. We looked into lots of skin numbing products and got permission from our doctor to try 20% benzocaine. I went to our local Walgreen's and asked our awesome pharmacist if she had any suggestions. She suggested trying teething ointment. Brilliant idea! It didn't work as well as lidocaine, but with Tylenol the sticks were much better. We're thirty minutes into the infusion, and she only complained when I stuck her.

Along with a new bigger pump we have new bigger syringes. I was very surprised by the difference in function. The smaller syringes were smoother to push (does that even make sense?). The bigger syringes are also more difficult to hold in my hand while sticking her and opening the bandages. The old syringes had blue caps on the ends, but the new ones have caps attached to the plunger, which have to be broken out of the plunger. I'm sure in time I'll get used to the changes.

You can see the new syringe at the bottom. It holds up to 50ml, or 10 teaspoons.

Fundraiser update

Thank you to all of the amazing people in our lives who have donated outside of the fundraiser. In the past few weeks we've been given more than $2,000. With every donation I can feel my stress level go down. Knowing that we will meet the out of pocket for our insurance in the next two weeks is a scary feeling. I'll be glad once we have though because it will be done for the year.

We've learned a lot about Fiona's medical expenses in the past week, and I'm sure we'll learn a lot more in the next two weeks. We have learned that most of her visits to Cincinnati Children's are about $9,000. We still don't know how much we will be charged by the medical supply company for Fiona's infusion medication.

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reminder

Today I've been reminded that we are the lucky ones. Fiona is growing. Fiona isn't sick. No matter how scary or how difficult this is for us, we are blessed. Her infusions and antibiotics are protecting her. What we're doing now is keeping her safe until we can figure this out.

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new pump!

Our medical supply company sent us a new electronic pump that will accommodate 50 mls. Her current dose is 5 gms in 25 mls. That means they can double the dose she's on before she'll need a new pump. The new pump also allows a longer infusion time, which may help her feel less pain as the medicine goes in. When we got the first pump I thought it would last us at least a year. It's been 2 months and they've almost doubled her original dose.

I've been going over our insurance statements. Because we paid our out of pocket so early we haven't seen any of the statements in a few months. Since Fiona was diagnosed in July they've paid out over $78,000 (that doesn't include her hizentra or infusion supplies). I'm pretty sure we will reach our deductible and out of pocket in January. I'm so thankful for insurance!

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What I want...

Another test came back from an outside lab. In the charting system I have access to I can't see outside lab results. Once again I am impatiently waiting. I'm sure that if it was something really important they would have called me by now. Being in diagnosis limbo really messes with your perspective. I want to know things, and I want to know them now. I used to be such a patient person, I think I need to work on that.

Merry Christmas

Merry Christmas to you all! Fiona got plenty of chocolate in her stocking, and when she was done with it she took some from everyone else. She had 3 baths today because she was so sticky. Her favorite toy? Her brother's legos. She would sneak up and run away with a handful.

Limbo

 Fiona's fundraiser

It's been a busy day. I talked to Cincinnati about Fiona's emergency room visit last week. They were a little surprised that we were sent home without any testing. I think they are as worried about her as I am. Our coordinator said that we must feel like we're in limbo. We do. It's hard to wait for a diagnosis.

Because of Fiona's paleness, greyness, and dark circles we are increasing her dose of Hizentra. This increase means that her pump can't handle the volume. I'm not sure what the nurse for our medical supplier will decide to do. They have a few options. There is a bigger pump, but it's not electronic. We could use two pumps simultaneously. The option I don't like is that we could do two infusions a week (I won't let this happen). There may be one other choice, but I can't remember it. It's been a long day.

Today is her second treatment without numbing cream. The doctor's are still exploring pain management options. All of the topical creams have lidocaine, which I think is the problem. She is in moderate pain during the infusions, and I'm not okay with that. Tylenol isn't enough. She kept saying, "ouch, hurts". I'm going to ask if ibuprofen is okay.

I've been thinking about trying a gluten free diet and some foods to try to heal Fiona's gut. Cincinnati said no. They need to scope her before we implement any dietary changes. Any dietary changes that could heal the gut would change the results of a scope.

The test that was sent out to Duke is back. The results are normal. This is the test that can show what's wrong when the doctors are stumped. This isn't the last line of testing, but it is disheartening to loose that little bit of hope. These are the days that a bone marrow transplant seems like a great idea.

Emergency room visit, almost too lame to write about

So this morning Fiona was looking pretty bad. Grey, pale, dark circles under her eyes, again, but worse. I called Cincinnati, and after consulting with the doctor I was told to take her to the emergency room. I called and left a message with our local doctor and took her over. We spent about an hour and a half there. The ER doctor looked in her mouth, checked her cbc results from Wednesday and said we could go. I feel like there's something they're missing. I'm glad to be home and have her out of the germy hospital.

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What we've ruled out

So far I think we've ruled out bare lymphocyte syndrome and DiGeorge syndrome. We have a hospital account that shows test results as they come back. Our account doesn't show the outside test results though. a new outside test came back, but I'm not sure what it is yet.

Fiona is looking grey and pale still with dark circles under her eyes. She doesn't look healthy or pink. More than anything else, that has me worried. She isn't sleepy like last week though, so hopefully we can spend the weekend at home. I'm going to stock up on some juice to make sure we keep her well hydrated.

On Wednesday the doctor reminded us that t-cells can be suppressed by viruses. T-cell suppression can lower t-cell response and function. Her lower numbers may just be a virus, so they are checking for strange viruses. We know that she doesn't have anything normal, they checked all of those first.

They are running a more in depth test of her t-cell receptors. So far the normal t-cell receptor test came back normal. T-cell receptors are part of the t-cell that tells the cell what to do in response to other cells. Her t-cell receptors shouldn't be normal. Again, weird.

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confusion, tests, and mysteries

Today I feel like I've been in an airport waiting for a flight, only to have the flight delayed again and again (the flight is her diagnosis and treatment). Fiona is a mystery. There are many things that she could be diagnosed with, but there is also something that doesn't fit. When we get test results back they are sometimes confusing, most of the time we think we understand what they are testing, but we don't.

What we know is that her t-cells are low, her b cells are low, she is loosing protein somewhere during digestion, her t-cell function isn't good, and she isn't sick. We always come back to why isn't she sick? When they say she should be sick, they mean hospitalized, on her deathbed sick, not just a cold. In spite of replacing her IGG she still has an extremely compromised immune system.

They are sending off more genetic tests. One of them is only done by one person in the entire world, and it often gives an answer when no other answer can be found. Again, they drew as much blood as they could from Fiona.

Sometime in January, possibly the 8th they will scope her stomach and do a colonoscopy. The gastroenterologist wants to rule out celiac disease. Looking from the top and from the bottom of her GI tract leaves about 10 feet in the middle that they won't see. They may have her swallow a capsule camera to look at the rest.

We've been told to keep her away from crowds, and to mask her when she leaves the house. Have you ever tried to get a 2 year old to wear a mask? It's not her favorite thing. I think that in time she will get used to it.

One of the things we learned yesterday is that a specific t-cell function test called pha is the best indicator of how well her t-cells are working. In August her number was 203,250. Last month it dropped to 17,563. Normal range is above 135,190.

There is more to write, but naptime is almost over. More tomorrow!

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treatment pain

Yesterday I had to do Fiona's treatment without numbing cream. She's developed an unusual reaction to the numbing cream. It's supposed to make your skin turn white, which it did for the first 20 or so times that we used it, but now it leaves a raised, bumpy, red rash for about two weeks.

She says that you should all feel free to send sympathy gifts, mainly chocolate. :) It was horrible. She screamed for the first 10 minutes. Tomorrow we are going to Cincinnati and will be asking the doctor if there are any other options for pain control, someone mentioned numbing spray, but she may be too young to use it.

From what I can guess her treatment is roughly the equivalent of sticking three tacks into your skin and injecting about a teaspoon and a quarter of liquid under your skin. Most subcutaneous injections are less than half a teaspoon. Ouch!

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We're home!

It's so nice to be home. They didn't find any reason for Fiona's lethargy or her grey skin. The last doctor we saw wanted to take Fiona home with him, and our nurse told us that she loved us and wished that all of her patients were like Fiona. Santa and Mrs. Claus came to visit courtesy of Frontier airlines and he brought her a book and a stuffed animal. Thank you Santa! Fiona was watching for people to pass our room when he showed up. He made her day.

As soon as I got into the house with our bags Katherine walked out the door to find a lovely Christmas bag filled with fun surprises. Katherine is calling it the super secret ninja Santa bag. We're not sure how you timed that so perfectly, but thank you!

Fiona is feeling and looking much better. We appreciate all of the prayers and thoughts in her behalf. We feel incredibly blessed to have such amazing friends and family. Thank you all!

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Another 24

We're here for another 24 hours to let cultures grow. Our doctor wants to be cautious because of her immune deficiency. They are giving her IV antibiotics every 12 hour in addition to her normal oral antibiotic. The last doctor to visit us thought Fiona was the cutest patient he had ever seen.

hospital update

There really isn't a lot to update. So far all of her labs have come back normal, except the ones that aren't ever normal. We are still waiting for some labs and a respiratory panel. She's started coughing, but doesn't have any other symptoms.

Hospital stays are hard, doctors and nurses come in every few hours. There are lots of unfamiliar noises, and we are in an isolation room, which has constant airflow. There is also a double door entry so when anyone comes in or our we hear both doors.

Our TV is broken, so Fiona is extra restless. She gets short burts of energy then wears herself out and sleeps for hours. I'm convinced that a day in the hospital is like a week on the outside.

Fiona is drinking normally, but isn't eating much. She's about to crash, just in time for the doctor to come in. We've been up since 4 am.

Finding ways to play.

She thought this was lots of fun!

admitted

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We went to see the doctor and ended up being sent to the hospital. Our doctor was worried when she slept through an exam. Her energy level is way down and she's very pale. They said to be prepared for a blood transfusion.

We're in the emergency room waiting for a lumbar puncture before they send us to a room. I'm not sure how long they'll keep her. Her IV wasn't as difficult to start as it usually is. They are giving her some medicine to help with the lumbar puncture.

She hates hospital wrist bands and spent the first five minutes trying to pick it off. She almost has it. It's just hanging on by a tiny section.

greyish

Fiona is pale, and kind of grey. She has dark circles under her eyes and her eyes are a little sunken. It may just be another of her mysterious things, like the low grade fever and stomach issues. We are taking her to the infectious disease specialist tomorrow to get checked and to get her blood counts checked. The nurse I talked to seemed pretty concerned. She told me that if anything changes to take her to the emergency room.

I ordered a box of pediatric masks today. We are going to try to start masking her when she leaves the house. I'm sure it will take some getting used to. Going to doctor's offices and the hospital are making me increasingly nervous. Eventually she will get sick, but I'm hoping a mask will give her a little extra protection.

Our amazing daughter Katherine is working to get Fiona's story on the news. She is very driven and determined to help her sister. She is always coming up with new ideas to reach more people.

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blessings

When we met with the GI doctor we also met with a fellow. Cincinnati Children's is a teaching hospital, so we see residents and fellows often. It was interesting to hear her perspective on Fiona. She was amazed at how normal Fiona looks. She said that immune deficiency kids don't grow well, and they are always sick. For a few months I've felt like we are so blessed that Fiona hasn't been sick. As more numbers start to drop her health is nothing short of miraculous.

We are still waiting for the last labs and genetic tests to come back. I always feel so sure that her test results will find the thing that will confirm a diagnosis. Unfortunately, so far they haven't. This week the doctor's have ordered 3 sets of stool samples. TMI? Probably. They are trying to see if she is leaking protien and lymphocytes in her gut. They are also checking to see if she has any infections or parasites.

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Nap time update

It's nap time so I can finally write an update. Fiona had a CT scan yesterday, and a consultation with a GI doctor. The CT didn't find much, some enlarged lymph nodes. I don't know what that means, or how serious it is, but our hematologist is out of the office until next week. I'm sure we'll hear from them soon if it's a problem.

Fiona charmed all of the doctors and nurses. They said she was the cutest patient of the day. Because of her sedation she wasn't supposed to walk for a few hours. Once we let her out of the stroller she climbed up to stand on a chair. One of the nurses tried to help her balance and she said, "HEY YOU!", which translates to "leave me alone, don't touch me!".

The GI is planning on doing an upper GI scope, a colonoscopy, and a camera capsule. He mentioned that while she was under hematology should do a bone marrow biopsy. We're waiting to see if hematology agrees. We think she will also have a scope of her lungs.

Now we wait. I hate waiting.

bad news

Today we saw our home doctor. Fiona's t-cell function fell. Fell doesn't really do it justice though. It's more like it jumped off a cliff. Her numbers are scary low. She's being quarantined to home. She can't leave the house except for doctor's appointments. She isn't allowed to go outside. We can't have live plants in the house. She'll probably start a diet that will limit bacteria on her food.

The doctor here thinks our next appointment with Cincinnati will be scheduling the bone marrow transplant. He said her t-cells and b cells are failing. I'm trying to be calm. It's not working out for me.

Cincinnati Tuesday

Cincinnati called again today. This time it was to let us know that Fiona will be having sedation for her CT scan. Because she's a wiggly toddler who won't like being taken away from her parents and put into a loud machine they've decided to let her sleep through it. I think that's a good choice.

The day before we go to Cincinnati we see our doctor here. I'm going to ask him to draw some labs to see what her igg level is. I think she's metabolizing it more quickly than they expect her to. Hopefully I'm wrong. If I'm right they will increase her dose again. Increasing her dose means that we will have to do longer infusions or get two pumps or get a new pump that can hold a bigger syringe.

Being thankful

Sometimes it's hard to be thankful for trials. I'm trying to focus on the blessings we've been given during the trials. Here is part of my list.

• We know about Fiona's illness. 
• We have great doctors who are working to get her the best treatment.
• We have health insurance.
• We don't have to drive our old scary minivan to Cincinnati.
• We have great and supportive family and friends.
• We have 2 full sibling matches for bone marrow.
• In spite of her low counts she isn't getting sick.
• There are lots of people who love Fiona.
• We know what her future treatment will be, and we've had time to process.

 Fiona's fundraiser

infusion changes

We had to switch to Tuesday for her infusion this week. Our supplier thought she was still on Thursday and couldn't get her medicine in time for Monday. We also increased her dose and added a third site. I think the third site will be an improvement. Her sites aren't as swollen. She's complaining about her sites because I was in a hurry to get started and her numbing cream didn't have enough time. Next time I'll wait longer.

We see our home specialist on Monday. I'm hoping that he will check her igg. Based on her last labs l'm a little worried that we may have to increase her dose again.

I've been reading about immune deficiencies over the past few weeks. Waiting for labs to come back is anxiety causing. The genetic tests are especially hard to wait for. I want to know what is causing her deficiency, and I want her to have the best treatment for her condition.

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The day that everything changed

I was just sorting through pictures and found this....

I forgot that I took pictures that day, mostly to show Christian how pitiful she was. The yellow wrap is covering an IV. She was going on day 15 of a fever. She was dehydrated and wouldn't drink or eat anything. They had just drawn labs. A few minutes after I took this picture they did the nasal swab that showed she had 3 viruses. We wouldn't find out for a few months how severely compromised her immune system was.

When I see this picture all of my what if questions start. What if she hadn't gotten sick? What if I didn't take her to Primary Children's Hospital? What if my aunt hadn't suggested the test? What if we didn't catch it in time? It also takes me back to an appointment in July when our infectious disease specialist told us that her condition was life threatening.

As a parent I am living my worst nightmare. The funny thing is I think this is normal now. It's when I stop and think about it that I realize that this is not normal! The not normal feeling hits at weird and inconvenient times. I remember almost breaking out in tears at a grocery store checkout. I walked out quickly and crying in the car. This picture brings it all back.

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lab results

Cincinnati sent us Fiona's labs. All of her t-cells dropped again. I'm reading medical research papers to try and guess what she has. Crazy, right?

What keeps me from worrying too much is monitoring her condition is learning about it. I've started reading an immunobiology textbook. It's a slow read. I feel like it's my responsibility to know and understand what is happening in her body. When her labs looked better I stopped memorized her numbers. Now I can recall specific t-cell counts and most of her cbc (complete blood count).

The supplier for Fiona's medicine called on Thursday. Our rep forgot that we changed treatment day to Monday. She can't get her medicine here until Tuesday. We're going to keep her at home until she has her next treatment. Exposure is too risky, because her igg is probably less than 500. 500 is the lowest the doctors want to see her igg.

Thank you to our wonderful friends who have donated so far. We are really thankful for you and your support.

Fiona's fundraiser

Another call from Cincinnati

They are calling more than I like lately. Fiona's helper t-cells are down to 136. A normal range would be 500-2400. The only difference between her and someone with AIDS is that her condition isn't caused by a virus. It's caused by a defective immune system. Her white cells are up, which could signal an infection. She's starting her preventative antibiotic tonight, which will lower her neutrophils again. She's also metabolizing her IGG faster. I'm glad they increased her dose.

Increased treatment

Fiona's doctors took her off the preventative antibiotic to get her neutrophil count back up. To give her some added protection against opportunistic infections they raised her dose of hizentra. I talked to her rep about her reaction to the treatment and I've decided to use three sites instead of two for her infusion. I think this will be much better for her.

Yesterday she spent a lot of time resting. She seems to be feeling better. With cold and flu season starting I've noticed that she starts to get a runny nose, but it goes away instead of getting worse. I'm amazed at the difference her infusions make in her ability to fight illness.

Fiona's t-cell counts are worse than someone who has AIDS. In addition to her low t-cell counts she also has low b cell counts, and her b cells aren't maturing properly. How bad is all of this? Her condition is life threatening. Our pediatrician told me that I must be doing something right in my life for Fiona not to be extremely sick.

Where does this leave us? First, hizentra is a life saving drug for her. Without it she would have almost no protection from illness. Second, diagnosis will be critical for her future treatments. Right now she is getting weekly infusions, but it would be incredibly helpful to know what is causing her problems. Third, what we will have to pay for her treatments, doctor's appointments, and travel in the first three months of next year is more than 15% of our yearly income.

We are incredibly grateful to everyone who has made a donation so far. Please donate if you can, and share the link with anyone you think can help us. Thanks for reading!

http://www.youcaring.com/medical-fundraiser/she-s-a-fighter-/102797

Yesterday

Yesterday was long. We left our house at 9:30 am and got home a little after 10 pm. We spent 7 hours in the Hematology clinic. James and Fiona were so good and patient the whole time. I learned a lot about the direction of her diagnosis and treatment.

In the next month and a half they will be running a lot of tests. First, a genetic test for severe combined immune deficiency (SCID). This test will take 2-4 months to complete. Second they are checking t-cell function again because of her low t-cell counts. Third they will do a head, chest, and pelvic CT scan to check for autoimmune problems and possible infections that don't manifest outwardly. Depending on the results of the CT scan she may need a PET scan to look at specific areas in more detail. Fourth, she will have bronchial and stomach endoscopes to check for infection and autoimmune problems.

It's possible that she may have leaky SCID, she also may have hypomorphic SCID. If you think of SCID as a spectrum, regular SCID is what the bubble boy had, leaky SCID is less severe, and hypomorphic SCID is the least severe. If she is found to have a variation of SCID they will most likely do a bone marrow transplant. If she has hypomorphic SCID the will watch her to see if her t-cell counts and function continue to decrease. Yesterday I learned that t-cell counts decrease as you age, so a low count in a child is more significant than the same count in an adult.

They are also checking for autoimmune responses. An autoimmune response is when your antibodies start attacking your body. They are looking specifically at organ damage. If her body is attacking her organs they will do a bone marrow transplant.

The last thing that they are checking for is lymphocytic gastritis. That's a medical term for losing lymphocites and protein in the gut. Some of her labs suggest that this is a possibility, but some of them rule it out as a possibility. In an effort to make sure we check everything they are still running the tests for this. If this is what she has the treatment is continued IGG infusions.

After our discussion of tests and possible treatment options the doctor ordered labs. They needed 19 mls of blood drawn. For a small child that's a lot of blood. It took 4 sticks to get a good vein. It was horrible! Sometimes she's an easy stick, but if she's not, she's really not. From now on we will ask for the IV team every time.

Once our appointment was over we left the hospital, drove for about half an hour and decided that we should feed the kids. I didn't plan on her appointment going as long as it did, so I didn't bring dinner. We stopped at Cracker Barrel, James' favorite restaurant (he calls it dunkin' barrels). Just after we ordered our food Fiona got sick, all over both of us. I rushed her out to the car, cleaned us up, and waited for Christian and James to finish eating. Christian brought out our food, which we are eating for breakfast.

Because we are pretty new to the immune deficiency thing every illness triggers a call to the doctor. When I called last night the doctor was very thorough, and decided that it was probably a combination of a long day and a stressful blood draw.

Cincinnati called back morning to let us know that her white blood cells just over 10 (the highest we've ever seen them), which sounds great, but it could also signal an infection. Her lymphocytes are a little lower than last week, she's dropped from 2.09 in August to 1.2 yesterday.

Today we are exhausted, and glad to have a day at home to recover from the craziness of yesterday.

Long day

It was a long day in the Hematology clinic today. We were there for 7 hours. When we left we stopped to get some dinner and Fiona threw up. I'll write more about it tomorrow. The clinic visit, not the puke.

Day after pictures

After yesterday's pictures I want to show you what the day after looks like. I usually give her benadryl for a day or two after treatment, I think it makes her more comfortable. It's amazing how fast her body absorbs the infusion. She still has a rash, but it looks so much better. The rash should be gone by tomorrow.

The swelling is gone.

Treatment photos

 Today I wanted everyone to be able to see what her treatment looks like. It's so much better than the IV treatment. Next week they are adding 5 mls to her dose. This is the last increase before they have to switch her pump. Each infusion takes about an hour, and we do this once a week. She is getting used to it. She is really good about letting me put the needles in, and about not touching the pump or the tape. In the beginning it took two people to get the treatments started. Now I do them by myself.

This is how I apply her numbing cream. She doesn't like the way this feels. I'm not sure if it's the clear bandage or the cream. The cream stays on for at least half an hour. It usually ends up leaking at least a little bit.

While the numbing cream is working I gather all of the supplies. We also have a sharps container, but I don't keep it in the box anymore, it takes up too much room. Because she's two she loves to try to take some of the stuff when I'm busy with other things. She especially loves the little glass bottles.

These are the needles and tubing. The plastic pieces that are shaped like a butterfly are what I hold to insert the needles. We just switched to 6mm needles because the 4mm needles were leaking. I'm experimenting with different kinds of tape to hold the needles in place. I prefer the clear plastic because I can watch her reaction, but paper tape is easier on her skin.

This is her during treatment. The strap around her neck is hooked to her pump (inside the grey bag) and the thing she is holding is the tubing. I roll it up and tape it to keep it out of her way when she moves around. This is the easy part. About half an hour into the infusion I think she starts to feel the pressure of the liquid, and she gets pretty fussy.

This is a few minutes after treatment. I don't think the picture really captures it well. It's raised a little more than a quarter of an inch. I give her benadryl after her treatment and the swelling is gone in about 4 hours as her body absorbs the medicine. It's the equivilent to getting a shot, but instead of using a little bit of liquid each site gets 7.5 mls. That's a teaspoon and a half. Next week it goes up to 10 mls per site. If her reaction is bad they may let us use 4 sites.

Fundraiser

We are trying to raise $8,000 to pay for Fiona's treatments and travel to appointments for next year. That's 8 months of mortgage payments for us. Our insurance doesn't cover anything until we pay our $6,000 deductible. After that it's 80/20 until we pay another $2,000. Hizentra is in a specialty drug tier, so instead of paying 20% of the cost we pay 40% until we reach our out of pocket. Because of the cost of her treatment we know that we will reach our deductible in the first 3 months of next year.

Our sweet girls are trying to come up with ways to earn money to help pay for treatments. They are amazing! Katherine is going to do photography sessions. Abbie is trying to come up with some art projects. Savannah and Ellen will be making crafts. In the next few weeks they will be starting an Etsy shop. I'll post the link when it's up.

Here's the link to the fundraiser. She's a fighter

This is a journey

Lately I've been wondering why a diagnosis takes so long. We know there is a problem, we know what the symptoms are, and we are seeing the right specialist. I've come to realized that this is not an instant process, but a journey.

I've been trying to find information about less severe combined immune deficiencies. Go ahead and google it, there isn't much out there. In severe combined immune deficiency (SCID) there is usually a complete lack of t-cells, and sometimes a very low level of t-cells. Fiona's t-cell levels are low, but not low enough.

She may still be diagnosed with a type of SCID called leaky SCID. Leaky SCID is usually caused by a partial mutation in a SCID gene. It doesn't manifest as complete lack of t-cells. Children with leaky SCID usually manifest later. I think this is what she has.

Our next appointment in Cincinnati is on Wednesday. They will be doing lots of testing at this appointment including genetic testing for the genes that cause SCID. If she is found to have leaky SCID they will do a bone marrow transplant.

Sometimes I get frustrated waiting for the doctors to make what I think is the next logical step in the process. At each appointment we see her lymphocytes and neutrophils go down. The doctors keep telling us that a CBC (Complete Blood Count) is just a snapshot in time, that if we took them even a few minutes later we would get a different result. It seems irrational to me to dismiss her labs so easily, but I'm a mom, not a doctor.

She's two!

According to our doctor's Fiona is a miracle. We knew that before she was born. She overcame so many challenges to get here. We are blessed to have her. She is sweet, funny, loving  and spunky.

Good news, I think

Fiona's neutrophils came up to 1.9, normal range is above 1.5. I'm not sure, but I think that's good news.  Sometimes it's hard to know how much a change in numbers really means. Her lymphocytes are still 1.3, normal range is above 4. The doctor said we won't isolate her unless she gets below .5. Her lymphocytes are trending downwards.

We probably won't be taking her out much because of cold and flu season. Cincinnati has decided to increase her dose of hizentra. She hasn't been having fevers since we stopped her antibiotic, but she is getting a runny nose. She's also waking up a lot at night.

Patience is a virtue?

I impatiently waited all day to find out about Fiona's test results, but they never called or emailed them to me. It's such a routine test, but for us it means so much more. I feel like our future hinges on the results.

This life is starting to feel normal now. Waiting and worrying are normal. Treatments are normal. Stress and anxiety are normal. Putting all of my trust in Heavenly Father is normal. I have to believe that this is happening for a reason. I have to believe that we can handle this.

I've started looking at the Ronald McDonald house website. I'm trying to prepare myself for the possibility of living in a hospital. I read that bone marrow transplant families stay with them for a minimum of 120 days. I'm still trying to figure out how that would possibly work.

more tests

Fiona had labs drawn today for a complete blood count (cbc) with diff. That test will show what her blood counts are, but most importantly it shows what her specific white blood cell counts are. We are most concerned with neutrophils and lymphocytes. Lymphocytes are t-cells, B cells, and natural killer cells. If her levels from last week are the same or lower she is more likely to develop infections. We should have results back tomorrow.

Another call

Cincinnati called again today. They want to do some labs here this week and see her next week. Looking at the big picture her neutrophil count is down, all of her T-cells are down and her B cells are down. They are starting to talk about the bone marrow transplant again. Next week they will be running genetic tests to check for SCID or other deficiencies. I think this is a positive step towards diagnosis.

We started a fundraiser to help pay for Fiona's medical treatment. Please feel free to share it. https://www.youcaring.com/medical-fundraiser/she-s-a-fighter-/102797

tape allergy

Remember the post where I wrote about Fiona's allergy to tape adhesive? I was wrong. She's allergic to the numbing cream. When I wiped it off her skin was red and swollen, but only where the cream was touching. I hope they can give us something else to numb her skin.

Low ANC

The specialist from Cincinnati called today. They NEVER call. NEVER. I thought he would want to talk about t-cells. Nope, he pointed out that her Absolute Neutrophil Count (ANC) was low. Really low. Normal ANC is above 1.5, Fiona's was always over 2. Wednesday's ANC was .99. The plan for now is to stop her preventative antibiotic, wait a few days and test again. If it goes up we switch her antibiotic. If it doesn't go up we test for autoimmune problems. If she gets a fever we go to the pediatrician, and possibly get IV antibiotics for a few days. We are starting to feel stressed again.

lab results

I've been putting off writing this post. All of Fiona's t-cell counts have gone down. Her helper t-cells are the lowest, down to 172 (normal range is between 900-5500). We probably won't know more until Monday. We are going to isolate her until we can talk to her doctor. Things were just starting to feel normal.

versaderm adhesive allergy fix

Versaderm is the name of the clear plastic we use to cover the numbing cream and keep the cream from rubbing off for an hour before infusions. Fiona is developing a reaction to the adhesive on the plastic. We've been trying to come up with something else that won't make her red and itchy. We may have found the solution! One of the nurses from Cincinnati suggested glad press and seal plastic wrap. It's been on for 10 minutes, so far it's working. It stuck to her skin without any additional tape. She doesn't really like it on her skin, and the cream has spread out a little more than with verasderm, but I think it will work. 

We are moving her infusion to Monday's next week. Someone suggested moving it a day each week, but our doctor said we can do her infusion today and again on Monday. 

where we are going...

Our appointment today was good, and long. The doctor explained a lot about the plan for reaching a diagnosis. Our first step is to check her t-cells again. If they've gone down we do some genetic testing for SCID. If she comes back positive for SCID we do a bone marrow transplant. Autoimmune responses, opportunistic infections, and slow growth are all possible reasons for a bone marrow transplant.

She got her flu shot, which she may or may not develop antibodies to. The hizentra we give her contains antibodies from thousands of donors, which give her some protection. Unfortunately it's not a long lasting protection. Her body metabolizes the antibodies in about three weeks.

Sometime before January she will have a series of scans to check her organs. She is at risk for organ damage from autoimmune responses.

The doctor mentioned that she may still outgrow her hypogammaglobulinemia (low igg). It's not likely, especially with a t-cell defect and an extremely low igg count, but is possible.

Sometimes it's overwhelming to try to process everything associated with her condition. I'm sure that I'll remember more tomorrow. We're all exhausted and ready to sleep.

Not sure where we go from here...

Today's visit with our local doctor left me wondering how are we moving forward? We know what the problems with her immune system are. We are addressing the problems with antibiotics and Hizentra, but we aren't looking for the cause. I have heard that it can take a long time to get to a diagnosis, but it feels like we aren't progressing toward anything.

Our specialists are disagreeing about whether or not she should have a flu shot. Cincinnati feels that she wouldn't make an antibody against the flu, so there is no point. Our local specialist feels that the worst thing that could happen is that she wouldn't produce an antibody against flu, but maybe she would develop a mild antibody, which is better than nothing. We go to Cincinnati on Wednesday, so we'll ask about it then.

We've been hearing about a new development from the manufacturer of Hizentra. In September they released a formula that would only need to be given every two weeks. They are really pushing this new dosage. I asked the doctor about it today. He said that until they could show that it would maintain a more consistent level he wasn't recommending it to his patients.

What I'm hoping for on Wednesday is that they will give her the flu shot if we suggest using it to test how well she makes an antibody. This would involve drawing blood before giving the shot, and again later to see if there is a change in antibody levels. I'm also hoping that they will check her helper t-cell level. The plan was to watch and see if her t-cell level went down, but they haven't checked it since August. I'm going to request that they do more of the genetic testing before January, while our insurance is paying 100%.

The day after

The day after Fiona's treatment is crazy. She has lots of energy to be naughty. So far today she has emptied my wallet, scattered the contents across the kitchen, hall and family room, squished blueberry pancakes into the carpet, dumped a glass of water on the carpet, unpacked a diaper bag, climbed onto the table, ripped apart a box of pumpkin bread mix, colored on the fridge, colored on the inside of a kitchen drawer, emptied the sock basket, dumped the toy basket, spit chewed up peas all over the floor, took out a strainer from the cupboard, put the strainer on her head, decided the strainer made a better step stool, and unrolled waxed paper on the kitchen floor.

She likes to rest on a pillow between adventures. Enventually she will exhaust herself and take a three hour nap. Even though we've seen an increase in activity she can't sustain her energy for more than 15 or 20 minutes at a time.

She isn't eating very much today, but I expect a demand for chocolate and chicken this afternoon. I'm thawing chicken for dinner, and our trick or treating was moved to tonight because of the storm yesterday, so she'll be happy.

Her pants and socks have mysteriously disappeared, and I have an hour to find them before we go pick James up from school.

what is a treatment like?

As I give her today's treatment I'll try to describe it for you. An hour before her treatment we put on some numbing cream and cover it with a clear plastic bandage, like what they cover an IV with. Then I try to distract her for an hour while the cream works. Right now she is poking the cream and saying, "gushy, squishy, gushy."

While I'm trying to distract her I get the supplies ready for her infusion. We use a plastic mat, pump, bag, neck cord, 20ml syringe, y tubing, transfer spike, journal, gauze pads, alcohol prep pads, paper tape, clear bandages, coban wrap, and hizentra.

The first thing I do is to lay down the mat and wipe it with an alcohol pad. Then I pop the tops of the hizentra bottles and wipe the tops of the bottles. Next I stick the transfer spikes into the bottles, which are covered with a flexible membrane. One at a time I draw the liquid into the syringe making sure to get as much liquid as possible. After that I attach the y tube to the syringe and prime the tubing by pushing the liquid almost to the end of the tubes. Then I wipe the cream off and clean the site with an alcohol pad. The ends of the y tube have a small needle on them, and a tab on each, side to hold onto. I stick the needles in, taping each one down with a clear bandage and pull back on the syringe to make sure no blood comes out. If no blood comes out I unscrew the plunger from the syringe and attach the pump. Then I start the pump. I also have to keep a journal of her reactions, lot numbers for the hizentra, and start and end times. Sometimes we cover the infusion sites with coban, which is a self adhering elastic wrap, so she doesn't touch the sites. Each infusion takes an hour. We try to keep her sitting so the needles don't move. When the infusion is over the pump beeps, I take out the needles and put pressure on the sites so they don't leak. I cut the needles off the tubing, put the needles in our sharps container, and throw everything else out.

We think she is developing an allergy to the clear bandages. We found some new tape that is better than paper tape and more gentle on her skin, but we need the clear bandages so we can see her reactions and to keep the numbing cream in place.

Her usual reaction to the hizentra is mild, think of an immunization, but with a teaspoon and a half instead of a small amount. She gets really big goose eggs. As her dose increases we will add more sites to spread out the liquid. Her current pump is electronic and will only allow for one increase in dose, then we move to a spring driven pump.

Today was the first treatment I did without another adult to hold her arms. She did really well. I think she is getting used to the idea, but is still nervous when I stick her the first time. She doesn't really feel it, so the second stick is easier. We started her infusion at 11:50, and now, half an hour later, she is up and playing.

low grade fever

Fiona has had a low grade fever for a few weeks now. I'm starting to wonder if it's a side effect of her antibiotic. We've checked to make sure she doesn't have any bacterial or viral infections, and so far everything is negative.

Tomorrow is her treatment day. Treatment days are exhausting. I think I'll try to do her treatment while the girls are at school. I'm glad our community is postponing trick or treating. I think it would be too much for her.

The next few weeks are going to be busy with doctor's appointments. Next week Fiona will see her doctors here and in Cincinnati., and the next week is her two year checkup. I'm going to push for more testing, hopefully the genetic testing, before the end of the year. Since we've met our out of pocket I want to get some of the more expensive tests done while our insurance is covering 100%.

I'm really looking forward to seeing what her igg level is next week. Hizentra has introduced a formula that would let us do treatments every two weeks. I'm going to ask the doctor if that is an option for Fiona. I'm also hoping that she weighs more than 30 pounds. If she does she can get an epipen instead of the glass vial and syringe of epinephrine we have now.

my hands

This is more of a whiny post about myself than anything about Fiona, so feel free to ignore it. The specialists have told us that the best way to keep her healthy is to wash our hands often. I think I wash my hands at least 20 times a day. I also use a lot of hand sanitizer when I run errands. My hands feel like sandpaper. At the end of the day it's hard to straighten my fingers because my skin is so dried out. If any of you have great advice about dry hands please comment. Thanks!

treatment cost

We were able to look up a claim from our insurance company for Fiona's medicine. For a four week supply it's about $1,600. The price will change as her dose increases. It looks like it will be billed as a prescription, but it will go towards our deductible and out of pocket. I think our insurance company is adding a specialty tier, but I'm not sure what that will mean for us. It would be nice if the insurance company gave us a rep like our medical supply company does. Being able to talk to someone who really knows our situation and can give us specific details would make a huge difference in our stress levels.

eating like a horse

In the two days after treatments Fiona gets hungry. Really, really hungry. After her first IVIG she came home and ate almost as much as Christian and me. After a particularly memorable treatment she shoved handfuls of chicken into her mouth as fast as I could cut bite sized pieces. She seems to love protein and chocolate lately. Before she was diagnosed she loved fruits and vegetables, now it's hard to get her to eat them. I wonder if this is just a normal toddler phase or if this is her new way of eating?

Settling down

Really not much to update, which is great! Fiona has had some mild fevers, but they don't last more than a few hours. She goes back to Cincinnati in two weeks.

Christian gave Fiona her infusion yesterday. He did a great job. I'm trying to figure out a routine for infusion days. So far I haven't been successful. It's an exhausting process, and by the time it's over my house looks like a tornado passed through. I hope it will get better once the doctors let us change her treatment day. It would be nice to give her infusion when the older kids are at school, but I'm not sure if I can get the lines started without someone to hold her hands.

I had a great conversation with another mom last week. We talked about how as moms we can be judgemental of each other. I am embarrassed to admit that before James and Fiona I judged other moms. I used to wonder why some moms couldn't control their kids. I've learned that motherhood isn't about control, it's about working with what you're blessed with.

Fiona's energy level and attitude the day after her treatment are at best wild. She was in Tasmanian devil mode at our church trunk or treat tonight. It's hard to watch her energy come and go so quickly. I find myself wondering if her tantrums are because of headaches and joint pain that are side effects of her treatments. Because she looks so healthy and normal I forget how hard these treatments are on her body.

Pricing and pharmaceutical assistance programs

I still don't know how much Fiona's treatments cost. I've heard wild estimates, but I'm still waiting to hear back from our supplier. Not being able to plan for the cost is stressful. The lowest estimate that I've heard is $1,200 a month, and the highest is $60,000.

Most people that we talk to about the expense of Hizentra say that we should check with the manufacturer, Behring to see if they offer an assistance program. They do, but only for people who are uninsured, or whose insurance companies don't cover the drug. I found a nonprofit that helps with copays, but their primary immune deficiency program is temporarily full.

I know this will be difficult. I know it will be a struggle to pay for treatments. I know that we will always have to fight for Fiona, but she's worth it! How many parents get to raise a unicorn?

The Affordable Care Act and specialty drug tiers

This may not be a popular opinion, but I'm thankful for the Affordable Care Act. While there are many parts of the ACA I don't like (the entire exchange system, or penalizing people who don't have insurance), there are some parts that will benefit Fiona. Before the ACA people like Fiona could be dropped from their health insurance for no reason other than the cost to treat them. They could be denied health insurance because of a pre-existing condition. They faced yearly and lifetime caps. This is huge when you consider the cost to treat Fiona from birth to two years will be enough to pay for a nice house. Over the course of her life it will easily exceed the average lifetime cap of 2 million. We are so lucky that we never had to worry about any of this.

What we have to worry about now are specialty drug tiers. In the past there were three tiers, low cost, moderate cost, and higher cost. Insurance companies are starting to add a fourth and fifth tier that are paid with co-insurance rather than a co-payment. That means patients who need these drugs will pay a percentage of the cost instead of a fixed cost. For patients like Fiona this cost will be substantial and unaffordable. There is no generic for igg.

Healthcare reform hasn't really made healthcare affordable, but it offers Fiona some protection. I think we have a long way to go.

She's trouble

Today at the doctor's office we went over Fiona's latest tests. Her igg is at a good level, if she starts to get sick they will try to increase that level. Her white blood cells are almost normal. She looks good, and the doctor said, "we'll watch this one, she's trouble." He has no idea.

My pregnancy with Fiona was difficult. She was born 5 weeks early because of a placental abruption. She was in the NICU for 10 days for jaundice and weight loss. We went home and she was admitted to the children's hospital 2 weeks later for pneumonia. She had severe reflux and lots of gas in her belly. At one point they thought she had a condition that would have required a colostomy. Once that was ruled out we started seeing specialists to treat conditions caused by her prematurity. We saw an ophthalmologist, a geneticist, a gastroenterologist, and a pediatric surgeon. We had her hips checked for dysplasia, and she saw a physical therapist for 8 months for torticollis. Hopefully her igg therapy will give us a break from the trouble.

When to get tested

You should be suspicious if you have an infection that is…

Severe – requires hospitalization or intravenous antibiotics

Persistent – won’t completely clear up or clears very slowly

Unusual – caused by an uncommon organism

Recurrent – keeps coming back or if it

Runs in the Family – others in your family have had a similar susceptibility to infection

At the meeting yesterday there was a question and answer session with an immunologist and an allergist. One of the questions was, "can I wean off of the igg replacement?" The immunologist's answer struck me and has been on my mind since then. He said that he had a patient whose parents chose not to give him replacement therapy due to the fact that igg is a blood product and it was against their religion. Over the course of a year he was hospitalized frequently due to infections. Eventually his parents were convinced that they should give him therapy. He died that day from an infection. The immunologist said that he felt it was the biggest failure of his medical career.

Low igg (hypogammaglobulinemia) is life threatening. I wish I didn't know that. I wish my baby was healthy, and that we never had to worry about illnesses. Fiona was tested because I thought she was sick too often. My aunt mentioned checking Fiona's immunoglobulins. I thought testing her was crazy. When the results came back we were shocked.


We found Fiona's immune deficiency because it was mentioned to us. Primary immune deficiencies are more common than you would think. It's estimated that 1 in 1,200 people have a primary immune deficiency. If you think you have a problem, please go get tested.

The Immune Deficiency Foundation

Christian and I went to an Immune Deficiency Foundation (IDF) meeting today. We got to meet other people getting the same treatment Fiona is on. It was amazing. Most of the people there were patients or spouses. We were the only parents of a young child with an immune deficiency there.

A college student was sitting at our table who was diagnosed with an immune deficiency when he was a baby. It was hard not to stare at him. It was like seeing Fiona's future. For the first time I realized that this will become normal, and that she can have a normal life.

We learned about a great opportunity to go to a plasma donation center. We can learn about the donation process and thank donors. I wonder if they even know what they are donating for?

We also got to meet reps from other medical supply companies. I feel better knowing that the exist just in case. We met the reps from two of the drug companies too. The kids were thrilled to see all of the pens, toys, and hand sanitizer we came home with.

This felt like a great next step in our learning process. I have lots of information to read and research. I'm so glad we went.

No more nurse visits

I did all of Fiona's treatment yesterday with supervision from the nurse. At the end the nurse said she won't have to come back. I'm looking forward to being able to do treatments on my schedule.

I was really nervous about sticking Fiona with the needle. It really wasn't as bad as I anticipated. It felt like popping a balloon with a pin. It helped that we used emla (numbing cream) and left it on long enough for her not to feel the stick.

Fiona had a mild reaction to the igg yesterday. The infusion sites were really swollen and didn't go down as fast as they did last week. She also got a rash around the sites. I wonder if the rash was from the tape and not the igg? She seems fine today.

We're still waiting to hear about the labs from Wednesday. I think they will probably come back okay or they would have called yesterday.

Waiting

We went to our Indy specialist today. Fiona woke up screaming last night, so we thought it would be good to make sure that we aren't missing something. The doctor is running labs and a respiratory panel. Now we wait for the results. If they don't find anything we will assume that she has serum sickness. That just means that her body didn't like the igg from one of her treatments, but she won't get it with every treatment.

Tomorrow the nurse comes to our house for Fiona's first at home treatment. We'll get one more nurse visit next week. I think I can do it all myself, but I'll be glad to have a nurse here to make sure I can. When they sent the supplies for treatments they were supposed to send an epipen. Instead they sent glass vials of epinephrine and syringes. I don't even know the dose to give her if something happens.

We are still trying to figure out which doctor to call when something happens. Yesterday I called our pediatrician first. The nurse thought I should take Fiona to the emergency room. Then I called Cincinnati, they said do not take her to the emergency room, it's too risky. Then I called the Indy office and got an appointment today. Next time I'll call Indy first. I'm always learning something.

elevated temperature

Fiona's temperature is weird, it goes up and down all day long. Her temperature hasn't really been normal since she got sick in May. This week she's been getting up to 100 degrees almost every day. Technically, 100 degrees isn't a fever.

Our Indy specialist says if she has a temperature above 102 we rush her to the hospital, tell them about her condition and ask to bypass the emergency room. Our Cincinnati specialist says that if her axillary temperature is above 100.5 we need to call them.

Sometimes I wonder if I'm thinking too much about a symptom and over reacting. We've never had a doctor tell us that one of our children has a life threatening condition before, so I'm not sure how to do this. Once our new normal becomes reality things will be better.

Today's treatment

The new treatment was very different. Our nurse didn't know how to program the pump they sent, but I figured it out. The pump is so small, almost the size of a deck of cards. The needles are tiny, about the length of a thumbtack's metal part and a litter thicker than a sewing pin.

This infusion went into her legs. Next week we will probably use her tummy. By the time the treatment was done Fiona's legs were pretty swollen. She's getting 7 mls in each leg, which is almost a teaspoon and a half. When we got home she slept for almost four hours. When she woke up the swelling was gone. She isn't eating like she does after IVIG. Maybe that will come later?

Next week the nurse will come to our house. The doctor told us that she would come for the first six weeks. The nurse said she will come for three weeks unless there's a problem. I think three weeks will be fine. It's not complicated.

I think the worst part of the treatment will be keeping Fiona occupied for an hour. She can walk around and play a little. It's good to keep her distracted, otherwise she notices that something is taped to her leg and she picks at it.

Overall I think it went really well. Much easier than IVIG. She was less traumatized. By the time the swelling went down she was back to normal. Next week will be even better because she will have more distractions at home than she had in the doctor's office today.

We met with the doctor for a few minutes and talked about an eventual diagnosis. She doesn't really have Severe Combined Immune Deficiency (SCID). Her's is a moderately severe type. They don't really know what to diagnose her with. They've talked about Common Variable Immune Deficiency (CVID), which is kind of a catch-all diagnosis. It doesn't fit well either because usually it's just one variable, and she has a couple. In the future they will do genetic testing to see if they can come up with something. Like I said, she's a unicorn.

Ten signs that you have a primary immunodeficiency

Here are the ten signs most doctors look for when they suspect a primary immunodeficiency.

1.         Eight or more new ear infections within one year
2.         Two or more serious sinus infections within one year
3.         Two or more months on antibiotics with little effect
4.         Two or more pneumonias within one year
5.         Failure of an infant to gain weight or grow normally
6.         Recurrent, deep skin or organ abscesses
7.         Persistent thrush in mouth or elsewhere on skin, after age 1
8.         Need for intravenous antibiotics to clear infections
9.         Two or more deep-seated infections
10.       A family history of Primary Immunodeficiency

Fiona had none of these.

The big box

There's a big box sitting on my kitchen table. It's full of medical supplies. I usually love medical supplies, but these give me anxiety. Suddenly this is all to real. It's easy to forget that there's something wrong in between treatments. The box won't let me forget today. I'm sure it will be fine, and that in a month this will be easier to think about.

To distract myself I'm thinking about how to organize all of the stuff. They send 4 weeks worth of supplies. I'm going to get a big storage container with smaller containers that fit inside. I wonder if I should organize it by the type of supply, or by what I will use in a week?

Every few weeks we find something new to think/worry about. One of our first realizations was the need to be near a great children's hospital. We also have to worry about the area we live in. For example, the northeast bears the risk of tick carried illnesses. We also have to worry about outbreaks of illnesses as she has very few immunities. Tetanus is a major concern, so we don't let her run outside without shoes.

In the box that came this morning there was a flier about natural disasters. It said to prepare for disasters that are regional. For us that would be tornadoes and flooding. While both are unlikely in our area, there is some risk. I'm coming up with a list of medications and supplies that she would need if something happened. I can't believe how many things I didn't have to think about 5 months ago.

Fiona is happily oblivious to all of my worry. She's talking into a vacuum cleaner tube and making her friend laugh. She loves to color. She doesn't hesitate to pick something up off the ground and put it in her mouth. She's joyous, and I hope she stays that way.

New design and new treatment

My lovely friend Rebecca gave us a pretty new blog. I love it! Fiona calls it birdy birdy, and asks to see it. It couldn't be more perfect for her.

On Thursday Fiona starts her new treatment. There has been some disagreement about when and where. Thankfully our medical supply company has demanded that it will be in our local doctor's office. Because there is potential for anaphylaxis, and this is a different formulation of igg I feel safer having access to a doctor. Next week's treatment will be at home.

Specialists are special

You never know what would expect when you meet with a doctor for the first time. Will they have great bedside manner, or act like a dead fish? Will they have amazing nurses and a great staff, or will they forget about you the minute you walk out the door? We have been spoiled by our pediatrician and the staff at her office. They are amazing.

Sometimes specialists are hard to reach. They don't return calls quickly. They have staff that can give you a generic answer. If you push for a better answer they say wait until your next appointment, which is usually two weeks away. They tend to drop bad news and run, leaving you to stew about it for weeks.

Sometimes scheduling a treatment is impossible, until they decide that it's an emergency, then it's scheduled for the next morning. When the next morning you might ask? They don't know. The hospital usually calls the next morning with a time.

We expected Fiona to start her new treatment this week. Our health insurance has approved it. The supply company has contacted us. We are waiting, as usual, on our specialist. I plan on calling his office today, but I feel like I already know what the nurse will say. We have an appointment next Thursday.

I feel a sense of urgency about getting this treatment started. Fiona's IGG is being metabolized and falling every day. Her IGG post treatment upper level will be lower with every day we wait. Maybe this time they'll surprise us.

The elephant in the room

I think about the possibility of a bone marrow transplant often. It would be a cure. No more IV treatments, and no sub-q. It sounds good to me. The doctor's have told us that her numbers aren't low enough yet. Her T cells are low, but she has good function. They are still watching her numbers.

What doesn't sound good to me is how much we would make Fiona suffer. Our family would suffer too. We've been told that she would be in the hospital for a few months, then outpatient for a few more. We've read the information they gave us. Life during and after the transplant would be difficult. The rules are crazy and complicated.

It's hard to know that there's a cure, but she has to get worse to be cured.

Tender mercies

Last night the medical supply company that will handle Fiona's treatment supplies and send the training nurse to us called. The rep assigned to us introduced herself and told me about her son. He had a bone marrow transplant for a severe combined immune deficiency when he was 5 months old. He's a freshman in college now. She was a great source of information. She gave me her cell phone number so I could call if I ever had questions or needed to talk. It was amazing to me that someone who has been through what we're going through was assigned to us. I can learn a lot about Fiona's condition, treatment, prognosis, etc, but I need to know about the experience, from a real person, who can understand where I am. She's been where I am.

Today I watched the video about Fiona's new treatment. I know I can do this, and I felt prepared to do it until I realized that every week I have to stick two needles into my baby. It was easy to hold her hand and comfort her while someone else was the mean person hurting her. Now I have to be that person. Ouch!

Here's a link to the super boring video about at home treatment. http://m.youtube.com/watch?v=u4mw5V-q_A4&desktop_uri=%2Fwatch%3Fv%3Du4mw5V-q_A4

She's a unicorn!

Fiona's condition is a primary immune deficiency. That just means that she was born with it. Most kids are diagnosed in the first three months after they are born because they get really sick. There is a common phrase among doctors, "don't look for zebras, look for horses." It means that instead of looking for uncommon illnesses look for common illnesses because they are more likely. We've decided that Fiona is a unicorn. She should be really, really sick. She should have been in the hospital with life threatening illnesses. She shouldn't be growing well. She isn't like most kids.

Finding her condition was tricky. At her 12 month checkup they did a CBC (complete blood count). Her white blood cells were low. That should have  been a red flag, except she had a sinus infection. Infections suppress white blood cells. Our pediatrician suggested that we repeat the test in a few months. Unfortunately that was the beginning of the winter long cold, so she never had a second test.

In May as I was packing for our vacation she got a fever. It came on high and fast, so I knew it was a virus. We left the next day thinking that she would be better by the time we got to Utah. That was the beginning of a 15 day fever. After a week we went to an urgent care clinic. The doctor spent two minutes in the exam room, declared it a virus and left. Three days later we tried another urgent care. Again we were told it was a virus. The next day we went to the emergency room. They gave her some juice because she was dehydrated and sent us home. I called our pediatrician's office and talked to the doctor on call. He thought she was dehydrated and needed an IV. I took her to primary children's where she got an IV. They did a test for viral infections and sent us to an infectious disease specialist. The test showed that Fiona had three viruses. The doctor said that was unusual, but that it happens. He gave her an antibiotic and said she was fine. He told me that he was giving us one of the happiest diagnoses that he could give.

We came home, and the fever was gone. I mentioned something on facebook and my aunt suggested having her immunoglobulins checked. When our pediatrician told us that all of her immunoglobulins were low I was surprised. We saw another infectious disease specialist here a few weeks later. He told us that she had hypogammaglobulinemia. He said it was very serious and that her IGG was very low. He said any illness could be life threatening. He said to be prepared for a $60,000 a year treatment. Then they did some blood tests and scheduled her for another appointment in two weeks. I called my husband and told him it was bad.

For two weeks I cried. We were devastated. Our baby was sick. She was going to need an IV monthly for the rest of her life! We had so many questions. We wanted to put her in a bubble, and so did our pediatrician (have I mentioned that we love her?). Our next appointment brought us some answers. Her condition was worse than we thought.

Future blood test would show that she had low T cells, low NK cells, and low B cells. We were sent to Cincinnati for more testing and a bone marrow transplant evaluation. When her first labs came back there were so many low numbers. I spent two weeks learning what they all meant.

This all seems like a blur now. IVIG has helped a lot. I have a cold, but for the first time ever Fiona didn't get it. We are still trying to figure it all out.

Her last appointment

For a few months people have been asking how Fiona is doing. We have been talking about starting a blog to keep everyone updated, and to help explain what this means.

Fiona has a combined immune deficiency. Her B cells and her T cells are defective. Her defective B cells cause her immunoglobulins to be low. Her immunoglobulin G (igg) is very low. The fix for low igg is replacement via IV(IVIG) or subcutaneous(sub-q ig). Over the past two months she's had three IVIG treatments. Next week she will start sub-q ig. The fix for her T cells isn't as simple. The only fix is a bone marrow transplant. Her numbers aren't low enough to justify the risk, so they are monitoring them for a few months to see what happens.

At her appointment in Cincinnati yesterday she had IVIG. They doubled her dose because her igg was dropping too low between treatments. They didn't give us much new information. We did find out that her low T cell count is not a result of viral suppression. That sounds like good news, but it's not. It means that the problem with her T cells won't go away on its own.

At our next appointment here she will have her first sub-q ig. After that we will do sub-q at home once a week. The advantage of sub-q is that her igg levels will be more consistent.

We've had a few people ask what they can do to help. Fiona's treatment is derived from plasma donations. If you have time, please go donate plasma.