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.