Friday, January 31, 2014

pharmacy drama

I called Accredo yesterday to ask about a payment plan. They advised me that they don't have payment plans, and that until they receive payment in full they won't ship more Hizentra. Our first bill isn't a problem. We can pay for it with the donations we've received. The next bill will be more challenging.

I wish I could talk to the person that made this decision for us. Biorx has payment plans. Switching isn't in Fiona's best interest, and eventually it could impact our ability to get her treatments. Saving a few dollars on our health insurance isn't worth risking her life.

Thursday, January 30, 2014

new needle sets

It's really sad, but getting a box of medical supplies was the highlight of my day. Accredo sent me the needle sets that was been using on Fiona before the switch. I think that having something more familiar may make a difference in her treatment. They also sent some new clear window dressings to see if her skin is less sensitive to them.

Wednesday, January 29, 2014

First bill

Our first medical bill for this year came. Fiona's hizentra for the month was $2,795. We are very thankful for the donations we've received to pay for her medicine. Because of her treatments she hasn't been sick at all this winter. Last winter she had a cold from October to April.

Fiona had a mild reaction to her last infusion. I think she may be allergic to the butterfly shaped plastic part of the needles we used. Her infusion sites (where I poke her) also leaked a little. Just like last week one of the three tubes connected to the needles didn't allow the liquid to move through as quickly as the other tubes. Accredo is sending us a different kind of tubing to try next week.

We go back to our local doctor next Monday. At our last visit when we told him about her pain during infusion he said that continued pain throughout the infusion wasn't typical. He mentioned placing a central line and going back to IVIG. I'm not sure we're ready to do that yet. Central lines are notorious for bacterial infections. If

Fiona's fundraiser

Monday, January 27, 2014

difficult infusion

Today's infusion was hard for both of us. Fiona is developing anxiety about being stuck. She's in pain during the infusion, which makes her fussy. I'm ready to go back to IVIG. I didn't have a problem sticking her until this time. She was shaking and crying and trying to grab the pin needle to keep it away from her skin. After about an hour she calmed down, but if she moved she was in pain again.

Two of her three sites leaked a little. I don't like the new tubing that they send us, it doesn't allow the liquid to flow evenly into the three needles. One of the three is always slower than the other two, so two of her sites get more medicine that the other one.

We go back to her local doctor in a week. I'm going to talk to him about other options. Hopefully we can come up with something that works for her.

Fiona's fundraiser

Tuesday, January 21, 2014


The box was delivered at 3 this afternoon. After going over how to work the new pump I started her infusion at 4:40. It took 3 hours, our normal time is half that. I think there may have been a problem with the tubing, one of the three tubes that feeds the injection site was moving slowly when I primed it. I'll call and ask about it tomorrow.

I like the new pump. It doesn't force the liquid through the tubes with as much pressure. I think it will be better for her in the long run. My only complaint is that it's huge. Her old pump was a little bigger than a deck of cards. This pump is the size of a carton of eggs.

There was a mix up about the dose of hizentra. We were sent 3 grams per week, but she takes 5 grams. They've cleared up the problem and are sending more bottles. Accredited will only send one size of bottle, so I have 5 bottles for each treatment. Filling the syringe takes a lot longer than it did with a 1 gram and a 4 gram bottle.

I'm glad to be done fighting to get her medicine.

Fiona's fundraiser

weather delay

The weather is causing a delay with the order being delivered. When the nurse tracked the package it said out for delivery. The nurse who was supposed to visit today can't because of the weather. She is going to call and walk me through the first setup. I'm not sure the shipment will be here before she calls. I think this is an opportunity to develop patience. The saddest thing is that Fiona keeps bringing me the box that I keep her treatment supplies in. I think she knows that she feels better after her infusion.

Fiona's fundraiser

Monday, January 20, 2014

Hizentra tomorrow

Fiona's Hizentra should get here tomorrow morning. A nurse is coming at 11am to show me how to use the new pump. The only unsolved problem is that the prescription is for 3 grams, and Fiona needs 5 grams. The pharmacist commented that even at 3 grams she is above the recommended level for her weight. I told her that at 3 grams Fiona's immunoglobulins were too low. She metabolizes the Hizentra too fast and with flu season near peak we need to keep her in the middle of the normal range. We're almost there!

Fiona's fundraiser

another delay

I called Accredo this morning to see where they are with Fiona's order. I finally got the direct number for the team that handles Hizentra. They said the claim for Fiona's medicine was delayed over the weekend because of our drug plan. So I called Express Scripts to find out what was holding up approval. Our drug plan allows a prescription under $100 to be filled without approval as a courtesy. Once they took that off the claim was supposed to be processed. So I called Accredo again to let them know. Now Accredo says that the claim is in adjudication. They said the insurance company has to tell them how much to charge us. I'm so tired of calling them. The pharmacists know my name. I'm sure they are just as sick of this as I am. The last call they said to check back this afternoon.

Fiona's fundraiser

Sunday, January 19, 2014


Friday morning I was desperate to get Fiona's medicine. It could have been shipped overnight and it could have arrived on Saturday. Now we won't get our order until at least Wednesday. That would be okay except this treatment is a major part of Fiona's immune system. I know I say this all the time, but she is worse off than an advanced AIDS patient. An AIDS patient still has functioning B-cells, and can take drugs to fight the virus that is suppressing their T-cells. Because a virus isn't causing Fiona's deficiency her best treatment is Hizentra. Delaying this treatment is putting her at risk.

Because of my desperation I called our local Walgreens to see if I could get a week's worth of Hizentra from them. While I was on hold I had the horrible realization that they would want us to pay for the prescription up front. I was trying to think of how I was going to pay for it. Then I remembered the fundraiser. Can I tell you how much of a relief it was to know that it would be okay. Thank you to all of our donors! Unfortunately, Walgreens doesn't carry Hizentra because they have a mail order infusion service.

Friday afternoon Accredo called and I was told that they needed to verify my information, and that they were working on the insurance approval. The woman I spoke with said that someone would be calling me to set up my order, and if they didn't have the pump I was used to they would also set up a nurse visit so I could learn how to use the new pump. I'm not thrilled that they didn't finish the order on Friday. Monday is a holiday, so my best chance to get Fiona's treatment will be a call to Cincinnati on Tuesday. At this point I wonder if Accredo will even have our order shipped in time for next week's treatment. Every time it seems like we are moving forward they seem to loose traction.

Fiona's fundraiser

Friday, January 17, 2014


I was getting really tired of whining about Accredo not doing anything. I'm not going to say anything more about today, except we finally have made progress thanks to a transplant coordinator at Cincinnati Children's and the manager at Accredo that I yelled at. I can't believe that I yelled at her. So embarrassing. Moving on. I think we will have supplies here sometime next week. I still plan on fighting to use Biorx, but in the meantime I'll take what I can get.

Fiona is pale again today. I noticed it when I was putting her in the car to take James to preschool. Speaking of preschool, the kind women who work in the office come down and take James in and bring him out so we don't have to expose Fiona to a crowd of children. We are lucky to have amazing people in our lives. Thank you all!

Fiona's fundraiser

new plan

It's unlikely Accredo will have our order here before Tuesday. I don't think they do Saturday shipping, and Monday is a holiday. If Fiona's treatment isn't here on Tuesday she will go to the hospital for a treatment. I'm not sure if our local hospital will have it. We may have to drive to Cincinnati. I feel better knowing that we have another way to get Hizentra for her.

Our rep from Biorx called last night. She is amazing! We went over the supplies I use for Fiona's treatment and she sent me a list with numbers so I can try to get the same things we are used to having. Thanks Lisa!!!

The best part of this is that Fiona is blissfully unaware. She plays and watches cartoons. She loves Curious George and Kai lan. She likes to put on James' power ranger mask and say, "I a power ranger!".

This week we are dealing with some sleeping issues. We are working to transition her to her own bed in her own room. It took her an hour of crying before she fell asleep last night. She is a fighter, and lately she's fighting sleep.

Fiona's fundraiser

Thursday, January 16, 2014

more Accredo disappointment

Every interaction I have with Accredo is more disappointing than the last. Today I had a 30 minute discussion with a man named James. He told me that they got Fiona's prescription three days ago. He is expediting her prescription, and we need to wait for the specialty Hizentra team to contact us. I'm calling our doctors today to see if we can go back to IVIG.

Fiona's Fundraiser

Wednesday, January 15, 2014

no infusion order!

I'm pretty sure this topic is getting old, but Accredo isn't going to be easy to work with. I'm sick of thinking about them, talking to them, and waiting for them to get our first order started.

On Monday Cincinnati faxed them a prescription. They faxed it to the number on the letter that said I needed to change pharmacies. They told me it would take 24 hours to process the fax. I called this morning and waited 23 minutes on hold to get a 2 minute answer. They can't find a record of the fax. They are calling Cincinnati to try to get the prescription. The woman I talked with asked what number Cincinnati faxed the first prescription to. I gave her the number from the letter, the one I verified with Accredo on Monday. She said she had never heard of that number. Way to undermine my confidence in your company.

When I got off the phone I googled Accredo complaints. There are a lot of them, mostly about them misplacing orders, not filling orders, or not mailing orders. I'm worried that they won't get an order to us by Monday.

Fiona started Tamiflu today. I feel better knowing that she has that extra protection. 

Tuesday, January 14, 2014

Immune system basics

I was just thinking that it's been a while since I wrote about the basic immune system problems that Fiona has. It would take a long time to read through all of the old posts, so I thought it would be good to write about it again. I also understand more that I did when I wrote the original post.

You are born with an immune system that can attack illnesses. It starts working right away. This is called innate immunity. Your immune system remembers the infections it has fought and can fight them off again, but in a better, faster way. This is called adaptive immunity. Adaptive immunity is the reason we get vaccinated. Our bodies remember the inactive or weakened viruses and can fight them off without getting sick.

B-cells protect your body from getting sick. They form antibodies that fight illnesses. There are three types of antibodies that B-cells form, IGA, IGG, and IGM. IGA protects your eyes, mouth, nose, lungs and intestines. IGG travels in your blood to fight germs. IGM protects your blood.

T-cells are another part of the immune system. They fight infections too. There are three types of T-cells, regulatory T-cells, helper T-cells, and killer T-cells. Regulatory T-cells tell your body when to stop fighting an infection. Helper T-cells tell killer T-cells where to fight, and they tell B-cells to make more antibodies. Killer T-cells kill germs.

Together T-cells and B-cells form your adaptive immunity. B-cells and T-cells are both called lymphocytes, and are produced in your bone marrow. Fiona's B-cells are defective. They don't produce as many antibodies as they should. Fiona also has low T-cell counts. Because of her low counts Fiona has very little innate, or adaptive immunity. If she is exposed to the illnesses we vaccinate for she would have very little response from her vaccinations, and her body would have a hard time fighting them off. The only fix for her problem is a bone marrow transplant.

The doctors don't understand why her immune system isn't working the way it should. They have tested for everything that could cause her low counts and everything comes back normal. We are still waiting to see if her genetic tests show any mutations.

Fiona's fundraiser

Insurance update

We've called the benefits department for Christian's employer 4 times in an effort to get an exception to use Biorx (our current specialty pharmacy). The first time they wouldn't talk to me because I'm not the employee. The second and third times the people Christian contacted were out of the office. The fourth time they told us that they don't give exceptions, and that we had to file an appeal with Express Scripts (our prescription drug plan).

I called Express Scripts 3 times last night. The first time my phone dropped the call and they didn't call back. The second time I was told that I had to go through Accredo (Express Scripts' specialty pharmacy) to get any information about how to continue using Biorx. Then I was transferred to Accredo, and they didn't know why I was trying to get any information from them because they are just a specialty pharmacy. The third time I called the woman I talked to was very helpful. Even though she didn't know exactly how to get the answers I was looking for she took the time to find the answers for me. The answer is that Accredo is the only specialty pharmacy that we can use and have Fiona's treatment and supplies covered.

I also talked to our rep at Biorx. She told me that they tried a test claim and it came back as zero pay. Express Scripts later told me that it had been rejected. One of the reps at Biorx did some research and found out that Indiana insurance code says that any healthcare provider (doctor, hospital or pharmacy) that is willing to accept the insurance company's terms has to be accepted into the provider network. I've contacted the state's department of insurance to see if they can help.

I used Fiona's last dose of Hizentra for her infusion yesterday. That means we have to order from Accredo to get her medicine for next week's infusion. I really wanted to avoid that. I'm disappointed that it didn't work out, and that it took more than a week to get the answers we are looking for.

The good news is that she is currently healthy. We can get her treatment. We have the ability to go back to an IV treatment in case something goes wrong.

Fiona's fundraiser

Sunday, January 12, 2014

keeping my fingers crossed

We've now talked to 3 people in benefits about getting an exception to stay with our specialty pharmacy. The first got some kind of answer for us, but wouldn't talk to me because I'm not the employee. The second told Christian that she would get back to him. He called two days later and she's out of the office for a week. The third is working on it. I hope we get an answer soon. We are down to the last dose of hizentra. I'm sure it will take some time to get a new order set up and shipped. I'm hopeful that we will have an answer on Monday.

Fiona's fundraiser

Saturday, January 11, 2014


It really does hit at the strangest times. I've been looking for a photographer to get some nice pictures of Fiona before the transplant. The thought of her loosing her hair kills me. I love her hair! When I think about the pictures I tear up. I don't know if I can handle the photo session.

Today at Trader Joe's one of Fiona's favorite employees asked where the little one was. It was hard to tell her. She remembered when Fiona was having stomach problems, and when she wasn't growing. She had tears in her eyes when I told her.

This is hard, the hardest thing I've done so far as a mom. It's funny how normal treatments, daily antibiotics, and lab draws seem now. I'm sure we'll look back in a few years and be glad when this is over. I'll be glad to send her to kindergarten with a normal immune system.

Fiona's fundraiser

Thursday, January 9, 2014

Ronald McDonald house

Next month we have two appointments scheduled. They couldn't be scheduled on the same day so we have to be in Cincinnati on two days. The Ronald McDonald house has a room available for us that night. I'm very thankful to have a place to stay near the hospital. I'm looking forward to seeing what it's like since it's an option for us to live there during transplant.

Fiona's fundraiser

Wednesday, January 8, 2014


As usual we are exhausted and overwhelmed by today's appointment in Cincinnati. Fiona doesn't have pancreatitis. Her labs from December didn't show any cause for her low lymphocyte (t-cells and b cells) levels. The labs that have come back so far from today show that her her lymphocytes are lower than last month. Unless something shows up on her genetic tests we don't know what is causing her immune deficiency. If nothing shows up on her genetic tests she will be having a bone marrow transplant. If something shows up on her genetic tests she will be having a bone marrow transplant.

I asked the doctor what was left as far as diagnosis. If her genetic tests show something that will be her diagnosis. If nothing shows up her diagnosis is unknown combined immune deficiency. When I asked what our course of action was he asked how we felt about a bone marrow transplant. This was the first transplant discussion that they didn't say, "we're not there yet."

We talked about how I think it is better for her to go into the transplant healthy. Because Fiona has full siblings who match her type of bone marrow completely there is virtually no risk to her. The chemo that they will use to kill her bone marrow is less harsh than what they use for cancer. I also told them that I would like for her to have a healthy, normal immune system by the time she's five.

Our only alternative to a transplant at this point is to wait. We wait to see if she gets sick and risk her going into the transplant sick. We wait to see if she has an autoimmune reaction and risk organ damage. We wait to see if her numbers get worse and risk opportunistic infections. Waiting is not in her best interest.

Her next appointment will be in February. We are taking Abbie and James (her sibling matches) to that appointment to be tested for the transplant. That is huge. That is the first step. This is becoming too real. I thought when we got "here" that I would be relieved. I'm not sure what I feel now. Dread? Maybe things will be better tomorrow after I sleep. At least the kids will be home tomorrow as a distraction.

The good news is that she looks almost normal, not so grey, not so pale, the dark circles under her eyes are getting better. She has more energy. I think it's a combination of a higher dose of Hizentra and starting amoxicillin.

I feel like we need a plan. Right now there are too many details to make a plan. We've talked about renting an apartment in Cincinnati. They told us today to expect to be in Cincinnati for 6 months.

Fiona's fundraiser

Tuesday, January 7, 2014


We just got a test back that shows a diagnosis of pancreatitis. I know what pancreatitis is, and I'm not sure that she has it. Luckily we are going to Cincinnati tomorrow, so I can get some clarification.

The benefits department called me back this afternoon to say that they couldn't talk to me they have to talk to Christian. I hate it when companies do that. I understand privacy rules, but I'm not asking for information about him.

specialty pharmacy update

I talked to our rep at Biorx. She told me to call Christian's employer and ask for an exception because they make the decisions about our healthcare. I called, and they haven't ever had an issue like this come up before. They are making some calls to find out what they need to do. 

I did some research and found out that the company that administers our drug plan owns the specialty pharmacy they want to make us use.

Monday, January 6, 2014

leaking infusion

I forgot to check Fiona's infusion sites after they started. One of the three sites leaked all of her medicine. That means she only got 2/3 of her infusion. I'll never forget again.

Sunday, January 5, 2014


Fiona's fundraiser

When Fiona was a baby a friend told me that sick kid's hair doesn't grow. I started noticing about 9 months ago that Fiona's hair was thin and wasn't growing. I didn't really think about it again until last week when I noticed a lot of new hair. I think her body was putting so much energy into fighting illness that there wasn't much left for things like hair. It's nice to see that her body is finally getting the help it needs from her treatments.

Try to ignore her messy face, she just ate breakfast! You can see her new hair in the front. The rest of her hair is about 5 inches long.

Friday, January 3, 2014

Our specialty pharmacy

I just got a letter from our insurance company saying that if we continue to use our specialty pharmacy that they will no longer cover Fiona's treatments. They are happy to assist us with changing to another specialty pharmacy called Accredo. I've never heard of Accredo. We have 2 weeks to make the necessary changes.

I trust our current company BioRx. They are amazing. BioRx has given us a representative who knows what we are going through. She is available to us anytime, and not just for pharmacy related questions. She is supportive and helpful. She looks for solutions to any issues that come up.

This is also going to change things about her treatment. We rent the pump used for Fiona's infusions, which will have to be sent back and replaced. I'm not sure if they will supply the same tapes and dressings as we are used to using.

BioRx has a payment plan, which I'm not sure if Accredo offers. This change may impact our ability to pay for Fiona's medicine. We may have to go back to IVIG and have a port placed if this doesn't work out. 

Because of the overwhelming nature of Fiona's condition this is distressing to me.Change and uncertainty are all we have. Using the same company is a small piece of stability that is important to my emotional well being. This is so not okay with me.


We saw our local doctor today. It was a great appointment. We talked about some of the things Cincinnati ruled out. Our local doctor told us that we are past the point of them being able to test and recognize a specific know condition. He said that Cincinnati has consulted with the National Institutes of Health (NIH), which is the government health agency. In terms of how rare Fiona's condition is, either she is the only case, or the first case they've seen. She doesn't fit any known diagnosis.

We realized today that the local doctor (infectious disease) is here to treat illnesses. Cincinnati is trying to diagnose her condition and treat it if possible. Having that clear distinction in our minds helps us know who to call when she gets sick.

In addition to bactrim and amoxicillin, both preventative antibiotics, we've been given tamiflu. Right after we left the doctor's office Walgreens called to say that they didn't have it, but that another location did. We were glad to find it since it's backordered. She'll start a preventative dose when peak flu season hits, she has first person contact with anyone who has the flu, or if she gets a fever.

We talked again about the low grade fevers and her general unwell appearance. His theory is that she is constantly coming in contact with viruses and bacteria, and this is her body's way of fighting off illness.

The doctor said that she is not allowed to go out until flu season is over. It's getting harder to keep her at home. There are so many things we want her to experience.

Fiona's fundraiser

Wednesday, January 1, 2014

more normal tests

Three more tests came back normal yesterday. One of them doesn't usually come back normal for kids with Severe Combined Immune Deficiency (SCID). Fiona is now more of a mystery. My hope now is that something shows up on the genetic test, which may not be back until February.

Our doctor called in a prescription for amoxicillin yesterday. I was hoping to pick it up yesterday so she could take it as soon as possible, but it wasn't filled in time. I'll feel a little better once she has that extra layer of protection.

We will see our local doctor on Friday. Since he's an infectious disease specialist I have a list of questions about avoiding illnesses this winter.