Pity Party

I hate Friday's. I hate them. It's so easy to pretend nothing is wrong when you aren't in a hospital full of sick children. It's such a slap in the face every third Friday when I'm forced to face reality. The adrenaline from diagnosis has worn off and I'm left with a broken heart that I like to ignore. In between the weeks I try my hardest to drown out my thoughts with mindless TV watching, knitting constantly, and keeping my mind busy with something else until the very second I fall asleep. This NEVER gets easier. I hate blogging too. It forces me to relive everything I didn't want to go through in the first place.

The doctor told me today that we only have five treatments left. FIVE. I feel like I should be so happy but.. I'm not. LCH is funny.. the prognosis is really high, and so is the relapse rate. I met with 3 other families a few weeks ago that have also been affected by LCH. Out of three, two have relapsed MULTIPLE times and the third just barely got done with treatment. There was a 12 year old boy there whose mom told me she's lost track of the relapses. Discouraging, huh?

Our doctors at the hospital say, "Well, it's not like a Leukemia relapse." That is such a punch in the gut. Relapse is DEVASTATING no matter the circumstances. If Avery does relapse she will most likely continue to respond to different types of treatments. The specialist likes to say "We have lots up our sleeves." But what difference does it make?

I feel like she has gone through enough. She is getting older and much more aware of what is going on.  Avery has now started to cry when they access her port. It breaks my heart that she feel like the doctor is "scary". How do you explain to a two year old that they are trying to help her? You can't.

I'm terrified to think about the long term effects that this disease as well as treatment will have on her. My mom tells me to not worry until we get to that point. But telling me that is like telling me not to blink.

I'm just so freaking tired of feeling like this. I guess all I can do is pray that He won't make her do this again.

Alright, I'm done with my pity party - for a bit of good news.

Avery is now eligible to receive a wish from the Make a Wish foundation. If you don't know who they are - look it up. It's the greatest club that you never want to join. :) In about 1 month Avery will be able to "declare her wish" at the Make a Wish building in Murray. She has been given a key and I've been told that it will unlock a door where she will be able to declare her wish to the Wishing Wizard. They won't tell us anymore than that. I don't think she really understands but I do know that she deserves this.

 4 children diagnosed with LCH even though it's considered a "rare disease"

 Avery in her Ariel costume :)

 Avery at her ballet class!

 I think this is supposed to be 1st position...? :)

 Loves being outside

 Playing with the kitchen during clinic

While kids are getting their chemo they can watch TV, play video games, do puzzles, read, play with toys, or eat popsicles! :) 

More tests

Yesterday might've been the longest day of my life. I set my alarm for 5:30 - which I ended up sleeping through and we rushed out the door at 6:37 AM (seven minutes after we were supposed to be at the hospital for check in time). We got up to the hospital in pretty good time and they checked us in to Imaging.

 Waiting with Grandma before they access her port. So so grateful that we had her there! It would've been so hard to do this on my own and she cut out basically her whole day so she could be there with Ave.

 Right before the MRI. This part is the hardest. She's dead asleep and it's so hard walking away from her. 

 Totally zonked out.

 This is the skeletal survey. They make her lay on a hard cold table (and I mean really cold - they have to keep the rooms freezing for the equipment) she has to be completely naked and they are holding her down so she can't move. The last two times we've had these she was practically hysterical - crying, shaking, kicking the nurses.. and they tell you that you can't be in the room because it makes it worse. So I requested this time that they do the survey while she was still sedated. It was sooo much better. She did wake up and cry but not nearly as bad as before.

After the skeletal survey they took us back to recovery so that she could wake up and eat some things. She is was so sweet while she was waking up. She wanted me to hold her the whole time and she kept talking about Mike and Sully from Monster's Inc! 

We had a few hours to kill before clinic so we went to the cafeteria and let Avery sleep a little before we got some lunch. My sweet sister came and met us to see Avery and it made Ave so happy to see her.

While in clinic the doctor came to talk to us about Avery's test results. And we got good news!! Her tumor is continuing to shrink and her bone is still healing! We are so grateful that she has continued to respond to the treatment she's on. After getting her chemo we got to go home at about 2:30. 

The rest of the day Avery was really subdued and just wanted to lay on the couch. She wasn't eating much but she was drinking and the nurses that discharged us after the MRI said that if she didn't feel like eating it was alright because they gave her so much fluid.

Around 5:30 I sat down by her and she felt really really hot - she had a fever of 102! Standard protocol when you have a port is that you have to go to the ER if you have a fever over 100.4 ever since the port is an infection risk. I couldn't believe that we had to go back up the hospital after spending 8 hours there earlier.

Telling Ave we had to go back up to the hospital sucked. She already felt like crap and just cried and kept saying it's not fair! It kind of broke my heart to hear her say that. It's basically the most unfair thing that a two year old has to go through all of this crap. I so wish I could do it for her. Plus after having tape on her chest all day from the port her skin was already really irritated and red. 

We got checked in right away at the ER because they were expecting us and the nurses came in to access her port. It was horrible because anywhere else in the hospital they don't really know what they are doing when it comes to accessing. They just don't do it as often as in clinic. The nurse made Avery lay down (which she really hates because she feels like she's being held down) and then when she put the tape on she put it on way too tight. So they had to take the tape off her already irritated skin and redo it. They had to take a blood draw from her port and for some reason they could flush her port but it wouldn't draw. We had to hold her down for a good half hour while they moved around the needle to try and get it to draw. It was so awful. Luckily after getting a lot of fluid and some tylenol her fever came down and her labs looked good so we were able to go home around 11:00. 

Good news is that Avery is doing much better today. No sign of a fever and she is running around and playing like usual. This girl is such a fighter!

Specialist

These past few weeks I've had an overwhelming need to try and connect with some other mom's or people who have what Avery has. From the beginning, our doctors have told us not to research LCH online... mostly because it would scare us. They were right! I became a member of the Hystiocytosis Association so I could connect with others that have this and do more research. There I found that the chances of having LCH is 1 in every 200,000 and even there there is such a wide spectrum that the chances of finding someone who has something similar is very rare.

There I also found that although the pronosis for LCH is very good, the chances of relapsing is also very high. Not only that but there are things called "late effects" or "permanent consequences" that affect patients with LCH long after they are finished with treatment. These can include neurological problems such as memory loss, stunted growth, learning and concentration difficulties, and higher chances of depression. A really common permanent effect of LCH is Diabetes Insipidus. A lot of tumors from LCH occur in the scull close to the pituitary gland and diabetes insipidus occurs from damage to that glad. The likelyhood of developing DI depends a lot on where the tumor originally started. 

On the Hystiocytosis Association's website there is a section where you can go and read about others who have LCH and read their stories. I went through and read most of them... and many of them had LCH reoccurrence 3 or even 4 times. 

This to me is so discouraging. I feel like I've been living in complete ignorance to what this disease can affect. I was expecting to be done after a year of treatment and that this all would be over. Just thinking that this could happen again is almost unbearable to think about. Even if we are lucky and Ave doesn't have to go through Chemotherapy again, it seems it's likely that she will have other problems to deal with. 

I've connected with another mom in Canada who's daughter is 2 and also has LCH. She expressed to me that there are no specialists and almost no doctors who have dealt with this before. Our oncologist is not a specialist in LCH but there is one where Ave is being treated. I felt really strongly that since we have this specialest that is available where Ave is being treated anyway that we should meet with him. So while we went in for treatment this last Friday we were able to meet with an LCH specialist. Thomas and I wanted to know if extending Avery's treatment longer then a year would lessen her chances of relapsing. Or if the risks of extending her treatment would outweigh the benefits. 

While meeting with him, he basically told us that there isn't enough information to know if having two years of treatment versus a year would be better. Not too long ago, they did a study where they randomly picked LCH patients and treated them with a six month treatment plan and some with a full year. The only difference was that the patients with the longer treatment plan were much less likely to have an LCH reoccurrence. This is originally why we thought that extending Avery's treatment would lessen the likelihood of her relapsing.

We knew that Avery had single system LCH which means that it was only found in one part of her body. Multi-system means that LCH is found in the bones and the organs. Avery's tumor was in the skull and had a soft tissue component to it. Because of where it is and how aggressive her tumor was, Avery is single system with risk of central nervous system involvement and has a higher risk of developing diabetes insipidus. 

We also learned what what our doctors had been telling us previously that if Avery does relapse, it will come back in the same place, was completely false. After talking to the specialist, he told us that if it does come back, she could develop tumors on the top of her skull, in her legs or arms, or even develop into multi system if it does in her organs.

Although living in the dark can sometimes be easier, I'm glad we met with the specialist and that I'm at least informed. It makes things scarier but not knowing is always worse.