My Little Guy

This is my wonderful baby boy Ryker. He is 2 years old and the most amazing little guy in the whole wide world. He is my rock. He finds humor in everything, loves to make everyone laugh and has the best personality EVER. I wish I had a few of his traits....well as many of you know Ryker has always been a little different. His voice has always been hoarse and grawly, almost like if you watch The Little Rascals, he sounds like Froggie. Its just always been Ryker, but as time went on Chase and I started to become worried because it wasnt going away. He talked like this all the time, never giving his vocal cords a break and if any of us even tried to mock him we would have to cough or clear our throats. This just wasnt normal. He also has a very large bottom lip that we know wasnt hereditary....This thing has always been pretty big. Strangers or people that didnt know him very well would often ask me if he had fallen on it or if he had a cold sore or something....nope.....thats just Ryker. I have taken him to his pediatrition many times, and his ENT, no one ever had an answer. The reason why his lip was big is they were telling me that he chews on it....well I've never seen him chew on it but ok. Then the excuse for his voice started out as he was forcing it, just making his voice do that. Then I changed ENT's, he thought Ryker had acid build up on them, he scoped them and nothing.

As frustrated as I was, I just thought I needed to see if he would grow out of the voice thing so I waited almost 6 months and nothing was changing. The only thing changing was Ryker was starting to talk more and we were hearing this grawling voice more and more. I changed ENT's a third time and went to Dr. Heidi Heras (who I absolutely recommend) she was the first person who sat there and listened to him talk for quite a while, and was so interested in him that she wanted an answer, right then and there we made an appointment to place tubes in his ears, take out his adnoids, and do a throat scope to check for anything that might be causing this voice of his. She also wanted me to see someone a little more familiar with this sort of thing so she recommended me to an Oral Maxillofacial Doctor named Dr. William Mcbee. We got in to see him about a week later. At first he was more interested in his bottom lip and looked very closely at it and at first he had decided that it was clogged saliva glands and for me to bring him back in 2 weeks or so and we'll see if it looks any different. So in 2 weeks I take him back and Dr McBee has what he thinks another idea as to what it could be. He spoke to a lot of different pathologists and they think it could possibly be Granulomas, and they wanted to do a biopsy of his bottom lip. I told him about Dr Heras doing the tubes and everything and since he was already going to be asleep he was going to have her do a small biopsy for him so we could get this figured out.

March 15th comes around and its Rykers big surgery day! He was such a trooper and did so well....all except for the part he had to wear that gown...he was not having it!! They placed tubes, took out his adnoids, put a scope down his throat to look at his vocal cords and did the biopsy. Then we got to play the waiting game. I wasnt getting the results back for 2 weeks. The stress begins! I had to wait till April 1st to get in and see Dr Mcbee!!

March 31st I get a call from his office saying I needed to bring Ryker in so they could take more pictures of him, I asked if they had the biopsy results were back, the nurse said she would ask the Doctor when suddenly he gets on the phone.

"Hi Mrs. Cooper, this is Dr Mcbee, we got the results back, its not Granulomas."
me: "Oh well thats wonderful news!"

Dr:"Well okay heres the thing, the pathologist that did Rykers biopsy called me and the first question he asked me was does Ryker have an oddly toned voice? I told him yes, and he said alright then we have a diagnosis for you. Its something called Lipoid Proteinosis, its extremely rare in fact so rare that I dont think many pathologists would have caught it. "

me: "well then the voice and the lip are all tied together oh wow, so okay what do we do from here?"

He couldnt really tell me much over the phone, because he didnt really know much at all about this, he had never even heard of it...he wanted me to come in the next day as planned and we would talk more then.

So the next day I go in to the office scared out of my mind, the doctor comes in our room and the only things he could tell me were that this is so rare that Ryker will not be able to be treated here or anywhere close to here, in fact we dont even know where the study group is that studies this disease, but we're going to find it. Its so uncommon that it normally only happens when parents of a child are related.(Which in fact Chase and I are not!) Its so rare for 2 people to have this same gene that makes this ECM1 gene that this is probably going to be the medical discovery of the century, not only that but Ryker also is the youngest person to ever be diagnosed with it. He told me to go home and do some research and he would do the same and we would meet back in a few weeks and compare notes. He also took pictures and a video of Ryker that the pathologist wanted to show at a conference in May. So as of now we have no answers, nothing. Just that Ryker is a very special little boy which we always knew. Its just so frustrating that no one could give me any answers. All I know is what I have read online and off a few papers that the pathologist had faxed in, which are very scary. It basically said its the head and neck disease. It messes with everything, your brain, voice, face....The brain one scared me really bad, it causes severe epilepsy and mental retardation. It said that it mostly messes with their temporal lobe, which controls your emotions. Anyways, I'll let you know more when I find out more but as of right now I know nothing. Everyone I believe that everything happens for a reason, and there is a reason I was handed this. Please pray for Ryker now as you all did for Livi. I have very special kids and I wouldnt ask to change them for anything.

Utah US Continental Pageant

What a night! Oh my goodness I just want to start out and thank all the people who helped out that night. It all was an amazing surprise. They all surprised us by playing Alivia's video (located on the bottom of the page) and having Livi be their special guest for the evening. Livi got a beautiful crown and a sash that says PRINCESS ALIVIA on it. They also had an entire page in their program that was dedicated to Livi, saying all about the day she was diagnosed and where to donate in her name. It was seriously the most amazing surprise ever. I was balling of course and so was everyone else in the auditorium!

Here is Livi with Miss Utah Teen USA and Mrs. Utah teen USA, they absolutley loved her to pieces!

Here she is yelling at me because this was her stage!! What a goof....Then her and Kynzie danced the night away during intermission. They were quite the entertainers!

Her special job was to escort all the contestants to their place, she did a wonderful job!!

The three women who made this whole night possible. I would especially like to thank Mrs. Utah US continental Keara Wright for again touching our lives and helping us out so much. You are all truely amazing and I could never thank you enough for the joy you have brought to my daughters life!!

Maintenance Appointment #2

Livi was not so happy to be at clinic today, I didnt get many pictures because she kept getting mad at me....steriods for ya!!

What a wonderful feeling! Month #2 of Maintenance...Its been pretty easy so far...Livi hasnt been too sick. She gets sick after she takes her dose of Methotrexate which is every Friday then after clinic of course. This last time she was begging for a burrito after we left so we stopped to get her one. Well when we pulled her out of the car she threw up all over...no burrito for Livi. We went home and she slept the rest of the day. I though getting just one dose of one kind of chemo would be so much easier, but apparently I was wrong. Its fine though nothing we're not used to. We also got to start steriods again...Ohhh Myyyy Goshhhh.....It so hard to just look at your little girl while she is screaming at you at the top of her lungs and just say "I love you honey.." Then its hard for little brothers who have to be subjected to it as well. Ryker this last time ended up with a huge bruise on his face from his sister biting him. Steroids for ya.....I try to just keep them away from eachother and to just be super calm with Livi because she has no idea why or what is happening to her.

She went to school a few times this month too! She was so excited to see everyone! It makes me so happy to see her doing so well. When people ask me how shes doing I just want to burst in to tears because I am so grateful of how lucky we are...we really got handed the most horrible thing you could possibly imagine and to have our daughter handling it so well has been a huge blessing and I am grateful every single day. It just teaches you to not take anything for granted, to live each day to the fullest.

Photo Shoot!

What an amazing day this was. Livi loved every second of it! She is always in the mood to get her picture taken and even though it was freezing outside and I would wrap her in a blanket in between pictures, you cant even tell she is cold. Heather you did an absolutely amazing job! Thank you so much!!

I wanted to get pictures taken of her before her hair grows back and have them be absolutely extraordinary, so I called the right person! I guess just that out of all the horrible things that this little girl had to go through the last little bit, her being bald is the only thing I want her to remember. She rocked the bald head! Now that it is starting to grow back I am going to miss it. I went back and read my blog post about how awful this part was going to be, and really it was so amazing. It never once bothered her, she never cared and no one else ever did either. I must say though it was nice to get a break from doing her hair everyday. Just grab a hat and go!

A huge thanks to Heather Telford for these wonderful pictures!! You have given us the opportunity to take this memory and save it forever!

Best Day in a Long Time?

Maintenance....we have been waiting a long time for this, been through hell and back and just trying to keep our head above water from day to day. It feels good knowing that we're going to start having a little more freedom. But I'm also scared out of my mind not knowing what is in store for us next. In maintenance Livi will only be seeing her oncologist once a month, she will also be getting chemo just once a month....the chemo she has been getting once a week for the last 6 months. This scares me. Why do you ask? Well without that horrible poison keeping the cancer away there is a chance that it will come back. Relapse....this a word I hope I never hear anyone say to me. Im excited for Livi though. Counts will go up, this means hair starts growing back and she will be able to start going to dance and school again! Start playing with her dear friends again!! Im so excited for her. I will not let her know what a nervous wreck I will be though. It also scares me only having her ANC checked once a month.....what if its good one month so I let her go and do.....then when they check it again and its super low!!?? I will feel horrible for exposing her to all the germs! I hope after a while everything will just sort of stick to a good count and we wont have to worry about that.

What is Maintenance??

Maintenance (consists of repeating cycles lasting 12 weeks each, lasting 2 years from the start

of Interim Maintenance for girls and 3 years from the start of Interim Maintenance for boys)

Vinc: IV push/ Once every 4 weeks

Dexamethasone: PO (Orally, twice a day)/ 5 days each month

Methotrexate: PO/ Once a week

6MP: PO/ Days 1-84 (every single day of Maintenance)

Methotrexate: IT (LP)/ Once every 12 weeks

First Maintenance appointment!! Way to go Livi! You did it! 6 Months of horrible horrible courses and you made it so far! We are so proud of you our little angel!!

I <3>

Here is Livi with her pill tray...Yes I actually acquired it from my grandma...haha not really but you would think!!

Here's all the perscriptions Livi got sent home with today. They consist of 6MP which is a chemo pill she will be getting every day for the next 2 years, Dexamethazone....dundundun...yes the lovely steriod yet again....she's only on it for 5 days a month though...feeeww! Then the septra which she will also be on for the next 2 years but only takes it on Mondays and Tuesdays. Then she was put on Acyclovir because the poor thing can not get rid of those stupid mouth sores!! Last but not least Methotrexate, she will be taking 5 of these pills once a week.

Snuggling with her daddy :) Daddy's on the phone....

ATTENTION ATTENTION PLEASE!!! If you will please notice...you probably cant because I have a horrible camera but there is peach fuzz growing on this cute head!! Im sad to see the bald go. Is that weird? I feel like I didnt take enough pictures!!

Steriods :) New favorite is KFC

And corn dogs of course. This girl could eat us out of house and home when she is on steriods, No joke!!

Clinic Stats:

Weight: 18.4

Height: 101.5

ANC:800

Another awesome story, we had Livi's ANC checked 2 weeks after she ended DI. It was 1000! After being 400 throughout the whole course we were really happy. So when I had it checked the day before she started maintenance I didnt think twice about it. Here we are on Tuesday January 24 getting up at 6 am to drive up to clinic. Livi's appointment was at 8 because she had a scheduled LP. We are so excited, in the best mood ever, got up there and before we even get called back in to a room a nurse walks in to the waiting room and sits next to me with a piece of paper: her counts. The first thing she says to me is "Did no one call you yesterday?" with a really queezy look on her face.... Immediately I said "No, but her counts were great last week and she hasnt gotten chemo so I just assumed they were fine." She looked at me with a very symathetic look, "her ANC is only 600 and it has to be 750 to start." AHHHHHH!!! What?? And no one called me??!! Oh man was I mad, but I didnt want to make too big of a scene so I just walked up to make an appointment for the next week......Seriously though? That was crap!!!

Daddy's Surgery :(

This last week Livi's dad had to have surgery on his rotator cuff and his Labium cuff. They were completely torn from the bone, and he also had a piece of his bone floating around that had broken off at one point.....This bone was probably the size of a quarter, it was huge!! The doctor let us keep it which was pretty awesome....... His shoulder is so messed up and has been for a very long time. We havent been able to get it fixed because we didnt have insurance for the last 2 years. Finally we got it done. He is going to feel so much better once it heals. It was horrible not being able to move his arm over his head, or bend down to grab anything or throw a ball with his right hand. It got so bad that one time it popped out of socket in his sleep! It was popping out of socket at least once a week.

When the surgeon got done and came out to get me and tell me he was done he just kept saying what a bad shoulder it was and how he really hopes this will fix it and Chase will regain mobility. They said he probably will never be able to throw a ball again which is sad, he wont be able to teach his son to throw a baseball or a football :( sad day!! I really feel for the guy. But luckily Ryker has lots of uncles who will be more than happy to play catch with him!

Chase is just trying to heal now, he had a block which lasted 24 hours but now that it has worn off and he's feeling it all, he's one sad guy. Its good to have him home though. Hopefully everything will heal well and he'll be able to go back to work soon! 3 months+no working=NO INCOME :((( Looks like mama will be going back to work soon for a while until Chase can....I dont want to, but ya do what you gotta do!!!

A Break From it All!

Livi had a 3 week break from chemo between DI and Maintenance, it felt so good to have life be some-what normal for a little while. Livi's counts were still really low so we couldnt really go anywhere but we played lots of Wii, colored, and lots of games. It was really lots of fun. After the first few days it already seemed as if Livi was back to her old self and I was hoping (and I still am) that it sticks.

About 3 days into her break though she did need a blood transfusion so back up to the hospital we go! She wasnt acting weird or anything, she was just sleeping ALOT. She didnt wake up till almost 11! In normal parent land you just think how awesome it is, and you get that extra time, well in cancer mom land you automatically jump to something is wrong. We got to go to Utah Valley Hospital for her transfusion which was nice that we didnt have to drive clear to salt lake. Man did they spoil her rotten!! She got 2 blankies, 3 new hats, coloring books and crayons, stuft animals....they dont see bald little ones around this hospital very often. Nurses kept coming in to meet her, everyone loved her to pieces! Who wouldnt??

Look at this little boy! He is such an amazing brother. He is always so caring and so loving towards everyone. He was such a good boy for the 5 hours we were at the hospital. Livi is so lucky to have such a wonderful brother and vise-versa.....I have the most amazing kids anyone could ask for!!