Surgery Stay Day 27 - Appointment with the Bone Marrow Team

Ariauna had a bit of a rough night last night. Not sure why, but she was moaning and groaning a lot in her sleep. I think her tummy hurts, not necessarily from surgery but because things are not moving through there like they should. She was up at 6:00 a.m. this morning wide awake and ready for the day to start. The surgical team came in at 6:30 and she screamed BOO and scared them. It was pretty funny! Not much new, just continue to up the food slowly and they checked her incision to make sure it was doing okay. Ariauna continued to have a rough morning. They turned her food up to 20 ml's at 8:00 a.m. and she continued to feel nauseated. Poor girl. Some how we have got to get her tummy used to food again. Unfortunately, it has not had food for 5-6 weeks so it has forgotten what to do with it.  At 11:00 Ari wanted to go down to the play room. Home Depot was here to do one of their building projects. Ari chose to make and paint a bird house. She had fun, but as soon as she was done she was ready to come back to her room and get hooked back up to her NG tube (suction). I was sick to my stomach all morning. Today was the day to meet with the bone marrow team and I was a nervous wreck. Ari and I spent some time playing the card game 7-up for awhile before it was time for the appt. Jerry and grandma and grandpa came up to go to the appt with me. We had a volunteer from the kid crew come and stay in the room with Ari and Jaxon. We met in the conference room and we waited and waited for what seemed like forever. Finally a nurse came in at 2:30 and said that Dr. McManus was waiting for the blood tests on the other siblings and as soon as it came she would be right down. Finally the Doctor came along with Dr. Eldridge from hematology (she is the fellow we have been working with about Fanconi's). The meeting was basically to tell us what are options are, which is pretty much to do a bone marrow transplant or Ariauna will not live. They can not guarantee how long she could live without one, but the longer they wait the more chances she has of getting leukemia or some other cancer. The doctor was excellent in presenting the information in a way that was not too overwhelming. Of course, I was overwhelmed and a complete basket case about it all, but for the information she had to give us, she did a good job. With Fanconi Anemia some people can live a long healthy life and never know there was anything wrong. Other's their bone marrow stops working and they can go down hill very fast. As much as I do not want to say this, I am grateful that we were admitted to the hospital for Ariauna's intestinal surgery. Had it not been for that, she could/would have gotten very sick and then it may have been to late for the option of a transplant.  I guess God really does work in mysterious ways. Even though a surgery seems like a weird blessing, in reality it really was.  The next thing the doctor told us was that our other 3 children, unfortunately, are not a match to be a donor for Ariauna. My heart was broken, I could feel the tears start to stream down my face. So many people have been praying for us, our ward has fasted for us, I have prayed and prayed and prayed that one of the kids could be a match and yet...... They are not!  So the next step is to find a donor. They had already done research from the bone marrow transplant donor list and there are a few donor's that the preliminary results show they are a partial match, but not an exact match. They can try to do the full work up on those donor's to get the complete numbers and the other option is to use a cord donor. They actually found several of those that would be a really close match to Ariauna. There are benefits and down falls to a live donor as well as to a cord donor. A cord donor is blood stem cells that was collected from an umbilical cord and then frozen. Dr. McManus is thinking the cord donor is a better option for Ariauna. They will immediately start the full work up on the cord donor options we have. The benefits of a cord donor transplant is that the cells are so young and naive that they have a better chance of attaching to the bone marrow and doing their job then a live donor's cells. With a cord donor there are less chances of getting Grafts-versus-hosts disease because the cell's again are young and naive. One of the top reason's they like the cord donor is that the chances of rejection are less because they have not had years of illnesses, sicknesses, ect. The down fall to a chord donor is that it can take longer for them to attach and for the body to start making cells on it's own. The next part of the appt was to discuss what will happen. Right now they will start with getting the Insurance end taken care of, doing full work ups on the donor's that could be a match. All of this will take time, they are assuming the transplant will take place in 2-3 months. In the meantime they will start running all sorts of tests on Ariauna to get a baseline on how all her major organ's are working before the transplant so they know if there is issues after the transplant to any of her organs. They will be checking her kidney's, her liver, her heart, eyes, ears, etc. She will be looked over with a fine tooth and comb. Once they have everything in place they will have another conference with our family and at that point they will have everything ready including the donor. We will make sure that Ariauna is at a healthy point in her life that she can physically go through the transplant, they will then set the date for the transplant. As I have said before, Ariauna will be admitted to the hospital a week to 10 days prior to the transplant date and will start heavy doses of chemo. Most likely they will be putting a port in under the skin in her chest that all her IV's will run through. She will definitely be losing her hair as I mentioned in my previous post. The chemo that they will give Ariauna is different then most because of the Fanconi Anemia and she also most likely will not have radiation after because usually in Fanconi patients the radiation does more harm to their liver than it does good. The day they start the chemo will be considered minus days. The day of her transplant they will do a huge Bone Marrow "Birth" party and from then on out she will have 2 "Birth"days!  They will celebrate with balloons, presents, ect. That day will be considered day zero and then will continue to count up in positive days. They say that days 6-8 are usually when the sickness starts to hit the patient. They will become nauseated, won't want to eat, may get mouth sores, and that can last up to 3 weeks. Ariauna will not be able to come home until the cells are en grafted (meaning her counts start to go up on their own and the cells are grafted to the bone marrow and starting to produce on their own). Her white blood cells also need to be up high enough to go home, Most likely she will go home on TPN and lipids for nutrition because she still will not have much of an appetite. She has to be able to keep medicines down in her stomach before she goes home. She can not have any infections, and no graft vs host disease. They assume she will be in for about 4-6 weeks. Once released we will have very strict instructions on what she can and can not do and there will not be many visitors allowed. They will have a home health nurse that comes to the house and then she will be seen in the clinic twice a week for awhile. On day 100 they will lift a few of the restrictions and at that point she may be able to go outside in our own yard in the shade, may be able to eat something other than home cooked meals, may get a few more visitors allowed, ect. She will be immuno compressed and will not be able to go to school/church/big events for about a year.  If everything goes well with the transplant then she could live a long healthy life. She would have higher chances of getting certain cancer's, but would be monitored closely for them. Most of the time the chemo will make them infertile so she will probably never be able to have children. Before we go in she needs to have any dental work done that needs to be taken care of. About the time she hits a year from transplant she will have to have all of her child immunizations done over again. In time we will have to have our other children tested for Fanconi Anemia. We will actually make an appointment and take them in to the Hematology unit to have a physical and be tested. There was so much information given to us today, I am overwhelmed, scarred, heart broken for my little girl and just sick about it all. I think I am going to need some time to let it all sink in. I never in the world imagined something like this happening to one of my own little babies. Ariauna is a fighter and I hope and pray she can fight this just like she has every other obstacle in her life. We spent the rest of the afternoon visiting with daddy and grandma and grandpa. After they left, Ari and I just layed around and I held my baby a little closer to me. I am so grateful for her, for her sweet spirit, she is much stronger than I am. My heart is so full tonight. I can not thank everyone enough for the prayers, the fasting, the emails, texts,  cards, gifts, visits and food for my family at home. Every little and big thing that people are doing for us is a huge help in us making it through this trial one day at a time. I know my prayers may not have been answered in the way I was hoping, but I know each and every prayer being said in our behalf is helping Ariauna and helping our family to make it through each day. May our Heavenly Father bless each one of you for all you are doing in our behalf. We love you all and are so very grateful.

            The crazy ways Ariauna likes to rest

            The Home Depot wood bird house project

     Our beautiful little girl, finally clean tape on her face

            Jaxon going on a walk with Ariauna