MRI day, results day and everything in between.

Friday May 8th Alex had her recheck full body MRI. The last full body was done on July 11, 2019. She has had three other MRI’s since then of individual areas but not the whole body. The primary reason for doing this was to be sure her left femur is still the only part of her body affected by this disease. We are getting closer and closer to making some major treatment decisions for her and a large factor in which direction we go is whether the disease stays on the femur or appears somewhere else. Alex is a superstar and so strong. Her January MRI was about an hour and 15 minutes long and she needed an IV for contrast and she completed It without sedation. The doctors and hospital staff were so proud of her and so were we. When she found out she was not getting to do this one awake she was nervous because she hates the sedation and the way she feels when she wakes up. She also loved that with the awake MRI I was able to sit in a rocking chair in the room with her and never left her. She was a little upset that they wouldn’t let me go back with her. The sweet nurse that was with us during the July MRI was with her again and that helped a lot. It was hard having everyone in masks because even those who would have been familiar to her weren’t recognizable so it took a bit of time to warm up to them.

They promised her that they would call me and have me by her bed as soon as her eyes opened and they kept their word. She does not remember waking before I got in there so that was a blessing. It was very hard to send her back with them. This was the first time she had been sedated that Brent had not been by my side. It was harder than I thought it would be. The nurses told me to go enjoy some alone time and I got teary. I realized I didn’t want alone time. I didn’t want to sit and rest. I wanted nothing more than to be busy and not have to think about my little daughter being sedated, what the MRI might show, or what it would be like waiting days for the results. This is also when I realized that I probably needed this time to think and pray and just be. It was so hard for me and I think I often stay busy to keep from having to deal with whatever I am struggling with. This is something I am working on. Taking time for just being and for prayer even when it is painful to live in the struggle but so good and peaceful to know that I am never alone, that God is walking all of this with us and even more than that He is not surprised.

Later in the day Alex told me about what they did when they took her back. The sweet nurse played eye spy with her in the room until she fell asleep. Eye spy is one of Alex’s very favorite games and we play it a lot around here. It was sweet of the nurse to do this and keep her happy right up to the moment her eyes closed. The nurses told me she smiled all the way to sleep. They told me what a precious girl she is. We agree of course.

They nurse even put a mask on her hare that she loves so much. Alex said she liked the smiley mask.

She really was wonderful. When she woke she got the sprite and goldfish she had chosen. She woke up really well this time. Usually she has some issues with nausea but she didn’t this time around. We were able to leave within an hour of her waking and she was almost back to herself by this time.

We are consulting with a doctor in Baltimore about a possible treatment option and were able to have a disc of this MRI sent to him that day. I was also able to bring a copy of the disc home as well.

We got home and she bounced right back. She spent the rest of the day playing with brothers and sisters.

Knowing that we wouldn’t get results from the MRI until Monday we were thankful for a wonderful weekend enjoying family in many ways. Saturday we went to Dayton for one cousins birthday party. We took the non interstate route to really enjoy the drive and it was beautiful. The party was a lot of fun and the perfect distraction. Boone found the kittens right away and loved on them any chance he got. We had to keep telling him they needed to go to their mama to nurse so they could have a break and he would go put them down with her. He adores all animals.

Alex and Pap Pap with food and a porch swing on a gorgeous afternoon.

Cousin silliness! These cousins love each other so much and enjoy every minute they are together. It was a really sweet day. Thank you, Stone, for turning 1 so we had an excuse to celebrate! 😄

On Sunday my family made sure it was a special Mother’s Day. They made breakfast and we had a special worship service with church family on zoom while we were sitting on the back patio. It was a beautiful day.

After lunch we worked in the yard and played. We got some of the garden planted, which is something we love doing together.

The children made beautiful cards and gave me a mason jar full of little notes with acts of kindness written on them that they were committing to do for someone in the home. So far they have done a great job remembering the things they wrote down. Brent made me this beautiful spinner for my garden and I love it.

We ended the day with dinner and a loud and silly game of Pictionary. I’m pretty sure Alex was our teams best guesser!

We made it to Monday. It was such a blessing to have a weekend where we were able to make sweet memories together while we waited for results. I know God has our girl and her story in His hands but it is still a struggle for me to not be worried as we wait. This was all playing around in my head over the weekend. I am working on finding the balance of caring deeply but also trusting completely and not worrying.

We had appointments with both our Orthopedic surgeon and Oncologist with time in between for some pizza from the food court. That is Alex's favorite treat of doctor day. At the Orthopedics appointment we discussed the results from an ortho perspective and were told that the femur looks stable and that the radiologist believe there may be some shrinking of the malformation away from the hip joint so that was very positive news. We were thankful to hear this. Alex has not been wearing her brace much because it is uncomfortable and limits her movement because it is not hinged. Also, because the lift is so high in the shoe the cast heal pops out of the shoe every couple steps and that is bothersome for her so it is always a fight to get her to wear it. The doctors have always said follow her lead on what she does or does not do because we are working with a lot of unknowns in this situation. They decided to make her a new brace and hinge the ankle and make the foot flat where it was slanted in the other so she was toe walking. They are going to be adding a lift to several pair of her shoes so she can have a permanent lift rather than something that is inside the shoe and causing the heel to slip. This should be a really good solution for her. There was talk about hinging the knee also but to do that her knee would no longer be protected by the clam shell brace and since all of her breaks have occurred just above the knee and her most fragile area is there they still feel it is important to protect her knee. If this option does not give her enough function they will try again with a hinged knee. So much about this is trial and error but we really do have a great team who care deeply for Alex and want to find the best solution for her even if it takes several tries. Alex got to pick out a new pattern for her brace and she chose butterflies for this one. She has loved her unicorns, ice cream, and donuts but wanted to pick something different this time.

We had limb length x-rays while we were there, which showed a 3.5cm leg length discrepancy at this point and also a discrepancy in the size of her feet. They believe this is not due to the growth plate being damaged but instead due to circulation issues caused by the bone marrow cavity being filled with these vessels and other issues caused by the disease. This was pretty much the extent of the consult there.

We had our lunch date while we waited for our next appointment with Dr. Borst in Oncology. This was scheduled as a telemed appointment but since we were already there she allowed us to go ahead and come up for a face to face appointment which was better for me to be able to make sure we got all of the information and all of our questions answered. Alex was getting a little tired by this point of the day so she was a bit grumpy during the appointment but all in all it was still good. The news we got from Dr. Borst was a little bit harder to hear. She agreed that yes the femur is stable and there is discussion that maybe there is some regression of the disease at the neck of the femur but nothing very noticeable. This we were happy with because not worse is good news. She then told us that they had been able to be much more confident that the area of signal they had seen in July behind the knee is in fact part of the disease and not due to the fracture. There had been disagreement between her and her team and the tumor board as to what that signal actually was. This MRI was a new higher definition machine and was able to get a closer look. She said without a biopsy there is no way to know for absolute sure but that is what they are agreeing it is at this time. Because it is muscle and because of where it is they are not able to get a biopsy to confirm. This is not considered a new location of the disease but is where the vessels come through the bone and out into the muscle in that one place. This wasn't happy news but we are thankful to have a more clear picture of this piece of the puzzle. The next bit of news we got was that they saw a 5mm signal on her right humerus. Because of it's tiny size they are not able to get a biopsy to say for sure what it is. She had several different radiologist look at the imaging trying to be able to have an absolute answer for us of what this place is but said that sadly right now they are just not able to tell us for sure. There are two possibilities. One that it is a new location of the disease in her body. If this is the case it would change her diagnosis from Gorham-Stout Disease or GSD to Generalized Lymphatic Anomaly or GLA. Both are vascular malformations and are sister diseases. Last September while we were in Cincinnati we learned that one of the primary differences of these two diseases is that GSD spreads in connected areas but does not skip around and show up other places in the body. They were diagnosing her with GSD because it was only found in the one femur and said that typically when they find as much damage as she currently has to a bone if it is going to be somewhere else as with GLA they also find it in other locations but with the rarity of these diseases there was no way to be sure unless a future MRI showed a new location. This has been a hard reality to deal with and why the waiting for these annual MRI results is so hard for us. With the presence of this new spot there is a possibility it is GLA but we have had to schedule a follow up MRI of the humerus for August 3rd to take a closer look and give it time to either get larger, stay the same, or go away. This bit of information was certainly hardest but we are choosing to look at the possible positive until we know for sure. The reason we are leaning more toward the possibility that it will be gone in August, and our doctors agree this is a good possibility, is that in each MRI they have seen what they call hot spots in other areas of her body. These have always been watched and each time the following MRI showed that place clear. The difference with this spot is that it does look some different than those places that have gone away with time. This is why they don't feel completely confident telling us that it is nothing.

We have a telemed appointment with a doctor in Baltimore Maryland on Monday afternoon to discuss a possible surgical treatment for her. This treatment option makes the most sense for her if we find out in August that this place on her arm is nothing to be concerned about but if we find out otherwise that could change our options. We were getting very close to making a final decision for treatment so this setback has been hard for all of us. It does feel like a series of steps forward and then backward, forward and back.

Her chemo levels are still not quite up to the therapeutic level even though we have raised the dose three times in the last three months. We would like it to be within therapeutic range but since the MRI showed a stable femur we are able to hold off increasing the dose for now. We are thankful for this hold. Each time it is raised she has some new challenge to deal with. The newest challenge is patches of psoriasis that cause itching and irritation. We are working on effective ways to manage this for her. We are thankful that she is finally beginning to gain weight again and almost back to her pre chemo weight. This has taken a lot of work, creative feeding options, and supplementation but it is working. She is also beginning to sleep better at night. She still wakes up saying she is hurting or has had a bad dream a couple times each night but is going back to sleep more quickly than she was before.

Please pray for continued peace and patience for us as we learn to wait for God's timing in all of this. Pray for clarity and wisdom as we continue to talk with doctors and make decisions. Pray for Alex to continue to be strong and peaceful as things change and as she deals with new challenges. Pray for the other kids as their normal is constantly changing as well and they are faced with responsibilities that would not typically be required of them at their ages. They have all been truly wonderful to her and to each other. We have enjoying watching them grow and mature through all of this. There are lots of hard things but there are many more blessings. We sometimes have to look a little harder to find them.

I was going to publish this last night because I have been working on it for several days and keep going back and changing things as I wrestle with what to say, how much information to share, what is more than you are interested in hearing, and just what is right.

This morning we started to get on zoom for church and there was some technical difficulty so we had about a 20 minute delay. We had already had some frustrating incidences this morning so we were struggling some already. We decided this was a good opportunity to go on a walk together. As we walked, pulling Alex in the wagon she said she wanted to walk and got out and took my hand. She has been showing some signs the last two days of pain but has not been willing to say she hurt. Today she was clinging to my arm with both hands each step and we asked if she was hurting. She said not very badly but we could tell she was. We told her to get back in the wagon and ride and she got so upset. She said she just wants to walk. She started getting teary and then of course Brent and I broke down. We told her we all want that for her and we are talking to doctors and working on it. We told her how strong and brave she is and how much we love her. She is overall so happy and rolls with everything but certainly has these moments that are so hard for all of us. Moments after that Boone was running back toward home and wiped out on the pavement so we got back to the house and got on the worship service a bit of a mess but happy for the opportunity to worship with church family in this way. The sermon was just exactly what we all needed to hear. It was about worry and prayer. He talked about Phil 4:6.

Do not be anxious about anything,

but in everything,

by prayer and supplication

with thanksgiving

let your requests be made known to God.

He talked about how we are called to pray in such a way as to specifically ask for the specifics of our needs. And with Thanksgiving and praise to our Father in heaven. This is how we turn worry to worship with prayer. Even though we have just asked and the situation has not changed we still find peace from these things. God calls me to rejoice and I cannot rejoice when I am worried. I am praying specifically today for healing for Alex, knowing that healing comes in many different ways and also praying for peace in God's will for my child and my families life. Is this the first time I have heard this? Certainly not and it won't be the last time I need to hear it but it was the perfect reminder today to pray this way and know that peace does not mean there is an absence of pain or tears. I am so thankful for these beautiful reminders of God's love for me and my family.