On Schedule - Going Inpatient!

Many of you have probably been wondering how Ava is doing; thank you for your thoughts, love and prayers. It's been a little more difficult to connect over the phone with the different time zones/schedules. But we are grateful for the ability to still stay connected with you all.

There is a lot to update. Thanks for your patience.

Ava is doing really well. She is happy, playing and has been enjoying her time pre-transplant. She woke up this morning shocked and excited to see the sun out! (She has caught on to Seattle weather pretty quickly). *wink*

The baseline tests they did on Ava all came back normal: heart, lungs, kidney and liver functions; dental looks good. Praise the Lord! She did test positive for rhinovirus, as well as cytomegalovirus (CMV), which is not ideal. The presence of any virus is a concern, since she will essentially have her immune system obliterated, and it's crucial to have the cord blood graft into her system without complications. These viruses could cause pneumonia - which any of us could fight off in our healthy state, but not someone who has no immune system. In an immunocompromised person, pneumonia could be fatal. Thankfully, a recent nasal wash showed no presence of the rhinovirus (so hopefully that remains true through transplant) but the CMV doesn't go away and will be something to keep an eye on throughout the transplant & follow up. Ava also has HSV, which they knew... this is the cause of her eczema herpeticum outbreaks. Because Ava had a recent outbreak, they plan to keep her on Acyclovir (antiviral) for a full year instead of the normal month or so (?) for transplant patients.

Her bone marrow biopsy came back, revealing MRD positive with 0.007% blasts. Of the 3 tests performed, 2 revealed no disease, but the most specific one showed 0.007%. It is lower than her previous result of 0.03%, so the nelarabine last round did something. Still, being MRD negative going into transplant has been associated with the best outcomes. The doctors shared these statistics:

• There is a 75% success rate (meaning no relapse) in patients who are MRD negative going into transplant. (I don't know how long the follow-up period was here: meaning, 75% success rate in the first how many months/years?)
• This number drops to 40-50% success rate in patients who are MRD positive going into transplant.

The way they understand it, it's not the amount of disease/blasts present, but whether disease is present at all. While this is disappointing, we continue to trust in God, who is not bound by any statistic or probability, but holds the future in His hands. May He continue to grant grace, peace and faith for the journey.

So, consent forms were signed and we are forging on to transplant. Ava is on track with the schedule they outlined for her. Transplant is scheduled for next Friday, Nov 21. Today (Tuesday) is considered Day -10 (negative 10), counting down to her transplant day. Here is the rundown:

Tomorrow (Wed, Nov 12; Day -9): Ava will be admitted to Seattle Children's to receive an injection of Xolair to decrease IgE levels (which are related to the body's allergy response). They hope this will preempt/minimize any potential allergic reactions Ava may have to treatment.
Thu - Sat (Nov 13-15; Days -8,7,6): Ava starts high-level chemo for 3 days, through Saturday.
Sun (Nov 16; Day -5): day of "rest" - no treatment, but remaining in the hospital
Mon-Thu (Nov 17-20; Days -4,3,2,1): Four days of total body irradiation (TBI), twice daily. TBI will take place at a different location from Seattle Children's, so she will be transported via "cabulance" back and forth. They normally put children like Ava under general anesthesia for each TBI procedure, but they are trying to go without anesthesia. More info on that below.
Fri (Nov 21; Day 0): Transplant day!

So, Ava gets admitted tomorrow and will remain in the hospital through transplant and beyond, about 6 weeks assuming everything goes well. The only thing on the agenda tomorrow is getting the Xolair injection. They have never pre-treated someone with Xolair before transplant. So, they are dedicating tomorrow to the injection alone as well as to observe Ava and make sure she's doing okay. Note that Xolair is only used in patients with IgE levels under 1000; it hasn't shown efficacy in levels higher than that. Ava has had hyperelevated levels of IgE; the previous level they obtained was 22,000! But most recently her level dropped to 800, so they want to try using Xolair to see if this could further decrease her IgE levels and prepare her body to not mount an allergic response to treatment.

To elaborate on the TBI, it's imperative that the patient remains still during the 40 min procedure, which is why children are generally put under anesthesia. However, I found out that Ava recently had some type of reaction to anesthesia last week, I believe during her biopsy. Her oxygen saturation dropped to 80% and developed a wheeze about an hour into the procedure. This is the first time she has reacted to anesthesia. Her face/arms were a bit puffy afterwards, but she otherwise recovered quickly. Since Ava will get TBI twice a day for 4 days, this would mean getting anesthesia 8 separate times, not to mention all the hours she would need to be NPO before each procedure (having nothing to eat/drink). We need this little girl to get as much nutrition as she can, especially after chemo! Because of these above considerations, they are going to try having Ava get TBI without any anesthesia. She even had a trial run to test out laying still. Although Ava can lay still now, who knows how she will do post-chemo. Will she be able to lay still if she's feeling nauseous or otherwise unwell from chemo? She will have some protection placed over her lungs during TBI, but if she moves, her protection could be compromised. Please pray for successful TBI without anesthesia, and adequate protection of her lungs.

The doctors are very concerned about Ava; they have never been so concerned about a patient reacting to various steps of treatment. They haven't seen someone react as severely as Ava has. They want to be wise and cautious in preparing the cord blood itself as well. The cord blood is frozen in DMSO, which could potentially cause a reaction. An agent that they would use to "wash" the cord blood could also cause a reaction, so they are debating which agent to use. Of course, the physical delivery/transport of the cord blood itself is also important. We don't want to see any punctures/tears in the bag (or any damage to the cord blood itself). Please pray for God's hand to guide each step of the transplant process. We trust Him as the Great Physician. May He heal Ava fully, in whatever manner He chooses. 

You may recall that there is a study being done in Seattle, where patients are randomized to receive (or not receive) "expanded" stem cells during the crucial first month post-transplant. The thought is that receiving these extra cells would help boost their immune system during the critical time of waiting for the transplant to graft while avoiding infection. Ava was randomized, but was not selected to be in the group that receives this trial procedure. This was really disappointing to everyone, but we also remember that God's ways are higher than our ways, and His wisdom is so far beyond us. May we rest in His wisdom, for our "wisdom" is foolishness. We trust that He has what's best in mind.

The good thing is that the cord blood Ava will receive is pretty big - it has about twice the volume that a typical cord blood has. The doctors already felt that one cord blood would be sufficient for Ava's little body, so this extra volume is an added bonus.

An interesting tidbit: Ava's blood type is O+. The cord blood is B+. After transplant, Ava's blood type will become B+!

As you can imagine, there are many things to pray for. In summary, please pray for:

• no allergic reaction throughout transplant/treatment
• Ava to tolerate chemo well
• Ava to be able to remain still during all 8 sessions of TBI to avoid anesthesia
• successful transplant/grafting
• NO infection/complications
• Gwen - that she would feel adequately cared for when so much of these months has been focused on Ava
• Esther - I understand that her belly continues to grow! Please pray for safe development/delivery of Baby Boy Lee
• health for the whole Lee family, so no one will carry any new germs to infect Ava
• for the Lee family to be filled with God's peace and Sabbath rest on a most difficult journey; for courage and peace for Ava, the valiant warrior.
• May God continue to receive all glory and praise! He is good, He is good, He is good.

You all have played no small part on this journey. Thanks are not adequate to express the gratitude that Mike & Esther feel as they have seen you come alongside them, offering your prayers, love, support and all KINDS of amazing, tangible help and blessing. Thank you, thank you, a thousand thank-you's!

Life is an adventure, whether we want it to be or not. As we embark on this next rollercoaster ride, we trust God to go before us, hem us in from behind, meet us in the ups, meet us in the valley-lows, and cheer us on to the finish line, whenever that may be and however that may look. We trust Him to put a song in our hearts when we haven't a song to sing. We trust Him to bring His peace that transcends all understanding. Please pray for continued trust... our flesh is frail and weak. If we had to rely on our own ability to have faith, we'd be in deep waters. But Jesus is the author of our faith, the reason for our hope. It is impossible to imagine walking this road without Him. Let us walk by faith and not by sight.

Continue to come along with us on our journey. We welcome your companionship on this ride. It was Tuesday when I first started this post earlier in the day, but now it is Wednesday: T minus 9!