Family Time

It's been a glorious past few days! We spent much needed time as a family and with Uncle David and Auntie Hannah in town, it felt complete!

It's amazing to see Ava's thirst for life. She's only 7 and yet she has learned to appreciate each day, savoring the moments with us.

Last Friday, Ava was able to get a bone marrow biopsy due to the sudden increase in platelets, ANC, and hematocrit. Her platelets went from 17 to 75, her ANC from 265 to 1006, and her red blood from 28 to 35 in just TWO days! The nurses were amazed at her recovery. We stand in awe of God's ability to heal Ava and her resilience to continue the fight.


We know that this all stems from the prayers she is receiving from all over the world. Thank you, from the bottom of my mother's heart, for your love for Ava and for storming heaven's gate on her behalf.

Tomorrow we will consult with Ava's oncologist to see what our next move will be. The most exciting news is that we are officially approved for Venetoclax AND the insurance is covering it completely!!! What an awesome answer to prayer. The financial advisor at the hospital was shocked that the insurance company was covering this drug-so are all the doctors who thought, for sure, the process would be longer and harder. But with all the connections we were given by you, everything was expedited and....well, here we are!

Thank you for standing with us and for us. We draw some mighty strength from the knowledge that we are not in this alone. The battle is fierce, but we stand in our stronghold, fortified by the Lord and all those who love Ava.

There is beauty everywhere today. And, tomorrow, with all its worries, can take a backseat. For now, we are resting under the shadow of His wings.

We know who these blessings come from. And I'm pretty sure there is more to come.

Request for Prayer:
1) Gwen is now sick with the same thing I had. I am still recovering from whatever it was that hit me. We pray for quick recovery and that it would not pass to Ava. Any sickness could delay the start of chemo.

2) Prayers for clean CT scan and No Evidence of Disease in her marrow. We will find out the results in the next few days.

3) For Ava to be able to get the Notch 1 inhibitor if needed. This drug has shown even better response than Venetoclax in an ex vivo setting. We shied away from this option at first due to how difficult it would be to get it. But, with the help of our friends, we are actively pursing this as an option.

4) For complete healing and long life for Ava.

5) For our faith to stand firm, even in the face of death. For a renewed relationship with Christ and a trust that grows with each day despite the heartaches.

Thank you for praying with us!

Celebrating Ava's discharge by going on a paddle boat ride!

On discharge day, Ava saw this cloud in the sky and said, "This cloud looks like a heart."

Reading a silly book with Uncle David and Auntie Hannah

All three Loves. Please grant us more time with them.

On the way to our whale watching adventure. 

Looking for marine life! We saw 2 humpback whales, some dolphins, and a pod of orcas!

Family fun at Carkeek Park

Teaching Jude how to walk. He should take it easy though. I mean, he's only SEVENTEEN months and still not walking. No big deal. #thirdchild

Ava had a bone marrow biopsy on Friday as her blood counts increased dramatically since Wednesday. Ava was having some back pain from the procedure (not sure if she still is), but thanks for your prayers for full and quick resolution of that. We await the biopsy results.

In the meantime, the Lees are looking into their options for a haplo-identical transplant (possibly in Milwaukee), and Ava is scheduled to start Venetoclax on Tuesday. In fact, the drug was approved and fully covered by insurance! Thank you for your prayers, we praise God for His provision! It looks like there should be no side effects judging from what is currently known about the drug, though it's still in its experimental stage.

Thanks so much for your faithful prayers and loving support.

I recently came down with a bad infection that kept me out of Ava's hospital room (due to her low immune system), off the computer, and in bed for a few days.

It was really hard and Ava cried many tears over our separation. We face timed as much as possible but every conversation had her wiping her tears away. The redeeming part of this sudden illness is that I got to spend the last 4 days with Gwen and Jude. Holy cow! They are so cute.

Since I last updated, something very special happened. A few nights ago, Ava and I were drifting off to sleep when she initiated THE conversation. She asked me what would happen if the chemo didn't work. I wanted to hear her thoughts first so I asked her what she thought would happen. She said she would go to heaven to be with Jesus.

Her candor and her sweet faith broke me and fixed me in the same instant. How pure and strong is her faith to anchor to promises that even well seasoned Christians might stumble through at times? How heart wrenching is it that her mouth forms around words like "dying" and "heaven" while her counterparts talk about summer break and vacations?

I asked her if she had fear. She answered that she was so excited to meet Jesus and to be in a place where she wouldn't be sick anymore. But soon, she began to wipe away her tears as she told me how hard it would be to leave us behind.

I don't want to walk this lonely road. The way is hard and broken. There are thistles and thorns and we step with tender feet. But to know that she is leading us with gentle attention to our shattered hearts, that she is not scared even though we tremble, that she knows where she will be when all is said and done, it lightens our load a fraction at a time until we realize that she bore the brunt of the burden all along. We were just spectators cheering her on in this wretched, wonderful race that is called life.

I have told her this before and I will say it and envision it for many, many more times to come. There will be a day, when she approaches the threshold of heaven and God will be there. He will scoop her up in his arms and he will whisper, or perhaps he will shout, "Well done, my good and faithful servant. Enter into joy!" For there is no one I know that has lived out the difficult lot given her with such grace, joy, and honor as my love, Ava Bright.

Thank you to everyone who has prayed for this particular conversation. It was so natural and so filled with beauty that I know it was not coincidental but a culmination of so many loving prayers.

We are currently waiting on Ava's counts to recover. In the next week we will redo a bone marrow biopsy and a pet scan to restage her disease and get a more accurate read on how well Mylotarg worked. At this time, the doctors are still uncertain which chemo they will pursue next.

With that in mind, there is actually another chemo that would possibly work even better than Venetoclax. This drug, called a Notch 1 receptor inhibitor, would be ideal because it would target the Tcell component of her disease which seems to be the most aggressive part of her leukemia. Ava has a Notch 1 mutation that would make the drug a truly viable option. The problem we ran into early on was how impossible it would be to get the drug. Venetoclax was already FDA approved and therefore its biggest stumbling block was getting a hospital and a doctor willing to administer it. With the help of so many of you, we were given great advice on how to pursue Venetoclax and we have possibly secured it for her.

The issue with the Notch 1 inhibitor is that the drug is in trials right now and therefore the drug companies keep a very tight rein on compassionate use opportunities. One example of why they don't readily allow individual uses would be that if they gave Ava the drug and she were to pass away from some unforeseen side effect, the FDA could pull the drug and the company could lose billions of dollars. That type of risk is not taken lightly.

So far, all three companies that have this drug (Eli Lilly, Pfizer, and Merck) have denied us access. Since time is not on our side, we are desperately seeking a way to expedite an appeal. Does anyone know of a way?

Last night I couldn't sleep from the pain of my sore throat and Ava's situation. I emailed her oncologist begging him to help us get the drug. In the morning, I was a little embarrassed at my emotion and sheepishly waited for his reply. His response was filled to the brim with kindness.

There are times when I wonder if the Lord will answer my cries with tender care. There have been more moments of silence these past few years than I have ever experienced in all of my time walking with Him. But I know, without a doubt, if Ava's oncologist can be so good to us than God's kindness will be immeasurably more.

I am expectant, face lifted up toward heaven, awaiting his response.

It will be good. I am sure of it.

Treatment Considerations

Thanks for your continued prayers for Ava and the Lee family.

As you know, the cancer journey involves a lot of nerve-wrecking waiting. We have been waiting to see how well Mylotarg worked. We are waiting for Ava's counts to recover, for her ANC to show signs of an immune function returning. We are also waiting for the results of her bone marrow biopsy, skin biopsy, PET scan and lumbar puncture. With no immune function, it is highly possible for the test results to show a false negative (e.g. showing no disease when disease is really present). However, any positive results for disease would be reliable and true. These test results will help guide Ava's treatment course.

The plan and hope has been for Ava to reach remission in preparation for a second transplant. However, assuming Ava reaches remission, there are also concerns with pursuing a second bone marrow transplant (BMT). Even if the transplant is successful, it is certain to leave Ava with many serious, long-term health issues -- far more than the effects from a single transplant. Should Ava reach remission after this past round of chemo, the likely assumption/understanding would be that the chemo worked and brought her to this place. However, there would also be a thought/hope/wondering if Ava's body is finally kicking in to effectively fight the leukemia apart from the chemo (and maybe she wouldn't need any more chemo or transplant). There wouldn't really be a way to determine this, so depending on the test results, chemo or transplant seems to be the main two options. It's just that chemo is a double-edged sword as you know; while it has some fighting power against cancer, it also has toxicity on the "good guys" as well, so it's a mixed bag. A parent's heart is to use as little chemo as possible, and only what is required, but no one really has that kind of knowledge or certainty...

If Ava becomes ready for a second transplant, another question is: what kind of transplant should be done? Another cord blood transplant? Or perhaps a haploidentical (half-matched) BMT where Esther is the live donor? In the beginning of this journey, there was a lot of talk and search to find a perfectly matched donor for transplant. In the absence of such a donor, the option for cord blood transplant seemed the best route because the immature cells in cord blood could allow for a less than perfect match. But now that Ava has already had a cord blood transplant and also experienced GVHD with it, there is some discussion on whether a haploidentical BMT might be preferred this time around. With all BMTs, there's a delicate balance of wanting just enough foreign cell response to kill/attack the cancerous host cells, but not too much that it causes life-threatening GVHD. On the other hand, too little of the foreign cell response could result in the host cells attacking, resulting in a failed transplant.

If Ava reaches remission, there is a small window of time to make these decisions - no more than two weeks. As you can imagine, there are many steps involved with either kind of transplant, cord blood or haploidentical BMT, so a lot may need to happen in a short amount of time. But as we have seen with the progress on getting access to Venetoclax, nothing is too difficult for God.

Your prayers are coveted! Please pray:

• for Ava's continued pain: for total resolution of pain, no need of pain meds...or effective pain management that wouldn't cause her to sleep so much.
• for absence/resolution of any infection, whether on her skin or anywhere else
• for remission/true absence of disease
• for wisdom regarding whether to proceed with transplant and if so, which kind
• for wisdom regarding treatment plan if Ava is not in remission (e.g. doing Mylotarg again, if and when to use Venetoclax and how to dose it, etc.)
• for rest for the Lee family, rest for their weary souls and hearts; for the Lord's breath of life to sustain them, His embrace to uphold them, and His presence and peace to comfort them.

Ultimately, we continue to pray for unhindered trust and rest in our Heavenly Father who knows all and sees all. As He grants us insight into His plans, may we have the ability to recognize His hand and respond accordingly. We serve a mighty God, who is a faithful Shepherd and Guide. Thank you for walking with the Lee family.

Recap

You probably have many pieces of information already, but just in case, this is to summarize where we are in terms of Ava's treatment plan.

As you probably know from the Team Brighter Days page, Ava's pain medication regimen was changed a couple days ago which has allowed her to be more alert during the day (hooray!) and the radiation burn pains in her perineal area (and urethra) have improved a lot. Thank You, Lord! Thank you for your prayers.

Ava has remained hospitalized and in isolation due to very little immune function. Her ANC had been zero all week, but went up to 35 today. If her ANC reaches a certain threshold (not sure how many hundreds), she could be discharged! It would be a wonderful thing if she could go see some fireworks this July 4th!

Ava is scheduled to get another LP this Tuesday. Praying for no more disease in the CNS system. They plan to get a bone marrow biopsy the second week of July to assess disease level and efficacy of the chemo/Mylotarg regimen she just received. If disease is still present, Ava will get another round of Mylotarg. If there's no disease (i.e. remission), they will take next steps toward prepping for transplant.

Meanwhile, Esther mentioned on the FB page that there is a very promising drug called Venetoclax that was tested on Ava's cells and shown to be effective at eradicating her disease! There are a number of hurdles that make it challenging for Ava to get access to this drug right now, but the doctors' hope is that Ava would reach remission without it and be able to move onto transplant, making Venetoclax unnecessary. Yet should the need for Venetoclax arise, we pray that the Lord would make a way, according to His wisdom. Ultimately, our hope lies in Him alone; keep our eyes fixed on You, Lord, and show us what it means to walk by faith.

Thank you for being an amazing community to the Lee family! They feel incredibly blessed. As you pray for Ava, please lift up a prayer for other families battling pediatric cancer who may be walking the road alone without such a community; may the Lord bless them with people who can journey alongside them, even in a pass-the-baton kind of relay-style support. May the Lord bless you for your sacrificial love.

Join Us

Dear Faithful Friends,

I'm so sorry for not updating the blog more frequently. I am currently blogging Ava's progress, almost on a daily basis, on our Facebook page Team Brighter Days. I intend on continuing this blog but only for longer/deeper reflections.

So please join us over at Team Brighter Days for current updates on Ava's status and for ways to pray for our family.

Thank you so much for your continued support, love, and prayers! They are a source of immense strength to us.

Love,
Esther