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

Transferring out of ICU

Thanks for your prayers for Ava and family. Ava was extubated overnight and is going to be transferred out of ICU to the Cancer Care floor. As you can imagine, it was rather traumatic for her to wake up and find tubes in her with not much recollection as to what happened. The good news is that the MRI came back normal, which also means it's not very clear as to why she had the seizure. This isn't her first seizure, so perhaps she has some tendency toward them with the right stressors/triggers.

However, now that Ava's ANC is at zero (meaning she has no immune system), she may need to remain in the hospital longer; waiting to see what the next steps are, thanks for standing with the Lee family!

ICU - update

Thanks for keeping up with the Team Brighter Days Facebook page. You may already know that Ava was discharged yesterday and perhaps saw the video of doing her celebration dance.

Unfortunately, Ava is back in the hospital. She had an unexplained seizure lasting quite some time and was transported via ambulance to the ER where she was intubated and is now in the ICU. She will have a CT scan to assess what might be the cause of her seizure; her platelets are really low, so they probably want to make sure she doesn't have any bleeding in her brain in addition to assessing other things.

Your continued prayers are immensely appreciated. Please pray for Gwen as well, as she witnessed the paramedics coming for Ava. Thank You, Lord, for providing today's manna. We receive it by faith and cling to Your mercy.


Update: CT scan came back normal, no intracranial hemorrhage. It's possible that the seizure could be a side effect of one of her medications (methotrexate). If so, Ava can be pretreated with medication to avoid this in the future. They will get an MRI to see if Ava's seizure was medication-related or not. Thanks for your prayers.

Ava has been allowed to leave the hospital for 4 hours at a time these past few days.

Wow. It was like watching a caged bird fly free.

It's been 12 days in the hospital under isolation. She came to Seattle sicker than she's ever been. And now she is up eating potato chips and watching Dragon's Edge.

It has been hard watching her teeter on the edge for so many years now. It's like dying a thousand deaths, over and over again. But it doesn't take away from the joy when she is given more life, more time, more breaths. This time of uncertainty somehow expedites the ushering in of thankfulness.

I am so thankful.

Thank you for the house that has become a home.
Thank you for the food--the glorious food that has sustained us.
Thank you for the gift cards to all our favorite and most practical stores.
Thank you for the heartfelt cards and emails.
Thank you for the acts of service in so many behind-the-scenes ways that I don't even know them all.
Thank you for the prayers that storm heaven's door at all hours of the day and night.
Thank you for your love. It has covered us so.

As we walked the long, white hallways back to Ava's hospital room after our pass ended, we talked about gratitude. Sometimes I wonder if it is okay to teach her things even with the future so shaded with gray. Does it really matter that she learns life lessons? I struggle with what to do with our time together. Do we live like she will die or do we live like she will live?

I'm beginning to see that the timeline isn't as important as the journey. And our destination is Christ. So, what we do with what we have really matters. In our case, we don't know how many minutes, or days, or months, or years. But I know that when we train ourselves to thank God for every little thing, even when our hearts have the most venomous words ready to spew at Him, there is something that the heart does. It melts. It matters that I teach her that thankfulness is necessary for survival.

The nurse walks in with the lab reports and tells me that there are blasts in the blood again. My blood freezes, and I think everything is going to hell. I want to jump out the window and die so that I can have some face-to-face complaining time before God. (Kidding...but only slightly). But then I get a text from a friend who tells me she is praying, and I open an email with a Starbucks gift card and the message, "We don't know you, but we love you." Then my mom sends a warm plate of food to the hospital, made with love, from the many nameless people serving us right now...

My heart is so mad. My heart is so thankful. It is okay to be both and I will teach Ava this because she will need to know it for the days ahead.

I have been trying to keep up with it all but really can't even begin to thank everyone for the love and service you have shown our family. Thank you to everyone for doing the hard work so that we can see how thankful we ought to be.

*The doctor said that the blasts are not too worrisome right now. Her marrow was so full of disease that maybe it spit a few cells out in the blood. We will check labs again tonight. Please pray with us for zero blasts in her blood, her marrow, her skin, her CNS, and the other sites in her body. We know it's a long shot, but we also know who stands in our corner.

Update

(From Facebook):

We just finished talking with the oncologist and plan to go with the medium intensity chemo. Ava's disease is so advanced at this point that it is creating problems for some of her organs. With that in mind, and the fact that Ava is still sick and might have a fungal infection, this is the safest route to take.

Her oncologist clarified the issue with CNS relapse. The reason it changes our plan going forward is because T-cell ALL relapse in the CNS is extremely hard to treat. Since Ava's disease is more unique with both T-Cell and AML markers, it is hard to say how the disease will react. If you remember, her leukemia has always seemed more driven by the T-cell component but the AML markers of her disease make Mylotarg a very good chemo to treat her with. Of course, the higher intensity chemo would have been a better choice but Ava is too frail to receive that right now. If this round of chemo shows promise by getting rid of the majority of her disease, we may be able to do a second round with higher intensity chemo.

However, it still stands that CNS relapse after transplant raises your risk of relapse considerably. So does extrameduallary disease, and so does leukemia cutis.

Essentially, Ava has three terrible prognostic factors standing in the way of her chance at a second transplant and disease-free survival.

We know things are looking bleaker than ever before. But we just can't give up. Ava is not ready to stop fighting. If you could see the way she still greets people with a smile despite her body shutting down...

We are asking the Body of Christ to seek the Lord with us concerning Ava. Her life is carried so preciously in His hands. And who knows, He may heal her for His glory. But if not, the glory still belongs to Him.

Please pray:
1) That this is not a CNS relapse. The pathology report has not confirmed it yet. Although it looks very much like a relapse, it could be a process of infection. It is unlikely that is the case, but stranger things have happened.

2) Ava would tolerate the chemo well and not get any sicker as her immune system crashes. We pray that she doesn't have a fungal infection and that her current parainfluenza virus would quickly be resolved.

3) We would find a good pain management for Ava so that she wouldn't be in pain but also have more wakeful moments. The medication she is currently on makes her extremely drowsy so she spends a lot of time sleeping.

4) That the chemo would clear out her disease completely and that she would have a chance at a second transplant.

I have been allowing myself to believe that Ava will make it through this and that her story would bring so much glory to God. We know that you are praying this alongside us, and we are so thankful for those precious prayers.

CNS Relapse

(From Team Brigher Days):

With devastated hearts we share that Ava is showing CNS relapse as well as widespread disease in other extra medullary sites. Due to the CNS (brain and spinal chord fluid) relapse we are being told that even with the most intense chemo, the chances of her recovering are now very slim.

She has been sleeping many hours throughout the day and her intense pain is being managed with morphine and other pain medication. Without intervention, she likely only has weeks at most.

We are spending a lot of time bedside with Ava and, unfortunately, Gwen and Jude do not understand so they are confused and sad about being separated from us.

Mike and I talk to her oncologist tomorrow. Please pray for us as we navigate these impossible decisions. We thank you for your prayers as you plead with us for another chance for our precious girl. We need a miracle.

Very Sick

The Lee family has made it to Seattle. Thank you for your prayers! Mike, Esther, Ava & Jude arrived via medical charter on Sunday as you probably know from the Team Brighter Days Facebook page (remember, it's a public page accessible to everyone). Esther's parents arrived with Gwen Monday evening.

Ava is very sick. You know this, as her peripheral blasts went up to 18% on Sunday from 5% only the day before. Ava also has parainfluenza virus right now, which can result in a severe infection in immunocompromised patients. They're not sure if they saw a touch of pneumonia in Ava's lungs. Jude & Gwen are also sick, probably with the same virus, though their bodies fight it much more effectively than Ava's does.

Ava had a CT scan done earlier today (Monday). The CT showed more nodules, but the big question is: are they leukemic nodules, or are they from a fungal infection? Ava has already been on an antifungal for a while (just in case). But even so, a fungal infection would be serious and difficult to treat, especially with her weak immune system. Any chemo would further suppress her immune system which could exacerbate a fungal infection. In order to know for sure, they would need to biopsy one of the nodules, which would be an unpleasant, invasive procedure through her side. This would also require that one of the nodules is large enough to biopsy, which is unclear at this point.

So what are the treatment options for Ava's cancer? You've heard about Mylotarg as a key player and consideration in her treatment. Mylotarg itself is not chemo, but is given in conjunction with other chemotherapy agents. Mylotarg works best when disease levels are low. Chemo agents are used to bring the disease level down to a point where Mylotarg can do its "clean up" work. It is less effective when disease levels are high.

The real variables here are in the chemo agents used, and they are weighing the options:

1) most aggressive/intense regimen (Mylotarg + fludarabine + Ara-C): this is the treatment you've heard about, where it's so intense it would wipe out everything and put Ava in a severe state of immunosuppression. Ava would need to remain hospitalized for the duration of treatment/immunosuppression, which could be anywhere from 4-8 weeks.

2) "medium" intensity regimen (Mylotarg + cytarabine): this regimen results in immunosuppression for several weeks.

3) "light" regimen  (Mylotarg alone or with a hypomethylating agent).

You can imagine the factors that weigh into their considerations. The first option, while being most aggressive against Ava's cancer, also puts her at risk for other infections, especially with immunosuppression lasting so long. On the other hand, for Mylotarg to work most effectively, they need to get her disease level as low as possible. They would only consider the "light" regimen chemo if Ava had other factors/conditions that made the other options unavailable.

Ava's been on a lot of pain meds lately and also complains of a stomach ache, which could be something called typhlitis. Ava's pains may be due to chloromas; she also has a small pocket of fluid near her heart.

As it stands, Ava will get a lumbar puncture (LP) on Tuesday along with some injectable chemo. The plan is to start treatment (Mylotarg + chemo) on Wednesday no matter what, though they're not sure yet which regimen to use. There isn't much time to waste as Ava's cancer continues to spread, but there are important factors to consider in deciding on the best regimen.

Please pray for:
- a smooth LP on Tuesday
- God's wisdom and grace for the medical team regarding treatment plan
- absence of fungal infection
- effective pain relief for Ava
- rest, stamina and strength for the Lee family
- God's peace and presence to guide
- God's glory to be revealed unhindered throughout this journey
- comfort and peace for the whole Lee family, but especially for Ava, Gwen and Jude who are dealing with sickness and being apart from family in one form or another. Sick kids always want mama! Thank you to all who continue to love on Gwen and Jude.

Be sure to follow the Facebook page for any day-to-day updates in addition to this blog. We know that so many of you already (and continue) to pray for Ava daily, and there are a couple of prayer chains going. If you are interested in joining a prayer chain, you can sign up on this one for a 1-hour slot (note that the time zone is noted in PST).

As a reminder, the link to the Facebook page is also under "Ways to Help," where you can also find links for the prayer chain there and how to provide meals or other support for the Lee family. The Meal Schedule page has also been updated.

Your partnership has been invaluable. Thank you so much for walking with us. The Lord bless you for your sacrificial love.