We don't know how long our sweet Ava Bean will be with us. This has always been true, but we have never known so clearly that every day and every moment we have is a gift. In the past week, Ava turned 11 months old. She has lost the ability to eat by mouth and her brain is having more and more difficulty telling her body to breathe.  Many times a day she needs us to rub and stimulate her because she's starting to turn blue from the lack of oxygen.  She hasn't opened her eyes for more than a fleeting moment since Matt arrived home on Friday.

These are the signs of Alexander's Disease--too many astrocytes, cells filling up her brain, robbing our little one of her most basic abilities. Because Ava's disease is so rare, there is no path laid out for us. There is no one with whom we can compare stories or to whom we can look for more information.

Ava's hope is in the Lord alone.

We continue to pray that God would heal her in a way that would bring Him the most glory. It appears that He is taking her home.  A few nights ago, Ruby said, "Maybe when Ava gets to heaven, and God makes her whole, He can send her back to us." That sounds like a wonderful idea to me.

While we wait and hope and grieve, we are living on the razor's edge -- listening for her every breath and wondering if it will be her last, while willing ourselves to keep living life, caring for our girls and ourselves.  Our hearts are breaking over and over again and yet she could hang on for hours or days or weeks.

We don't know how to do this.

We don't want to do this.

We ask every day that God would let this cup pass. We plead, we bargain, we beg. Then we eat the bread of truth that His will is perfect, His ways are good and He loves her more than we do.

Pray for us.

Pray for Ava.

Daddy's Home

Ava Loveliness

Ava Holding Steady

As I go to bed tonight Ava is comfortable in her bed, hooked up to her pulse oximeter and her feeding tube. An alarm will sound of her oxygen levels dip or her heart rate drops for too long so we can sleep with a measure of peace. Her breathing is still concerning, but she hasn't declined, so for now we simply watch and listen and thank God for machines that can be our eyes and ears. She's not able to coordinate to eat much by mouth anymore, so we're very grateful for the ease of a feeding tube to keep her sweet checks chubby. She is also rarely awake, but then she'll suddenly surprise us and blink her eyes a few times and appear to be checking us out. Of course this means we drop everything we're doing and all descend to ooh and aaahh until she goes back to sleep. 

Matt leaves this afternoon for New Orleans for an annual work conference, where he will give a number of presentations....which means he's barely slept in the past three days. On the heels of his departure, my dear friend Abby will arrive from California to spend some snuggle time with Ava and keep me company over my birthday. Last week we welcomed Miss M, to live with us again too, so we're a full house of women for a while.

As you can tell, I won't be lonely while Matt's gone, but we still would ask for your prayers! 

*For protection for Matt while he's gone, and for blessed presentations. 

*For protection for the girls and I, along with Abby and Miss M. 

*Please pray specifically that Ava would remain stable and would not have any seizures. 

*For continued daily grace and wisdom to observe and rightly respond to Ava's breathing and feeding trouble. 

Thank you for faithfully carrying our family in your hearts. Hopefully I can post more pictures tomorrow. Ava gets cuter every day!

Quick Turnaround

After giving us a scare last night, Elizabeth is quite perky and doesn't have a fever this morning! We'll keep a close eye on her since she often starts to spike again in the afternoon, but for now it appears--since the other girls are also coughing and having runny noses--that it's just a virus.

Because of her already compromised breathing and her immunosuppression from steroids, Ava simply cannot get sick. So far, God has miraculously kept her well, so we are praying that continues.

***an end of the day update: Elizabeth's fever never returned, so she'll be back in school tomorrow (Tuesday). The other girls still have cold symptoms, but so far Ava is holding her own. She has started having some breathing trouble, which is causing us to hover a bit more. This is especially concerning in the middle of the night, when everything seems a little scarier. Thanks for your prayers! Keep them coming!

Prayers for Elizabeth

On Thursday, Elizabeth and I had a rare date, just the two of us. She was such a brave patient during her blood-draw, she deserved a treat! 

This moment was made possible by the many people who are loving and serving us right now. They're stepping in to babysit, making meals, running errands, picking up groceries AND bringing me caffeine to help me make it though the day! We're so grateful! And thank you especially for continuing to pray for us. 

Would you pray for Elizabeth tonight? She must have heard me mention that things were awfully peaceful around here, because tonight she's got a 104 fever and we're watching and waiting to see where it goes. We've done this dance with her for six years now, but it's still scary. 

An Anniversary of Sorts

About a year ago, I took a short break from my bed rest and sat through the worship time at our church's Easter Service. I asked God to heal me, and He did. The clot that was endangering my pregnancy and our 21 week old baby resolved and I knew in that moment that God had answered my prayers.

Following such a miracle, the year that followed has been so very different than I imagined it would be. Hope and heartache have been intermingled. I truly believed I was completely healed and my pregnancy would soon become "normal". Instead what followed was more bleeding, preterm labor, hospitalizations and eventually the delivery of our little angel at 24 weeks and 5 days. Ava was large and healthy for a micro-premie. She was "perfect" in every way. Then, she wasn't. Her hydrocephalus was a terrible shock. Her diagnosis with Alexander's disease was another blow. I am no closer to understanding the mystery of prayer or the miracle of healing, but I am amazed by how peaceful we feel today. And more and more I am understanding the reason for this peace.

I trust God's heart. Finally.

I don't understand why things seemed to resolve, only to get worse. I don't understand why any of this is happening. But the mercy of this moment is, I don't have to. I don't need to. In my best moments, I believe God is guiding this response and enabling our trust, and will--if it is required--give us more faith than we already have. Faith that would move us from knowing He can to knowing He will heal our little one. And so I simply stay here.

Be still and know that I am God.

God, you alone are God. You do all things well. Your ways are higher than my ways. There is none like you in all the earth. You ARE at work and will redeem all things and work them together for our good. You are behind and before us. And you are gently leading us.

Grandpa's Going Away Party

Click here to link to the video of Sid Harms' memorial service. It was such an honor to be present to celebrate his life.

Ava Sleeps

Ava is 10 months old. She is beautiful.

Her chubby cheeks are kissable.

Her hair has a sweet curl that compels us to rub her head at every opportunity. Her breathing is raspy at times, which is a relief, since it means she's resting, breathing and still with us.

Her sisters bring her their "kiki's" and dollies and sing sweet songs to her. She sits with us most of the day, and sleeps near us at night.

We snuggle her whenever we have the urge. And through it all, she sleeps.

We live for the moments when she opens her eyes ever so slightly and we can pour into them all the love and affection and adoration we have for her.

These moments are fewer lately. And we treasure them all the more.

Peaceful days

We've been in a bubble these past few weeks, and that's been made possible by Ava's stability on her steroids. Since we maximized the beneifit, and the risks of staying on them are many, we're begining a long, slow taper, which, if all goes as planned, means she'll be finished with steroids the day after she turns one on May 24th. She is not having seizures yet, but it's expected that her seizures could start anytime, since the steroids have an anti-seizure effect, and now we're weaning her off of them.

Her breathing has become more labored at times, and that too is to be expected because of the problems in her brain. Needless to say, we're trying to ride the fine line between hovering over her all day long, and enjoying the peaceful days while we have them. 

We continue to be blessed by family and dear friends who are coming to visit, encourage us and love on sweet Ava Bean. After Grandma Harms left, my dear friend arrived with her daughter, who is Ruby's bosom buddy! and while Matt took advantage of the opportunity to work his tail off, the 7 of us girlies and her baby girl-bun-in-the-oven had a sweet time just being together, cuddling, reading books and playing in the backyard. This evening Kristen left and my parents arrived in time to celebrate my Dad's birthday tomorrow. We're so looking forward to a few more days of family time. Each person who has come to visit has blessed us and in a very important way, borne witness to Ava's precious life. We can't express just how much their visits have meant to us. We also know we're able to fully enjoy this time together because of all the friends who are running errands, making meals and continuing to help us keep our boat afloat. Thank you so very much. You are blessing us tremendously.