TLC

Grandma Harms arrived to spend a few days with us this week. We are delighted to have her and soaking up every minute. It's good medicine for all of us.

Ps- Today is her 60th Birthday! We couldn't be more honored to celebrate with her in person. Grandma, we love you!!

26.2

It has been one week since we confirmed Ava's diagnosis and then increased her dose of steroids to try get a little more awake time with her each day.  We haven't seen the significant improvement we'd hoped for, but her breathing has been stable and she seems comfortable.  Because of that, we've been able to take advantage of Elizabeth's Spring Break to savor some togetherness.

We've managed to fit in some wonderful times together at the zoo,

 

feeding the ducks in Tower Grove Park,

 

 

 

feeding the fish at the Botanical Gardens,

 

 

 

...and picking out a new stash of books from the library. On the heels of all that activity, we're all completely worn out.  Last night it took an hour for me to make 3 tuna fish sandwiches for the girls! Clearly we're not firing on all cylinders.

This morning, a friend gave me a helpful picture to describe this season of our lives- we're competing in a triathlon (someone got picky about the term so we compromised by grouping events to keep from having to call it a "dexathalon"!).  We finished the swim leg (months of bed-rest and then 3 months in NICU).  The cycling leg is over now too (hydrocephalus, shunt failure requiring surgery and the hard-to-treat epilepsy that kept us in the hospital most of the fall and winter).  Now we're running the marathon, which started when Matt's dad passed away suddenly and we learned of Ava's genetic diagnosis and its prognosis...and there are still many miles yet to be run.

 

Each leg of the race is different, requiring different muscles, new energy, and new skills.  There's also a painful transition between each leg, when our bodies and minds are switching gears, shedding the experiences of the previous moment, and embracing the tough road ahead.

Right now we mostly feel awkward.

 

We're searching for our rhythm and struggling to pace ourselves because, in contrast to the 26.2 miles of a marathon, we have no idea how long this leg of the race will be.  And honestly, we're not even exactly sure which course the race is taking.  Is it the low lying flat course of a complete healing? Or is it the grueling mountain run with peaks and valleys that will end with Ava's home-going?

We're running.  There's no turning back.

And in many ways we're running blind. Except that we have a Guide who is daily ahead of us, calling us to follow Him.

Without a map of the course, this season is full of tension...desires and needs, hopes and realities each pulling us in a different direction. And while we ARE experiencing the grace of God, moment by moment, some moments feel more grace-filled than others. Some moments look like quality time with my girls because Miss M is folding laundry and a blessed soul has signed up to bring dinner. Other moments look like tears on the way to the grocery store because I have to get away because savoring each and every second of each and every day is...exhausting.

My brain simply can't hold onto the reality that my baby is dying and I have the choice to spend my time doing anything I want...so how can I put her down? or walk away? or take a break when I don't know how many moments I'll have?

 

How do we grieve Ava's condition which has robbed her of development and the ability to interact with us, and still celebrate her life?

How do we live fully in the moment, enjoying every day and still give ourselves permission to take the space we need, let off steam, and even distract ourselves without feeling guilty?

How do we work, parent, and live, so close to the veil that separates life end death? How do we pray with every breath for a miracle, and still release Ava into the hands of the one who formed her, for His will to be done in her life?

And how do we teach our daughters about the joys and delights of heaven, where their beloved Grandpa and precious cousins are waiting, and then tell them we're not ready to let any of them go there?

We can't answer these questions. But God hasn't left us alone with them. Daily we need His grace to hang on in the in-between. He has promised to send us a Helper. So we trust He is with us. And when we feel like we might split in two, He is here. A friend makes space to listen and encourage. My sister cares for my heart with her words and her prayers. A friend hauls Matt away for a mid-day break. A text, a card, a comment, an email, and a voice-mail bring encouragement and solidarity. Cookies from sweet neighbors. Packages from Angels to bless the girls. A friend to sit with Ava so we can share a meal with our big girls with a little less distraction. He is using you to care for us. 

And while we often feel we're stumbling along, we know He is keeping our feet on the path and making the path before us straight.

One mile at a time.

I lift up my eyes to the mountains—
  where does my help come from?  

 My help comes from the LORD,
   the Maker of heaven and earth.

 He will not let your foot slip—
   he who watches over you will not slumber; 
indeed, he who watches over Israel
   will neither slumber nor sleep.
 The LORD watches over you—
   the LORD is your shade at your right hand;
the sun will not harm you by day,
   nor the moon by night. 
 The LORD will keep you from all harm—
   he will watch over your life;
the LORD will watch over your coming and going
   both now and forevermore. 
Psalm 121

A Diagnosis for Ava

On Wednesday night, while Matt was in Chicago and I was enjoying girl-time down with my sister and our daughters, we received a call from Ava's doctor. We'd been hoping for and dreading this call at the same time. He informed us that the most recent testing done on Ava was positive for a genetic mutation causing Alexander's Disease. Ironically, it was Matt (with help from a dear colleague) who actually suspected the diagnosis and it was at his urging our doctors agreed to send off the genetic testing.

Because Matt had suspected Alexander's disease, the diagnosis hasn't really caught us off-guard. It has however given us a clear reason for all of her symptoms and now allows to STOP putting Ava through additional diagnostic testing and treatment trials that have no chance of working. With this diagnosis, we are spared so many "what if's?" We don't need to spend mental or emotional energy weighing the risks and benefits of treatments that won't work, and can instead focus on enjoying the time we have with our dear little one. Truthfully, every day we have is a gift from God, and Ava's life is giving us an opportunity to live like we believe that. Truthfully, it has also given us something to fix our sights on as we pray for her complete healing.

Alexander's disease is an obscenely rare disorder (1 in 2.7 million!) and if you want all the details, you can read very detailed information here. Briefly though, it's a mutation in a gene called GFAP which is a gene essential to the function and proper growth of astrocytes- the very set of cells that were found to be abnormal on Ava's brain biopsy. What her doctors had been considering "serial bad luck" (hydrocephalus, white matter disease, hard-to-treat epilepsy, problems with her brain stem, and lack of development) are actually multiple symptoms of one terrible disease. Ava's GFAP gene mutation has been seen many times before, almost always with symptom onset in early infancy, severe seizures, and usually with death before the age of two. Alexander's disease that begins as early as it did in Ava has no treatment and is always fatal. We have always known that Ava needed a miracle (especially since the last hospitalization when her breathing had deteriorated and we first sensed just how close we were to losing her). Now we know exactly how big that miracle needs to be and how to ask for it by name! As I said a few days ago, God has the power to heal Ava of this genetic mutation as easily as He could fix her vision or reflux!

But this I call to mind,

and therefore I have hope:

The steadfast love of the Lord never ceases;

his mercies never come to an end;

they are new every morning;

great is your faithfulness.

Lamentations 3:21-23

In the midst of our heartbreak, God's mercies are many:

The weather here has been absolutely beautiful, providing us with many hours of outdoor playtime and the mental boost that comes with it

Our older girls are finally over their colds and coughs and able to snuggle with Ava to their hearts' content.

Ava has been home now for several weeks, and for now, her seizures are under control (It was merciful that we didn't have to absorb this news while hospitalized).

Even though Matt was out of town when the news came, we were able to spend time together on the phone, and I was comforted to have my sister here visiting me.

You must be praying, because we have peace that we cannot explain apart from God. We are hardly sleeping, yet His grace is enabling us to put one foot in front of the other. In spite of everything, we feel truly insulated from fear and despair.

Cousin love

My sister and her daughter arrived on Sunday for a four-day love fest. We are delighted for our Ava and her Avianna to spend some quality time together. And sweet Svea has already been a tremendous blessing to us. Matt leaves tomorrow for a conference and Svea leaves Thursday...our house has a bit of a revolving door right now!

Ava has been mostly stable since starting steroids. She's awake more, and is having a bit better eye control, but in the past three days her breathing has begun to be odd again, so we're getting things in order to bump up her steroids to the next level. She'll likely have more side effects, (including being at greater risk from germs) but we want her to get the maximum benefit to her brain and mental status, so at this point, it's worthwhile to press ahead.

We are also awaiting the results of her most recent genetic testing. We know the testing is complete, but the results haven't been certified yet, so they could arrive to our doctor anytime between tomorrow and next week. Ultimately, while we are eager to have more understanding of what is going on in her brain, we know that God can heal her of a degenerative brain disorder as easily as he can stop her seizures and heal her epilepsy. So we are praying for the ability to view any information we receive through the eyes of faith. And, we're praying for the strength to stay upright! We are both exhausted to the core, and in great need of grace for each day.

Praise God for caffeine and new mercies every morning!

Milestones: Elizabeth's First Muscial Performance

Elizabeth's Kindergarten Muscial was last week...two friends cam over to help with Hannah and Ava, so we actually got to attend with Ruby. What fun! Even after all the build up and the work it took to get us all there, we still weren't sure she would be willing to participate since crowds and loud noises usually stress her out, but she LOVED it! She was a little nervous, but her Aide was with her the entire time, and once she spotted us in the seats Mrs. Reising had saved for us in the 2nd row, she seemed to calm a bit. She spent most of her song watching her classmates, but singing her heart out and doing some of the motions. My mama's heart was bursting. As soon as Ms. Reising said the show was over, she broke into a huge smile and bolted off the stage! We took the big girls to DQ for a blizzard...and thoroughly enjoyed our night of feeling normal.  What a milestone for our little Joy Child.

Grandpa's Goodbye Party

I work out my theology as I try to explain the realities of life and death to our girls.

They just take these painful things at face value. Grandpa was sick, but he's not sick anymore. He's in heaven now, so he can't come visit us.

We will miss him, but he's happy with Jesus.

This past weekend, with many, many others who were touched by his beautiful life and ministry, we honored the man most called, "Pastor Sid."

It was Elizabeth who first so aptly described the purpose for our weekend away. You're going to "Grandpa's goodbye party," she said matter-of-factly.

And I knew she was right.

God is good. Pain, suffering, hard and loss are real.

Husbands, Fathers and Grandpas die..."Should we send him a Bible?" they ask.

No honey, He has the Word Himself.

I want to give them an explanation. To reason it out and integrate it. And while I grapple to find the words, they simply see the truth.

Grandpa is where he most wants to be. With his beloved Savior. Whole. Forever.

Why wouldn't we throw him a party?

He was a man, worthy of the honor he received. A man who lived out his faith every day and gave glory to God through every mountain and valley of his life. A husband who honored his wife and wooed her with a pure love during every season of their marriage. A father who cherished his children and grandchildren, and prioritized his family, while still making everyone he met feel like an old and treasured friend. He lived a life of service, always drawing in, loving, caring and encouraging. Every day he acted a lot like Jesus.

So we celebrated him. We cried over our loss, not his. And we were blessed to see first hand the far reaches of his ministry and the impact of his life. Truly, heaven celebrated.

And as the day drew to a close, we all agreed...

it was the best sad day ever.