Hi friends and family,

We want to provide a brief update following Emma’s first enzyme infusion treatment —

Emma’s first enzyme infusion went well

This past Wednesday, Emma went to Children’s Healthcare of Atlanta (CHOA) Scottish Rite Hospital for her first infusion of enzyme treatments.

As a reminder, her disease causes a lack of the TPP1 enzyme, essential for breaking down toxins in the brain. In Batten Disease, toxins build up, leading to brain cell damage. You can view a visual explanation here.

Her treatment infuses artificial TPP1 enzymes into her brain stem. Artificial enzymes aren’t as effective as real, but they will likely slow down the progression of cell damage, giving her additional time walking, talking, and having some general independence as a person.

To receive the infusions, the neurosurgeon inserts a needle into the port she had installed in her brain during her surgery. Then, the enzymes are infused slowly over a multi-hour period.

It makes for a long day of sitting in the bed with an “Olaf hat” (see below), but Emma was a champ.

She did great during the port access part (all the staff were delightedly shocked), and she received the enzymes well — even taking a nap for a portion of the infusion.

This process will become a new “normal” for Katie and Emma. They will spend a whole day at CHOA every 14 days to receive these infusions. They’ll access her brain port, and then she’ll sit in the bed for 4.5 hours to receive the enzyme medicine. As long as the doctors don’t see anything concerning, she won’t need to stay overnight.

I (Stephen) won’t be able to accompany them on every infusion trip because of work, but I’m glad I could join for the first one, and I hope I can at least be a chauffeur for many more, even if I am only able to drop them off and pick them up.

The CHOA team members were all competent, thoughtful, and kind. It’s a comfort to know that we have a source of good medical care ongoing with our team there. We’re grateful.

Back home, our big kids enjoyed a great day with grandparents, even experiencing the Georgia summer it’s-so-hot-outside-let’s-find-something-indoors pastime of bowling for the first time. Grateful for the love they are getting, too.

Lastly, thank you for all the messages and gestures of support. We felt well-cared for this week.

Please join us for a Night of Worship & Prayer on 7/28

So much of this journey is spiritual as much as it is physical.

As such, we feel some calling to press into worship & prayer as deeply as possible as we get more closely acquainted with some realities:

1. There is almost nothing more certain in life than suffering / pain / disappointment.

2. If God is in charge of the universe, what we think about Him drives our perspective on suffering.

3. The Bible is chock-full of verses about blessings on the path of suffering.

These aren’t unique to what we’re going through, so we want to “press in” to God alongside others on this: going to Him with our pain, worshipping Him as the one in charge, and asking for deeper strength and comfort.

If you are a person of faith or think you might want to explore it, it would be an honor to press into worship & prayer alongside you.

Details: A worship and prayer service (featuring prayer specifically for Emma) will be held at CityScape Church in Columbus, GA, at either 5p or 6p, on 7/28.

We’ll do an announcement post about it in the next ~week or so that you can share with others if you’d like.

What’s next?

• Next enzyme infusion 7/17

• Night of Worship & Prayer @ CityScape Church 7/28

Resources that have encouraged us lately

• Song: Christ is All, Church of the City New York

• Devotion: In Affliction Look to Jesus, Octavius Winslow

• Devotion: The Test of Character, Oswald Chambers

Prayer requests

• For sleep. Emma’s still suffering from disrupted sleep each night (and therefore so are we). We recently received discouraging news on this front indicating it’s most likely her disease causing it. We are asking in prayer for some immediate relief and wisdom for our next steps on how to help her.

• For wisdom & guidance re: upcoming decisions. We are approaching some big decisions in my business, the kids’ activities, and our general commitments as a family. We want to be a source of blessing in what we say “yes” to (and don’t).

We’re thankful for…

• What seems like a great medical care team at CHOA.

• Family, friends, and church community and their care for us so far.

• A reliable car for trips to and from Atlanta.

That’s all for now. More to come. Thank you for holding us up. 🤍🙏

Blessings,

Katie & Stephen