More Manna

I never tire of the manna metaphor.

God continues to amaze me with his willingness to provide for each and every twist and turn on our journey, exactly when we need it. I was heartbroken, but prepared to stay in Missouri with our daughters while Matt organized and attended his Dad's memorial service on Saturday. Then last weekend Matt and I began to pray about the possibility of me going. It would take a perfect set-up: my mom available to care for the girls, a nurse to care for Ava, 24/7, Ava to remain seizure-free and stable without any deterioration in her breathing, and PEACE for my mama's heart to leave my girls at home.

God has provided all of these things and more: My mom is here, hurray! A nurse-friend (she's so much more than that) has generously offered to come stay with Ava all weekend...and her family is flexing to allow her to bless us. One of Ava's NICU nurses offered a meal for Friday night. We've gotten many offers of help to spell my mom and our nurse friend. While Hannah is just getting sick, it doesn't appear to be as severe, and the oldest girls, my mom, Miss M and I are all on the mend. And through all of the crazy illness, Ava has remained healthy. She has been seizure-free, and is even awake more each day (see those eyes?).

Lastly, and most importantly, Matt and I have peace. Not particularly because I feel good about leaving the girls...My heart is still torn about that. (I am a mom after all!) Yet I know, because I've seen over and over:

If God is making a way, He will bring peace. 

He has opened up the way for me to go. I'm humbled at the army of people who are helping make it possible. And I am trusting He has perfect provision for every single one of us.

What grace.

Sporting a Faux-hawk for Daddy

So far...Ava Loves Steroids!

Ava's been doing well since coming home. While her sisters, her parents and her nanny have been dropping like flies around her, she is still acting well, so keep the prayers coming! In addition, she has not had any seizures, she's taking all of her daytime feedings by mouth and her breathing has completely normalized. Because she got everyone worked up, she got to bring home two new machines with her from the hospital. She has been wearing the apnea monitor (at night) and pulse oxometer (night & naps) but so far hasn't set off any alarms!

We remain on quarantine, so Mom (thank God she's here!) and I do costume changes many times a day in order to keep the girls germs away. The checker at the grocery store must have thought I was preparing for biochemical warfare with all the sterile gloves, masks, and bleach products I bought today. While she was freaked out at first, Elizabeth is finally comfortable with me wearing a mask, so let's hope it all works!

Ava is definitely still sleeping a great deal but does show us her eyes at least a few times a day for 30 minutes or so. I didn't get a weight today, but I imagine she's gaining since she has only had one episode of vomiting since coming home. All in all, it's a good report!

A touching story from today: Since one of our pastors recently broke his foot, Ruby and I were talking about how difficult that must be for his family. We brainstormed about all the ways our Daddy is helpful around the house now, but couldn't be if he had broken his foot. Ruby looked up at me and said, "It would be ok mama, because Granna would just come stay with us to help us out!" apparently she has the best grandmas in the world and their willingness to swoop in during a crisis is simply a matter of fact. What a blessing.

Matt is still recovering from this bug, but is blessed to be able to be a help to his mom during this time. His brother was there and left, and his two other siblings will arrive this week. Everyone will gather on Saturday, March 3rd, to honor his dad with a memorial service at their home church, Abundant Life Christian Fellowship. Sid Harms was a man truly worthy of the honor he will receive. It was my privilege and great blessing to call him Dad.

Thank you for your continues prayers, emails, words of encouragement during this time. We feel so very loved.

Today's the Day!

We're still waiting for some home medical supplies to be delivered, but we've got her all packed up and ready to go! The doctors are very pleased with how awake she is and are hoping that her seizures will stay quiet. The next few weeks will be a period of watching and trying to keep her well. All three girls at home are sick, and both Matt and I are coming down with it, so it will be a miracle if she stays well. That's what we're asking for! My mom arrived an hour ago, so we at least have a professional baby-holder on hand and we can quarantine her if we have too. I'm pretty sure she wouldn't mind.

Thank you for all your thoughtful emails and texts about Matt's Dad. We are thankful for your thoughts, prayers and expressions of love.

Grieving

We are very sad to share with you that Matt's dad passed away last night. While we all knew his health was on the decline, we simply weren't prepared for the suddenness of loosing him. His fight with cancer is over and we are so thankful that he is finally whole again and with the Lord, but we already miss him terribly. Thank you for keeping Matt's mom and the rest of our family in your prayers. 

Matt flew out this afternoon to be with her and his other siblings and I will remain here in the hospital with Ava until we get clearance to go home. It looks like that might be as early as tomorrow as long as she has minimal seizures and stays stable. She is definitely more alert in the last 36 hours and is begining to open her eyes more and drink from her bottle! We can't be sure if it was simply treating the UTI that caused her to wake up or if the steroids are working. Only time will tell.

Miss M is taking care of our three sick girls at home and single-handedly keeping the home-front in order. Please pray for the quick recovery of the Harms girls and for protection for Ava, Miss M and me. My mom will arrive tomorrow to help while Matt's in California. Thank you for continuing to carry our family in your hearts, love us in practical ways and pray for us throughout so many difficult circumstances. 
Love, 
Matt & Sara

Still in the fight!

Ava made it through the weekend without any more bumps in the road. She did all the things we wanted to see her do for her sleep study, so hopefully when the report is issued, they can give us more clarity as to the underlying cause of her breathing trouble.

Since then, though, she's been much sleepier. While this actually makes breathing easier for her, it's not encouraging sign. It might be the effect of her UTI, but it could also be the progression of her brain problems. It makes us all the more eager to get started on her steroids! This afternoon we finally got negative cultures...which is actually POSITIVE news to us....that means her infection is being treated, so we can begin steroids this evening! Please pray that she has no negative side effects from the high doses she'll be receiving, and that this has a positive effect on whatever is going on in her brain.

Along with steroids comes another fight...to keep her healthy, smack dab in the middle of cold and flu season...oh and don't forget RSV! Her immune system will be suppressed so we need to keep her well. However, Elizabeth slept all day (till 3pm!!) which means she's likely coming down with something, Hannah has a runny nose and Matt's feeling worn down. Anyone who's sick can't visit Ava and even bringing her home becomes riskier. Will you pray for wellness for our entire family and Miss M?

"Don't be afraid, I've redeemed you.
I've called your name.
You're mine.
When you're in over your head, I'll be there with you.
When you're in rough waters, you will not go down.
When you're between a rock and a hard place, it won't be a dead end--
Because I am your god, your personal God"
-Isaiah 43 (The Message)

Friday Night Update

Today was long. Ava was mostly stable but we had visits from many doctors and thus many alterations to the plan. Most notable, we've taken two detours before starting steroids.

A routine urine culture to rule out an infectious cause for her sleepiness revealed a probable UTI. Steroids would make things worse, so this afternoon Ava was started on antibiotics and we'll have to wait for negative cultures before getting started on steroids. Unfortunately, we'd already officially stopped the ketogenic diet and given her some normal formula, so at this moment, she is in a window where seizures could reignite. Please pray that Ava wouldn't start seizing again.

Additionally, ENT weighed in and ordered a sleep study to help us better understand the cause of her breathing difficulties. So Ava and I are on a vacation to the 9th floor tonight where she was hooked up to many more wires and monitors and is now expected to sleep! Um...pray for sleep.

Miss M brought Hannah and Ruby up to the hospital today and I had so much fun playing with them on the rooftop garden. I crave time with all my girls right now and this morning was especially sweet.

To end on a fun note, Matt and I had made plans for Miss M to stay late so tonight we could have a date for Valentine's since let's face it, our Valentine's Day was a pretty depressing day for us. We cancelled those plans with Ava's admission, but were surprised this evening by a lovely catered dinner, hand-delivered by our sweet Marcia, complete with roses (which were confiscated by the hospital staff for health code issues...darn!) wine, candles and real china! Our sweet nurses played the part of waitresses and fetched us some water, and didn't even expect a tip! What a lovely way to end a really difficult week. Marcia, thank you.

And an enormous thank you to everyone who continues to pitch in to bless us, encourage us, call, text and email us, love on our girls, feed us, caffeinate us and pray for us day after day after day. Your efforts are allowing us to focus our whole hearts and minds on loving each other, and being the best parents we can be for Ava and her sisters right now.

Ava Update

Ava is still stable in the hospital and had a good night last night. We're still investigating the underlying cause of her intermittent airway obstruction but we have monitors to watch her patterns, and despite frequent dips in her oxygen levels, she's pulling herself out of the dips by herself, which is encouraging. We're getting our ducks in a row to start steroids, which is, apart from a miracle, the last tool in our toolbox. Starting steroids will mean stopping the ketogenic diet, which could result in an increase in her seizures...can you fully appreciate just how badly we need a miracle??

Please pray~

*that God would allow Ava to fulfill the purpose for which He created her. That He would get ultimate glory from her life...and ours. That she would live every day, and we pray there are MANY, with wellness, vitality, joy and peace in her mind and body.

*For wisdom for Matt and I. God says he'll give it if we ask. He has. And we believe he will continue to.

*Peace and wellness for our oldest three. This constant transition is wearing on them and we need God to shield their hearts and minds. It has, though, been heartbreakingly beautiful to process some of this with them. They are aware of what we need, and simply asking for a miracle from the only One who does miracles. They aren't hung up by the "Why?" and "but what if?". It does my heart good to learn from them about how to approach our loving Father with my most precious desires.

*Continued peace. Every step of this journey has been marked, despite pain and heartache, by peace, assurance and unfathomable grace. He has not left us. He is gently leading us forward. Though we cannot see what is around the curve, how could we not trust Him when He has been so faithful to us thus far?

Ava's back in the Hospital

Ava has been having a more difficult time breathing over the last few weeks, and things seemed to get worse tonight.  A few hours ago, we called an ambulance and she'll be admitted to the hospital tonight and probably for the next few days as we try to grapple with her deteriorating condition. Matt's with her overnight, and I'll swap with him so he can work tomorrow. I have no more words. Please pray.

Ava's MRI Results and a Mini Neurology Class

Ava came through her MRI beautifully. She managed to undergo sedation without needing a breathing tube, and that made her recovery much, much faster. Isn't she the sweetest little Valentine?

Immediately after her procedure, we met with our Neurosurgeon and Neurologist to go over the results. This was a difficult meeting because the results are heartbreaking. No one really understands what is happening in Ava's brain, or what it means. They don't understand why she's declining or know if it will continue. We do know what we told Ruby and Elizabeth this evening: that Ava's brain is very sick, and getting sicker, and we need God to do a miracle. In the mean time, we are trusting God for wisdom about how to best care for our sweet Ava.

Because the results were so confounding to the team, until now, we haven't laid out all the details. But the time has come to give you more details, because things are getting more complicated. To put today's results in context, Matt's going to take over and try to give you a clearer picture of the results of Ava's previous MRI and the subsequent brain biopsy results. So put on your thinking caps because this is going to get very technical! But if you can hang in there, know that you are blessing us by getting the scoop so we don't have to rehash all the details in person over and over again.

*****

Based on the previous MRI and biopsy, we know there are four primary problems in Ava's brain. Unfortunately, most of these problems have progressed based on today's MRI. Going forward, keep in mind that on an MRI scan, abnormalities can be revealed either by the color ("signal") seen or the by the shapes/structure seen.

1. Ava's deep brain structures and the areas surrounding her ventricles show "enhancement" when a contrast material is given by IV. "Enhancement" occurs anytime blood vessels are leaky and the contrast material leaks out into the brain. "Enhancement" is typically a sign of infection, inflammation or tumor. Because she's not showing signs of infection and her biopsy didn't show clear tumor cells, inflammation is the most likely culprit. However, none of the doctors or radiologists here have ever seen this pattern, so none of them can even venture a guess as to what might be inciting the inflammation.

Today's MRI showed that every area of abnormal bright signal is significantly brighter, and that new areas are now involved. An especially problematic question remains: if this is inflammation, is it the primary cause of her problems and decline, or a helpful part of the body's response to whatever it is that's going on?

2. On her prior scans, the areas surrounding the ventricles have been too bright even when contrast material has not been given. Often, when this is seen in a kid Ava's age, it is due to a lack of oxygen at some point, usually from birth trauma/asphyxia. When that's the cause, the involved brain tissue dies and is reabsorbed over several weeks, leaving empty space where it once was. Although the area around Ava's ventricles have been too bright in a similar pattern, the brain tissue had been hanging in there and NOT disappearing. This certainly means that the areas weren't injured by lack of oxygen, but what was injuring them was unclear.

Today's MRI suggested that the disappearing has begun. It seemed to indicate the the damaging force has been present strongly enough and for long enough that these central regions of the brain have begun dying off and shrinking.

3. In addition to all the color/signal abnormalities, Ava's brain also shows structural/shape problems. First, the same areas that have the "enhancement" appear too "full." Not quite like a tumor or a mass, but bigger than they should be for her age. This could be equivalent to swelling from the inflammation, or it could be because of tumor-like cells infiltrating between the normal cells. The biopsy was supposed to help figure that out. It showed abnormal "astrocytes," but the pathologists could not agree if they looked tumorous or not (the vote for tumorous was 3 of 5). Astrocytes are the nurse cells of the brain and they are supposed to live in and around the neurons to help keep them well-fed and happy. On Ava's biopsy, they were too numerous and appeared filled with abnormal clumps of protein.

On today's MRI, the enlarged areas did not appear to have enlarged very much. Everyone is taking this as a sign that the abnormal astrocytes/nurse cells many not be as tumorous as some thought.

4. Also related to brain shape/structure is the fact that part of Ava's cortex is not folded/pleated the way it is supposed to be. These malformations of the cortex are the probable cause of Ava's seizures.

These abnormally folded areas have not changed much. In addition, Ava's 24hr EEG last week showed quieting of many of the areas where the seizures appeared to come from. That's a bit of good news, and for it we're thankful.

Thanks for your prayers today. We are still absorbing the news and have a lot to process in the days ahead. It blesses us that you're with us on this journey. We are being sustained by your prayers and support.

The LORD is the One who goes ahead of you; He will be with you.

He will not fail you or forsake you.

Do not fear or be dismayed.

Deuteronomy 31:8

Slow Motion

Tomorrow, (Tuesday morning) is Ava's MRI. I'd be lying if I said my heart wasn't pounding in my chest.  Please pray for a smooth procedure and clear and helpful results.
It's a big day for our little Bean.

These past five weeks we've been living in slow motion. Life goes on, yet we cannot walk quickly. We feel tethered to home, and family and face-time with our daughters.
We simply don't know what tomorrow will bring.

We cherish each day to snuggle our girls close. 

We treasure the moments when sisters hearts are woven together.

We grab hold of the moments to laugh together, play together and breathe deep the feeling that we are somehow living normal life.

And we kiss, and cuddle, linger on the couch and take pictures. So not one of these moments is lost, in our hearts and memories. Whatever comes.

Multitasking Queen

How many times in the last 6 years have I wished I could have eat while sleeping in order to save time?? Ava's got it down to a science! As the picture shows, she's back home after a smooth 24-hour admission. Her EEG went well and the results are both encouraging and disappointing.

For the non-doctors in the group, an EEG measures brain activity, and a video EEG allows doctors to observe what a patient is doing while her brain is, or is not, having seizures. In the past, we could correlate Ava's rhythmic eye and hand twitches with definite seizures, and were also able to see many seizures that occurred without physical symptoms (called sub-clinical seizures).

This time, with the exception of an abnormally formed portion in the back her brain, they saw many fewer seizures over all. That's really good news. It's definitely possible that the ketogenic diet is working! Unfortunately that means the episodes she's having at home are not seizures...which means not treatable. They also have no explanation as to why she spends most of her time sleeping. So the burning questions we had are still not answered, and we'll have to sit tight until the MRI on the 14th.

A Lunch Date

Ruby came to the hospital this morning to have some one on one time with me, and now we're enjoying a little room service!

The Week Ahead

Ava's got a big week ahead. This picture is from almost two weeks ago. That was the last time she was this alert. She's spent much of the last week sleeping, with brief awake periods. Since her medication levels aren't high enough to be causing this, we need to rule out the possibility that she's having lots of seizures that we aren't detecting, which are wearing her brain out and causing this sleepiness. Tomorrow morning, (Monday) we'll be admitted for 24 hour EEG monitoring (remember this test?) and if she is not having seizures, we'll likely be home by Tuesday evening. If she is, then we'll stay for a while longer while we begin new medications.

Elizabeth has fully recovered from her endoscopy, so she'll be back at school tomorrow.

To her delight, we've decided to discontinue her hearing aides for now. She has gotten more and more agitated while wearing them, and any gain in her hearing has been undermined by her constant attempts to remove them. It's just too much sensory input for her right now, so we'll have to try again in the future. We did receive surprising good news this week: our insurance covered the cost of her hearing aides entirely! Since they're not covered in our policy, we'd expected to pay the thousands of dollars to provide them for her...and now we don't have to! Praise God!!

Finally, a number of you have asked if we've gotten back the final biopsy report. We have, but the information in it is confusing, and simply doesn't provide any clear diagnosis or prognosis. In fact, the first line in the report says, "we're having a very difficult time with this case."...Not a typical biopsy report! At the end of the day, they don't know why, and they don't know what it will mean, but her brain is in really bad shape. It's possible we'll get some information from the EEG tomorrow, but the most important information --whether the abnormal cells in her brain are growing and how fast-- will hopefully come from her repeat MRI on Valentine's Day, a week from Tuesday.

With every breath we ask God for a miracle, and meanwhile, we live life. We continue to receive each day as a gift.  We treasure each of our wonderful daughters.  And we live each day by the grace of God and with the practical support from countless friends, new and old.

 

Thank you for staying with us on the journey.

Sleeping Beauty

Elizabeth's in recovery and sleeping off her sedation. The endoscopy went smoothly but since they had to cauterize a big vein they'll keep her for observation for a while. Thanks for your prayers!

Waiting...

Elizabeth was taken back late, at 1pm, so we're still waiting to hear. The poor thing was a mess for having to go without eating and drinking all morning, and the "happy-sleepy" meds we gave her didn't help matters. They made her a sad-drunk of sorts. She desperately wanted to color....with markers...on blank paper...while sitting up. So crayons and a coloring book simply wouldn't do. She resorted to coloring on herself and the sheets...hey, whatever makes her happy, right?? Praise God, Matt had a 15 minute window between clinics and showed up at the exact right moment so he could hold Ava and I could walk Elizabeth back to the OR.

Now, Ava and I are hanging tight and Ava's even managing to help me with her feed! What a big girl!

Thanks for your prayers!

Elizabeth's Day

Not to be left out of the fun, Elizabeth has an abdominal ultrasound and an endoscopy this morning. So She, Ava and I (Sara) are taking a field trip to Children's Hospital. We monitor the status of the varicose veins in her esophagus with regular endoscopies and since she's been really stable, we haven't had to check her since June. We're praying this is just routine and they won't have to cauterize any vessels this time. The hardest part for her is not being able to eat or drink for the whole morning. Her procedure is at 11:30 and she'll be intubated, so the procedure is not without some risks, we'd appreciate your prayers for our Big Girl. I'll keep you posted!