MW Deca Project

Hi Friends,

No news from the IRB yet... still waiting...

But I wanted to let you know that a group of girls from Millard West DECA (where I went to high school) are doing a project to raise awareness for Now I Lay Me Down to Sleep, the organization that does free pictures for families who have a child with a lethal diagnosis. We did maternity pictures and newborn pictures for NILMDTS, it was a wonderful experience for us, and these girls are helping give back to an organization that has greatly impacted my life.

How can you help?

1. If you are a photographer and would like to get involved, the Omaha area needs more photographers. You can check out the organization at nilmdts.org, or contact Catherine Bosley one of the area coordinators at bosleycreative@hotmail.com

2. The girls are selling t-shirts for $10 a piece to raise money for the organization. They come in baby pink, baby blue, and lime green. If you want to order one email me your size and color at LindseyLE@gmail.com and I will let you know where to send your check (: Here is what they look like

Thanks for your support! "There's no foot too small that can't leave an imprint on the world" (:

No news yet...

Hi friends...

Just wanted to let you know I haven't heard anything yet about Evie's drug trial... hopefully tomorrow.

Evie weighed 7 lbs 5 oz this Monday... compared to just 7 lbs last Monday! Something new to Praise God for this week (:

Love you, will update soon!
Lindsey

where does time go!?

Another week has gone by... where does time go??? It has certainly gone really fast the past 5 weeks having Evie and John home... unfortunately John goes back to work tomorrow. That will be a weird adjustment for us! When John was first home Lyla would say... "uh daddy, you going to work now?" it was pretty cute (: Now we will adjust back to daddy working! I remember thinking when we got married that life was finally going to settle down and I wouldn't have to adjust to change so much... ha! That's a funny thought looking back (:

Evie is doing good, we still go to the doctor every Monday and Thursday to give urine and get a weight check, and give blood on just Thursdays, but she is a trooper. They do the blood/urine to check her calcium levels. We get to go to the doc really early and I leave her in her car seat with a big cover over it so hopefully she doesn't get one of the billion cold or flu bugs going around. I have had a cold for the past week or so, but luckily (by the power of prayer!) neither of the girls have gotten it.

I got an email last Friday that hopefully we will have a better idea of what the IRB is thinking by tomorrow (Monday). We may also have a better idea of when her trial would start. I am praying for God's hand to work in the situation because I honestly feel like although I want everything to go as quickly as possible, only God knows what's best for Evie. Since there have been so few kids in the study, I am relying on Him for answers... which also takes a huge weight off of John and I's shoulders! This "learning to take one day at a time" thing has actually been a huge blessing in disguise, and really helped me to enjoy these past few weeks with much less worry. I have felt really blessed through all of our circumstances. Praise God for that! "Even though I walk through the valley of the shadow of death, I will fear no evil, for you are with me;" (Psalm 23: 4) takes on a whole new meaning when you actually experience it.

On a lighter note, we took Ly trick-or-treating twice this weekend in her tiger costume, and went to the pumpkin patch last Monday. It was 70 degrees, and absolutely beautiful! I will post some pics.

I will also let you know if we find out anything tomorrow about the medicine trial. THANKS for your continued prayers for Evie and our family! Love you!

Lyla and her friend Ben - I love this picture because I have no clue what either of them are thinking!

Eve in the tub - she loves it (:

no news is good news (:

Hi Friends,

Sorry it's been a week since I posted, happily I haven't had much to post about!

Evie is doing so great, we took out her NG tube on Tuesday night, we only had to use it one time so we figured she didn't need it anymore... and she hasn't!

The drug company that has the clinical trial was in town Thursday, it sounds like she is a good candidate for the trial. That is a big relief to us. They met with our geneticist, who thought they might be able to start the trial mid-November pending IRB approval. It sounds like a while away, but I am actually glad to have a few more weeks of normalcy for Lyla's sake. Once she stops shouting "I'm so glad your home!" every time I walk in the door (after being gone for even just an hour!) I will probably feel better about being back in the hospital.

Evie's calcium levels in her blood and urine are on the rise, which is problematic. Kids who have hypophosphatasia can't absorb the calcium that is supposed to be going into their bones... and it circulates around in their blood and urine. We will be mixing low-calcium formula into her normal formula until she gets started on her medication, and her bones start absorbing things the way they are supposed to. If her calcium levels get too high it can be very bad for her kidneys and cause "failure to thrive" because she would feel all over crappy. So far she is still doing fine.

John, Lyla, and I are doing good (: I can't believe Evie will be a month old on Monday... it has gone so fast. Like I did when I was pregnant, I still feel like I go into "Lyla mode" during the day, trying to keep things as normal for her as I can, and "Evie mode" when I have some time to digest all that's going on with her. It is scary to think about all of the possiblities of Evie's condition and all that could happen with the drug trial, so I am still working on taking one day at a time. It feels good to cry out to God, and with God. I know he hears my prayers and the longings of my heart to take care of Evie the best I can with all of the unknowns. And sometimes it feels good to just cry it out.

Continuing Prayer Requests: 1. Evie's health - One specialist we have talked to thinks that Evie's bones will continue to weaken until we get her on the drug trial. Also prayer that we can keep her calcium levels under control. 2. That the IRB would pass the trial and the details of paperwork would go smoothly. 3. Adjustment for the rest of us as we become frequent visitors to the doctors offices to give urine samples, blood samples, and have check ups.

Thanks so much for loving us, and being willing to pray. We feel so blessed to have you as friends to go through life with (:

Here are some pictures from the hospital that my dad took:

Lyla had to "check Evie" every time she came to the hospital. She usually followed this by saying "I think she's workin!" or "Sounds like popcorn"

after the nurse told me that the cribs held up to 350 pounds, that's where Evie and I spent most of our time

Home! and thoughts for the day...

We are home again!
Praise God, I am so ready to sleep in my own bed... that plastic couch in our hospital room just wasn't very welcoming after so many days (: Evie is doing good, home with nothing but an NG tube (a tube up her nose) that we can feed her in if she doesn't take enough formula by mouth. Other than that, she is very close to back to normal.

I got to leave the hospital to take Lyla to church this morning (a much needed break). Rob was singing the song "Everything" by Tim Hughes and it just penetrated my heart. Here are the words from the verses:

God in my living

There in my breathing

God in my waking

God in my sleeping

-

God in my resting

There in my working

God in my thinking

God in my speaking

-

chorus: Be my everything (repeat)

-

God in my hoping

There in my dreaming

God in my watching

God in my waiting

-

God in my laughing

There in my weeping

God in my hurting

God in my healing

-

chorus: Be my everything (repeat)

What am I going to take away from this most recent bout of hospitalization?... well each day was a new day. Good or bad there was no planning, preparing, and nothing to do but be present, take care of Evie the best I know how, and trust God with that day. God WAS and WILL BE there in my hoping, watching, waiting, laughing, weeping, hurting, and healing. After 20 years of being a "worry-er" God is teaching me to trust
Matthew 6:34
Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own.

God isn't making a suggestion to me, it's a command. I can't even wrap my mind around how the next 6 months are going to go, and after thinking we may lose Evie twice now, I can't even go there.

So tonight, I'm going to go sit on the couch with John, watch Lyla play, and hold Evie. Today, she is doing great, and that is enough for me today.

Love you friends,
Lindsey

Saturday Evie Update

Hi Friends and Family!

I am sorry I have left you in the dark the past few days, we have been on such a roller coaster it's been hard to update, keep up with the doctors, read literature on Evie's condition, have snuggle time, pray, AND get rest!

After a day of decling a little bit in her recovery on Thursday, Evie did much better Friday, and even better today. They FINALLY took all of her cords off today, she is no longer hooked up to anything! Such a blessing for me to get to hold her without worrying about setting off a monitor. She still has an IV in her arm just in case she needs emergency medicaiton if she would happen to seizure again, and her nasogastric tube that she is getting half of her formula in, but we are going to try a full bottle later today and see how she does.

Evie has received a diagnosis of Infantile Hypophosphatasia. It is a rare bone disease, in which your body does not make enough alkaline phosphatase, the key enzyme in bone mineralization, and depletes your body of it's vitamin B6 stores, causing seizures. The good news is, we have gotten Evie on a regimen of B6, and she hasn't been seizing. She is also off of oxygen and her respiratory status is much better. The bad news is, there is no approved treatment for Hypophosphatasia except a clinical trial, although the clinical trial is VERY promising.

So what's next? Well, Evie needs to take a bottle and stay stable before we can go home, which will hopefully be tomorrow. Next, we need to get approved for the enzyme replacement therapy clinical trial. John and I are finishing up reading the literature and getting our questions answered. The company that makes the drug is coming out next week to hopefully expedite the process of getting the trial approved by the Institutional Review Board (IRB). This drug is the only enzyme replacement therapy available for Hypophosphatasia, so we are ready to get on board. It has shown remarkable improvement in the early stages of the trial. What's the rush? 18 out of 18 infants who had seizures in addition to severe hypophosphatasia passed away in the first 18 months of life. Ugh.

Prayer Requests: Stability in Evie's little body. That we can take her home as soon as possible. Prayers that the drug company successfully gets a clinical team to Omaha ASAP to get the trial started, and that the IRB feels a sense of urgency in getting the treatment passed. That John and I can have peace of mind and heart about the clinical trial, and that if it is not God's will for us to participate that he would throw a giant red flag out there for us. Preparation for me (John, Nana (Micki), Papa Steve, and everyone else), as the trial will require a 2 week hospital stay for Evie to get started, injections 3 times a week, and numerous blood draws, urine specimens, xrays, etc in the 6 months following the start of the trial. Prayers that Lyla will continue to adjust to our new normal and the ins and outs of taking Evie to the hospital... she is doing really good so far but we are both having a pretty hard time being apart during this hospital stay.

Thanks for loving us and praying for us! I can't believe how many emails, phone calls, texts, cards, and gifts we have gotten from friends, family and even strangers just letting us know they are thinking about and praying for Evie. God's people have truly blessed us by wrapping their arms around our family.

Love you!!!
Lindsey, John, Lyla, Evie

moving up (:

Hi Friends (:

We are out of the PICU, and back up to the 5th floor. More importantly, Evie has been off of oxygen since 8am, and her levels are staying good and stable! This is the first step toward going home. They also let her bottle feed a little, and put the rest in her tube. She sucked it down like a pro, so hopefully they will let her take more and more bottle, and less tube feeding.

Evie is getting a picc line put in her arm tomorrow, to finish a set of antibiotics. There were some white blood cells in her spinal fluid from the spinal tap, so they are treating for meningitis even though they don't think she has it... it's a precaution they always take until her cultures come back normal, which takes a few days. Once she is cleared they will stop the antibiotics.

We have an unconfirmed diagnosis that the geneticist and neurologist are looking into... it involves Evie not having an enzyme that she needs. With the missing enzyme her vitamin B6 levels get low, which can cause seizures. She has been on doses of B6 for 2 days, and hasn't seized since, Praise God! There is one experimental drug that is in the second phase of testing that our geneticist is looking into. We obviously have to weigh the benefits and risks before considering it, but it is something we are checking out.

Prayer Requests: Evie would stay stable, and without seizures. That she will be able to take a full bottle soon, so they can remove the nasogastric tube that they are feeding her through, and that her oxygen stats would stay good so we can GO HOME. I am praying praying praying that we can go home tomorrow, but they may have to watch her picc line for 24 hours, which puts us at Friday. Not only is it hard to be here, but I sure miss Lyla. She is a little "off" from us spending so much time here, and it would be so nice to get things back to normal for all of us.

Thanks friends, Love you!

Lindsey and fam.

She Woke Up!

GREAT news friends, Evie woke up this afternoon!

I decided to rub a little baby lotion on her dry arms and legs, and she opened her eyes and we talked for a while (: About 10 minutes later she had an EEG, so she had to go back to sleep for that, but she woke up one more time after that. She sucked her binky for a few minutes, which is another praise since she hasn't done any good sucking since yesterday and they were feeding her through a nose-tube (I'm too tired to think of the real name for a nose-tube).

So Praise God and thank you for your prayers. I think we get to move back upstairs to the Intermediate Care floor soon, I will keep you all posted.

THANKS for checking in and praying for my sweet little one!!!
Lindsey

PICU...

Hi Again,

We discovered yesterday that Evie is indeed having seizures, and during an EEG she was actually seizing with no outward signs of a seizure. This means she may be having small seizures between the large ones that we experienced this past weekend. She also had some interesting findings in her blood-work that the geneticist thinks may be leading him to a diagnosis of what she has. He is positive about finding a diagnosis, but not too positive about the way things are going right now, especially with the addition of seizures to her already long list of symptoms.

Last night Evie was brought down to the Pediatric ICU. She took a bottle around 3:30pm Monday and I tried to feed her in the hours following, but by 9pm-ish she wouldn't open her eyes, respond to my voice, or take any kind of bottle/binky. They thought she might be seizing constantly, so she was brought to the PICU to start seizure medicine and keep a close eye on her breathing. Our pediatrician, the neurologist, and geneticist were all here within the hour, which if you have tried to get ahold of any of these people, you know is pretty miraculous, yet clued us in to the severity of the things they were looking at.

After another EEG they ruled out constant seizures, and we may be going back upstairs sometime today. Her oxygen level is all over the place, staying at 100 for a few minutes then dropping into the 80s, low 90s, and back up to the upper 90s. She has to stay above 93 for a significant amount of time before they will let her go home. It is looking like we will be here for a few days at least.

Since moving to the PICU last night around midnight she has had a CT scan, another EEG, a spinal tap, a urine sample, and more blood work. She is getting the works to try to find the cause of this unresponsiveness.

PRAYER requests: #1. that Evie comes back to being her normal self soon. It is really scary to watch her just lay in her bed. #2. That they an come up with a TREATABLE diagnosis, and that the diagnosis will be less severe than they are thinking right now. and #3.That John and I get some sort of rest while still managing to talk to all the doctors and get the information we need.

God is good all the time. We know (from experience!) that no matter the diagnosis, her prognosis can change at any time, and life threatening diagnoses are not always life threatening (:

Love you friends,
Lindsey

We're in the hospital...

As Lyla would say... bummer dude!

After a great 2 weeks of being home, we are at Children's Hospital. Evie had a short seizure-like episode last Friday night but when we brought her in they didn't see much they could do... last night (Sunday) she had a much longer episode of straining, stiffening, some tremors, and some abnormal breathing, so we had her brought in by ambulance to check her out. They have admitted her to Children's and are going to do an MRI, EEG, have a neurologist check her out, and the geneticist look over her again. First they need to know if she is indeed having seizures, then they can determine if it is part of the skeletal issue that she already has, and then how to treat her.

If you could pray that 1. she doesn't have any more episodes, 2. that the doctors can find the cause of what's happening, and 3. we can get her unhooked from all of these monitors they have stuck all over her little body we would greatly appreciate it.

Love you friends!

Lindsey & Fam

Psalm 116:2 2

Because he turned his ear to me,

I will call on him as long as I live.

Thanks September and JD!

We had so much fun this morning going on KGBI's "Thursday Morning Praise" show. Thanks to September, JD, and KGBI for allowing us to share Evie's story of God's continuing goodness to us!