Every day is a gift...

"I want you to understand that this is serious...this is very dangerous to her...this could take her..."  These are the words we heard from Amelia's cardiologist just a week ago.  They still play over and over in my mind.  Amelia had a follow-up with her cardiologist because they noticed an increase in her aorta at 2 weeks old ("aortic root dilation").  This time it had grown drastically at an increase that he did not expect to see; so he had to prepare us for the worst.  He said they could do surgery and you could replace this part of the aorta, but then the rest of the aorta is affected, and therefore it wasn't an option.  The plan then was to put her on medication and hope for the best, but we have to know that it may not work that well.  Then it was question and answer time...  I just wanted to get out of there as soon as possible.  But to where?  Where can you run from news like that?  We asked some questions we never thought we'd be asking...  How long does she have?  Is all this treatment on her hands, legs, feet worth it?  Is this condition painful for her?  How will we know if something is wrong?  We don't know how long she has; high rate of mortality in the first 5 years...yes, the treatment is worth it....no, it's not painful for her..."for a baby with an aortic tear, it's going to take her and you're not going to know it."     

After the doctor walked out of the room, Nels was holding Amelia and staring at her through sobs... I looked out over the city and it was getting busy; it was 5:00 so the workday was done.  I had just heard that I could wake up tomorrow and my daughter could be gone...how could it be?!  Her life has hardly begun!  But, life goes on... Through many sobs and tears, we finally made our way home. 

Today...  this last week has been a whirlwind.  On Friday of last week, they wanted to admit Amelia to regulate the new blood pressure med they were putting her on, and then they decided to back off a little and first start her on a "beta blocker."  In layman's terms, I understand this will loosen up her vessels so that there's not so much pressure on the aorta.  The plan is to eventually start her on the blood pressure med (formulated for her size, but used frequently in adults).  Studies have shown that using this particular medication has given them some good results with patients diagnosed with Loeys Dietz Syndrome (LDS).  LDS has become a common term around here in the last few weeks.  Along with a few other characteristics, this heart issue leads them to believe Amelia has a connective tissue disorder, most likely LDS.  The blood work has been sent in and we will wait anywhere from 12-24 weeks before we know if this is, in fact, what Amelia has.  Therefore, at this point, they are treating her as such.

On Tuesday, we are going to get a second opinion from another cardiologist in the same office.  In fact, I was also able to connect with a genetics counselor from John Hopkins this week who works closely with the Dr. Dietz.  She gave me a phone number for the cardiologist to be able to contact Dr. Dietz directly!  Our Great God at work!!  

This week Amelia had occupational therapy on her hands and it went so well.  Nels and I were both very encouraged.  On Wednesday, Amelia had her last cast put on and was fitted for braces.  That appointment also went very well.  We will be revisiting the casts at another time, but for now, we are going to celebrate when that last cast comes off!  The bad news was that the braces will be on around the clock (except for baths) indefinitely.  The right foot is giving them a lot of trouble and until she has surgery on that foot (tendon transfer) she will need to be in braces.  Typically, they do not like to perform that surgery until the patient is about 5 years old, but in Amelia's case they will most likely do it a little bit sooner.  According to Dr. K., the tendons and bones have to grow in able to work together to accept each other.  However, my perspective has certainly changed.  We'll deal with braces as long as we have our little girl!  That's what matters most!

Removing the casts...just relaxing!  I'd say we're getting used to the routine!  :)

Lookin' good!

To end the toughest week in our lives, we celebrated Amelia's baptism this past Sunday...this was not by accident.  It was an absolutely beautiful service and without a doubt, I could feel God's presence.  As I looked out over the congregation, all I saw was love and care and support.  Then seeing our friends and family praising God with us for our precious Amelia meant more to me than words could ever express!  I realized at that moment...this is what it is all about.  God's plan for our lives could be drastically different than we ever planned or imagined; I NEVER thought from the ultrasound at 20 weeks that this journey would take us here, but I have come to believe (even when it's so hard) that God has a good plan.  Along each turn I have thought to myself, will I really be able to keep saying this if things take a turn for the worse?  I have to believe I will.  That doesn't mean it's easy and that's why I also believe this would not be possible without SO MANY lifting us up in prayer!  A line from one of my favorite songs right now, Oceans by Hillsong United, "Where feet may fail and fear surrounds me, You've never failed and You won't start now..." 

My dear blessings!!

"We want to do a silly one!"

So thankful to be celebrating with Gigi!  Great Grandma is 97 years old and Amelia almost 97 days old!  God is good!

Leaving you with a couple thoughts.  My mother-in-law introduced me to this book, Mo - A Loeys Dietz Syndrome Memoir.  This book was written by Kate Jurgens, whose daughter is 17 years old with LDS.  This is a quote from her book...

"So many activity restrictions...had lead to one of Maureen's deepest pleasure discoveries - reading.  Amelia Earhart captured her vibrant imagination and inquisitive mind.  'Mom, Amelia was so brave to learn how to fly.  She went ahead and did things people thought she could never do.  I don't know if I could be that brave.'" 

Psalm 61 has now become very special to us; it was the scripture at Amelia's baptism... 

Hear my cry, O God; 
listen to my prayer

² From the ends of the earth I call to you,
    I call as my heart grows faint;
    lead me to the rock that is higher than I.
³ For you have been my refuge,
    a strong tower against the foe.

⁴ I long to dwell in your tent forever
    and take refuge in the shelter of your wings.^[b]
5 For you, God, have heard my vows;
    you have given me the heritage of those who fear your name.

⁶ Increase the days of the king’s life,
    his years for many generations.
⁷ May he be enthroned in God’s presence forever;
    appoint your love and faithfulness to protect him. 
⁸ Then I will ever sing in praise of your name

    and fulfill my vows day after day.

Lead me to the Rock that is higher than I, dear God...

Oceans, by Hillsong United

What is arthrogryposis?

A lot of you have inquired about Amelia's condition and so I have been promising to deliver..  Also, an update on how she's doing.  

As many of you know, Amelia has arthrogryposis, which I also mentioned in an earlier posting.  The Wikipedia defines arthrogryposis as...

"Arthrogryposis multiplex congenita (AMC), or simply arthrogryposis, describes congenital joint contractures in two or more areas of the body. It derives its name from Greek, literally meaning "curving of joints" (arthron, "joint"; grȳpōsis, late Latin form of late Greek grūpōsis, "hooking").^[1] Children born with one or more joint contractures have abnormal fibrosis of the muscle tissue causing muscle shortening, and therefore are unable to do passive extension and flexion in the affected joint or joints.  Arthrogryposis is a not a diagnosis but a clinical finding. So this disease is often accompanied with other syndromes or diseases." (http://en.wikipedia.org/wiki/Arthrogryposis)

Amelia has distal arthrogryposis which means she has multiple contractures of her distal joints; her hands, knees and feet.  Her hands are clenched; sometimes they're really tight, meaning it's very difficult, if at all, to get one of my fingers in her grip.  Then sometimes they're a little more loose and I can slide my thumb in there for her to grab onto.  Her knees are bent at about a 100 degree angle.  And, then of course her feet; a severe atypical case of club feet.  It's hard for Amelia's doctors to tell us how long and what kind of treatment Amelia will continue to require because each step depends on how she responds to the different treatments.  However, they can tell us that there will be some form of treatment of the affected joints until she's done growing (or longer) because if you don't keep up with it, the joints will contract back into their original position.  Just out of curiosity, I looked up the average age a girl stops growing which is 18 years old.  We have fallen into a routine now of weekly (sometimes more) visits to the doctor and are starting to realize and accept that Amelia will require treatment for a long time.  

Tomorrow, Amelia will get cast #9 put on.  This last cast was on 2 weeks following her tenotomy and will be changed tomorrow.  Dr. K said the number of casts following this one will be determined on, again, how she responded to the surgery and casting this time around.  However, for future reference he did tell us that following this casting, she will be in braces around the clock and also start physical therapy on her knees.  Once they get a feel for what they can achieve with therapy, they will then do casting of her knees.

Her hands...  We are scheduled to follow up with Amelia's hand surgeon next week.  We met with him shortly after she was born.  He believes she will respond well to treatment, however he did confirm that because Amelia's hands will never fully open like ours, she will just function different than us, but she'll still be able to use them...that was good to hear.  So, in making sure that's the case, he said at this point, he does not suggest we get very aggressive with therapy and splinting.  He said splinting and exercises are fine, but whomever is doing it, has to really know what they're doing.  Relaying this to Amelia's therapist and then fine tuning her treatment was a little bit of a bump in the road, but I think we're on the right path now with a therapist who has specifically worked with arthrogryposis.  This was an answer to prayer!  Right now, we have to do 2 different stretches with Amelia at least a couple times a day, more if possible.  She also has splints now for both hands.  We got one fitted last week and since she was not happy about it, we had to get the other one done yesterday.  These splints are for "resting" only, because while she sleeps (or rests), that is when her hands are more loose so we're able to get the most stretch out of them.  The resting is difficult because Amelia doesn't really like to sleep for us during the day, so hopefully the nights will be enough.

Tried to sneak them on while she was falling asleep...without success  :)

On Thursday, we have an appointment with Amelia's cardiologist.  At 2 weeks old, he saw Amelia for a follow up from a diagnosis in the hospital of PDA (Patent Ductus Arteriosus).  At that time, he saw some dilated vessels in the heart that concerned him.  So, this appointment is important to us.  We hope to get some clarification on her condition and hopefully those vessels are measuring back to a normal size.  However, at her recent 2 month check up with her pediatrician, he still heard a heart murmur, which lessens that possibility.  But, we know that if this is God's will, anything is possible, so we hold onto that hope and if the news isn't what we want to hear, we will still trust Him.  

Amelia will also be getting some blood drawn this week.  I just received a call last week that our insurance company authorized genetic testing for a connective tissue disorder that could be serious, so her doctors have decided to go ahead with testing.  Results will take 6 weeks to come back.  Since Amelia was seen by her genetics doctor at only 1 week old before any treatment, they were able to point out some characteristics that line up with her arthrogryposis to make this syndrome a very real possibility.  Another reason her appointment with the cardiologist is an important one.

The days are going by so fast...  Not knowing what lies ahead for Amelia can weigh heavy on our minds, but I have also been able to put that worry aside so that I don't miss being able to take in this time with my little Amelia, and Elsie and Sam for that matter.  I will never have these moments of smiles and coos again once she passes that stage.  This again is only achieved through prayer, I believe!  I'm usually the worrier to the point it consumes me at times. So, when I stop to think about that, it's pretty amazing!    

Good morning!!

Please continue to pray that:

Amelia will continue to respond well to treatments and tolerate her splints...
That the appointment with the cardiologist will be a positive one with some clarification...
Elsie and Sam will continue to adjust well...
God will give us perseverance each week...

So, with that, I would like to share with you another verse that a good friend of my mom's, who battled cancer, shared with me.  God's promises...

"I WILL LEAD the blind by ways they have not known, along unfamiliar paths I WILL GUIDE THEM; I WILL TURN the darkness into LIGHT before them and MAKE THE ROUGH PLACES SMOOTH. These are the things I WILL DO; I WILL NOT FORSAKE THEM."  Isaiah 42:16

Thank you again for continuing to support us in so many ways!!  We have been SO blessed!  In fact, this Sunday our Amelia will be baptized!  We are so excited to officially commit her to Christ and in realizing she is His first, we will do our best, with His help, to raise her to love Jesus with all her heart!  If you would like to join us, you are welcome to come!  If you need the details, leave me a comment and I'll be in touch!

"I have called you by name; you are Mine."  Isaiah 43:1

So far, so good...

Surgery went well!  Dr. K said she did get a little upset at one point, but they got her settled down and for the most part, she did great!  He said she will most likely hit a rough patch tonight when the numbing wears off, but now that she is 2 months old, we are able to give her Tylenol, which I am thankful for!  So, we are going to stay on top of that and hope it helps! 

As far as surgery, he was able to successfully stretch the Achilles tendon in both legs and pull the feet up.  She will now be casted for 2 weeks straight instead of one.  She will be having a couple more casts after that and then they'll reevaluate to determine whether she will need more casting or not.  Pray she responds well to the treatment and hopefully this set of casting will be done sooner than later!

THANK YOU for your encouragement and most of all your prayers!  I can say, without a doubt, God heard your prayers today for our sweet Amelia!

A friend shared this verse with me this morning from Psalm 121:1-2.  What I love about this verse is that THE Creator of Heaven and earth was with each of us this morning (and always)!  How awesome that is!! 

"I lift my eyes to the hills--

where does my help come from?

My help comes from the Lord,

the maker of heaven and earth."

Amelia's very first surgery is coming up!

On Wednesday, our sweet Amelia will be having a minor surgery on both legs; a bilateral Achilles tenotomy.  This surgery will stretch the tendons in each leg in an effort to bring the feet up.  She will then be casted again immediately following the procedure for another couple weeks.  Her response to this part of the treatment then will determine how many more casts she will have to have. 

The plan was to have the surgery at the hospital, but because the doctors decided that the anesthesia would be too risky, they will be doing this in the office.  I am relieved because I was most worried about the anesthesia part of it, but on the other hand, I hope it's not more painful for Amelia.  Another reason they wanted to do it under anesthesia is because Amelia moves so much.  It sounds like they're pretty confident they'll have it under control, even if it takes more nurses to hold her down.  :(  The numbing will be similar to what you would get in the dentist's office if you have ever had a filling or any other type of procedure where they first numb it topically (applying the numbing cream to the area) and then give you a shot to numb it the rest of the way through.  So, asking for prayers on Wednesday that:

1.  She will respond well to this part of the process and surgery will be successful in correcting her club feet for now
2.  She will feel no pain or discomfort during the procedure
3.  That she will come out of the procedure itself with minimal pain over the next couple weeks, if any

Thank you again for ALL the prayers!  I have received so many messages letting me know they're already lifting us up in prayer for Wednesday.  It's so comforting and brings me to tears!  

Again, I promise to update you on Amelia's prognosis in the very near future!  I received a call from the genetics doctor last week that I want to update you on.  We are going to really need some prayers for upcoming appointments and tests, so I will do my best to update you on that very soon!

In fact, in the meantime, another prayer request would be for Elsie and Sam.  It seems to be taking it's toll on them lately, especially Elsie.  I know a newborn is an adjustment in itself, but the extra strain on all of us has been hard on them.  So prayers for them too would be very much appreciated! 

I am going to leave you with some verses that I didn't even realize were right in front of me while writing this post.  And, of course, the words are perfect and just what I need to hear from Isaiah 40:26-31.  Another AWESOME reminder that God is taking care of us!  He never ceases to amaze me!

²⁶ Lift up your eyes and look to the heavens:
    Who created all these?
He who brings out the starry host one by one
    and calls forth each of them by name.
Because of his great power and mighty strength,
    not one of them is missing! 
²⁷ Why do you complain, Jacob?

    Why do you say, Israel,
“My way is hidden from the Lord;
    my cause is disregarded by my God”?
²⁸ Do you not know?
    Have you not heard?
The Lord is the everlasting God,
    the Creator of the ends of the earth.
He will not grow tired or weary,
    and his understanding no one can fathom.
²⁹ He gives strength to the weary
    and increases the power of the weak.
³⁰ Even youths grow tired and weary,
    and young men stumble and fall;
³¹ but those who hope in the Lord
    will renew their strength.
They will soar on wings like eagles;
    they will run and not grow weary,
    they will walk and not be faint.