Saturday, February 17, 2018

A different outcome...

Well, some disappointing news today.  Surgery on Amelia's right leg did not go as planned.  When her surgeon removed the cast from her left leg that she had surgery on 5 weeks ago, he noticed that something wasn't right.  An xray showed the plates in her femur moved and the screws were coming out.  We're not sure of the cause for this, but her bones were not healing as expected at this point, but Amelia has had trouble healing at times for minor things because of her Loeys Dietz Syndrome.  So, Dr K decided to just focus on repairing the left leg today.  There is now a double plate and 6 screws instead of 4 to hopefully prevent this from happening again.

In 4 weeks we'll reevaluate, do an xray and if the the left leg is doing well, we'll schedule surgery on the right leg.

To make sure this doesn't happen again, Dr. K put Amelia in a spica cast - her left leg is casted and the cast then extends up around her chest to her arm pits to keep that leg from moving at all. 

One of the first things Amelia said after waking up in recovery was that the "wrong" leg was casted, she was so disappointed.  She is still trying to figure out why and is asking a lot of questions and of course, just wants to go home.

As I contemplate this tonight...I am thinking about all of you.  Our only hope is found in our Savior, Jesus Christ, and when you pray to Him on our behalf, we know He is listening and cares about every single detail.  Thank you for blessing us in this way!  We truly appreciate your prayers!  At this point, these are some of our specific prayer requests...

Please pray that healing goes well this time so we can move ahead with surgery on the right leg.  In the meantime, pray that God would give Amelia's doctor guidance and wisdom in making the best decisions for her.  He was surprised and very disappointed with this setback.

Pray for Amelia, that she will be able to accept her situation and thrive when she's just too young to understand why.

Pray for us as a family as we adjust to yet another casting hurdle; it's been a lot for Elsie and Sam to go though over the last 4 years.  Pray that we would have patience, understanding and kindness with each other when we're feeling discouraged.

Thank God that He is in control and nothing surprises Him.  We can trust He cares deeply about Amelia and will bring everything together in HIS time.

Our pastor saw Amelia before surgery and reminded us that "we are precious in His sight" not fear because we are His and He is always with us (Isaiah 43).

Thursday, February 15, 2018

Time for round 2....

Tomorrow Amelia will be having surgery on her right leg.  She had blood drawn a couple times since the last surgery and the most recent report showed a lot of improvement in her hemoglobin, so her blood management team gave the go-ahead for surgery #2.  The plan is to approach it similar to the left leg, but her right leg is more complicated and has always been more stubborn, so please pray with us again for a successful surgery!  They will also remove the cast from her left leg (in surgery) and her orthotist  will be in there to fit her for a KFO (Knee Foot Orthosis).  This is a leg brace that is like her AFOs (Ankle Foot Orthosis) that she has always worn, but will extend above the knee.  

Elsie had a birthday party last weekend
and Amelia wanted to try out the
"photo booth" too.
I always feel guilty that I am not good at keeping you up to date on Amelia's progress and I apologize for that.  But, I am happy to report that she has been doing really well the last couple weeks.  The first few weeks were tough, especially at night.  And she fought her pain meds at home just as much as she did in the hospital, so that was always a struggle.  Although, for the most part, her days were pretty good.  She would have periods of being very uncomfortable or painful at times, but it went much better than I expected.  Of course, she learned how to scoot around and get where she wanted to.  Then, the second week in, she came down with a high fever.  Assuming it was influenza, like what her older brother had the week before, we treated it as such.  But, she started vomiting a lot and Sam never did.  So, we called her surgeon in the morning and they had to see her to rule out an infection.  They removed part of the cast and the incision looked good - so thankful!!  They replaced that part of the cast and we went home with 2 different colors now.  :) 

Now we face round 2.  I'm more confident than I was before surgery #1, but yet more nervous knowing how difficult the left leg was and now with the more complicated leg...I'm not ready to go through it again, but at the same time, I'm ready to get it done!  She told me tonight that she will have surgery tomorrow, "but I do NOT want surgery ever again."  Unfortunately, this won't be the last, but we won't worry about that now.  

I can't thank you enough for all the prayers and thoughtfulness during this time!  I love to read all the emails, messages and texts - they're so encouraging and the thoughtfulness alone means so much to me - even when I'm not good at responding to those either.  :(     

I would also like to thank those who have brought us meals since Amelia's surgery.  That is such a huge blessing to our family and helps us out so much!  We have received so much support in other ways too and it means more than you will ever know!  Amelia has also loved all the cards and thoughtful gifts too!  I said it before, but to know that she means that much to others - I can't really even express how that touches my heart.  Our dear friends from church gave us a gift before Amelia's last surgery and part of it was a devotional by Joni Eareckson Tada called "Pearls of Great Price."  It couldn't have come at a more perfect time.  Especially with the recent loss of my dad too.  The devotion on February 12 was called, "A Soft Heart."  It seems to capture how important this encouragement from friends and family is when you're on the receiving end.  

"Focus on suffering is a dead end.  Rationalizations and justifications do nothing but harden our hearts, making things worse.  However, softness of heart comes when we encourage one another in our suffering - spreading truth, imparting hope, bearing the burden, sharing the load, praying alongside, and offering a comfortable shoulder to cry on."  

"For I am the Lord, your God, who takes hold of your right hand and says to you, Do not fear; I will help you."  Isaiah 41:13

Thank you for continuing to lift Amelia and our family up in prayer!  Of course, we pray that everything will go well tomorrow with the surgery, but this wears on our family as a whole too.  Please pray for understanding, peace and love for each other as we jump another hurdle together.      

Saturday, January 13, 2018

Update post surgery...

I know a lot of you have been waiting for an update; so sorry for the delay!  Amelia's surgery went well!  Praise the Lord!! However, her doctor only did the left leg because it did end up being very complicated - he said it was about as difficult as it could get, but he was ready for it!  Her stats, including her blood pressure remained stable, so prayers answered there!  She had a lot of nausea and vomitting after surgery until about mid morning today.  She also started with a lot of pain in post-op already saying her cast hurt :( and he even put an epidural in to numb that area, and it's still in.  But, she still has quite a bit of pain, so they added some more pain meds, so hopefully that will help!  She's just very uncomfortable.  No smiles today.  :(  She just begs us to take her home.

Amelia also lost a lot of blood during surgery and that's not helping anything either.  They started iron supplements through IV last night and added some orally this morning, but her hemoglobin kept dropping down to a 5 (normal is 13-14).  So, she is now getting a blood transfusion and we have already seen her perk up a bit.

Her heart is stable yet, but the cardiologist will continue to follow up on her here to make sure some of her abnormal stats are due to the blood loss.  Her cardiologist is through U of M, so we see the one here.  But, he's friends with Amelia's cardiologist so that's an answer to prayer!

Thank you for your continued prayers!!  We all appreciate them!

A dear friend texted me this verse this morning and our pastor read it before Amelia's surgery yesterday.  It was also read at her baptism 3 days after we thought we were going to lose her....God has been faithful!  I needed to be reminded of that!   "Hear my cry, Oh God, listen to my prayer: from the end of the earth I call to you when my heart is faint. Lead me to the rock that is higher than I, for you have been my refuge, a strong tower against the enemy."  Psalm 61:1-3.

Thursday, January 11, 2018

Fast forward to January 9, 2018....

Hello!  It's been a while!  Thankfully, I can say our Amazing Amelia has been thriving in many ways.  Her personality just shines!  She has been walking with special braces on her legs with and without her walker - which is amazing!  She's so determined!  She started swimming a lot this past summer, which she really enjoyed!  She also started a dance class doing "creative movement" and that has been a joy to see.  Elsie went with her to make sure she could be a part of the routines as much as possible - it's very sweet.  She also started preschool this past fall and loves that too!  Her teachers are wonderful and help her in any way they can. She is a very happy little girl and for that we are so grateful! 

"As for you, you meant evil against me,
but God meant it for good..." 
Genesis 50:20

"His pleasure is not in the strength of the horse, nor His delight in the legs of a man; The Lord delights in those who fear Him, who put their hope in his unfailing love."
Psalm 147:10-11

In the last post, Amelia was in the process of having both legs casted in an attempt to straighten her knees.  That did not go well at all.  Because of the position of Amelia's feet and knees, she could not tolerate the casts because sores had formed on her heels.  With Loeys Dietz Syndrome, the skin is thinner, so these sores took a very long time to heal - the effects of them are still visible (over 2 years later).  So, for that reason, another try at casting was out of the question. 

Since then Amelia has been wearing her AFOs (Ankle foot orthosis) and special braces made for her legs.  The braces for her legs are similar to what the casts did, but it was up to us to slowly stretch her knees with a ratchet on the side.  We could now take them off for baths or swimming - huge bonus!!

We call this the "Mia Bobber." 
Amelia's sweet Uncle Mitch made this for her!
As you can tell, she loves it!
Amelia still wears these braces, but only for about 3-4 hours at a time.  Then they become very uncomfortable for her.  However, she also wears them at night too along with braces on her feet (Ponsettis).  But, when she's not weight bearing (because she's laying down), they don't bother her as much.  In the end, conventional treatment to straighten her knees has not been working and her orthopaedic surgeon made the decision to do surgery, which was last resort because of the risks involved.  Surgery was always going to be necessary to move her dislocated knee caps, which formed way off to the outside of her knees, but this surgery will include much more.  The risky part is loosening and tightening those parts in the back of her knee just right (while also avoiding nerves) to assist in getting her knees straight.  The bones below her knees are twisted, so those will have to be realigned.  Then everything above her knee has to be adjusted as well.  Her surgeon doesn't even know everything he will have to do until he gets in there, which could include the shortening of some bones.  Because this surgery is so involved, he will only be operating on one leg at a time.  The first surgery will be this Friday and the second one is scheduled for 2 weeks later.  She'll be casted following both surgeries for about 8 weeks.

So, going into this Friday, we have a few specific prayer requests.  Please know we covet your prayers and do not take them for granted! 

- There's a chance if everything goes very well, her surgeon might be able to do both legs at the same time; not likely, but possible!

- That Amelia's pain will be manageable...

- The incision will be big, so there could be a lot of blood loss which means a blood transfusion might be necessary, but hopefully not...

- That she'll tolerate the casts and that her skin will be able to handle it...

- Amelia has a serious heart condition (aortic root dilation/aneurysm) and in addition to that, there was a concern about her blood pressure dipping really low during anesthesia for an MRI she had in November in preparation for this surgery. Please pray that her blood pressure stays manageable and her heart condition stable...

- That the surgery will be a great success and she'll be able to walk!  Without her leg braces would be a huge bonus!  Amelia has her heart set on that. :)

February, 2017
On December 9, we lost one of Amelia's most valuable supporters, my dad and Amelia's papa.  Our last prayer request would be that you would keep Amelia, and our family, in your prayers as we grieve his loss and at the same time face this surgery for Amelia.  We are so happy for him knowing he is finally at Home with his Heavenly Father, but we miss him so much - especially his love and support!  My dad was also disabled and had braces as a child, major surgeries and pain in his legs (even until the end), so him and Amelia had a very special connection and I already miss his ability to relate and understand, unlike many of us.  But, Amelia knows he is in heaven and confidently says "he can do anything now!" - and that's what's most important to her!  Her papa would be proud of her - an inspiration she is and has always been to us! 

I would also like to take this opportunity to thank many who have been so supportive throughout the last 4 years and especially lately in light of Amelia's surgery.  Our families and friends have been with us through this journey and continue to hold us up!  Many have already surprised us with special gifts or offered support in different ways.  None of it goes unnoticed and it means more to us than you will ever know!  

We are also thankful for our church family who has always supported us in many different ways!  We are so grateful that Amelia means so much to them!  Another family that is important to us is our school family.  They have provided and cared for Amelia in many ways as well.  Last night, some very special friends, that are also teachers at the kid's school, put together a prayer covering for Amelia and our family.  It was beautiful and such an honor to be a part of.  Thankful for a community of believers in Christ at church and school that know the power of prayer! 

Through all this love and support, I can feel God holding us up saying, "See Julie, I got this!  If only you just trust Me!  'I am making all things new!' (Revelation 21:5)"  Pray that we would trust and rest in the assurance of His faithfulness! 

My mom gave me this flip calendar many years ago and I still treasure it.
My Father's words for the 12th day of January are perfect.
Songs are a great source of encouragement to me and these are just a few, of the many I love, that I have been listening to lately - listen to the words and I hope they encourage you too today! 

Wednesday, October 21, 2015

Round 2 of casting

Four weeks ago, Amelia had both legs casted to try and straighten her knees.  Amelia did so good through almost the whole procedure that I knew our prayers were being answered and I was very thankful for that!  The first tears came after they were on; everyone left the room and she realized she couldn't move her legs.  I felt so bad for her.  The crying didn't last long, but it was only the beginning of five long, tough days.  The first couple days are supposed to be the hardest, but then as she gets used to them, it should get better.  The first night was terrible.  Amelia woke up every 20 minutes with painful screams; it was heartbreaking.  I think we only got through that night because we were assured it would get better... it didn't get better.  Prior to this, Amelia was taking 2 hour naps and sleeping through the night and now she was barely sleeping at all.  I had a feeling something was wrong right from the start.

On Friday afternoon (day 2), we determined that Amelia might be having muscle spasms.  So, her doctor gave her a prescription and we were looking forward to some relief for Amelia and us!  However, she couldn't keep it down.  We tried everything we could to disguise it...nothing worked.  We were so disappointed.  Over the weekend, Amelia seemed to be getting used to the casts and getting around a little more, but she still wasn't sleeping.  The muscle spasms seemed to be less frequent, but worse when they did happen and there was nothing we could do about it.  By Monday, Amelia was still very restless at night and had not taken any naps, so her doctor decided it was time the casts came off.  After getting them off, we discovered sores on both of Amelia's heels.  :(  The plan was then to keep her doctor updated on their healing and go from there.  Amelia fell right back into her routine of sleeping, which I was so thankful for! 

Sparkly hot pink, especially for her big sister, Elsie.  :)
Today, Amelia is scheduled to have the casts back on.  The sores are still there, but mostly healed.  The plan is to change the casts weekly for as long as it takes to straighten her knees.  However, this set will be on for 2 weeks. 

Thank you so much for your prayers for Amelia and the rest of us!  Those five days were so hard on all of us that we need and so appreciate your prayers!

Some specific prayer requests then would be...

...that the process of getting the casts on goes smoothly.  I'm a little nervous how it will go this time around because of her bad experience.

...that her legs, feet and especially her heels are protected from irritation.

...that she will not get any muscles spasms or pain from the stretching.

...that she is able to sleep comfortably with them on.

"In the natural, many things are impossible.  But in the supernatural realm with God, nothing is impossible.  God wants us to believe for great things, make big plans, and expect Him to do things so great that we are left with our mouths hanging open in awe... We can be bold in our asking, and Ephesians 3:20 challenges us to ask for big things."   - Joyce Meyer

"Now to Him who is able to do immeasurably more than all we ask or imagine, according to his power that is at work within us..."  
Ephesians 3:20
 Jeremy Camp - Same Power

Wednesday, September 16, 2015

The next step

Today is a day I have been dreading for a while.  This afternoon, Amelia will start casting on both of her legs.  Not necessarily related to her Loeys Dietz Syndrome, Amelia was born with multiple contractures, at which time they diagnosed her with Arthrogryposis.  This involved her hands, knees and feet.  According to WebMd,  "A contracture is a condition in which a joint becomes permanently fixed in a bent (flexed) position, completely or partially restricting the movement of the affected joint."  (  In other words, Amelia's knees do not stretch out all the way, they are bent at a certain degree and will not stretch out beyond that point.  While in therapy, the physical therapist was able to straighten it out more than it was at birth and we tried several different things to straighten them more, but with Arthrogryposis, if you don't keep working with those muscles, they will contract back to what they were orginally.  So because we were never able to get them stretched out all the way, she wasn't able to walk and therefore use those muscles, so in the end it would be a losing battle; in fact, they have already contracted back since we stopped therapy earlier this summer.  In addition to the contractures, an MRI in May showed that Amelia's knee caps were congenitally dislocated, which means they formed way off to the side of her knees.  {This is explained in a little more detail in the last post.} So, Amelia's orthopedic surgeon had to come up with a different plan.

Since we're dealing with two different issues, the first step is to get the knees straight and then perform surgery to move the knee caps and all the mechanics that go along with it.  Amelia's surgeon called me in June to let me know that he had been talking with other doctors around the country and even brought up her case at a "Complex Case Conference" because her separate issues are rare, but even more rare is the combination of them.  Her surgeon calls it a "unique scenario," so unfortunately there is not a lot of experience in her situation, if at all.  So, casting is a start, but he is not convinced it will work.  However, straightening her knees during surgery makes the already complicated surgery of realigning her knee caps even more difficult, so we have to try.  He anticipates the casting to take a few months, but some of that depends on Amelia's cooperation and he's not sure she's going to take it too well.  That's what I'm worried about...  That's where we need your prayers!  Right now, I can only focus on how hard this could be on Amelia; I don't want to have to watch her struggle or suffer.  But, if I'm able to put it into perspective, this is the first step we need to take to get our little girl walking.  We had a break this summer from therapy and appointments, now it's time to look forward to a positive change.  One step at a time never became more literal to me.

I love it how God knows exactly what we need and reminds us of it in ways unknown when we least expect it.  Yesterday Sam was home from school and while outside, he wanted to have a pretend picnic with treats.  He got it all set up and then proudly announced, "Let's pray!"  He started with the Lord's prayer and came to "Give us this day our daily bread as Amelia gets her casts on.  Amen."  I was stopped in my tracks...that's exactly what I needed to hear and it came from the lips of my little boy!  When I reflect on this, my prayer is "Give us just what we need for today dear God, remind me to take this one day, one moment, even, at a time."  What an awesome God!!  

Here's a short video showing how Amelia gets around...with a little music in the background by Elsie. ;)

Would you please pray for us today?  Some specific requests would be...
  • that the casting will go smoothly and Amelia will feel little if any pain...
  • that her doctor's hands will be swift so that the procedure doesn't take too long for Amelia to be held down...
  • that Amelia will adjust well to them and pleasantly surprise us...
  • for Nels and I as we long to have her walking, but hate seeing her go through this process to get there.  We hope and pray it doesn't dampen our little girl's spirit...
  • Pray that it works so that we don't have to do surgery on this too. 

Taken just this morning at my grandma's house

"Hope deferred makes the heart sick, but a longing fulfilled is a tree of life."  Proverbs 13:12

Sunday, July 19, 2015

Take Heart!

Earlier this month, we saw Amelia's cardiologist and I'm very happy to report that it was a great visit!  She was all smiles when we got there, but when it was time for the EKG, she slowly realized what was going on and when she did, she wanted nothing to do with it.  We made it through even though she wanted to keep pulling the cords off.  We were worried the echocardiogram would not go well either, but she did great!  I was so proud of her!
A little re-cap first since I didn't blog about this...Amelia had a heart catherization at the University of Michigan in May.  She was born with a PDA (Patent Ductus Arteriosus).  Every baby is born with this, but under normal circumstances, it closes shortly after birth.  Sometimes, as in Amelia's case, they don't close on their own and this is very common in kids with LDS (Loeys Dietz Syndrome).  Since Amelia's aorta is already dilated, her cardiologist was hoping that by closing it, it would take some pressure off the aorta and stop it from growing.  During the heart cath, her cardiologist used a coil to plug the hole so that blood would not flow through there anymore.   

When Dr. G discharged Amelia later that night from the hospital, he also mentioned that her pulmonary artery was dilated as well (in fact, he said it was 50% larger than normal).  At the time, I asked him what that meant, and he explained it to me, but said he would get a better picture of it at the echo (in July).  I couldn't stop thinking about it though.  At the appointment he said that it is very common for any of Amelia's vessels to dilate because of her LDS and the pulmonary artery is one of them.  When the aorta dilates, it has to be watched very closely because it can not handle that kind of growth and if it dissects, it can be fatal, but with the pulmonary artery, it can dilate quite a bit before there's any cause for concern.  That put me at ease.  But, he said Amelia will have an MRI at least annually to look at the arteries throughout her body to watch for any growth that is would require treatment. 
As far as the PDA, he explained that the coil he used to close it looks great!  He said they worry about the coil protruding into the aorta or pulmonary artery, but hers is sitting perfectly in between the two!  Praise the Lord!!  He then gave us a run down of the aorta itself as well.  It has grown a little, which we were hoping would stop with the PDA now closed, but he said that may take some time to kick in and make a difference.  So, the slight growth did not concern him.  We'll check it again in 4 months.  For now, we can breath again and say many prayers of thanks!  God has been so good to us!  

Dr. G telling Amelia what a good job she did!

Thank you so much for all your thoughts and prayers for Amelia and our family!  It means so much to us!  She continues to amaze us!

"May the God of hope fill you with all joy and peace as you trust in Him, so that you may overflow with hope by the power of the Holy Spirit."  Romans 15:13

Patent Ductus Arteriosus (PDA): "...persistent opening between two major blood vessels leading from the heart. The opening, called the ductus arteriosus, is a normal part of a baby's circulatory system before birth that usually closes shortly after birth. If it remains open, however, it's called a patent ductus arteriosus."  Mayo Clinic definition

Aortic Dissection:  "An aortic dissection is a serious condition in which the inner layer of the aorta, the large blood vessel branching off the heart, tears. Blood surges through the tear, causing the inner and middle layers of the aorta to separate (dissect). If the blood-filled channel ruptures through the outside aortic wall, aortic dissection is often fatal."  Mayo Clinic definition

Loeys-Dietz syndrome (LDS): "...genetic disorder that affects the connective tissue in the body.  Individuals with LDS exhibit a variety of medical features in the cardiovascular, musculoskeletal, skin and gastrointestinal systems. Information about the natural history and management of individuals with LDS continues to evolve."