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."

Sunday, June 7, 2015

Determined to walk no matter how long it takes!

So often lately, I sit down to write an update and the words just aren't there.  I type then delete, type and delete, and so on.  And then I happened to go to a garage sale and someone recognized Amelia and told me they have been praying for her!  I can't tell you how that felt!  And since then, I have had a few other incidences similar to that one.  So cool!  But, they also had a lot of questions about how she was doing because they haven't seen any updates...and this was over a month's time!  Because it's long overdue, I decided to do a few separate posts focusing on one area at a time so it's not too overwhelming. 

We (Amelia's therapists and I) just decided last week that we are going to take some time off of therapy at DeVos.  Let me tell you...I was not jumping for joy as I thought I would. The weekly appointments were cumbersome at first, but before I knew it, it was just our routine...every Friday morning for almost 2 hours for 17 months.  Amelia was just 2 weeks old when she saw her therapist for the first time...  

One of our many firsts...therapy at DeVos
Last day of therapy...
On May 5, Amelia had an MRI of her knees at U of M and we recently met with her orthopedic surgeon to go over the results.  He broke the news to us that therapy alone will not help straighten Amelia's knees.  This was a hard one for me to take because of all the work we put into it at therapy, through Mary Free Bed and at home.  So, leaving therapy felt somewhat like something you work so hard for only to get knocked down at the end.  Amelia's doctor was never convinced that therapy alone would do the trick (although her therapist was very hopeful), but he thought it was possible with some casting.  The MRI showed that Amelia has congenital dislocation of the patellas (knee caps).  The knee caps are attached to the tendon of the quadriceps which contract to straighten the knee and another muscle is attached to the base of the knee cap...all of this formed way off to the side of her legs. Therefore, moving them, and all the mechanics that go with it, will require major surgery, especially since this is how it formed from the beginning (congenital).  In addition, her Loeys Dietz Syndrome and knee contractures further complicate things.  "I'll be honest, she's a pretty complex case," is what we heard.  So, with that being said, Amelia's doctor is consulting with different doctors around the country on the best way to approach this, one of them being whether or not to do casting before surgery.  On that note, we asked him if we could wait until after summer to start this process, because with casting we would have to worry about them getting wet, they'll be hot, she won't be able to be mobile, etc., etc.  He was fine with that because he said we really won't lose any ground by waiting just a few months.  Phew...  In the meantime, however, she just won't be walking.  It didn't really bother me too much before to watch her with other kids close to her age when they're walking and she's walking on her knees trying to keep up because I was holding onto hope that walking was in the near future, but since this news, it has been much harder.  I see a long road ahead.  And, I know that a lot of it is perspective and it could be much worse, but this is where I'm at right now.  It is so hard to see the desire and fight in her and know it's not something she will be able to accomplish on her own.  More and more she tries standing with knees bent.  It's my heart breaking for her....I am grieving what she will have to go through to get there. 

So, as I wept in the early morning hours for my daughter, I was drawn to Jeremiah 1:5 and I know God had to literally take me to it.  Jeremiah 1:5 says, "Before I formed you in the womb I knew you, before you were born I set you apart."  I had to be reminded - in fact, it had to stare me in the face - that there is nothing I or anyone could have done differently to create my beautiful Amelia.  She is exactly what God wants her to be and He has promised to follow through with His plans for her (Jeremiah 29:11).  I have to believe this for myself, my family and mostly for her.  God has showed me so many blessings I would have never known if it wasn't for her...He definitely set her apart.  Another beautiful thing about my Lord is that, in my sadness, He reminded me of this in a very vivid way.  On the last day of therapy, Sam had to come with me.  He was supposed to stay at a friend's, but it didn't work out and it was a blessing in disguise.  God played a scene in my mind from that day that I will never forget.  Amelia was in the gait trainer at therapy and Sam was beside her hunched down with his hands on his knees cheering her on in such a tender way with every step.  And let me tell you, she already adores her big brother, so the look on her face looking at him was also priceless.  I never knew I would have to depend on my children this young, but I needed my little boy that day.  I also realized that I would not have those moments without this trial.  There have been many struggles over the last 17 months, but the joys that have made life sweeter than I ever thought possible, have topped them...thank you, God.  

While we wait for news on what, when and where, please pray that Amelia's doctor will have clear direction on what would be the best option for her.  Pray for us as a family, that we continue to find joy in the struggles so that we can be a testament to God's amazing grace.  

"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

Thank you to everyone who has been so faithful in prayer even though I have been absent in my updates!  Over the next few weeks I will be adding an update on Amelia's eyes and heart.  It's looking like she will need surgery on her eyes this winter and we'll see her cardiologist the first week in July to have an echo and get a better idea on what he did and saw on her heart cath that was also done on May 5 at U of M.  

Monday, December 15, 2014

Getting back to the heart!

It's that time again...  On Wednesday, Amelia will be seeing her cardiologist and also having an echocardiogram to be sure her aorta has not grown.  Please pray that we will not see any growth, that Amelia will tolerate the test well and that God will give her doctor wisdom regarding the results.  I will be sure to update the blog after the appointment and what else has been going on these last couple months!  In the meantime, as always, thank you for your prayers! 

Our little blossom!

Wednesday, October 22, 2014

Our little go-getter!

9 months!
First of all, thank you to all our awesome prayer warriors on behalf of our Amazing Amelia!  We truly believe He hears each and every one of your prayers!  A few weeks ago, Amelia had an MRI/MRA to get a closer look at her aorta dilation and the rest of the heart and also to look at the arteries from head to pelvis to rule out any other aneurysms.  We had a rough start with some miscommunication over what was actually ordered and what needed to be done, but once the kinks got worked out, it went pretty well. Through it all, Amelia was a rockstar!  We were so proud of her!  She did have a reaction to the anesthesia with some nausea and vomiting, but they gave her some anti-nausea meds and she was good to go!  We were already seeing smiles before we even got in the car.  

We received a call just over a week later and they did not find any other aneurysms!  Praise the Lord!!  They did find that Amelia has tortuous vessels in her neck, however we were not surprised by this news since a lot of LDS (Loeys Dietz Syndrome) patients have this.  Basically, her vessels are twisty, where normally they're straight.  But, as far as we know it does not cause any issues, praying that's the case!  Her heart remains stable which is great news!  Although, her cardiologist did increase the dosage on her meds because in talking with the cardiologists that were in on the MRI, they agreed that even though the dilation in her aorta remains stable, she's still not in the clear.  Pray that the increase does not cause any side effects and continues to do it's job!  

Right now, we continue to watch Amelia be her amazing, very determined self.  She does not stop!  She has been crawling for well over a month (with and without braces) and is now trying to pull herself up on everything!  In fact, one of my favorite moments was when she crawled for her therapist for the first time...she was so excited, she started screaming!  Oh, how that made my heart leap for joy.  Amelia has some wonderful therapists that have a special bond with her; although, she's been seeing them every week since she was only 2 weeks old!  I would say that makes them a pretty special part of this journey.  Thank God for these blessings!  

"The Lord is my strength and my shield; my heart trusts in Him, and I am helped.  My heart leaps for joy and I will give thanks to him in song."  Psalm 28:7

As part of her therapy, they continue to work on getting her knees to straighten out.  She has certainly made progress, but they're still contracted (unable to straighten), with her right leg being a little tighter.  So, now they'll be adding some equipment/gear to help straighten them even more.  Last week she was fitted for a SPIO and knee immobilizers.  The SPIO works on her trunk and the knee immobilizers gently stretch her knees.  Her therapist is also working on getting her into a "stander."  That's basically what it is and does...helps her stand.  She has the SPIO (and knee immobilizers) on in these pictures.  

Amelia is almost 10 months now and has exceeded our expectations!  She doesn't even seem to be bothered by any of this and is just so happy!  It's been so fun to watch her grow!  We can't wait to see what else God has planned for her!  

"We will shout for joy when you are victorious 
and will lift up our banners in the name of our God."  Psalm 20:5

 THANK YOU again for all your prayers!!!  I really mean it when I say it means so much!  

Saturday, August 2, 2014

Loeys Dietz Syndrome

An official word in our vocabulary now and used quite a bit these days...Loeys Dietz Syndrome.  About a week ago, we met with Amelia's genetics doctor and counselor to go over the results of the genetic testing and they confirmed Loeys Dietz Syndrome (LDS).  We expected these results, but it was a reminder that this is a lifelong's not going to end here.  LDS is a rare disorder that affects the connective tissue and there is no cure.  Because we have connective tissue throughout our bodies, people with LDS can have many complications, among the most common being vascular, skeletal, allergy and gastrointestinal issues.  Because of this, Amelia will be tested in the near future for scoliosis and have an MRA from the head to pelvis to identify arterial aneurysms.  

This past week we saw Amelia's hand surgeon and eye doctor.  Her hand surgeon anticipates some surgery on her pinky when she's done growing.  As for the eyes, we will follow up with the eye surgeon in 3 months to probably start patching and discuss possible surgery to correct an issue with coordination of her "v pattern."  This means that when she looks up and/or to the side, she loses coordination.  Please pray that surgery is not necessary.  All surgeries make me nervous, but any surgery on the eyes makes me really nervous!
Her weekly treatment continues on her hands, knees and feet.  She still has to wear her braces around the clock, but I took them off so you could see how good her feet look!  She loves to play with her feet when they're off, which I love to see, but makes me sad she can't do that all the time.  Oh the things we take for granted.  But, she doesn't even seem to notice.  In fact, regardless if they're on or off, she's trying to crawl!   

Despite these complications, we are very optimistic and hopeful for the future!  Maybe it's the known vs. the unknown.  I remember when I was pregnant with Amelia and the worry over the unknown would at times consume me to the point I could hardly breathe.  Now I look at my beautiful baby girl who brings us so much joy, I know everything about her was meant to be and I am beyond thankful for our Amazing Amelia.  Now we can move forward and hopefully provide Amelia with the best care possible in light of this diagnosis.  

"As far as I am concerned, God turned into good what you meant for evil."  Genesis 50:20

As part of this journey, about a month ago, Nels, Amelia and I were able to attend a Loeys Dietz Syndrome Conference in Baltimore, Maryland.  What a blessing that was!  We learned more about this disorder, what to look for, and possibly expect in the future.  I already feel more confident going into appointments with Amelia, knowing the posibilties. 

A very special man, Dr. Dietz!
At the conference, we heard a panel of physicians speak that specialize in LDS, as it specifically relates to the vascular system, orthopedics, allergy and gastrointestinal concerns.  We were especially excited to hear THE Dr. Dietz and THE Dr. Loeys speak.  
Amelia was star struck too!  :) 
Dr. Loeys!  He held her for a long time...I don't think he wanted to let her go.

Most importantly, we made some wonderful connections with other parents that are on the same journey, fighting the same fight!


When we first found out about LDS, I of course had so many questions, but most importantly I was concerned about the treatment options (or lack of) that we were being given related to Amelia's serious heart condition.  Gretchen, the genetics counselor that works with Dr. Dietz at John Hopkins University, contacted me personally!  This was our first connection to Dr. Dietz and his expertise, so we were very excited about this.  I still remember that first conversation I had with her over the phone.  She gave us the information we needed to pass on to our cardiologist...and what good information that was!  It saved her life!  Needless to say, we are very thankful for Gretchen and her part in our journey! 

In a previous post, I had quoted Mo Jurgens.  Mo's mom, Kate, wrote a memoir about Mo's life and the many obstacles they faced as a family through a lot of unknowns.  I have been reading this book, as it's been a great resource to me not only as a mom, but as we take this journey with our own daughter.  Mo was diagnosed with Loeys Dietz Syndrome at age 9. She has had 27 surgeries in her young life.  She will be starting her sophomore year in college for nursing this fall.  What a great nurse she will be!  I was so excited to meet them both...I have to say, I was star struck!  :)  

So, not only did I get to meet both of them, but before I really had a chance to get to know her, Kate took Amelia from me during one of the sessions so that I could listen, so cool!  I could only smile and thank God for stringing together every little detail; oh how He knew I would need that connection as a mom.  Not to mention many other moms that were instant friends!  Amelia was the youngest at the conference, so these moms have been where we are at right now and were quick to make sure I knew they were there for me if I needed them.  Exactly what I needed! 

The weekend went by so fast...I feel like I'm still taking it in.  Then I realize, Amelia is only 7 months old and I think back to all we've been through in this short time; life and death were thrown at us within months of each other and here we are now, watching our baby girl roll around, smile, talk and giggle!  Sometimes I feel like I'm just watching from the outside.  I can only say I have so very much to be thankful for!  God is without a doubt using Amelia to teach me so much!  

"Don't worry about anything; instead, pray about everything!  Tell God what you need, and thank him for all He has done.  If you do this, you will experience God's Peace, which is far more wonderful than the human mind can understand.  His Peace will guard your hearts and minds as you live in Christ Jesus."  Phillipians 4:6-7

If you would like to learn more about LDS, please visit In fact, as you pray for Amelia and our family, pray for the foundation, as well, as they continue to give so much of themselves in educating others about LDS, fostering research and supporting those affected by it.  I was reminded again this week of how rare this is.  Out of the many doctors and medical staff that have been involved in Amelia's care, her cardiologist has been the only one that knew anything about it.  THANK YOU again and again for your prayers and support!