Noah's Thoughts

Hi everyone this is Noah. I wanted to tell you all that my heart is doing fine. I'm happy that I haven't gotten worse I took this weird test to see how I would do in high altitudes because I want to go to camp and Big Bear. I'm able to go to Hume Lake this year which I'm super excited about. We are also planning some other fun activities since I got the ok to travel. Sadly there are still lots of doctors appointments and medications I have to take. Good thing I take pills now and not the yucky medicine. I still wish I could play soccer and hockey. I'm glad though that  I'm doing well and not in the hospital like some of my friends that I have made this year had to.  I'm a little worried about the actual surgery of the transplant but I usually try not to worry about it too much.  I have really been enjoying 412 my Church's youth group.  It is a lot of fun.  School has been a lot harder this year and there are many tests but I have been doing well.  Thank you so much for continuing to pray for me and my family.  I know God is working and has everything figured out.

Thankful

Thanksgiving is fast approaching and we have so much to be thankful for:

 Our sweet boy has made it almost three years since his first diagnosis in January 2012.  He has been on the transplant list for over two years now and has remained hospital free.  I can't tell you what joy it brings me to see some of you still faithfully wearing your bracelets.  Eric Twisselmann I was brought to tears the other day when I saw you on stage sporting your faded bracelet. I love how worn it was.  It was so faded the words were washed away but I could still tell what it represented and was so beyond grateful to see the support. I know so many of you are still so faithfully praying and we can't thank you enough.  We are so thankful Noah has remained well.  His pressures are stable for now but we don't know for how long so we celebrate today and the last few years knowing things could dramatically change tomorrow.  Really it's how we all should be living, thankful for today not knowing what tomorrow may bring.  Can I get an Amen?  Our sweet boy still needs a new heart but only God knows the timing.  He remains on the transplant list and we remain hopeful that it's in God's control and not our own.

I'm also thankful for the gift of driving and for the muscles in my body that still work.
As this muscle disease continues to progress it brings me more and more thankful for an amazing husband who carries his bride and lessens her load, supportive friends, family, adaptive equipment, and God's grace.

I'm thankful for our sweet girls successful surgery.  She is doing amazingly well and there have been no  surprises.  She loves that her momma has a pacer just like her.  She has regular check ups and positive reports.  So thankful.

Last we are thankful for all of you that would care enough to read our words and receive our updates.  It means so much to us.






Give thanks in all circumstances; for this is the will of God in Christ Jesus for you. (1 Thessalonians 5:18)

Bittersweet

Yesterday our sweet girl turned six.  It was a birthday to remember.  She has been in love with horses for a while now.  She was super sad when Jason, the boys, and I went horseback riding a few months ago.  She did not meet the age requirements and was unable to join.  She wanted a horsey birthday so mom began the hunt  to find someone who had a pony or a horse.  A sweet friend I grew up with had a connection to a ranch close by.  The people had never done a birthday party before.  After hearing about our family from our friend they were willing to host a party for us at their ranch.  This ranch holds several horses including ponies, a pig, chickens, and several peacocks.  It was the perfect place.  The hosts went above and beyond and truly blessed our sweet girl.  It was such a joy seeing her ride a horse for the first time. Hannah will always remember this special birthday.

At the end of the day looking back through all the pictures my heart began to break for Hannah's birth mom.  I think and pray for her often when she comes to mind.  She always comes to mind on this day in particular. We are so blessed to have Hannah's birthdate.  Since children are abandoned they don't often know a birthdate and are given one based on weight and other criteria.  Her birth mom felt like her daughters birthdate was significant or she wouldn't have taken the time to pin it to her clothing.  She cared enough to let me know and I will forever be grateful for that and so many other things.  I can not imagine the loss she must feel not knowing if her sweet daughter is alive and well. Oh how I wish she knew what an incredible gift she has given us and how brave we think she is for leaving her in an elevator of a hospital in hopes of her getting the care she needed.  I wish she knew how healthy and beautiful she is and how much we adore her.

 Lord please wrap your arms around Hannah's birth mom and give her the love and comfort that only you can give.  Lord I pray that if she doesn't know you already Lord I pray that you would make yourself known to her.  Lord thank you for choosing us to be Hannah's parents. Lord I pray that she would grow up with a desire to love and serve you all her days.  I pray that she would put you first above all things.  Grow in her a compassion for the fatherless, give her a gift of humility, patience, and self worth.  Thank you for saving her life and for continuing to heal her body.  Thank you for the fighter that she is and that you made her perfect created in your image.

It's not about the why it's about the who

The last two weeks have been rough.  I have been to at least eight doctor appointments. Six of which have been for me.  I have been told after allergy testing that I'm allergic to almost everything except foods(Thank you Jesus),I had a CT scan of my sinuses, told I need tubes put in my ears, after a PFT test was told my breathing is declining, and was told I can no longer drive with out adaptive equipment(that one was the hardest). There were days where I felt like Alexander in the book "Alexander and the terrible no good very bad day". Great book by the way.

Normally I can tackle each hurdle as they come knowing God is working out his plan through each obstacle I face.  This last week however I finally broke and was crying out to God Why?  Why so much?  Why my driving? Why does it all have to be so hard?  Many of these things I knew I would have to face with this disease I just thought it would be further down the road for me.  I knew all the answers to the Why in my head but my heart was buried in my circumstances and I was feeling very defeated. After a very good and much needed meeting with a friend I was super encouraged by an article he read to me.  Many of you know about Pastor Saeed Abedini.  He is an Iranian American Christian pastor imprisoned in Iran.  He has been incarcerated now for over two years.  He wrote a letter to his daughter for her 8th Birthday.  In the letter he talks about how he recognizes that this must be so hard and confusing for her.  He goes on to say that she is probably asking Why? Why is daddy in prison? Why are they doing this to him?  He says to answer your question you must first understand that it's not about the Why it's about the Who.

This pierced my heart and reminded me once again that it's not about me.  It's always all about Jesus.  Not about the why but about the WHO.  JESUS. Who am I to ask Why? Who am I to question his plan for my life? Jesus took on human flesh, went through more than anyone could bare, and was hung and crucified on a cross for my behalf. Yes I cry and I groan but then I'm reminded of how much I don't deserve but have been given much.  It's not about the why it's about the who. Powerful words.

Thankful for friends who remind me of God's truth.  The only way to get through these hard moments is to preach the Gospel to ourselves and to meditate on his truth daily.  For some of us hourly :) Thankful that he is working out his great plan and that he is sovereign over my life.  I pray that when hard days come again I will remember these truths, put on my battle gear, and fight the good fight.

Meeting Update

We are thankful for all your prayers.  We got word today from the transplant team at UCLA.  After much consideration they have decided that Dr. Changs opinions were very valid and raised great concerns.  They are sorry for not consulting him before they met with us but feel it is best that Noah remain on the list.  Our emotions are all over the place.  At the end of the day though it comes down to this.  God is sovereign.  He is the only one in control of what happens with Noah.  He already knows the timing, the donor, and if and when Noah will make it to transplant.  It really doesn't matter the decisions these doctors are making.  I have been resting in that a lot this week as we have anticipated the doctors decision.  Even if he was to have been taken off the list for awhile that list doesn't determine what and when for Noah.  God does.  This is what calms my emotions and helps me to breathe. I have absolutely NO CONTROL.  NONE!!!  I don't have the right answers in this situation.  I do however have a wonderful Father who even before Noah was formed he knew his story.  I will rest in that.  I will cling to that assurance.

Leaning On The Promises Of God

We are constantly amazed at how rapidly things change with our situation.  Last week we were thrilled to hear how well Noah's pressures were in his heart after his heart cath.  They were contemplating taking him off the transplant list for awhile.  We drove down to UCLA and had a wonderful meeting with the transplant team who answered all of our questions.  We walked away feeling at peace with the decision.  Over the weekend we started making vacation plans and getting excited about our family being able to travel and not worrying about "getting the call". Still I found myself not being able to fully  be excited.  I was waiting for the next shoe to drop, the next storm.  It seems over the last three years nothing has been calm for us and I was holding onto this new news very loosely.  I knew that at any moment things could drastically turn for Noah and I think it is just a way to safe guard my heart in this process.  Still as much as you safe guard yourself or prepare it still is hard when the storm comes.

Tuesday we had more sets of doctor appointments. This time Hannah and Noah were both seeing Dr. Chang for heart Echo's and check ups.  At this appointment the storm hit again.  The team at UCLA had not talked with Dr. Chang yet about their decision to take him off the list for awhile.  Dr. Chang was very upset and did not agree with this decision.  He has very strong reasons and after the team heard his thoughts they are taking them into consideration.  On Tuesday a team of several pediatric cardiologists, surgeons, and the transplant team will meet for a round table discussion to discuss Noah's case.  Right now Noah has not been taken off the list but is on an internal hold until this matter gets decided.  I can't even tell you what an emotional battle this has been this week.  On top of all that , Wednesday Noah had a bad episode.  He yelled for me from the other room.  When I got to him he was clenching his chest telling me it really hurts.  It lasted about a minute and he had many little ones that followed.  He started to cry and said he just wants it over with.  It breaks my heart to see him in discomfort.  He is fine and the pains are one of the things Dr. Chang is concerned about because he has had them consistently for the last two years with no improvement and somedays they are worse.

Please pray for this decision.  Pray for our nerves.  Pray that Noah would be ok with the outcome.  It is such a roller coaster ride and we would love to have some calm back. Pray that we would continually be leaning on the promises of God.  Grateful for you all.

Blessings Abound

Well Noah is Officially a status 7 on the list.  This just means he is in-active for now.  Since the news on Monday that this could be a possibility I have had a mixed bag of emotions.  Happy, worried, confused, anxious etc.  I had a ton of questions and not a lot of answers.  When UCLA called on Wednesday and asked if we could meet with the transplant team on Thursday I felt relief.  I was so happy that I would not have to wait a long time to get the answers.  I needed to feel good about this decision and was praying I would feel at peace once we got our questions answered.

We had a wonderful visit with the team.  Dr. Alejos who is Noah's transplant cardiologist is the nicest man.  He is so great at explaining hard things, and not making you feel like your questions are silly.  Believe me we have had plenty of doctors who are not great at these two things and it can be very frustrating.  I love the way he interacts with noah and encourages him to ask questions as well.  Dr. Alejos explained to us that they have seen great improvement in Noah's heart pressures over the last two years.  The medications have been very effective and Noah's pressures are in the normal range.  At this point if he accepted a new heart it would not be any more beneficial to him.  Noah still has Restrictive cardiomyopathy. He will eventually need a new heart.  His heart still does not relax properly and this will eventually lead to heart failure and stroke.  The benefit to taking him off the list right now is to buy this sweet boy some more time.  Transplant is great because it will save his life however only for a short time and transplant comes with lots of dangers and complications.  The longer he can wait the better it will be for him.  We do not know however how much time this will buy him.  He could start to show signs of heart failure tomorrow and need to be re listed or it could be another year or so. He will be closely monitored by the transplant team.

We feel very much at peace about this decision. For right now we are celebrating this unexpected blessing and will continue to give our son up to the Lord trusting in his perfect plan for Noah.

Thank you for your continued prayers and support.

Praise God From Whom All Blessings Flow

Yesterday was Noah's heart cath. We got up early and arrived at UCLA at 7:30 ready to check in.  Noah's procedure was delayed by two hours but we kept busy by playing games on the ipad.  Noah was nervous and asked us to pray for him.  Truthfully he wasn't the only one nervous.  We were scared for what the doctors might find when they went in to take a closer look at his heart.  It had been 15 months since his last heart cath procedure.  Finally they were ready to take Noah back.  We gave him a kiss and told him we would be in recovery when he woke up.  Jason and I went to the cafeteria to get some lunch.  We talked about the number of times Jason has had to say goodbye to one of us as they wheeled us off into surgery.  He remarked that it never gets easier and is not looking forward to the day Noah gets his new heart.  I was super tired from lack of sleep the night before.  My body had come down with Vertigo and the drugs that I was taking to help keep the room from spinning were making me loopy.  I wanted nothing more than to find an empty hospital bed and take a long nap. Yep you read that right Vertigo.  I laughed when the day before the nurse told me it could last for several months.  I looked up at the ceiling and said "really Lord?" "Throw me a bone or take me home".  My bone would soon come.  After we ate lunch jason and I went to wait in the waiting room.  The nurse came by and asked if our last name was Lietzau.  She handed me a phone and told me the doctor would like to speak with us.  Let me just stop and say it is torture when this happens because you have no idea what your about to hear.  They have your precious child in an operating room and you are not thinking roses and rainbows you are thinking the worst has happened.  The doctor started with your son is fine and we are all finished.  Then says you know what let me come down and talk to you.  I have no idea what he is going to explain to us but at this point I'm thinking a hospital stay is in our future. Here is the following conversation:

Hi Dr. Alejos.  He stands there with a smile and explains to Jason and I that Noah's pressures looked so good.  He said it's pretty unbelievable but they are in the normal range.  What?? He says, we thought his numbers looked well last year only increasing by one but they are actually better than when he was first listed.  This does not mean that he is cured but they think he could wait a year or two for transplant.  He goes on to say the medications he is on are obviously doing there job and we will just monitor him closely every two months.  WOW!!!  What?? What does this mean???  Well it means that we can't explain his nasty abnormal EKG, and he still has restrictive cardiomyopathy. but the longer he can wait to have a transplant the better for him.   They still need to go over all the results with the transplant team before they make him inactive on the list.  If they do decide to take him off the list he wont lose any of  the last two years of time he has occurred.  When he gets put back on he will still have all that time.  If he got a call tonight though Dr. Alejos said  I would for sure turn it down.  WOW!! This means we will be able to travel and not be stuck close to the hospital.  I asked him about Hume Lake.  Noah has been sad to miss the last few years. He said he had a test they could do to check how his heart handled the elevation.  If he handles it ok than yes he can.  WAHOO!!  He told us he would be in touch and walked away.

We both stood there stunned.  There was my bone.  A mighty big one too I might add. Unbelievable.  We are so happy but know at the same time how drastic things could change in a second. So we want to hold this loosely.   For now we are taking it and are so relieved in so many ways.  Thank you Jesus for this gift. We will keep you all informed as we learn more about what the transplant team has decided and what this will mean for Noah.  Thank you once again for being faithful to pray for us in this long journey.

Ps. Please look pass all my ugly grammar mistakes.  I'm not good with writing to begin with and having vertigo makes it even more challenging :)

It is well with my soul

Wow!! Time has gotten away from me and I can't believe it has been so long since my last update. We started school and it has taken some time to adjust to our new routine.  We are enjoying some much needed structure and it has gotten our minds off of all the medical jumbo looming over our heads.

  It's been so good for  me to look back at these posts and realize the ups and downs of emotions we have experienced as we have walked this long journey. My last post I was in a weary place and now I'm sitting here feeling relief that God doesn't keep me in that place for long.  Jason and I are coming off of a wonderful week.  We just enjoyed two nights away together just the two of us.  It was a great time of reflection and relaxation.  We were surrounded by beauty and I was able to just sit and recount God's goodness through our journey over the last few years.  It was good for our souls.  Another wonderful thing happened.  The day after we returned our sweet Jonah made the decision to follow and trust Christ.  The Lord has been working in his heart a lot over the last year. He has been asking lots of good questions and has had some break downs as the holy spirit tugs at his heart. Yesterday while I was driving his little sister to class, Jonah began to cry and told me he was ready to ask Jesus into his heart.  I was caught off guard and texted Jason.  Jason told me to move forward as he was convinced the holy spirit was moving in him.  We talked through his understanding of this decision and I got to witness yet another answer to prayer. My boy made the biggest decision of his life. The rest of the day's tasks seemed so insignificant and I found it hard to want to do anything else but sit with my kids and relish in God's goodness.  We are all rejoicing.  I love my boy but will never love him more than his savior and today he got that.  He understands that he is a sinner in need of a savior.  His heart is tender and the weeping comes from understanding the ultimate sacrifice his savior made for him. Glorious Day!!

Yes there are things that still loom over our heads.  There is much pain and uncertainty that is waiting for us with transplant, heart disease, and muscular dystrophy.  However I find great relief knowing that my boys( and one day God willing my daughter) understand that we have a hope that is far greater than anything.  They understand that this is not their permanent home.  One day Jesus will wipe away every tear and right every wrong and we will get the immense joy of spending eternity with our father in heaven.

Sweet friends we are thankful for all of you and ask that you would continue to journey with us in prayer.

Noah- Noah has been doing well but is growing inpatient as the days go on.  He is eager to get his new heart and feel better.  He looks forward to playing soccer and going to Hume again someday.  He has his annual heart biopsy Cath procedure this Monday at UCLA. Please pray for good results and no surprises. No sickness.

Jonah- We are thankful for Jonah's clear results from his heart monitor.  Jonah is doing great and we are proud of his recent decision to follow Christ.  Pray that the Lord would continue to work and use Jonah through our circumstances. Pray that God would continue to keep him healthy and strong.

Hannah- Hannah continues to do great since her surgery.  We pray that she would continue to feel great and for no surprises with her heart.

Jason- Pray that God would continue to help him grow in trust and patience.  As he cares so deeply for his family I pray that God would meet him and shower him with his love and grace.

Melody- Pray that I would not be defeated by my circumstances.  Pray for strength and energy.  Pray that I would be able to avoid respiratory illnesses this season.

Oh So Weary

I wanted to give you a brief update on our family and ask that you would continue to keep us all in your prayers.  This has been a looooooong journey and we want to thank those of you that are still checking in and lifting us up in prayer.


Noah-  Monday I dropped Noah off at golf camp and explained to his instructor all about Noah's condition.  A few hours later I get a call that Noah is not doing well.  Noah was dizzy, his breathing was labored, he had rapid heart beats, and was finding it difficult to keep up with the walking on the coarse.  I was super nervous when I got to the coarse. Noah was emotional and crying that he just wanted his new heart.  It was very hot and humid and that alone can do a number on cardiomyopathy patients.  Noah has learned over the last two years to self regulate.  This means he can run around but the minute he feels anything he stops.  This was a situation that he could not do that and unfortunately the instructor was not listening to Noah.  He knew his body needed to stop but the instructor was not letting him.  He will be seen at UCLA tomorrow for a full work up and we would appreciate your prayers.  As Noah is getting more and more fatigued and not feeling great he is also getting inpatient.  He has broken down a few times and wants more than anything to feel better.  It makes me anxious and it breaks this mommas heart.


Jonah-  Because of our family history Jonah sees the cardiologist once a year for a work up.  Since we found out a few months ago that Jonah tested negative for the rare disorder Noah and I share, I was expecting todays appointment to be uneventful.  Well I should have known that is never the case with our family.  Jonah's EKG came back showing some slow abnormal rythmns.  He left the office wearing a 24 hour holter monitor.  Hopefully this is no big deal as this momma is reaching her limit on how much more she can take.

Hannah-  Praise the Lord this sweet girl is doing amazing.  Her oxygen levels were 98 today.  she is not getting out of breath and has more energy than I can handle.  The doctors couldn't be more pleased at this point.

Jason-   My sweet husband carries the weight of this family on his shoulders and it is a heavy load.  Please pray that God would give him the strength he needs to endure each day.

Mel-  I recently went to UCLA for a swallow test.  We found out that my tounge and swallowing muscles have been affected by this wonderful MD disease.  It is mild now and nothing I can do except eat small, slow, and often.  I will be having pulmonary testing next to check my breathing.  The danger with this disease for me is that eventually it could effect my respiratory muscles.  Other than that I have been focusing on trying to do things I have always wanted to do before this disease progresses any further.  We recently had fun renting segways, and I even tried horseback riding for the first and last time.  Hey some things just aren't for everyone :)

We trust in God's perfect plan but friends there are days when we are weary. Days when the journey seems long and we don't have much steam to press on.  Pray that we would be obedient to press on in faith, that we would be consumed by his love, and that we would experience his peace that transcends all understanding.

Angel Game #3

Thank you so much for those that came out to support us at the Noah's Heart Angel game.  We had a great time.  It was a fun night but I  was not prepared for the flood of emotions that came over me.  It started when Uncle Josh told Noah he was going to get to put the game ball on the pitchers mound.  Noah was excited and super thankful.  He hugged his uncle and said thank you three or four times.  I held back the tears as I watched him. It was difficult being faced with my physical disabilities and realizing the progression from the last two games.  Next was seeing Noah have his moment on the field and realizing that on the same field last year our close friend Geoff shared in a special moment with us between his cancer treatments.  Geoff was the healthiest he had looked in months and so happy having a special date with his bride.  He died months later and I miss him dearly.  The next moment came when we surprised Noah with a special guest.  His co-op teacher of three years Mrs. Barneson came.  When he saw her his eyes lit up.  He hugged her tight for what seemed like 20 minutes and began to cry.  She holds such a special place in his heart and he was overcome to see her.  Also realizing we have done the Angel game for three summers now and we still haven't gotten the call was hard.  As much as we try to be patient it is hard to wait for something we know has to happen in order for our son to live.  It was so great seeing Noah have a blast with his buddies, the Angels won, and the fireworks were awesome.  A great night to remember for sure.  Thank you so much Josh and Jamie for giving Noah an incredible three games that he will remember for a lifetime.

Grateful

After only six days in the hospital, Hannah got to come home yesterday. It felt so good to have our family back together and in our own beds.  It has been amazing to see how fast this sweet girl has recovered. She is doing amazing.  She continued to blow away the doctors with her progress each and every day.  Hannah went in Tuesday morning for open heart surgery and the placement of a permanent pacemaker.  She now has a conduit shunt helping to supply blood and oxygen to her heart.  Her oxygen stats went from being in the low 80's to now 98-100.  We have noticed a significant increase in her color and look forward to seeing her run around without getting winded.

It has been an emotional six days for us.  We spent time with nurses and doctors who were all apart of saving our girls life four years ago.  Her hospital room was directly across from the room where we heard doctors tell us she wasn't going to make it.  The same room where they worked on our sweet girl for three hours, pumping her full of meds to keep her stable.  We spent time with another family from our church who had been at CHOC for days with there daughter who underwent brain surgery.  We had an interaction with a family I had met by chance weeks ago at Dr. Changs office.  She was waiting for a heart/lung transplant for her one month old.  Her daughter had gotten influenza and was in the CVICU.  Our hearts were burdened for this family who felt hopeless.  We prayed for another chance to talk with them and were disappointed when they were discharged the next day.  Happy she was well enough to go home though for sure.  You see so much pain and despair when you are in the CVICU.  It made us that much more thankful and aware of our need for a Savior.  I don't know how people walk this life without Jesus.  I chuckle as I look back and see how much better it is to have Hannah and my surgery behind us. It wasn't how I saw things going but once again God's plan is ALWAYS the best plan.  I feel hopeful as we anticipate Noah's transplant that we will someday settle into a new normal.

A few days before Hannah's surgery I received a call from a sweet girl Samantha.  I did not know her but she explained that she worked for Noah's orthodontist and had recently heard of our family's story.  The Lord had put our family on her heart and she felt extremely burdened to do something.  She asked if she could meet with me to hear more about our story.  I told her Hannah was getting ready to have surgery so we didn't have time before.  I told her she was welcome to stop by the hospital since we had anticipated a long 14 day stay.  Samantha had decided to come by Monday.  We were surprised to hear we were going home that Monday so Samantha met us at our house instead.  This sweet girl brought us dinner and a beautiful gift basket.  She sat and listened to us tell her all that God had done over the last five years.  There were tears and smiles as we all recounted the beautiful ways God was at work.  we got to hear Samantha's story and her struggles and it became apparent that God was using her in big ways to impact his people.  We are so thankful for a new friend and the way that God encouraged her to reach out to us.  Don't you just love the way God works.  I don't make this stuff up people he is constantly showing himself in beautiful ways.

 Hannah is still recovering and we have a ton of appointments over the next coming weeks.  Please continue to pray for her. that she would remain stable and continue to heal.  Thank you all for your outpouring of love to our family this past week.  We are thankful and have been so encouraged by it.

Hannah's Surgery

Hannah’s Surgery

Today we met with Hannah’s surgeon Dr. Gates.  I have to admit it was a little overwhelming to sit across the room from the man who played a big part in helping save our little girls life four years ago.  We discussed the process of the Fontan operation Hannah needs to have and what it would entail.  He mentioned that it should be done this summer and we told him we would like to schedule it as soon as possible.  We explained that Noah was expecting a call for a heart and they could not take him off the list for this surgery. He looked at his books and said he could do it this Tuesday.  So Tuesday it is.  This will be an open heart surgery where they will go in and put in a conduit shunt. We are feeling many emotions right now.  We have dreaded this surgery for the last four years but also know how necessary it is. We are eager to get it behind us.  We are fearful of getting a call at the same time and having to split ourselves up to care for two of our children across town from one another.  We are scared of the actual surgery, we are concerned how Hannah and the rest of our kids will process this all.  Concerned how I will keep up physically and so much more.  We do know and are confident though that God has been in control and has known the timing of this all.  He will give us the strength we need to face whatever comes our way.  So thankful we do not have to walk this alone. The song thats ringing through my mind tonight is   “His power Will Prevail.” The lyrics are below.  Please continue to lift our family up in prayer.  We would love more than anything to be a light at CHOC where so many people are experiencing darkness.  Thankful for you all.

Rest assured today that the Lord your God 

    is the One who goes ahead of you

He will put the enemy to flight; 

    what He’s promised He will do

Like a raging fire, He consumes the foe; 

    making Satan and his forces go

We don’t have to fear, God is on the throne, 

    His power will prevail

God’s deliverance is as good as done; 

    who could ever count the victories won?

With a watchful eye He protects His own; 

    He’s the Shepherd, we’re His sheep

When we go to Him with an urgent need 

    He is always there to intercede

We don’t have to fear, God is on the throne, 

    His power will prevail

Each temptation here, every trial we face 

is surrounded by His awesome grace 

Supernaturally strength is given us 

    so that we might persevere 

God is never late, help is on the way; 

    He is faithful in the day-to-day

We don’t have to fear, God is on the throne, 

    His power will prevail

Summer Fun

Summer has started and I officially have a Middle Schooler, a Third Grader, and a Kindergartner.  It is so hard to see my blessings grow older.  Every Summer we create a  list of fun things to do.  Since we will have a surgery (possibly even two) this summer the list is short.  We are determined even in the midst of a hard season to have some fun.  We will miss our favorite family vacation up at Hume for the third year in a row.  It breaks our hearts but we know one day it will happen again and are thankful for great friends who help us pull off Camp Lietzau as an alternative. We are thankful to live in a place that offers so many fun things to do.  Heres what we have planned. We look forward to hearing how you all spent your summer. Thanks again for following us on this journey.

Smoothie Making Contest

Video Scavenger Hunt Competition

Angels Baseball Game- come join us

Smores

Backyard Movie Night

 Shaved Ice

Beach Bonfire

Splash Water Park

Kids Make Dinner

Camp Lietzau

Individual dates

Fishing

Vacation Bible School  

Swimming With Friends

Homemade Ice Cream

Game Night

Reading Program

Movie Theatre

Baseball Camp

Lego Engineer Camp

Heart Cath Procedure on Monday

Some days are just so incredible that you never want the day to end.  Then there are some days that are so incredibly hard you think to yourself how did I make it through the day.  Well Friday was one of those difficult days.  Two doctors appointments, several errands, and our youngest blessings recital.  Two extra kids added to the mix that were suppose to spend the night but ended up going back home late that night because our youngest boy started throwing up out of the blue.  We think his body did not like the goodies and jumping on the trampoline.  It was a non stop, just take a breath, and press on kind of a day.  I'm thankful that I can look back and laugh at how crazy it was.  I'm thankful that God does not allow me to be defeated by these hard days or moments.  Instead he reminds me that he is carrying me through and has a plan in all this.


Friday we met with Hannah's cardiologist Dr. Chang.  We were there to discuss her upcoming heart surgery. It is now time to schedule it and there are so many emotions and concerns that are flooding our minds.  Tomorrow she will undergo a heart cath to take a closer look at her heart and check the pressures.  They will put her under anesthesia and either go though an artery in her neck or groin.  It's never easy when your child needs to be put out,  and it's even harder when your child has a heart condition.  Based on what they see with Hannah's heart will determine how soon they will schedule surgery.  We are thinking July.

Please pray for Hannah tomorrow.  Pray that God would protect her, and that the doctors would not see anything alarming or that surprises them.  Pray for Jason and I that we would not be consumed with fear but that we would have faith resting assured that God is working all things for good.

A More Detailed Update

Thank you so much Marla for giving everyone an update.  It has been a a lot for us all to process and we are still taking it all in.  Months ago Jason, myself, and Noah had a full genome sequence panel done.  We were hoping they would find a genetic link between Noah and I.  After waiting for over three years I was relieved to finally have some answers.  Noah and I both have a very rare genetic disorder called Desmin Myofibrillar Myopathy.  It's so rare that there is not a lot of information on the disease.  They have only recently discovered the disease within the last 6-8 years.  Our next step is to meet with genetics to go through counseling, gather information, and get Jonah tested.  This disease is very familial so the chances of Jonah having this disease are great.  If he tests positive we do not know how and when the disease will present itself.  I was 19 and Noah was 9.  I'm feeling thankful that God protected my life and I was able to get my pacemaker before this disease was even named.  We are also very thankful that we have caught Noah's cardiomyopathy early and that God's hand of protection has been on him. These are the biggest threats with this disease.  The other major concern is Respiratory failure.  The respiratory muscles become weakened because of the skeletal myopathy leading to possible death.

All of this is not what we had envisioned for our life and there is a lot of grieving however we still have hope. With Jesus Christ there is always hope  We can trust in him alone and trust that he is working all things for good.  We know and our confident that this is all part of his wonderful plan.  I know that he purposes these things for our good. I know that all these trials and all this suffering is so that the works of God can be displayed.  I believe these truths and hold onto them every day.  It doesn't mean I have to like this though, it doesn't mean that it's not painful and that I can't cry out to the Lord to take it away.

Romans 8: 22 says

 22 For we know that the whole creation has been groaning together in the pains of childbirth until now. 23 And not only the creation, but we ourselves, who have the firstfruits of the Spirit, groan inwardly as we wait eagerly for adoption as sons, the redemption of our bodies. 24 For in this hope we were saved. Now hope that is seen is not hope. For who hopes for what he sees? 25 But if we hope for what we do not see, we wait for it with patience.

Please pray that we would focus our minds on God's truths and his goodness and not be consumed by fear.  We desire to live each day with hope and joy.

Noah's appointment went well.  They can never really give us time limits for a heart for him.  They can only go by where he is on the Unos list, trends, and how often they have seen his name come up.  They did remove some avoiders. We did not ask what they meant by that.  We assume they mean peramiters they set at the beginning when they felt like we had more time.  They feel by doing this that it will help speed things up for him.  They also suggested we schedule Hannah's surgery right away since it needs to be done this summer.  They do not want to take Noah off the list for this which causes me great anxiety but again God will supply what we need and already knows the timing.  All I can do is trust.

It was a lot to take in so we appreciate your prayers as we make appointments and trust God with all the timing.  We are so thankful for the ways you all come along side us.  We are thankful to not have to walk this road  alone.  We feel very supported and truly thankful for you.

Noah's Appointment and News

Thank you to those of you who prayed for Noah's appointment at UCLA today. It turned out to be a significant appointment. After months of genetic testing, the doctors were finally able to give a diagnosis for both Noah and Mel. They both have what is called Desmin Myofibrillar Myopathy. They are excited to finally have an answer - especially this one which they have been waiting for for so long! They also found out that Noah's heart team thinks he will be getting his new heart soon, so they recommended that Hannah's surgery be scheduled ASAP.

There will need to be many appointments in the coming days and weeks - for Hannah to decide a surgery time, for Noah's next heart cath, and with the geneticist. Mel will give a more in depth update sometime soon. Please continue to keep them in your prayers during this time!

Surgery This Summer!!!

I apologize for the lack of updates.  I feel like at times I have been running a marathon and my body and emotions just can't keep up.  I want to sit and write and I emotionally can't put my thoughts into words.

I'm going to start with giving some details about Hannah and how she is doing.  We have noticed in the last month that she has been getting more and more out of breath with activity.  Her oxygen levels have began to drop slightly as well.  She has gone from the mid to high 80's down to the low 80's and sometimes below.  The doctor has been putting off her next open heart surgery for awhile as we have waited for Noah's transplant.  Today after her ultrasound he noticed some funky missed beats.  He decided to put a 24 hour holter monitor on her.  The holter will record the rythm and activity and allow the doctor to see if this is happening frequently.  He doesn't want to wait any longer for her surgery and is advising us to schedule it this summer.  I asked if we could wait to schedule it until after we talk with the transplant team on May 22 to hear there concerns about the timing.  We most likely would have to take Noah off the list for a week or two.  We will see Dr. Chang next month and will schedule the surgery at that time.  We would appreciate your prayers as we help our daughter prepare for this scary surgery.  Pray for both Jason and I as we know this is a necessary surgery but have so many fears as we walk this road again with her.  It's hard to believe it has been over four years with our sweet girl.  She recently got glasses and looks so grown up to me.  She is starting to read words and has a love for learning.  She is super brave and is a fighter and we have no doubt will do big things with her life.

Noah continues to do well.  we really haven't seen much change with his condition.  We take him on May 22 to see the transplant team.  Since it has been a year since his last heart biopsy we will schedule another one in June.  We are praying for God's perfect timing for the perfect heart.  Since it has been almost 21 months we really don't stress about getting the call.  In fact I don't even have a bag ready any more and rarely think to myself today could be the day. I should probably be more prepared but am thankful at the growth God has made in removing the fear and anticipation.  God will work out all those details when the timing is right. Noah has also developed a ganglion Cyst on each of his feet.  He had one drained a week ago.  They will wait to see if it goes down.  If it does not go down they will need to do an MRI.  The other cyst will be drained at the end of the month.  From what I have researched these ganglion cysts are sometimes common with heart patients.  They do not hurt him but could be harmful if they are infected.  It makes me sad that he has to deal with another thing but he handles it like a champ and it honestly bothers me more than him.


Jonah-  Jonah has enjoyed playing baseball.  He has been lucky enough to have the same outstanding coach the last three seasons.  It has been hard to keep up with his baseball schedule but we know how important it is for Jonah to play.  He deserves some attention on him and to do something he really enjoys.  Jonah will go see Dr. Chang on June 24 to have an echo and EKG.  So far Jonah has been ok but there is still the possibility that he could develop cardiomyopathy at some point.

We are winding down the last weeks of school.  I'm honestly amazed as I look back at the year we have had that we made it through.  We got our testing results back this week and I'm so proud of my kids.  They are all doing amazing in school and blow me away with what they are capable of.  God is good and I'm so thankful for the time I get to spend with them at home.  Hard to believe I will be a mom of a middle schooler, third grader, and kindergartner.  How does that happen? :)

Melody-  It was a hard month as I spent three long weeks without my much needed braces.  I had been told for almost two years that I needed feet braces(AFO'S) I fought against them because I was stubborn and not ready to admit I needed them.  I wanted to do as much on my own as I could.  After many hard falls it became necessary.  the first pair I got caused me immense pain.  I thought this was normal so i endured the pain for awhile.  After a few visits to the orthotics place, I was told I should not have any pain and that I should see a foot doctor.  I found a wonderful foot doctor from the help of a friend.  They casted my feet and fitted me for a different type of AFO.  While I waited for them to be custom made it was a dark time for me.  I had to be faced with the reality that I no longer had the option of not wearing the AFO's.  I relied on people to help me walk and used my wheel chair a lot.  We realized just how much this disease had progressed for me and it was very depressing.  I could not run a simple errand on my own and I hated every minute of it.  I  was angry and bitter and could not see past my limitations.  When I finally got the braces I can not explain the immense joy that has followed.  I'm so relieved to have this simple device that has given me so much independence and self reliance.  They do not cause me any pain and have allowed me to play baseball with my son. I even stopped hiding them and have worn them with shorts.  God has done and continues to do a work in me through this disability.  Thankful for the things he is teaching me along the way.

Jason- My sweet husband continues to care for us all.  He bears the weight of so many things and works so hard.  He continues to feel well and goes in for regular visits with his rhuematologist who doesn't see any concerns at this point.

We covet your prayers as this summer will be a busy one with lots of apointments, procedures, and surgery.  We are thankful for you all as you have prayed and supported us through this very long season.  Pray that we would continue to fix our eyes on Jesus the author and perfecter of our faith.

Fighting For Joy

The past three weeks have been difficult both physically for me and emotionally.  I feel like we have been dealt so much that I have reached my limits and am having a hard time maintaining joy through this process.  It's a struggle.  I don't want to feel this way.  I have so much to be thankful for not to mention that I deserve nothing yet God continues to bless.  It is a struggle physically to walk and do the things that once came so easy for me  and that has taken a toll on me.  Every fall is a reminder of my loss of independence and my dependence on braces,  wheel chairs, and others.  I want Jesus to be enough for me and so I struggle to fight for Joy.  We had a scare with Hannah this week that just sent me over the edge.  She is fine and we are so grateful but I cried out to the Lord enough is enough.  I'm weak and can't bare any more pain.  I long for heaven more than I ever have.  I just long for the day he will wipe my tears away and there will be no more pain. These are big things, and sometimes have been really hard to bare. There have been days where I have cried out to God to give me strength to face another day.  God has been faithful in his word to show me how intentional he is in our suffering.  It’s all so that the works of God can be displayed.  What helps me get through these tough moments is  knowing that Not only is God  in control of our trials but he purposes them for our good.  

• John 9 where Jesus heals the blind man.  Is a perfect example of this

"As he went along, he saw a man blind from birth. His disciples asked him, "Rabbi, who sinned, this man or his parents, that he was born blind?" "Neither this man nor his parents sinned," said Jesus, "but this happened so that the work of God might be displayed in his life."" - John 9:1-3 (NIV)

We might think that it was rather hard on this man to suffer blindness, just so Jesus could come along and heal him. But Jesus tells us that this made all those years of suffering worthwhile. As difficult as it is for us to understand and accept, serving as the object of Jesus’ healing power was of greater worth than a lifetime of perfect vision. Similarly, God may have a purpose for our illness that we do not yet understand. 

Heres a passage that has been an encouragement to me

2 Corinthians 4:16-18 

 So we do not lose heart. ^fThough our outer self⁴ is wasting away, ^gour inner self ^his being renewed day by day. ¹⁷ For ⁱthis light momentary affliction is preparing for us an eternal weight of glory beyond all comparison, ^18 jas we look not to the things that are seen but to the things that are unseen. For the things that are seen are transient, but the things that are unseen are eternal.

It'S NOT SAYING IN THIS PASSAGE that it takes the difficulty away and that all these things aren’t horrible and painful right now and that we should go through life saying everything is fantastic AND It doesn’t mean that because God uses these difficulties that we are to love them.  Its just saying that it is nothing compared to what is waiting for us.  Something far better than we can even imagine. An Eternity with our Heavenly father.

I will continue to fight for joy knowing these truths and knowing that these light momentary afflictions are preparing for us something better than we could ever imagine.  Thank you for allowing us to be real and vulnerable through this journey and thank you for lifting us up in prayer.  We are thankful for you all.

Thank You

Sweet Friends and family we are once again overwhelmed by the Love and support you all showed last evening.  Chik-Fil-A was hopping from 5-8.  It was so much fun talking with you all and meeting many for the first time.  We are honored that you would journey with us and love on us in this way.  We have really seen the Lord provide in such beautiful ways.  We broke last years record breaking night by $600.00 and we raised a total of $3,600.  It was so much more for us than the financial gain though.  You all took the time to come out and tell us how you are praying for our family and that means more than anything.  Our sweet boy had a smile on his face the whole night and this will carry him for years to come.  You all are helping us teach him what is says in God's word. Romans 12:15 Rejoice with those who rejoice, weep with those who weep.  Thanks for living this out beautifully.  Thank you to the Purtell Family for allowing us to have a spirit night at your establishment but even more personally working the event and for your generous contribution.  Thank you to Lu Lister for all your work in coordinating such a beautiful time.  Our cups are full and it gives us the encouragement to keep pressing on in faith.  We are trusting that Noah will receive his heart in God's perfect timing and are grateful for the ways he is working in his life.  Thanks again for such an amazing night.

Here is a video from Noah

https://socialcam.com/s/FhieUWkJ

A new set of feet

This week and last has been filled with lots of doctor appointments.  While I'm thankful to have lots of great doctors and there expertise I long for the days this is not part of our normal routine.  Today's appointment was for me.  It has taken me a year and a half to get to the point of accepting that my feet and legs need help walking.  I finally broke down and got AFO braces. Unfortunately I have been in major pain while wearing them. After two different orthotics places said there was nothing more they could do, that it was most likely my feet, and that I needed to see a foot doctor I made an appointment with one recommended by a friend.  Dr. Melissa was super sweet and full of compassion.   I think she almost cried when she saw the braces I was wearing and knew exactly what they were doing to my feet.  She explained that they were hitting a nerve along the side of my foot.  She asked if I was experiencing burning and numbness. YES!! I said.  This woman gets it.  She was flabbergasted that they never casted me and told me a custom fitted padded AFO would bring me comfort and relief and provide a more stable walk.  She brought in a picture and although it was bigger and bulkier I was relieved to hear that it could be solved by a better AFO.  I was thinking that surgery and shots were in my future.  This news was much better.  You bet I will be styling these bad boys and making them look just as cool as my pink wheels.  Unfortunately since they are casted and custom made they wont be ready for about three weeks.  I will have to rely on my dear friends to push me around in my sweet pink ride.  Any takers?

This weekend I got to go to our Church Woman's retreat.  It was such a rich time of fellowship and studying in Philippians.  I felt as though each speaker was talking directly to me and giving me reminders that I needed to hear.  I have really allowed my circumstances to still my joy.  I'm so focused on them instead of the gospel.  I sometimes forget things like Philippians 4:19 where Paul reminds us that God will supply our every need according to his riches in glory or Philippians 4:13 that says I can do all things through Christ who strengthens me . I was thankful to be reminded that our attitudes can be redeemed.  We were encouraged to pray for wisdom as we allow the Holy spirit to redeem our perspectives.  My biggest take away and reminder was that The GOSPEL is the Anchor that gives JOY and fulfillment in the midst of ANY circumstance even Muscle disease and transplants.  The GOSPEL is meant to penetrate EVERY circumstance in our life.  God  continues to work in our hearts even when we lose our focus and I'm so thankful for that.

Thankful even more for all of you who continue to walk this long road with us.  Tomorrow will mark 18 months of Noah being listed.  He continues to do well and we are so thankful for the ways God has protected our sweet boy and for the work the Lord is doing through our circumstances.  Noah has an appointment tomorrow with his Cardio team.  Please continue to keep us all in your prayers.

Somebody Turned 11

WOWZERS!!!  I have an 11 year old!! That is just crazy.  That means in two years he will be thirteen and then three years after that 16.   Oh man.  I’m very emotional today.  I’m just so blessed that God has allowed me to be this boys mom.  He truly is a gift.  Our prayer is that we get many more years but more importantly that God will use him to impact his kingdom.  It has been a hard couple of years for NOAH but if you asked him if it’s been hard he would most likely say “not really”.  He just has an amazing outlook on life and his circumstances.  He encourages my soul and helps me relinquish my fears because of how brave he is.  He understands that his days are numbered on this earth and deeply cares for others.  Because of his situation he could choose to be sad and depressed but instead he chooses Joy. Noah we love you so much and are so excited to see what God has in store for you this year.  We pray for god’s hand of protection over you.  We pray that you would continue to use your circumstances for HIS glory.  Thank you so much for all the sweet birthday messages for Noah.  He enjoyed each one of them.  Thank you for continuing to pray and support him.  He feels very blessed by you all.

Short Update

Today Noah had his appointment at the UCLA transplant clinic.  It was the usual visit of labs, echo, and meeting with each member of the team. The visit went great and everything looked the same. We asked our usual question of have you seen Noah's name come through for a heart. They see his name come up often but not as number one.  This just means he is close, usually within the top three for a heart his size but really no one knows when it will be his turn.  I get impatient because I want Noah to feel better, I want to not live my life in limbo,  and because I fear for his life.   However today another thought came to my mind.  I thought about Noah's future donor.   I told Jason that I don't want to be impatient.  I want to be grateful that we have this time and that Noah is doing well.  I realized somewhere out there a family gets to have more time with the child they will  one day lose,  a parent who will make the decision to give our son the gift of living more years gets to hug her baby another day.  The longer we wait the longer they have to hold and love that sweet child.  So I will be patient, I will trust, and I will be thankful.


For I am sure that neither death nor life, nor angels nor rulers, nor things present nor things to come, nor powers, nor height nor depth, nor anything else in all creation, will be able to separate us from the love of God in Christ Jesus our Lord. Romans 8:38-39

Recap of doctors appointments.

I wanted to update you all on the recent doctors visits for both Jason and Noah.  Jason was suppose to have a biopsy done last week but unfortunately they messed up his appointment and it is now rescheduled for February 13.  The biopsy is for a small lump that was found on his chest.  The doctors do not seem overly concerned at this point.  Given all his strange symptoms over the last few months they still would like it checked out.  Jason's appointment with the Rheumatologist went as good as it could have yesterday.  Jason had a positive ANA test and has a few markers for Lupus.  He does not have enough markers to officially diagnose him though and the ANA retest could come out negative.  They are going to retest and check his kidneys out.  If he has any other symptoms he is to go back to the doctor.  We are thankful that so far it doesn't seem to be anything serious.

Noah saw Dr. Chang and Dr. Alejos today.  There were no big changes from the last two months.  He is going to put him on a baby aspirin a day.  The doctors discussed the possibility of him having a stroke as time goes on and the need to prepare.  It's hard to believe that two years ago this week is when we started this journey for Noah.  I never would have imagined we would still be waiting but here we are.  Jason and I were talking about how nervous we were a year ago.  Jason wanted so badly to have his heart cath done before we got the call for a heart.  He just needed extra assurance and knowledge that his heart really was failing.  I think God knew we needed that extra time to wrap our minds around everything and to get to a place where we were more accepting that this was gonna be our new reality.  God has done so much work in our hearts to help us prepare.  We feel more ready now.  Ready as one can be anyway.  I don't think a parent can ever fully be ready for something like this but we are in a better place and trusting the Lord even more for his works to be displayed through this.

Thanks once again for following us along this journey.  Thanks for your constant prayers.  We are so grateful.