Our Newest Family Member

I would like to introduce you all to the newest member of our Family “Dawson”  

Dawson is a Golden Lab. Yep you guessed it he is part Golden retriever and part Lab.  Here is a brief description from our new boy.

I am one of those dogs that loves to work! I like to play too and cuddle for hours in the humans laps. I know the difference though for when its time to work and when its time to play. My trainers worked hard on teaching me this important detail.  I am really excited to take all of the training I have had and put it to good use for my special person. I know all of my easy tasks (trainers call them basic) and I know the hard ones especially for smart service dogs! I can turn on/off lights, open/close doors, push buttons, get my vest, retrieve items, brace, the list goes on and on. Pretty cool! I am very proud of how hard I have been working and I can tell my trainer is too! It makes me so excited to know that even though I am ending one part of my journey at the ranch, I am really just starting my BIG journey with my person. I will love my person so very much! I hope to meet you soon!  Love, Dawson

We will start our training in a few weeks at the Little Angels Ranch in San Diego.  The second week of our training we will be able to take Dawson home. YAY!  This is super exciting and such an answer to not only our prayers but many of yours as well.

We want to thank everyone who has been on this journey with us for the last two years. Wow! Can you believe it’s been two years. Many of you have contributed financially through fundraising, donations, your time, and prayers.  We really can’t thank you enough.  I’m so excited for you all to see and meet this sweet dog and for you to see him at work helping to make our lives a little bit sweeter.

Look for more training updates and lots more pictures in the coming weeks.

Christmas Reflection

A few years ago we created this timeline as a way to look at how our disease and medical journey has transpired.  We have since updated the time line to add events from the last couple years.  I did not put down every ER visit, test, doctors apt. etc.  I just highlighted the main medical events.  

As I reflect back on this timeline I’m filled with many emotions.  Mostly gratitude for the ways God has carried us through.  I also feel hopeful because I know that God will never give his people trials in which he will not sustain them and bring them through to everlasting glory.  This is not to say I don’t grieve my losses on a daily basis.  I certainly do but I grieve with hope.  The Hope and Assurance I read from 2 Corinthians 4:16-17 that says: So we do not lose heart. Though our outer self is wasting away, our inner self is being renewed day by day. For this light momentary affliction is preparing for us an eternal weight of glory beyond all comparison, as 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.

I hope this holiday causes you all to pause and take time to be still. To reflect and think about what you are putting your hope in. Hope is found in the promises God has given us – promises of freedom from sin. We can find so much hope in Scripture through the gift of eternal life made possible through His son, Jesus Christ. So on that first Christmas night God not only came down, He came WAY down. As Yancey puts it in his book, THE JESUS I NEVER KNEW, Unimaginably the Maker of all things shrank down, down, down, so small as to become an ovum, a single fertilized egg barely visible to the naked eye, an egg that would divide and re-divide until a fetus took shape, enlarging cell by cell inside a nervous teenager.? To rescue us GOD became MAN and he entered this world in the same way any man does as a tiny BABY. He is our Hope. No matter what trials, temptations or pain we may suffer, we can always hold onto the hope God extends to us. I hope you all Have a Wonderful Hope Filled Christmas.

1977- Melody was born and it was a normal delivery

1989- Melody could not keep up with her peers when it came to physical activity, always came in last for the mile and walked most of it, was nicknamed peanut because I was super small.

1997- Began having syncope episodes which led to pace maker implantation for complete heart block.

1999- Jason and Mel get married

2003

- Noah was born six days past my due date.  Complicated birth which led to an emergency c-section.  My lungs began to fill with fluid, my heart rate dropped and so did Noah's. Noah hit all his milestones early and we never saw any signs of MD early on.

2005

- Melody underwent emergency pacemaker surgery to replace a lead wire.

2006

-Jonah was born.  Planned c-section.  Healthy boy.

- I had dropped a significant amount of weight this year after just having Jonah and was now under weight and we started to become concerned.  Got my thyroid checked, blood tests, saw a nutritionist etc. No real explanation.

2007

- Noah starts T-ball and we notice lack of coordination but didn't associate it with anything and never found it cause for concern.

- I had pacemaker surgery to replace another lead wire

2008

- I became pregnant with our third child and miscarried at 10 weeks. We prayed about adopting and submitted our application almost to the day of Hannah's birth.

2009

- I began to trip and fall occasionally.  We just chalked it up to me being clumsy :)

2010

- We flew to CHINA to adopt our beautiful daughter who had a multitude of heart issues the major one being poor oxygen due to only having a single ventricle. Double inlet left ventricle, pulmonary stenosis, VSD, ASD and too many others to name.

- 3 days after being home Hannah goes into major heart failure, we were given a 30% chance of survival if they operated or the option to keep her comfortable and she would not make it through the night.  God performed the first of many miracles and she had her first successful open heart surgery.

2011

- I was still falling and hadn't gotten any answers about my weight loss. After being referred to a neurologist we discovered it was a form of MD.  Which one though? Lots of tests, biopsies and doctors visits.

2012

- Noah complains of chest pain a few times.  we don't think much of it but remember being encouraged by Hannah's doctor to get EKG on our boys.  I took the boys to get tests and got a phone call that afternoon that I needed to take Noah to the Cardiologist right away.

- We were able to take Noah the next day and I was told that they might have to airlift him to UCLA or admit him to CHOC.  We were stunned and confused. UCLA  agreed to let us take him first thing in the morning.  More tests, trouble with insurance, a switch to CHLA then back to UCLA.

- May 30, 2012- We were told Noah had Restrictive Cardiomyopathy and would need a heart transplant.

- September 19 2012- Noah was put on the transplant list as a Status 2.  Lots of meds, monitoring, caths, and procedures became our new normal.

2013

- Our family goes on a make a wish trip-Disney Cruise to the Bahamas

-Tons of fundraisers put on by friends and community

- I purchase my first wheel chair-pink of coarse for longer distance walking

- Pacemaker surgery to replace battery

2014

-Genetic testing confirmed that both Noah and I have Desmin Myofibrillar Myopathy a rare form of Muscular Dystrophy.  We find out that both our heart conditions are symptoms of the disease and that it manifests different in every person.  We find out that it is a slow progressive disease that will effect all of our muscles.

- Jonah is tested and the results are negative- Praise the Lord!!!

- Hannah has second open heart surgery "The Fontan" to place a shunt to help the blood flow to her heart and they also implanted a pacemaker in her abdomen.

- Melody finally breaks down and decides to listen to doctors and gets her first set of AFO's(foot and ankle braces).

- Melody gets adaptive equipment put in her car as her legs become to weak to drive.

- Discovered weakness in Mel's swallowing and breathing

- Melody started being seen at the UCI MD clinic and learned that her breathing numbers were very weak.

- Bi-pap was delivered as well as a cough assist machine to assist with Melody's breathing

2015

- Noah moved from a status 2 on the list to a status 7(in active) Praise The Lord!!!

- continued progression of weakness for Mel

-Started walking with a cane

- We sold our house in La Habra

2016

-Noah began getting out of breathe frequently and was referred to the CHOC MD clinic

-we learned Noah's breathing numbers are mildly weak

-Noah receives his cough assist breathing machine

-Noah gets diagnosed with Torsil Coalition and has foot surgery

-Noah comes off the transplant list(Praise the Lord)

-Noah has a hard time physically getting around at summer camp and keeping up with his friends doing anything physical.

-Because of more weakness in Mel's legs she gets her first pair of full leg KAFO's but unfortunately they do not work out.

- Bought a new home in La Mirada close to church, friends,school and support

** Updated Below

2017

-Melody has surgery to insert a G-tube.  This was a much needed surgery and helped to gain 6 pounds.  It has also increased energy and made it easier to get meds and nutrition in.

-Melody has three surgeries in 6 weeks. Making it four surgeries in 10 weeks. Melody had a Ventricular Tachycardia episode that made her heart stop for 11 seconds.  It was determined Melody needed a defibrillator.  Many complications led to two more additional surgeries.

-Melody starts using a walker to get around easier at home.

- Melody now needs help getting dressed, and turned in bed

-Through overnight testing it was determined Melody needed to be put on a liter of oxygen at night.

2018

-Melody, Jason, and Noah fly to Minnesota to visit the Mayo Clinic.  We met with a doctor who is a specialist in our rare disease. We leave feeling encouraged that research is being done to help people with this disease.  Also thankful to finally find a doctor familiar with our disease.

-Noah begins to show weakness in hands, feet, and abdominal muscles

- Melody continues to get weaker and has a hard time walking without the aid of another person.

-Jason has an unexpected seizure like episode and is taken by ambulance to the ER(everything checks out fine) he follows up with many tests that all come back normal.

Hannah Turns 10

For those that have been following our story for the last eight years will understand what an amazing feat Hannah turning ten is.  Our sweet girl has had two open heart surgeries, been on oxygen, had a pacemaker put in, meds, and many doctor appointments in the last almost eight years that we have had her.  She is our miracle girl.  Hannah has double inlet left ventricle which means she only has one functioning ventricle, many holes in her heart, and array of other things.  After her two surgeries she is doing amazing and you would never know by looking at her all that she has endured.  She is a constant reminder of God's beautiful grace and mercy.  I realize that many of you who are new to my blog may not know her story.  You also may be thinking I knew about Noah and Mel but not Hannah.  It's hard to believe but Hannah's story came first and we don't talk about it as much as the rest of us because praise Jesus she is more stable and doing AMAZING.  Below is an article sharing Hannah's story from the Orange County Register and her homecoming video.






https://www.ocregister.com/2010/05/28/little-girl-mends-hearts/

https://vimeo.com/10399991






https://vimeo.com/10883744

  

Mayo Update

As I reflect back on our week in Minnesota I’m grateful for so many things.  Grateful for the Lord's provision in making this trip a reality for us. Grateful for so many peoples prayers and support. Grateful for visits with long time friends and Grateful for the opportunity to have so many appointments and tests with incredible doctors all in a weeks time.

I had heard many incredible things about the Mayo Clinic but it more than surpassed our expectations.  It is an incredible place.  I have never experienced anything quite like it.  What do I mean?  Well for starters every doctor we met with spent over an hour with us and it was clear that they studied our files and talked with other doctors at the Mayo clinic about our case.  They have a team approach.  They all work on Salary so they have the gift of time.  Everything is done onsite.  Every test, lab, research etc.  In CA we see doctors and specialist all over town.  Every procedure is scheduled weeks out, and different companies have to mail things out to you.  At mayo we were able to do tests, procedures, labs, x-rays, and many doctor visits all within a week.  Some tests that the doctors wanted after we saw them we either did same day or next day.  I joked with Jason that everyone of the staff must take Happy pills.  Everyone seems to love their job and treated you like you were very important to them.  Everything is run so efficiently and set up super well  even for people who have disabilities.  They have special needs restrooms available on every floor, the check in Kiosks you can move down to wheelchair level, the drinking fountains are normal and wheelchair height and have dixie cups for those that can’t drink from the fountain.  They really thought things through.  People come from all over the world come get care from Mayo. If you want to know more Ken Burns did a documentary that aired on PBS this week.  You can go to PBS.com to watch it. After my first appointment I downloaded the Mayo Clinic app on my phone.  This gave me access to my schedule, labs, test results, and comprehensive doctors notes and records(they take amazing transcribed notes after each visit).  All of these things are downloaded on my app within an hour of completion.  I was able to see my x-rays, blood test results, doctors notes etc.  I have had other portals here in CA but nothing is ever loaded and the doctors don’t put any notes from your 10 min visit.  The place is huge and I can’t say enough great things.

Our main reason for this visit was to see Dr. Selcen.  A doctor who has the most expertise with our specific rare disease.  When we first met Dr. Selcen we got the impression that she was just as excited to see us.  She has a passion for studying Myofibrillar Myopathies. If you look up Myofibrillar Myopathies you will see her name all over many of the publications. She is a research doctor and sees other patients with our particular Myopathy.  She is brilliant and every doctor we encountered talked about how brilliant she is.  She spent close to two hours with us at our first appointment and we met with her a total of three times.  She explained what was going on with the genes to cause this Desmin protein to aggravate the muscle fibers.  We felt like we gained a ton of understanding and felt hopeful that she is working hard to find a way to help this Myopathy.  She gave us some handouts on some current studies that are being done.  She recommended a couple antioxidant supplements.  They have done some testing and have found that one may help to slow down the aggregation of the protein in the muscle that is causing the weakness and the other helps keep the muscles that haven’t been effected energized.  They are over the counter and is no way a cure but they have found it to help.  We are looking forward to giving them a try and keeping in contact with her as more questions and symptoms arise.  She is a part of a Desmin Facebook page that she invited me to.  It’s a place to connect, share symptoms, hear about Dr. Selcens research and advances.  I told her I was passionate about helping to educate others especially doctors so they can help others that may be struggling to find a diagnosis.  She had Noah and I take pictures and video for educational purposes.  So thankful to have met her.

Another reason for our visit was to get some clarity and direction on Noah’s heart condition.  This was another positive experience.  We met with a Pediatric Cardiac transplant doctor named Dr. Johnson. This doctor knew Both Dr. Chang and Dr. Alejos our current doctors here at CHOC and UCLA.  He had wonderful things to say about them both.  We love our doctors but have felt unsettled on the fact that they do not agree with one another and it has made us question if we are doing everything wended to be doing.  Noah came into this appointment really wanting to understand his heart condition.  He left feeling like this was the first doctor to really explain everything to him.  Dr. Johnson explained to us that most people with Restrictive Cardiomyopathy do not survive past two years and that most of them do not even make it to transplant.  This is why they put them on the Transplant list right away.  This is why Dr. Chang has been concerned with him being off the list.  Dr. Johnson agreed that his EKG is one of the scariest he has seen.  However his Echo looks amazing and doesn’t make since with his EKG.  His pressures and heart looks good for someone who has RCM.  Since his EKG has been the same since his first one at 9 years of age you have to assume that its because of his muscle disease.  Dr. Johnson agreed with UCLA to keep monitoring him every three to six months and if his echo starts to look bad to list him immediately.  We asked him if he has ever seen anyone get better with RCM and he said NEVER.  Dr. Selcen brought up the point that it will be better to be transplanted  before his muscles get weaker.  This is something we will have to consider in the future.  We came to the realization that it will be a decision that Noah will have to make as he will be an adult in just a couple years.  He understand this and I think its one of the reasons he really wanted to understand his condition.  We left this appointment  feeling more at peace about the decision to keep him off the list for now and feel more confident in our doctors at UCLA. 

My visit with the pulmonologist was difficult.  She was a wonderful doctor and really helpful in explaining how my breathing muscles are being effected by my disease.  It was disheartening to see such a big decline in a year and a half.  She talked through the tough things ahead like trachs, and 24 hour ventilation. These things could be far down the road but it seems like we get closer all the time.  It’s difficult to hear the hard things but also good to prepare our hearts and minds.  My visit with the cardiologist was good.  My echo looks really good.  Even though I’m stilling having v-tach episodes, the fact that my ICD hasn’t gone off is good.  If it starts to go off then there are other meds and treatments that can be done so we felt hopeful.

This is really long so I will end with a heartfelt thank you.  Thank you to those that our invested in our story and care enough to walk along side us. Thank you for all your prayers and words of encouragement.  We felt so lifted up by all of you this last week.  

2 Corinthian 4:16-17

So we do not lose heart, though our outer self is wasting away, our inner self is being renewed day by day.  For this light momentary affliction is preparing for us an eternal weight of glory beyond all comparison.

T- 3 days

T minus 3 days until we hop on an airplane and travel to MN to visit the Mayo Clinic. I have been feeling a mix of emotions as the time draws nearer. I mostly feel deep gratitude for how smooth and evident the Lord’s hand has been all over this process. It started with a conversation with a friend that led me to think about going. I have known about this specialist there that has done research on our rare disease. Just the fact that she has seen patients with our condition is helpful. I called Mayo Clinic thinking I would have to jump through many hoops and many phone calls to make this trip happen. This was not the case. The First Lady I talked to took down my insurance info and set everything up while on the phone. She registered me and told me to send all my medical documents over.  I thought this next step would be difficult so I went in person to two of my doctors to see about getting my medical files. Both places had me sign a form and printed them out right there on the spot. Whaat??? I expected to be directed to another building wait forever to sign papers and have them mailed. Nope not the case. I scanned all my documents and explained to them that I wanted to meet with a certain Neurologist at Mayo who had expertise with our disease. I told her we were willing to fly out and could spend a week. The doctor then called me herself and got more information about what we hoped to achieve at our visit. She then said she would set up apts. and procedures, and labs for us and looked forward to meeting with Both Noah and I. We got our apt. Schedule soon after that. Not only was that process smooth but we found out that Noah’s transplant Fund would cover the expenses for our trip. The Lord has provided for everything and I feel such deep gratitude. I wish that were enough, but I also feel very anxious. I’m nervous for some of the answers to some tough questions we have. Nervous for new information. At the same time I’am Hopeful. Hopeful to see someone that understands this disease and could possibly give us some encouraging news. New possibilities. I’m trying to pray boldly believing that God is big enough to give us some relief from the hardship of this disease if he chooses. My head knows that I should be ok either way trusting that his plan is Better than my own but my heart wants some relief.

Would you all pray for this trip? Pray that we would get answers regarding Both of our heart conditions. That we would get some clarity on Noahs lungs, that we would hear some encouraging new advances regarding our muscle disease, that we would get good encouraging answers to our questions. That we would process information well. That we would help Noah process things well. That we would be a good support to one another. That we would lean on the one who is bigger than disease and hardships.  

I was encouraged this week by Joseph’s story in the Bible. The suffering of Joseph physical, mental, and emotional agonies had been allowed by God so that Joseph could fulfill God’s plan to save many lives. I want to have a response of willingness, faith, humility, and meekness so that God can use me to fulfill his plan.  We would appreciate your prayers so much. Hoping to blog updates for you all during the week.

Melody

Overdue Update

I’m so sorry for not updating about Jason sooner.  To be honest it has been difficult to know just how to update when we are still left with not a lot of answers.  I kept pushing it off hoping I would have something better to give you. The biggest thing to celebrate is that all tests have come out looking fine. We are still investigating but are thankful at this point that there haven’t been any further episodes or cause for concern. No news is sometimes good news.  It took a few weeks for the nerves and anxiety that it might occur again to go away. Thankfully we all understand that  just like everything else that occurs in our family it’s all out of our control.  So we press on knowing and trusting in the one who is sovereign over all things.   

We will get another opportunity to practice patience and trust in a few weeks.  Jason, Noah, and I will be visiting the Mayo Clinic in Rochester MN.  When Noah and I were first diagnosed with Desmin Myofibrillar myopathy I researched it like crazy.  Unfortunately there wasn’t a lot I could find.  I did find a doctor  who did some research on it and wrote a few publications.  Her name is Dr. Selcen and she works out of the Mayo Clinic.  She has actual patients with our same disease. This is a big deal because all the doctors we see are not as familiar with our disease.  They can treat the symptoms but not really help with what to expect.   We had talked about going over the years but Noah was on the transplant list and life just happened.  The Lord put it on my heart again as I was chatting with a girlfriend and  he has literally helped make this trip happen down to every last detail. We leave September 22 and Both Noah and I have appointments and procedures all week long.  We would really appreciate your prayers.  We are really hoping to get some answers and gain some understanding on several big areas of our disease.  More on this in another post.  Because there is no cure for our disease these appointments can be difficult.  We know we will hear many things that will be hard to process. We are hopeful also to hear some solutions and possibilities from doctors who have experience seeing patients with our same disease.   Would you please pray for our time.  I’m hoping to blog more about our experience so we will keep you posted. 

Thank you for continuing to be our support.  We are so grateful.   

Another unexpected ER trip

So you all know that our family has been through many terrifying moments, but last night we had a situation that shook me to my core.  I’m still processing, trying to cope, and trying to help my kids work through it all.

Jason came home from work around six o clock. He walked into our room where I was helping Noah pack for his Japan trip.  He walked into the kitchen, took a bite of a muffin, walked back into our room and sat down on a chair.  He said he didn’t feel well.  I begin to ask him questions and he had a hard time answering.  Out of the blue his eyes rolled back into his head and he fell back into his chair and had some convulsions.  He was unresponsive, I was yelling for him to wake up, my other two kids ran into the room and witnessed this all happening.  I called 911 and while I was on the phone with them answering their questions he came to.  He was very confused, pale, and still not feeling quite right.  The paramedics came and took him to the hospital.  It was a whirlwind and I had never felt so helpless.  I was trying to console my younger two, while my oldest put on my shoes so that I could get to the hospital.  When we arrived to the hospital  Jason looked good and the doctors were running some tests.  The doctors are unclear why he had this episode.  We will follow up with his regular doctors today and go from there.

I never thought I would be writing an update like this about my husband on this blog.  He is our Rock, our healthy one.The one we rely for everything. I’m thankful in so many ways.  Thankful that it wasn’t worse, thankful for Noah and the ways he stepped up for me and was so calm.  

Please would you all pray for Jason, for our kids, for me as we process this and move forward to get answers.  Thankful for you all.