Happy Birthday Madelyn Paige!

Two years old...How has it already been two years since my sweet Maddy was born.  From the time I was little I dreamed of being a mother and those dreams always included a daughter. When I had Alex I instantly fell in love with my mama's boy, but I always knew I wanted more children. Because of our journey with Alex, I wasn't always sure that we would get the gift of more children. We waited 9 long years to try again and what a gift it was on Halloween of 2013 when our Madelyn came into this world. It is all too perfect that Maddy is a Halloween baby because it fits her personality perfectly. She is both Trick and Treat. She is the sassiest, most strong-willed little girl, but also the sweetest, most loving. One minute she is standing with her arms crossed defiantly and the next she is putting herself in time-out for the 5th time in a row so she can get one more hug after she says she is sorry. She is quick to tell me no, but just as quick to say "I wuv you". She is all girl. She loves to play with her baby dolls and cook in the kitchen. She loves all animals. She loves to read. She loves her music and cooking classes. She LOVES her brother and her dog. She loves life! She is so very funny. She makes us laugh all the time. She has been known to thrown something when she is mad and then oh so sweetly say, "ooops..sorry....what happened?". When I ask what she wants to eat her first response is always "Hmmm...let's see." She has brought so much joy into our house.

My precious Madelyn-

I love every ounce of your being. You have so much personality and so much love in that tiny little body. You are so determined to find your way and explore and learn. You are so eager to show your love with a hug or a cuddle or saying "I wuv you". You are so incredibly smart. You already know your colors and shapes and are always counting to three. Your personality and sweet voice are infectious. We can't help but smile and laugh when you are around. Mia follows you wherever you go. You and Alex are the best of friends. Thank you for making me smile everyday. Thank you for completing our family. Thank you for giving your Buggy the sibling he always wanted. But most of all thank you for being the precious incredible little princess you are. I love you.
Mommy

Not the greatest of months

I am usually excited to blog about the fun we have had during the past month. Unfortunately this month didn't have a whole lot of fun, cool activities. Instead it was filled with a lot of medical appointments and hospital visits. What this month did give me is a reminder of how scary mito can be and an appreciation for our new norm which involves a lot less hospital time then it used to!

We started the month with a trip to Houston to check in with our specialists down there. It wasn't the easiest trip for Alex as he had several tests that are less then fun! On Monday, we started the morning with pulmonary. He was happy with how Alex is doing. Then both kids saw Cardiology. He also was happy with Alex and wanted to keep everything the same. He did confirm that Maddy has a hole in her heart, but wants to watch her and not repair it until she is a little bigger. Monday night Alex had a sleep study. He hates having all of that "stuff" hooked up to him, but he handled it better then ever before.

Tuesday we saw Dr. Koenig. She didn't want to change anything with Alex, but did let us know we need to do an MRI of his spine again as it has been two years. She was really happy with the progress that Maddy has made in physical therapy, but still does not like the way she sits, the positioning of her feet or her gait when she walks. She wants us to continue therapy three days a week. She also re-ran some labs that were off the last time we saw her. Once our appts were done Alex had to have cardiopulmonary testing done. It was really hard and took almost 3 hours to complete.

Wednesday we finished up with Dr. Pacheco. She is still watching Maddy closely as she feels like she has an immune deficiency that just isn't clear yet. She always likes to keep a close eye on our boy! After a TON of labs, we headed home.

We arrived home around dinnertime on the 7th and overnight Alex called me because he was extremely nauseous. He took his temp and it was fine so I gave him meds and went back to sleep. When I woke up at 6:00, he was crying that the meds didn't work and he was so nauseous. I went in his room and he had 103.9 temp. I quickly scrambled to pack a bag and head to the ER. Before we even got out the door his temp had climbed to 104.3. He was okay on the way down, but took a turn for the worse shortly after we arrived in the ER. His blood pressure that was fine on entry starting dropping rapidly and he lost perfusion (blood flow) to his extremities. His hands were as white as the sheets and ice cold. I know from past experience that this is not a good thing! The ER doc stayed in our room for almost an hour giving continuous orders. he was given a HUGE amount of fluids and a HUGE dose of hydrocortisone and finally started to stabilize. he was admitted to the PICU in assumed septic shock. However cultures never grew anything and he stayed stable! We were in the hospital for four days and the final decision was that he went into shock caused by adrenal crisis because of either a virus or his flu shot. It was the first time in a while that Alex scared us and was a reminder of how quickly things can turn.

We had a quiet week and a half recovering and then we were back to the hospital for surgery! Alex needed to have his port changed and a mole removed. Alex's current port was placed in January of 2011 after he was in septic shock in December of 2010. We were told that we couldn't leave a port in place longer then 5 years because it starts to adhere to everything and becomes difficult to remove. We feel incredibly lucky (and have worked extremely hard) to still have a port almost 5 years later without infection! So this was a planned surgery. The surgery went as planned. Alex has two incisions on his chest. One that is pretty small and one that is almost 2 inches long. He also has a long incision on his jaw line (maybe an inch and a half?). He proved once again how tough he is as he made it through recovery without ever taking a dose of pain meds.

We are happy it is almost November and hoping this month will bring back fun times and less doctors visits! I am continually reminded of how strong and determined my two precious children are and how very grateful I am I get to be there mom!

Ali

Crazy, Busy Life

2 months...the days, weeks and months are just flying by! So often I sit down thinking I need to update our blog and then life starts happening around me!

We have had a good summer. I'll start with Alex. Alex amazed all of us this summer by participating in his first sleep away camp. He spent a week roughing it with his friends at Boy Scout Camp. It truly took a team effort to keep Alex safe and healthy, but there are no words to express how proud I am of my amazing boy. Boy Scout camp is hard for all the kids, but the amount of strength and fortitude it took for Alex to push himself through the week is remarkable. His efforts did not go unnoticed. The two times I went to camp to take care of some medical needs, I was stopped by several people saying they had watched Alex and how amazing he was. The nurse running the health center even commented that instead of Alex coming for care he would bring others because he knew where it was! I am constantly reminded about the strength God packed into his little body. He is an example everyday of pushing ourselves to be the best we can be. Alex was able to make his first rank advancement in Scouts. He also continues to work hard in TKD. He has done a week of Mission camp with our church group and a week of arts camp at our hospital. Medically he continues to do well. We are getting ready to do growth hormone stimulation testing as he is finally stable enough to try and help his growth. We started school in mid July and have already completed 5 weeks of 6th grade. He continues to thrive academically and especially loves Science.

My little Maddy... If I had to choose three words to describe my Maddy they would be sweet, determined and strong-willed. That little 20 pound peanut has more determination in her then most adults. She is such a hard worker. Nothing physically has come easy to her, but she works SO hard to try and move her little body. She is in therapy 3 days a week and amazes us with her work ethic. She will be exhausted and shaking, but still try and take a few more steps. She is getting so close to walking. Our hope is that she can take some independent steps by her birthday. She is still getting her neupogen shots to boost her blood counts twice a week and they have helped immeasurably. She doesn't gain weight very easily, but she loves to eat! She is the sweetest little girl who loves all animals, music, babies and especially her family. She is talking up a storm and always asking for hugs. She says please and thank you and sorry. Her brother is her favorite playmate besides maybe Mia. Maddy and Mia are inseparable.

We are gearing up for a busy fall. Maddy and  are leaving tomorrow for Houston to do some specialized testing to see if we can determine if her issues are muscle or nerve related. Then we are taking our annual trip to Mimi and PopPop's. Alex and  look forward to our time at the beach all year. We start our homeschool co-op as soon as we return. Then Alex has a special event in September  as he was nominated for a fundraising event called Night of Superstars. We also, have a few Make-a-wish events and two trips to the National Institute of Health for both kids to participate in a study on Mitochondrial Disease and immune function. I have also been asked to serve on the family advisory council at our children's hospital and am working about 25 hours a week for Taste Buds Kitchen as well as dancing in the Dance Ministy at church. I am also siting on the panel of a new Special Needs Ministry at our church!

Whoo! Now you know why I haven't had time to update our blog.
Thank you to all who pray for us on this crazy journey!
Ali

PS- I promise pictures soon!

Happy 11th Birthday, Alex!

11 years. How my sweet boy did you ever get to be 11 years old? I am so very proud of the brave, strong, caring boy you have become. You always have a way of endearing yourself to everyone you meet. I have loved watching you mature this year. I look at you and still see my baby boy and yet you are growing up and becoming such an incredible young man. You continue to astound us with that curious, smart mind that absorbs so much of the world around you. I have watched you become the most loyal, protective, sweet older brother. Maddy adores you. I love to hear her call you "A-yex" and look up at you with so much love. I love how much you love her and how you always want her with you. I love to see you grow in confidence and independence.  My sweet boy, I hope you always feel the love that surrounds you.

Love,
Mommy

Exciting Month

I have been trying to find the time and update for weeks. Unfortunately, every time I sit down to write an update something comes up!

At the beginning of April we were contacted by one of the child life specialists at Cook Children's. They wanted us to represent Cook's at a Benefit Gala being held in conjunction with the ACM Awards. The gala was benefitting the Child Life Zone at Cooks. They needed to choose a patient who had been positively affected by the Child Life Zone. Alex has spent countless hours there over the last few years and especially the last few months as he created a Lego Stop Animation Movie with the production staff. I am not sure I can even put into words what that night was like. It was amazing to see my boy confidently meet celebrities and donors and speak eloquently about the hospital and what child life means to the patients and families. Everyone was so kind and genuine. It was so nice to see my boy with such a huge smile on his face. We are so grateful for the opportunity to attend this gala and help raise funds for something that means so much to us.

Garth Brooks and Tricia Yearwood

Nancy O'Dell

Jerry Jones

The magic continued...As we were leaving we were asked to come to the hospital the next afternoon to once again speak with Garth Brooks. While the child life zone was swarming with people anxious to get autographs, we were taken out to meet Garth as he came in. We spent a few minutes talking to him. He presented Alex with an autographed cowboy hat that a generous donor had bid on and wanted Alex to have. Such an amazing memento of our weekend. Monday as we were trying to recoup, I received a message on Facebook that Alex and I were on Entertainment Tonight! What a crazy, magical experience. As I profusely thanked the child life staff for this experience, I let them know what a great teaching tool it is for me to be able to point out that as hard as his life is, as much as mito has taken from him, we also gain...incredible experiences, amazing friends, and unforgettable memories. 

Our crazy life continues in full swing, but I see a light at the end of the tunnel and hopefully some slower days!  Alex is busy finishing up school. We have 2 weeks left in fifth grade! Homeschool co-op finished this past week. We are busy with Wish Night (The Huge Make A Wish Fundraiser that we participate in every year) rehearsals where Alex was given a featured role in the opening number. Alex is also still busy with TKD, boy scouts and has done some other volunteering with Make A Wish. he continues to do well medically. We are so thankful for the incredible team of doctors and nurses who have brought him to this place and found the right mix of interventions to have him the most stable he has ever been.

 

Miss Maddy Moo also has some exciting news. After months of therapy three times a week, she is starting to walk with the help of orthotics and a walker. She loves her new independence and is so proud of herself. It has been and continues to be such a struggle. Her PT always says that she only walks by sheer determination. She works so hard, but she has come so far from a few months ago when she wouldn't even bear weight on her legs. We are so proud of her.

We continue to work with hematology, GI and neurology to try and sort out this little sweet pea. We saw GI in April and they started her on a special medication to try and increase her absorption and gain some weight. She seemed to be making some strides, but she has been sick this past week and was unable to eat as she had blisters in her throat. Hopefully, now that she is feeling better she will start to gain again.

 

We did manage to sneak in time with Heather at H. Parker Photography to get some bluebonnet pictures. Our session was right around Maddy's bedtime and she was a bear. I was really worried we wouldn't get any good pictures, but somehow heather worked her magic and got some beautiful photos!

 

Thank you for sharing this crazy journey with us!

Ali

 

 

Happy Easter

Happy Easter!

 



I really am not sure how the months fly by quite so quickly! I cannot believe it has been 6 weeks since I updated!

Alex continues to experience the best year of his life. I am really unsure of what to say, but so very grateful for the incredible team of doctors and nurses who have gotten him to such a stable place. A few years ago, this "normal" life living without constant hospitalizations just didn't seem possible. To watch Alex thrive and enjoy being a boy is such a joy to me.

By far the greatest joy is watching the love between my two babies. While having another child was something I wanted so desperately. Alex craved a sibling every bit as much. He never takes his sister and his love for her for granted.  He frequently tells her that she is his greatest gift. he has even pronounced Halloween his new favorite holiday since that's when he got "his bundle of joy". Anyone who spends any time with my two comments on how special their bond is. It really is more then I ever hoped for.

Maddy continues to keep us on our toes. She was hospitalized shortly after my last update. She had a fever and croup. Her blood counts did not respond as they should so she had to be admitted for 48 hours. After that admission, her hematologist decided we needed to support her little body more. She was started on a treatment to force her bone marrow to produce more neutrophils (a kind of white blood cells). She has responded exceptionally well. The treatment can range from once a week to every day. It is a shot so they try and use the lowest dose possible. She had such a great response that we are treating only twice a week. This medication should keep her from future hospitalizations for "routine" illnesses because her counts are much closer to normal range. We also had an upper endoscopy to figure out her malabsorption. My understanding from the nurse is that the biopsies are normal so I am not sure where that leaves us as the doctor was sure they would be abnormal. We follow up with our GI next week. We also had a spine MRI trying to determine why Maddy's brain and legs don't communicate correctly. That was also normal so we need to move on to other testing. Dr. Koenig has ordered two other tests to look at muscle and nerve function. We are trying to get them scheduled ASAP. We are trying to determine if there is anything else we can do to help her besides continuous PT. We pick up her orthotics on Tuesday so hopefully that will help as well. Despite her challenges she is thriving socially and emotionally. She is so smart and FUNNY! My sassy little diva.

Mimi and PopPop came to join us on our trip to Houston. It was good to see them and have some fun times, but I wish we could have had an enjoyable vacation vs. medical appts. I can't wait for our trip to SC this summer!

Hoping for a nice quiet few weeks!
Ali

Boy Scouts and updates

Yesterday was such an emotional day for this Mommy! Almost 6 years ago we decided to enroll Alex in Cub Scouts so he would have an activity he could do with friends since we knew sports weren't going to be a good option for our boy. We had no idea what it would come to mean to Alex and to Bruce and I.

 

Over the last five years, we have watched Alex and his den work hard to meet requirements and achieve goals, but more importantly, we have watched them forge friendships that have changed Alex's life.

This sweet group of boys have grown up together.

Cub Scouts is all about teaching character. About being good citizens, helping others. These boys have exemplified that in everything they do. I have seen each one of them help Alex in countless ways. They have hung back with him when he physically couldn't keep up, they have stuck up for him when being made fun of, they have helped him set up tents, they have grabbed me or Bruce when he needed us medically, they have helped him complete projects. Simply put they have loved him.  So it was with pride, but also sadness that we watched these boys leave cub scouts and move on to boy scouts last night. It was the last time that Den 2, our flaming Arrows will be together as a group. And so in typical spirit they blessed my boy one last time. Two years ago when they became Webelos and chose a patrol name of Flaming Arrows, they made a flag. Last night they voted for Alex to keep that flag as the scout who showed the most Scout Spirit. A simple gift with so much meaning. For Alex he felt pride. For me, I felt love, love that has enveloped my sweet boy, by these incredible kids and their remarkable parents.

And so with tears in my eyes, I watched as my baby grew up, as he crossed a 15 foot rope bridge to become a Boy Scout.

I am so excited to see what this new chapter holds and so happy five of these boys have chosen to cross together to the same Troop.

Let's see, what else...
Alex continues to be more stable than he has ever been. He looks good, he feels good. We had one little hiccup where he got a mild case of pancreatitis and rhinovirus which landed him in the hospital for 4 days, but he recovered well.

Maddy is a mess! But boy is she a cute mess! She is such a doll who continues to make us smile. When Maddy is happy she is infectious, but look out when she is mad. She will let you know it! That spunk and determination is serving her well as she continues to struggle to meet gross motor milestones. It is her sheer will that allows her to do anything because nothing comes easy for the poor girl. She is in physical therapy 3 times a week. We are making progress, but it is SLOW. Her brain doesn't seem able to get her legs to do what they are supposed to. The question is why? We are trying to figure that out. She will have a Spine MRI on March 30th to rule out a tethered spinal cord. If that is the case we will do surgery to untether the cord and she should make progress pretty quickly once she has recovered. If it is not a tethered cord we will need to do some more testing looking at nerve problems. In the meantime, we are working on getting her some orthotics (SMO's) to help her feet and ankles. Her little ankles are so weak and loose. She cannot keep her feet flat on the floor when standing. Her feet roll. We are hoping that orthotics will at least help that issue as we work on the rest of our "standing issues". I have had a lot of people ask, so I will address this here... We have been told by multiple medical professionals that there is nothing we could have done any differently to change or prevent this. This is not a case of Maddy being spoiled or held too much. It is in inborn reflex to put your feet down and bear weight when you are held under your arm pits. Maddy WILL NOT let her feet touch the ground in this position. We are working as hard as we can to "teach" her something that should be natural. Every physical milestone has been like this. Trying to teach what she should just "get".  The other big issue we are working on is her GI/weight issues. She has had multiple tests and we know at this point that she does not absorb carbohydrates correctly. She will have an endoscopy and biopsies done on March 4th to determine which enzymes she is missing. Once we figure that out we will have to make significant diet changes and eliminate whatever is causing the problem. Despite all the medical challenges, our little princess continues to charm everyone she meets. She is so very smart and we love watching her learn more every day.

As if life wasn't crazy enough, I am dancing two nights a week. I have loved refinding my passion for dance. it is so good for me to get out and have some time for me each week. As much as I love the dancing, the fellowship is so great. I feel renewed when I leave each week. I am so grateful for the break! I have also taken a part time job! One of my dance partners and good friends is opening a business called Taste Buds Kitchen. I am her "sales manager".  For any of our local friends you need to check it out! www.tastebudskitchen.com/southlake

To say we are busy is an understatement! Between Taekwondo, scouts, school, homeschool co-op, doctors appts, therapy, Kindermusik and my work it is pretty crazy! However, we love that everyone is well enough for us to enjoy time with friends doing activities we love.

Thank you for those that continue to support, love and pray for us.
Ali



Maddy Update

This is an update I prayed I would never have to write. We met with Dr. Koenig yesterday morning to go over the results of the brain MRI. The MRI was normal, however at this point Maddy meets criteria for a "definite Mitochondrial Disease".  I have considered the possibility as Maddy has had some health concerns, but I thought there would be more testing. I did not realize she already met the diagnostic criteria. I am sad, but ok. Our children have totally different presentations and we will treat them as individuals. I know we will (and are) intervening much quicker with Maddy so hopefully she will not have to face all the struggles Alex has.

We spent the rest of the appt discussing the neurologic concerns with Maddy's development. Her brain is fine. There is a discrepancy between the development of her upper and lower extremities. Dr. Koenig said there are some definite problems from a neurologists perspective. The way Mad holds hers legs, their movement, the reflexes, etc are not normal. The next step is to do a spine MRI. If that does not give any answers we will have to look at nerve function. In the meantime, we are to continue PT.

I have not received any results on the bone marrow biopsy yet, but Dr. Koenig feels like the hematology issues are definitely related to the bigger picture.

On the bright side, Maddy did really well with the procedure and is happy to be home. She is a little sore, but playing and smiling. Alex was so good during the trip which was so helpful for me.

Please pray for my precious children and their health. Please pray for peace and for my mama's hurt that is hurting at what my babies have to endure.

Ali