HUGE Maddy Update!

It has been a really fun and crazy few weeks. Mimi flew in on December 12th for 5 days. It was a short trip, but we had so much fun! Mimi got to spend so much time with the kids just loving on them. She got to really enjoy Maddy and see her spunky little personality. It was so nice for me to have a second pair of hands with my "Mama's girl" for a few days. For 10 years I have loved watching the incredible relationship grow between Alex and my Mom. They have such a special, close relationship. Bug will always be, Mimi's boy. It was so neat for me to watch the love and closeness grow between Mimi and Maddy this trip. We cannot wait till March!

The day after Mom left, Dad flew in! We are busy getting last minute things done for Christmas and cooking some of our favorite home cooked meals.

On to Maddy... On December 4th, during Alex's GI apt, Dr. O and I got talking about Maddy's size. he was concerned by the amount of food she was eating and still not gaining weight. He gave me some samples of a formula meant for kids with trouble absorbing and asked me to schedule her for an appt. Surprisingly, she started drinking the formula right away with no problems. We were able to get an appt 1 week later. So on the 11th, we had her first "real" apt with our GI. He was not happy with how far down the growth chart she has fallen and that she is not gaining despite adequate calories. he was really happy she was drinking the formula. He is concerned that she has malabsorption. He had us do some tests to figure out if that is what is going on. He also mentioned if the tests confirm his suspicion he will want us to rule out a disorder that has malabsorption and neutropenia as its defining symptoms.

However, incredibly once we started the formula Maddy made HUGE strides in Physical Therapy. Like leaps and bounds. Total 180! If you look back at my last update she really had not accomplished any skills. Two weeks after starting formula, she is now rolling back to front and front to back. She is CRAWLING!!!! She went from not even being able to maintain on her hands and knees to crawling!!!! She is still not weight bearing, but we are so hopeful that the extra nutrition will continue to help her improve.

The day Mimi was flying in, Maddy woke up with a fever. Per our protocol, I called hematology. we had to head into the ER. We were so impressed with the way Cook's cared for Maddy and so thankful for the well organized protocols hem/onc has in place. We never even stepped foot in the waiting room (they had us enter a back entrance). They started an iv, drew labs, gave fluids and a dose of IV antibiotics and we were home 5 hours later (in time to pick up Mimi)! We followed up with hematology this week. They are both happy and unhappy with how she is doing. overall, she is doing great. However, they would have liked her counts to come up higher with a fever. Her neutrophils went from 20 to 800 which is a huge improvement, but they expect/hope for them to at least come over 1000 (1500 and higher is normal). Unfortunately, her counts were back to 30 on Thursday.  In light of this response and some other inconsistencies with her original diagnosis, they are trying to get a bone marrow biopsy added on to her MRI in January. This is not a small feat considering her hematologist is in Fort Worth and the MRI is taking place in Houston! Luckily, we have some good contacts/doctors in Houston who are trying to pull it together.

I think that is all for now. We are hoping for a calm, peaceful end to our year!
Ali

Incredible Month

What a busy crazy, fun month it has been!

We started off November celebrating Maddy's first birthday with her little friends and some of the important people in our life. We enjoyed showering our little girl with love and she had so much fun at her Kindermusik party!  Aunt Jenn-Jenn got to come and spend some time with the kids too which was fun. We also got some girl time shopping, getting pedicures and going out to dinner! As soon as Maddy's party was done my focus changed gears and we prepared for our first family vacation in a long time. Earlier this year I realized it was incredibly important to me that we have memories with the kids from vacations. We have not had a family vacation (that was not visiting extended family) since Alex's Make-a Wish trip. I set about planning a trip to Disney and Universal. Bruce can tell you, Alex and I were just a tab bit excited as it is all we talked about! On November 15th, we took off for a week in Orlando! I cannot begin to tell you how incredible this week was. Both kids had such an amazing time and Bruce and I did too! I had so many pictures (shocking, I know!) I couldn't decide which ones to post. I could go on for days about all the fun we had, but I'll just share pics instead!

Hogsmeade

 

Hogwarts Express

 

The Castle was GORGEOUS!

 

Hogwarts

The dragon on Gringott's Bank

Alex with the Wandkeeper at Ollivanders

Animal Kingdom

Animal Kingdom

Epcot

Magic Kingdom

Hollywood Studios

 

 

We cannot wait to go back! As you can imagine, we came home exhausted! I was glad to have a quiet Thanksgiving and a slow week to recoup!

 

 

I have had a few people ask where we are medically...

 

Alex- Overall, Alex continues to experience the most stable time of his life. We are still learning to manage his Adrenal Insufficiency. It can be dangerous (life threatening) and make him feel awful so we are learning how to adjust his medication in different situations to help him. Otherwise, we are keeping things pretty much status quo. There just is not much we can change at this point without major interventions that we are not willing to consider at this time. He amazes me daily with his strength and bravery. His life is so hard in so many ways, but he chooses to be joyful. He blesses everyone he meets with his huge personality and fun-loving spirit.

 

Maddy- Oh, my Maddy Moo. She is such a character. Anyone who has spent time with us will tell you she is not lacking in personality either. If it is possible to be a drama queen at 13 months, that would be my girl.  She is sweet and loves to cuddle, but she has a fiery temper and lets you know when she is not happy. She has started putting her hands on her head and throwing her head down on the ground when she is really upset! She packs a punch for such a tiny little thing. She is itty bitty at only 16 lbs, but she has enough personality for someone much bigger! We are still trying to figure her out medically. Her hematology counts are HORRIBLE. (Last check her neutrophils were 20 when they should be over 1500), but she is holding her own. Our biggest struggle right now is developmentally. She is severely delayed. She is still not able to roll belly to back, she can't crawl or even get on her hands and knees, she doesn't bear weight on her legs without assistance. For the last several months we have been doing physical therapy twice a week. I am trying not to look at the big picture, but instead focus on the progress she has made. We will be traveling to Houston in January to have an MRI of her brain to see if that gives any answers. In every other way she is developing right on track. She is so smart! She is starting to talk. She actually talks non-stop. But we understand about 10 words. ;) She is loving Christmas and especially the "pretty trees".

 

Alex and Maddy continue to be as close as possible. They would choose to be together every minute if they could. Alex is the MOST patient older brother. Maddy adores him and lights up when she sees him.

 

Thanks for reading, if you made it this far!

I wish everyone the happiest of Holiday Seasons!

Ali



Happy First Birthday!

Happy First Birthday Madelyn Paige!

 

 

525,600 minutes, 525,000 moments so dear.
525,600 minutes - how do you measure, measure a year?
In daylights, in sunsets, in midnights, in cups of coffee.
In inches, in miles, in laughter, in strife.
In 525,600 minutes - how do you measure a year in the life?
How about love? How about love? How
about love? Measure in love.
Seasons of love.

 

(From the Broadway Musical Rent)

 

How do you measure a year. If it is measured in love, then what a first year it has been. Our little Maddy Moo, our Smooshie has brought so much love into our house. I know everyone says you love your second child every little bit as much as your first, but I didn't think it was possible. Alex and I have had such a special, close relationship, I just wasn't sure another child could ever mean as much. But I fall more and more in love with this little princess every day. From the time Alex was about 3 or 4 he asked for a sibling. When I told him I was pregnant, (He was the first to know after Bruce) he was overjoyed. By far my favorite part of this year has been watching the incredible love between my babies. Maddy LOVES Alex and he is the most compassionate, caring, kind, nurturing big brother she could ever ask for. They look at each other with so much adoration. Alex told me this week that he needed to have a talk with Maddy on her birthday. He told her that she is the best thing that ever happened in his entire life.

The first time Alex held Maddy

 

 

 

 





 

To my precious Madelyn-

 

You have no idea how much joy you have brought me this year. I love every little thing about you. I love the way your face lights up when you see me. I love the way your hands shake with excitement. I love the way you ball your fists up and squeeze when you are mad. I love the way you look at your big brother. I love the way you call "Dia" so excitedly when she comes in the room. I love the way you smile when you see Daddy. I love the way you sign please and hold your hands up when you want me to hold you. I love the way you look down coyly when you see a stranger or someone aren't sure of. I love the way you scrunch up your nose when you are happy. When I chose the name Madelyn for you, I imagined a petite, sweet, dainty, well mannered girl. You are all those things and so much more. You are so sweet and so funny, but you have so much spunk. You are stubborn and let everyone know exactly what you want. And smart, you are so smart my baby girl. I love you, Madelyn Paige Beckwith. I am so lucky I get to be your mom.

Love,

Mommy


Long Overdue Medical Update

We just returned from a week of appointments in Houston. But many of my updates are weeks and/or months old! So I am going to just jump into the medical updates and then finish with another round of incredible pictures by our awesome photographer.

Alex: When Alex was in patient in April to do his fasting challenge, his endocrinologist ran a lab that has to checked between 7 and 8 am in the morning (thus making it difficult to test if not inpatient). It was low enough to warrant further testing. We did some testing and it was also abnormal, but before committing to treatment his doctor wanted to repeat the test one more time. At the end of July, we repeated this test for the second time. I got a call the day before we were leaving for my Mom's telling me that the results were very low and we had to start treatment that day. Alex was officially diagnosed with Adrenal Insufficiency. Basically, Alex's body is not producing any cortisol. Adrenal insufficiency can result in severe abdominal pains, vomiting,  muscle weakness and fatigue, low blood pressure, weight loss, kidney failure, changes in mood and personality, and shock. If you are in an accident, get really sick, have surgery, etc. and your body doesn't produce cortisol it can be really serious. Alex was started on a replacement dose of cortisol daily and I was given a crash course on raising the dose in certain circumstances and given an injectable form for emergencies. So far the medicine has been great. Alex feels good and he did better then ever on vacation this year! As for Houston...the doctors in Houston continue to be really happy with the care Alex is receiving locally at Cook Children's Hospital. We saw pulmonary first this week and while Alex's PFT's (Pulmonary Function tests) are still not normal, they are better. We are happy to see a bump in the right direction! The plan is to keep everything status quo. Next we saw cardiology. Alex has not been having any issues and his echo looked great! Tuesday we saw Dr. Koenig in the morning. We reviewed all of Alex's systems, talked some about his new diagnosis  and she didn't feel the need to make any changes at this point. In the afternoon, we saw Dr. Pacheco who ran a bunch of labs. Wednesday morning, we had an appointment with our endocrinologist who was happy to release us to the care of our local endo who has been so instrumental in the changes over the last year. It was nice to graduate from yet another Houston specialist. Thursday Alex had some testing and a thorough dental cleaning done under anesthesia. other then still trying to recover from the effects of anesthesia, he is doing great.

Maddy: I have avoided writing this update for a long time, because putting it in writing makes it feel "more real". Many of you probably don't even know that we have had any struggles with Maddy medically. In middy's early infancy, there were some things that seemed a little off, but everyone wanted to take a wait and see approach. We were able to do that until the end of May/beginning of June when Maddy was hospitalized for an infection. Just before she was discharged from the hospital the doctors came in and gave me a laundry list of instructions because her neutrophil count was "severely low". (Neutrophils are one of the kinds of white blood cells responsible for fighting bacterial and fungal infections). They let me know that this happens sometimes with infections and follow up with our pediatrician in 2 days and repeat lab work. Everyone's expectation is that it would be better by then. After repeating the lab work, I got a call from our pediatrician very concerned. her count had dropped farther to 147 (normal is over 1500). He let me know that he had called the hematology department and that they would be seeing Maddy the next day. At the hematologists office the count had dropped to 30 which is considered extremely critical. he ran a bunch of specialized lab work and we got the best results possible...a diagnosis of Autoimmune Neutropenia. Basically, her bone marrow makes neutrophils and then her body kills them off. Her counts have stayed at a critical level,  but she is doing really well. We have a strict protocol for if she spikes a fever and we have to be careful around people and vigilant about hand washing. But she has done really well overall. We continue to follow with hematology on a regular basis and will for some time. At Maddy's 9 month check-up, we discussed the fact that Maddy was not rolling either direction, was not attempting to crawl and would not bear weight on her legs. Dr. Benzick referred her for a PT evaluation. Starting on Monday, she will be seeing PT twice a week. The PT also noticed that she has bowing in her lower legs that seems more pronounced then normal. She referred us to an orthopedist to have that looked at. Dr. Koenig and Dr. Pacheco both examined her this week in Houston. Dr. Koenig does not want to jump to a diagnosis of Mito just because of Alex so she wants to look at her separate from our family history. She has ordered a brain MRI which is what she would do with any baby who came into neurology clinic at Maddy's age with significant gross motor delay. We will travel back to Houston when we can get the MRI scheduled and meet with Dr. Koenig again to go over the results and decide on the next step if necessary.

Please pray for my sweet babies and for my Mommy's heart as I so did not want to be in the "medical world" with my baby girl.

Now on to the fun stuff...Before heading to Houston we were blessed to once again attend family camp at Camp John Marc thanks to our awesome therapy facility. This is our second time at camp and it was just as magical as the first time. It is so much fun to have time as a family away from everything and enjoy some cool activities and lots of bonding time. We are so grateful to all the therapists who volunteer their time for camp, especially "our girls" from our clinic who let Alex stay glued to their hips all weekend. I can't wait to get some pictures uploaded from camp!

At the beginning of September we did a photo session with Heather at www.hparkerphotography.com. We have been working with her since Maddy's birth and she continues to amaze me with her talent. She is always able to "capture" the kids personalities in her photos. I can't share them all, because some are a surprise for Christmas, but here are a few of them!

If you made it this far, thanks for checking in and keeping us in prayer!
Ali

Vacation

Loving our time at the beach with family and friends! Every year we look forward to our visit with Mimi and PopPop. We love to visit NC and see family and friends that we don't get to see other times. We spend lots of time at the beach, swimming in the pool, playing miniature golf and creating memories. It has become such a treasured tradition for Alex and I. This year we have also been able to introduce Maddy to some firsts! One thing I have discovered is that (almost) 10 month olds and much more agreeable photographic subjects then 10 year olds!

I do have some medical updates, but I have chosen to wait till we are home from vacation before I share those.
Ali

Pertussis and Pictures

What a very long month this has been!

Alex started with a nagging cough on June 30th that quickly became pretty bad. By the end of the week he was wheezing and not sounding or looking good. He had no fever and no other symptoms so everyone's first thought was an asthma flare. We saw multiple doctors 6 times before finally being diagnosed with pertussis 10 days later! Alex is vaccinated, however because of his immune deficiency he doesn't hold the titers to his vaccines normally.  Unfortunately, many people now do not believe in vaccinating their children. Alex has paid the price for those choices. We are now almost a month into this ordeal and Alex is still struggling. he coughs to the point of turning red/blue. He cannot catch his breath. He has expressed a fear of dying. We are still doing breathing treatments around the clock. He is using twice his normal amount of Oxygen. He cannot go to TKD because he doesn't feel well enough. It is really sad. All of us were treated with antibiotics so we are not contagious, however, Alex's cough could linger for months.  we are praying he starts to feel better soon!

Miss Maddy Moo is doing well. We are so grateful she did not get the pertussis as I cannot imagine how scary it would be with a baby! Maddy had her 8 month pictures done on July 4th and they are precious! Heather at www.hparkerphotography.com has been so awesome to work with and she did a fabulous job capturing "Maddy"! My plan had been t get a few of the kiddos together, but because Alex wasn't feeling well, he didn't come. So here are Maddy's pics:

Hoping for a GREAT update next month!
Ali