Friday, December 28, 2012

9 years

My Dearest Abigail,

Its hard to believe that you are 9 years old today. I sometimes reminisce on your toddler years and feel like they were just yesterday. You were my little toe-headed, blue-eyed bundle of energy with an insatiable thirst for exploring the world around you. You walked early, talked early, and seemed to get stuck in every little place imaginable. I just chuckle when remembering the many times I had to pull and yank your stuck appendages out of chairs, trees, and any other small space you thought you could fit into. 

I am so thankful for the young lady you are becoming. You have such a God-honoring and joyous heart, and it makes me so happy to see you developing into such a beautiful person on the inside and outside. Your inner beauty and desire to please and honor God will direct your happiness and contentment as you grow older. 

My precious girl, your intelligence amazes me every day. You have always been so incredibly bright, but I have seen your knowledge take off at warp speed this year. I find it fascinating that you can remember so many facts and figures in that little head of yours. I have always said that your brain is indeed like a huge sponge, soaking up as much knowledge as you can at all times. Reading quite well for the past few years, you have found that you are actually enjoying reading this year. You often read for pleasure now instead of as a chore or homework. It pleases my heart to peek into your room and see you curled up with a book on your bed. 

But my favorite part of your attitude and personality is your compassion. You are perhaps the most compassionate child I know. You have a giving heart and are constantly trying to please those around you. Before blowing out the candles on your birthday cake, you closed your eyes for a couple of seconds and made a wish. When I inquired as to what you wished for, you sheepishly confessed that you wished that Nathan would not have mitochondrial disease anymore. Always thinking about others above yourself, your wish did not surprise me, but it did touch my heart. Your compassion is one that we all need to emulate. 

You are a loyal friend and trust-worthy in so many ways. You love unconditionally. You smile and laugh often. You are just such a joy to be around. And I love you with all my heart, my sweet Abby. Happy 9th Birthday, sweet pea. 

Sunday, December 16, 2012

Newtown, CT

I had sat down to eat lunch and check facebook during the first lull of that busy Friday morning when I first read of the devastating news of Sandy Hook Elementary School in Newtown, CT. Grief stricken and in a state of disbelief, I sat in front of the TV screen trying to comprehend the magnitude and heartache of what had happened...just like thousands of other Americans at that time.

I left early to pick the girls up from school and just sat in the parking lot. I was so anxious to put my arms around both of them and just have them under my watchful eye again. I couldn't help but imagine myself in the tragic situation that was happening in Connecticut. What if it was our elementary school? Our children? Our friends? Our teachers? What if that was our community?

People were already talking about trying to not expose your children to the horrific news of the day. "Protect their innocence" was one quote that I saw several times. I understand the thoughts behind such statements, as we do not wish to create fear in our children who are not familiar with the atrocities of our world. Nor do we want to unwillingly expose them to the heartache that death is not only reserved for the old and feeble.

Sadly, my girls are very familiar with the fact that some children do not live to adulthood. So many of our little friends afflicted with mitochondrial disease have passed away the past few years, and we all have learned to live with the silent daily reminder that the future of children born with this genetic disease is very uncertain. The poignant questions they have asked about their brother and our little friends who have died have provoked intensely emotional conversations that have bonded us together like nothing else and caused these two precious girls to mature in a way I had never imagined.

After picking my girls up from school Friday afternoon and hugging them for much longer than they ever anticipated, we sat down together as I explained what we knew had transpired that morning when we all were going about our normal schedules. I respect those who want to shelter their children from such tragedy, but in knowing the emotional level my girls can handle at this point in their lives, I wanted to be the one to tell them of the news of this act of utter and complete evil that had horribly broken the lives of so many families.

It was a precious but solemn time of communion with my 2 oldest children while explaining the unimaginable events of that day and answering any questions they had. I wanted to make sure they knew that we needed to be in constant prayer for the families who lost children, grandchildren, sisters, brothers, and wives during this terrible time...just like I stress to them the importance of praying for the families who have lost their children to mitochondrial disease when our little friends die. And just as I expected from past experiences of ending these difficult conversations with my precious girls, Abby departed to a place where she could spend some time to herself and think, and Emma stayed directly by my side so she could ask more questions to understand the situation better and be able to seek the comfort of touch she needed by being directly by my side.

Tucked tightly in my arms, Emma and I talked about this terrible situation a bit before she instantly sat up and locked her compassionate eyes with my own. With tear-rimmed eyes and trembling lips, she uttered something that will stay with me forever: "God can make good come out of this."

God can make good come out of this.

It was the emotional utterings of someone who was the exact same age as most of the children murdered on that horrifying day that spoke volumes to my soul...a 6 year old child with the spiritual maturity to minister to her own mother.

I trust that God is hearing the cries of an entire nation and holding these dear families up during this unfathomably heartbreaking time in their lives. I pray that he is holding each one of them up in his arms.  And I also pray that this disaster brings our families and our nation closer to Him. Come Quickly, Lord Jesus.

Friday, November 16, 2012

Dance Marathon at UF

26.2 hours. A symbolic marathon amount of time for dancers to be on their feet. 26.2 hours of students standing up for many kids who can not, in a prominent event that brings copious amounts of funds and awareness for Children's Miracle Network Hospitals. 26.2 hours for the kids at Shands Hospital for Children. 26.2 hours.

This is Dance Marathon at UF.
In 2012, DM at UF raised over $886,000 for Shands Hospital for Children at UF. That number and showing of dedication is staggering to me, and I look forward to witnessing the unveiling of the product of so much hard work from these University of Florida students when the 19th annual Dance Marathon at UF is held in April. It is always such an amazing fundraiser for Shands.
But to us, Dance Marathon is so much more.

Nate is a "miracle child" for DM. We honestly didn't have a clue what we were getting into when agreeing to join the Dance Marathon miracle families, but I for sure would have never imagined the degree of love we would have for these amazing people who are now a part of our heart. I have read several testimonies about how students' lives were changed by being around the DM miracle kids, but I don't think they can fathom how their lives have also changed our own.
The amount of care and devotion these ambitious and loving students show to our children is simply awe-inspiring. To put into words the emotions we go through while watching these students communicate, play, and pretty much spoil all 3 of our kids is seemingly impossible. To witness several of them trucking off with Nate, backpack in tow and completely uninhibited by his differences, and treating him like a very normal "rough and tough" little boy is unlike anything I can describe. But my favorite thing is watching the growing bond of friendship and love all 3 of the kids have with the DM at UF crew. Yes indeed, every opportunity we have to share with these amazing individuals is a life-changing experience for all of us.
Our family captain is like family to us...kinda literally. She was the CMN intern during radiothon who Nate became instantly close to, and he suddenly proposed to her on the day of radiothon. He introduces her to everyone as his "wife". In fact, he was telling his physical therapist this morning that he had a date with his wife this evening. Insisting on buying his wife a ring, he settled on a red ring pop and popped the question once more with ring in tow. It's pretty special seeing how much he adores Bailey.
We are so thankful to be able to participate in such a wonderful organization that brings so much care and help to our local children's hospital. But more than anything, we are thankful for having met so many incredible people with Dance Marathon at UF. Words don't seem adequate enough to express the impact they have had on our lives.

Friday, October 26, 2012

Houston: Fall 2012

Nate and I just returned from our bi-yearly trip to Houston, TX to see the docs in our mito clinic. This was our 7th trip there since Nathan's diagnosis, and it has most definitely become a home away from home.

Its amazing to remember the first few trips we made there and see how much the little guy has grown since then. Now, Nate really looks forward to these trips since he gets to see some of his best friends when he gets who are just like him, who have most of the same attachments, understand the language of medical "stuff", and truly feel the same apprehension, fear and pain for some of the same routine tests they all have to do on a regular basis.
These trips are always made so much easier by the people we get to see while we are in Texas. Nate had a blast playing with his friends, and I enjoyed catching up with my friends and meeting some new ones as well. Alex kept calling Nate his "younger brother"...those two were pretty inseparable all week. Thanks to our dear friends with Dance Marathon at UF, Nate and Alex got a phone call from a very cool Disney character when we were soon as the large video file uploads, I am going to share it.
The degree and amount of research the docs associated with the mito clinic are doing is simply incredible and exciting for the future of knowledge and treatment of these disorders. I am constantly in awe of the dedication these doctors have of trying to understand the "whys" to the complexities of this disease.
Anyway, Nate had a sleep study on Saturday night. This would be his 4th sleep study, and he is most definitely a pro at getting hooked up and ready for this test. He still hates to have 2 cannulas in his nose at the same time and the end tidal probe attached to one, but I really don't blame him on that one. That has to be terribly uncomfortable. We should get the results within another week or two, but I honestly don't expect any big changes.
Monday, we saw Cardiology, Pulmonology and GI. It was a very long day at the med center! His cardiologist is planning on increasing his dysautonomia med (florinef) in the spring to help combat with the hot, humid summers we have here. And he said that he really needs a significant increase in his fluid intake. Florinef works by increasing the blood volume and blood pressure to lessen the effects of autonomic dysfunction. In order for it to work optimally, the patient must have adequate fluid intake, and we have recently realized that Nathan does not take in enough fluids.
Pulmonology was good. She said we gave Nate a good run at trialing him off O2 for small prolonged times this summer, and it is clearly obvious that he does indeed still need oxygen supplementation. She thinks that his heart rate rising quite a bit when he is off O2 may be another reason why his lactic acid rises off O2. When asking about why he sometimes does okay and sometimes doesn't after he has been without oxygen supplementation for a longish period, his mitochondrial doctor said that it seems to depend on his ph. She said that we know his lactic acid rises when he is off O2...sometimes he compensates perfectly by making his ph alkalotic respiratory-wise to combat against the metabolic acidosis. But for whatever reason, his ph was significantly lower last time he had a blood gas. Lower than we have ever seen it, along with other labs that were more off than they usually are. She said what is happening now is that his lactic acid rises, and once his ph gets acidotic as well, he starts vomiting to help bring the ph back to a normal range. Its amazing how the body compensates. The key to helping him out of these episodes is fluid, fluid, fluid.
Anyway, GI appt was great. He was so interested in Nate's stimulator and progress since getting it placed. GI there doesn't have much to do with Nate's care, but his mito doc wanted us to continue to follow up with them periodically when we saw her last time.
Our appt with our head mito doc was great. She wants him to have a referral to endocrinology to monitor the periodic low blood sugars he has, and he needs to see his ophthalmologist again since it has been 2 years since we have seen her. She gave her blessing for him to start school in the fall, and they are forming together a letter to help direct everyone in how to hopefully make his school experience successful from a medical front. We'll start his IEP process in a few months to help get everyone ready for him next year, as he will be the most medically complex kid they have ever had there.
Anyway, Dr. K also ordered the Whole Exome Sequencing test for Nate to try to pin down the genetics behind his disease. This is a newer test and is able to look at more genes than any other test. It is entirely possible that there is not just one gene involved in Nate's disease, but a few mutations that together form the metabolic insufficiency in his body that is creating his mitochondrial disease. I am really anxious to get these results...and really hoping insurance will cover it so we can get these results!
I explained to his mito doc that in the 4-1/2 years we have been working with our team at Shands, I am extremely confident in their ability and knowledge of how to take care of Nate now. They really do understand how this disease affects him and how to treat each organ in his body. They are really doing an amazing job of taking care of Nate. So explaining that to her, and in conjunction with how well he is doing right now, I was able to convince her to allow us to come back in a year instead of our regular 6 month plan. That being said, if insurance does indeed cover the Whole Exome Sequencing, we'll come back in April to get the results and see everyone else then as well.
Lastly, we had an appt with our beloved mito immunologist. I simply love this woman! Out of all of our numerous docs, she is right up there at the top of my favorites. She and I have always just clicked extremely well, and I love getting to catch up with her every few months about family life, travels, and what not. While I was excited about Nate's IgG being mighty close to 700, she wasn't quite so pleased that his labs haven't responded better to the SubQ. She said that she wants him to be much higher than that. We ran labs again, and if his IgG hasn't increased substantially, she wants to increase his Hizentra infusion dose. She also said that she is writing up the data on her mito kids with immune deficiency, so that is going to be a good addition to the research of this disease.
A full (and long) PFT test in the hospital rounded out our trip before heading home. He was one of the youngest to complete the test, and I was so proud of him. It required a lot of different breathing techniques, but he listened well and did awesome. It amazes me what all he goes through without batting an eyelash most of the time. It was a good trip...exhausting, to say the least, but good.

Wednesday, October 10, 2012

A mother's understanding

I could barely control the shaking in my arms from the muscle fatigue of helping hold down a strong little 2 year old guy who holds a special place in my heart. It was the team's second attempt at trying to get an arterial blood gas needed to determine how this sweet boy's breathing difficulties were affecting his body.

The needle snaked in and out of the corner of his wrist, searching diligently for the pulsing artery, as the exhausted little guy hoarsely cried "Owie! Owie!" and tried to wrangle out of what must have seemed like torture to him. It was heart wrenching to witness, as we tried so desperately to hold him down and keep him as still as possible.

It was during this time that his dear mother rested her head against her son's body and closed her eyes. From experience, I could tell that she was silently praying...begging God for mercy and grace on behalf of her son. It took everything in me to swallow back the ball of emotion building in my throat, for even though I did not hear this mother's prayer, I knew what she was saying.

Several painful memories flooded my mind of times when I was doing the exact thing...during my own son's arterial blood gases, numerous failures of trying to get an IV started, times when he was in so much pain that all he could do was shake uncontrollably. It was during these times that I would silently beg God..."Take this pain away from him. Direct their hands to find the right spot. Be with my son. Hold him close to you. Please let all of this be over soon."

There are times when you feel so helpless. As a mother, we are hard-wired to want to fix it when our children hurt. So having to stand by and watch your child endure intense pain that you can not immediately resolve is one of the hardest things we must do.

Keep Nate's little mito friend, Sammy, in your prayers. He's a precious soul who has touched my heart over and over again. While doing very well lately, he has had a recent set back and is in the hospital for a bit. His mom is one of the kindest and strongest people I know, and I am so happy to call her a friend.

Thursday, September 6, 2012

Hustle and Bustle

Most afternoons, after homework and chores are completed, the girls spend the last few hours before dinner riding their bikes up and down the neighborhood streets with friends, playing pretend games, and just enjoying being around kids their age. The whole neighborhood posse is about 8 kids strong, and it is so much fun watching them laugh away their afternoons without a care in the world. Reminds me so much of the hours I would spend on my bike as a kid with neighboring playmates.

The start of school has been a wonderful one for the girls. They both have amazing teachers and great classes. Emma was really happy to have tested into the gifted program here like her big sister. I have to be honest in saying that we are not fully aware of what Emma is capable of...we didn't know how fast she was until she won 1st place in the kindy Talbot trot last year, and we weren't completely aware of how smart she was until she passed the testing for the gifted program. Crazy kid!

Both girls have started soccer for the fall season, and it is so much fun to see both of them on the field. I'm proud of the girls for running with me several times this summer, even when they were hesitant at the unwanted exercise at first. At the end of the summer, they logged in their longest distance of running/walking: 4.1 miles! I was super impressed in how much they had improved over the summer in their distance. Seeing them both run up and down the soccer field with a lot more ease and endurance is a good pay back for those days we ran together.

Nate has been busy with CMN activities for radiothon. It sure has been a lot of fun coming up with different ways to raise money! He has had a fantastic summer of incredible much that we have been a little more lenient on his resting and continuous oxygen use. We thought his body was doing an amazing job of compensating, so we were letting him off his O2 for a couple of hours at a time with no obvious signs of long term damage. Well, that was until this weekend...

We had a blast swimming, playing, and jumping on the trampoline at a friend's house Friday evening. Sat morning, he woke up puny and vomiting, After getting sick a few times and not being able to hold anything down even after zofran, I knew that he was more than likely acidotic and took him to the ER. Turns out he was acidotic like I had expected and dehydrated: high lactate, low bicarb and CO2, low glucose, high BUN, ketones, base excess was -7. The theory was that the energy expenditure overload the night before coupled with the excessive time off O2 caused his lactate to rise and his acid/base balance to shift and probably caused him to be a bit dehydrated. That, in return, caused him to get extremely nauseous, like it always does. And that caused him to vomit and just make matters worse. It just seemed to be the perfect storm of stress on his body to keep him from being able to compensate for it all.

It took several hours of running D10 at 1-1/2 maintenance plus an additional fluid bolus to help the little guy start to perk up that evening. And by the next morning, he was back to normal. Usually, he only decompensates surrounding illness, but the last two times has left us scratching our heads at what triggers these more recent episodes of acidosis. A friend reminded me today of the words of our dear friend Samuel Knight: He is just "a delicate metabolic balance". I still can't say that without hearing Samuel's voice in my head uttering the phrase coined for his life. Makes me smile and miss him all at the same time.

These times cause us to step back and just watch him a little bit more...let him continue to live life as normally as he safely can, but watch him a bit more closely. Seems that the moment we get too comfortable in his well-being, his body likes to give us a wake up call. Thankfully, he is back to doing great now, and enjoying the hustle and bustle of what we currently call "our family life".
(I have TONS of pictures on my camera...if only I'll take the time to put them on the computer!!)

Thursday, August 23, 2012

CMN Radiothon

Please help us reach our fundraising goal for Children's Miracle Network Radiothon!

Thursday, July 26, 2012

Team Natester

I hated fundraising in school. Putting people on the spot by asking them for monetary support in exchange for horribly expensive wrapping paper or boxes of chocolates or other insignificant items seemed absurd and annoying, and I simply reached the point where I just wouldn't even do it. But every once in a while, I was tempted and pulled in with some pretty cool prizes...a glow in the dark frisbie, a water bottle, and even 30 seconds in a money box where you could try to catch as many $1 bills that blew around. Those incentives would sometimes entice me enough to knock on neighbor's doors and corner church members with fundraising equipment in hand.
Fast forward 20 or so years, and those early introductions into fundraising are continuing at a different level. The "prize" for our efforts this time around are unlike anything I could have ever imagined. They are for the betterment of my son's life and the lives of many of his friends who grace the halls of Shands Children's Hospital at the University of Florida. Fundraising for CMN has a direct impact on Nathan's life in countless ways.

As a new ambassador for the Children's Miracle Network, Nathan has been asked to be a captain for their annual radiothon. With technology constantly changing the world, CMN has decided to change up how they do the radiothon this year, creating a 6 week online campaign to help drum up support via social media. On September 18th, we will do the actual radiothon for a few hours, but the heart of the donations will be through online giving.
What we currently need are people who are willing to join Team Natester and help us drum up support through social media (emails, facebook, blogs, etc) over the next few weeks...just a mention here and there about Children's Miracle Network and/or Nate while adding the link to our team page. We are hopeful that between quite a few of us, we can gather support from others in the form of donations to help make a difference. Every last little bit earned in this fundraiser will go directly to Shands Children's Hospital at the University of Florida.

So please consider joining Team Natester and helping us by periodically petitioning your friends and family to help donate to the Children's Miracle Network.

Important Links:
Team Natester
To join Team Natester, just click on the blue words "Join our Team".
You will then have your own page that you can send the link to your friends and family in trying to gain monetary support. The actual start date of the fundraiser is August 1st and ends September 18th, but all of the teams are technically starting now that we have had our first official meeting.

This shows all of the groups and teams. We are in the Red Group. Each group consists of a CMN ambassador (Nate's role), an on-air radio host, a pediatric department (we are fortunate to have the Peds ER Dept), 2 pediatric doctors (we have 2 ER docs that we love dearly), and a corporate partner (American Integrity Insurance Group is our support company).

 And thank you dearly.

Saturday, July 21, 2012


For the past 23 years, my father has granted me an eccentric "Happy Anniversary" on every single February 2nd...groundhog's day. He has created a bit of confusion offering these salutations on my facebook wall the past couple of years, causing many to wonder if it was my wedding anniversary or the like. The reason for this greeting he has offered on that day for over 2 decades: February 2, 1988 was the day of my tonsillectomy. To continue this unconventional tradition (because the apple doesn't fall too far from the quirky tree), July 20th will forever be known as Emma's tonsilversary.
Emma has been looking forward to this day for months...complete with a countdown until her big day. I'm sure any psychologist would have fun with that interpretation. She did enjoy the undivided attention yesterday. The anesthesiologist was amazing...quite an old guy who had "pretty much already retired", according to his own words, but still worked here and there. He was a gem. She said that she laid against the sweet anesthesiologist while he stroked her arm until she was asleep.

The surgery went perfectly. Her ENT said that her adenoids were huge and nasty. She sure did need this surgery, and we hope that this will help her feel much better. She woke up perfectly, ate a popsicle as soon as she was reunited with us and sat wide awake and happy until we were ready to head home. With my only experience with kids and anesthesia being Nate, I was nothing but shocked at how well everything went. I was literally amazed at how well and quickly she woke and immediately wanted to eat. It was such a far cry difference than any experience we have had with Nate, whose body does not seem to like anesthesia at all.
Em has continued to amaze us once being home. She is doing fabulous. Obviously, her throat hurts, but she is doing so great despite it all. The biggest problem has been that she has so much drainage now that is making her cough and sneeze, and neither of those are comfortable at all on a really raw throat. But she continues to show us that she is made from some pretty sturdy stuff with how much of a trooper she has been through all of this. This kid continues to surprise us. 

Thursday, July 19, 2012

Crossing paths

I have shared many times in the course of this journey how amazed I am at how God has placed countless others in our lives the past 4 years that have played an integral roll in our family and found a special place in our hearts. I wish things were different for Nate...I really do. But seeing all of the positive aspects encircling his and our lives as a direct result of the trials he has faced has given us a true thankfulness for the aspects of these trials. This is the grace that God has given us all to continue through all of this joyfully. If we would have never gone through the aspects of this difficult journey, we would have missed out on some incredible blessings.
One of these blessings took the form of a beautiful, witty woman and her 3 precious blonde-headed boys. Beth and I first made contact a little over 2 years ago due to that fact that we had quite a bit in common. She was a photographer whose work I had admired and studied, and she had a son who was just a few months older than Nate who was battling his own medical struggles: leukemia. I found it even more incredible that after writing back and forth a bit, we found out that we lived in the same city and our boys were frequent fixtures at the same children's hospital, sometimes at the same time but in different departments. Talks of "meeting" were put on the backburner while we both fought the front lines of the medical world head on for our sons.
On one of the first days of school last year, I recognized an adorable little toe-headed boy walking with his class at the end of the day. From the pictures I had come to admire, I immediately remembered that he was her oldest son. And just a few weeks later, Beth and I "met" for the first time at pick-up. Her family had moved over the summer and her oldest was now attending school with my girls.
Throughout the school year, we would talk for the few minutes we waited at pick-up...sharing the news and sympathy of what our boys were experiencing, along with different everyday stuff. Nate and Ezra, who shared so much in common and didn't really know it, chased each other around the same tree day in and day out. With Nathan not having much experience with kids his own age, Ezra was quite instrumental in silently teaching him how to interact. Nate has a tendency to scare any kid his age with his unusual appearance coupled with his social awkwardness in learning how to be friends, but Ezra soon became accustomed to his oddities and the two created a bond over Mario Brothers, Angry Birds and whatever else they found entertaining while chasing each other around that big oak tree.
Through our encounters with each other, Beth and I experienced and underlying unspoken bond. A bond created from fear and stress and sleepless nights...from concern for the future, lonely walks through hospital walls, and difficult talks with medical personnel...from heartache and exhaustion and intense hope. The two of us have always carried that unspoken, unbroken bond shared by mothers of ill children...its a friendship that can not be replicated in the "real world" and one that most don't understand. But those of us that carry it understand that feeling to its core.
After an incredibly courageous 3+ year battle with leukemia, Ezra's last dose of chemo through his port was today. He will continue to take chemo by mouth for a while longer, but this ending of intravenous chemo means that he will be able to have his port removed next week by a surgeon both our boys share. And shortly thereafter, this precious family will move to Minnesota, where they will embark on a new chapter in their journey closer to their immediate family. We will miss them all greatly.
At the beginning of the summer, Beth (who I believe is one of the most talented photographers in this area) so graciously took pictures of our family through the organization Inspiration through Art, an organization that gives pictures to families with children who are battling life threatening diseases. Since I always take pictures of our kids, we do not have any family pictures. Beth changed that, and the gift of family portraits that she has left with us is one I could never repay. They are simply priceless. Memories of our family love captured in a split second by such a talented photographer...moments that we will all cherish forever. Irreplaceable memories caught on film...absolutely priceless.
Its continually amazes me to think of the relationships we have been blessed to create through this journey. We will miss the Mancuso family.

Sunday, July 15, 2012

After a long hiatus...

After a month of craziness and a huge hiatus from writing here, everything seems to have settled down at least a little bit. We were gone for the last bit of June and first of July, totaling about 3 weeks. While the trips were wonderful and much needed, it is so good to be home and settling back into the lull of summertime around here. I hope to add photos from our trips to Marco Island and Kentucky throughout the next few weeks.
The kids had a blast on our long adventure. I can't get over how much easier it is to travel with them the older they get, and how they can adapt to later bedtimes and crazy schedules with much more ease. I also can't believe that school starts in a month where Abby and Emma will be in 3rd and 1st grade. We hope to make the most out of the rest of the free time we have this summer...
...starting with Emma getting her tonsils and adenoids removed this coming Friday. She is a bit excited about the popsicles, ice cream, pudding, and added attention she is going to get (she has a count down every day until surgery day) and just a wee bit nervous about the actual procedure. Her best friend's dad is her ENT, so she is a lot more at ease with "Mr. Garrett" taking good care of her. And even though we are well versed and strangely more comfortable with medical procedures, it is quite comforting knowing that she will be under the care of one of our friends. And I am really looking forward to seeing how this surgery will impact her positively afterward...she really needs these huge suckas out!
After having a rather rough month of May, Nafey boy has been doing outstanding the past few weeks. It has truly been incredible to see the transformation in him once again with better eating, better energy, and pure happiness. His surgeon increased a couple of the settings on his stimulator the end of May because he was once again vomiting, nauseous and bloating pretty badly and was getting most of his calories through his gtube once again because of his lack of eating. The changes in his stim settings really helped him through the rough patches of his body trying to bounce back from being pretty sick, and I think it has definitely played a huge role in how well he has done through the craziness of this summer. He's consistently eating better now than he has in a really long time, and that along with still supplementing with formula at night has helped him gain some much needed weight and height.
Nate had an immunology appt in June that brought about some more changes for the little guy. He was diagnosed with hypogammaglobulinemia (low IgG) when he was just a few months old, and IVIG therapy has been mentioned at nearly every appt with our immunologist here and in Houston as a probable treatment at some point. We have been hesitant about it until just recently for several reasons. But looking at the trend of his IgG, along with his t- and b-cell subsets on his lymphocyte panel, he may have a common variable immune deficiency (CVID) now instead of just hypogam. Additionally, he had blood work sent to a lab in Cincinnati that tests for certain types of lymphoproliferative disorders, and this can help us determine if he has a true increase in double negative t-cells like his labs may be pointing toward.
So, Nathan's immune system is struggling, and his immunologist here has said that she believes that he will benefit from IVIG. In fact, she said that due to the detrimental effect illness can have immediately and in the long run for patients with mito, she would rather most of her mito kids partake in this therapy. From what we have been told, it takes a lot of energy to create properly functioning immune cells, so immune deficiency is often seen in mito disorders. We have finally "come around" to the idea of IVIG for a few reasons...his immune system is not improving at all, his organ function seems to decrease at some level each time he gets sick, we want to preserve the function of his ICC cells in his stomach so that his stimulator can continue to work well, we want to keep open the idea of him attending school, and IVIG is now offered as subcutaneous infusions that can be given at home.

Nate's first infusion will be within the next few days. He will be getting 2g of Hizentra each week and will see his immunologist every 4 months to keep track of his immune levels. A nurse will come to the house for the infusions for the first couple of weeks, and then I'll take over the care from there. He should have 2 subcutaneous needles inserted, and the infusion will more than likely last just an hour and a half. Our hope is that this treatment is the catalyst to keep him well, improves his wacky immune labs (and get his IgG to stay above 500!), and helps him continue to improve overall.
And finally on the changes with the little guy, we were approached a few months ago by our local Children's Miracle Network asking if we would consider Nathan being a local ambassador for CMN and Shands Childrens. Of course we were honored. The ball quickly got rolling with interviews and fundraising opportunities. We are participating in an online/radio fundraiser for about 6 weeks coming up. Team Natester will need participants on our team to help drum up support via online social media, etc for this fundraiser that gives directly to Shands Childrens. I will include more information once we have the initial captain's meeting next week. This is definitely going to be a fun adventure for our family, and we are so happy to be a part of such an amazing organization!
Okay, more pics will follow in the coming days. Just wanted to jot down some of the changes over the past few weeks for us all. I look forward to writing more on here again.

Sunday, June 10, 2012

Jolee's beach birthday party

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