An Update:

Once again, here I am posting an update that is very long overdue. We have been so unbelievably busy. Jonathan and I have just decided that is our new norm for now. We have a teenager who is looking to graduate high school in Dec. and possibly transfer to a university in Jan., a very active 8 yr old who is too busy growing, playing, and having fun to slow down and relax, and our sweet boy who is almost five and counting the days until he can go to school! We joke with each other that when we are too old to remember our own names, we will have the time to enjoy each other's company. Maybe someday... :-)
     So, on my last post, I explained that Lydon was having some increased GI symptoms. We went for the gastric emptying scan and as we expected Lydon was not able to consume the amount of food required for the four hour test. We were all a little disappointed because the four test is the most accurate test and can generally detect even mildly delayed gastric emptying. One thing we've learned in the last four years is to just roll with the punches. So Lydon consumed what was enough food for the 90 min test and was amazingly brave as always. He was taped and strapped to the scanner table and was so anxious because the room looked like a surgical suite to him but he relaxed and did a fantastic job of staying calm and not vomiting. We got the results back around the end of Feb that showed moderately delayed gastric emptying. His stomach takes about twice as long to empty compared to normal emptying times. Can you imagine what it must feel like to eat and feel the same fullness in your stomach hours later? That's what Lydon feels nearly everyday. We have seen that evidenced. He is eating less, less frequently and complains of a lot of stomach pain. After we received the results, we discussed them with our motility specialist. We decided to treat Lydon for bacterial overgrowth of his intestines and also try Erythromycin which is commonly used to increase gastric emptying. We are rotating the use of these two antibiotics and monitoring him to see if there is any improvement. At this point, I do think it has helped some with his stomach pain but we haven't really seen a difference with his eating. Most of the time lately, he is too full from what solids he has consumed throughout the day that we have to tube his formula. We will be following up with our doc again soon and discuss the plan moving forward. I am content with keeping things as they are for now as long as Lydon's weight is stable and his other GI symptoms do not get any worse.
     Pulmonary wise Ly is ok. He definitely needs his oxygen when he is active, sleeping or extra tired. We've been able to catch him with the pulse ox during or immediately after a couple of activities lately and he was somewhere between 88-92%.
     We saw the eye doctor back in Feb.as well. We had been noticing his left eye seemed to be turning inward. After his eye exam, the ophthalmologist told us that his eye is turning in and he is more significantly far sighted than is normal. So he picked out bright bright blue glasses! Our handsome boy is even smarter looking now! We followed up with the eye doc last week and his vision is even worse in that eye. So, we are now having to patch his right eye for two hours everyday in hopes of restoring the vision in his left eye. :(
     Our other concern for Lydon as always has been his endurance, fatigue, and frequent colds in relation to his hypogammaglobinunemia or low immune system.  We've been able to keep these symptoms well controlled with our daily use of meds, supplements, fluids and oxygen. However, that is much harder to do with him being exposed to the public school environment. Especially after his frequent colds and hospitalization earlier this year. So, after much discussion, Jonathan and I decided to add another doctor to our list of specialists and consult with immunology. Our appt was very positive. We are all on the same page of trying to achieve the highest quality of life possible in allowing Ly to go to school. We are waiting on the blood work to come back before any decisions are made regarding treatment. We did start him on an inhaled steroid.
     We fought through some challenging issues with our insurance and formulary coverage for Lydon's formula. It was an absolute nightmare but for the moment, that is all worked out. We are working on Lydons increased sensory issues with noise, textures and smells that trend to greatly effect his GI symptoms. We have an amazing team that does an incredible job of daily management for an even more amazing little boy. His strength is inspiring. He keeps going when he is literally blue and vomiting. Lydon strong...we will never stop fighting for him. Our next big challenge... Pearland ISD and Kindergarten!! Stay tuned... :)

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Say a little prayer

     Over the past 18+ months, our life has been incredibly busy and I have not had the time that I would've liked to have kept this blog updated. Hopefully that will change but I wanted to give a quick update about an upcoming test we have scheduled. 

     During the past year, Lydon has continued to have GI issues although they have seemed to worsen over the last couple of months. His appetite has decreased and he frequently vomits; sometimes several times a day. We have previously tried every gastric emptying med available but Lydon has not responded to any of them. There is one medication that we haven't ever tried but it does not come without risks and side effects. Our GI specialist who is not only one of the best but one of the only pediatric  motility specialists in our area feels that this medication would help Lydon but bc of the risks associated with it, we must undergo a gastric emptying scan that proves that Lydon has poor gastric emptying or gastroparesis. Because Lydon is older now and is able to consume solid food, this test takes four hours to complete. So on Wed Morning, Lydon will have to consume an egg and two pieces of toast (which will be quite a feat if not impossible by itself) and then be strapped to the exam table while his stomach is scanned for four hours! :( We are asking our friends to pray that A.) Lydon can remain calm and not be upset or traumatized by the exam and B.) that this test will give us the information we need for this new medication.  We know without a doubt Lydon has poor gastric emptying, we see the repeated vomiting and comfort him when he cries with stomach pain from eating; but for this medication, we need the results of this test to prove it. Some days are better than others... We are just hoping this is one of his more typically "poor" GI days so that we can get the proof that we need.He deserves anything that can or will help him GI wise. He doesn't deserve to hurt. So if you will, add his name and ours to your prayer list tonight and let's bind together... Lydon Strong!! 

What I want my children to know

Those of you that know me very well know that for the past 15 months, I have been taking care of my Grandmother during the day while my Mother is at work.  She is 91 yrs old and has end stage Parkinson's and Dementia/Alzheimer's.  Her health has declined significantly over the last year but we have seen a rapid increase in her declining health over the last 2-3 months.  I am struggling very much with her overall ailment and extreme physical, mental, and emotional pain and torment she has been in lately. In walking the journey we have with Lydon, Jonathan and I have had very real conversations about death and dying and what our wishes would be for ourselves as individuals and for any of our children should we ever be faced with that scenario. But seeing my Grandmother like this... Seeing my Mother struggle with her own physical and emotional pain in making decisions for my Grandmothers care has been so very difficult for me.  This has made me do some serious inflection and soul searching.  So this post today is mostly to my three beautiful babies but the rest of you who are interested can read along. It may not make a lot of sense to any of you but I hope someday it will to my children...

Hollie, Aidan and Lydon,

Momma wants to share with you some things that I hope you will hold dear and never forget.  I get really busy sometimes and often feel like I forget to tell you or teach you some very important things.  So if by chance you ever get the opportunity to read this, here is what I would like you to know. 
     First and foremost, please know that I love each of you so dearly and there is nothing that could ever replace or change that....Nothing!  I hope that you always know that you can come and talk to me and that even if you've made a mistake and  I don't agree with what you are doing or have done, that I will "be there" for you and do my best to support you in whatever way I can. And in speaking of mistakes...be careful not to judge others for what they have done.  Even if their mistake seems like something stupid or ignorant to you, remember that not everyone has walked the same path or had the same experiences you have.  Remember to be compassionate of other peoples situations and to be thankful for your own but never let that hold you back from working hard to do better.  When you do achieve "better" be careful not to think that makes you "better" than anyone else and that you are not above making a mistake. Remember that everyone has a story and that you can learn something from almost anybody you meet. The garbage man, the janitor at school, the Principal, the President....People are people. Regardless of race, age, education or achievement.  Some of life's greatest lessons cannot be taught...,.they are learned. Learned, from people just like you and me. So don't ever be afraid to share with somebody else something you have learned. When pursuing higher education, whatever route you decide, just please do something that you enjoy doing. Do something that makes you feel appreciated and something that at the end of the day makes you proud of who you are and what you've done. And know that if you do not feel appreciated or proud of what you are doing then you probably should look at other options.   Always be respectful... Hold on to tradition and those things from your past that are precious to you and have made you who you are but never be afraid to try something new.  Change is constant and it is the only thing that is guaranteed!  Stand up for what you believe in and stand strong. Sometimes you may be the only one standing but that may just mean that you are the leader of the pack. So stand strong and hold your head high!!  Stay close to your siblings...Family is forever!
     Hollie if you do indeed become the NICU nurse that you say you will (and I have no doubt that you can) don't be afraid to touch that new Mommys shoulder and reassure her that she can do this. Remind her that God picked her for this special baby. You smile and tell her and show her a thousand times that she can run this machine or that. Be careful not to get too confident and remember its ok to cry and remain sensitive to the reality of life and death.
     Aidan, my boy... Never never never lose your fun loving personality.  Remember that people are drawn to personalities such as yours and that comes with a big responsibility.  If your gonna be a leader, be a good one and lead people in the right direction. Show people that you can friends with anybody and that winning isn't everything.  Show them that its just as important to be a team player and sometimes you should let your most novice player be the star of your team. Aidan, pay attention and watch your Daddy for he can and will teach you how to be the husband that you should be someday... The kind that loves his wife with his whole heart,  puts her first and fiercely protects her. The kind that doesn't define his marriage by the traditional man or woman's roles but views it as a partnership and does what it takes to get it all done.
     Lydon, my angel baby... I want you to know that no matter how tired or weak you may feel somedays, you are so incredibly strong! I want you to know that I would take away every ache, pain, poke and prod that you've endured.  I hope that you never question the decisions that Daddy and I have made for you but if you do, please know that we have done our best with the knowledge that we've had.  Know that your adorable smile and zest for life exudes love and energy to all of those around you.  Never underestimate the power of that, Super Boy!  I know that there is probably tons that I can still teach you but truth be told, you've taught me more than you'll ever know. 
     Most importantly, my sweethearts, please find a time and a place that you dig your heels in deep and develop your own relationship with God. Something that is sacred and truly your own.... aside from us your parents.  I cant promise you a whole lot but I promise that there will be a time in your life when your friends and possibly even your family will disappoint you but your Faith will carry your through. 
     Finally, please know how much I appreciate each of you and these incredible life lessons that I have learned from raising you. I want for you to know and learn all of these things that I've written about but the irony is that it was you who taught me how to love unconditionally, how to squash my inclination to judge, how to listen and how to teach, and how to stand up for what I believe in. I know that you will do many many things in your lifetime that will make me so proud, but don't forget to pass these things along to your own children.

Love Always,

 Momma

Happy Fall Ya'll

     Once again, it seems like it's been forever since I updated.  I keep thinking that after this event or the next that things will calm down this year but so far we've completed nearly 75% of this year and it hasnt happened yet! Needless to say, its but an extremely busy year!!  So much going on in the Paxton household.  We had an incredible summer.  Lots of fun and resting. Both of the big kids got to go enjoy several different things with their cousins. Lydon was able to go swim a couple of different times with the younger cousins too. He enjoyed a fun filled beach day at Moody Gardens with his "girlfriend".
     August rolled around and we got the big kids off to yet another school year.  Aidan is in second grade.  He has an "more seasoned" teacher this year who isnt as appreciative of Aidans full spirited personality but I am so proud of how well he has adjusted.  He is learning how to adapt to different personality types... A lifelong lesson that could benefit some adults I know. ;)  Hollie is a junior!  Oh my sweet girl...she has a load to carry this year.  She is working on the dual credit/dual degree program and it is TOUGH!!  So tough in fact that we can't seem to find time to pull her away long enough to get her drive time hours completed! Somehow, someway, she figures out how to get it all done and passes with flying colors.  She says she is going AWAY to college  (Sniff sniff! Guess shes had enough of these little boys at home.) and plans to become a NICU nurse. We will all miss her deeply but are so proud of her.  I guess this will take precedent for now but somehow we gotta get this girl driving ASAP!!
     In September, Lydon started a part-time Pre-K program.  We are so happy for this program and the people in charge who were willing to take a chance and let our beautiful boy particpate and be a part of something that he needed so desperately.  This program is two days a week for three hours in the afternoon.  Lydon has done amazingly well. He really likes his teacher.  He has made new friendships, learned to share and take turns, and raise his hand and ask a ton of questions!  He is doing almost as well physically as he has mentally and emotionally. Hes had some cold symptoms but only missed one day because of being too tired.   He is able to make it through the biggest part of his class without using his oxygen.  He usually gets tired about half-way through and/or about the time they start singing and dancing.  He crashes pretty early on school days and is pretty low key with energy and eating the next day as well.  We are not sure where this leaves us as far  as a Kindergarten schedule next year but we will just take it one day at a time.

     After months of fighting through all the red tape, we were finally able to get all of the supplements/medications that his mito doc had prescribed approved by our insurance. We were very optimistic about the potential increased energy and positive side effects from the new combination of meds.  BUT..., let us not forget this is Lydon Paxton we are talking about and our mystery boy is never dull and boring! After everything we went through to get this for him, we started it and sure enough it did seem like it was helping.  He was running around, eating and playing and seemed great until he fell asleep that night.  We gave it about a week but it become more than obviously clear that the new combination was acting like a diuretic and were it not for the Pedialyte he receives overnight for fluid balance, the medication would have been dehydrating him. So, we had to stop that but we were able to separate them out and keep the most beneficial ones on board without any negative side effects.  Wish he would've informed me that he preferred it that way before I fought tooth and nail with our insurance.  Guess he wants to keep me on my toes and keep my brain sharp!  I wouldnt have it any other way my sweet boy....You are worth it all!

P.S.  Through an awesome organization called I Run 4, www.whoirun4.com,  Lydon has been matched with a runner who now dedicates all of her runs to him. We are so excited about this. Its exciting to know that Lydon is the inspiration for somebody to run hard and stay strong and raise awareness about Mitochondrial disease. So cool to see my baby's name on running shoes, especially when he isn't having the best day. He has the heart of a champion for sure and now he is inspiring somebody else's heart and "sole". ;)

Food for thought

Surprise, Surprise! This is a post that isnt about sweet Lydon.  He has been holding his own for the moment. Thank goodness because we've had plenty of other "stressors" to tend to over the last week or so.  We are STILL working with doctors and insurance to get the new meds and treatments started. I will update when I have any new information but for now, here's whats been on my mind. 

I’ve been thinking the last several days about diet, wt. loss, etc.    I’m an average All American female with three kiddos.  I’ve never been “obese” but I’ve always struggled with my weight and been “thicker” than I should’ve been.  It wasn’t until I was in my 30s and had my middle child that I had more control of my weight and even still it’s been a struggle.  I, as many of you, have often wondered why does it seem so difficult to eat right and exercise.  This past week, my Dad who has for most of his life been pretty fit and healthy was diagnosed with near complete heart failure and ended up needing an emergency quadruple bypass surgery.  So, this is the realization that I’ve come to.  For most of us, including myself,  I think that  our wt loss goals tend to stem from a pretty simple and somewhat superficial viewpoint.  Most of us want to look good and feel good about ourselves, right? But what if our goals grew from something deeper and more meaningful than that?  What if it was a life or death matter?  What if you were told that if you didn’t lose 25, 50, or even 100 lbs. that your heart would fail and you would imminently die? What if your ability to continue working and caring for yourself or your young children depended upon that 25 lb weight loss? If that was your reality don’t you think it would be easier for you to choose apples with peanut butter and a glass of water for your afternoon snack over the large fry and Dr. Pepper?  The truth is that IS reality for all of us.  I’m nobody special but I have been a nurse for over 15 years.  I have a full understanding of the anatomy of the heart and heart disease in general.   This past week, all of that knowledge and experience became something very real and very personal.  My perspective has changed…. I’m not gonna lie, I still want to look good in my clothes and feel good about myself  but my goal is more about being “heart healthy” and less about “losing weight”.  It may not literally be a life or death issue at this moment but for me, my long term health and quality of life has become the real “heart of the matter" in my lifes journey of being the best happy and healthy Mom I can possibly be for my three beautiful babies!

A "simple" explanation of Mito

I found this on a blog from the UMDF and thought it made a lot of sense for those that haven't really understood Lydons disease.

What Is Mitochondrial Disease and How Do You Explain it?

I often get asked for a simple definition.  Okay so here it is; mitochondrial disease occurs when the body cannot produce enough energy to sustain life. Of course this is sort of like saying the earthquake that caused the domino disasters in Japan is the result of an acorn that fell off an oak tree in San Diego.

Mitochondrial Disease is like:

• Replacing your car battery with two Double “A” Energizers and wondering why it won’t start. But your car still looks pretty good, that is until it begins to rust from inactivity.
• Trying to supply the electrical requirements of Los Angeles (pop. 3.7 million) with the one electrical plant in Ranchester Wyoming (pop. 701).
• Swimming against Olympian, 8 Gold Medal winner, Michael Phelps, and you’re pool is filled with molasses.
• That dream you have where you are trying to run away from danger but you just can’t move? Yep, that’s mitochondrial disease.
• The way you feel after running a 50 yard dash compared to the way you feel after running a 50 yard dash with a 50 pound knapsack on your back.
• The way you feel after working an 18 hour day or 18 days without a day off. Well, if you had a mitochondrial disease you’d probably feel that way after breakfast.
• Buying a perfectly good Volkswagen Beetle with a 110 horsepower engine.  It’s a great looking car and it runs terrific.  Now take out a ring, gum up the valves, add some sugar to the gas tank and put in an old head gasket.  The car still looks great but now it will only generate about 50 horsepower.  That will get the Volkswagen around the flat streets of Kansas on a spring day, but now load it up with 3 of your hefty friends, or more if they’ll fit, with a trunk full of luggage and take it to the hills of Western Pennsylvania on a 90’ day.  It won’t make it!  But it still looks great!

Are you getting the idea? And by the way, all these examples are fixable; mitochondrial disease isn’t.

     Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.

     Depending on which cells are affected, symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection

     I hope this makes it easier to understand why Lydon "looks so great" and still needs to wear oxygen , receive fluids every night, struggle with vomiting and stomach pain and needs so many meds on a daily basis. He deals with most of the symptoms listed above. Thank goodness he currently does not have all of them!  

Reality Check

     During the last few weeks of being home from our trip, Lydon has continued to be tired and irritable which in turn makes me quite tired and irritable and admittedly (despite my best efforts) not in the best mood! I have been feeling down or a little sorry for myself if you will because no matter how hard I try to get all of my chores done and get to bed early, it just doesn't seem to happen. I have been SO tired lately.  Every night before I kiss Lydon goodnight, I push several syringes full of meds through his G tube and connect him to a feeding tube for fluids, a pulse ox, and oxygen. Face it, night after night, this is tiresome. As a Mom, this is very upsetting to realize that this is what is required to keep Lydon hydrated and oxygenated and hopefully rested night after night. I am not complaining about having to do these things, I just wish they weren't necessary.
     Today, Aidan my 7 yr old. wanted to go to work with me while I sit with my Grandmother.  I felt somewhat honored that he had the option to stay home with our beloved sitter and play outside all day with his friends but he wanted to be with me! ;)  We hung out and laughed together and talked but after a while he retreated to the play room. My Grandmother was dozing, so I had the very rare occasion to watch something of my choosing.  I came across a show on ID that was about a lady who had been wrongfully imprisoned.  I figured it would be a "feel good, happy ending" so that's what I chose to watch. It turns out that this lady was a victim of awful spousal abuse.  She was repeatedly raped and beaten. One day she had finally had enough and in self defense took a gun and shot and killed her husband. A jealous "friend" who also happened to be an FBI informant reported that she had plotted this for the life insurance money. Based upon his testimony, she was convicted to life in prison. After almost 20 years in prison, her case was reopened. Despite there being evidence that A.) She was the victim of severe abuse, B.) the FBI informant had perjured himself on the stand C.)  Evidence that her husband had also raped their daughter and D.) She was a model prisoner who gave Bible studies and had even formed a choir for the inmate service on Sunday mornings her parole was denied for six years!! In an unexpected twist of events, she was diagnosed with Lung Cancer and so her attorneys filed for a Grievance/Merciful release and it was denied yet again! Finally, the support from the community was overwhelming and the information regarding the FBI informant was finally made public and she was released! She had ten beautiful months with her family before she died. Her attorneys wanted her to sue the DA's office for withholding such vital evidence that effected her case but she would not.  She has such a positive attitude and such unwavering faith.  She said she was going to enjoy every moment of freedom she had and let God take care of the rest!
     So, what is my point in all of this and what does it have to do with Lydon?  Maybe this is just for me and it will not make much sense to the rest of you but this is what I see.  I do have ample reason to be upset about Lydon and for Lydon, no doubt. BUT, I also have SO very much to be thankful for.  It was amazing to me to see the story of this lady who was repeatedly abused, saw her husband rape their young daughter and in trying to protect both of them spent most of her life wrongfully imprisoned. I tried to imagine how she mustve felt to see her young daughter grow up while she lived in prison.  She could have become so bitter and angry and mustve felt so abandoned by God when year after year, her parole was denied even after she was diagnosed with a terminal illness. But she remained positive and stayed strong! After her release, she lived for almost two years longer than doctors expected her too and in turn made a huge impact on California's laws regarding victims of spousal abuse. I dont know what the future holds for our boy and I most definitely have "bad" days where I worry about him and hurt for him but I will NOT give up! Somehow, someway, Lydons story is going to make a difference. Maybe he will help some of his doctors connect the dots to not only diagnose him but other kiddos who suffer with the same things that he does. Maybe it's so that Jonathan and I can be used to inspire other parents in some way or maybe these obstacles are molding Lydon to be the most tenacious,  compassionate, innovative Pediatric Neurologist in the world! ;) I dont know what the purpose of this journey is. We have encountered many obstacles already and I'm sure there will be more but today I was so encouraged and inspired to fight harder to remain positive even when my heart is heavy and things seem unfair. The reality is things in our life with Lydon could be SO much worse! Despite his need for oxygen, fluids, meds, etc, he is a very happy secure little boy who knows that he is unconditionally loved by SO many people and we make sure that he gets to do a lot of living!! So far, we havent gotten very far with getting our new meds approved.  We are waiting on insurance and we all know how that goes.  I will update when I have more information.