My Older Son is Not "Jaxson's Brother", His Name is Jeffrey and He is Amazing

All too often, siblings of special needs kids go overlooked and underappreciated. These children go through so much, and people don't even realize how strong they are. This week I realized just how much our family has to sacrifice on a daily basis to meet Jaxson's needs. The impact of Kabuki Syndrome on Jeffrey is wildly evident during the holidays.

This week, the final week in October, is typically filled with celebration. Kids bounce off the walls in anticipation of Trick or Treat, parents do their best not to rip out their hair, and it finally feels as if fall has arrived. With fall and the return to school, germs and bacteria are spread in vast arrays all over the country. A typical child will get the cold or whatever bug happens to be ravaging the school, and recover in a short period. Kids with depressed immune systems or who are more susceptible to illness are not so lucky.

Thus is the case with Jaxson. He has not been diagnosed with an immune deficiency yet. I say "yet" because it is possible that he will develop the problem as he gets older, it is very common in Kabuki. Jaxson also has a slew of issues that make it hard for him to fight colds, and a bout of any respiratory problem is bound to land him in the hospital. His mitral valve is causing blood to back up into the right side of his heart and put pressure on his lungs causing them to retain fluid and he has pulmonary hypertension. On top of that, Jaxson has Reactive Airway Disease, which is a step below asthma. He doesn't have attacks, but his airway is extremely sensitive. Nasal and sinus drainage give him major coughing fits, sometimes to the point he gets sick, and his body can't protect his lungs from such drainage properly. So when Jeffrey stayed home for a week with what we thought was a virus, we did our best to keep him away from his brother. Any parent knows how impossible that is.

I knew when Jeffrey got sick that Jax was going to get it and eventually need admitted. I hate being right, but I guess mom's just know their kids. Because of Jaxson getting sick, the boys didn't get to have the celebration we had planned for them. On Sunday, there was a Halloween party for kids with congenital heart disease through Mended Little Hearts of Columbus. We RSVP'd that we would be there, but Sunday was the day Jax was admitted. When we couldn't go to that, Jayson was sweet enough to take Jeffrey to a local event so that he could still have some fun even though he was bummed we missed the party. He and daddy also painted his pumpkin while Jax was in the hospital, Jayson wanted to give Jeffrey a distraction and show him some special attention. He's such a good daddy! But missing out on those things wasn't all. Jaxson could not go Trick or Treating last night because he's still fighting the remnants of the flu, so Jayson took Jeffrey with some friends while Jax and I stayed home. I tried to have him paint his pumpkin, but he wanted nothing to do with it. I know he was upset he didn't get to go, even if he can't voice it. And now today, Jeffrey has his Halloween party at school. Last year I dressed Jax in his costume and we went up to hang out with his class. I told Jeffrey this morning that we wouldn't be able to go, and he just says, "I know," and looks down at his donuts sadly.

Seeing that broke my heart. Jeffrey is such an amazing kid, and he's all too often swept to the side because Jaxson needs extra attention. Jeffrey will be 7 at the beginning of December, and for the last three years he's had to do things separately from his brother, which is unlike anything he's used to seeing. All the other kids he knows get to have regular celebrations and do all kinds of fun things with their families. But he doesn't complain. He gets sad sometimes, and sometimes he acts out, but he's also a 7 year old boy and they just do that. Jeffrey just has an extra reason to behave that way sometimes.

Jeffrey is the most empathetic child I have ever seen. He gets so upset when Jaxson is inpatient, he cannot stand to have the family separated even if we are close by. He's so smart, and silly and wild and crazy and all of the things a 7 year old boy should be. He loves his brother so much and will do anything to help keep him happy! God could not have given us two more perfect children. I want to find a way to show Jeffrey just how much he's loved and appreciated, but it's not easy at his age. Sometimes nothing is enough, and that's normal for a lot of children. So we do our best, but we never feel like it's enough to compensate for everything he's had to go through.

Not only is Jeffrey affected, but Jayson and I are too. I am the one who typically stays with Jax for a variety of reasons, so I miss out on a lot of fun things with Jeffrey. But we do try to take turns, so sometimes it's Jayson that misses out. I had to quit my job and stay home with Jax because of his health issues, and although now I'm working from home, I don't get the breaks I did before. Jayson has had to deal with numerous employers who refused to understand and allow him time off when he needed it (his current job has been great, except for one incident). We do not have the financial means to give our children the things that they want, and sometimes the things that they need. Jayson and I will go without eating if we have to, to make sure that Jeffrey has food and Jaxson has diapers. We live in a not so nice area of town because we can't afford to live anywhere else. Things are on the upswing now, but it's taken a long time to get where we are.

The impact of a medical diagnosis on a child affects everyone in the household. People always say we're so strong and that Jaxson is the toughest kid they know, but let me tell you something. Jeffrey is tougher than Jaxson. He may act like a sensitive, whiny brat at times, but all kids do. Jeffrey has more mental strength than any kid his age should have, and he's learning to control his emotions already which makes me sad. We would love for our boys to have some semblance of normalcy, like a family with "typical" children. We will never have that. Jayson and I, we are fine, we get it and we will do whatever it takes to care for our kids. But Jeffrey deserves so much more.

Kabuki Syndrome Awareness Day!

Today is Kabuki Syndrome Awareness Day worldwide! Since we are all sick in my house, I wanted to put out an informational piece regarding Kabuki to help raise awareness via the internet. The fact that we are stuck inside today is a prime example of how Kabuki affects our family and others.

Kabuki Syndrome is a rare genetic disorder, affecting just 1 in every 32,000 live births. It's so rare that there are only a few families in Ohio that live with it. It is usually clinically diagnosed by certain facial features common to Japanese Kabuki Theater

makeup, including high arched eyebrows, large wide-set eyes, ear abnormalities (large, low set, small or missing) and a wide nasal bridge. It can also come with a cleft lip or palate, hearing loss, vision impairment, feeding difficulties, and intellectual disability. All Kabuki symptoms range from mild to severe, and is considered akin to Autism for the sensory, behavior and mental disability problems. Kabuki patients also can have hypotonia (low muscle tone), persistent fetal finger tip pads, short pinky fingers/toes and large hands and feet. It can cause late-onset dwarfism, short stature and developmental delay. It can also cause dental issues like not having certain teeth, teeth coming in in the wrong order, gum disease and gingivitis due to poor oral hygiene because of sensory problems.

Besides the physically visible abnormalities, there are a slew of medical complications that may or may not come with a Kabuki diagnosis. Medical problems can affect nearly every part of the body: Lungs and airway, stomach, heart, kidneys, reproductive organs, skin, muscles and immune system. Some Kabuki patients have issues that other Kabuki patients do not have. Some patients have lots of physical and medical problems but not as much on the mental side, while others have severe mental impairment and relatively little medical problems. Some have it mild all the way around and some have it severe all the way around. Some patients walk normally, some are late walkers, some cannot walk without an assistive device and some cannot walk at all.

Every single Kabuki patient is different, which is what makes it so hard to treat. To this point, doctors are tailoring treatment to each patient's need, but there is no set standard that all patients receive. Most doctors recommend early and aggressive treatment for ear infections due to the chronic nature in which Kabuki patients tend to get them. Ear infections, if not treated properly, can cause hearing loss, which is already a problem with most of these patients anyway. It is also recommended to have all vaccinations at the time they are due, including the pneumonia vaccine. Because of their susceptibility to infection due to a low-functioning immune system, any respiratory illness can progress rapidly.

Heart and kidney problems are very common in Kabuki patients as well. Congenital heart disease contains a vast array of problems, including septal defects, valve problems, physiological abnormalities and more. The kidneys can also have a vast array of problems, from having one U-shaped kidney to having two different sized kidneys, to improperly functioning kidneys and more. There are too many possibilities that I cannot think of them all, let alone list them.

With regard to Jaxson, he is affected by Kabuki in all aspects and then some. He has KP on his skin and occasional eczema outbreaks. He has hypotonia that led to him being a delayed walker and gives him gait problems still. He also tires easily when walking a lot and has muscle aches. He was born with a cleft palate, but not his lip, and suffers from hearing loss and vision impairment. He eats by feeding tube due to swallowing problems (likely caused by hypotonia) and oral aversion (sensory). He has wide-set eyes and ptosis (upper lid comes down farther than it should), fetal finger-tip pads (typically disappear in infancy) and will need lots of dental work in the future. He has kidney stones, reactive airway disease and horrible reflux. His heart has a bicuspid aortic valve and his mitral valve had a membrane blocking it at birth, plus other abnormalities with the mitral valve. Because of his heart problems, he also has pulmonary hypertension and is even more susceptible to illness. He has developmental delays in speech, fine and gross motor, and feeding, but cognitively is on par with his peers.

Jax also has issues that are not necessarily proven to be part of Kabuki. He had a tethered cord at birth, had bones fused in his skull at birth, lives with hydrocephalus and Chiari I malformation. All of those things also contribute to his gait and balance problems, can affect his bowels and cause pain, numbness or tingling in his extremities. His 10 surgeries are not typical of a Kabuki kid either, especially since half of them cover the things not related to his syndrome.

Kabuki Syndrome by itself does not alter life expectancy for people diagnosed, but the problems it causes can contribute to premature death. The severity of the problems is what would cause that, and it is not unheard of for children with Kabuki to pass before they should. So while it's a little ray of hope that Kabuki patients can live a normal life span, there's still a little buzzing in the back of the head with "what if's".

Now that I've talked about the problems Kabuki can cause, I also want to talk about the positives. These kids, they are definitely special! Most Kabuki patients are extremely friendly and social, they love other people and constantly want to have attention. They LOVE music! Jax is definitely an example of that. They are generally happy kids, sometimes to the point where you might think they don't know what is going on. But most of them do, they are smarter than you think they are. Kabuki patients are sensitive too, they care if you are happy or sad and want to make sure the people they care about are okay. Jaxson, even with all he's been through, is the happiest kid I've ever seen. He's strong, like all of his Kabuki family, and doesn't seem to feel pain unless it's massive. He gets frustrated when he can't communicate, hates to ask for help and adores his big brother. He is the sweetest thing, asking for hugs and cuddles, wanting his bear (lovey) to have hugs and kisses and pretending his bear is sick when he is. He hates his feeding tube but refuses to eat so he tolerates it. He never wants pain medication, even if he tells me he hurts! He hates clothes, preferring to run around in his diaper. He loves to get under his brother's skin too, which is hysterical to us because he's totally paying Jeffrey back for the last three years of being in his face!

The one thing I want people to take away from this blog is this: Kabuki Syndrome is a diagnosis, but it does not define who our children are. Our Kabuki Kids are beautiful, happy, loving individuals. They have their own personalities and generally have a smile on their faces. Our KKs are nothing short of amazing! They literally have to fight every single day just to survive, and they not only do it, but are mostly happy in doing so. We can learn a lot from our Kabuki family, like how to live life to the fullest with a smile on your face because you never know what's around the corner. In that, it applies to all aspects of life, but the Kabuki patients exemplify what it means every single day.

Wait and See

It has been almost a year since my last blog post. It's been a very rough year for us with Jaxson having lots of issues, Jeffrey is having behavior issues, Jayson started a new job and I am now working as well. Rather than try to recap what's happened, I prefer to start with what's happening now. That's the reason I write, and the best way for me to relieve stress.

So, back in June Jaxson had open heart surgery for mitral stenosis. Prior to surgery, the pressure in his heart measured at 21. Anything over 10 is considered severe. When he was discharged, his pressure was at 10-11, but because it was such an improvement from before, we expected him to recover and be okay. The doctor had told us that Jax was in a gray area of whether he would need further intervention in the future. Of course, it's Jaxson, so we naturally assumed that he would need something down the road. What we didn't expect was to hear that at the beginning of September, Jaxson's heart pressure was back up to 16. Because he wasn't having any symptoms, we were not in a hurry to proceed with surgery, attempting to put it off as long as possible, but knowing he would need it within six months. We scheduled another procedure for him so the doctors can get a better look at what is going on, and he will have that done tomorrow. He has been retching a lot again, taking really long naps (but waking up of and on) and he's just not as energetic as he was even a week ago. We are happy he's having this TTE tomorrow so we can get this process going, as I'm pretty sure it's going to happen sooner rather than later. What we're looking for tomorrow is a plan. Can we get him to the cath lab? Not likely. Because his valve has been repaired, a valvuloplasty could cause leakage. If they think they can keep the leakage minimal to buy him some time, it could be a consideration, but likely not a feasible option. Can we do another repair? Again, not likely. There's going to be scar tissue from his last surgery, and after reading the surgery notes myself, I can't imagine that they'd be able to do anything else. Which leaves us with one option: Mechanical valve.

Mechanical, or prosthetic, valves are never an ideal choice in a child. For one thing, children grow. So as they grow, the valve will have to be replaced multiple times in order to keep his heart stable. The faux valve will be a foreign body placed into his, in a very sensitive area of the body. There's a chance his heart could reject it, although that's not common. The valves come in two different types: man made and animal. There has been a lot of success placing animal valves in people who need them, but there's also the chance the animal valve will fail before the man-made valve. Of course, no one is looking at the kids that just need them replaced because they're bigger. Studies show that man-made valves last longer, but they are so new, that it's unlikely anyone has done a study on kids who need more than one. But it's not time to worry about it because we don't even know the plan yet. After tomorrow, we'll have a better idea of what to epect with his heart going forward.

Besides his heart, Jax has also been complaining of back pain, leg and foot pain, and he's been extremely constipated. So, when he has his TTE tomorrow, they are also doing an MRI of his spine to see if there are any changes, and they'll check his Chiari as well. Any changes with his spine could lead to changes with his Chiari. Tethered cord and Chiari have a lot of the same symptoms, so sometimes it's hard to tell which is the culprit. Hoping that it's just growing pains and nothing has changed, but we know Jax and I'm not holding my breath. I am expecting another intervention with his spine at some point. I really don't want to touch it right now because he's already going through so much. He doesn't ever want medicine when he's in pain, so I'd rather not risk it. The fear being that his spine was tethered with arachoid, which is not a normal thing, and his previous neurosurgeon said that he did as much as he could without causing further damage. Because his spine has already been operated on, he'll have additional scar tissue in there to get in the way. There's no way to prevent scar tissue from forming, and even if the new NS can clean some of it up, there will be even more after another surgery. So, again, wait and see.

Jaxson likes this whole "wait and see" thing. It's never an emergency until Jax decides that it's time for intervention, and then it's a whirlwind of chaos and appointments and information overload. I've added more meds to my list to keep me sane, Jax has added more meds to keep him healthy, and even Jayson (daddy) is taking meds to help with his seizures (epilepsy) and mood. We are all coping the best we can, but I think none of this is real yet. None of it will be real until we have more information and know a plan. I'm terrified they are going to tell us there's something new and crazy going on, because Jax just does that stuff, and trying not to prepare myself for that is hard. It's a fear, one I try to brace myself for every time he is sedated. For instance, Jax is sitting next to me now and he's breathing really heavy and fast. It's almost panting, but not quite. He's only been awake for about 20 minutes and hasn't done anything crazy. No running, no jumping, no beating on anything. Just playing with his toys. It's a symptom of his heart, but I'm worried about his lungs too. If his mitral valve backs up enough, he will end up with blood in his lungs. There's already extra fluid there, so it won't take much.

On a brighter note, Jayson started a new job earlier this year that he absolutely loves. He's been doing some side jobs for extra money, and I recently started working part-time from home. Sports writing has always been a love of mine, and it's something I've been doing for about 10 years. I've been out of the game for a bit, but it's been nice to get back in the swing of things and write again. I think it will help me keep up with this blog too. The release is exactly what I need right now. It's been getting colder out, so taking Jax anywhere is risky. I get totally depressed when I don't/can't leave the house for days on end, so I try taking extra Vitamin D and B12, and if I can get myself to write on a more regular basis, then maybe I can feel like a regular person again. Maybe. For now, I'm going to enjoy watching football with my littlest man since it's just the two of us today. Can't wait for the big boy to get home from grandma's later, I miss him!

Reality Check

Over the past month, our entire family has been sick. Jaxson ended up with a double ear infection, Jeffrey with a stomach virus that lasted a week, Jayson caught the virus but managed to kick it in a couple of days, and I caught the virus and couldn't get rid of it, landing myself in the ER with a bleeding ulcer and dehydration. It took two full bags of IV fluids running over three hours to hydrate me, and I still wasn't 100%. Then I got a sty in my eye and Jeffrey brought home yet another cold, which Jax and I have been fighting for days. On top of that, Jayson started a new job two weeks ago that has him out the door by 7am. Normally that wouldn't matter, but since Jax is back to his old habits of not sleeping, it makes it difficult for me to get any rest. Jayson takes him on nights he doesn't have to work, but he is halfway through two weeks of working without a day off. I can't ask him to stay up with a cranky baby when he has to work so much, so the house has fallen apart. Laundry is at least a week behind, we are literally washing clothes to wear the next day, washing dishes as we need them (no dishwasher), and staring at our gross carpet because our vacuum broke and we haven't been able to get a new one yet. That's it hit me: this is my reality.

My reality includes little sleep, little food, bad personal hygiene, ulcers, stress, anxiety and massive amounts of frustration. I need to utilize my outlet of writing now more than ever, so I'm hoping to take time out every day to write something. Whether it's posted or not is yet to be determined. But this is my life, as is similar to many special needs moms. I think the hardest part to deal with is the frustration.

Frustration appears in my life on a regular basis and in a variety of ways. Frustration with doctors, with hospitals, with homecare providers, with the numerous appointments every week, with feeling like I'm not heard, with the constant amounts of paperwork and hoops to jump through to make sure Jaxson has the best care. Then there's the frustration of knowing he's behind, seeing how behind he is when he gets around other kids, and knowing that we're doing everything possible to help him and he's not catching up yet. Don't get me wrong, he's definitely progressing and doing AMAZING things that I never thought he would do, but that's what makes it frustrating. He's already worked so hard and come so far, and yet he still has so far to go. Our journey is not even close to over. Now add in the frustration of having another child who can't express himself properly, is defiant, angry and sensitive to everything and anything. Jeffrey is nearly six (next month!) and still throws himself on the floor and the furniture when he's mad. And it's mostly because he doesn't understand what's going on or how to express himself, plus he has no idea what questions to ask to get the answers that would help him.

Besides the medical stuff, most of that is normal frustration for any mom. So I want to focus on something that's not normal: Watching your child with special needs do something normal (play date, etc.) and having people give him (and me) looks of pity. We do not want pity. We do not need pity. We need support and understanding. We need awareness on all childhood diseases/syndromes so that people will understand what is being said to them and treat our kids like any other kid. That's what we do at home. Jax and Jeffrey run around and play, get into trouble, climb up and down the stairs, jump on beds, throw toys around and just act like boys. Jaxson is mostly non-verbal, we all know that, but he is in no way, shape or form delayed in his mental capacity, nor has he ever shown a sign of diminished cognitive ability. He understands everything you say to him, so when he's upset about something, I just calmly explain to him what the deal is and he usually calms right down. He knows what he's allowed to do and what will get him in trouble, but like any 2 year old, he forgets 10 seconds after you tell him or he just doesn't care. He has listening issues like any other toddler, and he wants constant attention like any other toddler. But he doesn't talk, and therefore people assume that he has some form of mental retardation. I use that term as a clinical description, not a derogatory one. But Jax doesn't have that problem. People just don't understand.

I also become frustrated when people look at Jaxson's face and see their reactions. Of course everyone thinks he is the cutest thing ever, or at least that's what they say. But you can tell by looking at Jax that he's different. His head shape isn't normal and probably never will be. He has large features (eyes, mouth, nose, ears, hands and feet)) but is undersized overall for his age. He wears hearing aids and soon he will wear glasses. He has scars on his head that are pretty well hidden when his hair is long, but it's not always long. He has very visible blue/green veins down the sides of his face and forehead. I thought taking him out in public with pins in his head was bad, at least I could explain to people it was temporary. Soon he will also be wearing SMOs to help correct his gait and balance issues. All of these things are visible and not aesthetically pleasing. Because of that, it's not only different, it's bad. Supposedly.

I know I was picked to be Jaxson's mom, and I am thankful every day that I get to spend with him. We've come dangerously close a few times to not having him home, but he's always fought through it and proved everyone wrong. And once people get to know him, it's impossible to ignore his happy disposition, ornery face and his desire to get a rise out of anyone he can. He's such a trouble-maker! Jeffrey was never like that, so it's a new experience for us, but he does it on purpose and will stop when he knows we're mad. He's constantly on the go!

So here's what I would like for Jaxson, for any child that doesn't look or act "normal": No more tight-lipped smiles or turning heads. No more judgmental eyes staring at moms and their kids. No more looks of pity or apologies for things not in someone else's control. I want the smiles you give to everyone else, the looks you give to everyone else and the comments you give to everyone else. All of these kids deserve to be treated with respect. So instead of assuming and judging, please say hi and ask questions if you feel it necessary. And when a deaf child signs "thank you", please don't assume he's blowing you a kiss. If you get to know these kids, and I can vouch especially for Jaxson, you will find that most of them are just as happy as "normal" kids, if not more so, and they're very willing to share that happiness and light with you. Most of these kids have fought hard their whole lives, don't you think you should be giving them hugs and praise instead of stares and judgments?

I do. And that's how I treat special needs kids and parents now. Maybe I wasn't the best about it before, no matter how hard I tried to not pass judgment, but that's how I do it now. And you know what? I've been inspired more in the last two years than in my entire life. Each one of the kids I come across and have the pleasure of interacting with not only makes me smile, but gives me a reason to keep fighting for my child and for what's right. If we all did the same, imagine the outcome.

The Emotional Side of Hydrocephalus

Many people have been asking how we are doing. I post a lot about Jaxson, and I try to include everyone else when I can, but lately that hasn't happened. So rather than post something horrifically long on Facebook, I decided to blog. I have no idea how long this will end up being, or how erratic my writing will be, so please be prepared.

We'll start with a quick bit about Jaxson. I've noted what's been going on with his head, but not how he is doing overall. Jaxson is actually doing quite well, believe it or not. He's always in a good mood, and even when he's mellow he's still ornery. Most days you'd never know there was anything wrong with him but for the bulge on the side of his head. His gait is off-balance, but we're not too worried yet. I wouldn't walk straight if there was more weight on one side of my head than the other either! He's just such a happy kid, it's so hard to even tell when there's something going on with him. He never lets anything get him down!

Jeffrey has always been one of those kids that goes in cycles. He will have a streak of fabulous behavior and then a streak of being horrible. The streaks of good behavior are coming fewer and farther between, so it's clear that he's struggling with this one. Jaxson has been to the neurosurgeon three times in less than two weeks, plus other appointments. Jeffrey knows something is up, we've had to make too many trips, and he's worried about his brother. He wants to play with Jaxson so bad, and he does, but we have to remind him to take it easy because of Jax's head. It sucks. All they want to do is play together and they can't even play like normal boys. So Jeffrey acts out, he throws fits, he cries for no reason and he's become increasingly violent and rough. We take him to behavioral health in November, and besides the ADHD they're going to diagnose him with, I'm worried about other things that aren't quite as easy to help him deal with. No, I do not think ADHD is something "easy", but I have experience with it and I have learned techniques that work with Jeffrey already. I will not medicate him unless there's no other option. I'm just ready for this evaluation so we can work on getting him the help and coping skills he needs.

I think the best explanation for Jayson and I is tired. We're tired, drained, scared and worried. We're tired of the appointments, of the endless hours of waiting for someone to get back to us with answers, tired of never having a definitive answer or course of action, tired of not getting to spend time together or even share a bed. We haven't shared a bed since surgery because Jeffrey has a tendency to sneak into Jaxson's bed. They both move around a lot at night, so it's not safe for Jaxson for them to sleep together. So Jayson sleeps with Jeffrey and I sleep with Jaxson. Going on two weeks now. Our marriage is as solid as it ever has been, and our lines of communication flow freely, but it just sucks. We're drained from worrying all the time. Wondering if Jaxson will be awake in the morning, or if the next surgery is going to go as planned. Terrified that anything might happen to our baby at any given moment. And on top of this we have to take care of Jeffrey, who is clearly the biggest loser in this situation. He gets less attention now and we worry that we are missing something with him. We haven't even had time to get his school supplies or uniforms, and school starts next week! He's always waiting on us for something because Jaxson needs so much attention. And Jeffrey is SUCH a good kid, it is heartbreaking to watch him deal with this. Almost as heartbreaking as it is to watch Jaxson deal with it. It's hard enough watching one of your children regress because of a medical issue, but add to that watching your other child regress to toddler-like behavior and you have a pretty good dose of guilt built up.

Earlier today, Jayson took Jeffrey to pick up some paving stones from a friends house for the back yard. That left Jax and I at home for awhile. He sat on my lap for awhile and we watching Signing Time. I am not an emotional person, so I usually know when I'm due for a good cry. I've been due for about a month, so I wasn't all that surprised when I teared up holding him. Jaxson doesn't see me being emotional very often, so he wasn't quite sure what to make of it at first. Then he just looked at me with those big blue eyes and gave me a big ornery grin. It was as if he was saying, "Hey mom, it's cool, I got this!" How is it possible that such a tiny human being can possess so much strength as to hold up his own mother? And while tears streamed down my face for a little while longer, I wasn't quite as upset as before.

For me, the biggest frustration is having to explain to people that even after he gets the shunt (if he needs it), he's still not okay. In fact, I have to keep a very close eye on him all the time after that to make sure the shunt does't malfunction, get clogged or get infected. Any one of those things is an ER trip with immediate surgery. Having surgery with a shunt in place creates all kinds of new risks we have to be aware of. We would have to take precautions anytime we took him somewhere where the air pressure is different. I have to look this up, but I don't think he will be allowed to participate in contact sports. So if that's the only way we're going to fix his hydrocephalus, then of course we will do it, but there is a whole new load of things to learn. But not everyone is educated on things like this, and why should they be? I knew nothing of it until I had to, so I wouldn't expect most people to understand. But it is frustrating. Hydrocephalus is a lifelong condition. It does not go away, so no, Jaxson will still not be okay after this. Not even with regard to his head, but everything else that goes on with him.

I have to say that this has been the hardest issue for me to deal with. I know it's a pretty serious problem, but I don't think I've been this scared of losing my baby since they took him off the heart medication in the NICU. Because, like heart conditions, hydrocephalus can sneak up on you at any time. There's often no tangible cause for fluid to suddenly build up, that's why people with hydrocephalus are monitored so closely.

It's now after 10pm and I can't believe I haven't turned into a pumpkin! I'm beat, Jax is asleep on the floor and J is asleep on the couch and Jeffrey is in bed. I suppose I should join them if I want to be able to function in the morning. Oh who am I kidding? I eat little and sleep less, I'm sure I'll be up for awhile and will still hop out of bed at 6am to get my day started tomorrow. Good night!

"Life is not the way it's supposed to be, it is the way it is. It's how you cope with it that makes the difference."

Insert Witty Title Here

I've had this blog in my head for days, and just haven't had the time to sit down and type it out. Between the two boys, I might lose my mind before school starts! But I digress. Already. Today's blog is solely about Jaxson and my personal opinions about what the future may hold.

First, I want to bring you back to January of this year. If you recall, we took Jaxson in for a CT because we were concerned about pressure. Because he still had his distractors and the CT didn't show anything, they took out the distractors and did an MRI. Even though his ventricles were enlarged, we were told no hydrocephalus. I was as confused as you are. Fast forward to March, I essentially forced neurosurgery to scan Jaxson's brain/Chiari because I felt he was having pressure again. Again we were told no. Fast forward to July and the eye doctor appointment. We are told his optic nerves are elevated and he needs an MRI to check for hydrocephalus. Lo and behold, proof. I had been screaming it for 8 months before I got the doctors to see what I saw. Ironically enough, it was our new NS that found it, which is a good sign I suppose.

So, hydrocephalus finally diagnosed, Dr. Governale discusses Endoscopic Third Ventriculostomy with me and how Jax is a good candidate and may save him from needing a shunt. I agree and we schedule surgery for the following week. When I get home, I let everything process and sink in, and then I reach out to my amazing Facebook groups for opinions. Some of the people I've met are extremely knowledgeable about certain conditions, and some of them are REALLY good at reading brain MRI's (which I am not!). The bulk opinion was that Jaxson's head is too small and needs to be expanded again. However, Jaxson's head is growing on the same curve it has been since his distraction surgery. I've been keeping an eye on it myself and there's nothing concerning there so far. So I agreed to do the ETV to avoid (hopefully) shunt placement.

Initially, everything seemed to be going really well. Jax was ready to go home the day after surgery and he was pretty happy. Then he ended up with a HUGE mass of fluid on the side of his head. I took him in to NS before another appointment just 5 days after surgery. NS ended up draining over 40mL of fluid out of his head and wrapping it with an ACE bandage for pressure. It sounds like there's a tiny hole in his dura, and keeping the pressure on it may help it heal. Now, almost a week after the fluid was drained, Jaxson refuses to keep his wrap on and his head is not getting better. It's not getting worse either, so that's good, but it's not getting better. So I finally followed my mommy instincts and placed a call to Dr. Jackson, Jax's first NS who we absolutely loved. He let me know that from the sounds of everything, treatment has been reasonable and we need to give the ETV time to do it's job. He said he once had a patient who had a similar bulge last 9 months! He said that was a different situation, but I'm glad he said that because now I won't have a heart attach when the swelling doesn't go down as fast as I think it should. He said that it's possible that as pressure goes down in his head, Jaxson's Chiari may get better as well, which is awesome!

The other thing I asked Dr. Jackson about was Jaxson's spine. Since he did the un-tethering procedure, I wanted to pick his brain about what the obstacles were during that surgery. That man has an amazing memory! He doesn't have access to any of Jaxson's records or his operating notes anymore. He remembered that Jaxson's spine looked rough on the initial MRI, and he remembered that the procedure itself was not satisfying for him. He said that Jaxson has a lot of arachnoid adhesions, and that he basically did as much as he could without potentially causing further damage. He said that Jaxson's spine is always going to look tethered on an MRI.

Time for my opinions. Please remember that these are just that, opinions. Based on conversations I've had with doctors and what my gut and brain are telling me about my baby.

Here's what I see happening as time goes on. Jax will likely need a shunt. Although Dr. Jackson said that if the ETV lasts 6 months then we have a better prognosis of it lasting a long time. Given that Jax has a huge bulge on the side of his head and that his hydrocephalus symptoms aren't getting that much better, I foresee a shunt sooner rather than later. I see another distraction surgery in our future. The joys of pins. Because I agree with the people that say his head is too small, I mean, a portion of the back of his head is still flat. And if none of that makes his Chiari go away (which it probably won't because of his tethered cord), then he'll likely need decompression at some point in his life. The Chiari may not need addressed for many, many years, but I'm sure that at some point he will need it. It will be a matter of distinguishing which disorder is causing his symptoms.

As far as Jax's spine, I'm thinking that any attempt to clean out scar tissue will be futile. The more Jax grows, the tighter his spine is going to be pulled, and any surgery can cause scar tissue. So if there's scar tissue on scar tissue, eventually his spine won't be able to pull any farther. Mind you, this is as he grows, which he is not doing much of right now. We go back to Endocrinology in February for more tests. Now, Jax is already having some symptoms from the tethered cord. He limps and drags his right foot when he walks, he has pain in his right leg and wants me to rub it all the time, and that same leg did not react to the knee-jerk test the last time we had him at Complex Care. Since it's not prohibiting him from doing anything, it's not even something I'm going to bring up to the doctor. I'm leaving that alone for as long as I can because there are only four options when the time comes: Leave it alone and see how he does, attempt un-tethering again, shorten the spine, or spinal transection. You can see my dilemma. He's 2. He probably can't even have his spin shortened or transected until he's done growing. I don't know that, but it makes sense. Not that I like either of those two options. Thankfully, that's not something we have to worry about right now, but it is something that weighs on me at times.

Jaxson is such a joy to have in my life and an amazing light to everyone he meets. I have no concerns about his cognitive ability (kid is smart!), just his current and future physical limitations. As long as he keeps that smile on his face, I know everything will be okay! I've seen so many things on the web lately, I can honestly say that I would rather have Jax here and need constant monitoring than for him to not be here at all. I cannot bear what the parents of kids with cancer go through, especially if the battle is lost. I cry every time. For the parents as well as the loss of a beautiful soul. So, I take comfort in knowing that, as of right now, there is no threat to Jaxson's life expectancy. I hope that doesn't change, but if it does, I'm sure we'll beat it like we have everything else!

~"Life is not the way it's supposed to be, it is the way it is. It's the way you cope with it that makes the difference."

Catching Up

We finally got our internet last night, and I logged on today to discover that I haven't posted in over six months. For that, I apologize. We are hopeful things will be back on track now!

The last six months have flown by. Jax had his distractors out and cleft palate fixed and after that we haven't had any inpatient stays, our longest stretch by far! We did have to go to the ER a few times for various things including a fall down the steps, but that's to be expected with you have kids and especially boys.

Everything was going very smoothly until Jaxson's eye appointment two weeks ago. They discovered that his optic nerves are elevated and that he also needs glasses. I'm pretty sure I called that from day one, but Jax cooperated well at the exam and they were able to get a really good look at his eyes. So they put in a call to neurosurgery who referred back to plastics and we have that appointment tomorrow. I had to push for an MRI because no one else thought of it, and we had that last week. I haven't seen the report yet, but I was told that his nerves are elevated about a millimeter. I just don't know if it's a CSF flow issue or a space issue. Well, the doctor didn't know when I spoke with him on Thursday. I know it's a space issue and I've been preparing myself for another surgery for months. I knew we weren't done and I'm not convinced we'll be done after this one. He just has so much stuff going on in his little body and I don't think he can handle it all. At least he's happy all the time!

I am pretty confident in that he'll need another distraction surgery. You know, the one with the metal device in his head and the pins that we had to turn for 30 days. Yeah, the one that was awful. But, if we have to choose between that and a shunt, I'm going with the surgery. A shunt is permanent, so if we can fix his issues without placing a foreign object in his body on a permanent basis, then that's what we're going to do. My hope is that it will also relieve his Chiari and he won't have to deal with a decompression/fusion surgery at some point.

I think that this is suddenly becoming difficult for me. I'm struggling more each day with how Jaxson is progressing and how his body reacts to everything. Not having a known syndrome is making me crazy and I scour the internet for answers that I can't find. We have genetic testing next month, and I know that will at least give us something, but it takes 3-4 months for the results as the test is sent to Baylor. We try our best to live each day to the fullest and to make sure that both of our kids are happy, but sometimes it just feels like it's never enough. Jaxson is always happy and smiling, but he gets left out of a lot of things because he can't handle being in the sun for very long. It doesn't matter if he's in the shade or not, the brightness of the sun crushes him, and the pressure in his head right now only makes it worse. That and it makes him sleep a lot, which concerns me. I know, before he wouldn't sleep enough and now that he is I should be praising God, but when you have kids, anything out of the ordinary is cause for concern. And I'm frustrated with the doctors for not having a bigger sense of urgency about this situation. They don't seem to be concerned at all, but part of me thinks it's just because they know they can only do so much and right now that means wait. I just want some answers for my baby. Until we get some, I won't know if he's getting the proper treatment or therapy recommendations. I mean, the therapists are great at seeing what he needs help with and adjusting their plan, but I am looking at the future. I want to be able to plan a course of action and I'm stuck here, helplessly waiting for the doctors to tell me what's next.

I've become extremely active in some great Facebook groups recently, and I've found a ton of support there. I've given a box of formula to a mom, I've sat with a mom while she waited to hear about surgery, and I've spoken with numerous moms who have supported me and answered my questions as well as me returning the favor for them. I'm closer with some of those moms than I am with my, now pretty sparse, circle of friends. Not only do we understand each other, but most of us stay home with our kids because we can't afford to pay a nurse and our "special" kids can't go to daycare. So they're around when I am, where my mom friends of "normal" kids tend to have jobs and extra activities to do with their kids. Which is awesome, I can't think of any of my mom friends that don't go all out for their kids, just like moms of special kids. It's just a different kind of "going all out" and the term "going mama bear" on someone has a whole new meaning. Because rather than it being another mom or someone else's bratty kid, we get to act crazy with doctors and there's nothing they can do about it! :) But I do wish that some of my friends would remember that I also have a "normal" kid in Jeffrey. He loves people and other kids, he's very social and extremely active. Jax isn't house-ridden either, we just have to find a place with shade for him. I don't think that's too much to ask.

So maybe this is more me getting used to being at home all the time. Jayson said it took him about a year to get into a good routine with the boys and I'm just about there so I guess I'm right on track!

~"Life is not the way it's supposed to be, it is the way it is. It's the way you cope with it that makes the difference."