IEP evaluation frustration

Venting, that’s the reason I originally started this blog. To set the thousands of thoughts running through my head free and hopefully end up with some sort of clarification or relief. So here we go.

Today was Wyatt’s IEP evaluation. In Osceola County it’s called the PEEP program which is just a fancy name for the school district’s early intervention program that the child can transition into once they turn 3 years old. Wyatt had a few speech therapy sessions here and there through the Early Steps program, which is for ages 0-3, but they were never able to find him a speech therapist who was fluent in ASL and at the time, Wyatt also wasn’t very receptive to therapy sessions. He simply passed out when he didn’t want to participate. So here we are 3 years on this journey and we have to put him through yet another developmental evaluation. Just for the record, I hate those evaluation! Yes, HATE them with a passion! The questions are very generalized and don’t leave room for more elaborate answers. It’s mostly a score of 0-4 on how well he performs a task and the parent has to answer a lot of yes and no questions regarding his abilities compared to what a “normal” 3 year old should be able to do. I have several issues with this type of testing. For starters, the questions do not differentiate between communication vs. speaking. That’s a big deal for a child who is non-verbal. Just because Wyatt does not speak that does not mean that he is not communicating. Therefore, a simple yes or no option for an answer is not sufficient. That’s a big issue and can falsely alter his score. Second, halfway through the evaluation Wyatt lost his patience so cooperation at that point was a problem. There were definitely some tasks that I know he can do but he simply didn’t feel like doing them at that time. The darn evaluation did take 2 hours to complete and what 3 year old has patience for that. And then there were questions that I didn’t even know how to answer. I was asked to specify the different sounds he makes. I don’t freaking know, they are just sounds. There are no actual words and the sounds are not specific vowels that fit nicely into the printed test booklet criteria. But there is a difference in pitch depending on the situation. Those type of things can’t be explained to an evaluator who has never met Wyatt and will probably never see him again. So the whole time I felt like we’re portraying him as this mentally retarded kid when in reality the things he will and will not do primarily just depend on his mood and comfort level with the person who is asking him to do these things on command.  I did request an ASL interpreter to be present during this evaluation to see for myself how well he can be understood by someone who is fluent in ASL since that is his primary mode of  communication. While this was very valuable information for me, it was also very depressing. The interpreter was only able to understand 3 signs Wyatt used. His signs are still too modified and would not be understood out of context. So if he was asked to look at a picture and point to the ball and would then sign the word ball, the interpreter could understand. But if he did it out of context it would have been misunderstood because his fingers are not pointing the right way and his wrists touched. So that information was very important for me to know but it was also very frustrating at the same time. It actually makes me very angry because I have been practically begging for help with ASL instruction and have been ignored for the last 3 years. Everything he knows at this point, though it may only be understood by immediate family, was because of the effort we put in. We’ve have been working really hard to get him to where he is today, to allow him a form of communication that cuts down on his frustration level and therefore his tendency to pass out. But based on this evaluation, outside of immediate family, Wyatt basically has the communication skills of an infant. He taps a person’s leg to get their attention, he point at things he wants and he uses facial expressions to show emotions. As far as getting services approved for him I could look at this result as favorable. Having the worst case scenario on paper sure is helpful. But as a parent, it’s devastating to look at such a comparison. All the hard work we’ve put in over the last 3 years seems like nothing on paper when compared to a “normal” 3 year old. Wyatt has come such a long way and we have made huge progress in our eyes but that progress is so small in the context of a developmental evaluation. I almost wish I wouldn’t have put him through this comparison again. I have such a hard time coping with this information. It took me a very long time to accept who he is and to stop comparing him to the “normal” 3 year old. On a daily basis, I can now happily look at who he is as a person and enjoy every little accomplishment. But when we have to jump through the necessary hoops to get him outside help it’s like a slap in the face. That’s why I HATE this entire process. I don’t ever want him to feel like he’s not good enough. I know he will do all those things eventually in his own time. I hate being pushed into the mindset of having to fit some sort of norm. But as a parent of a medically challenged child those are the uncomfortable steps in the process you have to put up with if you have any hope of getting outside help. Simply put, IT SUCKS!  So, this evaluator will now type up his report and then it gets sent off to a bunch of other people up the ladder to decide what services he qualifies for. That can be anything from nothing at all, to just speech therapy at the local elementary school, to a full school day with all therapy services included. But for that decision to be made could take another 2 months as the school psychologist who administered the assessment warned me. When that meeting is schedule the real battle will begin as to what the school district will offer vs. what we want. It’s very frustrating to fight so hard to simply get outside support. It’s been 3 year and so far this fight has gotten us absolutely nowhere. I’d rather just know once and for all that we’re on our own, as we have been so far, than continuously jumping through these hoops without any results.

continuous brain damage in CCHS

The information below was taken from the CCHS Family Network Facebook page and sums up the researcher's findings and recommendations. Even though this information is not new to us, I am posting it here because family and friends may not be aware of these findings. I marked one aspect in bold as it specifically applies to Wyatt.
For the specific research literature, please refer to the CCHS Network Website here http://www.cchsnetwork.org/literature

Dr. Ron Harper, PhD., UCLA, Los Angeles.

"There certainly are better terms I could use rather than [brain] "damage." However, there is no getting around the findings we have; the mammillary bodies lose more than half their volume in some subjects, and that certainly would include cell loss as well as supporting tissue. I showed slides indicating loss of fibers from various structures, including the fibers of the fornix from the hippocampus to the mammillary bodies, the corpus callosum, especially in the posterior corpus callosum, and fibers from the pons to the cerebellum. I also showed injury to the cerebellum, the insular and frontal cortex, and the cingulate cortex, with the injury indicated by both tissue loss and fluid exchange between cells, which is indicative of damage. The cerebral cortex also is thinner in several areas than controls. All of these findings are published in peer-reviewed manuscripts in very high quality journals. Some do not like the phrase "damage." However, it would be a huge mistake to close our eyes and pretend it does not exist; we have to stop it. I tried to focus the talk on the issue that, although we know the injury exists, much intact tissue remains, and we have to make use of that intact tissue, and retrain it to acquire/take over lost functions, and that we have to do everything we can to protect against further injury.
I noted at the conference that a number of the B vitamins, especially thiamine (B1) are flushed out by the profuse sweating and malabsorption in CCHS, as is magnesium (equally important), and loss of those nutrients would lead to further injury, even if ventilation is adequate, but especially if hypoxic episodes occur. It is certainly valuable to know if the CCHS kids are magnesium or thiamine deficient, and to take remedial intervention [i.e. taking vitamin supplements].
Meanwhile, the cause of the [phox 2b] mutation remains unclear."
(RH 7/11/14)

Making sense of things

After another typical evening of fighting with Wyatt to go to sleep, my husband asked if I saw the post on Facebook in the CCHS support group. Another child with CCHS has died. This time, it was a 9 month old girl who lived in Florida. Though I sometimes browse through the posts within the support group, I’m not nearly as involved as I used to be when Wyatt was younger. I clicked on the name to see a picture but I didn’t even recognize the family. I distinctly remember that age, the struggles we went through with Wyatt back then that make today’s struggles seem minimal, yet no less exhausting. This news came after a day of 15 breath holding episodes caused by a 3 year olds tantrum. 15 times yesterday I had to go into emergency mode to make sure he didn’t die. 15 times yesterday I had to use up all my mental energy to not give up on him when he refused to leave his life support on at a time when he needed it the most. 15 times yesterday I asked myself when this fight will end. And then I hear this kind of news. I couldn’t sleep all night. My mind kept circling around the thought of not being able to save him after one of his tantrums. How would it affect me? Would I feel guilty for not having done something right to bring him back around like all the thousands of times before within the last three years? How many times in the worst of days have I questioned whether it would be better just to let him go? Would I be silently blamed? How would the rest of the family cope, my husband, his brothers, and his grandparents? Would it be a shocking surprise or an expected outcome always lingering in the shadows? Either way, none of us will ever be the same people again we used to be three years ago before Wyatt was born. Our perspective on life has changed so much; there is no rewind button to the once idyllic world. This struggle has changed all of us forever already, I can’t even imagine what would happen if we lost the fight. Through the inevitable pain, the daily battles would disappear, the medical equipment would be returned and eventually we’d all move on in a less medically involved world. But would it really be any easier? Then my mind wondered off to the question of why it seems that so many children, and even adults, with CCHS have died in the past couple of years. Are these negative numbers really going up as I perceive it or have we simply gotten to hear about more people living with this condition so any loss seems more personal now? After all, adults and children of all ages and all health status die every day and we pay minimal attention to it – unless their deaths have some sort of a personal connection to us. The CCHS group has certainly grown in numbers despite the relative low number of know cases worldwide. Therefore, a death within this small community seems shocking and magnified in proportion. So I guess it’s the personal connection that hits home. A death of a child that young also reminds us that the odds are against us proportionally to the number of CCHS cases worldwide, and it is really a wonder we haven’t lost our own personal fight yet. Such news brings gratitude but also a reoccurring fear of the unknown number of days we have left. Personally, hearing such news makes me not only want to leave the support group to avoid further anxiety but also want to delete my Facebook account altogether and live real life instead of the virtual one we are all so attached to these days. For one, it makes me realize that I often know more about the lives of people I have never even met vs. my own family who generally speaking has never been very good at sharing or keeping in touch. Yet what I do know about all these virtual people is mostly insignificant. I often get the impression that most of us share things online to get an immediate response so we don’t feel so alone. We either blow off some steam, post our competing accomplishments or over share out of boredom. I think it’s sad that a Facebook message is often the first source of contact rather than picking up the phone or even sending an email. Maybe it’s the instant gratification we seek since most of us receive immediate alerts on our smart phones and therefore expect just as quick of an answer, a relief from our loneliness. Regardless, I feel shame for not actually knowing the family who lost their 9 month old daughter yesterday and who was in the same online support group as we are. I can’t even presume to understand what they must be going through right now. But what I can relate to are the struggles they went through since their daughter was born. Even though we all search for circumstances that are far worse than our own to make ourselves feel better, and therefore in comparison to other medical challenges most of the CCHS kids live a relatively normal life, the battles all CCHS families face are very similar and simply exhausting. Out of respect for the family who just suffered a loss, the question no one wants to ask now is “Why?” Why did this little girl die, or any of the other CCHS kids before her? Was it a spontaneous prolonged sinus pause which caused her heart to not automatically beat again – my worst fear and one of the major risk factors associated with CCHS? Were there other medical issues that her little body couldn’t cope with anymore? Or maybe it was life support equipment failure? In my mind, I need an explanation to make sense of it all. But I fear no amount of detailed explanation will ever put my mind to rest. I will forever wonder whether this daily fight is worth it in the first place. I will forever question the ethics of anyone living on life support. I will always fear that Wyatt one day will decide against this life and I wouldn’t even be able to argue with him. I will never get the reassurance that saving his life day after day, sometimes 15 times a day, will eventually bring some sort of a reward or reasoning. Therefore, I sometimes envy people who have faith that there is some sort of a big picture to live up to. I’m too factual and practical for that kind of thinking. And although faith and I do not agree, I often wonder if faith-based people have an easier time coping with life’s unanswered questions and challenges. Whatever the case may be, I hope this particular family can forgive me for not supporting them more in their all too familiar daily struggles, and that they can find strength in the memories they shared with their daughter.

No news is good news

No news is good news here. While Wyatt still has the remains of this lingering cold, it doesn’t really seem to bother him much aside from being a bit more sleepy. Everyone else in this family thankfully has finally kicked this bug to the curb.

One thing that is worth mentioning though, especially to the families out there using Keppra, is that we (once again) decided to stop using it. We left the hospital with full intentions to follow through on the twice a day 2.5mL dose at least until we do another EEG in about 6 months. After all, we had no way of knowing whether the Keppra was what actually stopped those silent seizures or whether getting over the trach infection itself was what made that EEG activity stop. From the time we were discharged on September 3^rd until that following Sunday September 8^th we were giving him his dose almost on the dot at 8am and 8pm. We did noticed that Wyatt was really irritable and just not his happy usual self but brushed it off to being the effects of the cold. In retrospect, we should have picked up on it right then that something different was going on because he has never acted that cranky with any of the colds he had previously. Then on that Sunday he was actually so cranky that he threw several back to back breath holding/passing out/blue spell episodes that we ended up having to give him Ativan to make him stop and go to sleep. That was a major red flag because we have not gone to that extreme in over 6 months. Still, we didn’t realize what was causing him to be so cranky until the following day. On Monday September 9th I was so busy with all four kids that I didn’t realize until after bedtime that night that I had totally forgotten to give him any Keppra that day. Ironically, he was happy as can be all day. That’s when the light bulb effect happened and I was curious. He still had the cold so that variable couldn’t be considered as the cause of his crankiness anymore. The only thing that had changed was that he no longer had Keppra in his system. Damn, we should have known and went with our gut feeling in the first place when the suggestion was made to try him on this medication again. This was exactly what happened when we first tried Keppra with him over a year ago. He doesn’t tolerate having any loss of control when he’s drowsy. That’s one of the side effects of this medication and that’s what makes him have increased breath holding episodes. Just to be sure, we went without the medication the next day and the same thing happened. We had our happy go lucky 2 year old kid back who was unstoppable running around all over the place. So once again, we had to conclude that while the intention of medication to reduce his breath holding and therefore seizures was good, it ultimately contributed to more episodes and therefore an increased risk of seizures and we have to say no.

Finally home and almost back to normal

I am having a hard time thinking straight since I now have the full blown cold that has been lingering and making rounds in this house since the beginning of school, back on August 19th. So forgive me if I may miss important facts or simply sound a little out of it.

After Monday’s battle of once again stopping doctors from experimenting on our son trying to fix his breath holding episodes, which we all know cannot be fixed, things finally took a turn for the better by Monday afternoon. We insisted that instead of yet another miserable attempt of holding the kid down to place an IV line to give the second dose of antibiotics, we went with an IM (intra-muscular) injection instead so that we had the best chance at preventing another breath holding event. With Wyatt in our lap and one nurse with a needle in hand on each side, and another person standing in front of him playing Signing Time on the laptop, it was a quick one, two, three, poke, and done. He cried but didn’t hold his breath and just gave a mean face when all was said and done. Neurology was pleased that there was no more seizure activity visible on the EEG but wanted to keep it running to now get a good baseline since Wyatt was starting to act like himself again. Although we were second guessing that approach, we ended up agreeing, under the condition that “if it happens to come off then it stays off”, just to have a good comparison of sick vs. normal crazy Wyatt self. However, once Wyatt totally returned to his normal self and started itching his head, we noticed skin irritation underneath several of the leads. The nurse of course had to first tell the ICU doctor before she could do anything about it. Well, not us, we don’t need a doctor’s order to make decisions so that thing came off before they even had time to think about it and then we were mad at ourselves for pushing the envelope when we instinctively should have known better than to leave that stupid EEG on that long. Well, the blisters will heal but it’s not pretty. Tuesday morning Neurology came in for another chat and seemed a bit more at ease. Wyatt being back to his crazy two year old self, he performed well when asked to show off his developmental skills which are all on task. The Neurologist then said he had spoken with another neurologist up in Chicago who is familiar with the EEGs done on their CCHS patients and she reassured him that it was a normal CCHS thing and as scary as the data and images look, they are just part of the deal. While he still stated his curiosity about wanting to do an arterial blood pressure, he also allowed for a good discussion and accepted our viewpoint. In our book, that’s what makes a good doctor whom we can work with. One who is willing to research and learn about CCHS but also accepts it when we step on the brakes. So the plan is to just do a follow-up appointment with Neurology in 2-3 weeks so that he has time to review Wyatt’s record regarding old EEGs and MRIs and we can then discuss when to move forward with a repeat MRI. Since Wyatt does have an underlying trach infection at this time, we cannot be sure whether the seizure activity stopped because of the start of antibiotics or because of the seizure medication Keppra. Therefore, the plan is to continue the Keppra daily until we do a repeat EEG in about 6 months. Upon news of the second blood work coming back negative on Tuesday, the ICU doctor also concluded that the first positive blood result was a false positive and contaminated and therefore, we were ok to discharge on Tuesday afternoon. In the end, all we know is that this cold that has been lingering in our family for a few weeks now progressed to an infection in Wyatt’s trach, which in turn raised his body temperature to over 99F (for CCHS that’s comparable to a high fever), and it is assumed that the combination of these factors then made Wyatt more susceptible to seizures. Whether or not he would have had the long seizure on Friday without his self-induced breath holding episode, no one knows. But we do know that he has more of those episodes when he is not feeling well. As usual, we dealt with the difficulties at hand and left the hospital without precise answers. Such is the life of CCHS. Once we got back home, it was nothing short of controlled chaos. Wyatt was bouncing off the walls happy to be home and so were his brothers, while Mommy and Daddy’s brains felt fried and we couldn’t form simple sentences. The adrenaline had definitely worn off and rest of that day is kind of a blur. All I clearly remember is that putting Wyatt to bed that night, still with the drama of last Friday in mind and knowing that he almost always throws a breath holding episode in protest when it’s bedtime, that night was the most nerve-wrecking breath holding episode ever. The anticipation of whether or not a seizure would follow made me hold my breath. Thankfully nothing followed and we all got some much needed sleep. We woke up Wednesday morning just hoping for an uneventful, boring day just to catch up and to let our brains recharge. Instead, that lingering cold was throwing punches for Calvin and Mommy, Wyatt still had diarrhea from the antibiotics and probably partially because of this bug that’s going around, and the two older boys had to catch up on school work from last Friday and Tuesday, plus what was scheduled for Wednesday – they’re doing virtual schooling. While it all seemed very hectic and overwhelming at times, that routine stress was far easier to manage than the hospital stress.

Since today is Thursday, I think, I am happy to report that we are all alive and for the most part well. So cheers to this f… cold leaving this house by the end of this weekend.

Here we go again

My timing on the last blog post must have been some sort of premonition. As with every hospitalization, there always seems to come a point when doctors stray away from the original reason of the admission, this time due to a blood and trach infection, to a sudden interest in Wyatt’s breath holding abilities. And then from thereon out it always turns into a snowball effect that brings out the most stubborn scientists in even the best doctors. Don’t get me wrong, I am scientifically minded also and I wish there were a concrete answer and a set treatment plan for Wyatt’s breath holding episodes because those are the most difficult part to deal with daily. And it certainly tends to start out well when a new doctor joints the team of fascinated spectators of the Wyatt Olympic Breath Holding Event. But when you stare at data long enough it can get the best of you, suck you right into the narrow medical analysis mode, and you fail to see the patient side of things. Basically what happened is this: The EGG showed what we, as his parents, already knew. There are no signs that anything medical is causing Wyatt to hold his breath. Once he starts the breath holding, his heart rate slightly starts to increase (opposite of what would be expected in a person with a normal autonomic nervous system response) and his EEG waves start to become more erratic while his oxygen saturations drop and he stiffens up and turns blue. Once he actually passes out and relaxes, that’s when his heart rate drops for a few seconds before climbing back up, his oxygen saturations climb back into the normal range also but his EEG waves actually give a near flat line while he comes back to himself and opens his eyes. The near flat presentations of the EEG waves for those 15-25 seconds while Wyatt wakes back up out of the breath holding episode is what set off major alarms in the mind of the new Neurologist of course. Then that once again started the conversation of the possible risks of brain damage with each episode and the need to investigate the sequence of events further to be able to medicate appropriately. Oh boy did that open a can of worms again that I am so tired of discussing. What was unclear in the EEG is Wyatt’s blood pressure response during such an event. Therefore, the scientists came out and suggested to go ahead with a procedure to check Wyatt’s arterial blood pressure. To do so, he would first need to be sedated to place such a device and then Wyatt would need to have another breath holding event to get any real data. Let’s pause here for a second.  If we’re so worried about the potential damage to be done by his breath holding and passing out, why are we suggesting tests that require us to make him mad enough to cause him to hold his breath? Are we the only ones who have a problem with that contradicting approach? So the good doctor’s reasoning, while well intentioned, is that even if we can’t prevent the breath holding, maybe we can gather enough data to map out the sequence of events that happen as a side effect of the breath holding (heart rate climbing, falling, then climbing plus the unknown blood pressure response and near flat EEG waves), and then maybe we can give a daily maintenance medication to at least minimize these side effects. Again, so we want to intentionally cause more episodes, so that we may or may not get a better understanding of the potential risks, by continuing a bunch of tests that ultimately increase the risk of long term consequences by intentionally causing what is supposed to be avoided? Aside from feeding into the good doctor’s curiosity, I can’t see a good reason to turn my child into a guinea pig. As with every new doctor that joins team Wyatt, we have once again reached the point of trying to diagnose and cure his breath holding episodes. Let’s pause here again. Just as a reminder, the reason Wyatt had an episode on Friday that started this whole mess was that I told him to finish the food in his mouth first before he could have another bite. That’s an intentionally behavior, a two year old’s temper tantrum. But just for giggles, let’s hypothetically go with the idea of wanting to stop his episodes altogether because of the risk factors involved. There would be two ways to go about it. One, Wyatt rules and we always give in to everything he wants when he wants it and how he wants just so we don’t tick him off. Or our second option is to give him daily medication that keeps him drugged up enough to not care about anything which then also means he wouldn’t be able to function properly, progress developmentally or learn how to deal with life’s frustrations. Given these two options, I’d rather acknowledge the risks but raise a child who’s mind is not numb from daily medication so that he has a fair shot at developmentally staying on track and learning that life sometimes just simply sucks and you don’t always get it your way. So we once again sounded like a broken record and made it clear that we weren’t in the hospital right now to find a cure or to gather data. We were there to treat the acute illness and nothing more. So sorry doctors but you may not be the superheroes that fly in and fix everything because the thing you are trying to fix isn’t fixable. We choose to accept Wyatt for who he is even with all his brain farts as I call them and any potential future challenges, and we choose not to run every imaginable precautionary test simply out of fear of the unknown.

So I guess this is our imaginary line that we are not willing to cross.

Where is your line?

As I am sitting here in this intensive care hospital room and take a look around, it all seems like a scene from the series ER. I see a two year old boy lying in a crib who is mostly unfazed by anything going on around him, who is disinterested in eating or drinking, has 20 wires hooked up to his head all bundled up into a neat head bandage leading to a big computer screen and camera that are recording his every brain wave and movement.  Then there is another set of leads hooked up to different parts of his body and a different monitor continuously checking all his vital signs. And then of course, there is this big blue box standing next to the crib with a bunch of overwhelming ever-changing numbers and tubes attached that lead directly to that boy’s trach making it possible for him to breathe. There are various other medical supplies and emergency equipment set up around the crib and various people in multicolored scrubs keep going in and out of the room writing down numbers, checking on the many wires, administering medication, and throwing out educated guesses trying to analyze the problem at hand with fancy medical terminology. This is the kind of stuff that most parents worst nightmares are made of. For us, this is the reality we have been living with for over two years now. Nothing could have ever prepared us for this and never will we be completely prepared for what might be ahead. Yet on the good days, you try your hardest to enjoy the calm waters. And just when you think you have reached some sort of a comfort level and routine, something else knocks you right back down. After two years, you shouldn’t be surprised by this sudden change of events anymore but somehow it still catches you off guard.

I have to let some thoughts out of my head right now that will raise eyebrows, so be forewarned.

Every medical situation is different and even among patients with the same disorder, there are a lot of variables that can make for quite a list of possible presentations and therefore management approaches. When you hit bottom due to a medical complication and you think you can’t take anymore, it is human nature to start to compare. In the ICU you may look around and wonder what other patients were admitted for and whether, in your mind, that situation would be easier or harder to deal with. In all fairness, this mental assessment is totally subjective as your opinion of easier or harder is solely based on your own personal experiences and you cannot possible presume to know what it is like to live in someone else’s shoes. Still, comparisons have a strange comforting effect. Likewise, they can also make you imagine worst case scenarios, again very subjective to your own imagination and experience. Whenever Wyatt hits a challenge that requires hospitalization, I always run into this wall of asking myself where the line is. My beliefs and expectations of life have been challenged on so many occasions, it is hard to distinguish which ones are scientifically based and which are emotionally driven. I recently read a blog post that examined the difference between the pro life activists and the true meaning of pro life compared to the socially accepted definition of only including issues related to abortion laws. It was fascinating to me that the writer pointed out that there is much attention given to the seemingly never-ending discussion over the value of the life of an unborn child, yet hardly ever is there mention of the value of life of a child born with a physical or mental disability. That blog post questions everyone’s subjective stand on the overall value of life, or quality of life as some may call it. So where does this fit into my never-ending struggle to redefine my beliefs? Well, it’s fascinating food for thought but doesn’t help the dilemma. As a scientifically minded person, I usually take the stand of not messing with nature whenever possible. I feel that medical advances and technology are a double edged sword. Survival of the fittest is a principle I strongly belief in. I believe that medical technology and advances get in the way of that principle and the overwhelming human population and all the challenges that come with it are the consequence. With that in mind however, I also realize that medical technology and advances are based on not only scientific curiosity but primarily driven by some form of emotional attachment. I often ask myself, knowing what I know now two years into having a life support dependent son, would I be(true) pro life? If I answer this question from an emotional standpoint, the answer would be a clear yes. With the right support system it is certainly possible to lead a meaningful and fulfilling life despite the challenges. However, if I didn’t have the emotional attachment due to the person I am referring to being my own son, I would most definitely advice against such a challenging and stressful life. In fact, just to make it clear how deeply rooting this belief is, if I was the one on life support depending on other people to take care of me, I would not want to live like that and I would seek legal action to establish my right to choose.  So that then begs the question of why as humans we tend to question our own beliefs when it comes to the lives of our children? Are we truly acting in their best interest by keeping them alive? Should we embrace medical advances and technology at least long enough to eventually let them choose for themselves what they consider a life worth living and true quality of life? Or are we just selfish, unable to let go of the person who we have become emotionally invested in, unable to bare the thought of letting go? Where is the line? As hard as it is to answer any of these questions clearly, what are we to do when the next hurdle requires even more medical intervention and possible technology? When is it too much? When does the emotional attachment outweigh logical thinking to even ask the previous questions? Remember, all these questions and potential answers are all very personal and therefore highly subjective.  There is no guide book that you can buy to point you in the right direction. You can only make decisions in that moment of time as they are required to be made and only with the perceived knowledge that you have at that same instance. As human being, we will always use retrospect to doubt our own decisions and if we’re smart enough we will realize that most of the time, there are no clear cut right or wrong answers. But what is one to do when that decision could be the difference between life and death? Is ventilator dependency crossing the line for you? What about mental retardation? How about cancer? What statistical survival rate subjectively justifies the side effects of cancer treatments? What if someone you know had been in a car accident and hasn’t woken up out of a coma in 3 weeks? How long would you wait before you can emotionally justify pulling the plug or would you cling on to the news stories of miraculous awakening and if so, for how long? What about kids who were paralyzed from the neck down unable to physically do any daily tasks by themselves and requiring a ventilator to breathe? Or to keep it even simpler, how do you decide which preventative tests to do and how often?... Where is the line for you?

Most people don’t give these kinds of questions too much thought because they are just too sad to think about and emotionally draining. And they naively assume it will never happen to them. Well, that is the kind of a mental game that living with CCHS or any other so to speak “abnormal” medical challenge brings along. Most affected families would never admit these thoughts publically yet they linger all too often and resurface whenever a new medical challenge presents itself.