Life as I know it now

I know, I know. I’ve been really slacking on writing blog posts. To be honest, I simply didn’t feel like talking about Wyatt anymore. It’s such a bitter sweet tool. This blog started out as my own personal therapy session. I could put in writing any and every thought that crossed my mind. I didn’t have to hold back and it allowed me to process all the ups and downs of this thing called CCHS. While this blog certainly has served its purpose and today I even hope that this naked truth of my emotional roller coaster ride can help someone is this world, it has now become a tool for me that can also easily backfire.

When Wyatt was first diagnosed, all the other families affected by CCHS told us, the first year is the hardest and then it gets easier. What no one told us was why. So here’s my viewpoint so far having surpasses that one year mark by a couple of months now. I think the term easier is misleading. Having a healthy infant is hard. We have to get to know that new person, their likes and dislikes, their personality and what life changes that will entail. As exhausting and sometimes isolating as it may be, every new accomplishment of that perfect little human being seems worth a celebration and we can’t wait to see when the next milestone is going to be reached. Having kids turns us into this whole new person who almost overnight becomes less concerned with their own personal goals and suddenly feels the urge to tackle the world in the best interest of their child. Having any subsequent children, we start to have less and less time focusing on all the little accomplishments and shift more towards tackling the bigger battles such as what school to choose. Now consider having a child like Wyatt in the mix of that already challenging balancing act. Initially when we got the diagnosis, it almost felt like a relief to me because at least the medical problems that remained a mystery for so long finally somewhat made sense. Somehow putting a name to it made it all just a little bit easier to deal with. While waiting for several weeks for the test results of course, I had already done my research on what such a diagnosis would mean and what future medical options we might have. I felt prepared even though my mind played tricks on me every second of every day wishing for a medical answer yet hoping I’ll magically wake up from this nightmare. Once we got the answer that we were and were not hoping for, I went even further into overdrive mode. Now the practical aspect kicks in of learning everything there medically is to know so that I can adequately take care of the daily needs of this special child. There wasn’t really any time to reflect and process what’s going on. The complete load of that freight train of emotions hit me much later after we were already settle in at home for several months. One day something seemingly unimportant just opened the flood gates. Suddenly I realized, to no fault of my own but simply out of necessity, I was so busy juggling the medical needs of this one special child and chasing the next hopeful treatment option that I didn’t really even know who that person who’s living in our house attached to all the medical equipment was. I suddenly realized how much time taking care of that one special child has led to me missing valuable time with our other children, how much we all had to give up to be where we are today. The first year is the hardest not because of all the medical reasons. Sure, those are challenging and come with their own up and own emotions. But what was the hardest for me was to take the time to grief the loss of my hopes for my family’s future and accepting what is, the unknown. I had to learn to accept that doctors are not the heroes with the magical answers and cures like most of us were taught when we were young. With such a rare and not well understood diagnosis with an unclear medical treatment plan, doctors don’t know anything. In fact they are even scared of your child and we as the parents are all alone in making the right medical and non-medical decisions. Looking that fear in the eye and believing that the unknown and the loss of control are not my worst enemies, that took time. Hell, I still fight the battle in my own head sometimes.  That’s what makes the first year the hardest. The first year was very isolating because we were just treating water and didn’t even realize that we were losing  a part of ourselves. If I was not dealing with insurance, medical equipment or yet another medical challenge, I was busy contacting every researcher and other affected family. While it is great to have those contacts and that support system, I’ve also noticed that because of my personality it can become a downward spiral. Not even the most experienced specialists nor the CCHS families who have been living with this condition the longest have clear cut answers. Just like a healthy infant, each child is so vastly different. I had to learn to stop looking for answers and stop looking at my child medically and stop over-analyzing every move he makes. Instead I had to pay attention to who he is as a person and how we as a family could repair that unintentional disconnect that slowly marched in between us over the last year. In my darkest days I realized that I will not be a good mother to any of my children nor a good wife to my amazing husband if I only focus on life with CCHS. Since I am an all or nothing type of person though, that meant I had to let go of doing research and making treatment plans which is the complete contrary to my hard to break habit of always having to have a plan. It takes a huge amount of effort for me to simply live in the moment and not make comparisons to the way things should have been. Coming to that realization and taking that dive into the deep also known as leaving my own comfort zone, that’s what made the first year so incredible hard for me.

I can’t say that I have succeeded in putting that realization into action just yet but it’s work in progress. This is the reason why I haven’t been posting on here much anymore. I have to make the conscious effort to get to know my four children all over again for who they are today. I have to ask out loud for quality time with my husband so we can get to know each other all over again in the middle of our new life together. I also have to speak up and demand time to myself so I can figure out who I am now instead of trying to chase down who I used to be. So here I am making the first step.

To all my friends and family out there: I know you care and I am sorry for previously turning down your efforts to help. I’m not good at asking or accepting help, nor do I even know exactly what kind of help I could use. I will work on that. It must be difficult for someone on the outside to know what to say or do. Sometimes the best help is to simply be there. Even if it hardly ever works out, please don’t give up on offering to include me or my husband in get-togethers. Don’t worry about how much effort it would be for us, suggesting a play date or a cookout is still a good idea. Discussing parenting or career issues is actually nice because in the end we all face similar difficulties.  So let’s face the world together again!

stoma reconstruction surgery with a surprise find

On Thursday, July 12^th, Wyatt was admitted to the hospital for a scheduled stoma reconstruction surgery to remove the access skin growth around his stoma site and to close off the additional stoma opening. Now post-op and sedated in the PICU, we are glad we had the surgery done now rather than later. It turned out that right under his vocal cords there was scar tissue that had grown from the inside and up his airway. That scar tissue had gotten so big that it was blocking any air from going through around his trach. Aside from the obvious concerns that this blockage could have caused a sudden serious issue with trach changes one day and ventilation by mouth may not be possible, this find explains why Wyatt used to be able to vocalize much better several months ago and lost his ability to breathe through his mouth and nose almost completely. We assumed it was due to his trach upsize, meaning a wider trach would leave less room for air to pass by his trach through his vocal cords and out his mouth and nose. While this unfortunately means that the surgery ended up being a completely stoma reconstruction and therefore has to be treated like a new tracheotomy in regard to recovery, finding and removing this blockage opens up the possibility again of better vocalization and therefore, less frustration and possibly even less blue spells for Wyatt. I’m not betting money on the last part but simply hearing him snore when he’s sleeping now brings tears to my eyes. Snoring means he can make noise. I’d give anything to hear him laugh or even cry and I will break all parenting rules and let him curse on occasion if he will just learn to talk.

Looking down his airway during surgery, this is what they found... Don't worry, I covered up the gross pictures of the actual reconstruction portion of the surgery.

bye, bye nursing

So after numerous discussions over the weekend, we cancelled nursing for good. We might have to revisit that dreaded idea again when we decide on daycare for Wyatt but that's still 2+ years away. Instead of being upset or frustrated, we have always been excited when a nurse would call out sick or the agency couldn’t find coverage. I don’t think my personality is made for home nursing and when I’m stressed Wyatt is stressed. It’s just a downward spiral. I have always noticed a night and day difference between the morning hours for example when we had a nurse and the afternoon or weekends when we didn’t have a nurse present. Not only does the nursing agency/FL Medicaid put restrictions on what Wyatt can do, where he can go and when but I recognized that having nursing made me treat Wyatt differently, too. I don’t want him growing up defined by nursing. I don’t want him to start to believe that he can’t do certain things or that he needs constant supervision to do anything. He will know his limitations and may have to make modifications but I want him to know that he can do anything he want to do, whenever he want to do it. He can and should be just as independent as his brothers and I cannot stand for anything less. We haven't had a nurse since Friday and our entire family including Wyatt has been so much more relaxed and acting normal around each other again. No more house arrest for Wyatt (stupid FL Medicaid rules). We took Wyatt grocery shopping and to Grandma and Grandpa’s house. We took him to the park/playground and let him swing and go down the slide for the first time. We sat him in the kiddy pool on our back porch. We have been successfully sprinting him (taking him off the ventilator to let him breathe on his own while awake) several times a day for 30min-1hour at a time. And the best, most amazing and reassuring part is, we did all that without a single breath holding cyanotic episode. Just since Friday, since we haven’t had a nurse, he has even greatly reduced the number of episodes when it’s nap time and now settles down on his own and even just. That is so huge, I can’t even find words powerful enough to explain how big of an accomplishment, how big of a difference that is. This boy just needed his freedom and get out into the world. No more explaining to nurses and the nurse case manager why/when we spint Wyatt and why we don’t have an official doctor’s order for it to cover liability for the nursing agency. No more explaining why we won’t feed Wyatt exactly 150mL every 3 hours on the dot. No more making endless phone call to update everybody and their brother on Wyatt’s latest appointment schedule, new orders and discussions with doctors. No more overanalyzing every little fussy moment or changed ventilator need as being medically related without considering normal growing discomforts. No more treating our son like he’s sick and holding him back from doing normal growing up kid things. No more of that. What Wyatt does, how and when is back to being a family business only.

I wasn't sure of the cold water at first but boy, splashing everyone sure is fun.

I better hang on to dear life, Mommy might get too excited.

Crawling is optional when you can just stand?!

Better not drop me. I'm hanging on to my vent circuit just to be safe.

upcoming stoma reconstruction surgery

Wyatt’s stoma reconstruction surgery is scheduled for July 12^th and will require a 3-5 days post-op hospital stay.

The enlarged skin growth visible in the picture was caused by a suture that cut his skin when he had his tracheotomy placed in July of last year. That tear in his skin created a larger than intended stoma meaning there is an extra opening behind that skin growth. ENT refers to it as a panhandle shaped stoma. In other words, a normal stoma is supposed to be round but Wyatt has a figure 8 stoma. During the reconstruction, they will ventilate Wyatt via intubation to get a clear view of the stoma. They will try to save as much of the good tissue as possible but will remove the excess skin growths and tighten up the stoma to make it round. The expected post-op procedures and discomfort level will be equal to having a new tracheotomy. Sedation will most likely be necessary for a few days post-op to prevent numerous cyanotic breath holding episodes caused by discomfort.

While we certainly don’t want to put Wyatt through another surgery, the enlarged stoma has only gotten bigger as he’s been growing. The air leak is getting worse making it impossible to know how well ventilated he truly is because we can’t be certain how accurate his daily reading are, especially his CO2 readings. The skin growth has gotten bigger over time as well and is often irritated. He has also been decannulating more frequently doing simple things like moving his head or coughing. We were giving it time to let him get bigger and more resilient but it’s simply not safe anymore and needs to be fixed. Our hope is that after the recovery period is over, he will be a lot more comfortable and we will be able to assess his ventilation more accurately. Once the air leak around his stoma has been minimized, he should be able to vocalize more again as well like he used to – fingers crossed.

seriously???

Alright CCHS family, I have to vent about my home nursing dilemma and I’m hoping you guys might be able to offer some advice. I’m so frustrated with nursing lately that I went through phases of anger, guilt and holding back tears today. Quick backstory.

When Wyatt was discharged from the NICU at three months old back in September 2011 we went home with nursing. We had a few good nurses but also had some experiences we could have done without. After some issues with the business ethics of the first home nursing agency, we switched to a new agency in November in a last ditch effort to give it another try. Even though the second agency was providing far better coverage and better overall service, by December we still went from needing a one week break from nursing to cancelling services altogether. And then came the Chicago visit. We were given the speech by doctors that by declining nursing services we were not acting in our son’s best interest and were not providing optimal care and safety. So after some soul searching we decided that maybe due to the negative business experience with the first nursing agency we didn’t give the second agency a fair shot and we agreed to start nursing back up at the end of February.

Well, here we are now and we are back to the same issues that bothered us about nursing from the very beginning. Aside from having a hard time finding good nurses who are willing to learn about CCHS and who can handle the unpredictability of this disorder, we’ve always felt uneasy about nursing for various reasons. From December to February our whole family seemed so much more relaxed and together without nursing. The obvious bumps in the road that come with home nursing are the loss of privacy and having someone else take care of your child. That never sat right with me and probably never will. My husband and I are both very involved in our son’s care and we don’t see it as burden. Of course, there are days when we get frustrated and we’re tired but we don’t consider it to be any more challenging than raising any other infant or toddler. We have always approached Wyatt’s care as family affair. In fact, one of my conditions of agreeing to return to home nursing was that we do not live our life depending on nursing coverage to make any plans such as doctor appointments, and that I will no longer make several phone calls each week to keep every case manager and nurse informed of ongoing treatment or medical order changes. I absolutely hate having to ask for a doctor’s order for everything we do with Wyatt. We made it very clear to the nursing agency as well as every individual nurse that comes into our home that either myself or my husband will always be there and that the nurse is only present as a second set of hands, and only in rare and brief occasions will the nurse get to take over complete care. Well, based on recent conversations with our nursing case manager, our outspoken and very involved approach seems to be limiting the agency’s selection of nurses that they consider a good fit for Wyatt’s case. Another recently discussed issue has been when and where a nurse can accompany Wyatt. Since our private duty nursing coverage is paid for through Medicaid, their guideline state that a nurse can only provide care within the home and to/from doctor’s appointments. We are expected to notify the agency every time we are taking Wyatt anywhere outside of the home and ideally the nurse would be cancelled in advance for that shift. I basically told them that these restrictions make home nursing pointless in Wyatt’s case. We the parents provide his care and having him on house arrest is not helping his social, physical or mental development. If we can’t have nurses sit with him in the backseat of the car for example when we want to go to the park, then what’s the point of having nurses? It’s hard enough living around nursing shifts but to have to ask for permission when and where I can take my son is just an unacceptable flaw in the Medicaid system. I want Wyatt to grow up to be independent and all he is learning so far is what he can’t do. I’m afraid eventually that is going to lead to him believing that he can’t do certain things or at least not without help. That’s bullshit. That is not how I raised any of my other kids and Wyatt will be no different. To top it off, our daytime nurse has been pushing my buttons lately by simply sitting out her shift at our house. She was great in February when she first started with Wyatt but has since lost that original enthusiasm. Wyatt doesn’t want to be near her now when he’s cranky so she has gotten more and more frustrated and I in turn have taken over even more to prevent Wyatt’s endless breath holding episodes. I have tried to explain to the nurse that this is normal baby behavior and that her getting frustrated is only raising the level of tension which Wyatt easily picks up on and therefore only makes matters worse. I have even given her hints on what to try to do to earn his trust but nothing has been working. Worse, over the last few weeks her frustration has led to her distancing herself to the point of slacking on her nursing duties. I even had to talk with the nursing case manager about the fact that she hasn’t been doing CO2 checks despite me telling her repeatedly that they have to be done. She also hasn’t taken any of our instructions on how to look for Wyatt’s cues to prevent episodes and when and how to intervene. When I spoke with the nursing case manager again today, I told her straight out that it seems that this nurse just doesn’t care anymore and no conversation is going to help that. I am done with her sighs and me explaining over and over again what to look out for, yet when I put tough love to use with Wyatt when I try to get to fall asleep by himself for example, she just sits there and plays with her phone instead of observing what I am doing to try to get to know his likes and dislikes better and to start picking up on his cues sooner. I told the case manager I cannot make someone love their job and I cannot teach her to want to get to know my son’s personality. She tried to tell me that during his four hour nap she couldn’t get access to do a CO2 check because he was sleeping on his stomach. She gave me that excuse even though I showed you several times how to do it and I even set it up for her and left the inline co2 monitor part in place already. But she couldn’t get to it in four hours? That’s called not caring. To push matters over the edge though ,the nursing case manager calls me back later today and says that she discussed these issue with the nurse and that the nurse said I am taking away her independence to take care of Wyatt. The nurse said to the case manager that she feels I have been increasingly frustrated, I am acting as if I don’t want nursing and that I am taking over more and more of Wyatt’s care and therefore she is unsure what I want her to do during her shift. Seriously?  Yes, I am taking over more and more because I can predict and prevent cyanotic episodes before alarms sound, whereas the nurse hasn’t applied any of our instructions and suggestions, she doesn’t do shit until a machine alarms at which point it is too late to prevent the cyanotic episode. So, excuse me for not just sitting back and watching her put my son through episode after episode. This is not like the first day of daycare where the child throws a tantrum and then gets used to it after a couple hours. We are talking about a child who does not know how to cry, who faces very real risks of further brain function compromise with each cyanotic breath holding episode. So no, I will not just hand him over to the nurse and put him through this, crossing my fingers that in a few days, a few dozen episodes later, he will simply get over it. Then the nursing case manager proceeded to ask me if I want that nurse to return tomorrow and simply shadow everything I do with Wyatt to give it another try. Their concern is that if I take this nurse of my son’s case then we have no coverage at all. No thanks, I don’t want coming back tomorrow because I cannot bite my tongue at this point. I have no trust left for this nurse and I have no patience left to be civil if she returns. I am perfectly capable of taking care of Wyatt and I don’t want her to stay on his case only to keep the agency out of hot water. Long story short, coming from the nursing agency, I’m one wrongly phrased word away from canceling home nursing altogether again. Based on my personality alone, I know I will never be comfortable with depending on or reporting to someone else about my son’s care. No one is ever going to know him as well as we do and no nurse will ever have the same sincere selfless drive for Wyatt’s best interest and safety as we do. I am so angry right now that I even have to put up with this and listen to these comments. I am angry at doctors for pushing home nursing on us when our gut feeling from the very beginning told us that it wouldn’t be the right decision for our family. I am angry that stupid bullshit like this still rocks me to my core and makes me doubt my parenting choices for my special needs child. I am furious at comments that indicate that a special needs child should be treated differently and that a special needs child should take advantage of special programs. Just because these programs and assistance like home nursing exists, doesn’t automatically mean it is the right or best choice for every case. It is not that simple. In the end it comes down to the parents deciding what they want for their own child’s care and future, how they want to raise their child and what works best for the child’s personality. I will not stand for being told how to raise my child or how much care to provide for him myself… I am not sure that after all this nonsense and unnecessary stress, I am even willing to start looking for another “good fit” nurse again. I think the nursing agency and this one nurse might be right about one thing, I don’t want nursing for my son.

success and revelations

I know, I know. I’ve been slacking with my blog posts lately. Honestly, I wasn’t sure what to write and I’ve been too tired to give it too much thought. And I don’t want to talk about the latest annoyances with home nursing, Wyatt's continuous stubbornness hindering the oral feeding and sprinting progress, the ongoing struggles with Wyatt’s blue spells and now temper tantrums leading to even more blue spells, or the promising yet disappointing new CO2Monitor. I feel like a broken record on these subjects. Let’s talk about something positive…

Well, last weekend my husband and I dared to get away for a couple days and we handed all the responsibility for all the kids, including Wyatt, over to Grandma and Grandpa. We picked the two older boys up from school a little early and were hoping to get out of town by 3pm when our nurse was scheduled to leave. Well, we underestimated the challenge of transporting everyone and everything for the short 3 mile car ride to Grandma and Grandpa’s house. Even our new minivan can’t fit six people, plus three days worth of supplies and equipment. So after a couple trips back and forth and everyone and everything was unloaded, we ended up just throwing our own luggage together and manage to leave by 5pm. It was a smooth ride after that and the two of us finally had random small talk again. This may seem boring and meaningless to most of you but to us, it is such a relief from the usual short and sweet dialog evolving only around the latest medical updates and kids related news. Since this was the first time that we went away overnight and considering it would have taken us a good 3 hours to drive back home if something were to happen, it was a bit nerve-wrecking. After all, this was a test for all of us. Wyatt is very clingy and we all didn’t know how he would react (a.k.a. how many episodes) especially when it was time to go to sleep. If he could handle staying overnight at Grandma and Grandpa’s, that would mean that we have found a greater state of normal again and it opens up the possibility of all the kids being able to have sleep-over every now and then instead of Wyatt being the only one excluded. Long story short, the weekend was a success!

The reality of what we all have been through in the last 11months definitely hit last Tuesday when I had a parent teacher conference with my 7 year old son’s 1^st grade teacher. I walked into the conference with the urge to apologize for this being the first year ever that I haven’t been involved in my kids’ education. I feel so much guilt for not knowing what is going on at school for my 10 year old and 7 year old. Most weeks I couldn’t even tell you what their homework was, whether it got done or how they did on exams. I knew they were doing well based on their progress reports and report cards but we’ve been so busy at home that it often went unnoticed how much responsibility and trust we placed on them. Before I could even say anything at all to my son’s teacher she started out with complimenting my son on how polite and responsible and smart he is. I wanted to cry. He managed to be the best student in his class despite coming home to having to help take care of his 3 year old brother every day and having to learn to handle emergency situations with Wyatt. I can’t even express how proud I am of all my kids for taking life as it is and just rolling with it. My goal and my expectations have always been to raise my kids from an early age to be responsible, independent and well-mannered. Never could I have imagined that they would learn these skills from their youngest brother Wyatt.

the balance of life

Anyone who has ever had a bad cold knows there are a few days when you simply feel like you were hit by a freight train and you may even utter the words “I feel like I’m dying”. Any parents knows that watching your child suffer from a bad cold is simply torture even if it is the best snuggle time in the world. Now imagine the possibility of dying from a simple cold to be very real and the person affected is your 10month old child. That’s the cards we’ve been dealt here. The last week, and especially the last three days, has been nothing short of hell. It’s bad enough when your child has a rare genetic disorder that makes him dependent on a life support machine because his own breathing capabilities are simply not sufficient enough when awake and are lacking completely when sleeping. Having a bad cold however drives that fact home just a bit more as if we needed a reminder how fragile he can be with any minor illness. This cold is taking away his awake spontaneous breathing altogether. The simple action of adding water to the ventilator’s humidification chamber, which requires us to temporarily disconnect him from the ventilator for just a few seconds, makes him turn blue so quickly that even the pulse oximeter can’t catch up and get an accurate timely reading. He is so miserable and in so much pain thanks to a nasty cough and sore throat that he constantly tenses his body up. Crying or not, that overall body tension even causes his airway to tighten up so that the breaths provided by the ventilator are sometimes insufficient and we have had to ambu bag him for hours to get enough volume into his lungs to keep the co2 down and the blood oxygen level up. Despite holding him continuously, he was so uncomfortable two days ago that he just couldn’t take daytime naps and we had to give him Ativan, a sedative, to allow him to get somewhat of a restful night’s sleep. Yesterday, nothing would calm him down and the ambu bagging was going on for hours. And since our nurse was out sick too, picture me sitting on the sofa with Wyatt lying on my chest. I’m holding him with my left arm and ambu bagging him with my right arm while timing out each manual breath so that I can still silence alarms and turn on his feeding pump. In the mean time, my three year old is bouncing around on the sofa next to me begging for my attention and all he wants to do is play with Wyatt. Priorities, ensure your 10month old keeps breathing or making your three year old a sandwich? You learn how to multitask very quickly and thankfully we have plenty of handy snacks around fulfilling the three year old’s need for independence… We were running out of options quickly with Wyatt because he was already on 3L O2, increase ventilator settings and still he kept desatting and throwing episodes.  We knew that if we have him admitted to the hospital they would just stick him with an IV which requires several attempts since he is a very hard stick and then they’d sedate him for a few days until he’s better. Well, we practically have a home PICU, are now thoroughly trained in all sorts of emergencies regarding Wyatt, know more about Wyatt’s specific needs than I could ever explain to a doctor in just one day,  and we can administer sedation through his G-tube without getting him more stressed out being surrounded by doctors and nurses. So as much of a guilt trip as this is, we knew we had to basically knock him out with sedation for a few days to let his body rest and not compromise his breathing/oxygenation any further. Since Ativan has shown mixed results with Wyatt when given more than one day in a row and we used up our last dose the previous night, Pulmonology called in a script for Chloral Hydrate. Supposedly the drowsiness side effects wouldn’t be as long lasting with Chloral Hydrate vs. the Ativan and it would only take the edge of and make him a bit more relaxed. So we gave Chloral Hydrate a try hoping it would work better than Ativan with less side effects. Holy Coconuts, what a messed up drug that is. You could definitely tell when it was starting to kick in because his eyes were losing focus. But then imagine the incredible hunk stuck in a 10month old body without any control or coordination over his arms or legs. He was fighting the drugs so bad and getting so angry. He wanted to cry but the sedation wouldn’t let him. It took over 15 minutes for him to finally give up fighting but he still managed to throw a few episodes before then. Even after he fell asleep though, he was restless. And when he woke up after only 1 ½ hours of sleep, he was frantically grabbing things to chew on. It was insane. While I don’t like Ativan either, I think I definitely prefer it over Chloral Hydrate. Either way, it seems so wrong to be putting your own child through all this even when you know, the alternative would be worse. How I wish Wyatt would just be able to cry all day like you and me can. I would pay for that annoyance over this life or death scenario.

short update

I know I am not usually one to be short and sweet on here but a lovely cold is kicking my butt right now so here’s just a quick update.

We met with ENT (Ear, Nose & Throat specialist) on Friday and have decided that while Wyatt’s stoma site is functional from a ventilation standpoint, it is definitely not normal and needs to be reconstructed at some point. Doing the procedure has its drawbacks of course as it will be similar to having a new trach in regard to cleaning and recovery time and it will require 3-4 days of hospitalization post surgery. However, the current “Panhandle” shaped stoma site (vs a normal round appearance) is creating an air leak that shouldn’t be there and the two areas of additional skin growth around his stoma site often get irritated. The way the procedure works is that after anesthesia kicks in, they would intubate him through the mouth to get a clear view of the stoma. They would then use the existing stoma but tighten up the area around it as needed, preserve the good tissue as much as possible and remove the excess skin growths. After surgery of course, there will be stitches again and the first trach change I would presume has to be done by ENT before discharge. There is no urgency to doing the procedure but we feel strongly that it needs to be done sooner rather than later and having the excess skin removed should provide more comfort to Wyatt and reduce the risk of infection and inflammation. The lead time is approximately 4-6 weeks so most likely it won’t get scheduled until June. Since Wyatt also needs his yearly MRI of the abdomen and chest done with sedation, I’m going to try my best to push the hospital to do the MRI during that same hospital stay.

Some other updates… While Wyatt has had some prolonged episodes that ended with seizure like symptoms on occasion, probably due to the prolonged lack of O2 to the brain, I do have some great news. Purely by accident I discovered a way to prevent 90% of his episodes. We had already figured out that most of his episodes are behavior linked by now. So one day I got so frustrated with him knowing he was doing it on purpose and he had already shown me two back to back full blown episodes that I decided screw this, he’s not going to rule this house. So I started bagging him as soon as he started to cry and made that face like he’s trying to stiffen up and throw yet another episode. And what do you know, that little stinker stopped crying. His skin still turned a little blue but he didn’t go below 60s on his SATs, whereas his bad episodes can go well into the 10s, even to the point of reading 0. So that was a huge victory for Mommy and an eye opener that we had finally found a way to win this battle of willpower with him. The game was on from that point forward. Now he has to go to bed and settle down by himself even if it means we are bagging him for an hour to prevent episodes before he finally gives up and goes to sleep. That look on his face was priceless the first time we laid down the law and put him to bed still awake. He gave us such a pity look. He knew exactly what he was doing and we had to prove to him that we are just as stubborn. Two weeks into this method of madness, he is getting the point and even has had days now when he will go to sleep in his own bed without a fight. That said however, the stranger anxiety is in full swing here though. He will not go to anyone else but me and my husband right now. We can’t dare have a nurse sit next to him without one of holding him and he sometimes even throws a fit for Grandma. I hope that phase passes fast because it’s rather annoying. And just to show how much Wyatt needs to be in charge, yesterday he proved that when he wants to he can eat by mouth just fine. After all the struggles and failed attempts to get him to eat by mouth over that last few months, yesterday he completely voluntary took 20 bites of baby food from a spoon by opening his mouth on his own and even signing “more”. He had never done that before. And he did all that despite the fact that he’s fighting the same cold as me right now. Wyatt, Wyatt, Wyatt. I can’t wait to see what a handful you will be once you can actually walk and sign a few more words.

Back to bitching

Alright, my last few posts have been quite cheerful and while I have been able to handle the daily stress a bit better lately, I have to get back to my reason for keeping this blog – therapy. So let’s get back to today’s episode of “What’s annoying her now?”

Today’s answer is home nursing. It’s been a little over a month now that we have reenlisted the help of nurses to assist with Wyatt’s daily needs. We are approved for 16 hours a day and chose to apply those hours to daytime care only. We have previously established that night nursing is more of a hassle than actual help to us because Wyatt sleeps so much better with us in our room. It just didn’t work out for our family to have Wyatt be watched by a nurse at night in his own room when it only led to him getting woken up frequently, then thinking it’s party time for several hours and throwing even more episodes than usual. In our room he now actually sleeps all the way through the night most nights. Is it ideal that we don’t have night nursing? That’s a simple “no” because there is the very real risk factor of us sleeping through or not responding properly to alarms in the middle of the night. But our family made the decision that the risk of our potential lack of response outweighs the risk factor of the potential short and long term consequences of Wyatt having an increased number of cyanotic episodes. To some doctors that decision sounds irresponsible; to us it has been a huge improvement in Wyatt’s overall daily behavior and therefore treatment. That said, once my inability to deal with the daily stress hit, we decided we need to try to get as many of the daily 16 hours covered as possible to try to find some relief. While we have found four good nurses so far, unfortunately only two of them have been put on Wyatt’s regular weekly schedule. I have spoken with our case manager at the nursing agency, as well as their staffing manager several times. Our case manager has yet to show up for her monthly visit to check on things and restock all the nurses’ paperwork, as well as pick up the stack of old paperwork. I had to make it clear to both of them on several occasions that sending me a nurse just to fill a time slot was the opposite of helpful. It takes a lot of time and effort to train a nurse on Wyatt’s condition and care and to also have Wyatt get comfortable enough with that nurse to even let him/her hold Wyatt. That doesn’t happen in just one shift, especially not at Wyatt’s age of 10months old when stranger anxiety hits full force. In order to find a nurse that would qualify as a good fit though, I unfortunately have to go through the hassle of having them come for a shift to see how they do, especially how they respond to Wyatt’s lovely episodes. Still they keep sending me fill in nurses who have no availability to stay on long term. And trust me, to save everyone from agony, that’s one of the first questions I ask the nurses when they come for their first shift. Well, let’s just say the last few weeks have been challenging to say the least. I’m done seeing my share of nurses who only went into home nursing because they couldn’t hack it anywhere else. I personally know some great nurses so I know they are out there but man, the scope of what I’ve seen lately has been right out of tragic comedy, except it gets old very quickly and I’m not laughing anymore. One nurses came to orientate, talked the big talk about his experience level, never asked questions or said that he wasn’t comfortable, and then walked right out after only three hours of seeing multiple episodes. You made another one run, good job Wyatt. Then you have the paperwork pushers who will write pages after pages of patient progress notes and logs while never lifting a finger or even looking up long enough to know what progress was truly made by the patient that day. We have also had the ones who don’t do any charting in their entire 8-12 hour shift, don’t help out with anything unless directly asked to do so but then make up stuff to make their paperwork look good at the end of the day. Some nurses simply lack the ability to interact with babies yet they stay in pediatric nursing. I don’t get it. And finally yesterday evening, we had the one with complete lack of common sense who insists on hovering over you at the worst possible moments, yet not doing what needs to be done until it becomes pointless. Let me give you an example of last night. She asked a lot of questions, took a lot of notes and kind of just sat back and watched. I figured, ok, she has to figure out how we do things first and warm up to us, no big deal. By late afternoon however she has taken up our entire table on the back porch for over 2 hours to transfer her notes from her notebook to the actual paperwork required by the nursing agency while I’m sitting there holding Wyatt, suctioning him, playing with him and keeping an eye on the other three kids while my husband was cooking dinner. Then when she got the hint that we were ready to set that table for dinner, she asked to switch seats with me to use my side table so she could finish her charting. I politely informed her that I had to stay next to Wyatt to keep an eye on him and that she is more than welcome to do her paperwork inside the house as Wyatt will be joining us at the dinner table and we have plenty of eyes on him. She rolled her eyes at me seemingly uncomfortable to be out of sight from Wyatt (even though she had yet to interact with him) and sat down on the bench a few feet away. After dinner my seven and ten year old carried the plates back into the kitchen and accidently dropped and broke a glass plate that shattered into hundreds of pieces all over the kitchen floor. My husband was holding Wyatt so I ran into the kitchen and see my kids panicked standing in the middle of broken glass barefooted. I tell them to not move, I get my flip-flops on and carry them one by one back outside onto the back porch so I could clean up the glass pieces. What does the nurse do? Totally oblivious to what had just happened even though she saw, heard and even commented on the whole drama, she waltzed right into the kitchen walking straight through the pile of glass because it was getting close to 8 o’clock and she wanted to get Wyatt’s milk ready. I was stunned. I couldn’t believe my eyes. I didn’t even know what to say. I loudly announced that NOONE was allowed in the kitchen until I was finished cleaning up the hundreds of broken glass pieces. In the middle of still cleaning up the mess, she asked me again if she wanted me to get his feed ready now. My blood pressure was starting to rise. I calmly told her that we are holding off on the feed until after I was finished cleaning up the glass pieces, then we will give Wyatt a bath and then we will hook up his feed. Not even two minutes later, she asked me the same question again. At that point I just snapped back at her nd said “No. Bath first, then feed!” Around 9pm when we had finished Wyatt’s care and all the other kids were already in bed, my husband was holding Wyatt on the sofa trying to get him to go to sleep. Louder than she had spoken all day, the nurse of course had to ask another question right as Wyatt was almost asleep, which then woke him back up and mad him so mad that he went into an episode. Another 30minutes later, again writing up more paperwork, it suddenly dawned on her to want to clean out the suction canister right as the suction bag is already hanging on my shoulder and I’m heading up the stairs to get Wyatt to bed. She actually came up behind me as I’m walking with the bag towards the stairs and tried to stop me by holding onto the bag. What the heck? I told her not to bother; we’ll just clean it in the morning. Common sense apparently was not in her genes. I couldn’t wait for her to go. Normally we have the nurses leave whenever Wyatt goes to sleep at night because that’s when we like to go to bed, too. Unfortunately, this nurse though being yet another fill in didn’t come in her own car and was unable to get a hold of her ride until the end of her shift. So I had the pleasure of hanging out with her until 11p… These are the moments that make you want to pull your hair out and make you wonder whether it’s truly worth it to have home nursing in the first place. Yes, on Wyatt’s bad days it’s nice to have a second person there. And there are days where we are simply exhausted. But then again, particularly on Wyatt’s bad days, he doesn’t want anyone else to take care of him. Even with the good nurses, it’s not all smooth sailing either. I have a different parenting approach than they do so we sometimes clash for that reason alone. We don’t treat Wyatt any differently than we did our other three kids when they were his age. We like the prolonged snuggle time because we know soon enough he will be at an age where those moments will start to fade away. So having a nurse constantly ask me whether I want them to hold him gets very annoying. I’m sorry I’m not keeping you busy enough but I will never be the parent to just hand over my kid and let you raise him. I like my time with Wyatt and I even like doing most of his care. Somehow though most nurses have a hard time accepting that they are just there to be my second set of hands. It’s a personality clash I guess. The other big issue is the handling of Wyatt’s episodes. Wyatt has learned to use his episodes to his advantage and 50% of the time now he uses it for attention rather than when in discomfort only. One of our good nurses is so set on not letting him have any episodes at all during her shift that she will pick him right up at the slightest sign of a grumpy face. I on the other hand will first try to distract him, remove him from the situation, play a different game or simply tell him no before resorting back to being stuck on the sofa all day holding him. It drives me nuts that she does that and only reinforces his wrong approach to getting attention. The same goes for the regular CO2 checks. They are not my favorite part of Wyatt’s care either but they need to be done because Wyatt’s CO2 levels are the primary issue and there are no other ways of knowing where he is at any given point in time without objectively measuring. Our CO2 monitor however requires us to disconnect and reconnect Wyatt’s ventilator circuit periodically to place the CO2 monitor line. It cannot be left inline with his circuit and therefore this procedure has to be done for each spot check. The nurses however are so scared they will wake him up when trying to do a spot check while he’s sleeping that they have started to forgo the CO2 checks altogether. I have told them that it has to be done otherwise we don’t know when he’s out of range and what ventilator setting adjustments need to be made. I have even shown them some tricks on how to minimize the risk of waking him up. Still I have to constantly be on them about doing the spot checks and it’s starting to piss me off – excuse my language. This is not something that we can slack on. Too low or too high of a CO2 level both have neurological consequences. Checking his CO2 is not something we can skip or take our chances with. It also cannot be predicted strictly based on Wyatt’s behavior whether we think he’s in range or not. Objective measuring is the only way to know where he’s at and when to intervene. Some days I feel like such a broken record and I get so frustrated with nursing. All the reasons why we stopped nursing before are coming back very quickly. I hate having to get up at a certain time every morning to let the nurse in or not be able to get to bed whenever I want to because I still have a nurse sitting in my house. I hate having to be back home at a certain time because it’s the start or end of a shift. I can’t stand having to make numerous phone calls a week just to get all the doctor’s orders updated for the nursing agency when half the time I need to make vent or medication changes in the moment without a new doctor’s order. Keeping up with everyone involved with home nursing is usually a bigger stress factor than Wyatt’s daily care. I’d rather do it all by myself because then at least I wouldn’t have to explain everything I do and I’d know that it actually gets done. It’s such a no win situation.

a perfect day

Yesterday was truly an AWESOME day. We are starting the month of right. My intention was to have a good old family breakfast around 9am Sunday morning so we invited Grandma and Grandpa over to join us. I figured on a day when my husband is off, Wyatt (who sleeps in our bed) tends to sleep in a little longer. So we arranged for the nurse to come at 8am instead of 7am hoping we could all sleep just a bit more. And sure enough, it almost worked. Wyatt slept through the night, woke up at 630am with a full blown episode just to keep us on our toes, but then went back to sleep till 730am. Unfortunately Wyatt was still really sleepy when he finally got up and didn’t want to be put down. Therefore, my plan of having breakfast ready by 9am wasn’t going to happen. Amazingly enough though that turned out to be good thing. I had the privilege to witness my family in action in what can only be described as a rare occurrence. Everyone worked together as a team to make this breakfast happen… and wait for it… without arguing. It was breathtaking. Oh, I probably shouldn’t use that word - just kidding. It was really cool to watch. Everyone did their share of picking up around the house, setting the table out on the back porch and cooking the food. And when it was time to sit down and eat, even Wyatt was contend with the nurse for a little while just an arm’s reach away from us. Afterward the three boys tossed the football around with Daddy in the backyard while Grandma got to sneek in some snuggle time with Wyatt. We even surprisingly succeeded to keep Wyatt awake and entertained until his lunch time feed. The two big boys then got to go on a ride in Daddy’s mustang while the two smaller boys took a well deserved long nap. The afternoon and evening concluded with water hose chases and throwing burgers on the grill for dinner. It was as perfect of a day as we hadn’t had in a long time.

Nothing but boys

Oh boy, oh boy. There is never a dull moment around here. Apparently our entire family should start taking happy pills. There is the ten year old who is very self-driven, argumentative and a big time planner (much like myself) but who still hasn’t made friends with spontaneity or a sudden change in his plans yet. Getting him to be a kid and just play is the equivalent of torturing him so he’d rather do chores instead. Then there is the seven year old who is typically the one who makes everybody laugh, can change the mood of the situation (much like my husband) and tries to skip out on chores, yet he’s going through the me phase mixed with a lack of control of his emotions right now. Then we have the almost three year old who is a goofball, has a remarkable understanding of cause and effect and consequences of certain actions, which almost naturally now has made him an expert at taking advantage of that knowledge and changing the subject in an instant if needed. And then there is 10month old Wyatt who can instantly switch from having the biggest smile in the world to showing you his evil twin, topped with major stranger anxiety leading to clinginess right now. I love them, I love them, I love them… but boy are they driving me nuts sometimes. They have a way of either all tag teaming and everyone’s having a meltdown at the same time so that you can’t even get a word in and wish you had a freeze button. Or they will stagger their days so that by the time day four comes around your brain is simply fried. The biggest challenge is to NEVER let them know that they could rule this house if they actually put their heads together and planned it out just right.

I wouldn't change it for the world. They make me proud! (http://www.aprilsgrapevinephotography.com/)

hope vs reality

Teething is such a bitch. I don’t really remember the details for my other three kids aside from them being really clingy, so I have to assume it wasn’t too bad when they were teething. Wyatt on the other hand is miserable.  For a kid who hasn’t figured out yet how to just cry, it’s been hell. He’s responding to teething just like most kids would. He’s clingy and only wants to be held by me (which leaves the nurses just sitting around helplessly). He wants to sleep but is too uncomfortable to get any good rest. And since he can’t communicate any other way yet, he cries. But of course in Wyatt’s case crying means a full blown episode each time. The biggest problem for a child with a trach is that teething causes more secretions, therefore we have to suction him more frequently. The strain of each episode unfortunately has the same effect and we have to suction him again after almost every episode. That in turn makes him mad and more irritable and here we start the cycle again. It’s a no win situation. I have lost count how many episodes he’s had daily for the last six days. He hates Orajel, so Tylenol has been our best friend. Tylenol around the clock though means it’s causing him some constipation. This poor guy just can’t catch a break lately. He has six teeth already and for one of them he was this miserable also. But of course given that only one of his six teeth caused him this much pain previously, we were hopeful that it might get easier with each tooth following. Wrong! Damn hope, I hate you! I disapprove of labels but there is a reason why I consider myself a realist. I look at things that are right in front of me to deal with but I also like to be prepared for the worst case scenario by doing the research or playing it out in my head. People call me pessimistic for thinking that way but man, it leaves so much more room to be pleasantly surprised when things work out better than you expected, instead of crushing your hopes that were statistically unfavorable in the first place. Apparently yesterday was a day for people to gang up and try to convince me to look at the best case scenario though. First our nurse kept saying that she’s hopeful that Wyatt will eventually grow out of this and learn to breathe better. If you’ve done the research on CCHS then you’d know that’s not in the roam of possibilities. Despite me telling her that it’s not an illness but rather a genetic mutation, that there is no cure for CCHS, and that he’s dependent on some sort of mechanical ventilation for life, she insisted that she will hang on to that hope of a miracle because she’s seen it with premature babies who were given a grim outlook. That’s not really comparable but reasoning with her was pointless. Her riding the subject when I had just finished bagging my son for the 8^th time in less than four hours though maybe wasn’t the best timing to push the envelope. She’s a great nurse and Wyatt really likes her on his happy days but I don’t have patience for conversations like these. And then our DME case manager came by to once again swab out several O2 tanks. We went through four big O2 tanks this week alone just because of all the Ambu bagging. Of course, he had to mention that he just came from his other CCHS case. He mentioned how this child is four years old now and “running around like a normal kid” and that should give me something to look forward to. He proceeded to try to give me the positive outlook that this child is only vent dependent at night now and can move around freely throughout the day. He threw in there that this child also used to have blue spells but not as severe as Wyatt. He explained that this child’s version of a blue spell was a tinge of blue in the skin color from time to time but the airway never clamped off completely. I wish it were that simple with Wyatt. And I do have hope that he will eventually be able to come off the ventilator again while awake. But there is no way of knowing for how long of a time frame he can tolerate with his CO2 levels staying in range or whether he’ll ever be only nighttime dependent. As of right now, it’s not looking that way. It’s not in my nature to cling on to hope alone. If it happens GREAT but if that hope of a drastic change in his circumstances is the only thing I’m waiting for each day, I’m missing out on the now. I have to look at how to make life possible for him even if he stays 24/7 attached the ventilator with Oxygen requirements. Diaphragm pacers are an option but not a reasonable one until he’s about school age. And even then, they have their own set of issues and are by no means a failsafe option… On my lost in depression kind of days, I like to look at pictures or read stories of other CCHS kids and how they are progressing. But in reality, all CCHS cases are so vastly different, I cannot let myself get lost in comparing cases. I have to look at Wyatt and let him determine what he can or cannot do and when.

Let’s put my issues aside and recap what’s been happening on the Wyatt front.

As you saw in my previous post, Wyatt finally showed off his skills of sitting up on his own. I can’t even explain how much of a relief that is. As much as I know not to look at the time frame of milestones too closely, there is always that nagging question of how much of a neurological impact his daily struggles have. Having him finally sit up on his own unassisted and for over 30minutes nonetheless playing with his toys, that’s more than reassuring that he has his own plans of what he can and cannot do, as well as when and how he’ll do it. The coordination is definitely there and the rest of his strength will come. His well visit at the Pediatrician last week confirmed that he is gaining weight appropriately though he is short for his age and we will need to continue to keep an eye on his height progress. Otherwise, he is doing well and the Pediatrician switched him to once daily multi-vitamin with iron instead of a twice daily iron supplement. On the Pulmonary front, well, I haven’t made another appointment yet even though he was supposed to be seen by this week and get his last Synagis shot. Everything that needed to be addressed has been done through emails and phone calls only since his last hospital stay. Quite frankly, unless absolutely necessary I’m simply scared to get in a car with Wyatt again since our traumatic car ride home from Chicago. And my frustrations with the daily vent setting jeopardy over the last few weeks are making me less than patient to see another doctor at the moment.  Since Chicago about a month ago and Wyatt’s following illness, we’ve had ongoing frustrating trials of once again finding stable ventilator settings. After every attempt has failed to hold true for more than 24hours and Wyatt still having an increased number of episodes daily, we concluded the only thing left that was recently changed is the actual operating mode of the ventilator. CAMP switched Wyatt to Assist Control mode vs his previous settings in SIMV mode. In simple terms, in Assist Control Pressure mode the ventilator does all the work for the patient and provides the same amount of pressure with each breath as preset on the ventilator. In SIMV Pressure mode, the patient can initiate breaths on his own and the ventilator will only kick in to the preset numbers should the patient not reach the minimum desired pressure on his own or should he not take a breath within a set time frame. Therefore, Assist Control means the ventilator is in control of each breath taken vs SIMV means the patient is in control of when a spontaneous breath is taken and with what intensity (a.k.a. pressure). Wyatt has always been on SIMV for that very reason so that he can initiate breaths on his own and be in control of his own breathing as much as possible with the ventilator being his fail safe backup system. However, SIMV mode makes it a little harder to find a baseline ventilator setting because his CO2 readings won’t be as consistent if he is breathing over the ventilator. Therefore, we can only speculate that this is the reason why CAMP switched him to Assist Control mode to find optimal ventilator settings in the short amount of time that they had. So after ruling everything else out at home we came to the conclusion that this mode change is the only plausible reason left for Wyatt’s continuous discomfort. Therefore, we talked with our local Pulmonologist and switched Wyatt back to the SIMV mode. The difference was instant. He was suddenly laughing again and wanted to play instead of being clingy and cranky all day long. Why that thought never crossed our mind earlier, I don’t know. Regardless, we had finally found the underlying issue and fixed it. That said though, since the beginning of this week we are now battling teething again and therefore have again started a vicious cycle of numerous daily episodes and clinginess. Apparently this struggle is never-ending. Tylenol is our best friend but unfortunately Ativan had to be used a few times also. I hate Ativan and I really wish there was an herbal alternative for infants to ease anxiety and act like a short term mild sedative. It would be so nice to find a holistic pediatric doctor to consult with. When Wyatt is not teething however, we have figured out a pattern to Wyatt’s episodes. Teething discomfort aside, he seems to always be at greatest risk for episodes right towards the end of his (G-tube) feeds at which time he is also the most tired and gassy. We tried gas drops, gripe water, different formulas, different volumes and rates for each feed but nothing seems to be working. Sometimes just holding him in our arms until he falls asleep prevents the episodes but then other times he seems so uncomfortable and nothing soothes him that we are literally just an arm’s reach away from the ambu bag and O2 tank ready to revive him again. You feel so helpless. Again, I wish there were herbs for infants that could ease his feeding discomfort and let him relax enough to fall asleep without the drama. If anyone has any suggestions, please fire away.

Milestones

On March 19, 2012 Wyatt once again proved that he will do things whenever he feels like it. This is a picture of him sitting up for the first time unassisted. Not only was it his first time trying but he actually sat there continuously for over 30 minutes. He probably knew how to do it for a while already, he just never felt like showing us his skills up until now. Little stinker. Over the last week, he has also managed to learn to click his tongue which is even quietly audible, and I swear he is mouthing the word mama.

Thank you, Wyatt. I needed to see you do that. Despite all the worries about the possible negative neurological consequences of your numerous daily cyanotic episodes, you once again proved that you're in charge and worst case scenarios are just that.

counseling

Supposedly, admitting you have a problem is the first step. I was told that being able to come to that conclusion on your own also means you are not as crazy as you thought because truly insane people don’t know they’re crazy. So last week I reached out to a clinical social worker and made an appointment. Ok, let me clarify. Without giving out contact information, I emailed a few counselors to scope out the field and get details about how the sessions are set up, financials, etc. After a couple back and forth emails with one particular counselor, he must have looked me up in the phone book and called me back. So I gave him credit for making the effort and I met with him last Wednesday.

It’s a very strange feeling walking into a counselor’s office for the first time. I know people seek counseling for all sorts of reasons but for me, I always looked at counseling as a last resort that I would only use after I have exhausted all other options and I can’t solve the problem myself. And even then it’s an absolute last ditch effort hoping the counselor can somehow magically give me the answer that will make things turn around. In other words, I am more than skeptical that counseling will help me but I didn’t know where else to turn. Despite everything within me being against this, I made the appointment and showed up. I’m sure my outward first impression also gave off that vibe of me being very nervous and resistant. First order of business was the uncomfortable small talk, “Did you find this office ok?” I hate general questions like these. They seem pointless. And then there was the “How are you?” Did he really just ask me that? Why does he think I’m here? I bit my tongue for this typical everyday no one really cares about the answer kind of question and replied with the socially accepted “I’m ok.” I have come to hate that particular question most of all over the last nine months. As I entered the small office room, there was a corner desk to my right filled with a few files and books. Straight in front of me were a couple of chairs separated by a small end table that had a bowl of candy and a tissue box on it. The wall behind the chairs had an oriental room divider which I assume was there for decoration purposes only because there sure wasn’t any space left to divide. There also was what looked like a child’s painting on the floor next to the divider. I began to wonder what that child must have been here for. I took a seat furthest away from the desk and kept scanning the room. My thoughts were interrupted by the counselor asking me how I would like to pay for this session. I handed him my credit card for the $80 charge and explained that I would prefer not to use insurance to avoid anything going on my medical record. What I didn’t tell him was that I wasn’t sure whether I’d be back to see him again after this first session. He turned around to face his desk, leaving his back towards me, to charge my credit card and to input my driver’s license information. I continued to scan the room. On the wall behind me he had also placed a fake plant next to the room divider. I again caught myself wondering what that room divider was for. It appeared that he made a good effort to make his patients comfortable and relaxed in his office but to me it just seemed fake. I was staring at the door in front of me, tempering with the thought of excusing myself to the bathroom and leaving. But damn, I already paid for the session. The door had a white noise maker attached to the bottom vent to keep conversations confidential. Before I could hatch out my escape plan any further, he finally turned his desk chair back around to face me and attempted to break the ice by telling me that everything behind this door is kept confidential, unless of course there is reason to assume that I would harm myself or someone else or that there is abuse involved or other legal issues. Blah, blah, blah. I get it, he was covering his legal behind. Then he proceeded to tell me about his education level and his 35 years of experience and proclaimed to not have all the answers but that we will work together to find them. This all seemed like such a scene out of a movie. And then came the big question “What brought you here?” Oh boy, where should I even begin to explain the dramatic changes in my life that occurred in the last 9 months? I briefly summed it up by explaining Wyatt’s health complications immediately following his birth, his 98 days in the NICU, the rare diagnosis and his ultimate long-term medical needs, and finally his numerous daily cyanotic episodes which I was able to deal with before we went to Chicago but that are now putting me into a state of panic. Just as a counselor is supposed to do, he sat there, listened and took some notes. He was however unable to hide that facial expression that I have seen so many times before when I first talk to someone about Wyatt. It’s that expression of shock and disbelief and pity. Ok, maybe it wasn’t pity and it really was just heart-felt empathy but still, these expressions have no meaning in my book and only make me more uncomfortable and angry. He also took some notes about the family dynamics and the ages of my other three children. Then he asked me to switch seats with him and do a computer-based self-assessment to gage my emotions at this point in time and to then be able to revisit this tool every so many weeks for comparison to track my progress. That all seemed eerie as it is a similar approach to what I do when I first consult with a new client for Personal Training. The test was comprised of different questions regarding your thoughts and feelings and you had to pick an answer ranging from never, to rarely, sometimes, often, and all the time. Before even starting this assessment I knew what the result would be. Of course, almost all the questions are aiming at your worst feelings and thoughts as if this test is trying to put you back into that mindset. Big surprise, I scored very high on depression and anxiety, moderate on PTSD and thanks to a few positive questions geared toward whether I actually have happy days too, I also scored mild on bipolar. Stupid test. I began to wonder whether this session was worth the money spent. Of course the counselor responded by stating that he is very concerned with my high level of depression and anxiety and that “we” have to address that first before psychotherapy can be successful. He asked me what my stance is on medication to which I basically explained that unless I’m dying, which I’m not, I’m typically against medication. I also can’t afford to be dealing with side effects that could cloud my judgment and responsiveness toward Wyatt’s continuous needs. As an alternative which he claims is just as effective, he then stated that he has a holistic doctor working with him in his practice who approaches psychological treatments through herbs, massage and acupuncture among other things. This doctor currently offers a free consultation and would then charge me the same rate for future appointments should I choose to go that route. That sounded more along the lines of my beliefs and approach on things so I agreed to a consultation with that doctor for this Wednesday, right before my next counseling session. Unfortunately, that agreement already marked the end of this 50minute $80 session and the counselor ended our conversation with saying something to the effect of how courageous I am for taking care of Wyatt the way I do and that while it may only seem like something that I feel I have to do, most people wouldn’t do it at all. Again, one of those bite my tongue moments. I replied “Thank you!“ but I didn’t mean it. What I really wanted to say was that I’m sick and tired of hearing these comments. These comments are coming from a place of insecurity and fear in other people and it is not helpful to me at all. I would hope that if someone else were faced with a similar situation happening to their own child that they would also do the best that they can with the tools they have available. So telling me that I’m somehow doing more than other people would is bullshit and just another small talk kind of response, wanting to make me feel better yet only making things worse. What happened to being honest? If you’re uncomfortable, can’t relate and don’t know what to say, then just say that. I’d think far higher of you for being straight up honest.  

I’m sure you can tell by my writing style that I am struggling with the idea of going back for another session. I want to meet with the holistic doctor because I know from my own profession that good nutrition along with the right supplements can make a world of difference, not just in how your body feels but also how your brain functions. Counseling though I’m not so sure about. I don’t think this counselor is a good fit. I’m not even sure if counseling is for me at all. They way I see it is if someone you love dies, you go through the stages of grief but then eventually it gets easier and you move on with your own life because death is just a part of life. Now imagine having to face the possible death of your own child over and over again every single day. Imagine going through those stages of grief every day that your own child turns blue and lifeless and you have to revive him. Every time he has to go back to the hospital, you again start all over. It doesn’t end. How do you cope with that when there is no end in sight? What’s the sense in discussing different coping strategies to something that can’t be coped with because it’s never over? How can you even find a counselor who can understand what that feels like every single day? It doesn’t matter how often or how detailed I explain everything. Unless you’re living in my shoes, you can’t possibly understand. So how can you possibly help me?

So here is what I know I have to do.

1.       I have to let go of always being the one taking care of Wyatt. I have to ask for and accept the help available. Anyone who wants to help but is not trained in all his care simply needs to be trained.

2.       I have let go of the idea of being able to protect Wyatt 100% of the time. No one can do that and shit can happen at any time. If I can learn to accept that as a given fact then maybe I can finally shut my brain off enough to live again.

3.       I have to get my nutrition back on track. Good fuel put back into my body will give me the energy back to make it through the day, will help me to regain my mental focus, and to balance out all these brain chemicals controlling my mood and thoughts.

4.       Only after my nutrition is back on track can I consider working out again. As much as those endorphins would help my mood, trying to workout now as weak as I am with all the physical signs of depressions such as nausea and lightheadedness, it is simply not save. Nutrition has to come first.

5.       I have to keep writing. It’s my way of letting my thoughts and feelings out instead of everything spinning like a revolving door.

6.       I have to find ways that my husband and I can go out again by ourselves so we can find topics to talk about again that do not evolve around our daily struggles. My husband and I are both very good at always putting the kids first and making sure they each get their share of special one on one time. We have to put the two of us back on that priority list also.

7.       And here is the biggest challenge of all that will ultimately determine my success in all the aspects above: I have to learn to recognize the negative thoughts and feelings. If I can learn to acknowledge them as soon as they start creeping in, then I can learn to shut them up and make them take the detour.

Acceptance is hard

In just nine months, CCHS has tested us in more ways than anything else I could have ever imagined. I’d like to say that somehow all these struggles will turn us into more empathetic and live in the moment kind of people. While it is true that we all in our own ways now have a different outlook on life and approach things from a different angle, I’m also a realist and know that eventually we will go back to bitching about the little unimportant things in life. But that’s ok. I’m actually looking forward to that moment because it will symbolize that we have a reached a point in which CCHS doesn’t have to be the main focus around the clock 365days of the year anymore. While some people hang on to the hope of finding a cure, I’m simply hoping and smiling at the thought of eventually having those petty moments of creating our own useless non CCHS related stress and then realizing how stupid that is. That’s my overall goal. In all honesty, I do not believe that there ever will be a cure for CCHS. And before you jump to conclusions and label me as a pessimist, let me explain.

CCHS is a very complex yet little understood genetic disorder. So far nine (9) different mutation levels of this disorder have been identified through genetic testing, plus there is another disorder called ROHHAD that represents in a similar pattern but cannot be tested for yet. Within these nine different mutation levels, there are vastly different outcomes. Based on current research, it is unclear what pattern each mutation level follows in regard to the associated risk factors of cardiac rhythm abnormalities, cancerous and benign tumors, hypoglycemia, growth hormone insufficiency, vision problems, balance problems, learning disabilities, facial abnormalities, and the list goes on. Some CCHS patients that fall into the category of the most common and most studied mutation levels (20/25, 20/26 and 20/27) have everything imaginable to deal with additional to the breathing management, and then some CCHS patients within those same mutation groups have no other complications whatsoever. And then there are the CCHS patients with the higher expansion numbers (20/28 through 20/33) who have even less research to go by. At first it was theorized that the higher the expansion number, the worse the outcome. But then over time that theory didn’t hold up. Current data only suggests being proactive about screening for these risk factors but it cannot even give a glimpse of a future prognosis because each CCHS case known today is so vastly different. In other words, all we know is that these other risk factors were identified because they have been found in other CCHS cases, some of them more often than others. No one has an answer however whether theses other complications are truly CCHS linked or whether they are just another one of those winning lottery tickets. Chances are that if it has to do with the autonomic nervous system, it is probably linked somehow. All other risk factors though I personally am skeptical that they can be blamed on CCHS alone. Learning disabilities for example, any child can have those for multiple reasons. Cancer, well there has been so much research done in that area over the years and we still don’t know exactly what causes it. Vision problems are an issue for millions of people in the world… So I try to stay away from blaming everything on CCHS. With all that said, this is why I do not believe there will ever be a cure for CCHS. Even if research eventually figures out when and how CCHS is caused, there are too many mutation levels with too many variables in outcomes to find just one cure for every CCHS patient. Does that type of thinking make me a pessimist? I don’t think so. I’m just a realist who is trying not to miss out on what’s happening right in front of me at this point in time instead of being too pre-occupied looking and hoping for a miracle.

So in this never-ending cycle of the five stages of grief that the family of a CCHS patient has to go through, let’s talk about acceptance. (By the way, it’s a never-ending cycle because you go through it all over again each time you hit a setback or a hospitalization. Your only hope is that you start to go through this vicious cycle a bit faster each time.) Here is my first draft of a list of things that I cannot change:

1.       I have a special needs child. I hate that label but it’s true. There is no point in fighting it, it simply is our job as the parents now to make sure that this label doesn’t define who Wyatt becomes as a person.

2.       Standard programs for special needs children will not suffice. Being a mechanically ventilated child ultimately means it won’t be as simple as developing an individual education plan when Wyatt enters school. In fact, he may not have academic problems at all. Wyatt will need someone who can be his shadow. Someone who can stay in the background as to not interfere with his social development, yet always be close enough to intervene for medical reasons. It will have to be someone specifically trained to attend to his needs. At the beginning of each school year, we will also have to discuss his medical needs with his teachers and possibly parents of other children who will be in his class. We also have to be prepared to deal with prolonged medical absences from school.

3.       My child will always need mechanical ventilation. No matter what the medical advances may be in the future, our son will always be dependent on artificial ventilation of some sort. There may be better options applicable to him as he gets older other than a tracheotomy and a ventilator but none the less, he will never be able to be weaned off mechanical ventilation.

4.       We will always depend on number to know how our child is doing. CCHS means we can’t depend on physical signs and symptoms to identify when something is wrong with Wyatt. He won’t run a fever when he’s sick and just because his skin color looks good doesn’t mean his CO2 and blood oxygen levels are good. Objective monitors are the only way for us to know and he simply can’t live without those. It will always be a balancing act of keeping his numbers in check.

5.       We will always have to be ready to fight. Medical bills won’t go away. Arguments with durable medical equipment companies, pharmacies, developmental therapists, doctors, nurses, respiratory therapists, and other specialists are bound to come up every so often. Continued research and education on our part are a must. Getting less than adequate, quality sleep is a given fact. Always being on high alert for the next unexpected road block won’t go away either. These issues may space out a bit more over time (or at least I hope they will), but they will never completely go away.

6.       The never-ending yet unintentional comparison. We have four children but only one with CCHS, we have friends and family with children, and we see children of complete strangers everywhere we go. It’d be naïve to say that we are not going to compare Wyatt’s progress with that of other children. But I believe that’s a good thing because it’s a good challenge. It forces us to not give up and always reach for higher goals. We just have to learn to let go of a specific time table when he is supposed to do certain things, and we may also have to steer away from the normal ways of accomplishing these goals.

7.       We will always have to deal with the looks. No matter where we go, there will always be people staring or making comments without thinking. Most likely it is because they don’t know how to deal with the situation and it makes them uncomfortable. Either way, this is a tough one to deal with. Some days I want to say something sarcastic back and some days I’m ok with ignoring it.

8.       Its’ a lonely path. Being a parent of a special needs child is tough enough. Being a parent of child with a rare disorder that is not commonly understood is even harder. It doesn’t matter how detailed you explain what daily life is like, you’re still in this battle alone. You want to connect with parents of “normal” children but at the same time, it’s so hard to hear those parents complain about the normal issues of raising a child.

I am by no means claiming that I have truly accepted these facts, but maybe writing them down will bring me one step closer. It is no secret that the last few weeks have traumatized me and that I am struggling to get up every morning right now. I’m not ashamed to say that I am scared to be around my own son. I used to be able to handle his daily episodes but now each one of them sets me into a silent panic mode. The anticipation of an episode alone sometimes proves to be too overwhelming for me right now. My self-defense mechanism tells me to keep my distance from him to preserve my sanity should anything happen to him. I don’t have the option to stay away though which makes dealing with PTSD so much harder. There are no breaks. No one can just come in and take over for a few weeks until I can find my grounding again. Even home nursing can’t provide that kind of a relief. So it is up to me to somehow find a way to cope yet deal with the ongoing stress at the same time.