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July 29, 2016

The War Continues: Battle of the C-collar




One of the other issues we've been battling with Aiden's medical team is his c-collar. I loathe his c-collar. I despise his c-collar. I want to burn his c-collar. I want to throw his c-collar out the window. I want to run over his c-collar with my car. I want to force his doctors to wear a c-collar for a day, and see how they like it! I can't wait until we can say "buh-bye, now!" to his c-collar for good.

I get it - one wrong move, and Aiden could be paralyzed forever. It's a real threat, and not something I want to risk happening to him. I would much rather be safe than sorry. But even then, I. Hate. His. C-collar.

And here's why:

1. Many nurses don't even know what a c-collar is, and hardly any of them want to put it on. And it's a huge hassle. It takes two pairs of hands to do it. You have to have one person unhook his trach, while the other person places the front part of his collar in-place on his chest, and then quickly plug the trach back in through the provided hole in the collar. And then one person must put their hand behind Aiden's back, slightly lift back his head, while the other person slides the front piece into place. Once the front piece is on, one person must lift him up, so that the other person can put the back piece of his collar underneath him, and then slowly lower him back into bed as you slide the collar in place. All the while making sure you aren't pinching his jowls or catching his ears. That's quite a process that can't be done any other way for fear of his own safety. And because of this, many nurses would rather not bother with it. Although he has a daily routine clearly outlined in his chart, they take one look at the collar, another look at him, and put the collar back under his bed.

Except for a handful of our primary nurses that have gotten used to putting it on, I walk in on an almost daily basis only to hear, "Well, I don't know how to do the c-collar, so I just waited for you to get here to put it on...". I can't tell you how much this agitates me. This is a nurse, in a hospital, full of resources and people that do know how to handle a c-collar. And rather than reaching out to those resources, they'd much rather not bother with him. And as a result, he lays in bed all day long until I get there and get him out myself.

So he is missing out on opportunities to be held, played with, and treated like a normal baby because everyone is afraid to put on his c-collar. That is not fair, and he shouldn't be deprived or treated differently then any other baby in the NICU because a nurse simply doesn't want to bother with it.

And, 2. He hates his c-collar too. He can only tolerate it for a max of four hours at a time before he starts to get fussy. It makes him sore, tired, and sweaty, and he gets very uncomfortable in it. I mean, can you imagine having a hard piece of plastic around your neck, half-way up the back of your head, and half-way down your back? I'm surprised he lasts as long as he does.

It limits his range of motion, and this frustrates him. It doesn't allow him to move his arms so that he can rub his eyes, or try to grab a toy. It doesn't allow him to move his head as much as he would like to. And it forces his body to stay in a rigid, stiff state so he can't freely wiggle his body around. When he is awake with it on, he will begin trying to do these things, but you can see the frustration in his face. He inevitably gives up after a few minutes, withdraws himself from his surroundings, and goes to sleep. And that kills me inside. A four and a half month old baby should not be withdrawing from trying out normal developmental things because he's forced to have a hard plastic neck brace strapped around him.



The hospital has been talking to us about transferring to a long-term care facility in the near future. A few months ago they had talked about wanting to get an MRI of his spine done to get a clearer picture of his ossification. This fell off the radar, but David and I brought it up again as this long-term care facility came up. We expressed that we would prefer to get it done while we are still at the hospital, rather than dealing with transferring back down to the hospital to do it later.

Right as we expressed this, he started getting jittery eyes and legs. This can also be a normal side effect of his sedation medicine, but his medical team panicked and thought they did something to damage his spine. They consulted neurosurgery for it, and in turn neurosurgery wrote an order that he needed to wear his c-collar 24/7, and rushed to get him into an MRI to check things out the next day.

The MRI results came back to show exactly what we all expected. His spine is short, compact, and tighter than an average person. Thus his diagnosis of SEDc, respectively. The MRI further showed there wasn't any damage done to his spine, and it even looked ever-so-slightly more ossified than his previous spinal xrays. His vertebrae is also aligned straight, which is great news as individuals with this type of dwarfism are prone to having severe scoliosis.

Further more, since his MRI revealed no damage, they concluded his jittery eyes and legs to indeed be a side affect of his medicine.

You would think all of this led to the neurosurgeon removing the order to wear his c-collar 24/7 - but it didn't. The order supposedly remained there.

David and I were very concerned about this. If there wasn't any damage to his spine, why would we force Aiden to be in his collar all the time? This was only going to cause a whole other set of issues for him - he could get break down and pressure sores, it would further flatten his already flat and funky head, it would be harder to notice when it was getting too small for him... And all of our primary nurses agreed with us - this was not a good thing for him and was unnecessary.

We finally took up our concerns with the director of the NICU, who in turn simply told us, "I think this is a good thing for him. It would force nurses to do more activities with him if it's already on." But that didn't sit right with me either. That is a nurses job. I wasn't about to torture my son with a neck brace 24/7 just to force a nurse to do the job they are there for.

As David and I played the political game concerning all this over a period of a few days, we noticed a very fast change in Aiden. He was starting to withdraw into a shell from being in that collar. He was distant, refused to engage with you, wouldn't interact or smile, and would avoid any contact you tried to establish. And he was very uncomfortable - to the point nurses had to give him PRN's around the clock just to keep him happy and under control.

So David went to the NICU director again, and this time they got into quite a heated argument about it. After laying out our concerns and subtly pointing out that we weren't going to allow them to force our son into a shell for their own liability concerns (because really, that is what this situation boils down to), the director agreed to set up a meeting with neurosurgery to discuss a better plan for his c-collar. The neurologist was going to be out of town for a few more days, and the director refused to talk to anyone but the doctor that originally made that order. She did allow his collar to come off at night when he was sleeping, but wouldn't make any other changes until that specific neurosurgeon came back from vacation.

In the meantime I was not happy or satisfied. So each day I walked in to his room, said to his nurse that day, "Look, I don't want you to get in trouble. I will tell the doctors I mandated this, and you are more than welcome to throw me under the bus as well. But I am going to kindly tell you to help me take off his collar right now." And the funny thing is, not a single nurse argued with me further. One of our primary nurses even "washed his collar" very thoroughly multiple times just to give off the appearance we were doing routine maintenance to it, and ultimately give him an even longer break from wearing it. For the few days I did this he didn't get out of bed once - but in the bigger scope of things, I knew at that moment mandating a break from his collar is what he needed most.

When the day finally came that our neurosurgeon was back in town, David went to the hospital geared-up and ready for another argument. We were both hell-bent on not letting them order Aiden is to wear his collar 24/7. Even if they made us sign paperwork stating we wouldn't sue in the event someone paralyzes him, we were prepared to do so. Whatever needed to be done to ensure his mental and emotional well-being was taken care of as well.

As David finally met up with our neurologist, he was immediately taken back when the doctor said, "I didn't mean for his collar to be worn 24/7 here on out, I only meant for the day he was going to MRI so that he was ready to be transported at any time. And especially because he was jittery, I wanted to rule out any damage to his spine that may have caused that. So just as before, he only needs to wear his c-collar as he's being transported or when he's out of bed." David simply said to him, "Sounds great, thank you. Would you not mind going in to the NICU director right now and let her know that?" Our doctor happily did so, and the NICU director didn't say another word to us about the collar after that.

This is yet another example of the inconsistencies we are continually dealing with. And I'm so fed up with it all. Where did the misunderstanding lie? Was this all just a big liability issue for this new, young NICU director that just started, in spite of what our neurosurgeon said? Our neurosurgeon was acting as if he didn't understand why he needed to come talk to us. Like this was always the plan and what he said from the beginning. And yet, David and I battled multiple people in the NICU about it for an entire week. And in the meantime, Aiden had to once again suffer through something he didn't need to.

It's so hard to be put into a position where you have to constantly question and argue against decisions being made for your son. There have been so many times I've wanted to march in there, guns blazing (figuratively, of course) and tongues flying. But if I only appear as an emotional, irate mother - how far would that get me? David and I have to be so careful in the way we handle things. We have to play the politics game, appeal to the right people, be careful in our wording so they take us seriously. When your child's life is literally in their hands as you do so, it takes every once of patience and strength in me to play the game. And nothing makes me more upset when a NICU director flat out says to us, "Well don't forget, we are the medical professionals..." as we question a decision they made for him.

Could I fast forward time until when he finally gets to be home with me?

Sir Chunks-a-lot 


July 25, 2016

War Has Been Waged

For the past week, David and I have been at war with some of the hospital staff over quite a few things. And I am so tired of this hospital. The level of inconsistency and lack of communication has been astounding and incredibly frustrating.

Now before I go on, let me first point out that the issues we have been having have not been with nurses. NICU nurses are angels, and have been on our side. We've even had some nurses stand up to doctors with us. The problems we are having are coming from doctors and, more than anything else, nurse practitioners.

They don't take notes. They don't write things down. They don't communicate with each other. They walk in, see that his CO2 level is currently at 63, freak out and want to change all of his vent settings and put him on more respiratory support. Then when I ask why and say, "No, that's normal for him..."; only then do they look at his chart and see that it's indeed a normal number for him, and then waltz out all puffed-up and besides themselves. David and I have time and time again voiced our concerns over how bad the communication is - but nothing has been resolved. It has gotten bad enough that David and I feel like we've had to babysit his medical staff. And today because of this, I lost my son for a few minutes.

I apologize if this post isn't as well-thought out or written this time around. I am just so beside-myself angry about today, that I just need a way to get it all out of my system and vent for a minute.

I've come to loathe quite a few NP's. Don't mistake me, we have also had many incredible NPs. And when they are willing to listen and do their job, they can be a viable asset and advocate for you. But when I hear some names assigned to Aiden for the day, my heart will sink. We have had many situations in the past where we have had to fight an NP for something that Aiden needs. And today, that went way too far.

All day today Aiden has been in a never-ending cycle of bronchial spasm attacks. The tidal volume that the ventilator is set to giving him is 35, and he was only able to achieve a tidal volume between 9-12. This means he was only able to achieve small little breaths, almost as if he had been running around for some time and unable to catch his breath. His heart rate was consistently high, and he was working very hard to breath. He was covered in sweat, he was hot and exhausted - and we were trying everything.

We gave him multiple Levalbuterol puffs, and that only helped him calm down for a short period of time. We gave him Tylenol to see if he was in pain and keeping him in this state, and it didn't even phase him. So we gave him every other PRN medicine he had available, and he still wouldn't calm. We tried suctioning him constantly, but nothing significant would come out. We re-placed his trach, we wiped him down with cool water, we sat and gave him manual breaths on the machine - and still nothing was helping him calm and reach his tidal volumes. His oxygen was turned up to 100%, but he was only satting at around 75.

He was completely panicked and upset all day. His nurse, RT and I sat by him, watched his ventilator, and tried every thing we could think of. And nothing helped.

It got bad enough that the RT thought it would be best to do an xray and try to get a picture as to what was happening. So the nurse called our NP back to look at him. She looked at Aiden, looked at his ventilator and said, "I just think he needs to sleep. Try to get him to sleep, and see if that helps first.", and walked off without batting an eye.

As she walked away, I mentioned to his RT that in the past, we have increased his tidal volumes temporarily to help him catch up and it worked. The RT agreed that was a good place to start, and also wanted to push the xray again. So he followed the NP out of the room to talk to her further about that.

The RT came back a few minutes later to announce that the NP still didn't feel the need to xray, and would only approve him increasing the tidal volume from 35 to 38. Now that's not really a significant enough increase to do anything for him. And in the past, we would always increase his tidal volume to 50 in these situations. The RT said he brought this up to her, and pointed it out in his chart, but she still would only agree on increasing it to 38.

I was about to have my own say in this and demand an x-ray and increase to 50, but Aiden got into a coughing fit and suddenly plummeted.

We did everything, manual breaths, a quick suction - but after an entire day of fighting to breathe, his little body couldn't handle it anymore and he flat lined.

I was holding his hands, rubbing his hair, trying to calm him down, and his stats just kept dropping until his heart rate and oxygen saturation was at 0.

For a few brief moments, he was completely lifeless. No matter how much I have desperately tried to erase the image of him like that from my mind, it's stuck sitting in there. I called out his name and shook him as the nurse and RT pushed me out of the way to bring him back. It was only maybe a half minute he was gone - but he was gone. His body was completely gray and limp, his eyes open and lifeless.

That half minute felt like an eternity. I thought I lost him for good. Did his body give up? Just like that, after a straight three weeks of doing great, was he gone for good?

When he finally gasped and came back, I realized the room was full of his medical team. I pushed my way back to his side and he looked at me. I grasped his hand, and his little fingers weakly held my finger.

His doctor began examining him and assessing the situation. As the nurse and RT went over the day's events, the doctor snapped her head around to the NP and asked, "Why is this the first I'm hearing about this?"

The doctor immediately upped his tidal volume to 50, called for an xray, told the nurse to give him a Pentobar to knock him out and allow his body to recover and rest, and asked the RT to give him yet another double-dose of Levalbuterol. As the doctor ordered each of these things, she turned to the NP and asked, "Why didn't you up his tidal volume? Why didn't we get an xray earlier today? Why didn't you tell me this is what he was doing?" And the NP just stood there in the background, looking dumbfounded and worthless.

I can't seem to calm the emotions I have tonight because of this. Why did my child have to die for this particular NP to do anything for him? Why should it even be a battle anyway!? Why does Aiden have to go through this?

And I can't get the image of his gray, limp, lifeless body out of my mind. David switched me out at the hospital for the evening, and I took the girls home. Once they got into bed I broke down and bawled. The fact that this happened today is unacceptable. I am so tired of having to continually deal with these type of situations. After today a part of me feels like I can't handle it anymore. Almost like I need a break from that hospital room before I completely break down myself - but how could I possibly do that? I no longer feel like I can trust his entire medical staff to give him what he needs when he needs it. I feel like I have to be constantly monitoring and babysitting people. I don't feel like I'm listened to or considered a valid person in terms of his care. Medical personnel are so quick to think they know better than everyone else. But what did I ask for today for Aiden? An xray, and increased tidal volume temporarily to help him out. I shouldn't have to ask, because it's in his chart that that's what he needs. And I shouldn't be treated like I don't know what he needs either. After all, as soon as the doctor was FINALLY notified, what did she do? Increase the tidal volume, and get an xray.

How much longer will my baby have to withstand the torture he's endured before I can finally get him out of that hospital? How much longer can I take this myself? After witnessing him die today because of a stubborn NP that thought she knew better, I don't know how any of us are going to get through this.

And I never want to watch my son flat line again. I have a feeling that memory is going to haunt me for the rest of my life.

July 13, 2016

Confirming Aiden's Diagnosis

Finally. Nearly 4 months after Aiden's birth, we got the results back from his genetic testing. And they have confirmed he has a gene mutation within COL2A1.

I sat down with two of his geneticists for over an hour as they went over the results with me. I am fascinated.

They explained to me that if David and I were to do genetic testing with blood work, it wouldn't gain any results or answers for us. They have been able to determine his gene mutation was stemmed from either a mutated sperm, or a mutated egg. In order to do any testing, they would have to test sperm and eggs. And the chance that they were to extract a second sperm or egg containing the mutation would be one in a million. Because I have a simian crease on my left hand, they still think there is more of a chance it came from me. And since it stemmed from a sperm or egg, the likelihood of us having another child with a Skeletal Dysplasia is a mere 1-2%. So long story short, Aiden came the way he was supposed to come and David and I were lucky enough to get him.

Now because Aiden has the mutated gene, his offspring has a 50% chance of being born with a type of Skeletal Dysplasia. Melodie and Lydia do not have any chance of this happening to their future children. This means David and I have officially introduced a branch of little people into our family tree through Aiden.

Due to his x-rays, they still think the type of Skeletal Dysplasia he has right now is Spondyloepiphyseal Dysplasia Congenita (SEDc Dysplasia). There is a possibility this diagnosis can change in the future depending on how he grows and other related health problems that can arise, but he will always remain within the SED family of dysplasias. There are many different types of dysplasias within the realm of the SED family. There is SEMD, Kniest, or SED Strudwick - which is what Dr. Jen Arnold has - just to name a few. I believe his diagnosis will remain the same as he grows, but if it ends up changing it will only mean he has different physical barriers to over come. 

A lot of people have been asking me the following questions about Aiden lately. And really, thank you for doing so. I'm glad people in my life haven't been afraid to ask me about him and his future/diagnosis. Please, keep them coming. 

Will Aiden have any mental disabilities?

No. I mean, I'm ADD myself, and depression runs in both mine and David's families... But besides those "normal" things anyone can have, he has normal intelligence. He will be behind the first few years with reaching developmental milestones, and obviously his speech will be delayed due to his trach. But his limitations are purely physical. 

The collagen defect that causes his body to not be able to grow bone properly does also affect other cartilage in his body. Namely his ears and eyes. He will most definitely need to wear glasses in the future, although his eyesight is perfect right now. His hearing is also just fine, but he can develop some deafness in later years.

Will Aiden walk?

Perhaps! Most people do, but some require wheelchairs. He may require a few hip surgeries in his future to help with walking, and it will take him longer than most people to learn, but it is very possible. I see him walking eventually. But if not, you can still live a perfectly happy life from a wheel chair.

Why does Aiden wear that neck brace? How long will he need to wear it?

Unlike Achondroplasia (most common type of dwarfism) that only affects the long bones in your arms and legs, SEDc causes your spine to be more of a rectangle shape and therefore shorter as well. On top of that, the collagen mutation that creates this type of dwarfism makes it harder for his body to ossify bone. When we are growing in our mom's bellys, our bodies first made cartilage. That cartilage then hardens- or ossifies - and turns into bone. This mutation prevents his body from being able to do that properly. Thus his smaller arms, legs, rib cage, and spine.

If you were to take a single vertebrae from your back and look at it at a bird's eye view, that vertebrae would look like a circle with spokes coming out around it. On Aiden, the spokes are ossified, but the middle circle isn't yet. That, on top of the fact that his head is big compared to his smaller body and he doesn't have much of a neck from his spine being smaller, puts him at a high risk of being paralyzed. His spine just can't contain him right now. Putting his head back slightly isn't that big of a deal, but if his head were to suddenly fall forward, that soft spine wouldn't be able to handle the pressure and you could easily snap those nerves that keep him moving. So he wears his c-collar when he's out of bed or being moved around as a way to keep him safe.

He will most likely have a neck-stabilizing surgery in his future. They won't approach that idea until he is at least 2 years of age. There is a small chance his spine can ossify on it's own and he won't need to stabilize his neck, but I forsee that surgery happening. After surgery he will have to wear a c-collar while he heals, and after that he will no longer require one. 

Why did you guys get him a trach? Will he have it forever?

Because of his collagen mutation, his skeletal structure is as so:

His head, stomach, hands, and feet are the correct size for his gestational age.

His arms, legs, and chest are four weeks behind developmentally.

Because of this, his lungs are tiny and his rib cage is short and bell-shaped. To throw even more odds in the pot, he was also born a month early - which means his lungs were two months early. And don't forget he was born with hydrops, so his chest and lungs were full of and surrounded by fluid. All of that stacked against you gives your lungs a real dousy of a work out. He physically cannot breath enough on his own to keep himself alive right now. He needs the trach and ventilator to force his lungs open enough to ensure he breaths to live. 

The possibility of him needing this trach forever is a reality, but I don't think it'll happen. Eventually his body will slow down growing, and his chest size will catch up. It seems like other children with the same dwarfism have a trach until around the age of three. But if he does end up needing it long-term, they have an attachment for trachs that allows him to speak through it. Until then, David, the girls and I have started learning sign language.

How tall will he be?

It's hard to say. These skeletal dysplasia's are so relative to the individual person! Aiden could range anywhere from 3'5", to 5'. I almost wonder if he will end up being on the taller side. Compared to other families I've spoken to he is pretty long in length for his age. But his growth could suddenly slow down at any point as well. So really, we will just have to wait and see!

Will growth hormones help him?

No. His body wouldn't know what to do with all those hormones. Because his dysplasia stems from his body not being able to properly turn protein into bone, hormones won't do a thing. Giving him extra protein won't help either for the same reason of not knowing what to do with it. One of the biggest battles we have continually had are NICU dieticians that freak out about his size, and give him ridiculous amounts of protein added into his food to try and force his body to grow faster. But all they accomplish is giving Aiden a tummy ache and bad gas. (And a nice earful from mama and papa bear)

His body grows the way it can grow - at it's own pace. Nothing extra that you give him will help. And even if it did, I wouldn't give it to him. He is perfect exactly the way he is. 

I have such a huge feeling of relief now that we have a confirmation on his diagnosis. I knew that it would come back positive. I mean this in the nicest, most non-derogative way possible - he very clearly will be a little person. But something about having it on paper makes me feel like everything is going to be okay. The geneticists have thrown the term "lethal" out the window, and it feels like a ton of bricks have been lifted off my shoulders after getting that word out of my life. I've heard the word "lethal" for far too long. We are getting even closer to the light at the end of the tunnel each day.

July 01, 2016

Mr. Grouchy Pants



Aiden has officially been out of surgery for one week - and he's a grouch! (Don't let that picture fool you *wink, wink*) He's been very quick-tempered; the moment someone touches him he tenses up and gives you a real crusty look. If you dare to do so much as clean out his mouth or change a diaper, he'll chew you out real good. And really, I don't blame him.

A lot of his grouchiness is due to having withdrawals from narcotic medicines. I find it so frustrating that these babies are made into little drug addicts in the NICU. Since their first days of life, they are often pumped full of every narcotic drug imaginable. Believe me, sometimes it's merited. I don't want Aiden awake while he's getting a PICC line. After surgery I want him on every medicine possible until he's comfortable. And he's had more then a few times where he's felt so air hungry from being upset, that the only way to get his body out of that panic attack is to knock him out.

But there has been so many other times that when he's the slightest bit fussy, and rather than checking to see if something could be wrong first, they simply go grab a PRN narcotic. These medical professionals can be so quick to medicate, medicate, medicate. I can't tell you how many times when I'm there and told them "no" to the extra narcotics, and to check to see if it's a burp, or wet diaper, or to change his position first; and 9 times out of 10, that's all that was wrong and he calmed right down.

It kind of bothers me. If these babies were at home, and they were fussy, would you automatically reach into the medicine cabinet? No. You would soothe them, burp them, swaddle them, bathe them, change them. There is a whole mental checklist of things that could be wrong.

Another problem is that a baby is constantly monitored in the NICU. If a child is upset and crying at home, I guarantee you that infant's heart rate is high, that their co2 levels are high, that they are desating and have low oxygen. And do you call the doctor in a panic when they are upset and purple crying? Well, maybe. BUT your pediatrician wouldn't write you a prescription for pentobar to knock your baby out while he or she is purple crying. You would soothe them, do bicycle legs for gassiness, burp them, try white noise...

You wouldn't automatically drug any child at home the moment they get a little fussy. So why do hospitals use this as an easy-out to a baby that is upset?

And the best part is, although these hospitals are the creators of these addicted babies, they will use it against you and tell you that because your child requires so many drugs, they are highly critical and don't have much quality of life.

And again, when he's in a complete panic because he feels air hungry, then I agree the best thing is to give him something to help his little body calm down. It's very similar to having an asthma attack, but the difference is that his body doesn't understand what's going on and you can't communicate ways to help him come out of it. So his body continues to panic, thinking it's not getting enough oxygen, and he spirals until he's physically exhausted and quite literally passes out. In these instances, narcotics and opiates can get him out of these spells before he's exhausted. But there are just as many instances when he's not air hungry, but he's pumped full of extra drugs. And all of these extra drugs really start to pile up.



So Aiden has become highly tolerant of any narcotic drug or sedatives. So much so, that immediately after his surgery in an attempt to keep him comfortable, he was given Ativan, Clonodine, Morphine, Methadone, and Tylenol around the clock. The first three days he was also getting Pentobar and Precedex on top of those others. That's every drug safe to give an infant, at the highest possible dose you can give him, every 3-4 hours a day. With extra PRN doses as needed.

Obviously this is a terrifying amount of drugs. And it's no wonder he's going through major withdrawal problems.

He's cranky, clammy, and shaky. He has major diarrhea and tummy cramps. But really my rant on medicine aside, he's doing well. Immediately after surgery, and even through his withdrawal issues, his oxygen needs and PIPs have been significantly lower. Even his current neonatologist exclaimed excitement over his ventilation needs- and that doesn't happen often! The trach seems to have opened him up and allow him to breath more easily. I can't wait until we get his medicine under control so we can start interacting with him like a normal baby.