November 21, 2016

Validation At It's Finest

This past Thursday, we had a huge meeting for Aiden. Our entire medical team at Primary Children's Hospital, the doctor from South Davis, and the skeletal dysplasia team at duPont Hospital for Children in Delaware was involved. And it. was. incredible.

Going in I didn't know what to expect. This being our biggest meeting with the largest number of medical professionals yet, I was definitely feeling nervous and intimidated. But it turned out to be more than David and I could have ever asked for or expected. And now more than ever, I am feeling very excited for Aiden's future.

Before the meeting started, I spoke with the geneticist in Delaware. She was kind enough to let me vent, and asked me to lay out all of our questions, concerns, and frustrations with our current medical team. I told her our three main concerns were his c-spine instability, his vent and not trying other modes or types if home vents for him, and his diet. She assured me she would address all if these concerns, and hoped our medical team would be willing to listen.

One of the main docs over the dysplasia team at duPont, Dr. Bober, started out the meeting by saying, "the first year of life is a very long, hard road, but there is absolutely a happy ending. And these kids go on to thrive." Wouldn't it have been nice for our medical team to have heard that months ago? He went on to describe how these children typically behave - that blue spells, passing out when they poop, requiring high ventilation settings, being easily agitated, and requiring high sedation needs in the beginning are all characteristic and normal. He laid out in detail a general medical history that many of these kids typically follow, and he literally described my kid to a T. After this, I felt confident the rest of the meeting was going to go well. But even then, this meeting still surpassed anything I expected.

We first discussed his c-spine stability. Now as a reminder to our past c-spine and c-collar drama, when Aiden was first born, he was handled as any other newborn baby would be. In fact, perhaps more roughly at times as he had to be intubated, extubated, and reintubated a few times. In order to do this, you have to hold the child's head as far back as it will go in order to have a straight shot down the throat. But when he was around two months of age and the geneticists diagnosed him, they threw out a comment of, "By the way, these children have a c-spine precaution..." and his medical team panicked. They wouldn't let anyone touch him unnecessarily, move his head, dress him in clothing (until I just started doing it), hold him, or get him out of his bed unless he was wearing a c-collar. They even placed "osteopenia precautions" signs all over his bed space to drive the point home and essentially scare anyone coming by to see him into thinking if they touched him, his bones could break. Now obviously Aiden's type of dysplasia doesn't cause him to have brittle bones. But they did that, nonetheless, all to "ere on the side of caution".

At one point they wanted to make him wear a c-collar 24/7, and David got into a very heated argument with the director of the NICU about that. And because of the c-collar, as well as insisting he lay in bed flat on his back with minimal movement most of his life, he has a severely flat head. In fact, many people have told me he has the worst case of Plagiocephaly they have seen in their entire careers. But they won't allow him to have a helmet to reshape it, either.

So when Dr. Bober asked what imaging the medical team had done to look into his c-spine and they couldn't answer him, I was both overjoyed and outraged when his come back was, "So you are telling me you are making Aiden wear a c-collar based upon an assumption? And you've never done what's necessary to explore that?" He went on to explain the MRI's they have done for skeletal surveys are not enough, and the only way you can really look into c-spine stability is if you do flexion and extension x-rays of his neck. I can't tell you how many times we have told them that ourselves, but they always refused to do it. Dr. Bober even said based upon what he can see in those MRI's, Aiden is fine and what you should expect to see. He explained that the c-spine stability precautions are always there for just about any type of dysplasia because they are "all head" when they are little. So you should always be careful. But, in his words, "risk is risk, and he still needs the opportunity to strengthen those muscles."

So this week, we are getting the images duPont wants to see in order to dig deeper into his spine. They will review them for our medical team, and we could very well be getting rid of his bleeping c-collars for good. I really hope so. In this case, they would even allow him to get a helmet for his poor, flat head! When Aiden puts his collar on, he shuts down. I could go on forever about the benefits this would have for him; developmentally, emotionally, physically, mentally... But if by chance duPont comes back and says, "You know, after looking at the images, the c-collar would indeed be beneficial for him", I would have a much easier time accepting it from the experts.

One of the biggest battles we've continually had but I've never really talked much about is Aiden's diet. There is no such thing as a comprehensive growth chart for SEDc children simply because it's rare enough that information on this dysplasia is just barely being tracked and recorded. David and I have continually made a stink about it, the Dieticians have bounced back and forth between plotting him on both average height charts and achondroplasia charts, and in the meantime Aiden has been getting more and more chunky. It's been incredibly frustrating. When I spoke to the doctors at duPont before the meeting and I expressed my concerns about overfeeding him, they asked me to email them progressive pictures of Aiden to see just how big he has gotten.

When we started discussing his diet, Dr. Bober told our medical team that the pictures I sent him were "shocking". The NICU director defensively jumped in and said, "Well we've been plotting his growth on the skeletal dysplasia growth chart...". To which David interrupted, "Actually you've been using the achondroplasia chart, there is no growth chart for him." Dr. Bober sighed and continued, "I don't recommend using an achondroplasia growth chart either. Individuals with achondroplasia have big heads, long trunks, and short limbs. Those with SEDc have big heads, short trunks, and short limbs. However, their limbs are slightly longer and end up looking a little more proportioned. Achondroplasia growth charts are going to be completely different curves."

Dr. Bober put his foot down, and told them he needs to only be growing 10 grams a day. 5 grams is OK, 15 grams is absolutely unacceptable. Our medical team has been letting Aiden continually grow around 350 - 400 grams a week. That's around 300 MORE grams a week than he should be gaining. My child weighs around the same as an average toddler with SEDc. As you can imagine, this momma ain't happy. Especially because this is something we have banged our heads against the wall with them about since he was a tiny newborn. His weight has gotten completely out of hand, and has gotten to the point that he can't move his arms and legs well anymore. The Dieticians are finally listening after duPont told them to stop. But why did it take the experts to make them finally listen? Average height babies should only gain 30 - 40 grams per day, achondroplasia 20ish grams a day. So why did they let a child with SEDc, who is SMALLER then average height babies, and EVEN SMALLER then achondroplasia children, gain 50 grams a day!? And if the growth chart isn't there to follow, his appearance should be more than enough to tell you you are doing something wrong. I'm relieved they are listening now, but I'm afraid we are so far off on his weight, it's going to take a long time for him to lean out and get back on track.

In the middle of all this, Dr. Bober made a really good point. He said, "If you weren't overfeeding him so much, he wouldn't need to be on three different Diuretics." Ya, go figure...

Based upon duPont's recommended daily weight gain, and Aiden's growth so far, David made this "growth chart" for him. Shocking, isn't it?

And as if all of this wasn't good enough, we haven't even gotten to the best part of the meeting yet.

I know you are all well aware of the frustrations we have been having with Aiden's home vent trials. The RT that has been giving us so much heartache was present for the meeting, as well as our pulmonologist that refuses to try other modes on the home vent. The RT began by laying out all the different ranges of numbers they have tried on SIMV mode on the home vent. She finished by saying that Aiden just can't do it, and they are running out of options. To which the RT at duPont replied, "Well, have you tried AVAP mode?" There was a long, uncomfortable pause as our RT squirmed in her seat before she said, "Uh, well... um, no." The RT at duPont quickly followed with, "These kids need AVAP mode, SIMV mode doesn't work well for them. They can also do well on LTV vents, but the latest Trilogy home vent model has more settings to play around with and could end up being more beneficial."

Can I get an amen? In this moment, I never felt so validated in my entire journey with Aiden so far. duPont echoed exactly what we have been trying to tell them for weeks.

They went on to suggest a PEEP study, which can help lead them to figuring out the settings he needs on AVAP mode on the home vent. This goes over my head a little bit, but they will essentially play around with his vent settings while there is a scope down his trach. This way they can increase and decrease vent settings as necessary, while watching how it affects his airway and lungs. Then they can take the results from that, come up with an algorithm, and plug that in to the vent.

The fantastic news to all this is duPont effectively broke down some egos - our RT and pulmonologist are going to do the PEEP study tomorrow, and then try the home vent on AVAP mode. This means we are no longer going to transfer to the long term care facility this week. That may still be in our near future if the team here can't figure AVAP mode out, but I have hope again to finish up at PCH. And I'm surprised, the RT that has been giving us so many problems actually went to the long term care facility and got a quick training in AVAP mode and got some supplies for Aiden. I can't believe she did that for us. Perhaps hearing that this is the only acceptable mode for certain children motivated her to finally learn about it.

We have a very busy week ahead of us. His PEEP study and flexion/extension imaging is tomorrow. We could potentially try a home vent trial on AVAP mode by the end of the week. And we reworked his food and will begin weighing him a couple times a week with a goal of gaining 7 - 10 grams a day. Big, exciting things are happening, and we are over-the-moon excited for it. Finally we have been heard, validated, and have experts advocating along with us.

But I must admit, a big part of David and I are also pretty upset about this meeting. Why were we the ones that had to reach out to the experts and have them consult? In our opinions, the minute we confirmed Aiden's diagnosis, the medical team here should have been humble enough to say, "You know, I'm not well versed in this type of dwarfism. I'd better find someone who is." The team at duPont made it overtly clear that the medical team at PCH has dug themselves into a hole in terms of Aiden's care. They even threw out a quick blurb that he could have been home by 6 months of age. That statement stung my heart pretty deeply. I also can't help but feel like the trust I had in his doctors here has diminished. But I am also so relieved that we have someone guiding us back on course. Now my biggest hope and prayer is that we can make some changes that will make a big difference in his life and he can get home to me quickly.

November 17, 2016

Happy Prematurity Awareness Day

3 years and 2 months ago, my little Lydie-bug suddenly entered the world 6 weeks early. She was purple, barely breathing, barely making a small whimper, and very sick. She was taken to the NICU at IMC hospital via ambulance, while I had to stay behind at Riverton Hospital. We were terrified. During her time there, I witnessed her completely flat lining and having CPR performed on her twice. She had so many tubes in and around her, you could barely see her tiny body. The body you could see was bruised and scraped. Doctors told us she might not make it, but she fought hard. She still has her own battles with seizures and VSD, but she's a vibrant, spunky 3 year old with an infectious laugh.

Then Aiden entered the world a month early, and while a long NICU stay was certainly expected for him, entering this world for a second time was even harder than the first. I recognized all the beeps, stress, and fear, and had to endure flash backs to Lydia's own battle while simultaneously living Aiden's struggles.

I've laid in a hospital bed, still feeling delirious from the anesthesia and so weak from my C-section that I was unable to move, as they wheeled my baby into my room on an ambulance stretcher so that I may say goodbye. I desperately bawled as the Life Flight Team wheeled my tiny baby away from me. The image of her gray, lifeless body as they took her away forever haunts my thoughts.

I had to experience this a second time, only in a helicopter, and cried as I watched my husband and son walk down the hospital hallway as I helplessly sat in a wheelchair.

Taking Lydia to IMC Hospital via ambulance

Taking Aiden to Primary Children's Hospital via helicopter

I've seen my babies so completely covered in medical equipment that you could barely see their tiny bodies. The little skin you could see was swollen, bruised, and scabbed over.

Too many times for both my children have I desperately watched monitors as their heart rates and oxygen levels plummeted. Too often during those times I yelled out my child's name and shook their tiny bodies in desperation to get them to come back, only to be elbowed out of the way as a swarm of scrubs entered the room and began to do chest compressions.

I have held my son's hand in a desperate attempt to console him as he silently screamed while the IV team poked him over and over and over again - once a total of 18 times.

I sat in the hallway and listened to my daughter scream as nurses attempted to put in PICC lines through her belly button, arm, and legs. My intuition surged through my body, telling me to run and protect her. But I couldn't - it was a sterile environment and I wasn't allowed in the room.

I've held back tears of frustration as I've attempted to get my sedated babies to look at and focus on me.

I've sat in a room full of doctors and ugly cried as they told me my son was very near death, and we needed to prepare ourselves and family for that.

I have witnessed more pain, sorrow, and helplessness after the birth of my precious babies then anyone ever should.

Being a NICU mother to premature babies is something I wouldn't wish on my worst enemy. It forces you to become a different type of mother and look at the world with a different pair of glasses. Not better by any means, but different. You experience every little detail of your child in a different light. Every breath they take, every smile they give you, every diaper you change, even the way they smell, becomes especially precious.

But in spite of the heartache and PTSD I'm sure both David and I have, I wouldn't change the way they entered the world. Yes they both have and continue to endure things no child should. But they have taught me how to love deeper, appreciate more, and showed me a strength I never thought I had.

They, including Melodie for being the big sister to two NICU siblings, are incredible children. I consider myself lucky to get to be their mother. NICU babies are a special breed of children, and their parents have the privilege of witnessing their fights. It's a terrible, emotional, often heartbreaking but just as joyful rollercoaster that makes you love them just that much more.

November 13, 2016

Major Disappointments

I know it's been awhile since our last update. A lot has been happening each day. And as I would begin to write an update, something else would happen that would cause me to start writing the update all over again. But I'm currently sitting at a birthday party for one of Melodie's classmates, and after trying to engage in conversation with the other moms that are obviously more interested in Pinterest, it's become clear to me that I have some time to dedicate to an update. (Side note, that drives me crazy. What happened to moms talking and getting to know each other at birthday parties?!)

Since our last care conference, David and I have been feeling very disheartened. And very frustrated. And frankly, pretty ticked off. At this point, what were we going to do? The feeling of desperation and helplessness plagued my thoughts and sleep constantly.

After praying, I felt inspired to consult the wisdom of the other parents with SEDc children. They directed us to the doctors at the skeletal dysplasia clinic at duPont Hospital in Delaware. I was aware of this clinic and experts before now, and have even asked our medical team to consult with them many times before on various issues. Unfortunately they have never done so themselves. So we determined we would have to do it ourselves. We weren't sure if the doctors would consult, but thought that the threat of us seeking a second opinion might at least put a fire under their bellies again.

After leaving several tearful voicemails with anyone whose name was attached to the skeletal dysplasia clinic, I anxiously kept my phone next to me at all times. The minute my phone rang, I'd jump to see the caller, only to feel a slight disappointment when the caller ID wasn't a Delaware number.

In the meantime we told our medical team that we reached out to the experts and to hopefully expect to hear from them. At this time, we happened to have a neonatologist assigned to us that had never had Aiden before. He's not new to the NICU by any means, but by chance was never put over Aiden during his rotation. Once this doctor found out we contacted duPont Hospital, he replied with a, "Oh, I know the head neonatologist at duPont as well. I'll email him myself."

David and I talked in awe over that coincidence. And really, can we count that simply as a coincidence? What are the chances that we happened to have this neonatologist for the first time, at the exact same moment we contacted duPont, that happened to know the head of the NICU over there personally? I'm continually overwhelmed by the ways we see Heavenly Father's hands in our situation. I know that He watches over us and ensures we have the resources we need to help Aiden.

After a very long week, duPont called back. Not only were they thrilled to learn another child with SEDc was born, but they were also anxious to help us as well. They contacted PCH, and we signed paperwork to release all of Aiden's medical records to them to look over. It took PCH another week to gather all of his info and send it out. But honestly, we don't blame them for taking that long. Last I counted, Aiden was up to 56 xrays alone - and I last counted back in July.

After we sent his charts off to Delaware, his medical team here decided to hook him up to the home vent and see what he did. (Putting a fire under their belly sure did work, ha!) David and I rearranged our schedules to be there for him when it happened. When they brought the vent into the room, I began to shake from nerves. I didn't know what to expect, but I stood next to him and held his hand. When they switched him over, he didn't even seem to notice. They kept him on for 15 minutes, and the entire time he chatted away with me, happy as can be.

He was able to do two more days of the home vent and got up to 30 minutes on it. But on that fourth day, he didn't even last a minute. The next day, same thing - couldn't do it at all. Every day since then, he hasn't been able to handle the home vent for more than five minutes. Every day his medical team has played around with the settings, trying different number ranges to see what might work - but nothing has worked.

At these moments, I've felt more defeated then at any other point with his journey thus far. And the biggest frustration lies from the fact that it's not him, it's the medical team. Everyone in the NICU agrees that we know he can do it, we just can't figure out the settings he needs to help him be successful. Everyone, even up to the director of the NICU, has expressed to us that they are "no expert" on the vent, and doesn't understand the machine well enough to help him.

I finally turned to the NP one day and said, "Do you even realize how incredibly frustrating and maddening it is to hear that no one in the hospital is trained on this vent? How much it ticks me off that a single person here isn't trained well and that you are incapable of providing the resources needed to help my son?" But all she could do was nod her head and say, "I know, and I'm so sorry."

David and I felt so desperate, we actually spent an evening looking at homes for sale in Delaware. We thought that maybe moving our family to the experts would finally do something beneficial for Aiden. But Delaware answered back, and mentioned that children with SEDc need to go home on either an LTV vent, or on a different mode on the Trilogy vent.

PCH doesn't have a single LTV vent available. So David and I spent an entire day tracking down an LTV vent at Home Care ourselves, only to find out the pulmonologist refuses to allow him be on it even though the skeletal dysplasia pulmonologist suggested it. Apparently the two of them have conflicting ideas about this vent. The expert claims it can be beneficial for our son's bell-shaped rib cage, while the doc here claims it's harsher on lungs. Whatever.

So after a whole day of phone calls and emotions wasted on our part finding this vent and ultimately doing PCH's job for them, we turned to the next suggestion, which is the different mode on the Trilogy vent. But the minute we brought up this mode, it was immediately shut down by everyone in the room. Apparently, and again, no one in that hospital is trained well enough on the Trilogy vent to try and play around with that mode, and refuses to even try.

David and I once again turned to the incredible families we have gotten to know so well, only to find a clear consensus - all the kiddos with SEDc that are on the Trilogy home vent are on this different mode of ventilation. Curious, and a quick Google search later, David and I found the Trilogy handbook online and looked up this mode. It's literally the exact same mode of ventilation Aiden is on now on the hospital vent, just a different name. Exact. same. mode. Are you beginning to feel the asinine frustrations we are feeling yet?

(Also, we've been doing this way too long if we can open a handbook for a piece of medical equipment willy-nilly, and understand every word of it.)

Out of sheer desperation again, we've brought these things up with his medical team, trying anything to get them to change their minds. But because no one is trained well enough, the pulmonologist doesn't think it's safe to try it for him. Even though Aiden's doing it - right now, as you read this. And we get the safety issue. But that's not the problem here. The problem is the obvious lack of training in the hospital concerning the Trilogy vent. And quite frankly, it's unacceptable to deny a child something he needs just because you are unable to adequately train your staff.

Unfortunately, we know that we just aren't going to win this one. There isn't much to fight about when people flat out don't know what they are doing. And this has left us thinking we now have no choice but to transfer Aiden to the dreaded long term care facility. Clearly we are at the point that sitting around at PCH is a waste of time. And ironically enough, South Davis has a fully capable RT staff that is well versed in the mode that Aiden needs. And if it turns out he needs an LTV vent instead, South Davis is more than willing to call Home Care about the LTV vent we found.

I'm terrified to be even thinking about this place seriously. I don't know how we are going to do it. But ultimately if they have the resources to get Aiden home, then we will do what he needs and figure it out. David keeps assuring me we've figured it out this far, so we can figure this out too.

We have a meeting with the head doctor and RT at South Davis this upcoming Thursday. We plan on very honestly telling them all the things we've heard that causes us to be nervous, and lay out our expectations for Aiden's care. I guess we'll see how it goes, as we have no other options left.