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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.

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