Pre-Op and Surgery details (Part 1)

After we scheduled the surgery and had already put things in motion we found out that because of Presidents’s Day and Dr. G being out of town on a skiing trip with his family, if we wanted to have any opportunity to really sit in front of him and ask a bunch of questions and talk through the surgery at length, our only chance was going to be the Thursday beforehand. So we decided that Henley and I would make a quick trip up and give her a chance to have some fun before surgery. If you know me well, you know that I make it my goal to infuse fun in at every opportunity. It’s a technique I affectionate refer to as “sandwiching” LOL. I like to sandwich all of the crappy stuff Henley has to endure in between some fun (or at least more enjoyable) stuff.

Case in point, last week when we had to get blood work at home (PRE-Pre-op bloodwork) I kept putting it off and Henley kept saying “Mom, don’t we need to get my bloodwork done?” …… “Hey mom, when are we going to go get my bloodwork?” Finally I was like “Wow, you seem really eager to go have bloodwork done, what gives?” She replied “We always get ice cream afterwards” and gave me the biggest grin. I was like “Okay sister, I can see what’s going on here.”

Henley knows that anytime there is something hard that she has to do, we try to make it quick and then get on with our day. When she has MRI’s, we  treat it like an errand we have to run. “We’re just gonna run downtown and have some pictures made for the doctors and then we’ll get back to our day.” When possible, we go grab lunch or a special treat or do something fun. Overall, it just helps the mental game that so much of this is for kids. I have found that the bigger of a deal I make of something, the bigger of a deal it is for her. She is following my lead….and how I frame these things will leave a lasting impression on her. If it’s daunting, dramatic, drawn out and emotional, then she is going to take my energy and run with it. Then one day she will be an adult and do everything to avoid all those things that feel hard. If I frame it like an errand, it’s no big deal, we knock it out and get on our way. Then hopefully she won’t avoid doctors later on and procedures and bloodwork because of her memory of her childhood. This is a long game I’m playing here.

Here are a couple of videos I did recently on my IG where I showed what it looks like on days we “get pictures made for the doctors”.

This is when we did the final CT scan in December that Dr. G requested.

This was one of Henley’s MRI days that landed on Halloween and so we tried to make it fun and wore a costume (anything you wear can’t have ANY metal in it at all for this to work just FYI)

So back to the appt…sorry this is already a long post. Probably going to need a part 2.

Yesterday we spent an hour with Dr. Greenfield talking through everything. His PA came in and ran Henley through a bunch of Neurological testing to confirm what had been reported and she confirmed that Henley’s reflexes are non-responsive in her legs and her coordination is “sleepy”. She was examining her scar on the back of her head when Dr. Greenfield walked in and I was explaining that Henley hates her scar being touched and she flinches and says “it feels weird”. Dr. Greenfield felt it and he was like “oh yeah, it feels really tight, like her muscle is pulling inward. This can absolutely cause pain”.

Dr. G sat down and we exchanged our usual “it’s been a while” and getting a general catch up on everyone.  Even though we have exchanged emails, and phone calls over the past couple months, its just different sitting in front of someone for the first time in several years. We talked about his weekend family ski trip and Henley’s upcoming horse show, my recent appt with one of his colleagues for the stuff going on with my health and we recapped the past couple of years and how things have been up until recently.

He went over some statistics about kids like Henley who have Chiari Malformation and said that sometimes age at surgery can really affect surgical outcomes. For instance, In 2-3 year olds, 25%  of them will need a second operation just because their skulls are still forming and overtime they may need more space opened up. A child who has a Chiari decompression surgery between ages 5-6 has even less of a chance of needing a second surgery. If you are new to this blog, and Henley’s story… Henley had her first Chiari Decompression at 26 months old and a second surgery at age 7. She had incredible results from both surgeries, but symptoms crept back in years later which required the second surgery. It’s been 6 years since her surgery in 2017 and about 6 months ago we started to see the decline that felt familiar and after lots of testing, loads of imaging and professionals weighing in, here we are again. Surgery is in 5 days.

Dr. Greenfield asked Henley how she was feeling about things and if she noticed things being different verses 5 years ago to which she replied “kind of”. It’s hard for kids to even be this self aware sometimes right? They don’t go through life examining their words and how they articulating their “s” and “r” sounds, They aren’t focused on how they are walking and if they are cutting with scissors or writing like they used to. They just live their life. If their friends are running, they run with them, they aren’t noticing their leg weakness, they just know they get tired and need to sit down and take lots of breaks. Kids are really good at compensating for areas that aren’t working well, and they adapt.

Dr. G addressed several of my technical questions about the surgery and he went over her imaging to make sure we were all on the same page. I think he could sense my need for reassurance that we were making the right call and he gave me that. He said he would prefer there to be a bit more cushion of fluid around her brain and assured me that this is not experimental procedure and that he was not stressed at all about this decision making process at all. He assured me that the team would take care of her and keep her safe, that they would get her in and out and back to her routine as soon as possible.

With that, we said our goodbyes and headed straight to the airport to catch a plane to Houston for Henley’s horse show this weekend.

New York: Round 1

Over the course of Henley’s life I’ve made it my mission as her mom to try to help her walk through all that she has experienced with a balanced perspective. Sometimes we go through seasons of hard and painful stuff, but if we aren’t careful of our mindset, it can take up permanent residence in our soul. As a mom, I’m doing my best to factor in not only the present moments, but the long term mental recall of everything Henley is walking through during these crucial years in her development. My goal is to help her develop a mindset and attitude towards the challenges she faces that will help her to be able to confidently and calmly walk through hard things while being able to advocate for herself and know what it looks like to trust her God given instincts when things aren’t right.

In our home we don’t  pretend that things aren’t hard. We know that what we are dealing with totally sucks, it’s not fair, it’s painful, it’s uncertain, it’s anxiety ridden and it’s physically and emotionally taking a toll on all of us. But none of that discounts the fact that these times can be really important character building moments. I truly believe that a test makes way for a testimony and the Lord will use our testimony for great things if we let Him. Part of that is being brave enough to speak when it’s easier to stay silent.

When we found out that we were going to have to come to New York early in order to be able to meet with Dr Greenfield face to face before surgery, we decided to add in an extra day or two and have some fun in the city before we spent a lot of it…not having fun. So Henley and I are here packing it all in. We’ve done things on this trip that we’ve never done before and we’ve managed to have a good time. Mixed in with a pre-op anesthesia meeting, bloodwork, and a sit down with Dr G about the big day next week. I’m thankful for a bunch of saved up airline miles and hotel points that have allowed us to infuse some extra days of fun into our schedule.

Now we’re heading back to Texas for one last horse show before the big day! Thank you so so much for all of you who have messaged and texted and who are praying. You have no idea how encouraging it is to hear from people I haven’t heard from in years. It blesses me more than you even know. ♥️

Reflection.

Isaiah 43:1-3 “Do not fear, for I have redeemed you; I have called you by name, you are mine. When you pass through the waters, I will be with you; and through the rivers, they shall not overwhelm you; when you walk through fire you shall not be burned, and the flame shall not consume you. For I am the Lord you God, the Holy One of Israel, your Savior.”

This verse pretty much sums up the promises we have relied on for the last 6 months. I feel like it’s important to be reminded that the Lord NEVER promised us that our lives will be easy and free of trouble. However, He does promise that we won’t ever be left “alone”. Our faith WILL be tested. The Bible tells us to “rejoice in our sufferings, knowing that suffering produces endurance, and endurance produces character, and character produces hope!” Romans 5:3-5

So much of what we have gone through has been a relentless testing of our faith. It’s hard to really even grasp what faith truly looks like until it’s put on trial. For me, it has looked like saying over and over “Lord, I don’t have this, but I trust that YOU are 10 steps ahead of me and will provide a way through this firestorm that I can’t even make sense of.”

This has been the hardest lesson for me to learn. Having almost no choice but to TRULY surrender my life and the lives of my children, placing them back into the Lord’s capable hands and stand to the side and release control. To truly trust at my core that HE has got this and doesn’t need my help, rather just needs me to put one foot in front of the other and just. keep. walking. down the path He put me on.

I have said many times “Lord, I DO NOT have the strength, I need you to give me whatever its going to take to get me through this season.” and He has. HE has made us Brave. HE has given us supernatural peace. HE has allowed us to have the perspective we have on life. HE has given us comfort when we don’t understand why things happen the way they do. HE has orchestrated schedules and conversations and and doctors and people to happen right on time. The Lord has NEVER left us alone in this. He has ALWAYS been way ahead of us and provided for our EVERY need. I would be foolish not to be shouting about His goodness from the rooftops.
Someone told me once “God rarely shows up early, but He is NEVER late. He is always right on time.” So true.

A friend shared this song with me about 6 weeks ago and its been our anthem. I pray that you will seek the Lord in whatever you are dealing with and let HIM show you what it truly means to Be Brave.

www.bravecampaign.com

Update: First week home.

We have been home now almost a week. This past week has been a complete blur for me. Most days I couldn't have even told you what day it was, what the weather was like outside, where my children were or what time it was. 

We arrived back in Texas Monday evening. It was about all I could do to make the trip home from the airport. Henley did great. She had no problems on the plane with the pressure and she has been off pain meds completely for almost two weeks. She is seriously making brain surgery look like it was a piece of cake after that first week. They always say children bounce back like you wouldn't believe, but seriously, It's pretty remarkable. The minute we got in the car at the airport I had to take all of my pain meds just to be able to endure sitting one more hour in the car ride home from Love Field. 

We got to the house decked out in our winter gear and it was about 90 degrees outside. In addition to the strangeness of the drastic weather change, walking into our house felt strange. We've never been away from our home for such a long period of time. It was perfectly clean and smelled like home but it took some mental re-adjusting to the space. We dropped our things and Henley wanted to go straight to her room. Her room had been completely re-decorated while we were gone by some of my very sweet friends. The whole room is just precious and I can't wait to share pictures soon. Henley is in love with her new space! I held it together long enough to be super excited with her about her room and then quickly headed downstairs, went straight to my room, put my pjs on and collapsed. I was OUT...for days. I mean literally, I did not wake up again until Wednesday. 

I felt like falling into my bed was like collapsing across the finish line of the longest marathon I'd ever run. I just laid there staring up at the sky....for an entire week. My mom spent the days here with us to help with everything. Laundry, meals, cleaning the kitchen, taking care of kids and me. You name it. She is a rockstar. Adam went to bed Monday night with a fever and spent most of Tuesday in bed nursing a cold. We were both down for the count. Our physical bodies and emotions just completely and utterly spent. 

Wednesday I woke up in a LOT of pain. Adam had been talking to Dr. McClure in New York almost daily. When Dr. McClure called to check in on me on Wednesday, he suggested we go back to the ER to get some more imaging to make sure there was no internal bleeding or new findings. Not wanting to go to the hospital and truly wanting to be able to get the pain under control myself {stubborn}, I spent the day trying everything I knew to get on top of the pain. I finally ended up throwing in the towel that afternoon around 3:00pm and told Adam to just take me to the Emergency Room. I could not understand why I was still in SO much pain and it had been an entire week. I was taking a LOT of very strong pain meds and I still felt like dying might be easier. I took my last two Percocet pain pills and Adam drove me downtown to the hospital. We walked in the door and the minute we told them the story of the past week, they went into high gear. I have never seen an Emergency department move so efficiently {I knew it was possible!}. From the time I walked in the door of the hospital until I was being wheeled into the CT scanner for a CT angiogram was about 15 minutes. It was truly impressive. They ruled out internal bleeding which was our main concern. They checked my kidney function, urinalysis and bloodwork. All came back within normal range {Praise the Lord!}. With those concerns at ease, then came down to pure pain management. The nurses came in and gave me a 25mcg of fentanyl. 15 minutes later I had ZERO relief. They gave me toradol next since it had worked last week...15 minutes later NO RELIEF. They finally came back in 15 minutes after that and gave me 100 mcg of fentanyl and that finally kicked the pain. The doctors concluded that the pain is because of what is called "Infarction" which is tissue death due to inadequate blood supply to the affected area. Most people have heard of Myocardial infarction which is the partial death of heart tissue aka: a heart attack. So basically, it feels like my kidney is having a heart attack. ALL. THE. TIME. Imagine every part of your abdomen from your belly button on your right side all the way around to your spine cramps up so intensely that you feel like you might explode from the pressure. That's about how I have felt for the past 11 days. Well, the good news is... It's just part of my kidney dying. Everything else looks Fantastic! {insert sarcasm here}

If you know me well, you know how much I can't stand medications. I lean towards all things natural and so having to rely on these medications so heavily and be subject to their unruly side effects has been a very hard pill to swallow {see what I did there?}. However, I was thankful to have them and I was thankful they were working. We were able to go home much more relieved Wednesday night and I was put on a very strict schedule of taking my pain meds in exactly 4-6 hour increments {alarm included}. I spent the next two days pretty out of it as you might imagine. Throwing up because of the meds, but needing them to keep the pain under control. Saturday morning, 11 days post-op was the first time the pain wasn't the reason I woke up. I decided to take advil instead and see if that work keep the pain at bay....it did. I went another 6 hours and took another round of advil and so on and so forth. I made it through an entire day without the hard core meds.

Sunday, I finally felt like the fog was lifting. Like I could see clearly for the first time in a week. Slowly but surely I was able to get up and take a shower and get ready to go to church. I was so ready to get out of the house. I expended way too much energy getting ready, but I made it. After church I came home and went right back to bed for a couple hours. I am really hopeful that the major pain has finally subsided and I can move forward without the drugs. Tomorrow I see a specialist at UT Southwestern who is a personal referral from the Doctors in New York. This doctor will be the one following my case from Texas. 

First of all, thank you for praying. We know that we have been on the front lines of a serious spiritual battle this year and we KNOW the power of prayer. Thank you for linking arms with us and praying fervently for our family. We have felt the prayers and there is no other explanation for the way we have been able to walk through this other than the fact the The Lord has provided for our every need. "but those who hope in the LORD will renew their strength. They will soar on wings like eagles; they will run and not grow weary, they will walk and not be faint." Isaiah 40:31 

We are so thankful to everyone who has signed up to bring meals, sent cards and gifts, who have donated to the go fund me site, and have bought t-shirts and necklaces at www.bravecampaign.com you guys are seriously amazing!! You have orchestrated and declared Brave Days at school and on the baseball field. You have overwhelmed us with love and support and shown thousands of people what it looks like to be a part of the body of Christ. You have encouraged our family and carried us through in ways you may never know. Our plan is to take what The Lord has done through our story and turn Brave Campaign into a non-profit and keep paying it forward for other families like ours who need help traveling for treatments and who are facing times where they have to step up and Be Brave. We hope that you will continue to help us by shopping for a purpose at the site (we still have lots of t-shirts, necklaces and bracelets!), knowing that the money from your gifts and purchases will be stewarded well. 

Serendipity

Made it to Serendipity. We wanted to make sure we did a couple things with Haven & Zane before the blizzard came in. This banana spit was AMAZING!!

CUPCAKES!

She got her cupcakes!! Thank you Jeri Tidmore & Grandmommie for these sweet deliveries. Henley is improving so quickly and we are so thankful.

Peace out PICU!

We are so excited to have been released from the PICU. Henley is actually OUT OF THE HOSPITAL. We are kinda shocked, but she is doing remarkably well and although she is still on some pretty major drugs, we are able to manage the pain without IV meds. We have moved her back to our little hotel across the street from the hospital and are planning to have a peaceful and uneventful recovery for her here over the next week. Our main concern is keeping germs off of the back of her head so we are doing a lot to keep hands clean and use the topical medications we have on her incision site. We also have to work with her on moving her neck and head because her neck is very stiff after the surgery. We are encouraging her to look SLOWLY from side to side frequently. Henley has a follow up appointment with Dr. Greenfield at the end of this next week and then we should be released to come home over the weekend. 

New York is expecting a Blizzard on Tuesday & Wednesday so we are excited to see this place covered in snow! 

We have changed out plans a little bit and decided to stay put in this one hotel until we come home. So our address for the rest of our time here is: The Helmsley Medical Tower 1320 York Ave # L, New York, NY 10021

Top 3 Questions I get asked about Henley:

I realize some of you want more details than just the "cliff notes" version and it just dawned on me that I hadn't written anything about the actual surgery that took place and the details of that. I also tend to get the same 3 questions about Henley over and over so I figured, it might be easiest to just address all of those questions in this post. 

1. What did they actually do in the surgery? Well, they first shaved the back of her head. Then they made an incision down the midline of the back of her head. They had to cut through the neck muscles first before getting to the skull, then they were able to access her brain through the area they had removed at the base of her occipital bone in her previous surgery 5 years ago (yes, that means she has been missing bone on the back of her head all this time). Once through the muscle and occipital boney area, they reach the dura (the covering of your brain). They cut through that and were able to get a good look at everything from there. Once they were inside, they determined that her herniated cerebellar tonsils were still healthy tissue and so they choose not to resect those (i.e. cauterize that part of her brain that is falling below her foramen magnum). He felt that he was going to be able to give Henley's brain the space that it needed by sewing in a patch to give her brain some more room to breathe and for spinal fluid to flow freely. There are many approaches to this and where the "patch" tissue comes from. Some surgeons use bovine tissue or other animal tissues. Dr. Greenfield was able to use Henley's own tissue by harvesting tissue from another area on her brain and then placing it over where they had cut the dura. 

So think about it like this, when you get a new pair of jeans and the knees are really tight, then over time you wear out the knees of your jeans and they fit looser or sometimes you rip them and have to sew in a patch. It's the same concept. Henley's brain is just too big for her skull and so in order to keep spinal fluid flowing, they needed to free up some space so that she doesn't have a fluid collection on her brain or in her spinal column. In the first surgery when she was two, they thinned out the lining of the brain (the dura) in hopes of giving her brain casing more elasticity so it wasn't so tight in there. That worked for her for a good 5 years but things change as children grow and so it's a moving target. This time we had to sew in a patch to give more space.

2. What kinds of symptoms was Henley having that caused you to start investigating? Man, if I had a dollar for all the times this question was asked! Here is a list:

* Speech Difficulties. As in, it became physically difficult for her to get words out of her mouth. She would sometimes mix up letters in words that she had been saying for years or not be able to say things clearly that she had always said before like "foot". By the end of the day she was so exhausted from a day of talking she just wanted to be quiet. Over the summer, we noticed ourselves asking Henley to repeat herself over and over because we truly could not understand what she was trying to communicate. 

* Trouble walking. Henley has always gotten overly tired and had trouble walking long distances, but she began tripping and falling a lot. One of her teachers noticed her trip over her feet about 4 times one day while she was walking with assistance from one room to another. She described this as if Henley's brain wasn't telling her feet to move as fast as her body was trying to walk. It became pretty apparent that this was happening more and more frequently and she seemed to just get more clumsy and would be bruised up all the time from one thing or another. When we tested her for physical therapy and occupational therapy, it was even more obvious that she was really overcompensating for basic tasks she couldn't do. For instance, walking a straight line or balancing. This girl would fail any and all sobriety test just walking "normal". We learned that she appeared to the average person that she was just like other kids because she would speed up the activity to make it look like she was able to complete something, but when we slowed her down, she could. not. do. it. She would get so frustrated and cry because it was like her body just wasn't cooperating.

* Difficulty Crossing Midline. This comes into play when you are trying to do things like driving, writing or basically anything that forces you to cross one part of your body over to the other side. Like moving your arm across your body to the other side to grab something. Henley would just overcompensate and do everything in her power to avoid crossing midline. This is a sign that your right brain is not communicating with the left side of the brain. This also can affect the top of your body not talking to the bottom part. For example, jumping jacks? hilarious. (sorry that was terrible) 

* Daily Pain. At school, you would have zero idea because Henley hides it so well. She doesn't want the attention and she really doesn't know what "no pain" feels like. Every. single. night. for months and months she would need an ice pack at bedtime and complain of a headache, leg pain, back pain etc. By the time she had finished holding it together for a day of school, she would just let it all go when she came home. Some days even though she really wanted to go outside and play, she would just lay on the couch while everyone else would go play in the backyard. 

* Trouble Swallowing. We started noticing that she was physically having trouble swallowing certain foods and sometimes would chew and chew on something that she had eaten forever (i.e. grilled cheese sandwiches) and just end up crying because she couldn't seem to swallow it and would have to spit it out. It was heartbreaking. She would chew on simple things like bread and then it was like watching her try to swallow rocks. During a swallow study, we learned that she was overcompensating by drinking out of straws and when we took the straws away and tried to have her drink from an open cup, she would choke. 

* Tiring Easily. Henley just started getting less and less able to handle walking any distance. She would ask to be carried or to ride in a cart constantly. At Target, the grocery store, pretty much everywhere we go, someone was needing to carry her. She is 7...not a toddler. 

3. What about that "other situation" you talked about? You said there were potentially two problems and this surgery was addressing the most pressing issue. So here is the story on that. It's hard to explain in words but I'll do my best. If you look at Henley's MRI from right to left you will see her cerebellum and the herniated cerebellar tonsils that travel down next to her brainstem. Then you see her brainstem in the middle and then her odontoid on the other side of her brainstem (looks like it's at a 45 degree angle). This angle changes when you move your head up and down. It's basically a joint. Henley has Ehlers-Danlos Syndome (EDS) which is a genetic connective tissue disorder that is caused by a defect in the structure, production, or processing of collagen or proteins that interact with collagen. Collagen is kind of like your body’s glue -it is what holds your skin, joints, blood vessels, and other major organs in place. It typically presents itself in childhood or young adulthood with hypermobile joints prone to misalignment, stretchy skin that tears easily, and fragile blood vessels prone to cardiovascular complications (such as aneurysms). Loose ligaments can misalign the proper angle of the odontoid bone causing it to push backwards, compressing the brainstem. Chiari Malformation is a downward displacement of the cerebellar tonsils (part of the brain) that puts pressure on the cerebellum and brainstem from the other side, progressively damaging them over time, and blocking the flow of cerebral spinal fluid (CSF). So, if you understand what I just wrote then you understand that we basically have two potential problems that cause compression of the brainstem. One is on one side and one is on the other. The reason for her surgery this past week was for the Chiari and what it is doing to the spinal fluid flow and the compression from the back of her head to the brainstem. Often times when you do the Chiari surgery to remove the pressure on the back side, it causes even more instability on the other side. SO, all that said, you have to have a surgeon who understands both of those issues and how one effects the other so that they can take all of that into consideration. There is a very real possibility that one day down the road Henley might have to have a Craniocervical fusion. It's an awful procedure and we want to pray against this for her. So please, every time you think of Henley or our family, pray against any instability in her spine and head. 

So, there you are. Those are the most commonly asked questions I get and there are your answers. 

Letting Go.

We were able to suit up and go into the O.R. with Henley. They gave her versed i.e. "Giggle juice" to help to make her relaxed before going back. Apparently that is not standard procedure around here, but we requested it because her emotional state is of HUGE importance to us. After all, we are the ones who will deal with the emotional aftermath in all of this and they will get to walk away. 

Going under anesthesia is terrifying for Henley. It is for most people to be honest. However, there is one memory that neither one of us can seem to shake and that is the memory she had when she came out of surgery the first time when she was two. Immediately upon waking Adam and I were right there with her. I leaned down to hug and kiss her and the first words out of her mouth were "I'm so sorry mommy." As if she had done something to deserve all of this. To deserve this pain. I have never been the same. 

We have spent years talking through this, trying to make it okay. It's not. I don't know that it ever can be okay. Those memories run deep and she remembers them even 5 years later. She remembers waking up crying and she remembers being sad. 

I can't even talk about it all without crying. 

Yes, I'm sure I need therapy....we probably all do. 

So, we were able to go into the O.R. and meet a few of the people who are with Henley this morning. We told them that there were thousands of people praying over them today. Henley was really silly thanks to the drugs. They began putting all of the monitors on her, stickers all over her chest etc. Henley started making comments about payback and putting stickers on all these guys when she was done! When it came to the mask and the anesthesia it was again....traumatic. You can see it in her eyes. You can see trauma happening. There are no words for this. I prayed over her and and begged the Lord one last time to stand in our place and keep her safe. We kissed her and were escorted out of the O.R. I lost it when I walked out of the room. The child life specialist was there with us and Dr. Greenfield followed us out of the O.R. He reassured us that she was going to be okay. 

There is nothing that makes this easier. Walking away and handing over the life of your child is excruciating. My only hope is knowing the I left the Lord in there with her and HE is watching over his baby.

Field Trip to the hospital

Well, this was just about the best idea ever. It made a HUGE difference for Henley. We have learned that when we can put Henley in a leadership role, it helps her to overcome some of her fear. So we told Henley that since Haven & Zane had never seen an MRI machine or she was going to need to "show them the ropes" and give them a tour. Honestly, she has never "seen" an MRI machine either since she has always been asleep, but she has way more experience in hospitals than the other two and so she took on the role confidently. 

Haven and Zane were more willing to jump up on the table and try it out. Zane was especially excited about the "movie goggles" they had. We told them they could all get a turn, hoping that Henley would actually get on the table and we could get her to have a non-fearful experience with the MRI machine. She did great and we all felt much better after this was all over. 

Tips for other parents: If this is something your child ever has to do, I would HIGHLY recommend #1 doing it at a children's hospital if you can, and #2. Ask them about taking a tour beforehand. I have learned from several child life specialist that if you can help them walk through the experience with a stuffed animal or themselves it helps because they know what to expect and they can go into a procedure knowing that they will come out okay. We also use a lot of simple terms with Henley when we talk about procedures. For MRI's we tell her that it's time to take her "special pictures" of her head instead of using the word MRI since that means nothing to children. We are very honest with her when something is going to hurt or she is going to have to have blood taken. I will prep her beforehand and say. Remember we are going to see Dr. R. Remember, he is just a "talking doctor" (which means we just talk about stuff, no ouchies happen in their office).  I tell her too, "Hey, we have to go get some blood taken because the doctors need some information and your blood has the answers they need. Yes, its going to hurt, and that TOTALLY stinks big time, but afterward we can go do something fun. Would you like to stop for Ice Cream or Cupcakes?" This way she knows she can trust me and I am always going to be truthful with her. She also knows that I am her warrior mama bear and that I wouldn't let anyone do anything to her that was not for a really good reason. She knows that I am on HER side and that, she and I are working WITH the doctors to make her healthy. Lots of kids who deal with medical stuff feel left out of the decision, they often feel blindsided when adults don't tell them things because they think kids don't understand. I am here to tell you that THEY DO! Henley remembers things from when she was two years old, and how we handled things and set the stage for her then has made a difference in her perception when she is seven.

During our MRI Field trip, we made sure to praise all of the small things she freely chose to participate in. She certainly held back and was more than willing to let her siblings go first, and they obliged because they didn't have any previous fear of the situation like she does. Once the other two had gone we asked her if she wanted a turn. We asked her every step "Would you like to put the movie goggles on so you can see the movie?" She shook her head. "okay, well you have to lay down like Zane did so that they work." She laid down and we let her sit there for a minute knowing that we were all right there and that there was zero pressure for her to do any more than that. Then we said, while you are watching a movie is it okay if you ride in and out of the machine like Zane did?" She was reluctant but agreed. The operator slowly moved the bed in, waited a minute and then moved her slowly out of the machine. We asked her if she was ready to get up and she said "yes" so we took the googles off and let her hop off. With each step we call this "collecting positive experiences". We don't ever push her, but just do what she is willing to do and if she gets stuck on a step, we stop and talk through it. I will ask her "Would you like to see mommy do it first?" or "How about we let Draff (stuffed animal) try first" Generally kids don't even know what they are afraid of, they are just afraid, so we have learned that if we can take very small steps and collect positive experience along the way then she feels so much less anxious about the situation the next time. For Henley this works, I realize all kids are not like her, but we have certainly had to learn a lot about helping her to overcome fearful experiences.

Prayers Needed.

Preparing for Henley's surgery in March, the neurosurgeon in New York felt it would be helpful to have a "flex and extension MRI" study done. For several reasons this is important information to have. Basically, they want to see what happens to Henley's brain when she tilts her head down and back. To do this MRI they generally have to hold a very uncomfortable position for about 30 min each way. Because it's difficult enough already to get children to be perfectly still in an MRI, this procedure at Henley's age requires her to be sedated. Anesthesia is the absolute scariest thing in Henley's world. But alas, this is part of her world and so sometimes we have to do things that really stink. 

Today I got an unusual phone call from Cooks Radiology about 11:00. The man I talked with said that their neuro-radiologist had just reviewed Henley's scans from September and felt this procedure was going to be too dangerous to do under anesthesia due to the severity of her Chiari. Basically, they are afraid it might completely cut off the oxygen and or fluid to her brain and she wouldn't be able to tell anyone. They went back and forth with our surgeon in New York, but the bottom line is that it's up to the Neuro-Radiologist to make that call. SO, with that said we are going to try to somehow get these images WITHOUT anesthesia and we will have to just get what we can with Henley's cooperation. She is still pretty young to even try doing an MRI without anesthesia so we need some major prayers over her tonight and tomorrow. Please pray for her to have peace and to be able to get this done without a panic attack like we saw in Boston. If she can make it through this procedure, we likely won't have to do sedation ever again for MRIs which would be HUGE for us! 

The guy I spoke with at Cooks was super concerned about Henley and wanted to do everything he could to help make her comfortable with a super uncomfortable procedure. He asked if I thought it would be helpful to let Henley come down to the hospital tonight for a "trial run" and to have a "field trip" of sorts. 

He agreed that we could all eat dinner with our respective families and then meet up at Cooks tonight at 7:30 for a field trip. We are taking all the kids in hopes that Henley can show her siblings around her hospital. Sometimes when we can put her in a leadership role it helps to lessen the fear factor she would normally have. 

So here we go. Wish us luck and say a prayer. Hoping tonight goes well.

Henley Update Part 2

The day after we got home from Boston I called up to school to talk with the school counselor. I expressed my concerns about all of the things we were seeing with Henley and asked what we could do. By the time I got up to school 3 hours later they had already had a meeting with her teacher, principal, counselor & speech diagnostics person. They had information for me and a plan to move forward. Let me just stop there and say that this school our kids go has almost 1,000 elementary students in this building (most schools have 4-500). Let that sink in. These people are so busy and within 3 HOURS of me calling up there, they were already on it! We are BEYOND thankful for the school our children are in. When I walked into the office, most of them already knew what had happened in Boston and knew how defeated I felt. The principal said to me “You know we will help you however we can.” What a blessing to feel this supported in our community. 

I felt like the day we arrived back home began a whole new chapter for me. I now had lots more information, lots more people expressing concern about Henley and I felt like I wasn’t crazy or over analyzing her all by myself. We started down a path of investigating her in every way that we could. We tested did a preliminary speech eval in the school. The Speech Therapist called me and told me that she noticed Henley seemed to have developed a bi-lateral lisp since kindergarten. When I asked her if this was something that commonly developed later in childhood she responded with “Not generally. It’s most common when a person has had a stroke, develops Parkinson’s or has some other neurological complication.” Super…that answers that.

I called and made an appointment with our pediatrician to talk with her about all of the new concerns. We met that week and I laid everything out for her that we were seeing. She helped us get a plan of action together to start checking things off the list. 

First up: Speech. We decided rather than waiting on the school to do a full speech eval, we would have one of my best friends who owns her own speech clinic in the area do a full speech evaluation on her since she would be totally comfortable with her and because she also knew Henley’s entire history since she has been around since the beginning. After the test she scored the results and sat with me on the floor and explained it all. She said “I generally get concerned and recommend speech therapy if a child scores as low as a 10 on this test. Henley is at a -1.”  So there’s that. I guess we add speech therapy to the list of things we need to do now. 

Then we tested her for OT (Occupational Therapy). During this testing, they are looking for complications with fine motor skills and gross motor skills. That seemed to highlight even more issues we had not noticed before. Both fine motor and gross motor were of great concern. This day just keeps getting better. Add OT to the list too. 

Next up was the ENT. We went there to see if maybe Henley’s tonsils were abnormally large and that was causing the recent problems with eating and swallowing. Nope. Normal size. We also addressed the headaches with her doctor and took in an MRI image of her sinuses to discuss. Apparently children don’t really even develop sinus cavities in their forehead until later in childhood. So that’s good to know. The headaches are not caused by her sinuses….because she has none. 

Next up: Allergy testing. We did this to make sure Henley’s headaches were not being caused by something in the air that she was just allergic to. Nope. Not she’s not allergic to anything. 

The ophthalmologist was next. Maybe the newly developed reading problems and headaches were caused by poor vision? Nope. That is not our problem either. She has incredible vision. 

Frustrated about still having some unanswered questions I started writing emails. I emailed the head of the research team for Henley’s genetic disorder and also a Neurologist at CHOP (Children’s Hospital in Philadelphia) that I had email addresses for. I listed out everything we were seeing and asked if these things were related to the genetic disorder or if these seemed more neurological. The response I got from both of those people was that yes, there could be some overlapping symptoms, but these things generally were not coming on later in life, rather had always been present. They both directed us back to our neurosurgeon for consultation. 

I posted all of my questions in our Facebook page for the 16p families. I got an overwhelming response that matched the two doctors replies. “Yes, we do see lots of those things, but not generally acutely onset. I would probably see a neurosurgeon, it sounds like what you are describing is neurological.” one other mother said.

I texted all of my Chiari mom friends and got their thoughts and opinions too. One afternoon I got a call from one of the other moms. We talked for a while and she told me about the new doctor they went to see in New York and how amazing he was. Another one of our mutual friends was traveling there to see him also. I got the doctors information and called his office. Adam and I talked about it and decided to get a second opinion from him. I started gathering information to send to him before we could schedule a phone consultation. 

That brings me to today…

This morning I got up, got ready and was heading out to BSF when my phone rang. It was the school nurse. “Henley is in my office and crying because her back is hurting. That’s a new one. Right?” “I’ll come up there and check it out. I’m already in my car.” I went in and could tell she was kind of a mess. I knew just by looking at her that her back was out of alignment because her shoulders were slanted to one side and she was standing funny. I called our amazing chiropractor and got an appointment. We went home to wait. During the time before her appointment I got a random message from my other friend who sees the doctor in New York. Her son actually just had surgery for his Chiari about 10 days ago. They are still there and she messaged me out of the blue. She said that she had been thinking about me and Henley and wanted to just pass along some info about this surgeon who had done her son’s surgery. I texted her back a picture of my stack of papers and MRI images I was getting ready to send him for a second opinion and asked her if she could call me. She did and we talked for an hour. During that conversation I texted Adam the message she had sent me before our call and he responded. “What do we need to do to get an appointment with him?”. I responded“I think we just need to fly there.”

After our call we drove to the chiropractors office. On my drive I called the doctors office in New York and told them we had changed our minds and wanted to come there to meet with him rather than just talking on the phone. We scheduled an appointment for December 8th and that was that. 

We got to Dr. Haggerton’s office and he adjusted Henley and made her back feel better instantly. He said to me “its kind of unusual for a child her age to have this type of back problem.” I reminded him of the Chiari and he said “Oh yes! Well, all of this is related to the cerebellum so that makes sense.” I asked him about some of the other things we were noticing just to get his opinion on everything from a physiology perspective. He said to me something I have never heard before and its exactly what I needed to hear. He said “Well, this only gets worse as she grows.” He further explained that when we grow our brain stays where it is and our spine stretches out and pulls on the brain. (not his words…he was much more eloquent.) With Chiari your brain is basically “falling out of your skull” already and so it just keeps getting pulled further and further down the spinal column. I asked him that if she were his child, what would he be doing every single day for her. He gave me some great suggestions to feed her brain and give it everything it needs to function properly, but more importantly he said exactly what I needed to hear. He pressed his finger on my shoulder and said “If there is pressure here in your shoulder and its causing all kinds of pain and problems and you keep growing and your arm stretches out and pulls even more on that stressor but you never release the pressure from here (where his finger was pressing), then you just keep having the same problems. You have to fix the source of the problem first and then you can worry about regenerating all the functions.”

Then he looked at me and said “I feel like the Lord wants you to know that you have done everything you can here and you are doing a great job.” “Go see that doctor in New York.”

I feel like I needed to hear everyone that I truly respect in the medical & holistic field tell me that I need to go in a direction. The SAME direction. I feel like the Lord has been so gracious in giving us absolute clarity in the way that we needed to receive it before moving forward. So here we go. 

Henley Update: Part 1

Today is the day. I finally got the answer to my recent prayer. Prayers for direction, for CLEAR unmistakable direction. I’ve been praying this prayer for months. While waiting on the Lord to give me the answers I have been wearing my investigator hat. I have been waiting to post anything until I really felt like I could wrap my head around what has been going on and what we were dealing with and how we needed to move forward from here. 

I can not even count how many people have asked over the last couple of months “how is Henley doing?” If you had asked me June, I would have said “Things are great, we are in a really good spot right now and things seem to be pretty stable.” However, if you have asked me the same question since August you may have gotten a less straightforward answer. Only because I truly didn’t know what to make of the little things we were seeing at home.

Over the summer Adam and I noticed ourselves saying the phrase “What was that?”, “What did you say?” to Henley more than usual. It seemed as though something was different, but just slightly. Even Haven would be asking her to repeat things because she could not understand what her sister was saying. We tried to find a pattern. Was it when she was overly tired? Was it when her head was hurting? But really, we could not narrow it down to any specific cause. As August went on, it seemed like the speech kept getting more lazy, more slurred, more “slushy” sounding, especially when the weather changes.

Henley was also complaining about a headache EVERY. SINGLE. NIGHT. for several consecutive months. Every night we would use her essential oils, fill her diffuser and she would ask for an ice pack to sleep with on her head. Same routine. Every night. In the morning she would wake up, report no pain and run on about her day like there was not a care in the world. What do we make of this? What is causing the headaches? Is it the Chiari? Is it that she is over exerting herself and its causing fatigue headaches? Is she allergic to something in the air? Are her eyes bothering her? So many questions. 

In early August Henley also started having some slight problems eating. I say slight because they were inconsistent and some times they would be a huge deal and some times not at all. Weird things too. Like all of a sudden she stopped eating the crust on her bread. And not just like a “I am 6 and I just decided today I don’t do crust on bread anymore” but more like she would put it in her mouth, chew it and chew it and then have to go spit it out in the trash can. It was like she couldn’t figure out how to swallow it all of a sudden. We even tried other breads, like a croissant. She would chew and chew and when it came to swallowing the bite she would grimace like it was painful to swallow the bite. She even mentioned a few times “It’s hard to swallow” or “It hurts to swallow”.

Henley had her yearly MRI and check up with her Neurosurgeon at Cooks the first week of September and he stated that things looked the same from last year’s MRI and that while everything is still very compressed in her head, unless there were symptoms, we did not need to intervene, i.e. have another surgery. He chalked up the food issues to a simple childhood food aversion and we told him about the headaches to which he basically told us we could put her on a migraine medicine but that was pretty much our only option. Frustrated with those answers, but feeling like my hands were kind of tied, we went on. I mean, I am no neurosurgeon….just a mom, right? What do I know about my own child?? ugh. 

Henley started First Grade this year and has a phenomenal teacher who we adore. I know that she was hand picked for Henley and it’s a perfect match for her. This teacher is unique and perfect for Henley because her own 28 year old daughter has had her own extensive medical journey similar to Henley’s, and so this momma, not only has many years of teaching experience under her belt, but she has also been in my exact same shoes with her own baby and understands it all in a way that so many others just can’t. I say all of that to say that the Thursday before we left for MIT in Boston, I got an email from her expressing concern about Henley. She mentioned that since the beginning of school she had noticed regression in her reading and speech and just connecting in general. My initial thought was that we were only about a month into school and that maybe Henley just was not as comfortable with her new teacher yet and was needing more time to adjust. I asked if I could have her Kindergarten teacher pull her to asses her as well and give me her opinions too. She did and came back with the same concerns. She stated that not only did she see the same problems her new teacher observed, but that she felt like Henley wasn’t quite “all there”. In addition to that she mentioned that while walking her back to class she witnessed Henley trip 4 times in the hallway. When I asked her to explain, she said “It’s not like she is tripping over her shoes, It’s like her brain isn’t telling her feet to move fast enough”.

We went to MIT in Boston to participate in a research study being done by Simons VIP Connect that is studying Henley’s rare genetic deletion 16p11.2. This specific study was going to just so happen to be looking at how the brain processed speech with a fMRI (Functional MRI). This study is done in an MRI machine while the person is awake and they ask the person to partake in activities while watching what happens in their brain when they do. THIS did not go well. Long story short, it was traumatic. I saw a side of Henley that I have never seen before. I saw her in a full out panic attack and it was….hard. I cried, she cried. It was not happening. We were able to complete some other parts of testing they were needing, they assured me that it wasn't a totally wasted trip, but it sure felt like it to me.