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.