Chiari Comforts

Chiari Carnival 6
Chiari Comforts
Therapy, tricks, or tools for symptom management

I asked Kyle how he manages with Chiari. He says, "I just do." I guess he's right. He just does 'cause what else is he going to do? Lately he talks about how much he hates all this. I don't blame him, I don't like it either. I'm often sad for him but I try not to show it. I wait to break down after my house is quiet and everyone is asleep. I watch him sleep from his bedroom doorway. I think about how much he has grown and changed. I think about how hard it has been for him lately.

His symptom management is to rest when he feels yucky. He doesn't push himself too much anymore to keep going. A week ago, I tried to make him go walking with me around the park in our neighborhood. He didn't want to go but I made him. We didn't get too far until he just sat down on the sidewalk and refused to move.  I was thinking, 'great, how am I going to get him back home?' Finally, I had to bribe him. He likes that actually. But I'm glad he refused to walk with me because he is understanding his limits; he knew he wasn't feeling good and unbeknown to us at that time, he was suffering from major swelling in his spinal cord.

Kyle doesn't focus on his Chiari or his pain. Well, maybe a little on his pain. He focuses on Minecraft and Call of Duty. He focuses on things such as how to be in the hot tub/spa without getting his halo vest wet; he focuses on making me take him to get ice-cream or Starbucks. He loves to manipulate me. And I let him. I know he's bored, really. But he's limited on the activities he can do.

For pain, all I give him is over the counter meds. He doesn't like to take many prescription meds (yet). I'm sure as he grows and if things change he will. My favorite pain reliever is naproxen, so I give that to him the most for his neck and head pains. I've used ice packs on the back of his neck too. Not sure if it physically helps but he can rest and relax a bit.

One of his 'tricks' I guess you could say is he chooses to drink his nutrition more than eat. I'm not sure if this is because of the Chiari or because of the limitations to swallowing due to the halo. I mean, he can't stretch his throat (tilt his head back or forward) like most people do to swallow. I'm curious how it will be for him when he gets out of the halo. I'm guessing his fusion might make stretching his throat difficult as well. Any Chiarians want to comment on how it effects them? But he's big on shakes and ice cream and avoids any foods that is hard to chew and swallow.

Other tips he favors: Take Diazepam/Valium right before MRI's; use a wheel chair for an all-day outing; and rest as soon as you feel dizzy.

Take care and stay strong.

Swollen Spinal Cord

Kyle has been sick since July 13th. He's been vomiting every day, he can't and won't eat. All he wants to do is stay in bed. I've noticed he is laboring more than normal to swallow, breathe and speak. I've been trying to figure out what happened; why does it seem his Chiari symptoms are returning all of a sudden. Kyle had a CT scan on the 13th. I was there. Nothing seemed to have harmed him. We saw his CT scan results on the 16th with the P.A. and the results looked good. Of course, the CT scan is mostly to see how his fusion is looking. It cannot show how his spinal fluid is flowing.

The P.A. on the 16th said Kyle's fusion is complete from top to bottom but the bone itself is not thick enough so he will be wearing his halo for at least 3 more months. Devastating to Kyle because he was hoping to be able to go back to school. Anyway, I explained to the P.A. that Kyle has been sick since the 13th but she didn't feel it was anything to do with his Chiari.

Four days later, I take him to see his regular pediatrician because he was getting worse. His pediatrician wasn't in on the 20th so we saw a pediatrician that was available. That pediatrician prescribed Kyle a nausea medicine that cancer patients take.

Four days later, Kyle still isn't better. His voice is nearly muffled. He won't stand or walk. He won't eat. I email a desperate message to the neurosurgeon. First thing the next morning another P.A. calls me and tells me to take Kyle to get another CT scan and then come see her. When we met the P.A. she said no change in the scan from the one on the 13th. So they don't know why he is feeling the way he is. I mention he needs a MRI. We need to know if his spinal fluid is flowing correctly. She calls the neurosurgeons, whom were in surgery at that moment, they tell her do the MRI immediately.

2 days later, we are rushed in for a MRI. Two MRI technicians and three radiologist were there during the session. And Kyle's neurosurgeon was reviewing the images live from his office. Kyle was in the MRI for about 1 1/2 hours. I stayed in the room with my hand on his leg so he knew I was there.

After the MRI we were told to immediately go to his neurosurgeons office. His doctor needed to speak with us.

We get to Kyle's doctor office. It is now after hours. No one is around except us and the neurosurgeon and his team. The neurosurgeon examined Kyle and appeared very upset. He asked us if Kyle has had any accidents or falls. He showed us the MRI images and explained Kyle's spinal cord shows signs of trauma. His spinal cord is swollen and the spinal fluid is pooling at one location. My heart dropped. Kyle, with his muffled voice, says, "Am I going to have more surgery?" "Not for this, Kyle, not yet," says his Dr.

Kyle's doctor prescribes a steroid for him to take for the next 2 weeks. The hope is the meds will reduce the swelling. The hope is Kyle's voice will return. The hope is he will start to feel better.

I want answers.....

I'm so angry and sad and...well I just want my Kyle feeling better. I'm afraid his Chiari symptoms are returning.  And yet he still is wearing the halo, "for three more months", his surgeons say. But in the meantime, Kyle is losing his voice again. He won't eat, he's lying down all down, and he's weak. I want answers. I want to know if his spinal fluid is flowing correctly. I want to know why he is losing his voice again.

Klippel Feil Syndrome

We haven't mentioned much about Klippel Feil in this blog but it is something Kyle lives with as well as Chiari Malformation.

Klippel Feil is an uncommon birth defect. We were told it is not genetic but likely caused by a vascular problem during development. We were told that during my pregnancy (around 6-8 weeks gestation) the blood supply to Kyle got cut off, which could have been for a split second, and it effected his spine, his heart, and his kidneys (for all we know). Of course, if you google Klippel Feil you may find other reasons for it but this is what we were told by two genetic doctors about 12 years ago.

So, before Kyle turned 1 year old we found out Kyle has Klippel Feil Syndrome and his related birth defects included fusion in his cervical vertebrae (absent arch at c4 and deformed body of c3), abnormal ribs, a heart murmur, and one kidney. It wasn't until Kyle was almost 12 years old we found out he has Chiari Malformation.

Kyle is very smart and all boy. He was very, very active until he was 9 years old when he started to slow down and I believe that is when Chiari started effecting him.

How we first noticed something was wrong with Kyle was the obvious issues of short neck, his head tilt to one side, and he could not turn his head (we noticed his shoulders turned with his head.) His doctor though it might be torticollis so he had Kyle in physical therapy to "stretch" his neck. A big no-no once we found out it was a bone issue. My suggestion to parents is to have x-rays done before any physical therapy.

Kyle totally outgrew the tilting of his head and I can't really say why or how but he did. Although, when he is sick or extremely tired I notice a slight tilt in his head. What people notice the most is they think Kyle is shrugging his shoulders upwards. Which of course he's not but because his neck is short it does appear that way. His shoulders roll forward more than they should and looking at his back one shoulder blade is higher than the other. Recently we noticed his upper rib on the right side is protruding out a bit. 

We were told there are a few precautions with Klippel Feil such as no contact sports; avoid extreme flexion, extension and rotation of the neck. Although Kyle tries to defy these rules quite often.

My other main concern for Kyle is his single kidney. Kyle has to watch what he eats and drinks to keep his kidney healthy. He has always had a higher level than normal of creatinine and blood urea nitrogen (BUN) in his blood. His kidney specialist watches his levels closely to make sure those levels stay within the norm for him.

Kyle has not had any surgery for Klippel Feil. Although his Chiari surgery did have to take his Klippel Feil into consideration which made his decompression surgery difficult and slightly different from other people with Chiari.

I love my Kyle with all my heart. I hate that he has many health issues, but he is a very strong young man. Not to mention he is fun to be with and a pleasure to have deep conversations with; ask anyone who knows him! ;)