Sleep Apnea?

Kyle still has something seriously wrong with him.

However, he amazes me. He is doing well (supporting his head) with his halo off. He has 2 neck collars and 1 neck collar/brace (that insurance does not cover, grrr) but Kyle doesn't like them. Keep in mind, Kyle has Klippel Feil Syndrome too so his neck is short, and apparently you need a neck for such devices because none of them truly fit Kyle.

Anyway, Kyle is just happy to be able to put his head on a pillow. However, Kyle still vomits every day. His voice is still muffled and hoarse. He still pauses in his breathing for long periods of time, consistently, while 'trying' to sleep. I've mentioned this to his neurosurgeons several times throughout the months but nothing came of my concern. So I got fed up and emailed his regular doctor at 1 o'clock in the morning on October 10th. At 8am his pediatrician told me to come see him that afternoon. At that appointment, Kyle's doctor scheduled Kyle for a sleep study to check for sleep apnea; also referred Kyle to a Head and Neck Surgeon to check out his throat.

October 12th, I took Kyle to see the Head and Neck Surgeon who used a scope down Kyle's nose & throat to see his voice box. He said Kyle's voice box cartilage is severely swollen and weak. So swollen that it is obstructive and flaps when Kyle breathes in and out. He believes stomach acid (reflux) has inflamed this area. He's not sure why the cartilage is as weak as it is but he suggested to start with medicines to reduce Kyle's stomach acid (which helps with his vomiting issue). If his voice box does not heal then Kyle will need surgery BUT the doctor said it would be an extremely dangerous and difficult surgery because of Kyle's restricted airway.

The Head and Neck Surgeon then told us he will review the sleep study, which is scheduled for October 16th, and let us know if Kyle will need a CPAP or not.

Should know more by the end of this week.

The Halo is Off

Kyle got his halo removed yesterday. He did great!!

His procedure was scheduled for 9am in the OR. Generally, a halo can be removed in an doctor's office but Kyle's neurosurgeon team wanted to removed it in the OR. I'm guessing the issues that occurred in May caused quite the stir so they wanted to be 'a little extra cautious with Kyle'.

The day before this procedure we received a phone call from the Pediatric Anesthesiologist who was with Kyle in May when that procedure went terribly wrong. He spoke with my husband and wanted to make sure we were okay with him being on the team for this procedure. My husband told him yes because he knows Kyle's history. A few hours after the phone call I received an email from the Pediatric Anesthesiologist explaining he will make sure Kyle is safe and comfortable for the halo removal. All of the fuss over Kyle's halo removal procedure does seem a bit out of the ordinary but we are happy that the neurosurgeon team is being cautious.

Kyle only took valium for the procedure. The doctors were planning on numbing each pin site with local anesthetic but Kyle refused. Kyle's dad and I was not allowed in the OR so Kyle took his video camera with him and asked one the nurses in the OR to video some of the procedure, and they did!

About an hour later we were called to meet Kyle in the recovery ward. I was excited to see him so I rushed to the ward and I forgot Kyle's bag of clothes and shoes in the lobby. ;)

He looked good! He had a collar on, hooked up to an IV and monitor leads. His long hair covered his right eye. He looked at me and said, "I feel good". He was rubbing his chest that we haven't seen in 11 months. He looks a bit soft which means he needs to tone up now that he is free from his halo.

The 10 pound halo and vest was now in pieces laying in a bag at the foot of his hospital bed.

The pin sites healed quickly. The doctor put a little circle band aid on the front two pin sites  but a few hours later the sites were nearly closed. The back two sites are hidden by Kyle's hair but they look fine too.

The neck collar they put on Kyle didn't fit. Kyle's neck is short thanks to Klippel Feil. So his doctor told us to go to the orthotist office after he got released from the hospital to have a collar and brace made to fit him.

The orthotics visit took forever! Kyle was hungry and tired but we couldn't leave until he got a collar and brace that fit him. The orthotist worked a long time on customizing a brace to fit Kyle but gave up and started working on a different type brace. It was frustrating but they finally came up with something that worked. However, when we left the orthotist office we walked to a nearby restaurant and Kyle was beyond frustrated with eating with his new brace because the brace pushes under his jaw preventing him from opening his mouth to eat. On the ride home he ripped the brace off.

Halo is Coming Off!

It's time!! The Halo is coming off!!

Kyle has been in the halo for nearly 11 months. November 8, 2011 - October 4, 2012. Decompression on Nov. 8, 2011. Fusion implants on Nov. 16, 2011.

Took Kyle to see his neurosurgeons on Sept. 24th. Explained to them he is still having difficulties in swallowing and his voice is muffled. Told them he is depressed. They agree the halo might be messing with his psyche and his sleep. They assessed him and agree the halo should come off. But they want to see another CT scan first. They ordered him to get a CT scan of the spine on the 25th to check out the fusion.

Two days later, I received an email from them mentioning the scan looks great. The halo can come off. Yahoo!! But they want to do it in the OR .... poo. Last time he was in the OR was a nightmare for a 'simple procedure'.

Took Kyle for pre-op yesterday. He is scheduled to get the halo removed on Thursday, Oct. 4th in the OR at 9am. The plan is to numb the pin sites and give him an oral sedation-type med. Kyle asked if his dad or I can be in the OR with him. No definite answer, so we will see. I'm hoping so and I hope they will allow us to video it.

My 11 yr. daughter says we need to celebrate "4th of Kyle" (because the procedure is happening on the 4th.) Time for a Halo Off party!!

Depression & Halo Issue

The month of August, depression hit Kyle. He didn't feel like eating. He didn't want to get out of bed. He wouldn't leave the house. He stopped hooking up with his friends online. He suffered from headaches every day. He vomited every day. He couldn't sleep. Part of it is the halo, he wants out.

Kyle's voice became muffled again and he is having major issues swallowing solids again. He's also congested, he's fighting mucus buildup in his throat and chest.

Why is he feeling so bad? He did well on the steroids for his swollen spinal cord. But a week after he was done taking the meds he no longer was ok.

I think, the halo is the problem. It is messing with his psyche. He's been in the halo long enough.....since Nov. 8, 2011.

By-the-way.....Is it possible to outgrow a halo?

To me, it appears Kyle is outgrowing the halo. It appears the halo is pinning him in a slightly bent position now. He won't strand straight. I can only imagine how difficult it is to swallow with a short neck (Klippel Feil) that is fused and being forced down and inwards to his chest now. The halo needs to come off. But sadly it's not up to me.

His neurosurgeons had him get a MRI and CT scan in late August. After reviewing the scans they told us everything looked fine. His swollen spinal cord healed. The pin sites were ok. But they didn't say anything about the fusion. They told us 'see you in November for another follow-up.' What? November? What about getting Kyle out of this Halo? I had to let Kyle know he might not get the halo off until November now. He screams at me. I don't blame him. I scream with him.

So I took him to see his Pediatrician. He needs help for the vomiting, the headaches, the mucus. Kyle's pediatrician puts Kyle on meds for a possible infection. Kyle has been taking amoxicillian, Flonase, Albuterol, and QVar since the first week of September. It's helping slightly with the mucus problem and his headaches eased off a bit too.

Kyle's voice is my alarm to 'something is wrong'. Losing his voice was one of his first symptoms of Chiari, before we knew it was Chiari. So it concerns me when his voice starts to disappear again, as it did in July when he had the trauma to his spinal cord.

Strangely, his voice is now mostly muffled when he is in an upright position, when he is lying down he sounds clearer. Kyle says he feels better lying down. Hmmm......

Painful Pin Sites - Upcoming Procedure

Kyle has been experiencing a lot of pain for the past month. He has been having headaches almost every day plus major pain at his four pin sites. His halo has been making clicking noises too but I couldn't figure out where the clicking was coming from. Plus Kyle has been having me help him lie down in bed at night and help him get up in the morning. He says he feels his halo shift every time he lies down or gets up and it hurts. My thought is Kyle is still growing. He has been in his halo for six months so the vest is tight and the pins are loose. For the past three weeks I have been emailing his physician assistant about these issues. Finally, we went to Oakland to see his doctor on Friday, (5/4). Unfortunately, we didn't get good news.

First of all, Kyle had a CT scan on Monday, (5/1) to check his implant/fusion. So, his doctor used this scan to give us the bad news/good news.

Good news - The fusion is improving

Bad news - The fusion isn't complete. Kyle will not get his halo off for a few more months.

Good news - His pin sites are clean, his halo vest is clean

Bad news - The halo pins are all loose. Kyle is in pain because the pins are rocking and scraping his skull. They cannot be tightened because the sites (the skull bone) are weak. If his doctor tightens the pins they will poke thru his skull.

Bad news - The pin sites must be changed immediately. To change his pin sites he has to be under anesthesia. So, Kyle is scheduled for surgery on Tuesday, (5/8).

Pray all goes well.

Yeah....A Good Sign

So we took Kyle to see his neurosurgeon today for a follow-up and to get the results from the CT scan from last week. Kyle's doctor was impressed with how well Kyle is managing the halo, and he said his pin sites look very good and clean.

The doctor assessed Kyle's strength and expressed Kyle has definitely improved even though Kyle is slightly weak on the left side of his body compared to his right side. Which is a bit strange because Kyle was extremely weak on the right side of his body before his surgeries. Anyway, the fluttering in Kyle's eyes are nearly unnoticeable now...which is a good thing. Two disappointing issues are Kyle's voice is a bit hoarse again and his swallowing is a bit labored again. Those two things may not improve.

So, the results from the CT scan did show 2 very small areas, where they grafted in bone for the fusion of his cervical vertebraes, has finally started to grow. Yeah! And there was no change (no movement) in the 2 bones (I can't remember the name of one of them but it is next to C2) compared to February's CT scan. Yeah! The downside is that Kyle may be in his halo for a bit longer than the original plan of 6 months. But I think he will manage.

Thank you everyone for keeping Kyle in your thoughts. It helps!!

Living With a Rare Disease

Welcome to the First Chiari Carnival Celebrating Rare Disease Day

Hello everyone. I am Kyle. I am 12 years old, in middle school, achieved green belt in Karate, and I have Chiari Malformation.

Chiari Carnival: Chiari is typically a birth defect that goes unnoticed for years or decades. When did you receive your diagnosis and how old were you?

Kyle: I received my diagnosis in September 2011 and I was 11 years old at that time. My parents knew something was wrong for over a year before we found out my condition. It took many, many trips to the doctor before being properly diagnosed. I had a MRI in September 2011 and that is when we immediately got a call from a neurosurgeon.

Chiari Carnival: Many Chiari patients have been diagnosed with other conditions. Do you suffer from any other conditions besides Arnold Chiari Malformation?

Kyle: Yes, I was born with Klippel Feil Syndrome too. So, my anatomy is different from normal people. Basically, my cervical vertebraes are deformed. We also found out I have only one kidney but that doesn't have anything to do with Chiari.

Chiari Carnival: Patients who have had decompression surgery are referred to as zipperheads. Are you a zipperhead and have you had any other Chiari related surgery?

Kyle: Yes, I'm a zipperhead. My decompression surgery was in November 2011. It was scary for me to find out I have Chiari then have surgery in less than two months later. But my case was severe. I was getting so weak and partial paralyzed. My procedures included decompression surgery then I was in traction for a week (to try to straighten my cervical vertebraes a bit). Then I had the fixation surgery (implants for fusion) 8 days after my first surgery which puts me in a halo for at least six months.

Chiari Carnival: Western medicine focuses on medicine and medical procedures. However, many people benefit from ‘alternative’ therapies. What is your favorite non-medical/non-medicinal treatment for symptom relief?

Kyle: Before my surgery, I had to lay down all the time. I didn't have strength to do much and because my parents and I didn't know what was wrong with me until September 2011 I didn't take many medicines besides Tylenol for my headaches and albuterol for my breathing (thought I was suffering from asthma but it happens to be a Chiari symptom). I don't have a favorite non-medical treatment for symptom relief.

Chiari Carnival: Since Chiari affects the brain and nervous system some patients report symptoms which are difficult to describe. What is your most bizarre symptom?

Kyle: I was getting weak on the right side of my body and I couldn't explain how it felt. My parents thought I was just giving up using my arm and they thought I needed to work out or lift weights to build muscle. But I couldn't explain why I couldn't use my arm, or why my right leg was dragging. I guess because I didn't know what was going on. Then I would fall all the time. My parents would look at me and ask why did I fall and I would tell them I tripped but they were confused because nothing was in my way to trip me. I would just lose all feeling for a moment that caused me to drop to the ground. The loss of control embarrassed me and frustrated me.

Chiari Carnival: Dealing with pain and symptoms day after day can be very challenging and many state that their loved ones just don’t understand. What would you like others to know about living with Chiari?

Kyle: I don't like to complain but....it sucks. People expect everyone to be able to do the same things. When I say I can't do something it IS because I CAN'T not because I don't want to. I want to feel good and do crazy things with my friends but I'm limited. Chiari changes you and I don't know if I will be able to go back to doing stuff that I use to do. Such as going on roller coasters, or jumping on trampolines, or snowboarding, or sparring.

Chiari Carnival: Attitude plays a huge role in feeling healthy. What activities do you engage in to keep a positive attitude?

Kyle: My friends, my neighbors, my family and all of my parents friends have said they have never heard me complain. (I do vent to my mom though). I just want to feel better and not have surgery again. I'm still in a halo and I want to get out of this halo and go back to school and be with my friends. Unfortunately, I'm expecting my neurosurgeon to tell me more surgery is to come because some of my symptoms are returning.

For now some of my activities are playing video games with my online friends from around the world;  flying kites at the park with my dad; go on walks with my mom and sisters; sweet-talk my mom in taking me to Starbucks; go to the movies with my cousins; and my not-so-fun activity of doing homework.

Chiari Carnival: A great quote can remind us to keep the faith during those dark moments in life.  Can you share a quote or saying that provides you with inspiration?

Kyle: "Learn from yesterday, live for today, hope for tomorrow. The important thing is not to stop questioning." - Albert Einstein

Chiari Carnival: On behalf of Chiari Carnival thank you so much for sharing some of your Chiari journey with us. 

Kyle: No problem! I'm happy to be a part of Chiari Carnival.

My mom helped me with this post. Thank you so much for stopping by and reading my story.

Photo-3 days Post-Surgery

This picture was taken three days after Kyle's second surgery

Halo Screws

There are four titanium screws to hold the halo in place which are screwed into my skull bone.

Recovery Notes from First Surgery

Kyle's first week in traction had major ups and downs. The first four days he was in so much pain.

On the third day post surgery: Kyle had a feeding tube put through his nose to his stomach to administer him Tylenol and Valium. No food.

One of Kyle's P.A. also adjusted Kyle's halo and tightened the screws. This was extremely painful for Kyle and hard to watch. My husband was so angry that they were hurting Kyle but the P.A. said there was no easy way to do it.

So, that night the doctors allowed him to have a PCA (patient-controlled analgesia) which means he was in control of his pain medicine. He would push a button and a certain amount of pain medicine (morphine) went into his vein through the IV (intravenous) tubing. Only he was allowed to push the button.

On the fourth night at the hospital one of the nurses and the E.R. doctor moved Kyle's head to try to see the stitches on the back of his head. This scared me a lot and it hurt Kyle. The doctor moved Kyle so fast by rolling him to the right side of the bed, but didn't stabilize Kyle's head or the traction. (Kyle now had 4 lbs. of weight on the traction/halo). Then the doctor rolled him to the left side. They didn't position his head correctly after the move and it ended up hurting him a lot which took the next 3 hours to try to correctly reposition him and control his pain again.

So now, Kyle was a little more comfortable but very bored.

By the fifth day, Kyle was moved to another room in ICU. His new room was larger, had an awesome view of the S.F. Bay Bridge and had a T.V. in a position where Kyle could see it.

Unfortunately, Kyle was not allowed any water or food in fear of aspiration pneumonia so he was extremely thirsty all week.

Fitted for Halo

I got fitted for my halo today. Actually the technician just took measurements of my head and chest. I ask if it comes in different colors. He said no. Too bad beings I have to wear it for 6 months. I wanted it to be red. I also found out I will not be able to take showers. :(

Halo Traction

I found this site that describes the halo traction. I don't want to think about it. The thing gets screwed into my head and weights are added to it. I'm told I will be kept somewhat sleepy for the week that I am in this thing.

I don't know if everyone who goes through decompression surgery has to have this but I think I do because I have Klippel Feil.