Hmmm, The Magic of Steroids

Kyle took his prescription of steroids from Dec. 11th to Jan. 4th for treating his swollen spinal cord.

Major changes occurred during this past month.

First of all, Kyle slept all night on Dec. 17th WITHOUT his BiPap autoSV machine. I was nervous to let him but he fell asleep fast and I checked on him several times throughout the night and he did fine. However, I still tell him every night to use his machine but it's been a hit and miss since 12/17. But it's weird, right? To think he was pausing in his sleep over 100 times an hour and it was because his spinal cord was swollen. All the sleep tests Kyle had to go through and waiting for a sleep machine could have been avoided...possibly. Grrr.

Secondly, he's been full of energy. I know steroids does that but he's been off the steroids for about 20 days now and he is still full of energy.

His voice came back! My favorite change!! His voice is slightly scratchy but definitely louder.

He has NOT vomited since Dec. 11th. Kyle's favorite change. ;)

He CAN sneeze. When he sneezed normal for the first time last month it surprised me because it's been a long time since he sneezed normal.

Swallowing food is easier and he does not have to drink water with every bite of food to push it down.

No headaches. A good thing!!

I'm truly thankful that Kyle has done well this past month, and I just want it to continue!!

His next MRI is this weekend and we meet the neurosurgeons on Monday, 1/28, to find out more about the next procedure. I'm hoping the next procedure can wait a while so Kyle can be Kyle!

Spinal Cord Swollen....AGAIN!!

Kyle still vomits every day, he still can't sneeze normal (I know this sounds weird. I'll explain in a bit), he still has headaches daily, has high blood pressure, his voice is still muffled/hoarse, and he still does not breathe in his sleep. Frustrating!!

By-the-way, no word from the speech therapist since the barium swallow test. I didn't think we would anyway. The test was a waste of time because I know there is nothing she can do.

So, remember on Nov. 5th I posted that one of Kyle's neurosurgeons mentioned that Kyle's brainstem is compressed. He based this on Kyle's Nov. 2nd CT scan. He told us the odontoid (bone) is the cause for the severe anterior compression of the brainstem. Based on Kyle's images it appears the compression is on or near the medulla oblongata area of the brainstem. So I researched what the medulla is responsible for and I read it contains the cardiac, respiratory, vomiting, and vasomotor centers and deals with autonomic, involuntary functions, such as breathing, heart rate and blood pressure. Yikes, right? So, we had to wait from Nov. 5th to Dec. 9th for another MRI to be done.

So, Kyle had a MRI on December 9th. (Kyle had his birthday a week before this appt....he's now 13).

We met the neurosurgeons and team on Dec. 11th. The neurosurgeons immediately started asking us if Kyle had fallen recently. Which he hasn't. Kyle hasn't done much for months because he feels so yucky. Then they tell us they are not sure why BUT Kyle's spinal cord is swollen.....AGAIN!! At this point I wanted to scream 'I knew it!' but I was thankful knowing Kyle would be prescribed steroids again to fix this problem. It hurts me to think about how long Kyle has been suffering from this swollen spinal cord. I have no doubt that it has been swollen since before the halo was removed. Now, this doesn't mean he is out of the woods for the face-splitting procedure. The doctors mentioned the procedure again but says they will address the issue next month after his spinal cord heals. For now, they put Kyle back into a full brace and he is on steroids for about a month.
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SNEEZING ISSUE: For some reason when Kyle has to sneeze he basically sneezes inwards and then it causes him to vomit. He cannot launch a sneeze outwards like a normal person would.

Swallow Test

Kyle had a barium swallow test on the 15th because of his long term difficulty with swallowing. I think the speech therapist wanted to see if he has reflux and if he aspirates.

Kyle has explained several times to his doctors that solids get stuck in his throat so he pushes it down with three hard swallows and a sip of water. Many times he ends up vomiting because the food will not go down no matter how hard he tries to swallow. The speech therapist breifly mentioned that his trachea is partially paralyzed. I haven't confirmed this with the head & neck surgeon yet but obviously something is wrong because his swallowing isn't normal.

His speech therapist doesn't want him to swallow water with food. She feels Kyle could possibly aspirate the food and water. She says water slides down too fast so she wants him to only swallow foods that are moist, or swallow with a thicker type liquid such as a smoothie so the food and liquid would go down slower. Kyle didn't agree with anything the speech therapist said; even though it makes sense but he's the one that has to deal with it.

Anyway, the xray did show food getting stuck in his throat and it did take him about 3 hard swallows to get most of the food down without any water to push it down. Luckily, we did not notice any reflux.

What? ....... Split Kyle's Face Open?

Kyle had a CT Scan on Nov. 2nd, one month after the removal of his halo. Then Kyle had an appointment to see his Neurosurgeon on Nov. 5th. We don't have all the details yet. I pretty much was stunned when the doctor told me about another possible surgery for Kyle. MRI is scheduled for Dec. 9th and we will have another meeting with the neurosurgeons after that.

The bad news:  Kyle's brain stem is still compressed. Hence, the symptoms he still has:

Central sleep apnea
Hoarse voice
Partial paralysis in trachea
Difficulty swallowing
Shortness of breathe
Weakness in arms
Chronic vomiting

The doctors briefly mentioned the next procedure but to get to the bone that is causing the issue they would have to split Kyle's jaws and tongue in half. As soon as I heard that I went mentally blank!

Sleep Apnea Diagnosed

Sleep Apnea Confirmed.

Sleep apnea is a condition where you stop breathing while sleeping. There are two main types of sleep apnea, Obstructive and Central. In obstructive sleep apnea you cannot breathe because your airway is blocked. In central sleep apnea you do not try to breathe because something is wrong with your brain that controls breathing during sleep. A person is considered to have sleep apnea when they stop breathing for 6 or more times per hour.

Kyle's lab test showed he stopped breathing 101 times per hour

The following is an excerpt taken from a post found on the Web:

"My son passed away at the age of 8 on March 29, 2008. His cause of death is listed as "complications due to Chiari Malformation Type 1". Due to sleep apnea that was caused by the Chiari, my son simply, slowly stopped breathing and passed in his sleep."

This scares me.

There are many, many web site discussions over the link between Chiari and Sleep Apnea. Of course, I didn't know much about sleep apnea until my son starting showing signs of this disorder. So it quickly became my mission to research sleep apnea for Kyle. Not sure why Kyle's sleep apnea rear its ugly head months after Kyle's decompression surgery but I'm determined to find out.

If you suspect sleep apnea - get tested immediately because the process to getting a machine for sleep apnea takes time.

For Kyle, he met his primary care giver on Oct. 10th. Then he referred Kyle for a home sleep study. We had to wait a week for an appointment with the sleep clinic to pickup a home diagnostic device. Kyle did the home sleep study on Oct. 16th. We returned the device the next morning. Kyle's Head and Neck Surgeon called us with the results Oct. 18th and told us Kyle has severe sleep apnea. The Sleep Lab Clinic called us the next day to schedule on overnight sleep study at their lab for Oct. 31st. We didn't want to wait so we were referred to an outside clinic for Oct. 20th. Oct. 20th he stayed the night at a sleep lab and did the titration study. Oct. 24th our Sleep Lab Clinic calls and tells us Kyle's sleep apnea is central and obstructive so they want to do another titration study on Oct. 31st for a  Bilevel CPAP Auto Servo-Ventilation. So while we are waiting for that appointment, we requested a loaner CPAP machine. The doctor doesn't think the CPAP will do much good because she believes Kyle will need the ASV, but we received a temp CPAP machine today with the pressure setting of 7 based on his results from Oct. 20th.

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.

New CT Scan

Kyle had another CT scan today. This scan is to be compared with the previous scan. My hope is this CT scan shows signs that the fusion is taking place as planned. According to the PA they are also looking to see if 2 specific bones have slipped since Kyle was in traction. I tried to understand the x-rays and it does appear one spot is being pinched off a bit preventing his spinal fluid to flow correctly, but I'm not sure. Unfortunately, Kyle's voice is getting hoarse and he is getting tired in the middle of the day which could mean his spinal fluid is not flowing correctly again. We meet the neurosurgeon next week for more information and what to do next.

No Good News

We met with Kyle's neurosurgeon team to get the results from the recent CT scan and we didn't get the news we were hoping for.

Before the PA showed us the x-rays he asked if anything has changed. My husband and I mentioned that we noticed Kyle is laboring to swallow again and his voice is starting to be hoarse again. Then the PA showed us the x-rays and explained why those symptoms are returning. Unfortunately, the bone that they expected to show signs the fusion is successful is slipping and causing his Chiari symptoms to return. Kyle was completely stunned and upset. He was expecting to hear that he only need to wear the halo for maybe another month or so.

The Plan:

So, Kyle will get a series of CT scans again within the next month and we will see his neurosurgeon on March 12th. If the bone is still slipping then Kyle will need more surgery BUT no definite plan on how to handle it. If the bone stays where it is at and starts to fuse then possibly they will leave it and he will have to live with his current symptoms forever.

Time will tell.

Please pray all will be well.

Headaches

Kyle says he has been having headaches/pain in the back of his head. He says the best way to describe it is like an ice-cream 'brain freeze'. They happen several times a day. Definitely something to ask his doctor about when we see him in a couple weeks.

Chiari Awareness

Taken from Chiari Awareness Flyer

Chiari affects about 1 in 1,000 people. Most common symptoms are severe headaches, balance problems, pain and weakness in the arms and legs, trouble speaking and swallowing.

Shannon's Story - Chiari the Killer

I was searching for some meta tags and came across a person's blog about a woman who died from complications related to Chiari. Please read when you have time. 

Excerpt from 'Shannon's Story'
Chiari is a highly undiagnosed condition and is widely unknown and misunderstood.  The longer you delay a Chiari diagnosis, the greater chance of additional neurological damage.  Shannon’s Chiari diagnosis was about 5 years after the onset of symptoms, which initially included severe headaches and anxiety, later followed by depression. After she began to lose the use of her hand and leg, Shannon was finally diagnosed and underwent Chiari decompression surgery. Unfortunately, it did not succeed for her although it apparently does relieve symptoms in about 80% of attempted procedures. During the remainder of her life, Shannon lived in excruciating pain with extreme insomnia plus about 6 other Chiari symptoms. Unfortunately, Shannon was unable to survive with the condition and left us earlier this year (2011). Chiari is not a rare disease, just rarely diagnosed on a timely basis!!!  Chiari affects 1 in 1000 people, and 3 of 4 of those are women.

One More Day

Kyle is definitely nervous about his surgeries. He keeps telling me he doesn't want to go through with it. He knows he only has one more day before his first surgery. As much as I don't want him to have surgery I know we have no option.

Kyle was weak today. We had to go to two parties today and by the end of the day he was completely dragging his right leg and couldn't move his right arm at all. He can't do little things we take for granted like opening a car door or buttoning a shirt anymore. I still pray that he will get his strength and feeling back after he recovers from his surgeries.

Let's hope.

Getting Weaker

Kyle fell yesterday and couldn't get back up on his own. I can tell it upset him badly. Later yesterday he couldn't even pick up his android tablet. Today, I took him to get his pictures taken at a portrait studio. He was weak and tired but did the session anyway. As I was reviewing the pics I looked over at him sitting in a wheel chair and he literally looked like he was going to pass out. He said his stomach hurt. Instant fear came over me as if I'm going to lose him. He has two more weeks before his surgery. I pray we can wait that long.

Good Days / Bad Days

I'm Kyle's mom. I've noticed Kyle has good days and bad days, but regardless, he is getting weaker by the day. Kyle is experiencing neuromuscular deficits because the flow of his spinal fluid is getting blocked. Some of his issues are weakness of arms, hands, legs and feet. He is having more difficulty with balance and coordination. He also has major swallowing difficulties. Watching him go through these issues is heart-breaking. I'm trying to prevent him from exhausting himself because I don't know if he will decline more or not if he does too much. I also don't know if he will get strong again after his surgery. I pray he does.

Symptoms of Chiari

Here's a list of MY Chiari symptoms:

• Headaches
• Neck pain
• Loss of balance
• Loss of feeling in right arm and hand
• Difficulty swallowing
• Vision problems
• Fatigue
• Vomiting
• Choke easily on food and water
• Dizziness
• Weak
• Hoarse voice (Vocal cord paralysis)

note: my mom helped me with this post

What is wrong?

At first I didn't know what was wrong with me and I didn't know how to explain to my parents what or how I was feeling. Which was: I was tired a lot; I wanted to lay down all the time; I had headaches; I had dizzy spells. Then I started not using my right arm and hand. I got weaker in Karate. Then I started falling/dropping for no reason. And because I couldn't explain it and my parents didn't know what was wrong they did a lot of guessing.

They would ask me: "Why are you being lazy?" "You are not eating right, you need more vitamins and fish oil." "You need more sleep." "Try harder." "Use your right arm."

Of course, nothing changed.

I also have Klippel Feil, I was born with a single kidney. So my parents insisted something was wrong with my kidney. But nothing showed up too abnormal. So my parents started worrying that maybe my symptoms meant I had MS or Parkinson disease. They kept taking me to my doctor to find out what is going on.

Finally, nearly a year after my parents started getting concerned with thinking something was seriously wrong with me, my doctor ordered a MRI scan. The scan took 2 long, uncomfortable hours. The next day, my doctor called my parents and told them that a neurologist will be contacting them and that I should not be doing any physical activities.

We met a neurosurgeon about three weeks ago. And that is when they told my parents I have Chiari malformation.

Everything is going to change.

Background

Hello. I'm Kyle. I started experiencing changes about 2 years ago. Not knowing what was wrong with me until recently. A MRI scan showed I have Chiari Malformation. This blog serves as a journal of my experiences with Chiari. I plan on updating this blog before and after my surgeries. My parents will help me blog when I cannot.