Noah’s Journey

Born July 27th, 2008

5lbs 13oz

Noah’s Journey

Born July 27th, 2008. 36wks premature Noah was

amazingly healthy! We were thrilled!

We soon realized Noah’s health was not what we thought. At 6wks he started projectile vomiting & after

3wks of testing he was placed on a predigested formula. This helped the vomiting but he would still randomly

vomit with no known illness.

He developed a kidney infection at 4 months old. He was then tested for kidney reflux & also had an

ultrasound to make sure his kidneys were properly developed. All tests were normal. Noah was placed

on high dose antibiotics.

Other than your occasional cold & ear infections Noah seemed to be doing much better. He always

had a smile on his face!

Before we knew it he was turning one! Wow how fast

time passes!

We took Noah on his 1st trip to the ocean! He had lots of fun but developed a nasty cyst on his gum. By the end of the week we were ready to get him

home to the dentist!

Once we got home things changed. Noah began spacing out & moving his

hands in front of his face. The first time I saw him do this I was overwhelmed

with this horrible feeling that there was something seriously wrong with my little bug. I videoed his new behavior & took

him to the pediatrician. She thought this might be seizures & wanted to do an

EEG & MRI. She scheduled the tests & an appointment with a pediatric

neurologist. That day in the office was the day that Noah’s journey began.

Noah had a 30 minute sleep deprived EEG. His arms were wrapped tight in a blanket to help restrain him. He screamed & cried until he finally feel asleep. We were terrified! We had never seen Noah so scared.

Little did we know this was just the beginning.

He then had a MRI of the brain. He was sedated for the MRI.

The EEG & MRI were read as normal. We were thrilled but still very concerned because we didn’t know what was going

on with Noah. His episodes were occurring everyday now. We waited for our appointment praying for answers.

The day arrived & we finally got to speak to a neurologist. He said he felt that

these were possibly stereotypy's (controlled behavior that children

develop to sooth themselves) & that he wanted to do a 6 hour videoed EEG to

rule out seizures.

Noah underwent another EEG. This

one was 6 hours long and was videoed.

They didn’t restrain children at this

hospital so I had to hold him down for an hour while they glued

electrodes to his head. It was horrible! Once he was hooked

up he slept for a couple hours and then

we played for the remaining 4. The

EEG was read & again it was read as normal.

With all of Noah’s tests coming back normal Noah was diagnosed with

stereotypy’s & sent on his way. I knew with all my heart this was not right. Noah started developing ear infections in both

ears. He had 3 in a row & was referred to an ENT who scheduled Noah for surgery.

During this time his episodes became much worse. They were lasting up to 2

hours a day & were become very intense. I decided at this point we needed to get a

second opinion. Little did I know how many opinions we would get!

Noah's 1st surgery of 2010. He had tubes placed in his ears to help eliminate his ear infections. He did

extremely well. The surgery was a success and Noah's infections stopped completely.

Now the ear infections were gone so we could address

the main issue at hand, Noah's

episodes. We had an appointment with a

new neurologist. Noah's episodes were still very intense & we were noticing a new symptom. He began

spiking random temps with no illness. Every afternoon he would

get rosy read checks.

After meeting with the neurologist we felt again we should get another

opinion. She had put Noah on seizure meds but felt it was a stereotypy & the meds wouldn’t help. We were shocked

when the meds did work. Noah's episodes decreased to 15 minutes a day. We decided then we needed to

find someone who specializes in movement disorders.

Our new neurologist felt we needed to

repeat the EEG & do a CT Scan to rule out

Neuroblastoma (cancer). We were so

scared! Noah was admitted into the EMU unit were he spent 3 days of monitoring.

This time we decided to sedate Noah before hooking him up to the

electrodes. We felt this would reduce the fear he experienced

prior.

He was such a trooper!

His head was so itchy from the glue!

The Playroom made everything better!

Today was the day for his CT Scan. He had to get an IV to be sedated. Took forever to get the

IV. Once they got it he was sedated & had a reaction to the sedation. Was out of it for almost

10 hours! Very scary!

All test results were normal! We were so happy! While waiting on the

test results we had contacted John Hopkins & gotten a diagnosis of

PNKD (which is a rare movement disorder that occurs when the body is at rest). The neurologist on call in the EMU unit decided that we

needed to take Noah off his seizure meds & wait until our appointment

at John Hopkins. We agreed & thought 3 months would come before we knew it. We were so

wrong.

Over the next 3 months Noah got much worse. He started projectile vomiting. He would do this once a week on & off all day. He lost 3

pounds over a month and half period. He was scheduled for a

barium swallow. This is an x-ray of the esophagus & stomach to

rule out blockages & narrowing. Tests were negative. We were

then referred to a GI doctor who ordered an endoscopy of the

esophagus and stomach.

The day came for his endoscopy & he was sedated again for the procedure. All test results were normal. The GI doctor had no

answer for Noah’s vomiting. We were so frustrated! With no answer came no treatment just watch our son continue to get worse.

And he did get worse! Much worse! He developed decreased reflexes in his arms, he began have

nystagmus (eyes darting left to right) during his episodes, he began falling all the time, & developed a

tremor while using his fine motor skills. He would scream out in pain & at this point his episodes were up to 7 hours a day. He had good days & bad but at this

point they were all becoming bad. He started shouting out random words during his episodes & talking to people who were not there. It looked like he was

developing tics on top of the movement disorder. He had a hard time sitting down & eating because every

time his body came to rest for a period of time his episodes would start. He would wake up at 3 am & have episodes on & off for hours. We were watching our son slip away & there was nothing we could do other than watch and wait for our appointment at John Hopkins.

Noah woke up one day from his nap & after taking a couple steps he clasped to the floor screaming in pain. I took him to

the pediatrician who ordered an x-ray of his hip & knee. Again the tests showed nothing! What was happening to my son? Everyone looked at me as if I was crazy & no one could

give me answers!

The day finally arrived for our appointment at John Hopkins. We had our hopes so high that they could make our son better. We were so wrong! They said his new symptoms made them feel that he did not have PNKD but possible seizures. We couldn’t understand? He

had 3 EEGs with no seizures? They sent us home with no answers an prescription for strong seizure meds. If he got worse bring him back!

We were at a wall with no were to turn!

We came home & started the meds. We were thrilled! They worked! He

was getting better! The episodes were down to 30mins-an hour a day. Each day got better &

better. We thought “ok we don’t know

what this is but with meds he’s

better so this we will take.”

We meet with a geneticist the

following month. I was determined to

convince her to repeat the MRI. Call it what you want but

my heart told me they were missing something! She

agreed & also did some genetic testing.

The MRI was scheduled at a

different hospital & Noah was again sedated for the 1

hour MRI.

The geneticist called me with the MRI results. This day I can say I will never ever forget! My heart sank! Finally we found something but what was it and how do you fix it? The first MRI was misread and Noah had a Chiari Malformation type 1. She recommended seeing a neurologist right away.

• A Chiari Malformation type 1 occurs when the skull is to small for the brain.

Therefore pushing the brain down into the spinal cord. Noah's herniation was 18mms

extending down to his 2nd vertebrae. I knew after doing my own research this was

serious & had to be what was affecting him. We consulted with 2 neurologist who said it was incidental & no big deal. We

then scheduled appointments with neurosurgeons.

We meet with the first neurosurgeon. He said he felt very uncomfortable doing the surgery. He would have to remove the

1st vertebrae & part of the second & this could lead to instability of the spine & head. He wanted Noah to have

another MRI of the cervical spine & a CSF Flow study to see if he had sufficient flow to the brain. He said with a herniation

this large he felt certain the CSF flow study would be abnormal.

Noah waiting patiently!

Noah was sedated yet again for

another MRI to check his cervical spine for a syrinx’s (cyst in the spinal cord) & the CSF Flow Study. The results showed no

syrinx but very abnormal CSF flow with no CSF flow

in parts of the brain.

We met with Dr. Herbert Fuchs at Duke Hospital. He is the Chief of Pediatric Neurosurgery. He went over the MRI results &

recommended surgery. Noah was scheduled for a Chiari decompression surgery the following Monday. We were so

scared! Our baby is going to have brain surgery! Is this really happening? He told us of the dangers of waiting to do the

surgery. A minor head injury could be life threatening & with the 3rd vertebrae controlling your respiratory system sleep apnea

& death are possible side effects of a Chiari. Those were risks we were not willing to take.

On September 22, 2010 our lives were forever changed. I did something I never thought I could do. I laid my son on the operating table & watched as they sedated him. I was then taken out of the operating room. Thank God for the nurse who held me tightly in her arms & walked me very quickly down the hall. For the first time it hit me what was really happening & there was nothing I could do to stop it! This

fear no one should every have to feel.

We sat in the waiting room with our family by our side. Time stood still. Finally they called & said we are

starting the surgery. I didn’t want to know when it started just when it was over. 2 ½ hours later they

called & the neurosurgeon said everything went well. He said “Noah should have a much happier brain.” Ah

this was music to my ears! We finally get to see our little bug!

We went to the PICU & after seeing Noah we quickly realized he was having issues. He had an allergic reaction to the anesthesia & had developed strider

causing him to stop breathing. We were terrified! He was repositioned all night to help him breath.

The second day we thought would be better but Noah had developed to much acid in his blood & again he was struggling to breath. On

top of the breathing difficulties Noah was alert & in lots of pain. He started vomiting mid day. His blood pressure spiked to 188/120 & he began running a low grade fever. I actually thought to myself “what

did we let them do to our son!”

By the third day he was feeling some better & we were ready to take him home. He did his physical therapy but

was still having problems walking. He had difficulty walking

for almost 2 weeks after surgery.

Noah was released to go home! We were

so happy but dreading the 3 hour

trip home. They gave him a big dose of pain meds & he

did great. He was so happy to be home & really hungry. This

was Noah’s new smile. It really hurt

to turn his head.

After being home 2 days I noticed Noah wouldn’t eat & he started vomiting again. I called his neurosurgeon & he said to take him to his

pediatrician. Noah had developed chemical meningitis & was put on high dose steroids for the

next week.

6 days post op

After a couple of weeks Noah started getting sick again. He didn’t eat all day & that night he started vomiting. We called the neurosurgeon & he told us to take him to the ER. He was running a low grade temp & had vomited for the an hour & a half. They did blood work & his blood count was elevated. They then decided to transport him to Duke by the critical care life flight ambulance. They arrived at 4am. Only one person could ride up front in the ambulance so Nathan

followed us down.

After arriving at the hospital Noah had a spinal tap & a CT Scan. The spinal tap was done to rule out bacterial meningitis &

hydrocephalus. The CT scan was done to rule out pockets of CSF. Because of Noah's reaction to anesthesia after surgery he was required to have the tests without sedation. He was given a

medication to help him forget. It took me, Nathan, & 2 nurses to hold him down while they did the spinal tap. Thank God for Nathans strength because I didn’t know if we could do this & for the meds to

help Noah forget that horrible day!

All of Noah’s tests were

negative. He had developed chemical

meningitis for the second time! We were sent

home with another round of

steroids & prayed for him to recover this

time!

The steroids worked & within a few days he was feeling much better. He was finally recovering from his surgery!

9 days post op

He was feeling so much better! Within a month he was starting to get back to

himself!4 wks post

op

Noah was scheduled for a repeat MRI 8 weeks after his surgery. He was again sedated but this

time had his cousin with him which made him feel much better!

The before & after MRIs proves how much better Noah is doing! Its amazing!

Before After

Each day Noah is getting better!

And Better!

Noah is 4 months post op & we are amazed at how well he is doing. All of

the symptoms before surgery have improved dramatically. He is now

enjoying each day of his life! His bad days are few & far between. Each day

we see an improvement in Noah. We are praying that he will one day be free of all of his symptoms. For now we will thank God for the improvements that we see

with Noah.

Noah’s Journey has taught me to never give up! To always follow my heart!

Noah, the day that I held you in my arms for the first time I

never dreamed that you would have to go

through so much. You are the most amazing little boy I have ever met. Your courage,

bravery, and strength amazes me. My

promise to you: I will never stop holding your hand. I will

always be here for you & I will never give up

on my fight to help you become 100 percent better! I love you my

little bug!