Hopeful

Nanan left today and I'm so sad.  I had great snuggle time with her, especially at night when mom and dad were putting Aidan to bed and trying to get dinner cooked.

Aunt Destin manages to keep me dressed in the the most fashionable outfits even if Daddy and Uncle Widg can't seem to figure out how to put them on.

Thanks to Mom and Marmee's friend Gail my family is following leads from Canada to the Philippines about a little boy who had a similar diagnosis as me and is now doing well, seizure and medication free.  We are trying to reach out to their doctors in Canada to see what direction they might lead us in.  We are sticking to our motto, hoping for the best but preparing for the worst.  Just in the few days that my Phenobarbital dose has been reduced I have been more alert for longer periods.  I still tire very easily and become very overwhelmed/over stimulated when too many things are going on.  It's nice to get out of the house but it does take it's tole on me.  I took Nanan for a birthday brunch with Mom, Dad, Aidan, Uncle Widg, and Aunt Destin.  I pooped ALL over Mom - just to keep things interesting.  I became really over heated and had to be rushed to Uncle Widg's house to cool down.  Mom and Dad worry if I get stressed.   It will be easier when it is not so hot since I enjoy sitting out on the patio in the evening with my family.

A lot of firsts.....

What a good day ....... I had many firsts today and yesterday and it has been great.  I started off at the Chiropractor and Katlin's dog Kyra greeted me at the door with my first big kiss of the day. 

 
I was able to go on my first shopping trip with Mom, Nanan, and Aidan for school clothes for Aidan.  Now that I'm off the ACTH I can begin to go out in public.  It's nice to get out of the house and not be at the doctor's office.  We went to lunch and I had my first bottle in public. (Aidan drew a pic of a giraffe for me.)

Aunt Destin and Uncle Widg took me to my first birthday party.  Mom talked to the doctor on Friday and I will begin coming off my Phenobarbital.  It will take 6 weeks though but that will be one less medication I will have in my cocktail.  Then we will meet with the doctors in August to discuss what our next steps are as long as my seizures do not return (then that changes everything). 

Tomorrow we are going to Brunch for Nanan's Birthday.

Slow start....great end.....

I had a really difficult night.  My tummy was giving me a lot of trouble and kept Aunt Destin up most of the evening.  Nanan came in today from Louisiana and I was so excited to see her that I decided to stop eating and not sleep.  I skipped several feedings and Mom started to become worried.  So I decided to turn it around and had the best PT session I have had so far.  I held my head up like a champ and Ms. Beth (my physical therapist) said I looked awesome today....she was so proud. 

I had the best ending to my evening when Marmee and Aidan flew in from Montana.  Marmee leaves tomorrow....so I won't see her for a while.

A Normal Day

Mom and I had a talk this morning and decided we would start to venture out and have "normal days".  We started off at the chiropractor, which went well.  I spent most of the morning working hard on my OT/PT exercises.  I often miss these because I'm so tired from my medications.  Mom and I decided we needed to go to Target if we were going to eat today.  It was one of our first outings to run a normal everyday errand and we had a great time.

After getting home from the store decided to surprise mom by reaching out, grabbing, and hanging on to one of my toys that is in my car seat.  You might not realize it but this was a HUGE event. We were both so excited that mom started calling everyone to share the good news. 

Mom and Dad are going out to dinner to celebrate and I going to stay home and snuggle with Aunt Destin and Uncle Widg.  Tomorrow Nanan(Jamie), Marmee, and Aidan get into town.  I can't wait.

Many visitors..........

Now that I'm finally back on track I am ready for visitors.  Mom's friend Liz came to visit after I had a bath and then I got to spend the afternoon with Grandma while mom was out.

I had OT today and Ms. Julie said my head control has improved a great deal.  She gave mom a lot of exercises for me to work on this week.  She is concerned about my eye tracking though. 

Back on track again.....

I decided late last night that I would listen to mom and dad and give the new bottles a try.  I'm now eating like I should be and mom is no longer worried.  My last 20 hours have been really good.  I'm making good eye contact, eating well, breathing well, and my tummy is not acting up as much.  I'm sleeping a bit more than my parents would like but I'm working hard at doing well and it takes most of my energy. 

I saw Dr. Caitlin today and she feels my head adjustments are improving.  She continues to do craniosacral massage (pic).  I'll be seeing her 3 times a week (just to add into my already busy schedule).

I had a visitor today and will be able to start seeing people soon (just waiting for the ACTH to get out of my system and my immune system to get back o track). 

Hot Weekend

I stayed inside all weekend since it was really hot.  I continue to have tummy issues and decided to cut back on my eating since it makes my tummy hurt so bad.  Mom has been keeping a log of how much/little I eat because she is getting worried.  I have eaten a little more over my last 2 feedings and now my tummy is hurting again.  It seems I just have to find the right balance of eating and tolerating tummy pain.  There are feeding specialists at Children's Hospital that mom can take me to if needed, but we are trying to not go there if possible.  I seem to be there every week and mom and I are needing a break. 

I'm getting excited......Aidan will be back home soon and I will have someone to dance for me and make me smile.  I miss him. 

                Hanging out in my cradle.

Splints.....

Today mom took me back to Children's Hospital for an OT eval to see if I need splints for my thumbs....I like to hold them in tight and that is not a good thing.  So now I have pretty lavender and purple splints for my hands that I have to wear in the evenings when my tone is poor.  My tummy continues to give me issues but seems to be getting slightly better.  I'm not a big fan of my new bottles but Mom and Dad keep telling me they should make me feel better if I just give them a chance. 

Doctor News.....

I had a bit of a difficult night and a not so great start to my day this morning.  My tummy difficulties are getting worse.  Mom started me on my new bottles and we are hoping this will help.  I am on the max dose of tummy meds and still don't see improvement.  

Dr. L called today after consulting with the doc in Chicago.  Bottom line is the docs do not want to pursue further meds to treat my current state.  My diagnosis is some sort of Encephalopathy since my seizures are currently under control.  As far as what type of Encepalopathy I have no one knows so it makes it difficult to find meds to use.  So the waiting game begins......waiting to see if the ACTH stopped the seizures for good or just temporary.  If the seizures are only stopped on a temporary basis then the docs will work with Mom and Dad to see what other med options there are at that time. So for now I continue with my busy weeks, consisting of phsysical therapy, occupational therapy, chiropractic, and vision therapy.

Hanging out with Daddy

Sight Visit....

I'm still having tummy issues, but the nurse at Children's told Mom that I don't seem to have any blockages on my X-ray so I am to continue to follow up with OT about any eating concerns.  Mommy and Daddy took me to the Anchor Center this morning for my eye eval.  I qualify for services as I have a disconnect between my eyes and brain and don't really process the way others do.  I become easily overwhelmed and can only track and process information for moments at at time.  I will be able to go twice a week and learn different techniques to encourage me (and my brain) to utilize my eyes more. 

Mom, Dad and I are missing Aidan and can't wait for him to return from his vacation with Marmee and Papoo in Montana. 

I did get some snuggle time with Grandma and Aunt Destin today while Mommy was out, and got to spend some one on one time with Daddy as well.

Sleepytime. . . .

Another hot day in Denver and another difficult day for me.  I did have my first real jogging outing this morning with Aunt Destin and slept the entire time (yeah)!!!!  Afterwards mom got to cart me off to yet another doctors appt.  I met with the GI specialist who sent me down for xrays to rule out any blockages.  We are still waiting to hear back from them.  Then I got to spend sometime taking a nap with Grandma since the doc poked me so much I didn't sleep all morning.  Ms. Julie came after that for OT and she is wanting me to try a new bottle and new sleep position to see if that helps with my tummy issues.  After she left my sleeping was so fretful (from being over tired) I could only sleep if Mommy held me.  So here I am after several hours......

......trying to catch up on my sleep since I have my big eye appt in the morning and need to have the energy to participate.  Now off to sleep on Uncle Widg....

Another chapter....

Today was my last ACTH injection.  So we are hoping and praying that my seizures don't return.  Mom and Dad are happy and sad the medication has stopped.  The doctors have told us that they don't know why this medication worked.

Tomorrow I go to the hospital for a GI consult since my tummy is giving me a really difficult time.  I'm having a hard time staying on schedule with sleeping and eating.  Every aspect of my day seems to be dictated by my tummy.  Hope the docs can help me tomorrow.  On Wednesday I go to the Anchor Center (school for the blind) for a functional assessment to see if I qualify for services.  My parents and docs are concerned about my vision and are worried there might be a disconnect between my eyes and brain that impacts my ability to see correctly.  I have OT tomorrow as well and am ready to show Ms. Julie how well I'm doing on holding up my head.

Daddy has a client dinner tonight so I get to spend the evening with Uncle Widg and Aunt Destin.

Up and down weekend

Saturday I took it easy, sleeping most of the morning and early afternoon.  After waking up my mom and dad realized I must have allergies as my eyes had been red and swollen all day.  It was not one of my better days.  I did sleep all night but have mom and day worrying again as I have not been eating very well.

Today is a better day.  I woke up smiling and even let mom get a picture (not one of my best).  We went to the Farmer's Market.  It was one of my first outings with my parents that was not doctor related.  So far I'm eating better today and seem to be getting myself back on track. 

Lazy, Lazy Day....

I decided to sleep most of the day.  I did do some of my OT/PT exercises though.

Heading Home

I stayed over night with Aunt Destin at the hospital and had a difficult evening.  Guess I don't really like being there.  Mom came at sunrise so Aunt Destin could get some sleep before going to work.  There were many meetings with many doctors.  The reviewing doctor said my Hypsarrhythmia is gone thanks to the medication.  There were lots of crazy big words thrown around and at the end mom told me they just don't really know what is wrong with me or why the medication did what it did.  Mom and Dad spent some time on the phone with the doctor in Chicago who also admitted he is unclear what I really have and is not sure what the best course of treatment should be.  After all the meetings and the ride home I was wiped out and slept through all the tornado sirens during the big storm.  Beth my PT was late due to the weather so Dad and Aunt Destin stayed for my PT and mom went to spend time with a friend.

Tomorrow I am scheduled to go to the Chiropractor and spend some time with Grandma so mom can run errands.  I am super tired so mom said will give me my bath another day.  Once again I have blue marker all over my head and mom calls me her little Smurf.

Not the best day.....

Had a difficult night last night.  I can't shake these really bad tummy issues that are causing me a lot of discomfort and keeping me up most of the night.  My tummy issues followed me into the morning and I did not get much sleep today.  Mom took me to the Hospital this afternoon for my 24 hour EEG.  I got to see Catherine the EEG tech who has run several EEG's on me and is the only tech that does not put permanent blue marker on my head.  So I should look like my normal beautiful self when I leave tomorrow.  I finally got settled in and my tummy started acting up again.  I became so upset that I pulled some of my probes off. 

                                                         (check out my cool hat)

Mom and Dad left to get a good night sleep and I am snuggling with Aunt Destin.  I'm going to try really hard to have a good evening.  Mom will be hear bright and early in the morning to meet with the doctors.

A good day

Today was a good day.  I got to take my first long car ride with Mom.  We took Aidan and Marmee to the airport and on the way home mom only had to pull over once to fix my head in the car seat.  After that I had my first social outing.  I got to go to lunch with Mom and one of her friends.  We made a special effort to sit outside on the patio and away from ever body as I am not suppose to be near anyone who is not feeling well.  Ms. Julie came over today for OT and told my family that my head control is improving and she is so proud of me.  She suggested I go and get and eye evaluation at the Anchor Center to see why my tracking is poor and why my eyes cross so much. 

We went home and I got to spend the evening with Uncle Widg and Aunt Destin who gave me an infant massage.  It was wonderful.  Uncle Widg cooked a large Gumbo to give to some of our neighbors who have gone out of their way to help our famly.

I'm going to try and get a good nights sleep in preparation for my 24 hour EEG tomorrow. 

Just another manic Monday...

Today started off with my Chiropractor appt.  She is working hard trying to improve my acid reflux and tummy issues.  I got moved around the house most of the day while Mom and Marmee tried to entertain Aidan and get him packed for his 12 days in Montana.  Mom let Aidan feed me my 'snack' bottle.  He was so proud of himself....so mom took pictures and video.  Then Grandma came and stayed with me for awhile, while everyone was out running errands.   

Mom called my doctor this morning and expressed her concerns about some spastic movements I began showing over the weekend.  My family is concerned this could be related to the decrease in the ACTH (the med that stopped my seizures).  They had a long talk and decided to run a 24 hour EEG to see if I am having any silent seizures or infantile spasm like activity.  So on Wednesday I'm off to Children's Hospital and Mom, Dad, and Aunt Destin will take turns staying with me.  Mom and Dad will then talk to the Doctor in Chicago on Thursday to see what his thoughts are. 

Lazy Sunday

Today started off slow and quiet.  My Mom and Marmee went to the Farmer's market and I stayed in bed with Dad and Aidan listening to stories about superheros.  Afterward our friend Jay came over and took pictures of our family with Aunt Destin and Uncle Widg.  That really wiped me out.  We had some friends from Louisiana stop by to give us some love and well wishes.

Mom and Dad will be calling the doctor in Chicago on Thursday to see what his thoughts are. 

Marmee read a poem to Aidan and I that was written by one of her students that she taught many years ago.

Nezbah’s Bro

 

What a great brother Aidan

Lights up Nezbah like the sun

Now there’s a sister

But with you mister

Your parents hit a home run.

 

Your sister needs extra love

From you and the Lord above

Harper will make it

With love and true grit

But it’s you she needs most of.

 

Your sister needs extra time

A mountain she has to climb

Help your mom and dad

Squeeze them when they’re sad

A big hug is not a crime.

 

Your sister needs extra prayer

Tell the Lord how much you care

One thing is for sure

They will find a cure

Then you’ll be quite the pair.

 

One day she will help you back

Share a cookie or a snack

Cheer you in football

Paint your bedroom wall

Maybe carry your book sack.

 

One last thing for you to know

No matter how high the snow

With these trying times

And this attempt at rhymes

Sis will always love her bro.

 

-Bill (Medford) McGill

Aidan's Birthday

Today we celebrated my brother Aidan's Birthday.  He turned 5 today and spent the day at the pool with Mom, Dad and his friends.  I spent the morning with Marmee waiting for everyone to come home to have cake.  Today was a good day....no doctor appts.  I got to spend some quiet time on the porch with Aidan and we had our picture taken.  He showed me all his presents and gave me lots of kisses.

Tomorrow I get to watch Mom and Aidan pack to go to Montana.  He is leaving for 2 weeks.  Now who is going to kiss me on the top of my head every night and say, "Love you Nezbah!!"?

My Mom and Dad decided to call the Doctor in Chicago again to talk to him about other treatment options as my doctors in Denver feel I am at my best now and see no need to pursue further options at this time.

PT/OT

I had OT yesterday and Ms Julie to my mom I am doing an excellent job at trying on holding my head up.  She feels I am only 6 weeks behind other babies my age (2 months).  I do my exercises daily and work hard with my mom, dad and Aunt Destin.  I have PT tomorrow and see my Pediatrician.  My plan is to impress them as well.  My mom has me seeing a Chiropractor a couple times a week and she is working hard as well.

Getting back on track

I had a great time visiting with my Uncle's, Aunt's and cousins who were visiting from the Maryland.  I surprised everyone and have been showing big smiles everyday.  In the process of coming off of the ACTH (the med that stopped my clinical seizures) due to side effects.  We are hoping and praying that my seizures don't come back and my little brain has a chance to catch up as I'm behind on some of my developmental milestones.  My mom and Aunt Destin are trying to get me to drop one on my night bottles so my mom can get some sleep on nights that Aunt Destin doesn't take care of me.  I have a long week with many doctor's appts and hoping all news is positive.

4th of July weekend

I am preparing to spend the 4th of July weekend with family who are visiting from out of town.  I am really excited to meet my Aunt Anne, Uncle Jeff, Grace and Adam for the first time.  Due to side effects from the ACTH I am coming off the injection and am so happy to be down to just one shot a day.  I am having fun keeping my family on their toes as my schedule changes daily because of the changes in my medications.  Gave my family a great surprise.....I started smiling.  Looking forward to my first BBQ on the 4th.

My story

My name is Harper Johnson and I was born on May 2nd, 2011 weighing 7 lbs 3 oz.  My parents began to worry about me on day 2 as I began to have seizures.  After two EEG's, an MRI, and a Spinal Tap and 5 days in the NICU at Rose Hospital I was moved to Children's Hospital in Denver via Flight for Life.  I spent 3 weeks at Children's, had an abundance of tests and continued to baffle my doctors.  I was initially diagnosed with Ohtahara Syndrome after numerous EEG's.

Before I was born my brother Aidan would call me "Nezbah" as my parents hadn't decided on a name yet.  Unbeknownst to them, Nezbah is a Navajo word meaning 'woman in battle'.  This is my story.


My parents and I continue to research, seek other opinions, and explore all options as far as treatment goes for me.  The doctor's can't seem to decide what diagnosis I have.....whether it is Ohtahara syndrome (OS) or something similar.

"Ohtahara syndrome is a neurological disorder characterized by seizures.  The disorder affects newborns, usually within the first three months of life (most often within the first 10 days) in the form of epileptic seizures.  Infants have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures.  Ohtahara syndrome is most commonly caused by metabolic disorders or structural damage in the brain, although the cause or causes for many cases can’t be determined.  Most infants with the disorder show significant underdevelopment of part or all of the cerebral hemispheres.  The EEGs of infants with Ohtahara syndrome reveal a characteristic pattern of high voltage spike wave discharge followed by little activity.  This pattern is known as "burst suppression." Doctors have not yet seen any inherited cases of Ohtahara syndrome, but have observed that boys are more often affected than girls.  The course of Ohtahara syndrome is severely progressive.  Seizures become more frequent, accompanied by physical and mental retardation.   Some children will die in infancy; others will survive but be profoundly handicapped.  As they grow, some children will progress into other epileptic disorders such as West syndrome and Lennox-Gestaut syndrome."

The doctor's did tell my parents that they were surprised that my seizures stopped at 6 weeks old, in connection to ACTH injections and are not sure where to go from here.  So here goes my story, my daily routine, ups and downs as we try to figure out what my future holds.  My goal is to prove the doctors wrong, to be able to walk, talk, and live a productive life which they are thinking is not going to happen. 

I am Nezbah and this is my battle.