2010 Year End Review

Fern (my cousin), Bertrand, me, Matthew, Belinda and Aury (my sisters).

We are currently busy in Florida with several family events (such as my aunt's wedding, pictured above), but I just had to check-in one more time for 2010. :)

Closing out the year, I feel like our family is staggering across a metaphorical finish line. Any year that starts with your child having brand new seizure types on the very first day is bound to put you through the wringer.

Borrowing the idea from my friend Fawn, here is a representative post for each month of 2010:

January

Tonic seizures

February

How did Bertrand break his arm?

March

Developmental Evaluation: No Progress

April

ACTH, here we come!

May

State of the Pookie Report: May 2010

June

Hospitalization Update

July

The new Bertrand, and our biggest fear

August

Post-Infusion Report

September

Pneumonia Scare 2.0

October

Falling Down

November

A Big Change

December

Holiday Update

Re-reading all the 2010 posts this morning was quite the effort. And honestly, I was too disheartended, exhausted and sick for most of the year to write regularly. Much of the really hard stuff was left out. Hopefully, 2011 will bring positive changes to our family as well as many of our friends.

We're wishing you all much love, happiness and peace in the new year!

Holiday Update

Bertrand is cuddled by his 1st Lt. Uncle John during a post-lunch nap.

Our family is spending the first half of this holiday season in Park City, Utah at Bertrand's grandparents' new house. Bertrand is enjoying every moment! He is clearly a winter sports guy if those sports include: being pulled in a sled, hiking via kelty backpack, and sitting by the fireplace. Oh wait, that last one is *my* winter sport! ;)

It's been a relief having extra pairs of hands and eyes around to care for Bertrand. The introduction of a new seizure medication, Lamictal, had me particularly worried due to potentially fatal side-effects such as Stevens-Johnson syndrome and liver failure.

However, so far Bertrand is doing great! In less than a week on Lamictal, all of his seizure-types have seen significant reductions. For example, his atonic seizures were down to 2 yesterday from 30+ daily a week ago. (Today, he hasn't had any! Knock on wood.)

Even more telling than the number of clinical seizures (since so many of his are sub-clinical) is the eye-contact, the number of smiles and daily laughter! We couldn't have received a more beautiful and perfect gift than hearing our son's joy manifest itself!

Lamictal is a drug with a long half-life, so we are no where close to seeing its full effect. Bertrand is on day 6 of taking a 25mg orally disintegrating tablet (ODT). His final dose of 100mg will be reached mid-January, and full effects won't be seen until early February.

Potentially complicating this titration are several factors: upcoming travel, hospitalization, potential sickness, changes in school/therapy schedules, and a wean from zonegran. Timing the wean from zonegran--should we start in February? or start next week?--consumes my brain.

Bertrand stands to gain as much as 6 hours of consciousness back each day since that drug has made him so sleepy without contributing any additional seizure control. But, in November, reducing the amount by 25mg caused a temporary increase in seizures while his body adjusted.

The scientist in me has no doubt that we should hold off on the wean until we can hold other factors as stable as possible. The mother in me simply wants her baby to stop suffering from the drug's awful side-effects as soon and as safely as possible. Can there be compromise?

On the non-pharmaceutical front, Bertrand will be alternating occupational and physical therapy at Shriners Hospital every Tuesday, starting the 2nd week of January when we return from Cleveland Clinic. And speaking of Cleveland, these are the updates to B's team there.

• Sumit Parikh, M.D. - neurometabolic & neurogenetic specialist
• Debabrata Ghosh, M.D., D.M. - movement disorder specialist
• Deepak Lachhwani, M.D. - epileptologist

Wordless Wednesday

What will 2011 be like?

The ornament Bertrand made for us at school! Yay for popsicle sticks!

Today was an administrative day. I began addressing the logistics of our end-of-year travel to Florida and Ohio--such as completing inventory & ordering of necessary medication, ketocal and other supplies. I finalized Lamictal titration and Zonegran wean schedules, as well as picked-up the long-awaited Lamictal. (Bertrand starts the $630/month drug tomorrow.)

This morning, Bertrand was also evaluated for occupational therapy services at Shriners Hospital. The session went really well! He'll be recommencing with physical, occupational and speech therapies there in January, since he aged-out of Early Intervention.

Our 2011 calendar, which once seemed so empty, is already packed. Bertrand will be attending preschool for fewer days next year--only Mondays and Wednesdays--and he won't be receiving EI services, but we've already filled the gaps. The therapies at Shriners were the last pieces of the puzzle.

Bertrand's "extracurriculars" will also include "Dance Class" (the dancers with disabilties class at the University of Utah's Virginia Tanner Dance Program) on Tuesdays and "Horseback Riding" (hippotherapy at the National Ability Center) on either Mondays or Wednesdays, and a possible reintroduction to the MOMS Club playgroup on whatever day is left.

That's one busy, little man!

Home for the holidays!

Bertrand and his Uncle John being all manly together.
Today, Bertrand's uncle John (Booj) arrived home from Afghanistan! The 1st Lieutenant is on leave from the war for two weeks and will be driving up to see us this weekend. We couldn't be more excited! Bertrand in particular is looking forward to his hero's visit. Uncle John said, "I hope your belly's ready, B, because I'm givin' you the motorboatin' of a life time." ;)

We are so proud of you, John, and all the other wonderful servicemen and women serving our country! THANK YOU!

Cleveland Clinic, here we come!

Picture from OneMedPlace

Bertrand will FINALLY be seen at the Cleveland Clinic! Cleveland Clinic's neurology program is currently ranked #1 in the US. (Coincidentally, it also performs the highest number of pediatric epilepsy surgeries in the US--not that Bertrand is a candidate.)

We'll arrive on January 2. Bertrand will be admitted on January 3rd. And, we should be home by January 7th. Among the specialists Bertrand will see are Drs. Ajay Gupta and Sumit Parikh. Dr. Gupta is an epileptologist and Dr. Parikh is a neurometabolic/neurogenetic specialist.

We don't expect a diagnosis or, realistically, even treatment. (At best we can address his seizures but not the underlying condition causing his white matter loss, liver and nerve damage.) We're just confirming that we've done everything we can for Bertrand and hoping to improve his quality of life.

THREE! And counting...

Bertrand celebrated his THIRD BIRTHDAY today!

He had an Elmo cupcake pull-apart for his birthday celebration at school.

The class enjoyed cupcakes and apple juice while the birthday King took an I-am-ignoring-you-all snooze.

Bertrand was happy to come home to find wrapped presents and a yummy lunch.

For dinner, Bertrand got a special birthday treat: carrot cake flavored, low carb, dairy blend. The not-so-special treat was having to listen to his dad, mama and aunt sing "happy birthday". (Bertrand's hearing may never be the same again.)

Bertrand really enjoyed his birthday "cake".

Bertrand received LOTS of presents (in spite of mom's pleas for "no gifts!") thereby continuing his transformation into "most spoiled boy on the planet".

Bertrand attempted to open a present for the first time! This was very emotional for all!

Bertrand was also FLOODED with birthday well-wishes and love from across the world: mail, email, twitter, phone, text message, facebook message, skype, visits, and more!

It has become clear to us that our son is LOVED by more people than we knew.

And that in his short life, he has touched more lives than many people do their entire lives.

He really is special!

Happy birthday, B! We love you!

Individualized Education Plan

Bertrand's Individualized Education Plan (IEP) was completed and signed only one day prior to his 3rd birthday! Since we've gotten a wide range of questions from "What is an IEP?" to "What is in Bertrand's IEP?" I'll give the Cristina-fied version of what an IEP is and what our experience was with Bertrand's.

The Individuals with Disabilities Education Act (IDEA 2004) made parents of kids with special needs members of their child's education team. Parents work with educators to develop a plan--the individualized education plan (IEP)--to help their kids succeed in the public school system. So, the IEP is simply a document that describes the goals the team sets for a child during the school year, as well as any special support needed to help achieve them.

IEP meetings must be held at least annually, but may be held more often if needed. Parents may request a review or revision of the IEP at any time. It is worth noting that federal regulations do not require states to provide an "ideal" educational program or a program the parents may feel is "best." The state must provide an appropriate educational program, one that simply meets the needs of the individual student.

Going into Bertrand's IEP, my anxiety was ratcheted up to HIGH because of this last point. Endless amounts of parental anguish can be found online due to a divergence of opinion between the school district and the parents on what is "appropriate" for their child. My anxiety was unfounded. Matthew and I came out of the meeting with a strong sense of support and understanding from the people who'll be working with Bertrand. Furthermore, we felt that the goals and support outlined in the IEP document (and Health Plan) were appropriate.

Summary of Bertrand's "Team Evaluation Summary Report":

Primary Disability Considered: Multiple Disabilities

Definition: Concomitant impairments (such as intellectual disability--blindness: intellectual disability--orthopedic impairment, etc.) that affect a student's education performance. The combination of which causes such severe educational needs that they cannot be accomodated in special education programs solely for one of the impairments. The term does not include deaf/blindness.

Assessment for Classification

Bertrand qualifies for the "multiple disabilities" classification under all following 6 criteria: intellectual ability, assistive/augmentative communication and motor systems, sensory/motor skills, vision & hearing, additional assessments by the team, medical history

Areas of Evaluation

Cognitive: Battelle Developmental Inventory 11/12/10 - Percentile 0.2

Adaptive: Battelle Developmental Inventory 11/12/10 - Percentile 0.1

Communication: Battelle Developmental Inventory 11/12/10 - Percentile 0.1

Hearing: Normal peripheral hearing

Vision: Cortical visual impairment

Motor: Battelle Developmental Inventory 11/12/10 - Percentile 0.1

Medical: Bertrand has a vast medical history. Health Care Plan to be developed.

Bertrand has a disability, as defined by the Individuals with Disabilities Education Act (IDEA), that adversely affects educational performance and requires special education and related services.

Summary of Bertrand's "Individualized Education Plan":

1. Student Information

2. Present Levels of Academic Achievement and Functional Performance

This is a LONG summary going into detail on Bertrand's Battelle Developmental Inventory scores, medical records, therapist notes, and parent statements. I am too lazy to type it out. Suffice to say that Bertrand has "complex health issues" and is severely globally delayed.

3. The IEP Team considered the following Special Factors:

• Bertrand has special communication service needs such as a student who is deaf or hard of hearing.
• Bertrand needs for special communication services are addressed in his IEP.
• Bertrand needs assistive technology devices and/or services without which he would not benefit from special education.
• Bertrand's needs for assistive technology devices and/or services are addressed in his IEP.

4. Measurable Annual Goals

• Annual Goal 1: Bertrand will use eye gaze with an adult in the classroom to indicate turning the pages in a book or continuing a preferred activity or toy.
• Annual Goal 2: Bertrand will stay alert for 5-7 minutes during group activities.
• Annual Goal 3: Bertrand will tolerate a variety of textures and various sitting positions throughout the school day.
• Annual Goal 4: Bertrand will demonstrate anticipation of upcoming events when presented with a touch cue.
• Annual Goal 5: Bertrand will indicate a choice among two preferred items.
• Annual Goal 6: Bertrand will use a switch to activate a toy consistently within the classroom.

5. Services plus Adaptation Support

• Special Ed. Preschool - 150 minutes 2 times/wk
• Speech Services - 30 minutes weekly
• Occupational Therapy - 30 minutes monthly
• Physical Therapy - 30 minutes monthly
• Visional Services - 30 minutes monthly

A registered nurse will assist Bertrand in class through Spring in order to train his teacher and her aides in seizure identification, treatment, handling, etc.

6. Participation in State & District Assessment (N/A)

7. Notices & Participants

• Eligibility for Extended School Year (ESY) services will be determined later.
• Parent Prior Notice for Free Appropriate Public Education.
• Medicaid Consent

While Bertrand is at school

Bertrand's 2010 class photo. Love it! This expression is SO Bertrand! :)

If he isn't sick, seizing--too badly--or suffering from broken bones, Monday through Thursday, 8am until 11am, Bertrand can be found at the WONDERFUL Carmen. B. Pingree Center's Clinic Program.** Our family loves the program, the facilities, and most of all the staff there! They have worked wonders with Bertrand by augmenting the therapy and work I do with him at home. In particular, much of the social development Bertrand has experienced since February can be attributed to them.

That said, having Bertrand in the program also gives me an uninterrupted 2.5 hours to work or run errands. It's been a sanity saver. Here is a quick rundown of what those precious hours were like this morning:

• Eat breakfast
• Read medical section of newspaper (today: food allergies in children)
• Load/run dishwasher
• Upload and edit Bertrand's school photo
• Order prints of photos from Wallgreens.com
• Call Shriners physical therapy department
• Call Bertrand's orthopaedist for new therapy orders
• Call Shriners to schedule orthopaedic follow-up
• Call PCMC to schedule gastroenterology follow-up
• Email volley with Matt regarding American Express statement
• Receive, read and edit Bertrand's Classroom Health Care Plan
• Send Health Care Plan revisions/updates to SL County School Nurse
• Finalize documents to be taken in for IEP meeting tomorrow
• Schedule orthopaedic follow-up for January 11th
• Call Shriners to confirm wheelchair order execution
• Confirm that wheelchair order sent with insurance
• File EI graduation paperwork
• Confirm email receipt with Shriners

I know I've forgotten a few more items. But, all this was sprinkled with tweets, facebook and email replies while I was on hold (holiday muzak is the worst), random swiffering of kitchen floors, clorox wiping countertops, and, oh yes, repeated door openings for indecisive, barky, little dogs.

Best part? As I was running out the door to pick-up Bertrand, I told Matthew, "I don't know where my morning has gone. I've done nothing but I'm already exhausted."

__________________

**Please email me if you ever want an exceptionally biased and positive review of the program.

Graduation from Early Intervention

Not impressed with the certificate or stuffed bear, Bertrand would prefer that his therapists keep visiting him.

Today, Bertrand "graduated" from Early Intervention (EI). EI has been a part of our lives practically since moving to Utah: about August 2008. Bertrand's EI team consisted of four wonderful, talented and compassionate women who'd each come to our house twice a month--rain, snow or shine. They came under the auspices of providing therapy services (physical, speech, occupational and vision) for Bertrand, but honestly they did just as much for me. I know I should be celebrating that my boy is turning 3 years-old, but I am going to miss Kirsten, Meghan, Lisa and Linda. And, so will Bertrand.

Ladies, we love you. Thank you for all you've done for Bertrand and for our family!

Another Drug

Pictured above is Bertrand's current "breakfast". (Missing from the picture are miralax and phlexyvits, but Daddy took the picture, not me.) It seems like we can never add just ONE item without requiring several more. In fact, more than half the stuff in the picture is needed because of a problem caused by some other item in the picture (e.g., keppra binds with B6, zonegran causes stomach ulcers, the ketogenic diet is low in vitamins and causes constipation, etc.).

This is actually not a lot of medication compared to some other kids we know, but it is still more than Daddy and I would like for Bertrand. That's why I cringe at the thought of adding another medication. But, that's the point we are at with Bertrand's seizure management. Bertrand's seizures, particularly the atonic (drop) seizures, have gotten much worse. We would be adding a new seizure medication in the hope of reducing this number and to be allowed to wean another medication that is not working. (Zonegran has provided no seizure relief but plenty of nasty side-effects.)

So, at Bertrand's neurology appointment last Thursday, the neurologist laid out three medication options: Valproic Acid (Depakote), Topiramate (Topamax), and Lamotrigine (Lamictal). These three drugs are metabolized in the liver, and up to this point we've shied away from putting any additional stress on that particular organ. Liver damage and liver failure are not topics doctors or parents take lightly! However, according to Bertrand's gastroenterologist, the current state of Bertrand's liver damage and treatment is stable. Since Bertrand's quality of life is suffering significantly, we can proceed with hefty doses of caution (extra blood draws, constant monitoring, etc.). After all, a liver doesn't do you much good without a brain--and vice versa. Striking a balance is important.

• Depakote: Used for generalized convulsions and absence seizures. Fast results. Side-effects include liver failure, stomachaches, increased appetite, weight gain, change in personality, irritability, tremor, hair loss, decrease in platelets, Stevens-Johnson Syndrome (allergic rash), and tiredness.
• Topamax: Used for several seizure types including partial seizures. Moderately fast results. Side-effects include tiredness (hence the nickname Dopamax), irritability, language problems, behavioral problems, decreased appetite, weight loss, dizziness, kidney stones, acute glaucoma, decreased sweating. (Effects made more severe in conjunction with ketogenic diet.)
• Lamictal: Used for several seizure types including partial and complex-partial seizures. Slower results. Side-effects include Stevens Johnson Syndrome, tiredness, dizziness, vomiting, ataxia.

The choice of medication was left up to us, Bertrand's parents. We are well-versed on the pros and cons to each one from the handouts given to us by the doctor and our own excessive research. We also reached out to our vast online network consisting of parents of children with intractable epilepsy and movement disorders. This was invaluable, but it was like drinking from a fire-hose until patterns started to emerge.

While it is the drug our neurologist has the least experience with, we feel that Lamictal is a conservative first choice. In comparison, Depakote's liver risk is just too high and Topamax's cognitive effects too severe. The greatest concern with Lamictal is an allergic response called Stevens-Johnson Syndrome, which is addressed through a slow introduction. (This is why results are seen much later than with the other drugs.) But, liver involvement is minimal, it is used with success by kids with multiple seizure types and, in particular, complex partial seizures (some of Bertrand's most insidious seizures). Furthermore, a child similar to Bertrand, with a movement disorder AND seizures, went seizure-free with Lamictal.

We have to start somewhere, so Lamictal it is.

Bertrand watches "How To Train Your Dragon"

For a change from his typical Elmo fare, Bertrand sat down to watch "How To Train Your Dragon". It is a DreamWorks production that came highly recommended from other special needs parents for its portrayal of disabilities. Several characters in the film, including the title dragon, have need of prosthetic limbs, but this doesn't define their characters or limit the greatness they are capable of. It's a cute movie and, as you can see from his youtube review above, Bertrand enjoyed it!

3 Month Post-Infusion Report

Starting early November, our family started to receive "gifts" that are possibly the courtesy of stem cells. This is the three month post-infusion report to Dr. Kurtzberg, who conducted Bertrand's autologous stem cell infusion at Duke University in late August 2010.

Hi Dr. Kurtzberg,

I've been putting off this email because I didn't want to "jinx" the small, positive changes Matthew, Bertrand's therapists, and I have seen in Bertrand. ;) Up to this point, there has been no change in his medication, therapy or other interventions (but he'll be starting a new AED next week because there has been an increase in seizures since his steroid wean). That said, here are some of the changes we've seen, starting early November.

• Improved eye contact
• More frequent vocalizations and greater range
• Less extraneous movement (he feels more stable and calm)
• Playing with new toys
• Opening hands more (not as fisted) and will brace himself
• Using fingers
• Posture is more relaxed, with shoulder forward and arms down
• Initiates trying to get up or change position
• Can abdominal crunch to get into sitting position
• Can wipe at his nose
• Things such as a stuffy nose now bother him
• Enjoys getting his hair brushed, being touched
• Engaging in more stiming behavior - teeth grinding, rubbing textures, playing with things

We don't know if these changes were natural, the result of the infusion, or what, but we are grateful for them. I've spent over 2 years working with Bertrand, every single day, on improving his sitting and having his shoulders forward, to then one day have him spontaneously start doing it on his own?! It is fantastic!

Thanks and happy holidays!
Cristina

"Santa, all I want..."

Bertrand tells Santa, "all I want is a sister who looks like Elmo and parents who don't make me sit for stupid photo-ops."