Conference Results - Hemispherectomy

Yesterday was the Neurology multi-disciplinary discussion at the Blue Bird Clinic at Texas Children's hospital regarding Lillian's case. I didn't attend because it was for medical professionals only, it's probably best that parents aren't in the room for those things I guess!

My pediatrician Shannon Hayes attended along with the team of neurologists, the neuro psychologist, epileptologists, neuro surgons, other doctors and specialists. So a very seasoned room of people I would imagine!  Our epileptologist presented Lillian's facts, video footage of her EEG's, seizures, MRI's, and other pertinent medical history and information.  Then the room discussed next steps, such as new meds, keto diet, vegus nerve stimulator, and surgery as options.

According to the message relayed back to me, the entire room was in almost instant agreement that the only solution for Lillian will be a functional hemispherectomy.    She is still having about 180 seizures daily (according to her last in patient EEG) and despite maxing out on various meds the seizures continue to get worse and worse.

While researching this I found that probably about 100 hemispherectomies are performed in the US each year and about 20 or so of those are done at TCH* (*note this is just a quick estimate relayed to me not official statistic).   I can barely handle typing this but a hemispherectomy is the removal/disconnection of an entire hemisphere of the brain (Left or Right), leaving the patient with half of a brain effectively.

Dr. Quach told me that Lillian will need a RIGHT SIDE FUNCTIONAL HEMISPHERECTOMY.  I need to ask TONS more questions but online  it says during this procedure the temporal lobe is removed, a corpus callosotomy is performed and the frontal and occipital lobes are disconnected from the rest of the brain. So the parts that are not removed are disconnected and basically floating around so that the fluid does not overtake the brain.  The hope is that Lillian could be 100% seizure free after the surgery.

Obviously its a very risky surgery and we can't go back and undo anything if it should happen to go wrong!  But the risks of NOT doing the surgery are also great. 1. Lillian has many risk factors for  SUDEP based on her frequency and timing of seizures (most of them happen while she sleeps) 2. The doctor thinks the seizures will continue to get worse and worse and could eventually start attacking the good side of her brain.

So we have weeks and months but not years to make this decision for Lillian. It is very scary and we are praying and doing research to do the right thing.

Some websites about hemispherectomies:
<http://www.surgeryencyclopedia.com/Fi-La/Hemispherectomy.html>
<http://hemifoundation.intuitwebsites.com/facts.html>

Lillian's MRI....I can't sleep so of course I am looking at all these pics over and over. I have had the disk since August but try not to get them out.  I'm not a medical professional but some obvious issues here.  NOTE: MRI images are flipped so you see the right on the left and left on the right side.

 

Lillian's MRI August 2012

 

 

Please keep praying and/or thinking kind thoughts for my daughter!! Thank you :)

 

 

Our Texas PMG Family

After finding out my daughter had a rare condition called "Polymicrogyria" (too many small folds on the brain)...I ran to the Internet and searched for answers to understand what her outcome would be. One thing we all must realize is that each case of Polymicrogyria is very individual and the outcome for the child varies GREATLY.   It all depends on many factors, such as what other conditions are present as well as the AMOUNT of the brain impacted by the PMG. For example, Lillian has a UNILATERAL case, meaning that only half of her brain is impacted (the right side).  However her 2 new friends have BILATERAL case, meaning that both sides of their brain is impacted.

So on to Lillian's new friends Cora and Kaylee! They are PMG families living in Texas. One family in San Marcos (in the process of relocating to Houston!!!) and another family in San Antonio. We all share the same Neurologist (Dr. Clark at TCH) as well as Dr. Jane Edmonds (neuro-opthamologist), and Kaylee and Lillian have the same Epileptoligist Dr. Quach. 

Kaylee Rae

https://www.facebook.com/#!/KayleeRaesStory

Kaylee's mommy sent me a text one day that her daughter was in TCH for a one hour EEG at the same time my daughter was inpatient. So we decided to meet up! She came and sat with me in the hosptial room for a few hours. <3  It was like we had already known each other for ages. Kaylee has generalized Polymicrogyria meaning that her entire brain is impacted. She has cortical vision impairment, epilepsy, cerebral palsy, and is fed by G Tube.

Then we formed a team together for the Houston "Stroll for Epilepsy" :)

And we plan to meet up lots more once she gets settled in Houston!!

 

Cora Jane

 

https://www.facebook.com/#!/CoraJanesJourney

 

Yesterday I finally met Cora Jane's family for the first time!! They will have their first trip to the Texas Children's Hospital Epilepsy Monitoring Unit next week so please pray for good results for them!! In addition to PMG, Cora also has Agenesis of the Corpus Callosum, Microcephaly, Epilepsy, Infantile Spasms and Cortical Vision Impairment.

 

 

Her family is so sweet and we talked for hours about lots of stuff - insurance, types of therapies, how we felt the day we found out our child had PMG, etc... Kind of like free therapy if you ask me! Like Kaylee, Cora is more severely impacted by Polymicrogyria because she has it on both sides of the brain. So while Lillian needs your prayers there are two other babies out there who need them even more!

 

<3 Speaking of Prayers Lillian's case is going to be presented at a multidisciplinary conference on Tuesday May 7th so please keep her in your thoughts! The team is going to discuss next steps for her because so far we are unable to control her seizues with medication (we have gone through 5 different ones). I still see seizures every day (the strange staring spells) but she continues to smile through all of it.

 

 

 

Thanks for reading!! I feel so blessed to have a PMG "family".

                  

To my new friends:     

"Maybe I can't stop the downpour, but I will always, always join you for a walk in the rain." - Unknown

 

 

Sandra

 

Texas Children's EMU (Epilepsy Monitoring Unit) - Take 3

Yesterday was our third time visiting the EMU at Texas Children's Hospital.  I am starting to remember faces and some names a lot more! Everyone is so nice :) We arrived to do a 24-48 hour video EEG to follow up on the progress of Lillian's seizure reduction meds.

The room is outfitted with a video that is watched 24/7 by the EMU Technicians. Lillian is hooked up to wires that monitor her seizure activity the entire time, which are also watched by the techs. The doctor reviews and gives us the results.

First Lillian cuddled with her buddy Teri before she had the wires attached.  Teri is the Physician Assistant for the Epilepsy Monitoring Unit.

Then our old buddy Dean attached the wires to Lillian's head. She really doesn't like that part. It takes about 45 minutes to complete but she did as good as we could expect!

 

 

Afterward Lillian smiled for Dean for the first time!

 

 

Lillian wanted to ride around in one of the toys from the Child Life area.

 I think we made it rather difficult for the video tech's because they have to zoom in on her at all times! Plus we couldn't leave the room because she was hooked up to the wires! But she smiled anyway.

 

 

She is super mobile now which we are certainly pleased about ...however we had to keep a close eye on her so she wouldn't get into anything!!

 Child Life also hooked us up with a huge play mat for her to use.

 

Vivian attended the evening "Radio Lollipop" session at the Hospital. They offer crafts and music, it is alot of fun! She also got to be a "guest DJ" to say hi to Lillian on the air! Lillian and Neal listened to it from the room.

 

Here is the view from our room on the 10th floor. It was a little overcast today.

And Lillian with nurse Tina prior to discharge! Tina was our day nurse both days, she is so nice.

 

We learned that Lillian's seizures are back up to 80 or so during the daytime and 100 at night time.   Yes around 180 daily! This is despite the fact that she is taking 2 anti seizure meds (Zonagran and Klonipin). We are also seeing more physical manifestations with her seizures. She exhibits alot of blank staring where she is non responsive for about 10 seconds. This just started happening within the last month or so. 

 

After seeing 180 seizures in the 24 hour period our Epileptoligist, Dr. Quach said he had enough footage and that we may plan to increase meds at home and add a new one called ONFI.  Dr. Quach is so patient with our family and takes time to show us video footage of the seizures so we know exactly what to look for. He is also going to take her case to a multidisciplinary doctor's conference to see if they can brainstorm other alternatives/ideas for Lillian. The other options (outside of medicine) that we talked about so far are: ketogenic diet and surgery (hemispherectomy).  The surgery would be the ultimate last resort because it is obviously very invasive.

 

So I am hoping for a miracle, that maybe this new medicine combination will work for Lillian! I have hope for her!

 

Sandra 

AFO's

This past week Lillian was casted for a LEFT SIDE AFO (Ankle-Foot Orthoses) .  Due to her right sided polymicrogyria she has difficulty with fine and gross motor skills on the left side of her body.  The goal of this device is to help position her ankle so that she can more easily bear weight and practice standing/walking :) :)


They will send her cast off to be molded into a customized orthotic and we will have it back in 2 weeks! Vivian picked out a pretty pink heart print for her sister.


Waiting and just chillin - I brought Vivian along too, she is so supportive of her little sis!

We had to have Lillian sit really still while the orthotist casted her foot .  The gentleman casting Lillian's foot had never heard about Polymicrogyria so I told him all about it!

 Here is a picture of the itty bitty cast that will be used to make her first AFO!

 

That day was a busy day! After getting fitted for the AFO we went across town to have lunch with my husband so we could tell him all about the appointment!

Then big sis Vivian had a regular dentist appointment.

Then we raced back home just in time for therapy with Ms. Monica!!  

 

 

What a fun busy day!

Lillian Grace - Circle of Hope

Lillian Grace - Circle of Hope

I watch the news way too often and sometimes get discouraged by how much bad there is in the world.  But then every day I am reminded that the GOOD in our world outnumbers the bad many times over!

Sometimes it's hard to appreciate the everyday things in life, but I am always sincerely amazed when we meet with Lillian's doctors and therapists and see their genuine interest in working with our family.    So late one night I worked on this "Circle of Hope" in recognition of the people who work with Lillian to make our lives wonderful.

First is the ever expanding network of doctors and services we receive at Texas Children's Hospital.

**The Neurology department including our Epileptoligist Dr. Michael Quach and our Neurologist Dr. Gary Clark. And I greatly appreciate the awesome staff in the Epilepsy Monitoring Unit at the hospital. 

**Dr. Gail Demmler-Harrison and Holly Corwin (Infectious Disease) helped me retrieve Lillian's newborn bloodspot and had it sent to the CDC for testing.  Plus they answer my calls and emails at any hour and are super fast to respond, no matter what the question is :)

**Our PT for Aqauatic Therapy William Lynch works for TCH and he does an awesome job with Lillian every Monday morning. 

**Also I have a great audiologist, Amy Magruder, who checks Lillian's ears every three months during an ABR Procedure.  We will meet our new ENT Dr. Robert Williamson next week.

**Lillian was evaluated at the Meyer Center for Development by doctor Sherry Sellers Vinson. She helped get all the necessary referrals that we needed and reassured us that Lillian would do wonderful.   She will keep seeing us every 6 months.

**Just last week we met Dr. Mary Lynn Chapieski and she did a cognitive evaluation on Lillian.  She was surprised at everything that Lillian was doing, despite her number of seizures and other setbacks!

**Dr. Jane Edmond is our neuro opthamologist, she will keep a close watch on Lillian's vision.

**We haven't been to the Physical Medicine and Rehab clinic yet, but will be going there next month and hear it is awesome!

Second is the Early Childhood Intervention Program, the program where we receive Lillian's therapy services in home.  PT, OT, Speech, Auditory Impairment, and a new Specialized Service Therapy.
I will do a separate post on that :)

Our Primary Care Physician for Lillian, Dr. Shannon Hayes has been an excellent partner in our journey with Lillian.

And finally online support groups, prayers, love, and family are the glue that hold everything together!!!

Thank you.

Texas Children's EMU (Epliepsy Monitoring Unit) Take 2

March 5th was our followup video EEG to check and make sure Lillian's anti seizure medicines were working.  It was nice to see everyone in the EMU again, and by this visit I recognized most of their faces and remembered a few names.  *Hello EMU friends, thank you for all you do for my daughter Lillian, you are awesome!! <3*

Right before our inpatient appointment we had a quick follow up with infectious disease doctor Dr. Gail Demmler-Harrison. Then we rushed on over to the west tower to get checked in!

Outside the elevators on the 10th floor is an AWESOME outrageous PINK color! I just thought I would throw that out there...next time I will take a pic.

Here is a pic of Lillian getting hooked up by the nice tech's! The blanket that she has here was given to her last visit and is a PROJECT LINUS blanket....learn more!! What an awesome organization :) http://www.projectlinus.org/

We had a pretty uneventful day but had an inpatient ENT consult. The ENT resident confirmed that Lillian still has fluid in her ears and recommended antibiotics. We will try that and follow up with the ENT in 3 weeks.

The CMV researcher Holly Corwin came in to visit us and we discussed filling out the necessary paperwork to get Lillian registered with the national registry. She is sooo sweet and a few years younger than me, and I really enjoyed her visit!!

At night time we listened to "Lollipop Radio" and  also completed a craft :)  I think Lillian enjoyed listening to the music.

The next day the epileptoligist on call came in with his team and informed me that Lillian's EEG did not look good at all. It was a huge disappointment to me but we just kept on going! At that point the nutritionist came in and consulted with us. Lillian's meds are causing a decrease in her appetite, and her weight (8 Kilo's) is in the bottom 1 percentile for a 14 month old so we need to get some meat on her!! We started concentrating her toddler formula and pray she will start eating more!

 

Also our wonderful neurologist Dr. Gary Clark came to visit and offered encouraging words of advice. He always makes us feel better.

 

Then I saw my epileptoligist, Dr. Quach waving at us in the window! Although he wasn't on call he follows Lillian very closely and wanted to give us an update. He came in and said that while Lillian's EEG showed a lot of abnormal activity, she is pretty stable compared to how she was on the last day of our prior visit to the EMU.  Lillian is having about 60 seizures a day, with 20 of them during the daytime and about 40 at night time. They cause her to wake up 2-3 times per evening.

 

He said there was no point in keeping us in the EMU, that he felt comfortable sending us home to ramp up the meds rather than doing it in the hospital.  So we are home and increasing the meds, and we will go back to the EMU next month again for a 48 hour EEG to see how everything looks!! Currently Lillian is taking Klonipin and Zonagran for her seizures.

 

My friend Katie and her lovely little boy Connor came to visit us just before we were discharged :)

 

 

 

Bye bye doctors, techs, nurses, PCA's....we will see you next month in the EMU!!!  Thank you for helping my family :)

 

 

 

 

Sandra

 

 

 

Genetic Test Results

The genetics department at Texas Children's hospital called me today to say that there were NO connections to Lillian's conditions based on the genetic testing.  As I mentioned before, she had extensive genetic tests done and they found 2 variants that had "unknown significance".  I submitted my blood work and had the same 2 variants.  The genetics department indicated that these are 2 normal variants to have and that they are in no way related to any of Lillian's health issues.   They said that many of us have slight mutations in our genes that do not cause problems, and we never know about them because we do not all have genetic testing. So these variants are just "normal" to have and in no way relate to Lillian's diagnoses. 

So we have one less specialist to see... (Genetics) ...we confirmed that ALL of Lillian's health problems are the outcome of Congenital CMV, a cold like illness that I had during my early 2nd trimester of pregnancy :(  I have many prior blog posts about congenital CMV so please read and be aware!

HERE ARE A LIST OF POTENTIAL PERMANENT HEALTH PROBLEMS DUE TO CONGENITAL CMV    http://www.cdc.gov/cmv/congenital-infection.html

Permanent health problems or disabilities due to congenital CMV infection (source:CDC)

• Hearing loss  - YES, Lillian has this
• Vision loss  Hope not!
• Mental disability YES, delayed development
• Small head size   - YES, Lillian has this
• Lack of coordination   - YES, Lillian has this
• Seizures   - YES, Lillian has this
• Death (in rare cases)

In addition to the conditions listed above Congenital CMV is known to cause Polymicrogyria, which Lillian also has.

It is good to have an answer and close the genetic chapter and move forward. It is easier accepting the congenital CMV diagnosis as 100% causal for Lillian's conditions because we have the confirmation through her newborn bloodspot.

Love you Lillian Grace!

www.stopcmv.org