Grace Web Log

To keep friends and family up to date on Grace.

[March 1, 2007]

We have a date.  Grace's surgery will be April 20th.   The Intraoperative MRI is up and running and will be ready for use on HH patients at the end of March.

We'll be driving to Phoenix the weekend before surgery as Grace will have several pre-Op doctors appointments.  We are blessed that we have several family members that live in the Phoenix area and we'll be staying with them prior to surgery and when Grace is released after surgery.

We're still in the planning process and as we find out more over the next few weeks, we'll post here.

Thanks for all the prayers for Grace!!!

[November 15, 2006]

We made our long awaited visit to Phoenix .   During our visit, we met with Dr.Rekate (neurosurgeon) and Dr.Ng (neurologist). The consensus continues to be Grace is a candidate for the TC approach and should have surgery as soon as it makes sense.

Although we were hoping to have a surgery date when we left, Dr.Rekate recommends we wait for the Intraoperative MRI to be available. He believes this new technology will really be beneficial for Grace's HH removal, primarily because the HH is so large. He also mentioned that about 90% of the HH is attached one side which will be a benefit during removal/detachment.

The I-MRI is currently being calibrated for surgical use and should be ready sometime in January for initial patient use.  Then there is the learning curve and familiarization time needed before they start using it for HH surgery.  We are still estimating Feb-Apr time frame.  It should have been up and running already but they had a mishap during installation.  Some yahoo walked into the room w/ a metal tank and the magnet was broken when the tank slammed into it.  Let’s hope there are no more delays.

 So, for now we wait to get news of when the I-MRI equipment will be ready. Right now we know the system is up and running and in preliminary testing and calibration.  They are hoping it will be ready in January 2007 for initial patient use by Dr. Spetzler.   Once he's comfortable with the system, Dr.Rekate will assist in a couple of his surgeries and then will be ready for use on HH patients.  Dr.Rekate said that Dr.Spetzler would be assisting on Grace's surgery as he would be the expert on the Intraoperative MRI.

We are really happy with Dr.Rekate's conservative approach with Grace's surgery. We know her HH is one of the largest if not the largest they've had to remove, so waiting for the Intraoperative MRI is definitely okay with us. My only disappointment from our visit is not having a specific timeline for when we can schedule Grace's surgery.

We'll be contacting Dr.Rekate and team throughout the next couple of months to keep informed of their progress. We are still hoping for surgery by March 2007, but will have to wait and see.

 On a happy note, we got to visits several other HH families during our visit.  Some were there for surgery, others for consultations and other local families just at the hospital to support the rest of us.   We also had a wonderful visit with some of our aunt and uncles that live in the Phoenix area.

As for Gracie, she is a trooper and doing well.  We have not seen any cognitive decline.  Her speech therapy is improving the amount of words spoken.  Her seizure activity is about the same.  There might be a slight up tick in the amount and severity of the seizures.  We are hoping we will be able to keep this in check over the next few months.  We have noticed a “honeymoon” period in her seizure activity every time her medication levels are modified so we are hoping we can keep them low by lowering and raising her levels every few weeks.  

And for Sarah, well, she is everywhere, talking, climbing, and all around wonderful precious little angel.

We’d like to thank everyone for their continued support during this rollercoaster.  We appreciate all the thoughts and prayers you’ve been sending to Gracie over the past 1.5yrs and ask that you continue to do so. 

Love and warm regards,

Perry, Erica, Grace and Sarah

[October 5, 2006]

Most of you may have received an email and flyer from Erica’s Aunt Kathy Gibb about our future plans and fundraiser for Grace.  She has done a wonderful job taking the initiative to getting us started on our next difficult step and I would like to thank her.  Thank You.  With that said, we felt it was also time to do a log update to try to let you know more detail about our next.

We've decided the best thing for Grace is to move forward with surgery.  It has been a decision Erica and I haven't been looking forward to, but we've made it and have faith it's the best chance for Grace to live a healthy and normal life.

So, we are going to Phoenix in early November to meet w/ the neurosurgeon and the neurologist at BNI. During this trip we will learn more detail about the two surgical procedures that Gracie will require.  On our previous trip we did not get into any specifics w/ the surgical team since Grace was not having seizures at that time.  As you can imagine we have a ton of questions about what lay ahead.  We do not know if the procedures will be done at the same time, days or even years apart.

We will also learn more about their Intraoperative MRI that is being installed.  We hope we will be able to gather enough information to make a definitive decision about scheduling the operations. At this point we can see how their new MRI would greatly increase the chances of a successful operation (they would not be putting it in if it did not help) but we are not sure if it out weighs the amount of damage to Grace’s development that the seizures will cause during this delay.

As always please keep us in your thoughts and prayers and we will do the same and of course we will update you all as we know more...

Love Perry, Erica, Grace and Sarah

Link to Amazing Grace Webster Fund website

[July 24, 2006]

We have a few things to report based on Grace's visit with the neurologist two weeks ago.  This appointment was a follow up to discuss the medications she'd been taking for the past nine months.  

When Grace's seizure activity began last August I started to maintain a seizure log.   This log captures how many seizures she has per day during her waking hours, as well as the catalyst for the seizure, duration and description of the seizures.   This log became a great tool during our medication trials as we could easily monitor changes in seizure activity as we added a new medication or changed the dosage of a medication.  

So, after logging over 1200 seizures these past ten months and providing the data to the doctor, the consensus is the medication indeed don't appear to be helping in the control of her seizures.   Now this isn't a surprise to us.  We knew from the beginning the typical epileptic medications don't seem to have any positive impact on seizures caused by HH, but we gave them a try in hopes Grace would be the lucky one.  

We are now in the process of taking Grace slowly off the two remaining anti-seizure medications.  This is going to be a process that takes several months as we reduce the dosage of one medication every two weeks and then do the same with the other medication.  Perry and I are both very happy the doctors agreed to take her off the medication.   

We do have some not so happy news to share.  Grace has been showing signs of one of the other side effects of HH, which is what doctors call "hypothalamic rages".   These by far are the worse side effect of these tumor we've experienced.  Several of the parents from our HH support group have shared very candid stories with us regarding their children's rage activity and it's definitely something that could expedite Grace's surgery.   We are fortunate Grace has had less then 10 of these over the past two months, but we're worried they might increase in frequency overtime.  Unfortunately, we don't know exactly what to expect, so again we have to just wait and see.

Thank you everyone for keeping Grace in your prayers!

[April 9, 2006]

Lots of things to update you on, hopefully I'll be able to capture everything in this post without overwhelming you with too many details, but not only is this log a way to give all of our friends and family an update on Grace it's also my little way of keeping all these things straight. My memory just isn't what it used to be :)

MEDICATION SAGA CONTINUES....

So, let's start with the latest on the not-so-wonderful world of anti-seizure medications.  

During my last post, I wrote the new medication, Tegretol, seemed to be working.  Based on this improvement her dosage was increased on February 24th.  Within a day of this increase we noticed it was making her extremely tired for several hours after taking it.   We also started to see a *new* type of seizure, which was very different than the gelastic seizures.  While having these *new* type of seizures, Grace is very quiet, becomes pale and very weak and won't respond verbally when you talk to her during the seizure.  Once the seizure is over, her hands will tremble and it takes her up to one hour to 'recover' and be her normal little self.  In addition to the evident drowsiness and new seizures, we also began to see an extreme and disturbing change in Grace's personality within one week of the increase in Tegretol.  We contacted her neurologist and it was decided to decrease the dosage of Tegretol and see if that helped.  Luckily within a day of the decrease we saw our little Grace begin to come back to her normal happy self. 

Since the decrease of Tegretol on March 3rd, we've increased the Tegretol yet again (yes, you heard me right, increased again), but this time we're spreading the dosage over the entire day.  So instead of 6ml 2x per day she is having 4ml 3x per day.  The good news is she isn't drowsy and it doesn't seem to be modifying her personality/behavior.  The bad news is she's still having seizures (4/day).  So, I'll be talking with the neurologist this week to see what the next step is with Tegretol.  

BOSTON SPECIALIST REVIEW GRACE'S RECORDS...

Perry and I decided we wanted to get yet a 3rd opinion on Grace and her HH, so we sent all of her recent records (MRI, VEEG, seizure activity, and medication levels) to a Dr. Black at Boston Children's Hospital.  This doctor has been performing HH surgeries and has been very successful based on the research we've done and by the input of several folks on our HH Support Group.  

Now before I tell you his recommendation, I need to let you know that this doctor only utilizes one type of surgical procedure for the removal of HH tumors and doesn’t specialize in HH patients.  This procedure is the transcallosal (TC) approach, in which they enter from the top of the head and go between the left and right lobe of the brain to reach and detach/remove the HH.   The team at Barrow Neurological Institute (BNI) that we visited with in August 2005, utilize several different surgical procedures to remove the HH and had already explained that Grace would require two different procedures (TC and endoscopic) to completely remove the HH as her HH is extremely large and has a multiple connection points.

So, now that you have the background on the surgery options...on to Dr. Black's recommendation.  At this time, he would not recommend surgery as he would not be able to successfully remove/detach the HH and as a result would not be able to stop the seizure activity.   His assistant asked if Grace had been reviewed by any other doctors and when I told her BNI she indicated that was exactly who we should be talking to.

Looks like if and when we need surgery it won't be at Boston, unless Dr. Black begins to utilize the other techniques already in use by the doctors at BNI.

BARROW NEUROLOGICAL INSTITUTE REVIEW GRACE'S RECORDS A SECOND TIME

At the same time Dr. Black was reviewing Grace's records, we also asked the team at BNI to revisit our Gracie’s case.  The team reviewed her records last Wednesday and had quite a bit to share with us regarding next steps for Grace.

The first thing they noted was a concern with her expressive speech delay.  Now this is something that we've been concerned with for about 9 months, but initially all the doctors, including the BNI team, told us not to worry as lots of kids don't talk until 3yrs old.   

So why is BNI concerned now?  Basically, because when they had reviewed Grace's records in May 2005 and examined her in August 2005 she was not having seizures.  Obviously this is no longer the case so they believe there is a very high probability that the expressive speech delay is a result of the seizure activity and are concerned that other developmental activities might be impacted by the seizures.  As a result, they have recommended she have a Neuropsychological Examination when she turns 2 1/2 as this is the earliest age this exam can be performed....so, we'll be heading off to BNI (Phoenix) late this summer.

We were also told about research the Neuropsychologist has completed.  It appears there is a relationship between the probability of cognitive decline of the HH patient and having a combination of three symptoms, which Grace has all three.  These symptoms include a large HH lesion/tumor, precocious puberty and early onset of seizure activity.   So, based on this research it is likely that there could be more declines in the future although Grace's only apparent delay is with her expressive speech.

When I asked the Program Director exactly what we should do next she indicated that BNI likes the conservative approach to surgery and would like us to continue with the anti-seizure medication to see if we can find a medication that works for Grace.  However, they feel a trial of 3 medications is sufficient to determine if medications will be successful in treatment or if surgery is the next step.  She also indicated that if we see any drastic changes in Grace's development to contact them immediately.

The Program Director stated the decision for surgery is something each family needs to think about and determine what makes sense for their child.  She indicated some families decide to remove the HH to eliminate the seizures, regardless of any other symptoms, while other families wait to see cognitive decline before deciding to have the surgery. Unfortunately the history shows the medicines rarely works and it is just a matter of time before decline is evident which could happen slowly or in a matter of days.

Obviously this is something Perry and I am constantly thinking about and have been since Grace was diagnosed.  Right now we feel that we really need to give the medications a chance to help eliminate the seizures, however, if and when we see any development decline we will begin the steps to having the HH removed.

[February 18, 2006]

Grace is doing very well on the new medication.  So far the last increase of Tegretol seems to have reduced her seizures from 4/day to 3/day, with only 1/wk of the really big/strong seizures that make her tired.  We're hoping this isn't just what I call the 'honeymoon' period of the medication and this will be the long term effect.  I'll be contacting Dr. Tharp's office next week and assume they'll suggest another increase of the Tegretol and decrease of Keppra.  I'm hoping eventually we can get completely off the Keppra.  Grace is such a good sport about taking her medication most of the time but it would be great if we only had to deal with a single medication.

In a week or so Grace will need to have a blood draw to check her white blood count.  This is because one of the possible side effects of the Tegretol is a decrease in white blood cells which would make her more prone to infection.  If she shows any signs of this we'd have to find another medication for her.

Thanks again to all of you that keep Grace in your thoughts and prayers.  We love you all very much and are so thankful to have you in our lives.

[February 6, 2006]

Today was the follow up appointment with Dr. Tharp to review the Video EEG results.  As expected, the seizures Grace had during the 4 hours did show up on the EEG and were confirmed by doctors reviewing the captured video.  Dr. Tharp indicated the main purpose of the VEEG was to ensure she wasn't having generalized seizures, which is when the entire brain seizes.  If this were the case, he'd have to change the medications.  

However, Grace's seizures are not generalized and in fact are typical of those that result from HH.   The seizures are originating from the hamartoma and then spread through the frontal and right hemisphere of her brain.

We discussed the past weeks seizure activity, as Dr. Tharp was curious if the new medication was having any impact.  It appeared to be helping a bit by reducing her seizures from 6/day to 4/day, but more importantly the strong seizures that cause Grace to be very tired had decreased from 5/wk to 3/wk.  It was agreed we should increase the dosage of the new medication and decrease her dosage of Keppra. 

We asked Dr. Tharp how would we determine or qualify a medication as being successful in treating Grace's seizures.  He restated what he has told us during previous appointments.  We want to get Grace's seizure level to a point such that her quality of life and development are not hindered.  He said this criteria is different for each child.  Some kids get to the point where they don't have any seizures, others have a couple a month, while others can have 1 a day and still be thriving. 

So for now we are continuing to watch Grace's development to ensure she doesn't show any decline and we'll continue to try these medications to see if we can reduce the activity to less than 4/day.

Please keep Grace in your thoughts and prayers

[January 27, 2006]

We had an appointment at UC Davis Medical Center for Grace's Video EEG.  It was scheduled for 4 hours of actually taping, but the overall appointment was from 8am to 1:30pm.  It was quite an experience to say the least.  The setup required over 30 electrodes to be placed on her head, cheeks, behind her ears and on her chest.  As always, Grace surprised us on how patient she was while the technician marked her with a blue marker to identify each electrode placement.  He then had to clean each spot, glue the electrode down and then used an air brush to activate the glue.  Once he finished attaching on the probes, Grace's head was wrapped with gauze and then a stocking around all of the wires coming from her head, in order to keep her from being able to pull anything loose.  After setting up the EEG machine and the video camera, we spent the next 4 hours trying to keep Grace as happy as possible.  This included watching TV, coloring, reading books and lots of new snacks.  During the 4 hours she had several seizures of varying degree.  She had 2 large (45 secs) seizures, a small (15 secs) seizure, and several flickers.  Each time a seizure started, we had to push a button in order for the machine to mark when the seizure became visible.  This would then be reviewed when the VEEG was to be reviewed by the doctors.  

After the 4 hours were over, the technician went through the grueling steps to remove all of the electrodes from Grace.  This was the only point of the day which she got upset.  She actually cried, but who wouldn't after such a long day.

We'd have to wait a week until we'd get the results of the VEEG.  

We also began a new anti-seizure medication (Tegretol) today.  It was apparent the Keppra was not successful in eliminating the seizures.  We're hoping the addition of Tegretol will have a better impact.

[December 20, 2005]

Wow!  I can't believe it's already Christmas, Sarah is two months old and Grace will be TWO in four DAYS!!!

 What a year it has been and I can't believe how fast the last two months went by.  It's been busy with the two girls.  They are keeping us on our toes and me away from the computer and updating the website.  :)  

 Here's the latest on our sweet little Grace.

We started anti-seizure medication on October 14th.  Initially, we saw a slight decrease in the seizure activity, however she was still having 5-6 per day, so after 2 weeks on the initial dosage the doctors increased the dosage.   We've had to go through several increases in dosage over the past 2 months as we try to find the dosage that will work for Grace.  As of now the dosage can only be increased on more time before we reach the maximum dosage for her size/weight.  Although we are praying this medication will kick in and eliminate the seizures completely, I realistically think that we aren't going to be that lucky.  We'll have to wait and see and most likely will be trying other anti-seizure medications over the next few months.  

Grace is currently scheduled to have a Video EEG next month.  This EEG is intended to help them analyze the seizure activity over a three hour period.  As we understand it, the primary purpose of this EEG is to validate the location of the current seizures and eliminate the possibility that they are occurring in places other than the location of the HH.

[October 4, 2005]

It's been awhile since the last update on Grace. 

Grace had her 2nd MRI last month.  This was done to ensure the tumor (hypothalamic hamartoma) hadn't grown.  We received the results immediately and are happy to say that it is NOT growing.  This is great news!! 

Unfortunately, since our return from Phoenix, Grace has been having gelastic
("laughing") seizures.   They've increased from 3/day to 7/day.  These seizures are different then the two week episode of seizures we saw in May. These seizures seem to be longer and typically include a very distinguishable laugh at the end of the seizure.   We've found that about 1/3 of the seizures
are triggered by her surroundings.  For example, if she gets startled this will 'trigger' a seizure.  Good news is these seizures don't appear to be causing any behavior problems or interfering with her development.  If this occurs then we'd need to consider surgery sooner than later. 

We've been in contact with both BNI and our local Neurologist.  Both recommend we start trying medications to see if we can reduce the seizure activity.  We'll be starting with a medication called Keppra and have been told with 7-10 days should be able to tell if it's helping.  Although we are trying to remain optimistic this medication will help, we have found through researching other cases that these types of seizures don't tend to respond to epileptic seizure medications.  But we will pray this isn't the case for Grace. Grace is doing great through all of this.  She amazes us everyday!  She is such a strong and happy girl through all that she's gone through during the past several months.  She's much stronger than her parents.  

Grace has been featured on a special website dedicated to the many people living with various types of brain tumors.  You can see her little face at: http://www.braintrust.org/octfeature.htm

Thanks so much for all the thoughts and prayers and please continue to send them Grace's way. 

Much Love, Erica, Perry and Grace

[August 22, 2005]

[June 16, 2005]

[June 8, 2005]

We've had a couple more busy weeks.

- Grace went to see the Opthalmologist and he inidicated everything looks
good and she doesn't have any apparant vision impairments due to the
hamartoma. We'll be seeing him again in 6 months for a follow up.

- Grace started the Lupron treatments last week. Yikes...the injection is
huge. My poor little girl. Grace cried while the shot was administered but
was able to give the 'mean' nurse a smile once it was over.

- Report from the Barrow Neurological Institute (BNI) came back yesterday.
This is where we sent all of Grace's records to so we could get a 2nd
opinion from their team of doctors. Good news is they also do NOT recommend
surgery until seizures are present. However, they noted that her hamartoma
is one of the largests they've seen and they are VERY surpised that she
isn't currently having seizures as a result of the size alone. They've
recommended the following:

--- BNI Team highly recommends Grace see a Neurologist as she hasn't seen
one yet. They want someone that specializes in seizures to evaluate her to
make sure she indeed isn't having minor ones that we aren't picking up on.
It's crazy. These seizures are called gelastic seizures and are described
as inappropriate laughing, which of course is difficult to determine with a
16mth old. The concern is these seizures can progress into different types
of seizures (ex. epileptic type) so it's important to catch them early. I'm
currently trying to find a doctor to take her to.

--- BNI Team recommends a Development Assessment - Grace currently is on
track for a 16mth old, based on eval by our Pediatrician. However, BNI
folks indicated children with HH tend to develop slower than normal, so they
recommend getting an eval done now so we have a baseline to compare to in
the future. I'm hoping my pediatrician can do this or recommend someone in
Redding that can.

- Trip to BNI - we are still planning on taking a trip to BNI in Phoenix,
but this is postponed until she's evaluated by the Neurologist, which can
hopefully be arranged for sometime this month.

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