The Websters

When Grace was initial diagnosed, the information on HH was limited and when you did an internet search on hypothalamic hamartomas it returned less than 10 results.  We had been told the condition was rare, but it wasn’t until we began our search for answers did reality hit us.

I’m happy to announce that we’ve officially launched a nonprofit specifically for HH.  Below are the details from our release announcement.

We are pleased to announce the launch of  Hope for Hypothalamic Hamartomas (Hope for HH), a 501©3 non-profit organization and website  (www.hopeforhh.org) dedicated to providing information, support, and research data to individuals with hypothalamic hamartomas (HH).

Hope for HH was founded by Lisa Soeby, Ilene Miller, Dawn Cerven, and Erica Webster - 4 dedicated parents of children with HH.  Collectively, we bring professional expertise in business, non-profit management, website design, and social services.  We also have the support and blessing of an international community of HH doctors and researchers and will be adding a Medical Advisory Board to our organization later this year.  Hope for HH will provide:

*Information about HH symptoms, diagnosis, treatments, and specialized centers;
*Tips for living with HH, and education resources;
*HH research updates and articles, books, news stories, helpful links; and
*Interactive forum for HH patients, caregivers, and healthcare providers to dialogue
As HH parents and caregivers, we hope you will VISIT THE WEBSITE, PARTICIPATE ON THE FORUMS, JOIN US AS AN ACTIVE MEMBER OF THIS COMMUNITY, SPREAD THE WORD ABOUT THIS RESOURCE, and MAKE A DONATION. Mostly, we hope you will help us DRIVE and DIRECT this organization to be the best possible resource for our community.

We are forever grateful to the many families that have given so much to see that the concerns of this community have been heard in the past.  Through their tireless efforts, and the incredible dedication of many doctors that have heard our pleas - there have been many amazing advancements in treatment options and the understanding of the complex nature of our kids.  It is our hope that TOGETHER we can continue to make a difference for our family members living with HH and those yet to be diagnosed.

Perry and I are so appreciative for all the support over the past 5 years.  Your support truly helped give us the strength to get Grace through all the ups and downs associated with HH.

As many of you know, Grace had been seizure and rage free for nearly 2 years after her brain surgery, but unfortunately in August 2009 she began to experience episodes which appears to be morphed forms of the pre-surgery gelastic seizures and rage issues.  No one really knows what the future holds for Grace or any of the other HH kids, but our hope is with this new nonprofit we can make a difference in the life of all HH children, including our Grace.

Thanks so much and we hope you visit the website (www.hopeforhh.org).
With Love,

Erica and Perry

It took a bit longer than we had hoped, but we’ve received Grace’s VEEG results. After 50+ hrs of being hooked up and us witnessing over 12 *episodes*, the EEG is inconclusive.

The good news is no seizures showed up on the EEG, the bad news is no seizures showed up on the EEG.

No seizures showing up means Grace is not having the complex partial seizures that she had pre-op. This is truly fantastic news, but unfortunately both our local doctor and the neurologist in Phoenix did see the *episodes* while watching the video images. The problem is there was no corresponding seizure activity captured, so as a result the VEEG as a whole is inconclusive. These episodes could be caused by many things, including seizure activity (too deep in the brain to register on the EEG), hypothalamic rages, or a result of the traumatic brain injury as a result of the invasive surgery.

It’s almost as we are back to those initial days after Grace was diagnosed when we were trying to make sense of it all. Once again we don’t have a clear understanding of what is causing these *episodes*. I truly believe Grace has started to have gelastic seizures, but they’ve changed from what we typically saw pre-op. She now simply has moments she ‘checks out’ for a minute or 2 and then is very disoriented afterwards. She’s also having the more aggressive outbursts which bring back memories of the rage attacks.

Grace is now back on Keppra. She’s actually taking more than she did pre-op and it seems to be controlling almost all of the rage episodes, however, she continues to have the other type of episodes when she ‘checks out’. We are hoping right now that the Keppra continues to work, but realize based on our previous experience that we’ll probably have to modify meds at some point.

Tomorrow, we leave for Sacramento so Grace can have the Supprelin Implant put in on Thursday morning. This will eliminate the monthly Lupron injections, which causes Grace so much anxiety and days of leg pain. We have also noticed that Grace has more *episodes” right before and after her injection.  We think this is due to shifts in her hormonal balance. The implant should reduce hormonal mood swings associated with the Lupron injection and subsequently we are hoping that Grace will no longer have the increase in episodes she’d experience every 28 days. The procedure is fairly quick but Grace will be sedated. Please keep her in your prayers.

It has been awhile since our last update and with the recent events we wanted to make sure all of you knew what was going on with our little Grace.

We had weaned her off 1 of her 2 anti-seizure meds last November and it went extremely well. We decided to wait a couple of months and then began to wean her slowly off the last anti-seizure medication in late December of last year. By June she was down from 3.5ml 2x per day to 1.5ml 2x per day when we began to an increase in Grace acting out and subsequent “timeouts”. At the time we accounted this behavior to the fact that less meds in her system were allowing her to feel the full extent of emotions which she’s really never had the opportunity to feel before or after surgery. Prior to surgery these extreme emotions (happiness, stress, startling, anger) would typically result in a seizure and since the HH was removed and not acting as a catalyst for the gelastic seizures and subsequent partial complex seizures the anti-seizure meds have been helping Grace control her emotions and keep them within ‘normal’ range.

We chalked these new episodes to normal behavioral issues children go through growing up but that Grace never experienced because of all the past seizure activity.

In mid-July we completely eliminated the anti-seizure medication and things got completely out of control. Grace was having aggressive and violent outburst. She was constantly trying to strike anyone near her or throw items. Meal time was especially difficult. Once she finished her meal she would try to knock everything off the table to floor. These episodes would escalate to the point timeouts were no longer working and when Grace was sent to her room she’d do her best to completely destroy it by pulling out the drawers in the dresser, knocking things off the shelves, etc. About 1.5 weeks into this we called the neurologist and asked if this change in her personality could be the result of having withdrawals from the anti-seizure med. He said it wasn’t a withdrawal issue, but more likely sub-clinical seizures or behavioral issues. He agreed we should put her back on the med and see if things improved. Within 2 days things were dramatically better. Grace was still having these episodes, but they were much more controllable and no longer escalated beyond the point of return.

Grace started kindergarten on August 17^th. The week leading up to the first day we had a meeting with her IEP team at the school and let them know about the aggression we were seeing in Grace. We wanted to make sure everyone was on the same page and we had a plan for addressing any issues that might happen while she was at school. Of course, everyone was hoping Grace wouldn’t show any of these behaviors at school, but the first day of school proved us all wrong. She had several of these episodes which involved hitting, aides and classmates. That night I talked with a past teacher of Grace’s who had observed her during the school day and had been on the receiving end of her attacks. The teacher told me what she witnessed was not behavioral issues and that Grace was not in control. She said it looked very familiar to epileptic episodes of rage she’s seen before. After talking with the teacher I immediately called the neurologist and told him what had happened. He said it definitely sounds like there could be seizure activity going on and told us to put her back to the full dosage of the anti-seizure medication and plan on coming down to Sacramento for a 3 day 24hr video EEG in attempt to verify what Grace is experiencing and to ensure the medications she’s taking are correct. The good news is the increase of meds has definitely helped. It hasn’t completely eliminated the episodes, but as Grace’s teachers have said it’s like night and day from the first day of school.

We have come to believe that these episodes are 20% behavioral and 80% some form of seizure or rage attack like she had prior to surgery. They come on, usually, w/o warning. This seems to leave her very confused, sad and crying. The house has been walking on eggshells not knowing when she will go off. This is definitely not our sweet little Grace and we’re very anxious to find out what is going on and how we can help her.

This has been a very hard update to write since our news is not good news. We knew there were no guarantees for a completely seizure free life after surgery, so we’re doing our best to take each day as it comes.

Please keep our Grace in your prayers and we will post another update when we have some more news.

It’s so hard to believe, but it’s true!  Grace is officially 2 years post op and continues to be seizure free.  This is a huge milestone for her and moves us much closer to our hopes that she’ll be seizure free for the rest of her life.  We wanted to share this celebration with all of you that have been there supporting Perry and I and praying for Grace throughout her journey.

We weren’t sure what to post but I thought it might be interesting to look back at the day of her surgery  by reviewing some of the first Care Page post and see how far she’s come just 2 years afterward.

April 20th, 2007 - The day of surgery

1st post from April 20th:

We had an early start this morning. Check-in was at 5am and amazingly we
were early (combination of 2 alarms, 1 wake-up call). We met at 7am with
the anesthesiologist and at 8am Grace was taken back to the Interoperative
operating room to be prepped for the MRI and then for surgery.

Actual surgery got started at 11:00am and at 1:30pm we got an update that
tumor removal had started. We’ll be getting another updated in 2hrs. At
that time we are expecting them to do another MRI and then we’ll have to
wait until the doctor can review and determine if he goes back for more or
has gotten at much as he safely can.

Internet access isn’t as readily available as we had hoped, but will
continue to post updates as we can.

Perry and I want to thank everyone for all the support provided in the
months leading up to this day and for the prayers you’re continuing to do
for Grace today.

2nd post from April 20th:

We got another update at 2:30pm. Dr.Rekate came out to let us know he was
finished with the initial phase. He said everything so far was going great
and Grace would be going in for another MRI. He’ll then review the results
to determine if he will go in and try to get more of the tumor or close
up. We should know in another 1.5hrs or so.

3rd post from April 20th:

We just saw Dr. Rekate and the secondary MRI is indicating 90-95%
resection of the tumor. He mentioned multiple times the surgery had gone
better than expected, but also stated the next several days would be
critical.

Grace’s surgery also involved Dr.Spetzler the Head of Neurosurgery which,
according to Dr. Rekate, assisted with several difficult areas near blood
vessels. We were also able to speak with Dr.Spetzler separately. His
assessment of the procedure was as optimistic as Dr. Rekate’s but also
restated the next few days will be critical.

We are so happy surgery went so well, but now we move on to the initial
recovery. Grace was under for more than 8hrs, so they are expecting her to
be asleep for majority of the next several days and we won’t know the
success of the surgery until she wakes. At that point we’ll need to watch
for seizures and if she suffered any complications from surgery.

She’s currently in recovery and hopefully we will be able to see her soon.
She will hopefully be moved to PICU in the next two hours. We’ll post more
once she’s settled in.

Please continue to keep Grace in your prayers as the next several days
will be very critical for her recovery.

(Sarah is doing great on this trip but is missing here sister very much
and a little out of sorts being away from home. Hopefully we will be able
to see her tomorrow)

April 20th, 2009 - 2 yrs post-op!!!

April 20th, 2007 was the longest and most stressful days Perry and I have undergone.  As you all know the stress and worry didn’t end that day.  Grace spent more than 60 days in the hospitals and neuro-rehab.  The magnitude of the difficulties she faced during recovery was nothing we had prepared for and truly stumped the medical teams.  But she made it through and continues to amaze us with her ability to meet the daily challenges with a smile for her family, therapist, teachers and school mates.

Several of the medical staff still read our updates and to them we’d like to say thank you again for everything you did for Grace.  I won’t identify you by name, but you know who you are! 

Here are two photos 1 day after surgery.   Looking back at these brings back so many memories and reminds us at how little and young she was at the time of the surgery.

I recently took this photo of Grace and think it really is a great example of how far she’s come since being released from the hospital.  When she came home in June 2007 after those 60 plus days in the hospital, she could stand or walk for more than 10 minutes at a time, now she’s standing on one foot and kicking the other one out to the side.  Simply amazing!!

Has it really been over a month since we’ve posted?  Unbelievable how time flies!  We have 2 things for you.  The first is an update on our sweet Grace and the second is a request for all of you to help Perry and I raise money for Wings of Angels (see the end of this post to see how you can help us help).

We were in Sacramento January 23rd - 26th to meet with two of Grace’s specialists (endocrinologist & neurologist) and to have a new Physical Therapy assessment completed.

The appointment with the endocrinologist went well and he was amazing at how tall Grace has gotten since our visit last August/September.  She grew an inch and a half!  He’s made some modifications to her DDAVP dosages as we had a hiccup in our ‘normalcy’ when Grace’s sodium level jumped to 165.  This is after months of everything moving along so smoothly, but hey, it’s DI, so we’ve learned by past experience, that just when you think you have it figured it out that’s when it either drops or spikes.  Anyways, the adjustments to the morning dose seems to have leveled things out again and we’ve reached ‘normalcy’ again.  Next steps are for a full blood work up to check her hormone levels and thyroid to make sure her current doses of meds is still appropriate.

The appointment with the neurologist went great.  We received the okay to begin weaning her off the last of the anti-seizure medications.  She’s been off Tegretol since Thanksgiving and we’ve really noticed an increase in energy, balance and in her expressive speech.  Needless to say were are excited to begin the weaning of Kepra and how getting her off of it will improve other areas of her development.  We are doing this weaning very slowly as we don’t want to take any chances that residual seizure activity takes place.  We’ll keep you posted on this as we progress.

We’re working with the school on providing services to Grace to make her successful in the school environment.  She’s currently receiving speech, OT, adaptive PE from the school.  There is a dispute regarding physical therapy needs and as result we had an outside group assess Grace.  Let’s just say the results from the school are completely out of sync with the assessment we had done privately.  So, we’ll be meeting with the school again to figure out next steps.  Wish us luck!

Now here’s where we are asking all of you that have been so supportive to us during the past 4yrs to help us help Wings of Angels.  For those of you not familiar with them all I can say is they have gone beyond the call of duty to help our family and other families with children suffering from life threatening illnesses.  Last year was our first chance to start giving back to them and to pass on the support to other families.  Last year our team, which includes Perry’s parents, succeeding in raising nearly $4,000.  Our team was 2nd in amount of funds raised.  We’d like to match or surpass that for this years event on April 4th, 2009.  We need you to make this happen!!

1.  All donations are tax deductible

2.  Two ways to donate (make sure to include ‘Team Webster’ in your donations):

- Make Check Payable to ‘Wings of Angels’ and note ‘For Team Webster’ and mail to

The Websters, 18962 River Ranch Rd. Anderson, CA 96007

- Go to Online Donation (click here) to make a donation via Paypal. Make sure to enter ‘For Team Webster’.

We realize this is a tough time of year for everyone, but please realize there are several children in our area that are undergoing painful medical treatments and the support they get from Wings of Angels helps them make these treatments possible.

Last but not least here are some recent pictures of the Webster crew:

Thanks again for everyday of prayers and support!