Ketogenic Diet and an Amazing Gift

Yesterday, after waiting a few weeks for the appointment, we finally met with the Ketogenic Diet "Team" at CHOC.  We had already decided this was the route we wanted to go and  had labs taken while we were inpatient a few weeks ago just so we'd be a few steps ahead in the process.

I've mentioned that I'm not 100% sold on the Sabril.  Perhaps it's because when Riley was on the ACTH her IS's went away, completely.  On the Sabril she'll still have a few a day but, they are less frequent, not as lengthy and not as severe.  So I suppose this is a small victory in the grand scheme of things but, long term, we need a better solution for these awful things.

When I say we met with a "Team", I'm not kidding.  I was expecting a few people in the room.  When the door opened  in walked a pharmacist, dietitian, nutritionist, social worker, psychologist, therapist and NP.  I only recognized one so this was a new audience to talk to about Riley.  I also always feel a bit of anxiety when there are so many people grilling me with questions.  I even commented to them that it reminded me of the days in the PICU during morning rounds.

After a series of questions the NP looked Riley over and Riley was a rockstar.  We had taken her "ear" off because Riley is now startling to loud noises. We think this is HUGE for Riley as it must mean she's hearing the loud sound and knowing she's supposed to have a startle reaction.  She has only started doing this recently and when we both had the same jumpy reaction to a sound in the neuro waiting room yesterday it made me smile.  Perhaps her sense to sound really is reconnecting?  Riley followed the NP's light when she tracked her eyes, reacted to her when she tested her reflexes and moved her head towards the NP when asked.  This was a good exam and make me think Riley liked her.  If Riley doesn't like someone examining her she basically plays possum.  Riley is much smarter and aware of her surroundings then most give her credit for.

After our exam and discussions Riley's doctor, Dr. Tran, came in to discuss moving forward.  I had a list of questions in my head and was trying to make sure I got them all in before Dr. Tran was done!  I feel confident that we did address everything and that we have a plan in place.  We're on the same page in regards to the number of meds Riley is on and she knows what our expectations are as Riley's parents.

Riley is approved for the Keto Diet (almost).  We're waiting on 2 more labs (see why it was so great to get these going early) and as long as those look good we'll prepare to go inpatient to switch over to the Keto Diet.

We're looking at a date in July and a date in August and we'll be back inpatient for 4 or 5 days as they figure out the right formula for Riley and switch her meds to ones that are "keto" friendly.

I'm actually excited about this hospital stay and cautiously optimistic about what the keto diet can do for Riley long term.  We'll let you know once they give us the final go ahead which I am hoping will be in the next couple weeks.

I also want to share with you all  ( sorry for the repeat if you're my fb friend) something so wonderful that is being given to Riley.  And a bit about the family that is making this possible.

One of the things I least expected when Riley got sick was to be so touched by others who had medically fragile children and their stories.  I also never expected to be so overwhelmed with the generosity, concern, care and support that we as a family have received.  To this day  we'll receive a gift or a message and it touches me the same way it did day 1 of Riley's journey.

I "met" Patrick's mom shortly before Riley was to start ACTH.  I had known Patrick's Aunt for a few years and she put us in touch because I had so many questions and concerns about the ACTH.  Stef had lost her angel baby Patrick and was still so open to replying to my emails and giving me information that I needed to be prepared.  I think she gave me the most realistic explanation of what ACTH would do to Riley.  Prepared the most for what was to come.

I'm going to share a video from Stef's  facebook page that tells their story and shows what an amazing family Patrick has here on earth.  They truly are incredible.

Team Patrick

It's hard to watch without tears because they describe what our life felt like when Riley was first diagnosed and yet they don't have their sweet baby to love on today.

This Saturday it will be 2 years since Patrick passed away.  He was 27 months old.  I was thinking about this last night and Riley will be 28 months old on Patrick's Angelversary.  Gave me the chills when I really started to think about Patrick and his family.  They decided to honor this day by gifting Patrick's adaptive medical equipment.  If you don't know equipment like Riley's stander is expensive and rarely covered by insurance.  Ridiculous.

When I saw that they had an adaptive stroller available I emailed Stef. We've wanted this exact stroller for so long but, didn't think that we could justify the expense as we will need a wheelchair in a few years(possibly next year) and we needed to save our medical coverage for the day that we pulled the trigger on the wheelchair.  Well they picked Riley and now, again, for the millionth time in the last two days I am getting teary.  Being that they live in WI I was doubtful that they would pick Riley.

From Stefanie Gerberding's FB page.

  "So many things with Patrick felt meant to be and, honestly, a bit magical. We are feeling that magic with finding homes for Patrick's adaptive equipment. Riley is a beautiful girl in California with Infantile Spasms, like Patrick. Her mom, Megan Hopper, and I have talked a few times as my sister put the two of us in touch. They have participated in Team Patrick fundraisers even. Turns out the adaptive stroller is something they have been searching for and need! They will be flying out to pick up the stroller because they want to thank us in-person...doesn't that tell you how amazing they are?! We are so thankful Patrick's stroller is going to Riley. They are giving us the best gift of letting us share Patrick's memory and legacy. Please go and "like" Team Riley!"

I am blown away by the generosity and kindness that The Gerberding's have.  So, after a quick chat with my mom and Dave I decided to go pick up this stroller myself.  Shipping versus plane ticket is not that big of a difference and my heart is telling me that I must give Stef and Tim a hug in person to say thanks.  I know that parting with these items can not be easy for them.

So I am sharing this today because it is my hope that you will think about the Gerberding family tomorrow.  Whether you say a prayer for them to get through the day, just think about Patrick and the impact he had on all that knew him or do your own random act of kindness in his honor.  We, as a family will be keeping  Patrick's family in our prayers and will be finding ways to pay it forward in Patrick's memory tomorrow.  And soon, hopefully in a few weeks, I hope to be meeting Stef and Tim in person and giving them a gigantic hug from Team Riley.  We're just blown away by this amazing family and their incredible generosity.

Riley can not wait to use Patrick's adaptive stroller and feel his love through this amazing device that will allow us to include Riley in more of our daily activities.

People are good.  These special children that are placed in our lives can teach us more about humanity then most adults can.  Acts of kindness really do make an impact.  If you doubt what a simple act of kindness can do for one person I hope your doubts are calmed by Patrick's memory and the impact his family is having on so many.

As a family we will continue to pay it forward in Patrick's honor.  I hope you will too.

Fight On!

Love,
Dave, Megan, Logan, Mason and Courageous Riley

Decisons

Having to make big decisions for our kids (all 3 of them)  is probably one of the most stressful things we've had to do as parents.  I remember the angst we felt (well more me then Dave) last year about whether or not to send Logan to Kindergarten or wait another year.  And Logan did great, exceeded our expectations and reassured us along the way that we didn't mess up the rest of his life (at least for now :))  I know as we navigate parenthood there will be tons of other decisions we need to make and I am sure it won't get easier.

For Riley we need to make decisions that scare me sometimes.  It's the same type of anxiety I had when trying to decide to send Logan to K but, different.  I am not an MD.  I was never in medical school.  My hospital experience before Riley got sick was working with nurses to help cover them with insurance policies. So yeah not exactly a hands on medical background.

As time has progressed and we've started to add words to our vocabulary that I never knew existed we're learning that our decisions on behalf of Riley are as important as the input we receive from her doctors.  Now we always go in to these situations with as much information as possible but, having the ability to use the words we've learned to work through scenarios with Riley's doctors makes us feel as if we are doing everything we can for Riley.  We have to go with our gut though in most instances because again "we're only her parents".

Thursday we will be back at Neurology.  This time in the Keto Clinic.  We will turn in our zillion page questionnaire about what Riley eats (remember everything is via gtube so the paperwork has had me rolling my eyes) we'll meet with the nurses and dietitian and we're making a decision to put Riley on the Ketogenic Diet.  It's what my gut is telling me that we need to do for Riley.  It's also something her doctors agree to.

Unlike other families who have children on the Keto Diet we are lucky, if you can even say that, in the sense that Riley is 100% gtube fed so we won't need to adjust her diet or what she eats but, just change formulas again.  Here's to hoping the keto formula is nicer on Riley's digestive system then the one we are on now!

We also hope to discuss taking Riley off the Sabril.  We had our first appointment last week with the Ophthalmologist and although everything looks perfect in regards to the health of Riley's eyes, I don't believe Sabril is doing what we were hoping and I don't want this to be a med that is given on top of other meds if it is not helping. Remember a lot of children end up on a handful of seizure meds because the doctors just keep trying and adding and don't take away.  So we'll see where that discussion ends up.  I have a feeling though that they won't want to go this route.  Perhaps it's the pessimist in me.

The good, although frustrating news is as I mentioned everything looks great in regards to the health of Riley's eyes.  It's her brain that struggles to help her see. (which we've known since day 1)  I was skeptical when we first arrived at this particular doctors office about what his demeanor would be in regards to Riley.  His encouraging words as we left about the health of her eyes and his use of the term we've come to know well "plasticity" of the brain reassured me that we are doing all that we can to help Riley.  She has all the "equipment" so to speak it's just a matter of continually working with her and for her to see what she will be able to do.  We'll see this doctor again for another exam in September.

Riley has been doing well given all that is going on.  She is amazingly strong and brave.  We had to take her to the pediatrician last week for a couple of shots and she took them like a champ.  At first I think she was having ACTH injection flashbacks but, she didn't even cry.  Just held my hand and whimpered a bit. Logan can hardly believe that his sissy was so brave.  He could use some of her strength for his shots!  We were able to have lunch at my parents house after which made for a nice afternoon.

Summer is definitely here.  We had a wonderful time this weekend swimming a ton and  Logan loved having Tobin and Uncle Scott over to swim on Saturday.

I messed up registering the boys for VBS  this week so we're spending a lot of time together.  And really it is probably a blessing in disguise.  Dave also happens to be out of town until Friday so I am just hoping we make it until then! And yes I know we will.  We have such an incredible support system and "back up" team in place in case anything goes wrong and at the end of the day I am thankful for that.

Just today I met a grandmother whose daughter had spoken to my mom at the park before.  She noticed Riley's suction bag (it's hard to miss if you know what it is) and we struck up a lengthy conversation about her grandson who got "sick" at 9 1/2 months old and has "S" words and also has a gtube and trach.  It's funny and may sound silly but, the decision to go to the park was pretty last minute but, was what I needed.  Meeting this grandmother and hearing her story and the struggles her daughter goes through (as a single mother) with a child very similar to Riley (although he is 7) was the kick in the booty I needed to see that although some days are rough, others have it much harder and do this without the kind of amazing circle we have surrounding us.  I've learned that you just never know when these moments will be placed in your life and can't stop thinking about it.  I also hope to meet this mom/son one day as I am pretty sure we'd have a ton to chat about!

Because of our amazing support system, the kids were able to spend some time with Grandpa and Grandma Hopper yesterday while I attended a funeral and I think I can speak for everyone that they had a nice time together.  Spending time with the Grandparents while mom and dad are not around really is good for everyone!

We hope your summer is going well!  Hard to believe the 4th is just next week.  We're living in the moment and taking advantage of time together before school starts again!  In between the craziness that is naturally our life,  Riley is working hard at her therapies and should start ST again in a couple weeks. She's also continuing to prove to us that hope, faith and love really can get you through!

Fight On! and Go Team USA!

Love,
Dave, Megan, Logan, Mason and Courageous Riley

An update of sorts!

First, I am sorry for our lack of an update.  Life. Is. Crazy.  And I'm not sure this update will provide many answers.

Sunday, finally, for the first time in 2? 3? 4? weeks I finally really had a chance to sit down and let you all in on the craziness that is our life.  Dave and Uncle Scott took the boys to the beach and Riley had  her normal lazy Sunday, which always involves sleeping in.  She's a lot like her mama in the sleep department. Much like most of you, we've been running around like crazy people with end of the school year activities and just life in general.  And my delay in posting may also be a result of me trying to finish OITNB as fast as I could!

We've had birthday parties, tball games, Kindergarten pictures/parties, activities, CHOC Walk awards, Kindergarten graduation and celebration, and a fun wedding!  We've also moved out of our office in hopes to find something more suitable for what we need (which means our garage and home office are overflowing with "stuff").  We snuck away for a couple of days of grown up time which was good for our marriage and good for my soul. (7 hours on a lake with no cell service is amazingly cathartic)  We've had doctors appointments, therapy appointments, insurance fiascos, evaluations and decisions to make regarding Riley's care.  We've also tried to keep everything balanced and we're working hard to continue to make sure our boys are happy and don't feel forgotten or less of an importance to us as their mom and dad.  Through all of this there has also been an elephant in the room.  A fear.  A rock in our stomachs that makes it really hard to go about each day.

Last week I finally decided to just roll with everything going on and stop worrying about what I can't control.  You know the teacher gifts that never made it in to the teachers hands.  The classmates birthday party that slipped my mind until it was over.  The fact that Logan missed closing day for Tball because before we left out of town I forgot to mention it to my mom.  I had to clear my mind so that we would be ready for Riley's VEEG.  We had tons of questions.  We needed a plan.  I had to make sure that the new team we'd be meeting knew where we stood.  Knew what our feelings were in regards to Riley's care and knew that we wanted to be able to work together for the common good of Riley. They also had to know that we meant business.  I'm finding that more and more children that have a diagnosis like Riley's have doctors and family members often "give up" on them.  Not all of them but, the stories I hear make me sad.  We will never stop fighting for our little miss.

The elephant in the room is the return of Riley's Infantile Spasms. (They have a new name btw but, I'll get to that in a sec.

Riley is proving that she isn't a textbook case.  She's going to make the doctors work to figure this all out.  Even her audiologist seems to have a hard time working with Riley as she isn't the typical CI patient or more obviously a typical 2 year old.  It's not her fault.  Riley is a bit complicated.  But, as her parent's we will make sure we can develop a "Team" willing to work with us and work in the best interest of Riley.

Riley started Sabril (pronounced Say bril) on 5/22.  Her VEEG was 2 weeks after starting it and although we'd seen some improvements in her spasms they are still around.  I had a nice long chat with Riley's now old neurologist before starting the Sabril, about our concerns, about other options etc.  If, it doesn't start doing it's job soon we're going to need to move on.  We also do not love the idea that one of the main side effects is peripheral vision loss.  In fact this is the one that keeps me up at night.  He reassured me then that there was a very low chance of this happening but, when your child's vision is mainly peripheral to begin with it't a tough chance to take.  So we started the Sabril and hoped for the best.  We have an appointment with a new Opthamologist next week and we'll be tracking Riley as she continues on Sabril.

Friday morning, after being on the monitor all night we met with the doctors.  Dave had spoken to a few of them the night before but, this was the chance for us to really put a plan in place.

It was a team of doctors which included a few of the newer residents who just stand there, most likely told not to say a word, the floor pediatrician, a neuro fellow (who has been around since the PICU days and had very little to say positively about Riley then, wait until you hear what Riley did for her :)), and our new epileptologist,  We're impressed with her so far and she seems to know her stuff.  She also is very open to constant communications in regards to Riley.  If we don't like something and want to change it up she's open to that.  Most importantly though she looks at Riley like she is a person.  She talks to us, as Riley's parents, like we are human.  She's open to our ideas.  If I ever have to give another family advice on picking doctors those 3 things would be at the top of my criteria list.

We started the chat with the fact that Riley's EEG is still abnormal.  Her spasms are still there.  And for funsies my poor child's constipation issues are also showing up on her EEG as she's using all of her muscles to go.  Don't worry we consulted GI while inpatient and got Riley something to help with that!

Is Sabril working:? Will it work?  Really we don't know.  Over the next couple of weeks we will watch her (at home), keep track of how often her spasms occur and see if by giving it a few more weeks at a higher dose it will have a chance to do it's job.  As the days tick by my faith in this new med actually working is diminishing.

I asked again about why the are still called IS's and if Riley will grow out of them.  They have a new name.  Eplieptic Spasms.  And, if left untreated, can turn in to tonic seizures.  For the first time in the last year and a half it actually hit me that Riley's epilepsy has the possibility of taking her from us at some point.  We are playing with fire in regards to the possibility that if we can't get these under control they can develop in to something much worse.  It's scary.

Because we are unsure about the effectiveness of the Sabril we're also looking to switch to the Ketogenic Diet. It's a high fat diet sort of like Atkins.  It is also very restrictive and can have it's own side effects.  To be proactive, Riley had a ton of blood drawn Friday morning and once we have the labs back we'll have a better answer as to whether or not this is something that we can try with Riley.  Our main goal for Riley is to manage her seizures with the least amount of medication possible.  I may be oddly optimistic about the Keto diet but after reading so many success stories it gives me hope.  Ideally, we'd like to look at weaning her off of so many meds.

As I said to the team of doctors Friday morning, I don't want Riley a zombie in life.  I won't let them over medicate her without trying the other options available.  Riley is our fighter who was supposed to be a "vegetable".  Remember the fellow I mentioned who had little faith in Riley in the PICU.  She pulled Riley up by her hands Friday morning and Riley not only lifted her head up for her but held it there.  Take that doctor know it all :)  Dave was there when this exchange occurred and I could hear the excitement in his voice as he told me.  She also tested Riley's reflexes as they do often and they were ALL there.  This doesn't usually happen.  And this is a first for Riley.  We celebrate everything in this house and each and every little thing Riley does is huge for our family.

For now we continue to wait and watch.  We also continue to fight and believe in Riley. I'm sorry we don't have anything more solid to share.  This is just another one of the bumps in the road that we are unfortunately becoming accustomed to.

I don't think that we'll ever get used to this life.  It's ever changing and unpredictable.  One day at a time, together we're doing okay.  All 5 of us.  I promise.

Thank you for your continued prayers, support, messages, calls, texts, friendships and love.  We love all of you!

Fight On!
Love,
Dave, Megan, Logan, Mason and Courageous Riley