Sunshine on a cloudy day...

Shaun's older brothers, Stephen and Scottie, came to visit yesterday afternoon.  One of Denise's friends, Erin Sullivan drove the boys down to UVA from Berryville so that they could visit their brother and spend time with their Mommy.

Denise said that it was wonderful to see them... and terrible.. and fantastic.. and sad... all these emotions rolled into one.  While they were visiting, the boys asked her, "when are you and Shaunie coming home?"  And she had to reply that at this point she doesn't know if Shaun will be coming home... as she put it to her boys "home to play with you, or home in Heaven to play with Sarah".

It is so hard.. not knowing.. not having answers.. waiting.

Still, Stephen and Scott added so much sunshine to the room, not only by their loving presence, but they decorated Shaun's room with pictures that they colored, and made paper "chains of love" with pictures and messages on them.  Stephen wrote on some of his chains, "You are my little champ, Love Stephen", "We love you!", and "You get better"!  Scottie wrote, "Scottie Loves Shaun" and "You are a Super Hero!".

They both loved kissing on Shaun and spending some time with their Mommy.  Really, it was so good for them to come visit.. for them and for Denise (and Shaun!).
Thank-you, Erin, for taking the time to serve our family in this special way!

Shaun "sleeps" on, as we wait, hope, and pray that the new medicine will work (apparently it takes 2-3 days for the load dose of Topamax) and the brain activity will return to normal so that Shaun can be woken up to interact with us again.

A new plan...

Here are the updates from Denise's facebook page today:

New plan for Shaun this morning. We are going to start to ween him off the pentobarbitol as it has long term side effects on the body (especially the heart and edema) and he has been in a "restful" state for a while now. The new meds he is on helped lessen the seizure activity last night, not fully taking it away, but helping. It is time to see what his body tells us, mainly his brain, and we will go from there. This is a multiday process so keep praying for our little fighter!

During the day they were able to back off the pentobarbitol some (not completely) and see some increased activity.  Shaun - and Denise (and our cousin Mike, staying with Denise at the hospital) - enjoyed having many visitors come.. Shaun's Great Grandad Cole, Great Grammy Pat, Great Aunt Karen, Grandad (Denise's dad), Grandmomma, and Aunt Christiana were among those who visited.

Denise, the ever watchful mother, noticed that Shaun would have some more active periods that lined up increased and more prolonged (than the seizures) activity on the EEG.  She inquired about this and so tonight the neurologist came to review Shaun's EEG readings for the entire day.

Denise updated us again tonight on facebook with the new plan based on what the neurologist found:
One step forward and two steps back... Shaun seemed to be doing well until I noticed longer awake periods with scrambles in the wave patterns. I asked the docs what it meant (good or bad) and the news was that they would eventually build into seizures again. So new plan again : keep him at this dose of pentobarbital for now, other meds stay the same and load him with Topamax for the next 2-3 days. Shaun always beats to his own drum and we just need to listen and get on the same rhythm. Pray for us to have a breakthrough soon. Thanks.

As Shaun fights on, we join him in this fight as we pray for this precious life.  Our hope is in the Lord, who is our refuge and strength, an ever-present help in trouble (Psalm 46).  While we cry out to God and implore him on Shaun's behalf, we trust that His will will be done on earth as it is in Heaven.

Thank-you for taking the time to pray and send encouragement to Denise, Peter, and the Steyaert family.

Seizures continue

Today seemed to be fairly uneventful as Shaun is still in the pentobarbital induced coma... completing over 24 hours .  Sadly, the continuous EEG still is recording spikes in Shaun's brain activity which tells us that his brain is still having seizure activity instead of the intended rest - despite his calm exterior. This is not good news.

For tonight, the doctors are going to maintain the induced coma, continuing to give him the max dose of Keppra, and adding Vitamin B6 and Lacosamide to try to stop the seizures. They will re-evaluate in the morning depending on how things go tonight.

Because of Shaun's unstable state, they have not been able to take him for any scans of his brain, and are yet to have anything definitive as to why this has occurred.  The only possible explanation given at this time is disease progression.

So Shaun continues to "sleep" as we pray that this seizure activity stops and the doctors have wisdom in his treatment.

Seizures

"Today was a terrible day..." This was one of the first things that Denise told me on the phone tonight.

The day started with a fever, which is not completely uncommon for Shaun... but then while the Hospice nurse was visiting in the morning, Shaun started having grand mal seizures.  Denise and the nurse maxed Shaun out on the home meds he could have but was unable to get control of his seizures, so they called 911 (per Dr advice). The paramedics also were unable to control the seizures, so they took Shaun to Winchester hospital.  The doctors at Winchester tried as well with anti-convuslants, but were unsuccessful in controlling Shaun's seizures and therefore he took a life-flight to UVA, with his anxious parents driving as quickly as possible to get to Charlottesville.  Shaun was taken to the PICU at UVA.  After some time of unsuccessfully stopping these seizures (he was knocked out on a sedative but every time he woke up he would start seizing again), the doctors decided Shaun needed to be put into a medically induced coma with pentobarbital for the next 24-36 hours to allow his brain to rest, and when they wake him up, hopefully the seizures will not reoccur.

Shaun has a continuous EEG monitoring his brainwaves - as they are looking for signs that the anti-convulsives are working.  Pray that they work.

The doctors have not been able to determine why this has happened - Shaun is too unstable to have any scans of his brain done currently since he has been seizing all day.  Pray that the doctors have wisdom to know what course of treatment for Shaun, to determine why this has happened, and to prevent it from reoccurring in the future.

Shaun is on a ventilator, but we need to continue to pray that his oxygenation/CO2 levels remain where they should be.

Shaun's fever returned later in the day, so the doctors are looking for infection.  Pray that his body is able to fight off whatever pathogens may be coming against him!

As we all lift up Shaun, let's remember Denise - who is Shaun's constant companion in the hospital; Peter who went from work to one hospital then to the other and now back home to help with the other boys; Stephen, who is worried about his brother and misses his Mom; and Scottie, who witnessed a scary seizure at home and has a really hard time when Mommy is gone.  For all of them, it is so hard to see Shaun this way, not knowing when his last day will come and fearing that any day could be it.  But for now, life goes on while Shaun is "sleeping" and Denise is trying to rest as best as possible in the hospital. May they hold on to the Lord and be quieted by the Redeemer and Preserver of life.

The Lord is my Shepherd, I shall not want. 

He makes me lie down in green pastures, He leads me beside still waters. 

He restores my soul: He guides me in the paths of righteousness for His name sake.

Though I walk through the valley of the shadow of death, I will fear no evil; for You are will me, Your rod and your staff, they comfort me.  

You prepare a table for me in the presence of my enemies;

You have anointed my head with oil, my cup runs over.

Surely goodness and mercy shall follow me all the days of my life;

And I shall dwell in the house of the Lord forever.  Psalm 23

Today was terrible...bleak...dark.  But God is still an ever-present help, and we trust Him for tomorrow, whatever may come.

Results

We got news that the results of the muscle biopsy came back late last week. On Tuesday Dr. Lins~ Shaun's pediatrician, called us to share some of them with us. It showed that although his enzyme levels were good (CoQ10) and Respiratory Chain Defects (RCD) were not detected, his body is not creating the mitochondria as it should. This is called Mitochondrial DNA Depletion Syndrome (mtDNA DS). His levels should have been around 2000 and Shaun had 943. They don't know what is causing this and want to get some more testing done called exome testing. We have been referred to TGEN who is doing research in rare childhood genetic diseases to do these tests. It is just a blood test requiring 2 viles of blood, so it shouldn't be very invasive. It will not be for a cure but to understand the gene sequencing better and try to find a way to slow the progression down. Shaun has also been put on a modified mitochondrial cocktail  CoQ10 100mG, B-Complex 50 mG, and Alpha-Lipolic Acid 100mG. The other one we are starting is Carnitine 100mG. These are all taken 2 times a day and is a high level to try and help his body out. Pray for him, that his body will produce the mitochondria that it needs to survive, the progression to slow down, he is comfortable, and that our family will keep making precious memories.

A special thank you... (first of many!)

For a year or so now, we have been graced by meeting some wonderful people. They give of their time, talents, knowledge, and self. I want to say thank you to each of these people and let them know what they mean to us and Shaun. Each has touched our lives~ all of us, in a special way.

We met teacher Chelsea last summer after Shaun had to get a G-tube because he wasn't eating well. She came in an introduced herself, saying that she wanted to take Shaun to "school" and give me a much needed break- time for a shower!
Chelsea worked with Shaun every time we were in the hospital. She brought in toys, sang songs, played IPad, and challenged him even when in the PICU or on isolation. She brought joy to both of our lives. Chelsea taught me to challenge Shaun's mind, make picture boards, have him make choices, work on puzzles, sing "Row Row Row your Boat" with an alternate ending. She genuinely cares for "her kids" and wants the best for them. Totally dedicated. Shaun came to life when our "school hour" took place.
This last trip, we found out that Virginia is cutting the program for the 0-2 year olds from UVA. This was very upsetting to me and so I tried to send letters out to different delegates and representatives. This program was vital in helping me learn how to teach Shaun and that he wasn't just "sick," that he was still able to learn in his own way. It deeply saddens my heart that other families will not have the same privilege that I did, having the program and a teacher totally dedicated to her work and "kids."
Shaun decided that he wanted to write Ms. Chelsea a letter thanking her for her kindness, love and support.

Thank you, Chelsea, for all your love and time you share with us. You will always have a place in Shaun's heart and mine.

Row Row Row your boat, gently down the stream. If an alligator comes, don't forget to scream! (Grab your face!)

Sweet Family Moments... Enjoying Shaun

 One of the things on my bucket list is to get a good family picture... This was taken without warning and I am so happy to have it. Hope we can get a picture that we are all dressed for but if not, at least we have a family photo!

The boys love each other so much. It does my heart so well to see how much love they have for one another. I like to say that they make my Heart SING!!

 Shaun is loving playing on his little mat lately. He can pull the birds and rings to make it sing and light up. Its what he spends lots of time doing each day.

Sweet moments with my boy. He is so affectionate, as long as you give him the chance. He loves giving kisses if you will let him. Lately he learned about butterfly kisses and eskimo kisses. These are the moments I hold onto.

 Scottie wanted to be "twins" with his brother. Its so amazing to see the bond between these two boys. Scottie will sit and hold Shaun for hours. It's so sweet!!

 Shaun loves music. Scottie, Shaun and I love to dance to different songs and be silly. One of our favorite is the "cha cha slide". It has easy directions to follow and a fun beat. Otherwise we love dancing to the Wiggles and other kids songs and could do it all afternoon!

Dancing Cheek to Cheek!

Momma and her Boys!!
Mommy Heaven!