January 26, 2016

Seizures, Seizures, Everywhere.....

On the morning of Wednesday, January 13th around 8a.m., we checked in at our local children's hospital for a previously scheduled 24 hr EEG. Since Jeff needed to be at work we arranged for Jeff's dad to come up the night before and stay with Ethan and I at the hospital for part of the day on Wednesday, and for my mom to come up and spend the rest of the day with us at the hospital, until Jeff could join us after he got off of work.

Since this was a previously scheduled EEG, I expected that things should go pretty smoothly, as far as being admitted to the pediatrics floor and getting the procedure started. Ethan has had EEGs in the past and it has never been pretty. He hates to be held down and having 26 electrodes attached to his head is not something he finds enjoyable (at all)! In the past the EEGs have lasted about an hour, and this time it was supposed to last 24 hrs!!!! We knew that this wasn't going to be pleasant. We knew that it was going to be rough. 

Unfortunately, there were lots of barriers to even get the procedure started, and all of us were getting rather irritated about the fact that due to miscommunications and possibly other things between multiple parties, the EEG didn't actually start until after 3p.m..

Ethan cried so much that by the time the tech was finishing attaching the electrodes/leads that he had worn himself completely out. 
Ethan's nap didn't last too long though and then he was up. 

For the most part Ethan did pretty good after he woke up. The wires were long enough that he could get out of the crib, a little, and we could roll everything over to the bathroom, etc. However, since another component of the test was video monitoring him, he did need to spend the majority of the time in the crib. 

Not long after Jeff arrived after his long day at work my mom left and headed back home. Ethan was extremely happy to see his dad, and everything was okay until it was time for Ethan to go to sleep. He was so tired, but the electrodes were a huge issue because when Ethan is tired he rubs his head, so not once but twice we got him almost asleep and then he would rub his head and try to pull off the electrodes. This meant that we had to intervene and then there would be real tears and he'd be worked up and wide awake again. Thankfully, the third time which was after 10p.m. Ethan was asleep for the night and Jeff headed home to sleep and I tried to settle down for the night at the hospital. 

Ethan slept through the night, but was pretty restless. I didn't get much sleep, partially because it is just hard to sleep when your child is in the hospital but also because I kept looking at the EEG monitor (which I have no clue how to read, but it was clear that the seizure activity was much worse while he was sleeping than I was aware of) and worrying and praying for my little boy. The nurses assured me that someone in EEG was monitoring Ethan's test remotely and would immediately contact the nurses if it looked like a seizure was potentially life-threatening so that his rescue medication could be administered. Obviously, we don't have this capability at home, so that made me worry even more.

In the morning we hung out for several more hours, saw the doctors on round and the neurologist checked in on us and said hello and that he'd see us on Friday for our scheduled appointment. After that we were able to start the process of getting discharged.

Ethan was so happy to have all of the electrodes/leads removed.

Ethan remembered the lights from our previous visits. He enjoyed being able to turn them on and off.

I'm ready to go!

Wake me when we get home!
Ethan was very happy to be home. The next morning (Friday) Ethan seemed tired, so I decided I would just keep him home, especially since we had our post EEG neurology appointment at 1pm. The appointment with the neurologist didn't go very well. We knew that the EEG was bad, but we didn't realize how bad it was. Basically, our little boy is having seizure activity almost nonstop, and by the time that we "see" one it is really bad. Also, some of his staring episodes are likely seizures as well, and we had assumed it was just a side effect of the medications he is taking. So, know we don't know when he is staring off and having a seizure or when he is just sort of zoned out. The neurologist also suspects that Ethan may have sleep apnea, which could be causing the seizures to be worse.

The game plan for now is to stay on the same medications and to explore the possible sleep apnea aspect. A sleep study has been scheduled, but isn't until near the end of March (first available) and we are trying to get in to see an ENT.

Prayer Requests

  • An end to the seizures --- that God will either miraculously take them away and/or that the doctors will have the wisdom to know what has to be done so that the seizures can once again be under control. 
  • Get an ENT appointment scheduled and see an ENT before the scheduled sleep study, because the sleep study is most likely going to be even worse than the EEG. We would like to know if Ethan's tonsils and adenoids might need removed, and if so wisdom about surgery.
  • Return of Ethan's balance and independent walking. Ethan is still walking, but because of the seizures and/or the anti-seizure medication he continues to be very unsteady. His walking now compared to what it was like in the fall before the seizure activity increase is dramatically different. 
  • Praise for Ethan's continued toileting success. Pray that he would improve in his ability to be able to communicate his need to go to the bathroom. He is beginning to take my hand and walk me to the the bathroom here at home -- sometimes he goes, sometime he doesn't, but it is a start.
  • Increase in communication. Ethan's communication skills are still very limited. This is frustrating for us as his parents and for him too. I'm sure he thinks that the adults around him are really dumb for not being able to understand him.
  • That all three of us would be able to remain relatively healthy this winter despite all of the illness that seems to be going around. I worry about this a lot because a regular illness can be extremely tough on Ethan and although he seems to really enjoy school, there are so many germs at school!
  • Praise for Jeff's job that has allowed me to stay home so that I can be available for Ethan's many doctor appointments, therapy appointments and when he is sick or having a bad seizure day. Pray that Jeff would be strengthened and have the wisdom and knowledge necessary to continue to do a good job at work.
  • Wisdom about adding to our family someday. The desire for additional children hasn't gone away. Ever since Ethan's diagnosis we have felt that if we were to have additional children that they would come to us through adoption. We have been licensed foster parents for nearly a year and we have not yet said yes to a placement, as there are lots of things to consider. Our desire is for a child under the age of 5, but we would prefer under the age of 3 and to be perfectly honest I would love to be able to adopt a child who we had cared for since they were an infant. So many children in this world experience trauma while they are still inside the womb and/or after they are born. My heart aches for them. In the recent month or so my heart strings have especially been pulled for the millions of orphans outside of the USA and although I haven't felt that international adoption is the way that we will build our family, I still find myself praying for forever homes for children everywhere.  My heart also aches that in order for us to add to our family by adoption that means that a child isn't able to be raised by it's birth family. This is a very humbling thing to think about and at times brings me to tears. Please pray that we would lean on God and that we would be patient as we wait and that we would be very aware of when we should say yes to a child and that hearts would be protected. 
  •  Pray for wisdom and clarity of understanding for the doctors and researchers who are studying PBD-ZSD and/or treating individuals with PBD-ZSD. Although there are many others who are working hard in labs in the US, Canada, and in Europe, if you'd like to pray for members of GFPD's Scientific Advisory Board by name, you can find them on our website. Pray that 2016 will be the year that breakthroughs will be made that will lead to life changing treatment options for those impacted by PBD-ZSD. Members of the GFPD's Scientific Advisory Board have dubbed this to be the "Year of the Peroxisome," and I pray that they are right. I was corresponding with one of the top PBD-ZSD specialists today and here are her exact words to me, "Thank you Pam, the ' Year of the Peroxisome’ is definitely unfolding in many exciting ways! Please tell Ethan- this is his year!" Even now it is hard to not be emotional about the possibility that this could be the year in which effective treatments that would improve Ethan's quality of life are discovered. 
  • Pray for the leadership of the GFPD. We are all volunteers and we are parents of children (living or who have passed) impacted by PBD-ZSD and it is our desire to serve and support families like ours and to help in furthering the research efforts through fundraising and grants to those that are studying PBD-ZSD. 
  • Pray for all the parents whose children have passed away from PBD-ZSD. Tonight as I talked on the phone with a dear friend whose child is in heaven, and shared with her through tears the email message above, my heart was breaking in the midst of the joy that perhaps the doctors and researchers are on the brink of something amazing, because it already too late for my friend's daughter and so many other children. PBD-ZSD is a complex disease, and I don't know if it is God's will that this will be the year in which discoveries will be made that will give Ethan and other children the chance of a better quality of live, but as optimistic as I am that something great is coming very soon, I am still hesitant because there is simply so many unknowns and saddened by how many children have not and will not survive long enough to benefit. We have been part of the Global Foundation for Peroxisomal Disorders for more than five years and so many of my friends have lost children to this devastating disease in that time. For a better understanding of how these losses affect me, please take a few minutes to read the post, "When a Child With Your Child's Disorder Passes Away." 
  • Salvation of friends and family who do not yet know the Lord. Pray that we would be faithful witnesses and that they would see our dependence on the Lord and that hearts would be opened and lives transformed by the power of God's grace and love.

January 4, 2016

Rough day....

It wasn't a great day for Ethan. Although he did okay at church, he had several seizures during his nap after we got home from church. For the last several days the seizures seem to be contained to nap time, and we have no idea why and this evening, Ethan was just not his usual self. It took him much longer than usual to fall asleep -- more than an hour later than what is pretty typical for him. We aren't really sure what is going on, but he did just have another medication change on Friday (the dose of Onfi continues to slowly decrease, as the dose of Phenobarbital continues to slowly increase) so that might be why he is acting different. 

I am scared. I hate PBD-ZSD so much. I hate the seizures, I hate the physical and cognitive disabilities and the health issues that come with it. I hate that we don't yet have effective life-saving treatment options (or a cure). I hate that so many of my friends can no longer hug their children and kiss them goodnight, and I hate that someday I will likely be in the same situation. I love my son, but I hate this disease. I hate that PBD-ZSD touches every aspect of our lives and that it is so hard for me to be able to find joy, peace, and contentment when my human mommy heart just wants my son to be healed. I want to be able to have a conversation with with him, I want him to be able to do all the things a normal, typical five and a half year old boy does, but all of those things have been stolen because of PBD-ZSD. I'm pretty sure that PBD-ZSD has made me not a better person but a more bitter person. I am in a place right now that I am really struggling with fear, worry, hatred, jealousy, anger, discontentment and envy [to just name a few of the sins that I am currently wallowing in]. I just want to scream at the top of my lungs, "It's not fair. Lord, I know You have the power and ability to heal my son, but that that might not be Your will for Ethan and for us, and I don't like that at all!"

I've read more about grief and suffering than I think I would have ever done if our world wouldn't have been completely shattered because of PBD-ZSD, and I have to admit that although I have been a born-again Christian for more than half of my life and I still don't fully understand a lot of things. I also know that although I am sad and mad and frustrated and all the other things I mentioned above, that I'm not completely hopeless, because ultimately I have hope because of what Christ has done. God's love for me, a sinner saved by grace, is amazing, because there is nothing that I've done to earn it and there is nothing that I can do to lose it. 

"For God so loved the world that he gave his one and only Son, that whoever believes in him shall not perish but have eternal life."
John 3:16

" I give them eternal life, and they shall never perish; no one can snatch them out of my hand. My Father, who has given them to me, is greater than all; no one can snatch them out of my Father's hand. I and the Father are one."
John 10:28-30

For I am convinced that neither death nor life, neither angels nor demons, neither the present nor the future, nor any powers, neither height nor depth, nor anything else in all creation, will be able to separate us from the love of God that is in Christ Jesus our Lord.
Romans 8:38-39

This is love: not that we loved God, but that he loved us and sent his Son as an atoning sacrifice for our sins.
1 John 4:10

This morning our pastor's sermon was titled "Marveling in God's Message," and focused on Psalm 119:1-16. It was a powerful and convicting sermon. One of the songs we sang this morning is "All I Have Is Christ," and as we sang I couldn't hold back the tears. I am so guilty of frequently not living a life that reflects the words that I sang. Jesus is my Savior and Lord.  I have assurance that because of His sacrifice, His love, His grace, and His mercy that I have been forgiven of my sins (past, present, and future) and one day when I stand before God I'll be accepted because Jesus paid the price for my sin. However, I'm not always a very good witness to the world of the hope that I have in Christ. 

I think part of my reaction to "All I Have Is Christ," this morning stems from my failing to spend the time I should in God's Word. There have been times in the past where I seemed to be doing pretty good at digging into my Bible and other times which I have pushed it aside -- not because I no longer believed it, but simply because I chose to not make it a priority. My prayer is that in 2016 that I would grow closer to God, and I know that reading God's Word is a crucial component of deepening that relationship. 

My reading/reflection for today (January 31 Day Scripture Writing Plan) really spoke to my heart, since I will freely admit that I am struggling to see the beauty in our current circumstances.

He has made everything beautiful in its time. He has also set eternity in the hearts of men; yet they cannot fathom what God has done from beginning to end. I know that there is nothing better for men than to be happy and do good while they live. That everyone may eat and drink, and find satisfaction in all his toil - this is the gift of God. I know that everything God does will endure forever; nothing can be added to it and nothing taken from it. God does it so that men will revere him. Whatever is has been, and what will be has been before; and God will call the past to account. 
Ecclesiastes 3:11-15

Notes about these verses from my Life Application Bible:
3:11 - God has "set eternity in the hearts of men." This means that we can never be completely satisfied with earthly pleasures and pursuits. Because we are created in God's image, (1) we have a spiritual thirst, (2) we have eternal value, and (3) nothing but the eternal God can truly satisfy us. He has built in us a restless yearning for the kind of perfect world that can only be found in his perfect rule. He has given us a glimpse of the perfection of his creation. But it is only a glimpse; we cannot see into the future or comprehend everything. So we must trust him and and do his work on earth.
3:12 - To be happy and do good while we live are worthy goals for life, but we can pursue them the wrong way. God wants us to enjoy life. When we have the proper view of God, we discover that real pleasure is found in enjoying whatever we have as gifts from God, not in what we accumulate.
3:14 - What is the purpose of life? It is that we should revere the all-powerful God. To revere God means to respect and stand in awe of who he is. Purpose in life starts with whom we know, not what we know or how good we are. It is impossible to fulfill your God-given purpose unless you revere God and give him first place in your life.

If you happen to see me or talk to me while I'm stuck here in this valley, please know that I have good days and bad days. Some days I don't let PBD-ZSD get the better of me, but other days the sorrow is nearly overwhelming. Although it might not always look like it, I am leaning on God for strength, and I might be a mess, but I'm thankful that I am His mess. I'd also like to ask that if you do not yet know Jesus as your personal Savior and Lord that you don't let my shortcomings, sins, and struggles get between you and eternity. If you are interested in learning more about God and His love for you, please check out the "Why do I have hope?" and the "Their Words, My Thoughts {Recommended Reads}" sections. 

Would you please pray for Ethan, Jeff and I? 
  • Pray that we would feel God's presence, eagerly seek Him and remember to keep trusting His promises when we find ourselves overwhelmed by the current struggles of this life.
  • Pray for a solution to the continued seizures and that our little guy would be able to get back to where he was several months ago in regards to his overall happiness, alertness, health, and mobility.  
  • Pray for breakthroughs in science that will lead to effective life-changing, life-saving treatment options for children born with PBD-ZSD. 

January 1, 2016

Goodbye, 2015! Hello, 2016!

As 2015 came to a close last night I couldn't help but think about all that has happened and how much has changed in the last twelve months.

  • Ethan got his own car, thanks to the Metamora H.S. robotics team!

  • Ethan had PT in the pool for the first time!

  • We celebrated Rare Disease Day 2015!

  • I spent the night away from Ethan for the first time EVER. I actually did this on two separate occasions. Jeff and Ethan did better the second time around then they did the first, but I'm so glad that I was able to go on the short trips, even if it took me away from them. The first was to OK where I was able to visit with two fellow PBD-ZSD families. For the second I road the train from Bloomington to Chicago for the first time and attended a NORD conference in Downtown Chicago. 
  • Several dear close friends were forced to say goodbye to children who died because we still don't have life-saving treatment options for PBD-ZSD and thousands of other rare diseases.

  • Ethan went to a dairy farm for the first time!

  • We met and have gotten to know another family who has a little boy who was born with PBD-ZSD that only live an hour away from us. This is practically being next door neighbors for those of us in the rare disease world, since PBD-ZSD has an estimated incidence rate of 1 in 50,000 births. We consider ourselves blessed to call this family our friends. You can learn more about Max in the video below. 

  • We went on a mini vacation with Jeff's family in June and spent the 4th of July weekend with my family. 

  • We attended the 2015 GFPD Conference in Omaha, NE. We always look forward to the opportunity that conference provides us to be with other families who understand this disease and how it affects us as a family. It also allows us to have Ethan seen by some of the top PBD-ZSD specialists in the world and to learn about the latest PBD-ZSD research. 

  • Ethan turned 5 years old, and we celebrated with a house full of friends and family.

  • Ethan started his third year of preschool at a new building in a Deaf/Hard of Hearing Plus classroom. 

  • Ethan's mobility improved dramatically in August, September and October only to then rapidly decline when his seizures came back with a vengeance. Ethan is now incredibly unsteady, almost always needs to hold someone's hand and his endurance has dramatically decreased. 


  • Jeff joined Ethan and I for the class field trip to the pumpkin patch.

  •  Ethan went bowling for the first time when his class went to the Bass Pro Shop.

  • We enjoyed our first visit to our local Children's Museum/Playhouse during a family night sponsored by Easter Seals. 

  • Unfortunately, November and December were marked with multiple trips to the ER and admissions to the hospital, all seizure related. Ethan and I had our first ride in an ambulance since he was transported from DMH to St. John's shortly after his birth. Sadly, we got to do this not just once, but twice -- both times from school to the hospital. 

I know that 2015 was filled with as many good memories as bad, but it is hard to think of those when the last few months have been mainly consumed with fear, frustration and a sense of hopelessness, as we struggled with finding a way to get Ethan's seizures under control. Sadly, we are still struggling to determine what medication combination will be the best for him. Seizures are horrific. We hate them and how they (and the medications that are supposed to treat them) affect our little boy.

January and February are going to be filled with doctor appointments as we'll see Ethan's orthopedic, neurologists (yes, he currently has 2 as we transition his care from one who is two hours away to someone local), geneticist, endocrinologist, dentist, GI, and eye doctors. School will also be starting back up for Ethan soon, and hopefully the seizures won't keep him home too many days and/or I won't get too many calls to come get him due to the seizures, because our little boy really does seem to love school. We are very blessed that he has amazing teachers and therapists.

My plan is to follow a 31 Day Scripture Writing Plan this month as a devotional. This morning's reading/writing/reflection was Isaiah 43:16-19:

This is what the Lord says -- he who made a way through the sea, a path through the mighty waters, who drew out the chariots and horses, the army and reinforcements together, and they lay there, never to rise again, extinguished, snuffed out like a wick: "Forget the former things; do not dwell on the past. See I am doing a new thing! Now it springs up; do you not perceive it? I am making a way in the desert and streams in the wasteland. 

I freely admit that not dwelling on the hardships, heartaches and disappointments of the past is easier said than done. However, I do know that as we begin this new year that I find myself, more than ever, depending on God to make a way in the desert and streams in the wasteland [of this life]. Not only do we face so much unknown in regards to Ethan's seizures and all other matters of our family life, all one must do is turn on or read the news to know that our world and people everywhere are hurting. As we begin 2016 it is my prayer than none of us will forget all that Christ has done. Rend Collective has a great song for this time of the year titled "For All That You Have Done" that is set to the tune of Auld Lang Syne that I encourage everyone to check out (see below).

Current Prayer Requests:
  • Salvation of friends, family, and all who don't know Jesus as their personal Savior and Lord.
  • A closer and deeper relationship with Christ for all those who already know Him [this especially includes myself] and that believers would feel His presence in mighty and powerful ways, and that our lives would reflect that we know Him.
  • Wisdom for all those who are studying PBD-ZSD and for the discovery of medical breakthroughs that lead to the creation of effective treatment options and ultimately a cure.
  • Patience, wisdom, guidance, strength, comfort, contentment, joy and peace for Jeff, Ethan and I. PBD-ZSD is hard on all of us!
  • An end to Ethan's seizures and that he will be able to regain his stability and strength of walking.
  • January is Sanctity of Life month and our church is collecting formula, diapers, pullups, children's socks and underwear, coloring/activity books, and crayons/colored pencils with the goal of blessing our local crisis nursery, pregnancy center and various foster care agencies with these much needed items.