December 19, 2015

An earlier than expected start to Christmas break for Ethan

Thursday morning Ethan had a previously scheduled EEG, that had been ordered a couple of months back when all the crazy seizure breakthrough stuff begun. Obviously, the hope was that enough time would pass and that the medications would be working well and that the EEG would show that Ethan's seizure activity would be "under control." Since the last five to six weeks have been a real rollercoaster in terms of Ethan's seizures and so we weren't really sure what the EEG would show us.

EEG's are stressful for Ethan. He is sleep deprived, to start with, and then he is held down so that more than 20 electrodes are attached to his head. Then we have to try to make him sleep and not allow him to pull the electrodes off of his head. Ethan gets extremely agitated because he is being held down and people are touching him. After that, he is pretty exhausted from fighting and typically is able to eventually rest for the test. It is preferable for an individual to be asleep during the test, although it isn't required.

Thursday's EEG started out well, all things considered. Ethan got hooked up and pretty quickly after that was able to settle down and sleep. Jeff has been able to accompany us to the EEG's and he curls up with Ethan so that Ethan feels safe and will go to sleep. More than half way through the test Ethan began having seizures. They were lasting anywhere from 10 to 20 seconds each and began to cluster, one after another. At five minutes we gave him his rescue medication, diazepam, and although it took several minutes for the seizures to stop, they did. During this time the EEG technicians were still recording/monitoring because it allowed them to "see" that the seizures were stopping. The diazepam makes Ethan very sleepy, and so as began to really kick in the electrodes were taken off and Jeff and I comforted him while we discussed whether we needed to head down to the ER or if we'd be okay at home. Ultimately, we decided to go home since Ethan was sleeping and the seizures had stopped. We did ask the EEG technicians to go ahead and call the neurologist who would be reading the EEG and have them contact us if they felt that we needed to come back to the ER.

So, we came home and Ethan was resting, taking a nap, so we felt comfortable enough for Jeff to head to work. Sadly, Jeff wasn't at work long before I called him to let him know that I had received a phone call from the nurse at the neurologist's office and that he had read the EEG and wanted Ethan to be admitted to the hospital for further observation with the hope that something could be figured out to help get the seizures back under control. Thankfully, because Ethan was doing okay at home, we were told that unless something changed that we could stay at home until someone from admitting called to let us know that there was a room available on the pediatric floor for him. This allowed us to not only take the time needed to pack and prepare but also eliminated the need for us spend hours in the ER. Several hours later the three of us were up at the hospital, and although it still took nearly an hour of waiting before we were settled into our room, I was so thankful that we didn't have to go through the ER to be admitted.

Soon after we arrived the nurses and the residents touched base with us and let us know that there wasn't going to be a lot done except having Ethan being observed. We spoke at length with the resident about why we were even being admitted if nothing was going to be done, but ultimately it came down to the fact that if we stayed that we'd get to see the neurologist in the morning, so we stayed.

On Friday morning we saw the neurologist, Dr. S. There is a huge shortage of pediatric neurologists nationwide, but specifically in IL, especially in our area of the state. Our local children's hospital has had a lot of trouble staffing the neurology department and we have been trying to get a neurologist here since we moved in July 2014. One way that they are doing this is by "contracting" neurologists, so Dr. S., comes to our children's hospital one week a month, during which he not only sees children who are hospitalized but will also see a limited amount of patients in clinic (for regularly scheduled appointments). Jeff and I both liked Dr. S., who admitted that although he had seen two other children with PBD-ZSD in the past, Ethan is the oldest and most "mildly" affected that he had ever seen and that he was surprised to see Ethan doing so well. Dr. S. spent a long time with Ethan and us, and told us that he would do a bit more reading and would comeback in the afternoon with a game plan. He was also very open to contacting the PBD-ZSD specialists, which is very important to me, because not only have many of the top PBD-ZSD specialists in North America seen Ethan in clinic during the GFPD conferences, but it also lets us know that he is a team player.

After Dr. S. returned in the afternoon it was decided that we would start slowly decreasing Onfi, increase Topamax, keep Keppra the same (this was recently increased), and begin introducing Phenobarbital. We also have an appointment to see him in 4 weeks and we will be admitted to the hospital for a 24 hour EEG earlier in the same week as the appointment so that he can see how the new medication regiment is working. We have a detailed decreasing Onfi and increasing Phenobarbital schedule that we will be following between now and then. While Dr. S. is only in our area for one week each month he is available by phone and email. There is also a nurse practitioner, who has extensive experience in neurology, who will meet with us during our upcoming  appointment who will also be a part of Ethan's care team.

By the time we arrived home last night we basically bathed Ethan, feed him, and then it was bedtime. We were all exhausted. Being in the hospital, for even one night, is draining, and then was an "easy" stay because Ethan didn't have an IV and wasn't hooked up to anything. Although, we hadn't expected to start Christmas break off this way, since Ethan was supposed to go back to school on Thursday after the EEG and then have outpatient PT that afternoon, and Friday he was going to have an orthopedic appointment in the morning before going to school for the last day of school for the year, it is what it is.

Ethan was really tired today. The changes in the medications seem to really impact his energy level, and we are hoping and praying that over the next two weeks while he is on Christmas break his little body will begin to adjust and he will be less sleepy. We want to (and need to) stop the seizures, but we also don't want Ethan to be drugged up all the time. I want to see my little boy's smile and personality and there is always a fear that the drugs that are designed to control the seizures could steal his smile and personality away from us in the process. We do not want that to happen.

Prayer Requests
  • Wisdom for the medical professionals to prescribe the right medications and doses of those medications so that Ethan's seizures will be controlled and he will have the least amount of side effects possible.
  • An end to the seizures and that Ethan (and us) would never have to deal with them again.
  • Effective treatment options and ultimately a cure for PBD-ZSD in Ethan's lifetime, that would be available in time to help us give Ethan a better quality of life.
  • Wisdom, guidance, patience and strength for Jeff and I as we join in the decision making process in regards to how we treat the seizures and manage the possible side effects.
  • Strength and energy for Ethan as his body adapts and that he will be able to continue to learn and make progress in his own way.
  • Peace and joy during the Christmas season, as it is extremely difficult to get into the Christmas spirit while things seem so uncertain and our energy is spent elsewhere.
  • For hearts and lives to be transformed by the gifts of love and grace of Jesus Christ.

December 9, 2015

Big Day - Field Trip to Bass Pro & Easter Seals Family Night at the Playhouse Children's Museum!

Ethan was so tired tonight. He had a busy day at school, which also included a field trip to Bass Pro to meet Santa and to go bowling, and then this evening we had our first visit to the Playhouse Children's Museum.

More importantly though, here's a quick medical update:
  • We are thankful to report that for the last two days no one has seen any seizures, so we are hoping and praying that this most recent increase in his medications might be making a difference.
  • Our current neurologist (the one who is 2 hours away) called in a stronger dose for the diazepam. Ethan had been prescribed a 2.5 mg dose by the neurologist who saw him when he was hospitalized last month, but since it has not stopped the seizures the last 2 times it was administered, our neurologist calculated that a 7.5 mg dose is more appropriate for his weight.  So our hope is that the new dose will stop the cluster seizures, if they occur again. Obviously, the goal is to not have a need to use the rescue medication and to find a way to keep the seizures under control for good, however having the diazepam stop the cluster seizures allows for us to care for Ethan at home, unless there is a change in his breathing, etc., without needing to go to the hospital. A dear friend is going to be sending us a pulse ox, which we will be able to use to check Ethan's oxygen levels during and after a cluster seizure and the administration of the diazepam.
  • I heard back from our DSCC care coordinator, and just like we had both assumed, Ethan is still nowhere near close enough to scoring the points required to be eligible for the MFTD Medicaid waiver.
Now to the fun stuff!

Pictures from the field trip to Bass Pro:

"Look, Mom, I found a door. Can we leave?"

"What do you mean we have to stay? We've been here for 10 minutes already."

"I don't care for bowling, but the lights and this shark were pretty cool."

"Mom, why do you want pictures of me with a shark? Should I be worried?"

"Really, another one?"

"This is as much of a smile as you are going to get from me."

"Can we please go back to school now?"

Pictures from the Playhouse Children's Museum (It was a family night for families of children with special needs sponsored by Easter Seals and we were very thankful that it wasn't crowded and admission was discounted. Ethan had a good time, and we stayed an hour, but that was about all that he could handle.):

"Are you sure you know what you're doing, Dad?"

"I've always wanted to drive a combine. Great Grandpa Troutt will be so proud of me, when he hears about this."

"Wow, they put corn in a lot of stuff!"

"Thanks, Dad for helping me play the matching game, but why do they put corn in everything?"

"Light table and magnet shapes - score!"

"You want me to put my hands in there..."

"I'm not so sure about this..."

"Okay, maybe just a little..."

"Look at all the butterflies!"

"They are landing on our shadows."
"How does this work?"

"Oh, I figured it out!"

December 8, 2015

Here we go again!

Yesterday, our family got to take another trip to the ER.

Just a little after 7am, after I had fed Ethan breakfast and gave him his morning medications, he and I came into the living room to play before we had to get ready to leave for school. Ethan had 5 seizures, each lasting 10 to 15 seconds, back to back. Jeff had not yet left for work so I had him get the Diazepam, Ethan's rescue medication, and we gave it to him. The Diazepam is supposed to stop the cluster seizures, but it didn't. The seizures continued and didn't show any signs of stopping so the three of us packed up and headed off to the ER.

By the time we arrived at the ER the drowsiness that is a side effect of the Diazepam was very evident, and although Ethan wasn't sleeping, he was definitely out of it. He had another small seizure shortly after we got to the hospital while we were being checked in. Once again his vitals were monitored and blood taken. The doctor spoke on the phone with our neurologist (who is 2 hours away) and she directed us to increase his Onfi again (we had just increased it on Saturday).

We were home before 11am and Ethan was a little tired but otherwise playing like he usually does. He did take a good nap from about 1:15pm to 2:45pm and woke up ready to play, and still went to bed for the night a little after 8pm without much fuss. We didn't see another seizure all day and friends from our small group at church made us dinner, which was very nice (and delicious).

This morning I took Ethan to school and then stuck around for a little while. I spoke with the nurses who care for him at school and then saw him working with his OT and then during circle time. I left around 10am, and so far, things must be going okay (it is a little after 2pm right now) because I haven't gotten a call from school.

I've spent much of my day on the phone.

  • I spoke with Ethan's DSCC care coordinator to see if the recent changes in seizure activity and the subsequent ER visits make a difference in making Ethan eligible for our state's Medically Fragile and Technology Dependent (MFTD) Medicaid waiver program, which would allow Ethan to have Medicaid as a secondary insurance and access to other services such as respite care. Unfortunately, the care coordinator does not think Ethan will "score" high enough to be eligible, but she is going to double check with others in her office who are care coordinators for children on the MFTD waiver. [Every state gets to decide under what circumstances a child with disabilities, whose family's income is above the cutoff to financially qualify for Medicaid, can be eligible for Medicaid, and the qualifications/requirements vary greatly by state. IL is one of the worst states when it comes to services for children and adults with disabilities.]
  • I spoke with the nurse at Ethan's neurologist's office and she is going to talk to the doctor and get back to me. I wondered if the strength/dose of Ethan's rescue medication might need to be raised, and if the reason it wasn't stopping the seizures was because it simply wasn't strong enough. My hope is that a higher dose is still considered safe for my little guy and that if the need arises to use it once again that it would actually stop the seizures, and then we could simply monitor him closely at home as long as his breathing is fine, etc. 
  • I spoke with Ethan's nurse case manager through his pediatrician's office, filling her in on the last several days and asking her to talk to Ethan's pediatrician about a number of things, including if she could find out if the new neurologist that we are hoping to get Ethan into next month (we still don't have an appointment) is a doctor who is a supporter of the use of medical cannabis (marijuana) for children with uncontrollable epilepsy. I also asked her if she would get our pediatrician's thoughts on it. At this point we are very open to learning about all the possible avenues we could take to try to get these seizures under control. 
Prayer Requests:
  • All the same requests from Sunday
  • Ethan has a field trip tomorrow. I pray that he would be able to go on it (I'll be going with) and that he won't have any seizures while we are out in the community visiting Santa. 
  • Wisdom about medical cannabis. I have begun reading more about Charlotte's Web, which is a hemp oil, and therefore is legal and available for use without a medical cannabis card. We have not yet pursued a medical cannabis card for Ethan, and the oils are not yet available at the Illinois dispensaries, so even if we had a card and the required script we would not be able to obtain it "locally." We also want to see if the clinical trial for Epidiolex is still accepting new patients, and if they are, find out if Ethan a possible candidate. My understanding is that the trial is not accepting new patients at this time, but you never know until you try.
  • I would like to start some annual local fundraising events for the GFPD to raise money for PBD-ZSD research. The top PBD-ZSD researchers in North America and Europe have several promising ideas that they would like to pursue, but it takes money to do so, and research dollars for rare diseases are rather limited, so the GFPD and other rare disease patient/family organizations find themselves in a place where they need to raise the money needed to help find possible treatments and ultimately cures for the diseases that affect their loved ones. However, I'm going to need a team of people who will help me with this. Please pray for a team of 5 to 10 people to step up and volunteer to be part of a planning committee who can help me establish a fundraising event or two that can be successful in raising awareness and much needed financial support for research. 

December 4, 2015

Racking up the frequent flyer miles at our local ER

On Wednesday, I got a call from Ethan's school that he was had a cluster of uncontrollable seizures, that lasted about 4 minutes, so I went and got him from school and brought him home. He was exhausted and rested a majority of the day, but we didn't see another seizure all night.

Yesterday, I decided to keep Ethan home from school and let him continue to rest, because he slept in and seemed extremely tired. We had a nice pajama day here at home, he was extra affectionate and took a really good afternoon nap. We didn't see any seizures all day.

This morning Ethan woke up and seemed well rested and was acting like his usual self, so I took him to school. School begins at 8:30am and just about an hour later I got a call from his teacher letting me know that Ethan had had another seizure episode and that it lasted over 5 minutes so the nurse had given him the rectal Diazepam. Of course, I was freaked out and I let them know that I would be there as soon as possible. As I was driving to school the nurse called and said that the Diazepam had not yet stopped the seizures and asked if I wanted her to call 911. Obviously, I said yes, and called Jeff to let him know what was going on and ask him to meet us at the ER.

It is about a 10 minute drive from our house to the school and the ambulance beat me there and Ethan was already strapped onto the stretcher when I arrived. This momma held it together, but I was a mess on the inside. The seizures had stopped by the time I got there but they had lasted for approximately 12 minutes. It wasn't one continuous seizure, but rather a cluster, so he would have one, it would end, and then he'd go right into another one. Nasty stuff, none the less.

I'm not sure what it is about school that seems to make his seizures appear to be worse, and everyone who works with him has tried to determine if there is something that is "triggering" them, but so far there is no indication that that is the case. So, just like our ambulance ride from the school to hospital back on Friday, November 13th, off we went. The paramedics were great with him and there were ready for us at the hospital when we arrived. Jeff got there just a few minutes after we did.

They started an IV and drew blood for testing, did a chest x-ray to help rule out a respiratory infection, swapped him for the flu and RSV and thankfully everything came back normal, which is good, but it also doesn't help explain why the seizures continue to break through. Ethan was given an IV dose of Keppra before we were discharged with a plan to up all of his anti-seizure medications. So, this evening we increased the Keppra and tomorrow we will increase the Onfi and the Topamax (which was already scheduled to increase tomorrow anyway).

We were at the ER for about 4 hours and we are very appreciative that one of the pastors from our church stopped by to check on us, to visit, and to pray with and for our family. Pastor Aaron read to us a well known passage that we keep going back to over and over again ---

"Rejoice in the Lord always. I will say it again" Rejoice! Let your gentleness by evident to all. The Lord is near. Do not be anxious about anything, but in every situation, by prayer and petition, with thanksgiving, present your requests to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus. Finally, brothers and sisters, whatever is true, whatever is noble, whatever is right, whatever is pure, whatever is lovely, whatever is admirable - if anything is excellent or praiseworthy - think about such things. Whatever you have learned or received or heard from me, or seen in me - put it into practice. And the God of peace will be with you." (Philippians 4:4-9)

Jeff had just reread this passage to me earlier this week before we prayed one night before bed. We know that God is good and loving and that He loves Ethan even more than we do. We know in our hearts and in our minds that God is going to take care of our son, but as Ethan's parents it is really hard to not worry about this stuff.

We are in a place right now that I feel like the seizures (and PBD-ZSD in general) are our "fiery furnace." For those who might not know the story, you can learn about the three young Hebrew men, Shadrach, Meshach and Abednego and King Nebuchadnezzar's fiery furnace in Daniel 3 and/or from the Shane & Shane song "Burn Us Up" (thanks, Pastor Aaron).  So many times in the last several months as we have been really struggling with the seizures I think of the reply that these young men made when facing uncertain death by a foreign king -- "If we are thrown into the blazing furnace, the God we serve is able to deliver us from it, and He will deliver us from Your Majesty's hand. But even if He does not, we want you to know, Your Majesty, that we will not serve your gods or worship the image of gold you have set up." (Daniel 3:17-18).

Obviously, our battle with seizures is not the same as being thrown into a fiery furnace to die (if you don't know how Shadrach, Meshach, and Abednego's story turns out, you should really check it out), however we are standing up and saying that even if God doesn't take these away and even if he doesn't heal our son this side of Heaven we aren't going to turn away from Him. I'm a simple sinner saved through grace who continues to fail my Savior each and every day, and who still has questions and doubts especially when it comes to the suffering that occurs in this broken world we currently call home, but I know that God has a plan and that He has not left us, even when it is hard to see the purpose behind all of this and it is nearly impossible not to ask why. (Romans 8 is a good place to read about God's presence in the midst of suffering).

Are we upset, sad, worried, concerned, and scared about the seizures? ABSOLUTELY. Do we desperately want relief and healing for our son? YES. However, even in the midst of our frustration and our sorrow we are not hopeless and we haven't given up on God. Rather we know that it is now that we need to cling to Him.

Hebrews 13:5b - 6, assures us that "because God has said, 'Never will I leave you; never will I forsake you.' So we can say with confidence, 'The Lord is my helper; I will not be afraid. What can mere mortals do to me?" and Psalm 46:1 tells us, "God is our refuge and strength, an ever-present help in trouble." I know these things deep down in my soul, but to be completely honest, I still have a hard time sometimes, because being Ethan's mom is tough. I love our little boy so much and it hurts so much to see him suffer and there be so little that I can do to make it stop. I can't cure PBD-ZSD. I can't make his little brain stop seizing or fix every single cell in his body that is damaged because of this horrible disease. As his mom, I want to be able to do that for him and selfishly for us. I know God has the power to heal my son, this side of heaven, but I also know that just because God can do something doesn't mean that He will (that's a whole different topic).

So, right now we are just going to focus on trying to do our best to love Ethan and get him access to the doctors and medications that we pray will help stop the seizures in addition to praying for them to go away. We would like to ask you to join us in praying for:

  • Ethan's seizures would go away -- whether that be through a supernatural miracle or medical miracle --- because we do believe that God has allowed for medications and treatments and that He often uses them. 
  • Ethan to not be scared or frightened or in pain during the seizures and for the medications to not have any adverse side effects.
  • The doctors treating Ethan and that we could get an appointment to see the local pediatric neurologist ASAP.
  • Jeff and I would feel God's presence and His peace, strength, and comfort so completely that it is overwhelming and undeniable. That we would be able to recognize and enjoy all the great moments that we have with Ethan and not let them be overshadowed by the "bad"ones and that we can be the parents Ethan needs and deserves.
  • Healing, the development of effective treatment options for PBD-ZSD, and ultimately a cure. For the doctors and scientists who are dedicated to finding ways to improve the quality of life for our children. For success in awareness campaigns and fundraising efforts so that more research can be done.
  • The teachers, nurses, therapists, and other staff members who work with Ethan at school. 
  • Our country and world. For all those who are currently suffering because of the sinful acts of others. For the refugees around the world who have been forced out of their homes due to violence and fear. For the families who have lost loved ones in the recent mass shootings in CO and CA. For the thousands of families who lose a loved one every year because of gun violence.
  • Our witness and relationship with Christ and with one another. That despite our sinful nature and human failings, those around us would be able to see that we have put our faith in Christ and that it is because of Him that we can still have hope and joy even in the midst of suffering. That we would cling to one another and support each other and Ethan during these difficult times.
  • Our family and friends who don't know Christ. Our hearts are heavy for the many people we deeply care about who do not know the forgiveness, grace, and love that only Jesus can give.