A father's perspective

Justin wrote this and I couldn't have said it better....

It’s a Friday night at 10 o’oclock and I just got out of bed, unable to sleep due to another episode that disturbs the calmness of an unfamiliar apartment in a new city. The lack of sparkle in her eyes as she enters yet another cluster of spasms tears the peaceful slumber away from my child. Hour after hour, waiting for the next one to come. Counting. Tracking. Watching. There is nothing we can do. They come at all hours of the day. Without warning. Everything is dropped to helplessly watch. Is she still breathing? Does she need emergency medication? When will it stop?

Everyone tells you that having a kid will change your life. I don’t think this is what they meant.

For the past two years, my wife and I have watched our first born seize uncontrollably every single day. Piper was born with a rare genetic condition, Aicardi Syndrome, which resulted in the corpus callosum to never develop and an intractable form of epilepsy called Infantile Spasms. Since the age of three months, she has seized every day. This is definitely not what I expected fatherhood to be. But, I have learned some truisms in these past two years. I have learned what unconditional love really is. I have learned that an education bought at an elite school of medicine does not lead to supreme knowledge. I have learned that you can only rely on yourself and the wise people around you.

Over the past two years, my wife and I have battled for our daughter. Every stone has been turned. 17 doctors, 9 anti-epilepsy drugs, 1 severe diet, 6 supplements, 50,000+ seizures, 1,300 miles, thousands of friends, 1 last doctor prescribed drug that could lead to liver failure. We have learned to live by numbers. Numbers that have altered our perspective on parenthood. Numbers that bring a community together for a controversy.

On August 1, 2013, my wife and daughter entered the state of Colorado. We have moved away from those thousands of friends to join a new controversial community. A community that has become addicted to marijuana. We have moved to Colorado to take part in a movement of parents of epileptic children who are trying cannabis as a last ditch effort to heal their children.  

Marijuana. The word has carries some weight that will not set easy with many of you. Trust me, it was not an easy decision. I too once believed that medical marijuana was a smokescreen for people to use the drug recreationally. Not too long ago, my mind was changed. I learned of a 5 year old girl named Charlotte Figi. Charlotte has Dravet Syndrome, another form of intractable epilepsy. Charlotte’s parents were in a similar situation as us and were at the end of pharmaceutical treatment. Charlotte’s parents then found a group of brothers growing a strain of marijuana with low THC (the only psychoactive compound in the plant) and high CBD (cannabidiol, another compound in the plant). They had nothing to lose. Guess what…it worked. The first dose, Charlotte went a week without a seizure. As a frame of reference, she had 300 the week before.

Hope.

She’s not the only one. Now hundreds of parents, including many from Tennessee, are moving their children across the country to get access to the now named Charlotte’s Web. There’s been some media. Perhaps you’ve heard.  Guess what…it’s working for a lot of kids.

To give you an idea of our expectations, we do not think it will work. But, we don’t think anything will work at this point. Another truism, skepticism. We have accepted that this is our daughter and she has influenced more lives in two years than I have in my 28. She has a purpose, to teach.

Piper started on Charlotte’s Web on October 24^th. We give three doses per day in an oil form. For three weeks we have been increasing the dose. It’s never easy.

This week, we have had two newspaper articles and two local news channels report on our journey. Through social media, I have read 600+ comments on these reports and come across 3 dissenters. I don’t know what is coming next, but if you are still reading, I ask for you to remove your assumptions, your fears, and the unknown and ask yourself why.

These past three weeks, we have seen bad days, like today, but we have seen some of the best days. Compared to one of the worst days, Valentine’s Day 2012 – 379 spasms. Three days this week she has had 2-3 spasms. Can we say it is attributed to cannabis? I’m optimistic, but it’s not where I want to be – zero

I don’t know where our journey will take us, but I hope that Piper has fulfilled her purpose and taught you to examine the possibility. I don’t know that we will ever be able to come

Charlotte's Web First Two Weeks

Piper had her first dose of CW on October 24. We are hoping this is a date we will always remember, a date that replaces the anniversary of the onset of seizures, and other things we would like to forget. To be honest the first two weeks have been rocky. The day after her first dose, I started having symptoms of a stomach bug. Piper did not seem to have any symptoms so I figured it was something I ate. After the initial dose, I didn't see any immediate changes except she was a little sleepier than normal. The next day she looked terrible and had symptoms of a stomach bug too. She slept more than I have seen her sleep in a very long time. I hoped it was the new medicine calming her down and letting her get some rest but it seemed she was just her fighting off a virus. It took five days for her to start feeling better and then she had three excellent days! Halloween was the best day she has every had in her life. She was smiling, giggling and playing all day. She was very aware and responsive to us. I think she was excited to finally have her dada home. She also had the closest to a seizure free day that we have ever seen, she only had on single spasm!  We haven't had that kind of hope in a very long time.

Since Halloween we have had a rough time. We noticed that she was cutting a molar and started seeing an increase in seizure activity. Teething has always been a trigger of seizures but it has been almost a year since she had a tooth come in so we don't remember how bad it is. Seizures have been all of the place and we have seen some pretty rough ones. We have seen tonic "typical" seizures that we rarely ever see. She had 5 in one day and that never happens. We normally see 1-2 tonic seizures a month. We have used "rescue" diazepam multiple times to help her body get a break from the seizures. We noticed three days ago that she has another tooth coming in as well!We are praying that teething is the cause of all of the bad seizure activity and that she will get over it soon. We have heard from a few doctors and the dentist that teething is a developmental milestone. The interesting thing is right after we started CBD oil she has an influx of teeth appearing and we are curious if there is a correlation.

 Since we have so many additional factors like sickness and teething it has been hard to see how well  the medicine is working. We do know that she has never had a seizure free day and she was very close and that is very promising. When she had a break between being sick and teething she has some really good days so we think the medicine is helping but we won't be able to tell how well until she feels better. She is still on a low dose and we will be increasing to her full dose over the next two weeks. We have talked to a few families who saw results after 4-6 months of adjusting dose and weaning pharmaceuticals and this may be the case for Piper.

We have been involved in getting our story out to the public. We were contacted by a reporter from the Salt Lake City Tribune about a week before we started treatment. She was interested in doing a story about us and wanted to fly in to Denver ASAP to interview us. She actually was able to be here for Piper's first dose of the oil and did an interview. The story should run tomorrow and we will send out the link for everyone to read. We also were able to participate in an interview done by the local news in Denver. All of the families who are involved with the Realm of Caring met at the grow and were able to tour the grow houses and see the plants. While we were being interviewed Piper had a seizure on camera and made the news lady cry. I guess it was the right time to have one since they are trying to show people how desperately our children need this treatment.

We continue to pray and have hope that we are doing the right thing for Piper. Even if it doesn't help her as much as we want we have seen how much it has helped other children. I sat next to Charlotte Figi at dinner the other night and could not take my eyes off of her. It is truly amazing what this oil has done for her. She was talking, playing and fed herself dinner. To see where she was in the videos at her worst point and to see her in person, thriving, was truly amazing. We hope that we can at least spread the word about this treatment so that one day every sick child has a chance to try it without having to move across the country.

Overdue update

Sorry it has taken so long to write an update. Things have been pretty much the same as always here so I haven't had much to write about. We have been enjoying our time here and exploring the city. Justin finally made it out to visit at the beginning of October. After 7 weeks of being apart, it was so good to finally see him. While he has been here we have been able to explore Denver and just spend time together as a family. He couldn't believe how much my belly had grown while he was away! He will be going back to Knoxville on Tuesday and will be making the official move in November.

 Piper's Nana and Papa came out to visit and we had tons of fun and hated to see them go. We all celebrated my birthday and found some great restaurants in town. We went for a hike in Colorado Springs and toured the Coors brewery. I heard the beer was delicious. We are so lucky to have family that is able to visit. Being so far away from family has been a challenge for all of us but we just keep in mind the reason why we are here and it helps. January will be here in no time and we will get to see the rest of the family then.

We found out last week that the harvest is done and that the CBD oil is being made now. We may be able to pick up her medicine on Thursday!!!! This is what we have been waiting so patiently for and it seems unreal that it is finally here. We will go to a new patient meeting tomorrow to learn more about dosing, etc. We are so excited but it is also a little scary. This treatment could change her life but we also know it may not work as well for her. We are praying so hard that she gets the relief she deserves from these terrible seizures. I am filled with so many emotions (pregnancy enhanced) that have been building up the past few months. I will update more frequently because I know you are all as anxious as us to see what happens with the treatment. Please pray that this is finally the answer to this nightmare.

This week we will also go for an evaluation with the early intervention system here to get Piper started again in therapy services. I have been putting it off because it has been so nice to not worry and stress out about getting her to appointments. Her days are never consistent so it was hard to have a normal schedule. They do all in home services here so hopefully it will be a little easier for both of us. I know she needs to continue therapy but its really hard to see her so upset and unhappy. We also will be applying for a waiver that was not available in TN that provides assistance for families who do not qualify for Medicaid. Under this waiver I could actually get paid to be Piper's caregiver if I obtain my CNA. The waiver also provides financial assistance for respite care, nursing services and modifications to your home for accessibility. 

I have felt really good throughout this pregnancy and I am in my 27th week. I have been exercising and keeping in shape to be able to lift and keep up with Piper. So far I have felt stronger than I did before I got pregnant! Imagine doing squats with 30 lbs at least 50 times a day. It is a workout just taking care of Piper! I am just now starting to enter the uncomfortable phase but I still have a lot of energy. We have been seeing a high risk specialist just to be safe. So far everything has been great and we have had no issues. He is healthy and growing fast. He is kicking and moving constantly and is so strong.  He is actually a lot bigger than average so I will be doing my gestational diabetes test this Friday to see if that has anything to do with his large size. Hopefully his growth will level out over the next few weeks because it may increase my chances of having another c-section if he is large. I really don't want to have surgery again since the recovery was so hard but we won't know more until later. We are just happy he is healthy and will worry about the rest of it later. We have been so preoccupied with Piper we keep forgetting that we will have a new little one in just a few months! We are so excited to meet him!

Waiting

Waiting.... My least favorite thing to do. After the past two years you would think I have mastered patience. If anything, I think I have become more impatient then when this all started. We are always waiting on something. It becomes an internal struggle because we want the time to pass quickly to get to what we are waiting for but as time passes Piper seems to be stuck in the same place. The cliche thing you hear from parents, "they grow up so fast". They do, but for us she is growing bigger but basically still the same child as we saw at 6months when it comes to physical, cognitive and emotional aspects. It's a very disheartening thing to watch. It's like the world around you is going full speed but you are stuck in time. It makes it much harder to see children her age and what she is not able to do. I try not to think this way but I'm only human. 

As most of you who have been following us (we thank you) know; Piper, my dad and I moved to Denver at the beginning of August. After numerous failures with seizure medication we chose to try cannabis therapy. Picking up and leaving everything you know does not sound easy but surprisingly it has not been that bad. We are adjusting well and are ready to try this new treatment. She is legal to start treatment when the product is available. We knew there would be waiting involved and were not surprised. It's much harder waiting here knowing that very close to us could be the thing that finally helps her. We are on the waiting list with the group that treats pediatric patients and if things go well we may be able to start in October. (Prayers please!)  

Another hurdle for Justin and I is being away from each other. We haven't seen Justin in 2 weeks and he won't be visiting again for 5 more weeks. This is an enormous challenge for both of us. We are very close and not being able to talk about things and make decisions together is very hard. I know he misses Piper very much and hates to hear of her recent rough days. Justin's easy going personality and jokes always help change the mood on the really bad days. We could use some of that right now. He also hasn't felt Evan kick yet, which I get to feel all day.I know it must be killing him to miss out on so much. It takes an emotional toll on your heart but we are both trying to stay strong for Piper. We made this choice and knew it would be hard. He will be visiting in October and back to traveling until thanksgiving. Hopefully, he will be able to move out here by then. I am counting down the days. 

I know you are just as eager as we are to see if this treatment works. I will do my best to keep everyone updated! 

 

Le Bonheur

We will be going to Le Bonheur children's hospital in Memphis on Sunday to do a complete seizure study with some technology that is not available here in town. Our last EEG gave us results that were pretty unexpected. Piper has been having a new or modified type of seizure that did not show up on the EEG. We think that these seizures are originating deeper in her brain and cannot be read by a typical EEG. Our neurologist was pleased with the results on paper and suggested that these new seizures are random body movements due to a poorly controlled motor system. We didn't agree and based on our experience of watching seizures, we know that they are in fact some type of seizure. So we talked with him about other tests that can detect deep brain seizures. There are only a few hospitals in the country with this technology and Le Bonheur is one of them. Advanced testing from a team of epileptologists will be done to evaluate and determine if there is any new information to be learned. The evaluation usually takes a week and she will have all of the testing done in a few days. It may not be the best few days for her but it will definitely be nice to have it all done in a shorter period of time.

As most of you know we have exhausted our resources as far as anticonvulsants go. At 22 months old she has been on 9 medications and the ketogenic diet. Only one of these medicines have shown to be effective and does not control seizures completely. This medication has the potential to cause serious and irreversible vision loss. The longer you use it the more likely you are to have these side effects. If things stay the same she will not be coming off this medicine anytime soon. There are more seizure medications out there but the side effects are absolutely terrible. We know from experience what these medications can do. We have had a drugged up, zombie like child and do not want to see that again. Her current medications make her sleepy but not able to sleep, aggressive and just in a bad mood for hours after she gets them.  One of the medications we keep getting recommended has caused liver failure (which kills you) in many patients, especially under the age of two. They do routine testing to check if the levels are toxic but once they are there is nothing you can do about it. We don't feel comfortable with these circumstances and won't be trying this with Piper. That doesn't leave many choices. We have been researching for months on other options for Piper and have made some new decisions. We will see what the results say and go from there.

Even with the increase in sezuires, she is mostly happy and in a good mood. She hasn't had a chance to learn a lot of new things but is not regressing so that is good. She is improving with regards to eating, she can eat a variety of textures with no issues. Tonight she had fish sticks and carrots and ate the whole thing! I was very impressed.

For those of you who follow the blog regularly, I would like to add your email to a group list. We have some other issues that I would like to share with you about Piper but would rather not share with the whole world. In the next month I won't be updating the blog as much but will be sending out a group email. Please email me at AnnieKoozer22@gmail so I can add you to the list.

Thanks for the continued prayers, concerns and support! We appreciate you all.

EEG results and next steps

I haven't updated this in a while mostly because I don't have a lot of good things to say. The past 6 weeks have been really rough on all of us. Seizures are completely out of control and our options are slim. She has anywhere from 8-12 clusters of strong spasms that usually last 10-15 min each, sometimes longer. Most of these clusters occur at night so she never gets rest which triggers more during the day. We have cancelled therapy at least once a week and have given her rescue meds on a regular basis to just let her get some rest. We haven't seen this much activity since this all started. The most frustrating thing about it all is that this new activity did not show up on her most recent EEG. Our neurologist was actually pleased with the results from the study. We told him that although they did not show up they are in fact seizures. We have seen enough seizures to know that they are. He said it was most likely random body movements due to a poor nervous system. We disagree completely and have told him numerous times that something needs to be done. We have researched and suggested that these may be deep brain seizures that a regular EEG can't read. He agreed that it is possible but we would have to do a specialized test to confirm. The only place in Tennessee that has that machine is Memphis and he has connections there. We told him to try and get us an appointment ASAP. It really concerns me that we have to be this persistent just to get suggestions from our doctor. We feel like we are the doctors and really wish we had more support. I worry about other families who don't have the resources or personalities to question the doctor when it's needed.  We really feel like a bigger city would be more beneficial for her needs but moving again is not something we want to think about. 

 The amount of seizure activity she has experience in the last two months has really taken a toll on her. She is tired, irritable and "out of it" all day. She never feels like doing anything and we haven't been going out a lot except for outside our house. Her seizures are so strong and violent that she needs to be in a place where we can lay her down. It's almost impossible to hold her while she is  seizing. Her arms stiffen and slam on the ground so now we worry about her hurting herself. We both have taken some punches in the face.  Not all of her episodes are this bad but about half of her daily clusters are. 

She has regressed with most of the skills she was working on and isn't able to learn a lot of new things. She does have a couple hours a day where she is alert and plays but it's just not the same as it was a few months ago before this got bad. She just wants to sleep all day long. 

The stress of all of this is hard for us but we are actually able to manage it better than we expected. Although Justin did get shingles a few weeks ago! The doctor asked him if he had a lot of stress at home...yeah just a little. He is fine and probably not happy I shared that with everyone. He is training for a big triathlon in the fall so that helps keep his mind off things. I went out of town last weekend for a bachelorette party and it was so fun! It was nice to have a break and hang out with friends. We will be celebrating our 3rd anniversary on the 29th and hopefully we can do something fun. 

I will try to end on a positive note. We should be getting Piper's gait trainer soon and it will be fun to use at home. She spent 30 min in at PT last week and I think if we practice more she will get the hang of it. 

Justin put up her swing at our house so we go out and enjoy that as much as we can. She loves it! She falls asleep every time. 

Seizures, seizures are no fun

Well it has been a long time since my last post. Honestly, March was one of the worst months Piper has had in a long time so I haven't had a lot of postivie things to write about. We have kept busy getting the stuff ready (tshirts, bracelets and brochures) for the expo this weekend and training for the 1/2 marathon. We are putting all of our energy into the big fundraiser this weekend for Piper and Aicardi syndrome.

She was sick for about 3 weeks in March and it started with a fever of 104. Since Piper has never had a fever it was a whole new experience for us. She had the worst seizures we have seen in a while and it was not fun at all. She had a pretty bad cold for about 2 weeks and then she started getting better. We thought she had finally recovered until her fever spiked up again. We took her to the doctor and found out she had an ear infection, which was another first for her. It took her about a week to start feeling better but she is still not back to her usual self and it has been a week since she had been off antibiotics.

During the time she was sick and now her seizures have been out of control. We know that is to be expected when she gets sick but now that she seems better we thought they would slow down a little. While she was sick we noticed a new "drop" seizure that we haven't seen before. We actually took her in yesterday to the neurologist to see what he thinks we should do. He wants us to do another 6 hour EEG next week so he can see what is going on. Not a lot of fun for either one of us but it needs to be done to see exactly what is going on. With all of the seizures she has started regressing again and we are reteaching skills. We always know this will be an issue but it is really hard to see her basically starting over and having to try so hard to learn again.


She is doing okay with feeding so that is a good sign. She has forgotten some skills so we are back to the basics again.   While she was sick she missed a lot of therapy and we are back to just trying to get her to stay awake. The last physical therapy before she got sick she was using a gait trainer, which is a very big deal! She was strong enough to keep her body, head and chest up while taking a few steps. We were absolutely thrilled! Now the issue is getting her strength back up to be able to do that again. We learned today that both our speech therapist and physical therapist are moving in 8 weeks so we will have to start all over with someone new. So we will be losing 2 of our amazing therapists and we will really miss both of them. Luckily, we are all flexible and used to things constantly changing.

 using the gait trainer at therapy

In the middle of all the sickness and seizures Piper has had a few good days here and there. She had a great day Saturday and was happy and vocalized all day. We can only hope that we start to see more days like that. We are all praying that this weekend goes smoothly. We want to thank everyone who has supported us so far by purchasing tshirts and bracelets. We still have tons of shirts and look forward to selling more at the race this weekend. If you have a shirt, please wear in on Sunday while we run for our sweet girl. If you are downtown Saturday come by the convention center and see us.  Thank for supporting our family and GO TEAM PIPER!

ERG and MRI results

Piper had an ERG and MRI done in December and we just got the results back.

"Electroretinography (ERG) is an eye test used to detect abnormal function of the retina (the light-detecting portion of the eye). Specifically, in this test, the light-sensitive cells of the eye, the rods and cones, and their connecting ganglion cells in the retina are examined. During the test, an electrode is placed on the cornea (at the front of the eye) to measure the electrical responses to light of the cells that sense light in the retina at the back of the eye. These cells are called the rods and cones."

Her previous ERG was done at 5 months old and showed normal results for her age. This one also showed normal results for her age. This is what we expected and these are good results.


Her MRI was done at this time last year and showed the malformations that are typical of Aicardi syndrome. She had polymicrogyria, hypoplasia of the white matter, arachnoid cysts and complete Agensis of the corpus callosum.


I am going to use my "kindergarten" explanations because it helps me understand, ha ha!

Polymicrogyria is basically wrinkles in the brain. Our brains have natural folds but her brain has extra folds that look like wrinkles. This can be a cause of the seizure activity, delays etc.

Hypoplasia means underdeveloped, so it her white matter did not develop properly. Your brain has white and gray matter. The analogy I have heard is the white and gray matter are like a computer network. The gray matter would be the computer, the white matter would be the network cables connecting the computers together. Basically, her "computer" isn't always connecting.

Arachnoid cysts are pockets of fluid in the brain that can stay the same size or get bigger.

Complete Agenesis of the corpus callosum is when the bundle of nerve fibers that connects the hemispheres is missing. Some girls with Aicardi have partial Agenesis and may have more success developmentally.


The most recent MRI appears to be the same as the last one. He was unable to compare from our previous MRI but will be doing this soon. She still has all of the malformations mentioned above but it doesn't seem like anything new is going on. I will keep everyone posted once he compares all of the test results.