Chasing the Cure
The Catherine White Story
By Jason White
August 16th is one of the best and worst days of my life. On August 16, 2003, I married my beautiful wife, Carna, with a traditional ceremony followed by a legendary reception with our family and friends. Seven years later on August 16, 2010 our son, Logan, was having kindergarten orientation. I stopped on my way home to grab flowers for Carna, who had Logan and our 5-month-old daughter, Catie, ready to go to the school. Shortly after the orientation began Catie started having a seizure and continued having a seizure. After five minutes of continuous seizures, I took Catie out and gave her the rescue medicine Diastat to stop the seizure. The seizure stopped but only for a few minutes. Then Catie started having another seizure. This is not supposed to happen and never had before.
If a seizure lasts more than five minutes it begins to affect the brain and anything over twenty minutes can cause permanent brain damage or worse. As the second seizure neared five minutes I called Nationwide Children’s Hospital (NCH) in Columbus, OH for instructions, they said to administer another dose of Diastat and get to the NCH ER. It is a two-hour drive from our home to Columbus and about 45 minutes into the trip Catie went into another seizure. By this point, we were out of Diastat and had a long trip left to the ER. I called NCH to get a new prescription called in to a local drugstore, we were able to get the medication, but luckily the seizure ended right about the five minute mark. Catie slept till we got to Columbus and then went in to another seizure, once in the ER they rushed us back to a triage room and began working on Catie as her seizure continued despite IV medicines. 14…15…16 minutes…
Catie was born on February 25, 2010 and was a very healthy baby. She scored 10 on her assessments immediately following birth and was making noises within hours. Catie was developing on or ahead of schedule; she rolled from her back to belly at 6 weeks and she was beginning to sit and continually gain strength. Catie was a great baby; she slept well, was happy, smiling and cooing all the time. She was healthy and on target at her three month check-up. Unknowingly to us our lives were about to take a drastic change.
Carna noticed that Catie made a weird body movement when she woke up one night but wasn’t sure if she was stretching or something else was happening. Carna began keeping a closer eye on Catie’s behaviors and a few days later brought a similar instance to my attention. She said, “I think Catie is having seizures.” We weren’t certain what was wrong but wanted to get her into the doctor.
Catie’s appointment was on June 3, 2010. I went to work as usual, expecting a normal appointment, but I decided to leave work and go to the appointment with them that morning. Carna explained to Dr. Dailey, our pediatrician, what she had observed Catie doing. Even though the incidences were few and undocumented, Dr. Dailey recommended we take Catie to Nationwide Children’s Hospital in Columbus, OH. Dr. Dailey insisted we go immediately; if we weren’t able to set up an appointment Dr. Dailey recommended we take Catie to the ER. She wanted Catie to have an EEG and see a neurologist to get answers right away. We headed home to pack a few day’s clothes, doctor’s suggestion, and left for Columbus. We had never been to Columbus and I wish it were a trip I never had to take.
The drive from our home to Columbus is not bad, a little over two hours, but way too long to think about what’s wrong with your 3-month-old baby girl. Carna rode in the backseat with Catie because Dr. Dailey told us to watch and video record an episode on our phone to help with the evaluation from the doctor in Columbus. To this point, I had only seen a small glimpse of one of Catie’s seizures. Her eyes would roll up in her head and her arm and leg would stiffen, so I was trying to convince myself that it was still a simple issue. Then Catie had a seizure on the way to Columbus lasting about 20-30 seconds, which Carna recorded. We called family and let them know what was going on and we prayed – a lot. Catie had another seizure outside Columbus near Pataskala, OH. I was starting to worry, but then an eerie sense of calm and security set in. I remember the exact spot on the highway and I can still hear the voice telling me, “You and Carna are strong, you are the ones who can handle this.” I believe it was God talking to me – I have no other explanation. I think about that trip a lot and sometimes I’m not sure if I can handle this.
Catie, on the other hand, can certainly handle it! She is super tough. At Nationwide Children’s Hospital (NCH) Catie had an EEG, and we were told the EEG probably wouldn’t show anything unless she had an episode during the test. Catie did great. She didn’t cry or fight while they were gluing the leads on her head. She didn’t have a seizure during the recording of her brain activity but we could tell that the tech was not seeing a normal EEG. Eventually, a doctor came in and explained that the test was abnormal and we would be admitted for observation and more test. These results were something we grew accustomed to. Catie was kept on a long-term video EEG overnight. I figured out what some of the EEG leads meant as I watched them spike repeatedly through out the night. To this day I have not seen a normal EEG but I can watch Catie’s and get an idea of how hard a battle she is fighting at the time.
We met Dr. Vidaurre from NCH Neurology Friday morning and he explained that Catie was having spikes in electrical activity in her left frontal lobe and they were going to do an MRI to check for a cause. The doctors explained, again, that they probably wouldn’t see anything but it was the next step in the testing. Catie was diagnosed with complex partial onset epilepsy. She was prescribed Keppra to help control her seizures. The seizures stopped, so we went home before getting an answer on the MRI results.
We had a follow up appointment with Dr. Dailey on Tuesday and she had received the MRI results. They DID see something: Catie’s left-brain hemisphere had not formed properly. It is severely malformed. She has cortical dysplasia and is missing sections of her corpus collosum. We have been through genetic testing, which was normal, and have no explanation or cause as to why this happened. There was also no known future outcome for her. Other parts of the brain could learn tasks to compensate for the deformity or she could be severely disabled. Not knowing how things may turn out is both the best and worst part of Catie’s illness.
The month of June was a whirlwind of hospital visits, medicines, and learning. What is epilepsy? What caused it in Catie? How do we treat it? How will it affect her future? We learned a lot about epilepsy and the drugs used to treat it, because we have tried most of them, with limited success. We have family members with grand mal epilepsy and they are well controlled through medication. We thought we would give Catie her medicine and she would be ok. We soon realized things aren’t that easy with Catherine Elizabeth.
The seizures came back within five days of the first Columbus trip. We went back to NCH and added new medications. We also got to sit down with Dr. Hamiwka and look at the MRI. She explained the severity and multitude of malformations in Catie’s left-brain hemisphere and had concern about potential issues in the right hemisphere also. Dr. Hamiwka did not believe Catie was a good candidate for resection surgery because of the multiple areas of concern. At this point surgery was the last option in our thought process. We wanted to try medication for seizure control. Dr. Hamiwka also discussed Catie’s future as unclear. The brain is an amazing organ and the theory that each part of the brain has a specific function was not a concrete fact. If a lobe of the brain is damaged or missing, other areas in the brain can learn the process, especially the younger the brain. She did explain that Catie could end up anywhere on the developmental spectrum from severe mental and physical delays to a somewhat normal life.
The doctors put Catie on a medicine called Topomax and the seizures stopped; however, the meds had a negative effect on Catie’s alertness and development. The drug made her very lethargic and she slept much more than normal. At three months old, this state of alertness was slowing her developmental progress. Catie remained seizure-free for 10 days on this Topomax combination and then the seizures returned at an alarming rate.
At the onset of Catie’s condition her seizures were short 20-30 seconds with her eyes deviating to the right and her right arm and leg stiffening and rhythmically jerking. As the seizures returned they began to grow in length, going on for minutes instead of seconds, and her entire body was convulsing. She was prescribed Diastat, a rescue drug that will stop the episode within seconds of administration. The dose is to be given if a seizure lasts more than five minutes. This is something we hoped we never had to use but the seizures had created the need. Now we were headed back to NCH for the third time in less than a month.
Epilepsy is either easily controlled with medication or not controlled at all. About one third of epilepsy patients are resistant to anti-epileptic drugs (AEDs), and if the first two drugs do not achieve a level of control nearly 80% of those people will be diagnosed as intractable or resistant to AEDs. At NCH Catie was put on more medication but this time the seizures didn’t stop. Her diagnosis was now intractable pediatric epilepsy. The AEDs brought the seizures to safe limits but Catie was now seizing 8-10 times a day. We were released just before the fourth of July and went on a family vacation to the beach. While there we were giving Catie Diastat every day or so and were in constant contact with NCH and Dr. Vidaurre. We changed dosage amounts until we finally got the episodes regulated to a few minutes each, but occurring 10 or more times per day. We spent the next month researching drugs, diets and treatments to help find an answer for Catie. She was slowly regressing in strength and development due to the number of seizures and side effects of the multiple AEDs.
We were learning much about epilepsy and the long term affects associated with the number and length of seizures. Catie was in a pattern of seizures and sleep. The awake time was spent in a zombie-like state with her eyes looking to the right almost constantly. She no longer smiled or responded to us. Anything she had learned in the first three months of her life was gone.
This isn’t Working…
17…18…19minutes. Catie was having the worst seizure of her short life in the ER triage room and the doctors couldn’t stop it. The room was bright, noisy, and chaotic. Catie was on a large hospital bed in the middle of the room. Her body was covered with tubes and wires and oxygen on her face, machines were beeping and hospital staff was clamoring all around her. They had given her two doses of Ativan, an intravenous drug used to halt status epilepticus. Status epilepticus is a recurrent or continuous seizure lasting longer than 30 minutes in which the patient does not regain baseline mental status. The doctors were concerned and you could see it and feel it in the room. There is no more hopeless and helpless feeling than seeing your baby laying there seizing not knowing when or if it will ever end. It felt like forever watching them work on her and watching the clock knowing the damage each extended moment in this state was doing to her mind and body. She came out of the seizure as the drugs finally did their job just over 20 minutes after the episode started. Catie fell into a deep drug-induced sleep as we tried to compose ourselves and collect our thoughts.
The next two weeks were spent at NCH with little sleep as we kept a watchful eye on Catie. For the first few days Catie had prolonged seizures lasting 10, 15, 20 minutes, with the longest one lasting 24 minutes. The doctors would push the ativan through her IV, sometimes needing multiple doses, and then she would sleep 8-10 hours, wake up a little then start the cycle over. The doctors were trying different AEDs as often as they could with combinations up to five medications at once. Nothing was making a significant difference, but they did get the seizure down to a tolerable duration. Once Catie was controlled they did another EEG and the findings changed the course of action once again.
Catie’s epilepsy had progressed to the point that her brain was in a state called hypsarrhythmia – basically her brain was electrically hyperactive. She was now diagnosed with Infantile Spasms (IS). Infantile spasms is considered to be a “catastrophic childhood epilepsy” due to the difficulty in controlling its symptoms and the developmental problems that can occur as a result of IS. This was a turn for the worse, as IS affects the entire brain and can cause major delays and permanent damage. We chose an injection treatment called adrenocorticotropic hormone (ACTH) that was only recently approved by the FDA. Its novelty made it very expensive and a little difficult to obtain. The doctors started the injection and trained us how to give them at home. The ACTH worked to stop the seizures completely for three days. We were discharged, put Catie in her car seat, and she started having a seizure. After about four minutes we went back to the ER. The seizure lasted about 7 minutes, and two hours later we were able to go home.
The ACTH changed things at home. Due to the effect on the immune system Catie was not allowed to return to daycare. This meant Carna had to work from home 4 days a week and I was working Saturdays to give her an office day each week. The biggest change at home, though, was administering the ACTH. Twice a day Carna had to hold Catie down while I injected the medicine, alternating legs with each shot. Catie again proved her toughness. She never cried as day after day we gave her shots leaving bruises on her thighs. This ritual went on for three months along with other AEDs, yet we had little success controlling the spasms.
Infantile spasms are a different beast than a normal epileptic seizure. Medically the EEG is highly disorganized and chaotic compared to a normally patterned EEG. Each spasm episode usually consists of a cluster of short flexions or jerks of the trunk, head, or extremities. The flexion will last up to a few seconds then there is a short calm before another jerk creating the cluster effect. Catie was having 10-12 clusters a day and each cluster consisted of 20 to 150 spasms. Do the math: 200 to 1800 three-second seizures each day. Catie’s spasms were usually around a hundred per cluster. It is easy to imagine the damage that does to an infant’s brain and in turn limiting development of the entire body.
After thirty days on ACTH we had a 25-minute EEG, the normal observation period, it showed an improvement in the hypsarrhythmia but not significantly. Dr. Vidaurre prescribed another AED called Sabril. The drug has a long list of bad side effects and required vision tests every 90 days to keep an eye on the damage to her vision. As with most medicines Catie takes, the effectiveness was limited and the spasms continued within tolerable levels – a length that didn’t require a rescue med. Catie was on a three-month ACTH schedule which ended in the middle of November 2010. At that time we returned to NCH on a scheduled stay to introduce the ketogenic diet as the next attempt at treatment.
The ketogenic diet is a high-fat, adequate-protein, low-carbohydrate diet that is used primarily to treat difficult-to-control epilepsy in children. The diet makes the body believe you are starving and instead of using glucose for energy in the brain the liver burns the fat and creates ketones which are used as the energy source. The diet greatly limits the foods that Catie was allowed. She was put on a prescription formula, ketocal, which provided the fats and proteins needed. Eventually, solid foods were added. She had a set amount that had to be measured out to the gram and mixed with canola oil. It must be followed very strictly to keep her in a state of ketosis. Once more the treatment did not greatly affect the spasms. After about six weeks on the diet we decided along with Dr. Vidaurre that it was time to explore other options to give Catie the best chance in life.
What are the Options?
We spent the Holidays with family and friends enjoying it as much as possible while still struggling with Catie’s daily seizures. A lot of people got to be around Catie and see the fight she was putting up each day. This inspired my cousin, Mike, to take initiative and help Catie and others. He established The Catie White Foundation. The CWF is a non-profit foundation to help with Catie’s ongoing medical and developmental needs, raise awareness about epilepsy and help fund research. As the cornerstone fundraiser the CWF created Catie’s Race 5k run/walk. Giving family, friends, and community a reason to think about their own health and fitness while supporting a cause and raising awareness of epilepsy. Epilepsy affects over 3 million Americans of all ages – more than multiple sclerosis, cerebral palsy, muscular dystrophy, and Parkinson’s disease combined. Almost 500 new cases of epilepsy are diagnosed every day in the United States. Epilepsy affects 50,000,000 people worldwide.
After the holidays, Dr. Vidaurre referred us to his colleague Dr. Lee at Cincinnati Children’s Hospital (CCH). We took Catie to meet with Dr. Lee and the CCH neurology team in January of 2011. We discussed the treatments and AEDs that Catie had been on and what options we had moving forward. We set up an appointment for a week long observation and testing period. Cincinnati is about a four hour drive so we had to travel the day before and spend the night in Cincinnati before the morning appointments. When we arrived at CCH Catie had the leads glued to her head for an EEG. The entire four days would be recorded on a video EEG. Along with the EEG they did an MRI and PET Scan during the stay.
We did not learn much new about Catie during our time at CCH but researched information about a surgery called a hemispherectomy. A hemispherectomy is a very invasive surgery that involves the partial removal and total disconnection of one brain hemisphere from the rest of the brain and body. Basically, that half of the brain is just filling space and the remaining hemisphere controls the entire body. The effects of this procedure vary depending on age and health of the remaining hemisphere. If the brain is still developing and growing, up to about age three, then the useable hemisphere will attempt to take over and learn all the functions needed. But there is almost certainly going to be a motor delay, resembling a slight stroke, on half the body. It will occur in the opposite side of the body from the disconnected side. The brain works across the body, right brain controls left side and vice versa, except for vision. Your occipital lobe controls the eye directly in front of it.
Our thinking going in to the test at CCH was to get the results and have a hemispherectomy done. We had been in contact via email with a renowned neurosurgeon in California who specialized in the procedure. But Catie had different plans; the PET Scan showed almost normal function in her right hemisphere and only about 20% in the left. This correlated with the MRI which showed the physical malformations. Dr. Lee offered some information about possible surgeries before discharge but needed time to study the results more thoroughly with his team.
Catie’s condition was not improving on the diet and drug combinations so we were preparing for surgery expecting to go out to California for the hemispherectomy. Dr. Lee set up a meeting for the end of January to discuss the findings from Catie’s test. We first met with Dr. Lee and the neurology team and went over the findings. The EEG corresponded with the imaging results showing multiple areas on the left hemisphere were seizures were originating. They explained that this made Catie a candidate for hemispherectomy surgery. However, the Doctor was not sure if it was left side only. There was an area of the right frontal lobe that they were suspicious of. If we chose the hemispherectomy, with the definite side effects, Catie may have seizures afterwards originating in the right hemisphere.
We then met with Dr. Mangano, CCH pediatric neurosurgeon. He had the same concerns as Dr. Lee and recommended a procedure called complete corpus callosotomy. The surgery could be used as a first step in her treatment plan. The corpus callosum is a thin band of nerves that connects the two hemispheres of the brain. It is used to transfer signals across the hemispheres. The surgery cuts the cord, essentially eliminating communication between the two hemispheres. The hope was to isolate Catie’s seizures to the left side, if that was the only point of origination. The surgery is less invasive and has far fewer negative side effects than the hemispherectomy. The doctor explained that if the seizures were left hemisphere only we had options for more surgery down the road.
The tentative date for surgery was March 9,2011. We requested Catie’s test results and then sent them to the doctor in California. His opinion was the same as the CCH doctors but wanted to do his own observation and testing before ruling out the hemispherectomy. This helped make the decision to go ahead with the corpus callosotomy. We did not want to give Catie any permanent disability with the chance the seizures were still occurring. The infantile spasms were doing enough damage without us purposely adding more. Catie was absolutely not allowed to get sick in the month before her surgery so we limited her contact with people, even postponing her 1st birthday party. The surgery was scheduled on a Wednesday morning it was suppose to take 4-6 hours and the hospital stay for recovery is about 10-14 days. The weeks of waiting and worrying were tough. It is hard to think Catie is having brain surgery and everything would be ok. The doctors tell you how safe it is with the technology and their experience, and the outcome will be an improvement but won’t stop the spasms. It is still a scary thing.
Four days before the surgery tragedy struck our family when we lost Carna’s father unexpectedly. This made a bad situation much worse. The service was held Tuesday morning and we had to leave right after to get to CCH for bloodwork. We did not get much sleep the nights before and had a long day ahead of us.
Our Baby Girl in Surgery
The morning of surgery we met family and our pastors at the hospital. We registered Catie and then waited; waiting was something we did a lot that day, to go back to pre-op. Once the IV was in and all the procedural stuff had been done our guests were allowed back in pairs for a few minutes each before Catie headed to surgery. The anesthesiologist came back and gave Catie a small oral dose of medicine to prep her and she did not handle it well. Catie was choking and having trouble breathing which created a frantic couple of minutes as different nurses and doctors tried to figure out how to help her. They got her on oxygen and regulated her breathing some but decided it would be best to take her back immediately and put her to sleep for the operation so that they could put in the breathing tube. We walked down a long hallway to the surgery wing where we had to leave Catie. We got a moment to tell her we loved her, kiss her on the cheek and then watch as she was wheeled through the doors.
Then the waiting began, we sat and talked with our family and friends passing the time and anxiously watching the patient tracker to see where they were on Catie’s procedure. There was also a call station that would page us every little bit with an update from the surgery team. The technology at CCH is incredible. The first thing they did with Catie was an MRI, and then uploaded this image into the operating room. The tools used to do the surgery are equipped with a GPS-type chip that pinpoints their location on the MRI. This assists the surgeon’s view of inside the brain and assures he is in the correct location. Finally, around two pm the nurse paged us to let us know surgery was over and the doctor wanted to talk to us. We were led into a small room with a couple of chairs and there Carna and I waited for the news.
Dr. Mangano arrived shortly and explained that everything went great. He said that the MRI helped immensely due to a slight curve in Catie’s corpus callosum that they saw and were able to be sure to cut it entirely. But the MRI also showed a small malformation deep in the brain which was not present in the January MRI. The Doctor believed it was being caused by the Sabril and that we needed to stop taking it. The good news was that the malformation would correct itself once the medicine was discontinued. He explained that Catie was doing fine in recovery and told us to go up to the intensive care unit and we could see her soon.
Once again we found ourselves sitting in a small waiting room outside the ICU. This time it was not nearly as long, the nurse paged us and let Carna and I go back to see Catie. The ICU rooms are very plain, they look and feel sterile. Catie lay in a big hospital bed with oxygen and IV lines attached. She looked to be sleeping peacefully with her head wrapped in a massive amount of gauze forming a sort of bonnet. I don’t remember how long we were in the room before Catie woke up. It seemed like we had just arrived when she began to wake. As she awoke Catie went into a seizure. Everything the doctors had said and that we read about surgery indicated that there would be seizures possible, more acute seizures for the first few days due to the trauma. This still does not help with the fear and disappointment that we felt watching her have the seizure.
Catie slept a lot the rest of that day. The doctors told us to expect 2-3 days in ICU, but Catie did not like it there. She did so well that we were moved to the neurology floor Thursday morning. When the doctors did rounds they were very impressed with her alertness and said we could be released as early as the middle of the next week. They also took the wrapping off of Catie’s head on Thursday. The thought of brain surgery was scary but until you see the incision the magnitude is not understood. Catie was cut from temple to temple in an “S” to help make scaring in her hairline less noticeable once it grew back. There were nearly 100 stiches between the internal and external sutures. Seeing the cut for the first time was numbing, yet amazingly Catie didn’t care as she was cooing and rolling around in her hospital bed.
She had two seizures on Thursday and then one about 20-second episode around 6am Friday morning. During Friday morning rounds the doctors were amazed at how well Catie was doing. The nurses came in later and taught us how to clean the stitches. Well, they taught me because Carna did not even like the look of them, much less actually touching them. Catie didn’t care. She would let me clean them without too much complaint. Catie made it the rest of the day without any seizures. When the doctors made rounds Saturday they gave us the opportunity to take Catie home. Through her strength and the grace of God, Catie was released less than 72 hours after brain surgery.
Catie did not have a seizure on Sunday or Monday or Tuesday or at all that first week after surgery. The doctor couldn’t explain it. Maybe it was the swelling of the brain or possibly the new medicine. Hopefully the procedure had some unforeseen affect. It didn’t matter to us why they were gone just that Catie wasn’t having seizures was enough for us. It did not take long with the seizures gone to start seeing Catie’s personality. She was finally alert and looking around, acknowledging her surroundings and she even began to smile. I am sure every parent enjoys their child’s smile but until you have lost it and went months without seeing it you can not understand what such a simple thing means. Catie had been in physical and developmental therapy since she was about five months old. A lot of the early therapy sessions were just positioning because of her lack of alertness and muscle tone. Now that the seizures were gone Catie was improving very quickly, the high muscle tone which is usually associated with a stroke was disappearing, and low muscle tone can be overcome through exercises. Catie was rolling consecutively, and constantly gaining core strength. Along with her physical improvements she also was regaining some of her cognitive abilities. Catie was beginning to track objects and people as well as focus her attention for short periods of time.
We took Catie back to CCH about a month later to see the neurology team and have her stitches removed. They were amazed that Catie had not had a seizure since we left the hospital. The stitches were removed and the doctors at CCH decided that Catie was doing well enough to transfer our care back to Nationwide Children’s Hospital in Columbus. We were happy to be back with Dr. Vidaurre, plus it saved us two hours of drive time. That month without seizures was great for Catie’s development and for our peace of mind. It was wonderful not waiting for the next cluster or dreading when she woke up from a nap because a seizure was going to follow. Catie made it almost six weeks after surgery without a seizure. Then they returned.
The clusters of seizures were short and spread out at first but the longer they continued the worse they became. Dr. Vidaurre scheduled a twenty-five minute EEG to get an idea of what was going on to cause the reemergence of seizures. The EEG was scheduled on a Tuesday at a satellite office in Columbus. I sat in the room with Catie during the test and looking at the EEG I knew it was different, but there were still spikes occurring. We missed a call from Dr. Vidaurre. He mentioned that the EEG showed activity but was better. At about 9:30 pm that night we received another call from Dr. Vidaurre. The doctor said he was at home and couldn’t stop thinking about Catie’s EEG so he pulled up her records to check them one more time and felt it was necessary to call us. He started out by explaining that there were spikes in the left occipital lobe which explained the type of seizure that Catie was having. As he continued I could sense the excitement in his voice and he said, “the hypsarrhythmia is gone! The EEG shows no hypsarrhythmia, this surgery is not supposed to stop this. It doesn’t stop them. I have not ever seen it stop the hypsarrhythmia. It’s a miracle from God!”
We spoke a short time more but I was fighting my emotions and my mind was racing. I got off the phone and went to the bedroom where Carna was laying with Catie. Carna asked what was wrong and I told her the great news we had just received. We were ecstatic. It was such a relief to hear the hypsarrhythmia, which is the cause of the infantile spasms, was gone. The infantile spasms are a terrible disease that ravages the brain continuously. We called our family and passed along the good news even though the seizures were back the battle with IS was over for now.
The first annual Catie’s Race 5k run/walk was held June 18, 2011 at Brady’s Run Park in Beaver, PA. The event was a great success with over 150 registered participants and a group of strong sponsors. It is amazing to see the time and effort people put forth to support Catie and help raise epilepsy awareness. Catie even hung out near the finish line to greet everyone as they finished.
Once again we were adding new AEDs and adjusting the dosages of the existing medicines. At first we saw a decrease in seizures but as the new drug, Vimpat, was increase so did the seizures. We continued working with the medicines till our next appointment with Dr. Vidaurre. At this visit we explained our concerns with the Vimpat and showed him a video of Catie having a seizure. The doctor was not happy with the seizure and immediately began to wean Catie off of the Vimpat. He explained that it is a good drug for a very specific focal seizure but it has negative effects on other types of epilepsy.
In October of 2011 Catie’s seizures began to increase in length, lasting 5-8 minutes per episode. On Saturday the 8th Catie had a seizure last 15 minutes and for the first time in 14 months she had to be given Diastat. This was a very discouraging sign. We contacted the doctors at NCH and they scheduled a long-term EEG for the following week. This stay gave us a good opportunity to capture Catie’s seizures on EEG and get a MRI. It would take a few weeks to receive the analysis of all the data but the initial change is adding a new AED, Clobazam. Clobazam was not FDA approved in the United States so we ordered it from a pharmacy in Toronto, Canada. It took two weeks to receive the medication. We received the Clobazam on the last Monday in October and found out mid week that the FDA had approved the Medication for use in the U.S. for early 2012.
It took nearly a month for the results of the October test to be completed. The EEG results showed that there was seizure activity in both hemispheres of the brain. They could not conclude which hemisphere the seizures were originating in due to the fact that they were quickly synchronizing across the entire brain. Basically, this meant that Catie was having infantile spasms again. The hypsarrhythmia was not back but the synchronizing of the hemispheres was a bad sign. The MRI proved the EEG suspicions to be correct, the first callosotomy surgery missed a small piece of the corpus callosum. After a few meetings with the neurology doctors we came to the conclusion that another surgery to severe the remaining connection was our best option. We met Dr. Grondon, the NCH Epilepsy Neurosurgeon, in Mid-December 2011 for a consultation. Dr. Grondon explained the MRI, comparing the before surgery and after surgery images that showed the remaining callosom. We decided to go ahead with the surgery expecting a January 2012 procedure date. The Doctor went to meet with his scheduling secretary and they returned abruptly to ask if we were available around the Holidays. The surgery was scheduled on December 27th. Which worked out great since Logan would be on Christmas break and not have to miss any school.
The day of surgery arrived quickly and hectically just two days after Christmas. The pre-op went much smoother this time but there were more worries. There was an extra issue of concern due to scar tissue that forms after the first surgery. Unfortunately, this fear came to light early in the surgery. The doctor used the same incision on Catie’s head to gain access to her skull. Once inside the skull he encountered the scar tissue. While cutting through the tough scar tissue a capillary connected to the carotid artery in the right hemisphere of the brain was torn. He had to quickly cauterize the capillary to stop the bleeding before he could continue the surgical procedure. If it would have ripped even a small hole in the artery Catie would have had an aneurism on the operating table and there is no possibility of the doctors saving her. Once inside he disconnected the corpus callosom then did a MRI to make sure it was completely gone before closing up the incisions. Catie lost a little bit of blood and had a low blood count but her body made enough overnight to prevent having a transfusion. She was in the ICU overnight and only spent a total of four days at the hospital. The surgery did not give us the seizure freedom that the first one did but it seemed to isolate the seizure transfer. Most of Catie’s seizures seemed isolated to the left hemisphere. During her clusters of seizures Catie would jerk her head back to the left, roll her eyes and stiffen her right arm. This gave us hope that the procedure had worked in isolating them to the left hemisphere.
The next few months were tough on Catie from a health standpoint overall not just her seizures. She spent a week in NCH with a Gastro-intestinal virus which dehydrated her. As normal Catie was anything but by the books, most epilepsy patient’s experience more frequent or severe seizures while dehydrated but Catie has fewer some days even no clusters when dehydrated. We were lucky to get to stay on the Neurology floor for the week and discuss Catie’s future with her Doctors. We decided to try a steroid treatment once Catie got over her illness. Catie had responded well to steroids in the past and we looked forward to an opportunity to try the prednisone therapy. An added benefit of the steroids was that they interfered with the Ketogenic Diet giving us a chance to wean Catie off the fat based diet. We did not feel that Catie was benefitting from the diet and welcomed the switch back to normal foods. Unfortunately, we found out otherwise. Once Catie dropped out of ketosis the seizures began to increase in frequency, as the prednisone increased and her body got use to the new diet the clusters stayed around 6-8 a day.
March Madness meant more than just lots of college basketball in our house. Catie was finishing up the prednisone after very little improvement and we were preparing for a family vacation to Disney World. Catie needed special accommodations and medical documentation for the flight and stay away from home. With assistance from Dr. Vidaurre we obtained the cell phone number of Dr. Lee, who treated Catie at CCH and had transferred to Orlando, in case of an emergency. About two weeks before departure Catie began showing signs of a cold, cough and runny nose. This was not a common cold though it quickly worsened and we found ourselves headed to NCH for treatment. Catie was diagnosed with RSV, which she contracted from daycare, along with her entire class. What they said would be a day or so inpatient turned into a long scary week. Epilepsy babies seem to be more susceptible to respiratory illness and have a hard time fighting them, often leading to pneumonia. Catie had a difficult stretch in the middle of the week, in which she needed oxygen to help her breath. It was very hard watching her struggle to breathe and her condition would change rapidly. Twice during the week a doctor told us we could be released during their morning check but by the time the team of doctors made rounds Catie would be laboring to breathe. Finally, six days later we were able to go home and just in time to pack for Disney. We had a great time with our family at Disney World and the park was excellent in meeting all of our request for Catie’s needs. While in Orlando we spent a day at Sea World and had an opportunity to meet a family from the Facebook Infantile Spasm Group. It was nice to meet a family with a little girl who understands what you are dealing with everyday. That same day at Sea World I received a call from Cleveland Clinic to set up a Long Term Monitoring and Testing session for the end of April.
We headed to Cleveland Clinic with anticipation of getting answers and possibly a recommendation for another surgery to improve Catie’s outcome. We went to Cleveland hoping to get an answer on a possible Hemispherectomy Surgery. The week began with Catie being hooked up to the EEG leads for the video monitoring. Each day had a different test schedule, an MRI, PET Scan, and MPEG. They were able to collect a lot of good data through the test and during Wednesday morning rounds the doctor gave us a diagnosis that changed our lives, again. Catie was diagnosed with Aicardi Syndrome, a rare genetic mutation that only affects Females. The doctor scheduled Catie to have an eye exam to confirm his suspicions. We spent the day researching Aicardi Syndrome and trying to understand it. This was the beginning of a very emotional few days. The ophthalmologist identified the lacunae, unformed spots on the eye, which confirmed the diagnosis. Aicardi Syndrome is identified by three distinct characteristics: agenesis corpus callosum, lacunae or lesions of the retina and infantile spasms. These are all problems which we knew Catie had but now they were grouped into a “terminal” diagnoses with scary statistics attached. There are about 800 Aicardi Princesses in the U.S. and a couple thousand worldwide. The average life expectancy is 7-14yrs of age and the developmental outcome is just as bleak, most of the girls never walk or talk.
We returned to Cleveland Clinic June 1st 2012 to get the official test results from Catie’s neurologist. As expected, the diagnosis was Aicardi Syndrome with no chance of surgery for help due to seizure activity in both hemispheres of the brain. Unfortunately, the news got worse the MRI showed signs of atrophy in Catie’s Left Hemisphere. Cerebral Atrophy is a condition in which cells in the brain are lost. The seizures are slowly killing the cells and connections in Catie’s brain and will progressively grow worse over time causing more delays in brain function. Finally, we received the hardest news of the visit, in the opinion of the neurologist Catie’s seizures are uncontrollable and the best we can hope for is a 10-20% reduction. We’d never had a doctor tell us there was no hope for Catie. Every prior visit ended with a plan or idea to achieve seizure freedom. We left Cleveland very disappointed but if Catie has taught us anything through this journey it is that she is tough and handles things better than we do.
This was just the start of a crazy month. Catie was changing meds, increasing Depakote and weaning off of Zonegran, when she decided she did not want to eat solid foods very well. We continued trying to feed her as we prepared for the 2nd annual Catie’s Race. Catie’s Race 2011 was a huge success as the cornerstone fundraiser for the Catie White Foundation and created big expectations for the 2nd installment. Catie’s Race 2012 gained momentum quickly as the date approached and by race day had nearly 300 participants. It was a beautiful day and an excellent event pulled off by the entire Catie’s Race team of volunteers. The Race raised enough money for the CWF to create the Catie White Epilepsy Assistance Grant at Nationwide Children’s Hospital in Columbus, OH. It is the first ever grant at NCH dedicated solely to Epilepsy patients. However, during this time Catie continued struggling to eat and was now refusing her bottles as well. Catie lost about 3 lbs in 6 weeks and after multiple calls and visits to the doctor she was referred to a GI Specialist at NCH.
We took Catie to her GI appointment on a Thursday, we really liked the Doctor and her enthusiastic plan for Catie but she was concerned with Catie’s current state of health. Catie was barely eating and on the verge of dehydration, the doctor ordered some blood test and asked us to stay in Columbus till the results were in. The test came back with some low results but not enough to admit Catie. We were sent home and told to take Catie in to our pediatrician the next day to be checked again. We never made the pediatrician appointment on Friday; Catie had finally starved herself into dehydration. So, we dropped Logan off with his Grandparents along the way and headed to NCH expecting some fluids and a weekend stay at the Hospital. As we arrived at NCH the sky over Columbus looked very ominous, Carna had me stop at the entry to the parking garage so she could snap a photo. We got out of the car and heard an incredible racket echoing through the garage. As we walked to the elevator we saw the wind throwing tables and chairs from the outdoor cafeteria and trees bowing to the wind and rain. The storm outside was quickly forgotten as we began the admission and evaluation period in the ER. As expected IV fluids were started and Catie was admitted to the GI Unit. Then the calls and text began coming in describing the damage from the Derecho wind storm that hand torn through our home town on its way from Chicago to D.C. and the Atlantic coast. Our home survived with only minor cosmetic damage and a few days without power. Thankfully family and neighbors took care of things while we were gone. Catie still refused to take any nourishment by mouth and on Saturday June 30, 2012 the nurses inserted a NG Tube. The NG Tube is a small flexible tube passed through Catie’s nostril, down her throat and into the stomach. She did not like the tube taped to her face or tickling her throat which caused her to cough and gag. She did good as they slowly increased her feeds overnight but the next morning she began vomiting. In typical Catie fashion this was going to be a struggle and meant we would spend the 4th of July in the hospital. Doctors tried different formulas, rates of feed, and medicines over the weekend trying to prevent the vomiting with no luck. They scheduled a litany of test for the upcoming week in an attempt to find an answer.
On Monday, doctors attempted to do a video swallow test, which of course did not work out since Catie refused to eat by mouth, so they moved her NG tube down through her stomach and in to the small intestine , NJ tube, in a effort to control the puking. This made no difference in Catie’s condition. Tuesday brought another test, an upper GI scope, and another day of unanswered questions. Wednesday was the fourth of July which meant no Doctor’s and no test. We spent most of the day researching Catie’s symptoms and picking the brains of fellow seizure families on the internet. This led us to a few medications that could help and reaffirmed our belief that depakote may be contributing to the sickness. Another thing we learned, from Aicardi Families, is that it is very common for Aicardi girls to stop eating orally between two and three years of age. We did have an awesome view of the city of Columbus and the surrounding horizon from our 10th floor window so Carna and I had an array of firework shows to watch counting at least five simultaneously at one point in the night. Over the next few days we worked with doctor’s from GI and Neuro to introduce carnitine and erythromycin to help calm Catie’s stomach as well as setting up a weaning schedule to get the depakote removed. After another weekend at NCH, we spent Monday learning how to care for Catie’s NJ Tube. Catie was sent home with the NJ Tube while we waited to meet with the surgeon about a permanent feeding tube.
July also brought about our first chance to meet other Aicardi families. We attended the Aicardi Syndrome Foundation Conference in St. Louis, MO. This was an incredible experience for our family. We were one of 95 families to attend who had girls with Aicardi from 4 months up to 21 years old and the developmental ranges were just as wide. It was encouraging to see girls who could walk and communicate and hear the stories of them learning on their own terms. One friend we made did not sit on her own till 4 years old and was now running around at 7. It gave us so much hope and perspective. This is a daily battle that most families cannot relate to but these people truly understand. The connection with the people was awesome it was as if they were old friends or distant family. The conference was also very informative. We learned a lot about meeting Catie’s needs with equipment, therapies and medications. It was an excellent experience and we headed home with a renewed energy to continue Catie’s journey.
Catie had her surgery to place the Mic-Key Button feeding port in her stomach in August. She did great with the surgery but when she woke up the following day she was not happy. Catie has had two brain surgeries and recovered quickly and easily. Apparently, the G-tube surgery was much more painful. Catie was very cranky and scared us to death by holding her breath until her lips would turn blue. They placed her on a morphine drip for two days to help with the pain. We now had a safe way to ensure Catie received the nutrition she needed and we soon realized how little she was eating. Catie gained 6 pounds in five weeks on the tube. The feed rates were adjusted to keep her at a steady growth rate and she has adjusted well to the tube. The best thing to come from the g tube surgery was home health nursing care. Once Catie got the NJ tube no daycare center would accept her. We were fortunate that our insurance covered the nursing and with Catie staying at home she avoids all the germs and illnesses which she battled the previous winter.
Catie was gradually increased to bolus feeds, large amounts at a time, and we eventually put her on a blenderized diet. She sees a speech therapist weekly to try and get her to eat by mouth with little improvement. We also ordered her a wheelchair and other adaptive equipment to help with her strength and mobility around this time. It was tough to process the fact that Catie was going to be in a wheelchair but it is for her benefit and ours. As she was growing and it was becoming increasingly difficult to transport Catie even within our own home. The wheelchair creates a whole new set of problems which forced us into getting a van for transportation and creates the need for a single level home. As for Catie’s seizures, she continued struggling with increasing clusters. We began exploring the Ketogenic diet again and another medicine to stop the progression of the seizures as well as once again looking at surgery as a possibility of reducing them.
The next few months were spent connecting with doctors from Orlando to Detroit to Los Angeles and after discussions it was once again concluded that none of them were willing to operate on Catie. This may have shut the door on the surgery option for good so we started looking for a medicine that we hadn’t tried. Unfortunately, the few AEDs Catie had not been on previously were for more specific types of Epilepsy and also not an option. This left us with the Ketogenic Diet for a second run. We returned to NCH in January 2013 and spent four days at the hospital to implement the diet. Catie continued with the blenderized diet but adjusted it to fit the strict and fatty needs of the keto diet. For the first 2-3 weeks we saw small improvements but then Catie’s seizure clusters went back to the before diet rate of 8-10 clusters per day. After fighting the rollercoaster of Ketosis from 160 – 40 ketones on any given day and no seizure reduction we began looking at an adjunct treatment called the Vagus Nerve Stimulator or VNS.
We had reluctantly talked about the VNS on many previous occasions with different members of the Neurology team at NCH and always had a better plan come about. But in April 2013, there was no medicine or surgery or other option. As always we did our research and probed the Aicardi families for input on the VNS and a majority of them saw enough of a benefit that they would do it again. So, once again we were going to put our princess through her fourth surgery in 2 years in hope of seizure control.
The VNS consist of a small oval stimulator about the size of a thumb and a ¼ of an inch thick that is placed under the skin on the left side of the chest. There are tiny wires run below the skin along the collar bone and then wrapped around the vagus nerve in the side of the neck. It is a simple outpatient surgery involving an incision in the middle of the chest and another small cut on the neck. As always Catie did excellent during surgery and we got to go home that night. Unfortunately, the VNS could not be activated till the nerve that had been exposed to receive the wires had some time to heal.
After a few weeks we returned to NCH to activate the VNS. Once turned on the stimulator sends an electrical pulse to the nerve for 30 seconds at a time every 5 minutes. We also received a magnet that turns the stimulator on for 1 full minute by swiping it over the VNS during a seizure or before if we think one is going to begin. Turning the VNS on is a very long process requiring us to travel to Columbus every other week to increase the shock wave by .25 until we find a level that is both helpful and tolerable to Catie. We saw small improvements after each adjustment for a day or two then returned to her normal 8-12 clusters of epileptic spasms per day. The VNS did make improvements in Catie’s mood and behavior. She gradually became much more alert, aware and connected with her surroundings. The personality that was pent up began to show up in looks, squeals, and smiles. The attitude that she has used to fight through all the long hospital stays, surgeries, and test also came out. If Catie does not like something she has her ways to let you know. Although, the seizures were not significantly reduced the VNS has been worth it to get these changes in behavior.
Once again we were looking for the next new AED or treatment to help get some relief from the daily barrage of seizures on Catie’s body and brain. In mid August 2013 we decided there was no benefit from the Ketogenic Diet and began the slow wean back to a normal blenderized diet. During the summer we began learning about and researching Medical Marijuana that was being used with promising results in children with intractable epilepsy. The obstacle that arose was that medical marijuana is not available in our state or any nearby. People hear the term marijuana and think it’s just a drug to get people stoned, but in reality the medicine, CBD, made from the plant to treat epilepsy does not get them high. The chemical THC, which creates the high, is only in trace amounts in the CBD which is a black tarry liquid is given by syringe. The cases of children with epilepsy and more specifically epileptic spasms are few but growing so there is not a large field of studies or results to look at. We know of one Infantile Spasm girl who has moved to Colorado and had incredible results in both seizure control and her development. There is also an Aicardi Family that made the move to CO and is working to get the CBD treatment. This may be the next or only option left to help find some kind of seizure control for Catie.
Catie continues to battle everyday with a disease that is slowly destroying her brain a seizure at a time. As hard as it is to think about these outcomes we try our best to look at every small accomplishment and continue to work to give Catie the best life possible. We enjoy every smile, every noise, and every opportunity to expose Catie to a new or different experience. We will keep fighting as hard as Catie does each day to spread awareness of epilepsy and its many disorders like Aicardi Syndrome. We will always be Chasing the Cure!