Mom's Journal, MAY 2009

- May started out beautifully. I was invited to be “Mommy Helper” with Hannah’s kindergarten class on a visit to the Calgary Zoo. We had a fantastic time and Hannah and I enjoyed a great day together.

- Hannah’s antibiotics for her strep throat and ear infection were complete on May 4. I was worried that the infection wasn’t completely gone, because the frequency of the seizures was still quite bad, so I took Hannah to our family doctor for a check-up. He said that her ears and her throat looked clear, so we again had no explanation for the 4-5 tonic seizures per week that she was having.

- Seizures are still occurring rather frequently and Hannah is experiencing a lot more tonic seizures as she falls asleep and in the early hours of the morning. If she has more than one in a row, she tends to go into non-convulsive status following the second tonic and this requires Ativan to stop. The Ativan is getting to be incredibly frustrating. Hannah’s baby teeth are falling out and being replaced by adult chompers. Now that she has bigger teeth, it is virtually impossible to slip the Ativan into her mouth when her teeth are clenched. We were told to put it in her cheek but it doesn’t dissolve because she is so dry or else she has so much saliva that it comes back out during the seizure. If the Ativan does make it into her system, it takes forever to work. I contacted the hospital and voiced my concerns and they indicated that they are trying to schedule Hannah for an admission for prolonged video EEG monitoring and did not want to change anything until after the EEG. So we wait.

- Hannah had IVIG on May 5 and it went reasonably well. She was very tired and didn’t have a lot of energy afterwards and that night she had a series of seizures that again required Ativan. This continued for the next few days with Ativan every day.

- The weekend of May 15th, 16th and 17th was the worst. Hannah was having frequent absence seizures, head drops, and tonic seizures and the emergency medications were just not working fast enough or for a long enough duration to combat them. On the 19th she was admitted to the hospital for assessment and video EEG. As soon as the EEG was attached I could tell that it was bad. I am not great at reading them, but over the past few years, I have learned some of the fundamentals of EEG interpretation, or at least enough to know “yuck versus yippee”. Her EEG was chaotic and she had very frequent absence seizures and epileptiform spikes everywhere. Luckily there were no bullets to swallow at the hospital, or freight trains to lay in front of in the parking lot, so I was forced to accept that her EEG was once again horrible.

- Hannah was moved to the unit and we settled in for a long 2 days of trying to keep a very active little girl in her bed. She can’t get up and move around because she is pretty much tethered to the wall with wires glued to her head. Mommy was a one woman show, doing a dance here, a song there, pretty much standing on my head to entertain her. After the first couple of hours, mom was not very entertaining and Hannah was bored senseless.

- We went to bed early, and I cuddled up beside her in her tiny little hospital bed. She had a tonic seizure right after she fell asleep and then was postictal for a few hours. At 2:00am I felt a little finger poking my back and woke up to my poor little girl crying. I asked what was wrong and she was very upset complaining that her lips were hurting. Hannah doesn’t feel pain appropriately, so I was trying to figure out what she was trying to tell me. Then she yawned and screamed and covered her ear. Okay, that I got! She had another ear infection! What the heck is with these ear infections! She seems to get so many lately, and they absolutely dump the poor kid and seizures take hold with fury. I poked my head out the door and tracked down her nurse to report the infection. I gave Hannah a Tylenol for the pain and the nurse came in to assess her.

- I’m going to vent again.....

- I asked her to page the doctor because this is the reason for all of the increase in seizures. We always lose control of Hannah’s seizures when she is sick. Well..... are you ready for this? The nurse told me that neurology would be “in” after 8 am to see Hannah and there was nothing that could be done tonight because there was no neurologists in the hospital. What a load of crap!!!! I work for EMS and I transport patients to this hospital on a regular basis. If we bring in a child with a head injury, there is a neurologist waiting for them. If a child has status seizures, we are met in the emergency department by neurology! This is ridiculous. The nurse left the room...mommy stewed for a few minutes and then I totally lost my mind. Yes... I became one of those moms that all nurses despise! I rang the emergency button at the head of Hannah’s bed and the nurse came running in. I told her that I wanted a doctor, any doctor, to see Hannah right now. She told me that because Hannah was admitted to neurology for monitoring, that no doctor would come in to see her and that we would have to wait until the morning. I looked at her and said.....”Are you telling me that my daughter is admitted to the Alberta Children’s Hospital and there isn’t a god damn doctor that will come and see her when she is sick and in pain?”. She walked out! I was absolutely livid. I pushed the button again and said, “Unhook her from this EEG and I will carry her down to emergency so she can get treated!”. Within 5 minutes a paediatrician was in the room. He was actually wonderful. He was very very kind to Hannah and he gave her a thorough exam. Sure enough, she had a horrible ear infection and a sinus infection. He thinks that the sinus infection had been plaguing her for some time and this might have been the reason for the significant increase in seizures. So there we were, back on antibiotics, only this time on double the dose for twice as long. Maybe she will finally be able to fight off this endless infection. She couldn’t sleep and we sat up together until 6 am. We finally fell asleep only to be awakened by a student nurse at 7:30 am who wanted to assess Hannah’s vital signs and level of consciousness. With one firm, “No....go away”, she quickly left. Mom is a bear sometimes! We slept together until 10 am.

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- The second EEG day was better because Hannah got some visitors to entertain her. My good friend came with her daughter and her son, Grandma came, and then Sparkle the clown came and painted Hannah’s face! That night was “Pet Night” in the hospital, and the lovely Child Life specialist arranged for a service dog to visit Hannah in her room. Wow!!! Was she ever excited! It was wonderful to see Hannah light up when this very special dog arrived. I can’t wait for her to get her own service dog in December! It will be life changing for all of us. Watching this dog lay in Hannah’s hospital bed illustrated another point.....we need a bigger bed!!! Haha. He took up ¾ of her hospital bed. We are going to need to move Hannah into a double bed for sure when she goes back to her own room after the dog comes home.

- Our neurologist came in on Thursday afternoon and broke the news to us that we were already expecting. Hannah’s EEG is much worse (although she is sick). She is having very frequent absence, atypical absence and complex partial seizures that are very difficult to detect with the naked eye. These are often called, “subclinical”. She also had the three tonic seizures. He changed her emergency medication from Ativan to intranasal midazolam because Midazolam is stronger, and has a quicker onset than Ativan. He arranged for Oxygen to be installed in our home because her oxygen saturations drop after seizures and this can increase brain damage. He ordered a repeat MRI to see if there has been any change from the previous 2 MRI’s that she has had. He is looking for atrophy or areas of dysgenesis in the brain. He also ordered a muscle biopsy to complete the full metabolic/genetic screen from 2 years ago.

- We talked at length about where to go from here. The sad reality is that there is nothing left for Hannah. There is only one medication remaining that might work for her, called Rufinamide, but we can’t get it in Canada. Steve and I have been all over this, emailing everyone in the pharmaceutical company to try to access this medication on a compassionate protocol. I will keep you posted. The sad reality is that when this medication was first being marketed in Germany, we received an email from the pharmaceutical company asking if they could use some of her pictures from this website for their patient care guide. We agreed and she is now the child in their book, but she can’t get the medication. Life is so bitterly ironic sometimes!!!!!

- I spoke with a pediatric neurosurgeon in Ontario and there is a possibility that Hannah might be a candidate for a much more invasive treatment called a Vagus Nerve Stimulator (VNS). I have been pulling every study and clinical trial that I can get my hands on about the VNS. This was never offered as a treatment option for us because Hannah’s seizures are global but it has been shown to be effective in some children with Lennox Gastaut Syndrome. The VNS is like a pacemaker implanted in the chest that sends an electrical current to the left vagus nerve when a seizure occurs, essentially counter-shocking the nerve at the time of the seizure. This can interrupt the seizure and stop it. The problem with LGS is that there is so many seizure foci that the VNS couldn’t possibly fire with every seizure so the sensitivity would have to be tweaked to only activate on certain types of seizures like tonic or atonic. We are waiting for the MRI results before we can pursue this any further.

- It is pretty clear from Hannah’s clinical presentation and from her EEG that Valproic acid actually worsens her absence seizures, so this medication is no longer working for her that way. The problem is that there is nothing left, so we are all reluctant to completely pull this medication. We are leaving it alone for now. The doctor wants us to increase her ketone level through the night so her Ketogenic diet ratio had to be changed to incorporate more fat and less carbohydrate. Hopefully this will help with the night time seizures.

- We are heartbroken and for the first time in a long time, we feel like we are losing the battle. The only ray of light right now is that she was very ill and had two infections at the time of the EEG, so maybe it wouldn’t have been quite so bad if she had been healthy at the time. We are hoping that they will repeat the EEG sooner than the standard one year time frame that we have grown accustomed to.

- We asked to be released from the hospital when the tests were complete because Hannah’s birthday party was the next day. Sometimes you just have to put the bad days away, and focus on something good. Hannah invited 14 friends to her 6th birthday and she had a wonderful time.

- Grandma and Grandpa bought her the scooter that she had been wanting for over a year and she was absolutely delighted, she squealed “All of my wishes come’d true!!!”, when she opened it. Grandpa and Grandma (aka Captain and Mrs.Caution) bought her elbow pads, knee pads, and wrist guards, to be worn with her bike helmet that she already had. She will be very safe now!

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- Hannah had IVIG again on the 26th of May, and she slept through half of it....even the poke??? Tough little girl!

- On Wednesday May 27th, we had a very special event!!! Hannah’s kindergarten class was invited to a special wedding. Her super cool kindergarten teacher planned a fancy wedding for the letters “Q” and “U” to be joined together in reading and writing from this day forward, as long as they both shall read. It was so sweet. The teacher picked one boy to be “Mr. Q” and one girl to be “Mrs. U”, and guess who was “Mrs.U”??? Miss Hannah! She was a beautiful bride, in a long white flowing sundress, a tierra, white gloves, and ringlets in her hair! Mr. Q was quite smitten with his new wife and the ceremony was lovely. At the reception, all of the parents and children enjoyed sparkling apple juice and wedding cake (Hannah had rice, hemp hearts and water but it was still lovely).

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- Friday and Saturday Hannah had a series of seizures and required Ativan both days. Sunday was a good day!

- Hannah and Connor and I went to my good friend’s family farm to visit the brand new baby horses that were born over the past month. What an awesome day! Kalen was playing baseball with Daddy in Lacombe, but Connor and Hannah were able to spend the day on a real farm!!!! How exciting!! Both Connor and Hannah were invited to ride a beautiful horse named Simon. Hannah was able to ride independently as she was lead around the field. The smile on her face was priceless. Connor was over the moon!

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- When it was time to get off the horse, Hannah layed on the grass and talked to the horse for a few minutes. It was so sweet to see her have a little conversation with such a huge animal. Within a few minutes, the horse walked over to Hannah and you will not believe what happened........okay, I just have to show you.......Hannah the Horse Whisperer!

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- Daddy and Kalen arrived home safely from their weekend of baseball with only one small mishap. While warming up by playing catch before game 3, Daddy threw the ball to Kalen, who looked away for a split second to talk to another player ......opps... crash....ouch!! Poor Kalen! Still smiling! When people ask what happened to his eye he likes to say.....”That’s nothing, you should see the other guy!”. Haha.

- We are looking forward to June! Only 3 more weeks of school and then we are on summer vacation. Yippee! We are hoping to enjoy lots of camping this summer, if the weather cooperates.

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