Ears...

Since the hospitalization in January, Avery had been recovering  very well, and near 100% again. Up until a few weeks back, when she got an ear infection, causing a fever ,which triggered a seizure sending Avery via ambulance to the ER for the afternoon. Unfortunately this is to typical for Dravet kids. A simple illness or infection turns into a life threatening seizure for them, and nightmares for the parents. A worry we have every minute of every day. This is just another day in our lives...

This is the 2nd ear infection this year, and warranted a prompt visit to a ENT specialist. He quickly assessed that Avery would benefit from getting tubes in her ears, and we scheduled an appointment for surgery to be this morning.

  The surgery was at 8am, and went very well. We were able to be home by lunchtime. Avery's a bit groggy, hungry, and sleeping quite a bit, but doing OK. Overall she recovering as expected, and we are hopeful she continues with no seizures or complications.  Thanks for your prayers.

Pneumonia and More...

Avery's Pneumonia continued through the month January. On Weds, January 25th Debbie took Avery into the clinic to get checked out. Avery's condition had been getting worse, as the previous weekend she starting sleeping more of the day, and cough half the night. The antibiotics she was on, weren't doing anything for her. I got a call from Debbie, that the Dr. said Avery will need to be hospitalized, as her O2's were too low. So off to Gillette she went. When Avery got there, she was running a temp as well. She had been running a fever off and on all month. We'd give her advil for a few days, and it would go away, then a few days later fever again, and repeat... So, Advil was started in addition to IV antibiotics and nebulizations.


Avery hasn't lost her appetite!

By Thursday afternoon the fever was gone, and Advil doses were ceased. Friday morning came, and with that a fever again! Avery got a Nebulizer dose of Albuterol at 8am, and was in the middle of her morning meds when her temp spiked, and she began to seize. Debbie quickly called for a nurse, and administered 2 diastats with no effect. They then pushed Ativan via IV also with no effect, but with Avery's lungs still in rough shape from pneumonia, her lips started turning blue, and her O2 stats dropped. They quickly rushed her to PICU to intubate and sedate her, requiring her breathing on a vent. After 55 minutes, the seizure was finally stopped.




Avery on vent with her puppies.

 Avery remained on the vent for 48 hours until Sunday afternoon. She was extubated, but was still very tired, so she slept the rest of the day. But woke up for a bit when Debbie got to hold her in the rocking chair.

Avery continued to come around a little more each day. On Tuesday, I spent most of the day with her while Debbie took a break from the hospital and went to work for the evening. To Avery's surprise, the Music Therapist stopped in for a visit, and she loved every minute she was there.



Avery playing with Coral the Music Therapist



Dr. Avery Checking out her puppy

Finally on Weds. after she had been off O2 for a few days, and breathing well on her own, she was doing well enough to go home. Yeah!!! Avery was all smiles the drive home, and we decided to pick up Nick from school early on the way home.




Happy to see each other!

 Thank you everyone for your thoughts and prayers for Avery while she was in hospital. We are thankful to be all be under one roof again.

Happy New Year!

Hope everyone had a merry Christmas and Happy New Year! Avery had a great Christmas with both sides of our families. Avery received a number of generous gifts, and she's enjoyed all of them. Thank you.
She's especially proud  of her pink guitar.



Avery playing a song on her guitar

 Avery absolutely loves music, her singing and playing musical instruments has certainly struck a note as the house has been filled with her singing and playing songs. A future musician?

New Years Eve was spent with the Franklin side of the my family at Renae and Bob's. Always a great seeing everyone and spending time with all of the family. Avery was having a ball playing with the other cousins. She also couldn't stop looking out the window, watching the dogs.

Later that night, it was clear Avery was coming down with something. As she was sleeping, she was "shivering", I quickly grabbed the thermometer, and checked her temp, Normal, waited a minute, checked it again, normal. Seeing her "shiver" quickly made us think of last February, when she was doing this same thing in the middle of the night, same "normal" temp, but minutes later turned into a generalized tonic clonic seizure, calling for 2 Diastat's, and a ambulance ride to the Hastings ER. Which they then transferred her up to Regions ER for the rest of the night. Needless to say, when we saw her shivering, we were immediately on edge! In efforts to prevent it evolving into a seizure, we gave her some Advil to help prevent a fever, and oral Valium to help prevent a seizure. It worked! She slept the rest of the night, and we felt like we dodged a big one there.

That same night we happened to be getting a few snowflakes for once this winter, enough that I went out snowplowing in the morning. But when I got home later in the morning, Debbie said Avery was lethargic all morning, and not herself. She was way "off", something was up. I took her into the clinic, only to find out it was closed being new years day, So, we headed over to the ER. Once we got in the ER to get checked out, she was already running a temp. Dr. evaluation proved she had a bad Ear Infection, as well as a chest infection! Dr. gave her antibiotics to cover both to avoid an X-ray. A dose of Tylenol, and we we headed home.

Avery seemed to be responding well to the antibiotics all week, as she was getting better each day. She still had a bit of a cough, but seemed to be getting in the clear, or so we thought. Today, Avery got home from school, and we were told she was "off" all morning in school, poor balance, and frequent eye closing head nod seizure activity. Debbie got her into the clinic, and this time they did an evaluation and X-Ray. Good news- Ear infection is gone, Bad news- She has Pneumonia in her right lung, Here we go again...

On a positive note, our friends Brian and Amanda Meyers welcomed a beautiful baby girl into their family last week. Baby Klaire is home now, spending time with her big brothers, Kael and Kyler. Congratulations to them!

Happy 5th Birthday Avery!

It's hard to believe Avery had her 5th birthday on December 15th. We had a birthday party for her the Sunday before with Family and Friends. It was a great turnout and good time by all, especially for Avery, she couldn't stop running around with all the kids, She was having a blast every minute of it.



Avery 5 years

She's such a big girl now, potty training is in full swing again, and Avery seems to be making good strides in getting there, lately she's been going on the potty 1-2 times a day with some prompting, and we celebrate each one as one step closer. School has been going very well. She is excited to get there every morning. She's also made an impression on Shay, the nurse assigned to the class for Avery. Shay has been great, she is very hands on, as she sits in the class, and helps the teachers, and Avery. She gives us a report on ow she was in class, if it was a good day, or an off day, if she sees any head drops, or seizure activity.  Having her there, gives us great comfort.

With only a couple gifts left to get, we look forward to spending holidays with our families. We wish all of you, our families and friends, a very Merry Christmas.    

Happy Thanksgiving!

Monday morning we heading up to Gillette for the 24 hour EEG scheduled a few weeks back. This EEG was scheduled to 1. take a look at how she's doing, and see if theres any change since March with the addition of Clobazam, and 2. observe nighttime "twitches" and movements she's was making more frequently soon after the clobazam was started.



Avery playing puzzles while they hook up the EEG

After the EEG was hooked up, it was hang out in the Hospital room while they monitored the EEG and video Avery for external signs. We had a good time, played LOTS of puzzles, read books, even watched a movie. 

We didn't spend the whole time hanging out in the room, Avery was unplugged from the monitor to stretch her legs and take a walk around the unit.

Tuesday morning finally come, and we sat down with Dr. Feyma to discuss the results of the EEG. We got some good news, First, the "twitches" we were seeing at night when she was sleeping were NOT seizures. They were just involuntary movements while Avery sleeps, something considered normal. Second, the EEG captured the subclinical and Myoclonic seizures Avery has everyday while awake, typically when stimulated or eating, the positive news was although those seizures were still there, and showing up, they were not as long of duration as shown in the March EEG. So, we are not making any med changes for the moment, going to stay the course, and continue to ween off the Topamax in hopes that Avery continues to improve.

With this news, we had a lot to be thankful for this Thanksgiving, and we had a great day with our families. Hope everyone had a great Thanksgiving as well.

Happy Halloween

Avery and Nick had a great Halloween this year. Avery dressed as Bella, and Nick as Mario, we headed out visiting a few friends, and ended up at grandma Jeanne's and grandpa Tom's to hang out, and hit the neighborhood. Cousins Marae, Harley, and Addison also stopped while we were there.

Later in the week, Avery had a Neurology appointment with Dr. Feyma. In the beginning of September, we started Avery on a new med called Clobazam, This is in effort to try and reduce the number of daily myoclonic seizures, as well as the subclinical seizures she was having that we couldn't see. The plan was to add Clobazam, and then ween off of Topamax, a med she's been on for nearly 4 years. Topamax is known to significantly effect cognition and development. Some history- about 2 years ago we try to ween Avery off it, only to have significant increase in seizures, and 2 GTC's within a week, so we had to put her back on it, but agreed to it, at a significantly lower dose than before. So, at the end of Sept, we began weening Topamax for the 2nd time, we lowered it about a 1/3 of her daily dose, and then Avery got a respiratory infection/cold. So we stopped the ween until she gets healthier, today, she's still sick a month later, but slowly getting better, with the hope to start weening again soon.
    Also something to note, Clobazam is a commonly used med for most Dravet kids, and it's only been available through Canadian Pharmacy, an out of pocket expense to the family. Unfortunately this is too common for most of the meds effective for Dravet kids, they aren't available in US, and families have to go through other resources, and pay out of pocket, some meds at the cost of a mortgage payment. The good news is, just last week Clobazam was approved in the US. Hopefully we can start getting it through our pharmacy soon.
    We also discussed at the appointment that Avery has been having more twitches when she's been sleeping, seizure related? from her cold? from the Albuterol neb? we don't know, so Dr. Feyma has scheduled a 24 hour EEG on Nov 21st. to take a closer look. Keep you posted.

School Nurse Update

    School Nurse Update. Last Friday Debbie and I met with the District head nurse, Special Ed Coordinator, and Avery's teacher to go over Avery's IHP (Individual Health Plan) as well as if they could provide Nursing coverage  in the morning based on Avery's needs. The district Nurse contacted Avery's medical doctor, as well as Dr. Feyma, her neurologist. I also spoke with Dr. Feyma earlier in the week, to share with him what was going on, and discuss our thoughts on the matter.
     Our meeting began at 2pm, and right away we were told that they would be bringing in a Nurse! She is currently working parttime in the district, looking for more hours. She is going to cover Mondays, Tuesdays and Thursdays, and the District head nurse will be there on Weds mornings. She had put together the IHP prior to the meeting, which we reviewed during the meeting. The IHP is a thorough document, stating what to be looking for, in all states of seizures, and when to initiate the seizure protocol, and administer the diastat.
    First day with the nurse on site begins tomorrow, and she will be sitting in the classroom with Avery to get to know her better, and observe her (subclinical) myoclonic  seizures for a few weeks. Please pray that all goes well!