Make A Child Smile

Heather's Story
written by mom Denae

Heather Riley (we call her Ri sometimes) was born at 34 weeks and was a perfect little baby. Tiny, but perfect. By 5 months old, she had been in the hospital many times for respiratory distress. Finally they discovered the culprit was laryngomalacia, asthma, and reflux. Once she was on medicine for the asthma and reflux, she was back to being perfect.

Her development was always slow, but no one ever expressed any concern. I was told many times at her check-ups, that she was the youngest in a large family, so we needed to make sure that she was not being "babied." She did not sit up until almost 9 months old, she took her first steps at 23 months. She did not talk at all until just before her third birthday thanks to extensive speech therapy.

In July of 2001, she had her first seizure and our happy universe crumbled. The first seizure occurred at daycare, I was told that day that she couldn't come back until she had seen her doctor. They also told me at that time, they were very concerned about her development in general. She was almost 2 years old and was not doing any of the things that the other kids her age were doing. I was devastated. We went to see her primary care doctor and left with a referral to see a neurologist.

December 29th, 2001 she was diagnosed with epilepsy and global developmental delay. We had a few years where life was quiet, Heather Ri had therapy several times a week. She was making progress. Then in 2005 everything changed. The little bit of seizure control was gone. After many medications failed to control the seizures that were obviously holding her development back, she was evaluated for a Vagus Nerve Stimulator (VNS). It was during that evaluation period, that I was told she had childhood epileptic encephalopathy, a devastating form of epilepsy. With even less hope of ever having seizure control we agreed to try the VNS.

Dr. Heafner implanted the VNS in February of 2006. It has helped a little bit. She did seem "brighter" and more with it after the VNS was turned on. I have mixed feelings about the VNS, sometimes we are able to stop a seizure with the magnet, other times it does nothing. Heather's seizures remain uncontrolled.

In January 2008, she started the Ketogenic diet since all the medications and one surgery had failed to give her acceptable control. I believe she may have become seizure free on the diet, but it was impossible for her to continue. She was refusing to eat, and never drank enough. Eating has always been a battle for Ri. We had to stop, the all day screaming and obvious misery was not worth any amount of seizure control. It was at this point, in March 08, that Dr. Parrott had us come in to talk about what to do next. We had two options:

1. Surgical evaluation (which would likely lead to a corpus callosotomy)
2. Try another drug, Felbatol.

We chose the Felbatol. By that point, she had already had several VEEG's (Video Electroencephalogram) and they all told us the same story "Generalized Slowing, multifocal spikes (frontal and temporal lobes, more on the left than on the right)". Felbatol is a drug that is only given when everything else has failed and the benefits outweigh the risks.

We took that chance. By May 08, she was on high doses of 3 seizure meds, plus the Felbatol. We went to Dr. Parrott with some concerns as we were weaning the Zonegran. So he scheduled her for another VEEG. During this VEEG we took her off of everything except the Felbatol. Felbatol was increased and she was put back on a short course of Klonopin.

We came home with a different child. She wasn't having half of the seizures she had been before. It was amazing to see, our lives for that short period were as normal as they had ever been. Heather Riley has three older sisters, we were able to co camping and go with Nana on the boat. It was amazing, we hadn't been happier in a long time. I was in love with Felbatol. This lasted for almost 3 months before the world came crashing down again.

Starting in August, she was having a life threatening seizure activity called Status Epilepticus, both convulsive and non convulsive. Not only this, but after those seizures she would be in what was called Postictal Encephalopathy. During this time, her breathing would become erratic, her heart rate would become dangerously low (down to 20bpm and stopped twice) this would last for hours to several days after the seizures. The only way to know if she was in this postictal state or again in nonconvulsive status, was to have an EEG. She would be hooked up to the EEG for days at a time waiting for her to wake up on her own. This was happening every 7 to 10 days from August of 2008 until March of 2009.

During these months, they added more meds, changed her VNS settings, but nothing seemed to matter. We were watching the seizures take our Heather away. She started to regress and her already challenging behavior was even worse. She stopped eating and drinking and was completely dependent on a g-tube for her nutrition.

A few days before Christmas, she had a cluster of tonic seizures on the stairs. She aspirated and we didn't know at the time. The night before Christmas, she had almost constant tonic seizures, we gave her an ativan and the seizures seemed to stop. Christmas morning she woke Robert and I up with the sound of gasping for breath, I checked her temp because she just didn't look right, she had a temperature of 108.

Christmas morning, instead of opening her gifts from Santa, and spending what is likely to be the last Christmas her Poppi lived to see, was spent on the critical care transport ambulance being sent to Levine Children's from our home in Lexington. We were terrified, she spent the next several days in the hospital recovering. At first, they didn't know what was wrong, thankfully the x-ray confirmed it was aspiration pneumonia and not one of the many other infections that they had thought she may have.

This was when her neurologist, Dr. Parrott, told us that there wasn't anything else they could do, she has failed every medication available as well as combinations. The VNS isn't helping enough, the Ketogenic Diet failed and then we were only left with surgery, even that wouldn't stop all of her seizures. The seizures seem to come in cycles now. At this moment she is still having seizures, but they are not as bad as they were a few months ago. Her VNS was just set to a rapid cycle and she is on 5 anti-seizure medications.

She was granted a wish by the Make-A-Wish Foundation this Spring and our family spent a week of wonder at Give Kids The World and the Disney parks.

We wake up each morning and pray for a good day, thank God when she has one, and pray harder when she doesn't. Heather is a beautiful happy little girl who makes everyone around her smile.

(Story and Interests were last updated July, 2009)