In your words

In your words: Outshining LGS

Julie Neal hopes her daughter Bailey’s experiences with Lennox-Gastaut syndrome will shed light on this severe form of childhood-onset epilepsy.

Julie Neal
Julie Neal with her daughter Bailey.
Bailey was born in January 2001. I thought she was the best baby ever. She was beautiful and doing everything normal. Then one morning in June, when she was six months old, I had her on the changing table and all of a sudden her breathing changed. It was like she had the hiccups, but she was staring at the wall and her arms and legs were moving together in an outward motion. It lasted about 30 seconds, then she stopped and cried.

A few days later while we were driving I heard Bailey making the same noise she had made during that strange episode. I pulled the truck over to check on her and she was doing the same thing. I called the doctor and he sent us to the hospital for an electroencephalogram (EEG). The results were fine because nothing happened during the test. The doctor advised us to video her so he could have something to reference.

Bailey was in her baby swing on a Friday night when she had another episode. I got it on video and planned to show the doctor that following Monday, but the next day, while we were at a festival, she had a full-fledged seizure. God was watching over her, though, because there were three nurses nearby. All three nurses came over and started caring for her. The seizure lasted about three and a half minutes. It was by far the worst and the longest.

The nurses wrote down all of her vitals with a description of what happened. When we got to the hospital I was able to provide the video from the night before and the nurses’ notes from that day. Doctors started her on seizure medication. In hindsight she was probably having seizures long before we ever saw one.

We followed up with a neurologist in Fort Worth, Texas who continued to see Bailey for about two years. She had multiple types of seizures: grand mal, myoclonic, and atonic. Along the way she’s had other types as well. We went through many different medications trying to control them. After a couple of years, her doctor thought it was best that we see an epileptologist — a neurologist who specializes in the treatment of epilepsy.

In 2003 we tried the ketogenic diet for about six weeks. It’s a special high-fat, low-carb diet that helps control seizures in some people. She was in the hospital for about eight days to get started. We had to weigh her food down to kilograms. It was life changing for the entire family. Her seizure activity actually increased while on the diet, though. So the neurologist took her off of the diet and we changed medications again.

The next recommendation was a vagus nerve stimulator (VNS). It’s a device similar to a pacemaker implanted under the skin near your collarbone or on your skull that stimulates the vagus nerve with pulses of electricity. It trains the brain to not have seizures. At the time, I considered it an invasive surgery for a 2½ year old and couldn’t put her through it.

“She’s definitely taught me more than I will ever be able to teach her.”

Sometimes as a parent you question whether or not you’re doing all that you could be doing for your child. I started wondering if we should seek another opinion. In 2006, right after she turned five, I researched top epilepsy clinics around the U.S. and found a hospital in Houston. I wanted them to start from square one with a fresh look. Their findings were consistent with our original neurologist, which was reassuring, but I had also been hoping they would find something different that could be fixed.

While we were there they performed an MRI. She hadn’t had one in a couple of years. Afterward they told us she had scar tissue on her brain, which was the cause of her mental disability. It was the first time we were made aware of this. She started off so normal, but every year that passed there was more and more delay. I thought the seizures were causing the delays, but they weren’t. Doctors don’t know what caused the scar tissue; more than likely she was born with it. This was also the first time we heard Lennox-Gastaut syndrome (LGS) was a possible diagnosis.

After returning home we learned our primary neurologist was questioning LGS as a possibility as well. At that point, though, Bailey wasn’t experiencing the right type of seizure activity to confirm a diagnosis. LGS has a very specific pattern of spikes and waves as seen on an EEG, but it also encompasses cognitive impairment and that’s why doctors were considering it.

It was June of 2007 before we got an official LGS diagnosis. I told our neurologist we were ready to try VNS and it was the scariest thing I’ve ever done.

Bailey has been free of grand mal seizure activity for over five years now. She still has atonic seizures, also known as drop seizures, and myoclonic seizures, which appear as brief, sudden jerks. On a bad day she might have one or two atonic or myoclonic seizures, and they’re strong.

If she’s standing and has an atonic seizure her feet go so far off the ground her knees will hit her forehead. It’s not even a second long, but it’s long enough to force her body into a ball and throw her down. She’s had over 100 stitches from having these types of seizures. She’s also broken her arms, her ankle, and her nose. Even though she wears a protective helmet, it doesn’t always protect her teeth when she hits the floor. Broken bones and teeth are something kids with LGS have to endure.

Bailey is 14 now but developmentally she’s somewhere between two and four years old. As she’s gotten older, she’s also developed behavioral issues, which is common among people with LGS. It goes back to the fact that she cannot communicate to us what she needs or what she wants or what she feels. Maybe that’s where some of this stems from — the frustration she must feel from not being able to communicate. Her poor little body has gone through so much. It’s hard because she doesn’t always know the words to be able to relay what she’s feeling.

Neal Family
The Neal family

Although her speech is like that of a two-year-old, she can still get her point across and she comprehends at a higher level. For example, she had a profound connection with my Dad, who passed away in 2007. When she was little he jokingly taught her the word “nincompoop.” She couldn’t even say “Mama” yet, but she could say that, and to this day she still uses the word when picking on someone. It’s her favorite word and one of the few that she has retained over all the years. It makes me smile that a little memory of my Dad, her Papa, is still with her. She still walks by a family portrait with him and says “Ahhh Papa” like she understands he is no longer with us.

Our reality is that more than likely I will survive Bailey, and that’s a hard realization. It’s scary. I fear that the grand mal seizures will return and lead to sudden unexplained death in epilepsy (SUDEP), which is one of the main causes of death for people with LGS. In addition to all that they have to go through, it’s sad to think that people with LGS are at a high risk of SUDEP and a high risk of injury from traumatic falls that sometime prove to be fatal. It’s a matter of trying to prevent the things that could possibly happen.

Every LGS child has so many different scenarios and unfortunately there isn’t much information out there. I’ve never met a caregiver or someone else with LGS in person, but the LGS Facebook page (through the LGS Foundation) is a fabulous way to connect with others and learn. When you’re having a terrible day these people understand. They know what you’re going through. It’s incredible to know you’re not alone.

I recommend caregivers join the Facebook page and find someone who is living it. Don’t believe everything you read on the Internet unless it’s from a credible source. The LGS Foundation website offers legitimate answers to your questions. It’s important to remember that one person’s experiences compared to another are going to be different and you have to find information that’s pertinent to you.

Raising a child with LGS takes a village. My 17-year-old son, Hudson, is my rock. He often gets Bailey off the school bus and stays with her until my husband and I get home from work. He takes on the responsibility because he knows that we need his help. There are times when I have to call in the troops. Bailey’s grandparents help out when they can, and a friend’s daughter watches Bailey for me in the summer.

Sometimes I wonder how we will do it in 20 years. Those are things I have to think about, but for as long as possible, I want her living with us. Hudson says Bailey will live with him one day, and my other daughter, Addison, who is 10, recently told her best friends that she and Hudson have to live next door to one another when they grow up so that she can help him take care of Bailey. Her friends agreed that they would all live near each other so that they can all take care of Bailey. It’s good to know our village will always carry on.

Julie Neal
Bailey Neal loves to hold babies.

I try not to put a label on Bailey. She’s more than LGS. She has likes and dislikes and her own personality. She loves babies. That love that we feel for our own children, she feels for all children. She loves music; her face just brightens when a good song comes on. Although she can’t sing the words, she gets the melody and pitch, hums the tune, and knows the songs. It’s absolutely incredible.

I recently took Bailey to a Miranda Lambert concert. She was glowing. There were people sitting near us that kept watching her. Toward the end of the concert I unlocked the brakes on her wheelchair and let her wheel around and “dance” in the aisle. As soon as I did, the couple came over, grabbed her hands, and started dancing with her. The woman told me she had never seen so much joy coming from one human being. Then another little girl came over and sat in Bailey’s lap and danced with her. And another child came over and danced, and another. That’s the effect she has on people.

It’s moments like those that outshine the lows. It’s those moments that keep us all going — the incredible joy there. We’re fortunate to have Bailey. She’s definitely taught me more than I will ever be able to teach her.

This is what God gave us. We’ll keep educating ourselves about LGS, deal with it the best we can and make the best life possible for all of us.

Taking Precautions

Common seizure-related injuries include bruises, lacerations, bone fractures, burns, and, in some cases, drowning. Here are some things you can do today to reduce the risk of injury:

  • Avoid clutter in your house, and add padding to any sharp corners.
  • Keep lids on hot drinks, and use only the rear burners when cooking on a stove top.
  • Avoid lighting candles, swimming, or climbing ladders unsupervised.
  • Routinely check in with neighbors, friends or family that you trust. It may be helpful to give them an extra key to your home.

Source: “Seizure Safety and Seizure Risk: From First Aid to SUDEP” Daniel Friedman, MD, Assistant professor of neurology; NYU Comprehensive Epilepsy Center

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