While looking through baby name books several months before our bundle of joy was to arrive, one name stuck out among all the others, Karley. There are a few different interpretations as to the meaning of her name, but made the decision based on the definition in our name book; womanly and strength. We envisioned bringing another vivacious, intelligent, beautiful little girl into this world that will do great things for others and herself in her time on earth. Looking back, we are convinced that choosing Karley as her name is no coincidence; the Lord knows what he’s doing. Even though it may appear that we’ve been punished, we trust that the Lord has placed this burden on Karley and our family for a reason. We do not fully understand why yet and we may not for some time, but someday his plan for her will be unveiled. Karley will have to be strong-willed and determined to overcome her life-long ailment and still achieve her full potential as a person. We will be there for her every step of the way and through our faith, unwavering love and support from family and friends, and the determination that’s in her DNA, she will beat the odds and conquer her diagnosis of Dravet Syndrome.
On April 18th, 2012 we welcomed our second beautiful daughter into this world, Karley Ann Davis. She appeared to be healthy and weighed in at 7 lbs. and 14 oz. Karley had a hard time from the very beginning; she stopped breathing 10 minutes after delivery and had to be resuscitated by the nursing staff. Although it only lasted a minute, it felt like eternity and both of our hearts stopped. Luckily, the nurses revived her and she only had to spend a couple of hours in the NICU before we got to introduce her to our family and friends. We felt so relieved and thought the worst was behind us.
On July 31st, 2012, at 3 ½ months old, Karley had her first seizure. I was at my mother’s house when I noticed Karley shaking, like she was cold. She wouldn’t stop and it seemed to last forever. I immediately called my mother, who was out running errands, and she advised me to call 911 since it wasn’t clear what was happening to Karley. By the time the paramedics arrived, she had stopped but was very limp and lethargic. We were transported to the hospital by ambulance where the physicians performed a lumbar puncture, CT scan, blood tests, EEG, etc. All of the tests came back normal, so we left frustrated without a definitive diagnosis.
This was the first of many trips to the ER for Karley. To date, she’s been transported to the ER at least 15 times since her first seizure and has required intubation on three occasions. We’ve had to call 911 for most of her episodes because the rescue medication we have to administer to stop her seizures slows her respiration and requires skilled medical professionals to resuscitate her if she stops breathing. Her episodes started getting progressively worse over the next several months; the episodes started occurring weekly, lasting 20-30 minutes, and the convulsions would originate on her left side, shift to the right side, and then generalize across both sides her body. By this time, we were referred to a neurologist and it still wasn’t clear if she was having seizures or if she was suffering from a movement disorder. To prove to her neurologist that our descriptions of the episodes were accurate, we started recording the events with our smart phones. Even then, it was unclear what we were dealing with and as one can imagine, we were frustrated.
Following another long, 20 min. plus episode, she was admitted to the EMU and hooked up to the video EEG. The next morning she had a two minute episode and it was confirmed that she was having seizures all along, so we were devastated and relieved at the same time. We were told that they appear to be focal seizures since all of the convulsions started on the same side and she could grow out of them or in the worst case, brain surgery was an option if they failed to control the seizures with Antiepileptic Drugs (AEDs). At this point, we had gone from it’s not epilepsy, to it is epilepsy, and now we’ll just have to wait and see if she’ll grow out of it. The thought of her having to take medication for the rest of her life and/or having a portion of her brain removed before the age of five was tough for us to accept initially, but we did because there was still a glimmer of hope that she would be able to live a normal life. After she started her first AED, Phenobarbitol, she had a few episodes and then was seizure free for over a month. We were excited because she seemed healthy and happy and it appeared that the worst of this was behind us. We refer to this point in Karley’s life as the calm before the storm. It wasn’t behind us, it was just the beginning.
When Karley was 9 months old, she had a long status episode that rendered her unable to use her right arm for 4 hours (Todd’s Paralysis). We were really confused at this point and were told that it is not uncommon for kids with epilepsy to have loss of motor function following a long seizure. Like clockwork, Karley started having long status episodes every two weeks. She was given two additional AEDs in an attempt to slow down the frequency and severity of the seizures, but adding medication seemed to make things worse. Given her tendency to have status episodes, we were on strict orders to give her the rescue medication when the seizure started, wait five minutes, give her the second dose, and then call 911. This vicious cycle continued for a couple of months and a defining moment in her journey propelled us to take a stand and not take “we don’t know” or “we’ll have to wait and see” for an answer.
That moment was on a Saturday afternoon in March. We heard Karley choking on the monitor while taking a nap and rushed upstairs. She was having a full blown, tonic clonic seizure in her crib. She was salivating so much during the seizure that it was choking her, so we had to remove the saliva from her airway until the paramedics arrived. She had drooled during previous seizures, but not as profusely as this one. She didn’t respond to the rescue medication initially and turned blue due to the lack of oxygen. During the panic, Karley’s older sister sat in the corner of the room crying as she watched strange people come into her house and take her sister away in the ambulance again. The paramedics had to manually intubate her in the ambulance to get her oxygen saturation back to normal. The look of helplessness and fear in my husband’s eyes and the tear that dropped from his face as he hugged me before leaving with Karley in the ambulance unleashed a series of emotions in me that I wouldn’t wish upon my worst enemy. Not knowing if your child will be alive when you get to the hospital is the second worst feeling one can experience outside of it actually happening. My husband and I have been together for 14 years and married for 9 of the 14. Of the 14 years together, I haven’t seen him shed a tear or be rendered helpless. He did and was helpless that day and from that day forward, we were going to get answers.
Karley’s case was assigned to an epileptologist following the episode in March. I demanded genetic testing for Karley and much to my surprise, the request was granted. She was started on another AED with Phenobarbitol after this visit and continued to have seizures. Up to this point on her journey, she had only had partial complex and tonic clonic seizures. After she started the new AED, the seizures started to come in different forms; tonic, absence, myoclonic, etc. Around her 1st birthday, she started having frequent eyelid myoclonus, so we took her in for a 48 hour video EEG. Much to her doctor’s surprise, she had developed an epileptic encephalopathy and was quickly put on steroids to counteract the epileptiform activity depicted on her EEG. The steroid treatment halted her clinical seizure activity and brought her sharp wave activity back to acceptable levels. It was almost too good to be true and as it is with most things in life, it was.
On May 31st, 2013, after three months of torture waiting on the genetic testing results, we were informed that Karley has Dravet Syndrome. As one can imagine, we felt heartbroken, angry, confused, and in denial all in one wave of emotion. We say that we are dealing with it well, but those that have children with this awful disease know that we’re not. Instead, we’ve been in a dark place since we found out, but we are getting better every day. One thing that has helped us out tremendously is to meet other families in the area and help out with a local fundraiser for the Dravet Syndrome Foundation. Shortly after Karley was diagnosed, we made a pact to fight rather than feel sorry for Karley and ourselves. Raising money for Dravet research is the only hope we have for a cure and we sincerely hope that the families and friends of those diagnosed join us and Dravet families across the country in this fight.
Karley is just now starting to show signs of development delay, so we’re doing all we can to counteract the effects of the disease by putting her in speech, physical, and occupational therapy and treating her with an aggressive medication routine. We don’t know what the future holds for Karley, but we’re going to do everything in our power to make it brighter for her and kids like her.