On Monday of this week, Johnathan noticed Ailbe having what looked like a spasm. Each day since then, we’ve noticed 4-5 clusters of 3-5 movements. We became very concerned after Monday. We yelled at each other that it was just a coincidence and not really a spasm, we sobbed with each other hoping it was just a one time thing and then, when we saw it again together, we decided we needed to get ourselves together and call her neurologist. We sent him a video of what we thought was concerning and he said “it looks suspicious we should do a VEEG.” We scheduled the appointment for Thursday and then we waited.
We hadn’t seen a spasm in almost 4 years. I had just recently gotten to the point where I was comfortable saying “seizure free” to other people without feeling superstitious and we had let our guard down. We weren’t following her around anymore and watching her every move. We had begun to enjoy life, without the worry of epilepsy in it. Spasms are so small in general, that very few people can notice them unless you are looking right in a child’s eyes at the point of which they are having one. Doctors have difficulty seeing them and even neurologists who specialize in all type of epilepsy need to see a VEEG over several days to diagnose it. Ailbe used to have very severe spasms that made her body jerk as well as her eyes droop and they would hurt her. She would wince and cry during them. She was non verbal and it was very difficult to watch. This time around the movements we were seeing were concerning because they looked so familar but were so different. For instance, she could talk through these and she didn’t seem like she was in pain. Our emotions were all over the place because the last time spasms returned they went from 1 a day, to 20, to a 100. The fear of that returning was/is too much to bear.
Ailbe was diagnosed with Infantile Spasms at 18 months. She took zonegran and prednisone and they were controlled after two weeks. When she was first diagnosed her VEEG showed Hypsarythmia (a condition/state of the brain that can cause brain damage and be life threatening). 6 months later Ailbe turned two and we celebrated with a super fantastic second birthday and thanked everyone for supporting us through the toughest time in our life. At this point she had been 6 months seizure free and she was strictly on Zonegran. Two days after her party we noticed a spasm. Our hearts broke. It had returned. It went from a few spasms, to 100s a day. We tried Prednisone again, but it did not work. We were panicked. We tried Vigabitrin, but we couldn’t get her to swallow the pills. We tried the ketogenic diet, but it didn’t work. We were slowly losing our baby one attempt at time, so we went to a nuerosurgeon and he recommended a hemispherectomy. He told us that was her “only hope at life.” At this point, Ailbe wasn’t talking and another medication choice was considered to have around a 3% chance of success. In knowing that we weren’t behind surgery at this time, we tried Sabril. Sabril is a medication that is the powder form of vigabitrin. About a week in, we got the spasms to stop. A year later she transitioned to Topamax, Ailbe began to speak/had a developmental explosion, Isla was born and this continued until 3 months ago when she weaned off Topamax. We felt great! Very confident. Ailbe was blossoming, she was hungry/eating (her only major side effect of Topamax) and she was happy! It never felt so good to go somewhere not worrying that I wouldn’t have to bring an arsonal of meds with us. But, as of Monday, that has all changed.
We checked into UCLA Thursday and Ailbe was hooked up to the VEEG. That process is a little hard for her since she has to sit so still and there are 23 leads with 5 steps each. The glue smells terribly and makes it a little hard to breathe during some parts of the placement. She cried for me to take her away from “the man” and asked when she could get her “helmet” off. The head of neurology visited us to see the video we had. He thought from the video, that she had what is called a focal seizure or a partial seizure. He stated that she was a good surgical candidate and again it amazed him that she could use both sides of her body and both hands. I told him she can swim, climb, dance, run and do many things that show her gross motor skills are very strong. He was amazed. He told us that they didn’t have enough information right now and so they would come back tomorrow morning with the data from the VEEG. 24 hours passed and we had seen episodes for them to record so we were hopeful to have some information. Dr. Sankar returned with his team of nuerologists and he told us that they didn’t have enough to definitively diagnose a condition but that the imaging looked worse than the last time we were there. Not our initial (18 months) VEEG but the one we had several years ago after we weaned of sabril and went to topamax strictly. They asked us to stay another 24 hours so we reluctantly agreed. Ailbe was sobbing on and off for her sister and really missing home. (Child life at UCLA is incredible. They really helped us get through this stay. The brought Ailbe a variety of toys and teddy bears for her to play with and many that she could keep. Thank goodness for these awesome volunteers!)
This morning the pediatric fellow visited us to let us know the team would be making their rounds soon. We asked that Dr. Lerner (he hadn’t been coming around with the team but was the one reading the EEG) come visit us as well with his input before any decisions were made. Dr. Lerner visited us and as he opened the door, I could tell by his face. “It’s spasms.” He must have seen our faces drop as he followed quickly with “the imaging did not show hypsarhythmia. She is having spasms. They are so miniscule that you missed some while sitting right next to her. We will start Sabril treatment again and our goal is to eliminate the spasms as quick as possible.” We flooded him with questions and after answering all of them, he basically told us, one step at a time.
It’s hard to take things one step at a time. I’m learning to not completely go into panic mode. That with a diagnosis of cortical dysplasis (the reason for her spasms) Ailbe may have to deal with seizure activity throughout her life. But even if she doesn’t and this is the last time, she will always have to be aware of her brain abnormality. She may have to undergo testing routinely and we need to be prepared for this. From this point on, we are going to take the first step. The first step it to attack these new spasms, called Epileptic Spasms (now that she’s older) with the frontline med Sabril. It will be hard to get back into the mode of dealing with pharmaceutical companies and the stress of depending on shipments (since this isn’t a drop off your script at cvs med) but, we will do it. We are praying we don’t see any regression or side effects but will be prepared if we do.
A month from now we have to return for step two. They will check Ailbe in for another VEEG, a PET scan and an MRI. From this testing they will be able to check to see if the spikes and abnormalities in her EEG have reduced/left, and compare the scans from three years ago to her brain today.
We are definitely shocked and very saddened that this has happened but we have no choice but to step into go mode and take care of this. Now is the time to be strong and push for Ailbe. To make sure she gets everything she needs
On a personal note, I want to thank those of you that have reached out to our family. We very much appreciate your prayers, positive thoughts and concern for our well-being.
Ailbe is a warrior. She has always shown that. She will overcome this, I know it. I feel it with every fiber…
mypurplepack 