Warning: This is gonna be a long one!
Epilepsy is a chronic disorder in which nerve cell activity in the brain is disturbed, causing seizures. This has hands down been the one complication meningitis left Madison with that has been the most difficult to control and the hardest to watch her go through. According to the CDC, about 3.4 million people nationwide suffer with epilepsy, over 400,000 of those being children. It is the 4th most common neurological disorder and often the cause is unknown. There are so many different types of seizures and they vary based on where and how they begin in the brain. For the purpose of our journey, I’m only going to discuss the type of seizures Madison is suffering.
Subclinical Seizures & The EEG
A subclinical seizure is one that does not present any clinical or physical signs or symptoms. Madison suffered these when she was intubated and fighting meningitis at just 2 weeks old. They were caught on an EEG. An EEG or Electroencephalogram, is a test used to evaluate the electrical activity in the brain by using small metal discs (electrodes) attached to your scalp. Even when sleeping your brain cells communicate through electrical impulses and all of this activity shows up on an EEG. The EEG records said brain waves and is largely used to diagnose irregular activity or changes in the brain from brain disorders, epilepsy, inflammation, sleep disorders, etc…
At the time Madison was sick, she was given 3 different seizure medications in an effort to stop her seizures. Once we finally found one to stop them, we slowly weaned the other 2. Although her EEG no longer showed seizures, she still had irregular activity, which is common with brain injuries, and she was sent home on one medication called Phenobarbital.
I HATED phenobarbital. It’s like a bad word in our house. It is said to have minimal side effects and commonly used with children, but Madison was so out of it on that medication it was impossible to get her to do anything. It is classified as a barbiturate and has addictive qualities to it, especially with long term use. She slept A LOT when we first returned home from the hospital; she still does sleep more than the average child, but I’ll get into that another day.
On phenobarbital Madison always seemed loopy or high for lack of a better term, anytime she woke up. I hated seeing her so out of it and I pleaded with her neurologist to switch her medication. At the time, she wasn’t even having seizures and we weren’t 100% sure if she would. It’s possible she only had seizures at the time of meningitis because her brain was inflamed and that she would no longer suffer from them; or she could develop them again due to her injury which is unfortunately very common.
Due to my pleading we decided to have an EEG in early November to check on the activity in Madison’s brain. She did sometimes do some weird movements, but babies tend to be jerky in general, so we weren’t quite sure if they were seizures or not. The EEG still showed irregular activity (again, common for a brain injury), but no seizures were detected at the time. Her neurologist did however feel it to be too risky to stop the medication. Because there was still so much irregular activity going on, he was fearful that the medication was in fact preventing seizures from occurring, and that by taking her off or switching at that time, we could put her at risk for status epilepticus. SE is a very severe seizure that can last more than 30 minutes, causing further brain damage or loss of consciousness, and requiring medical support to come out of. This was not something I was willing to put my 4 month old up against, & so we stayed on the awful phenobarbital. It was a hard blow. We wanted to give our baby girl the best chance at developing, knowing she would be behind already, and we felt this medication was severely holding her back.
Then, strangely, a few weeks later, on Thanksgiving night, Madison had her first clinical seizure. We weren’t totally sure at first what was going on; upon waking up she would seem super startled. But my mom gut knew it was a seizure. I started googling like I always did, and stumbled across Infantile Spasms. I was pretty sure this was what my sweet girl was suffering, but only an EEG would tell.
Originally her neuro team asked that we increase her phenobarbital dose to see if that stopped them. Something I was obviously against being I hated that med already, but we tried. A week into increasing and her seizures seemed far worse. I asked to come back in for an EEG, as I was not increasing this medication any further. Sadly with seizures, sometimes things get worse before they get better with medications, but I was not messing with a medication I already saw take away her personality. We went in for an EEG in mid-December, and thus our journey with Infantile Spasms begun.
Based on her EEG she was diagnosed with Infantile Spasms & Focal Seizures. A focal seizure affects one hemisphere of the brain and can cause muscle contractions, unusual head or eye movements, and more. We added a medication called Keppra and it worked. Her focal seizures stopped after the first dose.
When it came to the Infantile Spasms diagnosis however, stopping them has not been so easy. IS is a very rare and severe seizure disorder occurring in children, usually under one year of age. The average onset is around 4 months, exactly when Madison began having them. It can be very subtle in appearance, making it hard for parents and PHPs to recognize as a serious problem, and therefore often misdiagnosed. Remember when I said it seemed like she had a sever startle reflex? Well, that’s what IS often looks like. Unfortunately because of that, and especially in regards to children who suffer it without a known brain injury or reason, it often gets diagnosed as reflux.
I’m sure a lot of you hear the word seizure and imagine someone convulsing on the ground- something very obvious. I was like that once too. But seizures can present in so many ways, something as subtle as an eye flicker or staring out into space for too long; something that looks exactly like a child’s startle reflex or a tiny head drop. Unfortunately children who suffer from IS are at a greater risk of a developmental disability due to the underlying brain pattern, called hypsarrhythmia. Hyps is a VERY high voltage and disorganized pattern of brain activity, making it hard for a child to develop. Patterns of hypsarrhythmia on an EEG typically confirm the presence of IS. A diagnosis of IS is very important because there are specific treatments geared towards this type of seizure.
Being told Madison had IS, even though I had previously googled it myself and assumed it was the case, was still devastating. We already knew she wouldn’t develop normally, we were already living with the fear of the unknown at how much she would develop in general, and now things were about to get 10x harder for her. The odds were stacked against her even more. To make matters worse, we were about a week away from Madison’s very first Christmas. The thought of spending her first Christmas in the hospital was soul crushing.
Treatment – ACTH
The frontline treatment for IS, is called ACTH. ACTH is a hormone produced in the pituitary gland. It is unknown why ACTH works to stop seizures, but has been found the most effective in stopping IS in children. It’s given in a gel form through an injection into the muscle. It’s typical to begin at a high dose, twice a day, and slowly wean down the dose and amount of injections over a 6-8 week course of treatment. Most children see a decrease in spams early on, and another EEG would confirm whether or not the hypsarrhythmia has stopped.
ACTH can cause irritability, increased appetite, large weight gain, high blood pressure, low potassium, and high blood sugar. Because of the severe side effects, families typically remain in the hospital for the first 5-7 days of treatment in order for medical staff to monitor the child’s reaction. At this rate, we would get home just in time for Christmas.
And so we moved forward with the ACTH treatment. But, did things go smoothly? Of course not. Remember when I mentioned ACTH was made by the pituitary gland? Well if you read my post on Madison’s endocrine issues, you know that she has issues producing hormones that work with that gland already. We spoke with her endocrinologist about the treatment, and deemed it safe to try, although none of us knew if she would even take to it, being her body already has issues with this hormone. Prior to beginning the treatment, Madison had other routine tests done to ensure she was healthy and good to go. She was, and we began the treatment.
Now long story short (I know, haha, this story is nowhere near short lol), but she would up getting misdiagnosed with a UTI from the routine urinary analysis taken prior to treatment. She was given antibiotics, which meant we had to stress dose her hydrocortisone, on top of being given a very high dose ACTH injection, and BAM, her sodium skyrocketed. (See my post on Endocrine as to why.) We had to stop the injections, and wait for her sodium to drop. We wouldn’t make it home for Christmas. We were now days behind on treatment. We sat and cried.
All of these mishaps happened over the weekend, and when her neurologist returned on Monday, he couldn’t believe all that had happened. He decided to let us go home, so that we could ensure her sodium had time to drop back into normal range, and we were to return the day after Christmas to re-begin ACTH treatment. We were happy & sad. We would get to be home with our family for Madison’s first Christmas, but it was surely tainted knowing we would return to the hospital the day after to fight this rare and devastating seizure disorder.
The day after Christmas we returned to the hospital and started the injections all over again. John and I were taught how to give them, we purchased a glucometer and a blood pressure cuff, and after 5 days were sent home with Madison to continue treatment. For the next 2 months we gave her shots twice a day, pricked her finger to ensure her sugar was stable, and took her blood pressure to ensure that it wasn’t high. We also had strips to check her urine, and brought stool samples to a lab weekly to ensure there was no internal bleeding which can also occur. It was a tedious 2 months.
In addition, because she was on such a high steroid, her immune system was basically wiped out. Just what you want in a special needs child during flu season right? We never left the house with her. She couldn’t get vaccinated. Her therapists and the limited family we had over, had to wear masks and gloves. We had to cancel her baptism. It was an extremely hard and lonely winter. And to top it all off, the ACTH didn’t work. We injected our tiny girl with shots every day for 2 months, and it didn’t work. Our baby girl was still having clusters of seizures, anytime she woke up, every day.
I’d also like to comment on the cost of this treatment. A treatment that didn’t work for us. One vial of ACTH costs around $34,000. Yes, those are 3 zeros. $34,000, give or take, for ONE vial of a medication that might save a child. Treatment can cost more than $100k total for a family. We are extremely blessed and thankful to have wonderful insurance that covered this cost for us, but many families are not as lucky. As if dealing with a child with a rare & severe form of epilepsy isn’t enough of a heartache, parents must face the burdening cost of a treatment that may or may not work. Let that sink in.
Treatment – Vigabatrin
The next frontline treatment for Infantile Spasms, is a medication called Vigabatrin or Sabril. It’s an oral medication that comes in a powder packet that you mix with water and give with a syringe. While there has been great success with this medication stopping IS, one of the biggest side effects is the possibility of permanent vision loss. Terrifying right? Imagine watching your child suffer seizures multiple times a day, every day, and in order to stop them you now have to worry about them losing their vision. The decisions epilepsy parents are faced with are simply unfair. The cost of this medication is around $7k for 50 powder packets, something we are lucky our insurance also covered.
Although the vision loss side effect is rare, so is contracting meningitis, so rare means nothing to me anymore. We decided to move forward with the medication after ending ACTH, starting with a low dose and working our way up. It can also be common for doctors to add Vigabatrin during ACTH treatment as well. With Vigabatrin Madison experienced some severe drowsiness and low tone, but we did see her seizures improve. They became physically less intense and shorter. Because things were a little better, and since she was on both Vigabatrin and Keppra now, we were able to SLOWLY wean that awful phenobarbital! That was a huge win for us.
The wean was extremely slow, because like I said, it is an addicting medication, and even a child can suffer withdrawal symptoms or an increase in seizures when coming off. Luckily Madison did alright with the wean. Nothing got worse, and although I was hoping for her alertness to greatly improve, her personality did come back a bit. She no longer seemed high and loopy when she woke up. It was still a blessing.
We have now been on Vigabatrin since February. Madison sees her ophthalmologist every few months to ensure her vision isn’t being compromised, and so far, things have remained the same. Her vision is no better or worse. Even though her seizures did improve, we eventually reached our max dose, meaning we had failed this treatment as well.
Treatment – Onfi
A few months ago we added Onfi to her laundry list of medications. In the world of epilepsy, Onfi is typically loved or hated. It has been the miracle drug for some, and a complete disaster for others. All kids respond differently though, and so unfortunately you must take everyone’s advice with a grain of salt, and make the hard decision for yourself on whether a drug is right for your child.
Onfi is classified as a benzodiazepine. It’s an addicting drug that can lead to tolerance and dependence. A scary thing to give to a child. But a number of neurologists recommended this as a next step for Madison, and after doing our own research, we decided it was the right choice. Since adding Onfi, we have seen the greatest change in her seizures. It took almost 3 weeks before we saw a decrease, but once we did they have gotten much shorter, and less frequent. We were even able to begin weaning Vigabatrin.
In fact, we increased her Onfi dose right before Madison got sick with rhinovirus, and over our stay in the hospital we noticed her seizures to be very minimal. Since we’ve been home (one whole week now), she hasn’t had her typical cluster of spasms, not once. Some weird eye movements, but not spasms. This is really such a new development for her, and I’ve been told that whereas getting sick can increase seizures in some, it can also decrease them in others. The eye fluttering she is occasionally presenting could also still be IS. She does sometimes seem to have a single jerk-like motion as well, whereas before her seizures always came in clusters. The timing is strange, but we’ve seen Madison’s development pick up a bit this past week as well, which can also be a sign of her hypsarrhythmia slowing down or going away.
Has Onfi become our miracle drug? I’m not sure yet. I actually plan to touch base with her neurologist later today to let them know of the changes we’ve recently seen. I don’t want to get anybody’s hopes up just yet, including my own, but I’d like to believe that we are beginning to see the light at the end of the tunnel. Only an EEG however, will be able to confirm seizure control. Some kids stop presenting physical symptoms of spasms, but unfortunately still suffer with the hypsarrhythmia.
I’d like to mention that we have also tried a less mainstream and more natural way of treating seizures, through CBD oil. CBD is a hemp oil derived from cannabis, without the THC. It can be quite controversial, and I’ll be dedicating a whole other post on that, so stay tuned if you care to learn more! If the Onfi in fact hasn’t or doesn’t fully stop Madison’s seizures, our next step would be the Ketogenic diet, which is also deserving of it’s own post. For now, we will take it day by day.
Although Madison has been a teething maniac this past week, it’s been so refreshing to see her wake up without having a spasm. I almost forgot what that was even like. Her strength amazes me, and I hope God continues to give both us and her care team the knowledge to put her on the right path. Please continue to pray for her in that we find seizure control, as well as for the many other children facing this diagnosis. Hell, pray for the adults suffering epilepsy too. It’s a hard disease to watch someone you love go through, but we are so grateful for all of the support we’ve had thus far!
If you’d like to donate to the Epilepsy Foundation, click here.
**And please remember, I do not intend anything I say to be taken as medical advice. I am not looking for negative comments regarding our decision to give our daughter the aforementioned medications, nor am I recommending them for someone else. This is simply our journey with our meningitis survivor and her battle with epilepsy. Thank you!
September 11, 2018
Maddie is so incredibly strong + a fighter. Always keeping her in our prayers <3
September 11, 2018
Can’t say it enough you and John are doing a great job with your precious girl.
One of the things life and it’s tribulations have taught me is you never know how strong you
are until you need to be strong. You, John and Madison are warriors.
September 11, 2018
Thank you! It’s so true that you don’t know your own strength until your only option is to be strong. ❤️
September 11, 2018
Kelly, Madison is absolutely beautiful. Your posts are both inspiring and heartbreaking, and I will continue to keep you all of you in my prayers.
September 11, 2018
Thank you so much!
September 11, 2018
Always thinking of you three. You are so strong . Of course you do what you must for your baby girl but being able to then smile… write about it… share… you are amazing. 💜
September 12, 2018
Aw you are so kind, thank you Sara! Your girls are just adorable!
September 12, 2018
Good bless you and your family Kelly, Your strength is amazing. Madison is blessed to have you as her Mom. And John as her Dad.
I keep you all in my prayers and thoughts every night.
Sending you hugs.
September 12, 2018
Thank you so much!
September 13, 2018
Thank you for such an honest look at epilepsy. We’ve gone though our own epilepsy journey with our littlest one, and nothing has been more challenging. I’m happy to hear you have found a win with medication! That is a journey in itself! Wishing for great days ahead for sweet Madison.