A Treatment for TSC That Helped Stop Seizures From Ruling Our Lives
“OK, if she does this one more time, I’m going to call the doctor.”
I made this deal with myself one afternoon when my youngest daughter was 7 weeks old. My husband Mike and I were finally in the groove of having three children, and so far Cammy had been an easygoing and good-natured baby. Then one day my mom mentioned something concerning — she had seen Cammy suddenly punch her left arm out while shaking and looking off into the distance. We had never noticed this before, but saw it happen that night, each episode lasting about five to 10 seconds.
That moment I made a deal with myself — that I would call the doctor if she did it again — her arm shot out, and she started shaking just 10 minutes later. It was a Friday afternoon before a long weekend, but I wouldn’t wait. We showed Cammy’s pediatrician a video we took, and I knew he was worried when they made an appointment with a neurologist for Tuesday.
That weekend was horrible because it started happening more frequently. We sat there panicking, our minds going 100 miles an hour, wondering if we should go to the hospital. We made it to our appointment on Tuesday, and after the neurologist performed some initial tests, he told us we needed to get her an EEG and MRI right away. We went straight to the hospital and ended up being there for three days. I’ll never forget how little Cammy looked when they hooked her up to her first EEG.
The doctor came in a little after midnight. We could tell he was nervous when he diagnosed Cammy with tuberous sclerosis complex, and then immediately said, “I encourage you not to Google it.” Our whole lives flashed in front of our eyes. All I could think about was how different everything was going to be.
Tuberous sclerosis complex (TSC) is a rare genetic condition that affects nearly 50,000 people living in the U.S. TSC causes benign tumors to grow in the brain and other vital organs like the kidneys, heart, eyes, lungs, and skin. It often affects the central nervous system, which can cause a host of issues ranging from seizures to developmental delays. We learned TSC typically appears in children within their first year and that it affects each person differently, with signs and symptoms ranging from very mild to severe.
When we met with the TSC specialist the next day, Cammy was immediately put on medication. It helped with her seizures for a while, but over time they were getting progressively worse. We started another medicine, but that still didn’t help. Cammy’s seizures kept getting longer and more frequent until she was having a seizure every 10 minutes. Not only was Cammy cognitively affected, but her seizures also caused her to lose the use of her left arm and hand.
We talked with Cammy’s doctor and decided to put her on a particular diet on top of her medicine. It was a lot to learn, but we adapted. We’d have weeks of no seizure activity, and then they would slowly start increasing again. Every time we thought we found something that was working, we’d have to say those dreaded words, “It happened again.” Every time the seizures came back, it was worse than the time before. It was brutal, and her seizures controlled everything in our lives. Mike and I arranged our work schedules so that he or I could always be home with Cammy. The doctor told us managing Cammy’s seizures would be the number one predictor of her development, so we wanted to give Cammy every chance we could. It felt like we were in a race against time.
One day, it seemed like Cammy’s seizures just stopped. It was exciting at first, but two months later we got the feeling something wasn’t right. Cammy looked uncomfortable, and personality-wise, she seemed completely vacant and not like herself. Her skills would change from day to day — she could roll over one day, maybe sit up on her own, but then the next day she wouldn’t be able to do any of that. But we hadn’t seen any seizures, so I emailed our nurse. After an EEG, we realized Cammy had over 30 seizures that day, we just couldn’t see them. What do you do when your baby’s having 30 seizures every day?
Desperate to get her some relief, we talked with her doctor about our options. Since Cammy had seizure activity from different parts of her brain, her doctor recommended surgery and adding EPIDIOLEX® (cannabidiol) to reduce her seizures. EPIDIOLEX is the first and only FDA-approved prescription CBD medicine for seizures associated with TSC, as well as two other seizure conditions, Lennox-Gastaut syndrome and Dravet syndrome. She told us about the possible risks and benefits of EPIDIOLEX, including some potential side effects Cammy might have increased liver enzymes, sleepiness, diarrhea, or loss of appetite. Considering different treatment options always comes with mixed emotions — including the fear and anxiety that comes with making a change. However, since we had been working with our doctor and trusted her, we decided that if she thought something could help, we’d give it a try.
IMPORTANT SAFETY INFORMATION
Do not take if you are allergic to cannabidiol or any of the ingredients in EPIDIOLEX. EPIDIOLEX may cause liver problems. Your doctor may order blood tests to check your liver before you start taking EPIDIOLEX and during treatment.
Please see additional Important Safety Information & Indications below.
Soon after the surgery she started taking EPIDIOLEX, and we were so relieved when she started experiencing fewer seizures. It was like a light was back in her eyes that we hadn’t seen in months. She seemed less tired and glimpses of her personality began to peek through — it was almost like she got a gold medal! It took a while, but very slowly she started army crawling, and then walked on her knees for a while. We were working with a gait trainer at physical therapy. Then, on Valentine’s Day, something amazing happened: Cammy was in the kitchen, and she stood up and took a few steps! It was incredible! After 16 months of nothing but improvement, Cammy did start having seizures again. So we talked to her doctor with whom we have a great relationship, and after seeing a video of Cammy, she agreed Cammy was having more seizures. She decided to increase the dose of EPIDIOLEX to keep up with Cammy’s current weight, and we were able to keep moving forward with her progress.
Although Cammy is obviously delayed, she is light years from where she was or where she would have been without surgery and EPIDIOLEX. When I see Cammy playing with her brother and sister, I am grateful for where we are. I almost can’t believe it when I see all three children going down the slide, one right after the other. Every time Cammy does it, the third child in a row, it’s still emotionally overwhelming for me. I think of all the progress she has made, how she’s able to be a bigger part of her siblings’ lives, and the fact that she’s even been able to go to pre-school where she’s thriving. Seeing where Cammy is now at 3 years old gives us the confidence to handle whatever may come next. Cammy’s progress and where she’s at now have given us hope for the future. Cammy’s going to be the best Cammy she can be, and she’s proving that to us every single day.
Important Safety Information & Indications (continued)
What is the Most Important Information I Should Know About EPIDIOLEX (cannabidiol)?
EPIDIOLEX may cause liver problems. Your doctor may order blood tests to check your liver before you start taking EPIDIOLEX and during treatment. In some cases, EPIDIOLEX treatment may need to be stopped. Call your doctor right away if you start to have any of these signs and symptoms of liver problems during treatment with EPIDIOLEX:
- loss of appetite, nausea, vomiting
- fever, feeling unwell, unusual tiredness
- yellowing of the skin or the whites of the eyes (jaundice)
- unusual darkening of the urine
- right upper stomach area pain or discomfort
EPIDIOLEX may cause you to feel sleepy, which may get better over time. Other medicines (e.g., clobazam) or alcohol may increase sleepiness. Do not drive, operate heavy machinery, or do other dangerous activities until you know how EPIDIOLEX affects you.
Like other antiepileptic drugs, EPIDIOLEX may cause suicidal thoughts or actions in a very small number of people, about 1 in 500. Call a healthcare provider right away if you have any signs of depression or anxiety, thoughts about suicide or self-harm, feelings of agitation or restlessness, aggression, irritability, or other unusual changes in behavior or mood, especially if they are new, worse, or worry you.
Take EPIDIOLEX exactly as your healthcare provider tells you. Do not stop taking EPIDIOLEX without first talking to your healthcare provider. Stopping a seizure medicine suddenly can cause serious problems.
What Else Should I Know When Taking EPIDIOLEX?
The most common side effects of EPIDIOLEX include increase in liver enzymes, sleepiness, decreased appetite, diarrhea, fever, vomiting, feeling very tired and weak, rash, sleep problems, and infections.
EPIDIOLEX may affect the way other medicines work, and other medicines may affect how EPIDIOLEX works. Do not start or stop other medicines without talking to your healthcare provider. Tell healthcare providers about all the medicines you take, including prescription and over-the-counter medicines, vitamins, herbal supplements, and cannabis-based products.
What Additional Information Applies to Women?
If you become pregnant while taking EPIDIOLEX, talk to your healthcare provider about registering with the North American Antiepileptic Drug Pregnancy Registry (by calling 1-888-233-2334). The purpose of this registry is to collect information about the safety of antiepileptic medicines during pregnancy.
What is EPIDIOLEX (cannabidiol)?
EPIDIOLEX is a prescription medicine that is used to treat seizures associated with Lennox-Gastaut syndrome, Dravet syndrome, or tuberous sclerosis complex in patients 1 year of age and older.
It is not known if EPIDIOLEX is safe and effective in children under 1 year of age.
You are encouraged to report side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088. You may also contact Jazz Pharmaceuticals at 1-833-424-6724.