September 2001, Tiara is one month shy of her 3rd birthday and is finally implanted with the VNS at Choc. The surgery goes smoothly and her neurosurgeon starts her on the lowest setting. We are hoping for a miracle, and praying her seizures will decline. The first 2 weeks we see no change; she is still averaging 6 seizures per day. The third week after getting the Vagal Nerve Stimulator, Tiara is suddenly getting worse. She is unable to do certain things; she has become withdrawn, and is no longer babbling. She is lunging forward and crying out just like when she was a baby and diagnosed with Infantile Spasms. Her neurologist orders an emergency EEG and they discover she is having hypsarrhytmia again. This is the brain pattern associated with Infantile Spasm seizures. Oh my God, how could this be happening to us? Apparently, it is impossible to have infantile spasms at her age because she is no longer an infant and her doctor has never seen this before. But yet, despite the fact he has never seen this happen before, Tiara has relapsed and it is the worst possible scenario. Her neurologist thinks it is impossible the VNS caused this to happen, but I know in my heart, it was the VNS and I order him to turn it off immediately. He starts her on Lamictal hoping she won’t need to get back on Vigabatrin, but after a week, nothing has changed. Remember, I worked over 1 year getting this device approved for TT and now after 3 weeks it has caused her such harm, it is unbelievable. After the week is up and there is no improvement, I refuse to wait any longer and insist he put her back on Vigabatrin. She is no longer a part of the study at UCLA and the drug is not FDA approved so we have to figure out how to get it. Luckily, I had formed close friendships with several other families whose children were using Vigabatrin. Her doctor gave me the dosage she should start on and I called a friend and asked if we could “borrow” some. Of course she agreed and within hours I started Tiara back on Vigabatrin. Fortunately my friend’s husband was making a trip to Mexico so we put in our order along with several other local families. But, I remember thinking, what if we were really poor and I couldn’t afford to give him the $220 for the medicine? What if I didn’t even know other families who could help me out? Then what? She just turns into a vegetable? Maybe? That is how damaging infantile spasms can be on the brain and how imminent the situation was. The Vigabatrin stopped the spasms within 2 days!
Fortunately, I had reached out to the Tuberous Sclerosis Alliance soon after Tiara was diagnosed. They connected me with several families and together we started a local chapter for the Tuberous Sclerosis Alliance. We started fundraising, educating doctors in the local area, formed a support group and started an annual picnic. Because of these friendships I was able to get Tiara her drugs quickly and in the years to come the support for one another would become an invaluable asset. We don’t meet on a regular basis anymore, but if anyone ever needs any information or help it is always a phone call away. It is funny because of our core group we each had a “role”. I am the mom you call when you need help with seizures and drugs, one mom is a net-worker, and highly connected and can always get you any information you need, and the other mom is amazing at dealing with the school district. Support from others dealing with the same issues is always so important but there is a fine line in talking about the problems too much. Sometimes the support groups would be super depressing and make me feel worse. Because most of the people in our local chapter are really positive people we decided to spend more time on fundraising and awareness of TSC because we all felt the same way.
During this same time, Tiara is supposed to be starting public school at Harper Preschool. When you have a child with special needs, they are given therapy through the Regional Center up until their 3rd birthday. At that time, they are transferred over to the school district. There was lots of testing and each therapist also did an exit session with us and made certain recommendations and prognosis for the school district. I clearly remember her speech therapist telling me she didn’t think Tiara would ever be able to talk. Really? She then went on to explain Tiara’s aggressive behaviors, which had already started at this age, would worsen as her level of frustration grew because of her inability to communicate. Wow, that is super positive. Once again, she like so many other trained professionals were wrong about Tiara. She can talk, not perfect, but good enough. Luckily, the school district realized Tiara needed a one on one aide because of the seizures. I was really hesitant to let her go off to preschool weeks before her 3rd birthday. I mean the whole system didn’t seem to make sense to me. They wanted my kid who couldn’t even talk, was on a ton of meds, having seizures and always off balance, to get on a bus and go to school for 4 hours without me. Yeah right! Well, they hadn’t hired a one on aide yet, so we agreed I would be her one on one until someone was hired. So of course I drove her everyday and off to preschool we went together for the first month. One the first day of school, they took away her pacifier, we both started to cry. I was so upset. Remember, I am pregnant at this point and super emotional, but Tiara loved her pacifier more than anything. I understand their point, they said she couldn’t practice her speech with the pacifier in her mouth, and yes she was 3 years old, but I didn’t care. It brought her so much comfort and I could only imagine how terrible she must have felt all day, having all those seizures and yet still having to try and focus in class and therapy. UGH.. Well, after I cried and tried to immediately leave with her on the first day, her teacher agreed we could slowly wean her off.
After a month, they finally hired an aide for Tiara, but I was so scared to leave her. The teacher was wonderful and the aide seemed great too, but it took me a long time to get over the panicky feeling when I dropped her off everyday. I guess it was probably better for me, since I needed to get the nursery in order for our third girl, Trinity, scheduled to arrive late April. When I am pregnant I always have a lot of pre-labor pains for weeks and sometimes a month prior to the baby arriving. Well, this time, it was worse. After Christmas, my pains were so bad and I started bleeding. I went to my OB, who after another ultrasound and an exam put me on bed rest and said I couldn’t carry Tiara anymore. WHAT! This is the first week in January and the baby isn’t supposed to come until mid to late April. What the heck was I going to do for the next 3 ½ months if I couldn’t get out of bed. Who was going to take care of Tabitha and Tiara. Seriously, this must be a joke. Of course, you know by now, I do whatever the heck I want regardless of what anyone says but Lou was adamant about me listening to the doctor. Before she put me on bed rest he was always getting mad at me for doing too much. “Slow down, take a rest, stop painting by yourself, why do you always have to be doing something?” Blah, blah, blah, I thought and now my failure to listen to him was shooting me in the back. Now what was I going to do?
After the appointment we sat down and made a plan, well that is right after Tabitha’s birthday party at Girl Mania at Fashion Island. The day I was rushed in to see the doctor, was the day of Tabitha’s birthday party. Of course, Lou wanted me to stay at home in bed, but I walked, very slowly through the mall, and then I stayed seated the whole party. I mean that was really hard for me, but I knew he would send me home if I pushed it. Once we got home, we decided it was probably time to hire a nanny to help with Tiara after school and do laundry and stuff. UGH, I hate having to rely on others for help, especially needing paid help. That sucks. He wanted me to get help everyday but I agreed to 3 times a week. My mom and sister offered to help the rest of the time. The good news was that the doctor said once the bleeding stopped, I didn’t really have to actually stay in bed, I just couldn’t do too much. So my mom rented me a wheelchair so she could push me around the mall and fabric stores. I was in the process of making Tiara and Trinity matching bedding since they would be sharing a room. I even tackled making my own crib bumpers. What a psycho I was, I so don’t have that kind of energy anymore. I bet you are laughing at me right now. I also learned to use the motorized wheelchair at Target and Costco. I felt like such a loser at first, but then I figured who cares, I need to get my shopping done and I need to get out of the house. Lou let me drive Tabitha and Tiara to and from school so I was able to accomplish everything I needed during my bed rest.
At this point, Tiara is attending school, most days, but she is now having 100’s of seizures everyday. Going to school is practically pointless because she is unable to learn anything when she is constantly having seizures. Her doctor wants to turn the VNS back on and I let him because she has no other options at this point, except brain surgery.
Tiara is admitted to UCLA for Phase I telemetry to see if she was a candidate for brain surgery. We spent 4 days in the hospital, while Tiara was hooked up to an EEG, monitoring all her seizures. She also had an MRI and a PET scan. She hated every moment of it and was constantly hitting me because she wanted to leave and was so frustrated being confined to the bed. In addition she had to be sedated for the MRI and PET scan on different days so she was always super agitated when waking up from anesthesia. After all that, the doctors decided she was not a candidate because she had multiple tumors on both sides of her brain and they couldn’t operate on both sides. In addition, in patients with TSC, what typically happens is that if a tuber/tumor is removed, another inactive tumor may then start causing seizures. Since she had so many tumors it was very likely another would start causing seizures after they took one out. So the doctors ruled out surgery and said they couldn’t help. Why is it so many times in Tiara’s life, the doctors have told me they are unable to help? I was feeling so defeated again. None of the drugs were working, the VNS didn’t help, and now brain surgery wasn’t an option. What is next?