After buckets of paper work, a medical exam, an EEG and a review of her medical records Tiara is accepted into the Vigabatrin research study at UCLA on July 16, 1999. Dr. Shields, the director of the study starts Tiara on the medicine on July 19, 1999.
A miracle has occurred. Tiara has stopped having infantile spasm seizures after the first dose of Vigabatrin. All of the sudden she is sitting up again and now even trying to crawl. Her smile has returned and we are feeling hopeful for the first time in months. She is referred to the Regional Center and after reviewing her condition she is immediately granted status as a consumer. This means she will start to receive services such as speech, occupational and physical therapy to try and help her catch up developmentally. I had no idea the state and or county funded all of these services if you child meets certain medical criteria. I was shocked to learn our taxes paid for these programs. I felt guilty about it at first but everyone assured me that this is how the system works and I should accept the services and not worry about the funding, since I am a taxpayer. I clearly was clueless about our government and how it operated but super grateful that Tiara was going to be starting therapy right away.
The next few months before her first birthday were the beginning of the Tuberous Sclerosis rollercoaster. One day everything is fine and the next day we are back in the hospital. Several weeks after being on Vigabatrin Tiara starts to not feel well. Because she is so newly diagnosed all her doctors immediately are concerned every little thing is a huge thing. I am on this same boat with them but the ups and downs were constant. Her cardiologist told us she could start having problems with her heart at any time so to keep our eyes open to any changes in her health. Well it is a Saturday in August and she is very agitated and crying a lot. I take her to the walk in clinic and wait to see the doctor. Once I give him a history on Tiara he refuses to treat her. He told me he had never treated a patient with Tuberous Sclerosis and I would have to drive her to Hoag. Once we arrived at Hoag, the waiting room was overflowing. I called my friend the nurse and she got us in the back door. The Hoag ER physician came in and introduced himself. I explained her history and he immediately got an attitude with me and got super defensive. I was puzzled by his behavior but keep talking and requested he test Tiara’s heart. He finally admitted he too had never seen or treated a patient with Tuberous Sclerosis and he was not sure what tests to perform. He then said “ I will perform any test you want on her, just tell me what you want.” “OK, I said. Do an echocardiogram on her heart.” They did the test, her heart was fine and we left. Clearly she was just acting like a regular 10 month old but we all panicked. This theme would continue for years, until I learned how Tiara responded to viruses, seizures, infections, and fevers.
Fevers will always be problematic for Tiara as we learned 2 weeks before her first birthday. She had another virus and a slight fever; suddenly her whole body was mottled and purple. I rushed her to the pediatrician and by the time we arrived her fever had gone from 101 to 105. Her fever escalated so quickly that her body couldn’t adjust and her skin got mottled. I know this now after 13 years but no one knew this then. At that time, we did not know this was her normal response to a fever so once again her pediatrician told us to drive straight to CHOC. We also did not know at that time that Tiara was unable to sweat. One of the tumors in her brain interferes with her body’s ability to sweat so if she gets too hot from the environment or a virus her body temperature rises and quickly. She needs to be physically cooled down with cold rags or ice packs in conjunction with Motrin every 6 hours and Tylenol every 4 hours. I quickly learned to chart everything about Tiara as not to over medicate her.
Once we got to Choc, they assumed the worse and believed she may have an infection in her heart. After a three-day visit filled with numerous tests, specialists and interns she was discharged wherein they decided it was a normal virus. As you can imagine, Tiara always like to be held or carried and always slept with Lou and I. I had spoiled her so bad she would have nothing to do with her crib. In the hospital they insisted she stay in a crib but she would cry constantly and I couldn’t fit in the crib with her. So it was this first visit after the diagnosis that I struck a deal with the nurses on the fourth floor. If we could have a twin bed instead of a crib for Tiara, I would stay in the bed with her at all times and never leave. I had to sign special paperwork but I got her the twin bed. The only problem was if I had to go to the bathroom in the middle of the night I had to call a nurse to stand guard over her bed, while I ran to the bathroom.
And so Tiara made it to her first birthday having experienced more medical tests and probing than most people experience in a lifetime and it was only the beginning. Read Week 5 of my Journey with Tiara, here…