Tiara is still doing horrible and I am just trying to keep her going until she is approved for brain surgery. After the Pet Scan in January, we found out she has to get a Meg scan at UCSF. Well, that is a little problematic for us since she is a horrible flier, motion sickness and serious irritability, so we realize we will have to drive her up North for the test. We get a date for the test in the first week in March and we decide Lou will take the day off and we will drive straight to San Francisco, spend the night, get up, do the test and drive straight back to Newport. So that is what we did and funny enough the three of us had a great time. Tiara was super good in the car, our only problem was the urine issue. Around this time, Tiara suddenly started holding her urine for hours, like any normal adult. The problem is a Depends diaper cannot accommodate a huge rush of urine all at once like a toilet, so it gushes everywhere. We currently have a supply of astronaut diapers she sleeps in and we use for long car trips or doctor visits but at this time we didn’t know about the special diapers. So every few hours we had to stop, change her clothes, wipe her down and clean up her seat.
By the time we pulled into the hotel driveway our car smelled worse than a litter box. Louie’s best friend, Damon, had arranged for a hotel for us right next to the hospital. It was this really cool Japanese hotel that had this crazy traditional Japanese bath, which was a perfect square and you sit in it on a stool. Tiara was terrified of it but Lou had a blast trying to figure out how to use it. Lou is obsessed with baths and we have a really old normal size tub which doesn’t fit his 6’2 frame, so whenever we go to a hotel he can’t wait to use the tub.
Tiara loved the hotel and the next morning we got up and drove straight over to UCSF medical center for the test. Once we got into the hospital, we met the technician who was so kind and informative BUT she proceeded to tell us Tiara would have to lay still for at least 20 minutes WITHOUT MOVING AT ALL in order to complete the test. Every time she moved the test would start over and there was 3 parts. OMG, how are we ever going to do this? She then went on to tell us that if they sedated her the results wouldn’t be as accurate so they highly recommended we try without sedation. Well, since I wanted the most accurate results possible, as this test was the final hurdle in determining whether she could have the surgery, I didn’t want to take any chances. Louie and I each took a side and started singing every imaginable song and telling her every story we could think of. She did really well but we ended up staying in that room for over an hour in order to get the whole test done. On the way to the car, we were feeling so jubilant. We did it! We survived the drive up, the hotel stay and finally finished the test, it felt so great.
Once we got her to the car we realized she was soaking wet with urine again. Oh well, no biggie. Lou held up a beach towel as a little tent so I could change her in the parking garage before we started our 7 hour trip home.
When I pulled down her diaper I realized she had just started her period for the first time. Wow, what great timing, how exciting, what do I do with this? Guess I will wipe her up, put on the diaper and get into the car, and that’s what I did. I knew her violence was stemming from her hormone changes and once she started menstruating I was able to verify my theory.
Now that we had finished all the tests, we just had to wait for the results. The MEG scan took at least a week to read and interpret and then the team at UCLA had to discuss the options. I received an email on March 21 asking me if Tiara and I could come to UCLA in 2 days to meet with her neurologist and then her neurosurgeon. I was so excited and readily agreed. Of course in my mind, I had already decided Tiara was having surgery if they agreed to do it. I knew plenty of children with Tuberous Sclerosis who had brain surgery at UCLA with Dr. Mathern and their lives had improved significantly in each and every case. I had no reason to believe that the same wouldn’t happen for Tiara. Two days later we met with her Dr. Mathern and he reviewed all the results with me.
As I explain this, please remember I am paraphrasing my interpretation of what he said. Clearly he said it much more eloquently and scientifically than me.
He explained that Tiara had tumors on both sides of her brain and many different areas where the seizures were originating from. Typically what happens in a child with Tuberous Sclerosis is that if you remove an “active” tumor, one which is causing seizures, a non active tumor may activate and start having seizures. So just because you remove a problem tumor doesn’t mean the seizures will stop because most likely another tumor will decide to start causing seizures. When tumors on both sides are actively causing seizures, the surgeon has to decide which area is causing the seizures that are most detrimental to the child’s development and welfare. In this case, we were hoping to eliminate the vomiting seizure, but unfortunately there were a couple of potential areas in her brain that would signal a vomiting reflux and one was on the right side of the brain and the other was on the left side. So they had to guess based on all their tests and analysis which tumors to remove.
Initially the team was convinced Dr. Mathern should take out a few of the more active tumors on the right side but after the MEG scan results came in they decided the left side would probably benefit Tiara most. Since her most active tumors on the left were in the frontal lobe area, they felt strongly that removing them could help with her violent behaviors. After explaining the options to me, Dr. Mathern said if we wanted to proceed with surgery he would attempt to take out 2 or 3 tumors in the left frontal area of her brain. He then explained the risks, and the reality that the only reason he was offering this surgery to us was because she had no other options. He wasn’t convinced it would help, but he would try. He then said, if it didn’t work he would consent to a second surgery to remove the active tumors on the right side of her brain and if that didn’t work either, there was nothing left to offer.
He then looked at me and said “does Tiara have a father?” “Of course, he is wonderful, he is just working.” He kinda looked at me with this side ways glance as I then went on to say, “my husband will agree with whatever I decide and I have already decided we want the surgery. So what do we do now?” He seemed a little perplexed by my attitude and said even though I had already agreed he needed me to sign some consent forms and then explained we had to wean Tiara off of Depakote for 2 weeks prior to the surgery.
That made me feel sick. I was instantly terrified. I wasn’t scared of her having brain surgery, just not taking her Depakote. Depakote was her strongest, best medicine and her seizures were so out of control how could she survive without it? He said it wasn’t negotiable, she had to be off for 2 weeks before because of bleeding issues or he wouldn’t perform the surgery next month. Ughhhh!!!!! Now I was scared.