Back at the PICU - When we got back to the PICU the doctor there said they were going to do a spinal tap because she had a slight fever and they wanted to rule out any possible infection.  Also, since she was still sedated it was a perfect time.  After the spinal tap they showed us the clear liquid and said that sometimes the fluid will be cloudy if there was an infection, but not always.  Shortly thereafter they told us that there were no white or red blood cells in the spinal fluid and that was good because when blood cells appear it can be an indicator of infection.  Also early indications from the lab showed no signs of infection, but they would have to culture the sample for 3 days.

Monday evening with our Neurologist - Later that evening when many of our family members arrived we were introduced to the pediatric neurologist for our local hospital.  He took his time in explaining to us why they do an MRI and how it can determine if there are problems with a brain.  He proceeded to tell us that his diagnosis was Sturge-Weber Syndrome.  He said that when he examined the MRI it was a textbook case.  He then showed us Amanda's MRIs and explained what we were looking at and how the affected right hemisphere compared to the unaffected left hemisphere.  Well the whole family was pretty torn up, but Brandy and I pressed him to tell us what we could expect.  He mentioned seizures, possible glaucoma, developmental delay and other possible problems.  He said that the most important  thing right now was to get the seizures under control and then take one day at a time.  He said that cases of SWS range from the very mild to the severe.  This all sounded pretty grim to us and we were literally numb for quite some time.   They immediately started Amanda on Trileptal and Fosphenytoin (Dilantin) and ordered an EEG and ophthalmology evaluation. That evening I was allowed by the very kind nurses to use one of their computers to access the internet to look up information. Anyone reading this probably knows that there is very little information about SWS on the internet. The best and most comprehensive web site I found was the Sturge-Weber Foundation (www.sturge-weber.com). Much of the information that I did find from the medical websites talked mostly about worst case scenario. I had to pick out little pieces of information that gave me hope and remember them. For instance, Amanda's seizures did not start until 18 months and many children start much earlier. After starting the Fosphenytoin and Trileptal Amanda's seizures decreased in quantity, but were not eliminated. She was still having about 3 a day at about 8 hour intervals. Ativan was administered whenever the seizure went longer than 5 minutes and, to a varying degree of success, helped stop the seizures.

Tuesday - PICU - On Tuesday Amanda was very groggy and irritable. I just want to mention this because we were not prepared for the side effects of the medications. She was impossible to console and would whine and fuss for hours. We spent a lot of time just holding her in our arms sitting in a chair. Of the whole ordeal this probably was the hardest thing for us to watch. Early this morning they set up the equipment to perform a Video EEG. I am sure you parents are very familiar with this, but for those of you who don't know an EEG is a measure of the brainwaves. They place electrodes (in Amanda's case 21 of them) on the scalp of the head in various predetermined locations. These electrodes can then measure the activity between two points in the brain. By reviewing the EEG they doctors can determine many things. They can sometimes tell where the seizure has started (if the patient has a seizure while being monitored), they can tell if the patient is having subclinical seizures (seizures with no visible characteristics such as hand clenching) and they can sometimes determine how badly the brain is damaged. We were instructed to write down the starting and ending time of any seizures that we witnessed so they can find them quickly while reviewing the EEG. When the EEG technician put the sensors on Amanda's head she mentioned that it may leave scabs and may not look pretty when it is taken off. We later found out at Miami Children's Hospital that this should not have happened for only 3 days of monitoring and the scabs that Amanda had on her head upon removal they would only expect after 3 weeks of monitoring, not 3 days. They said that it was probably due to the type of paste that was used to secure the sensors. Amanda was then monitored off and on over the course of 3 days. Several seizures were witnessed which helped out the Neurologist.  The Ophthalmologist came in and measured Amanda's eye pressure to determine if she showed signs of glaucoma and said her pressures were well within normal range.  We later found out that since Amanda's port wine stain does not cover either eye lid she is at a lower risk of glaucoma compared to a SWS patient who has a PWS that covers one eye lid.  She is still at a higher risk of glaucoma than the general population, so we will need to do more frequent eye exams.

Wednesday - Amanda had been doing well and the seizures had subsided when she had a breakthrough complex partial seizure on Wednesday. If I recall correctly she had gone about 26 hours since the last seizure. When she had the seizure on Tuesday they added an additional medicine - Phenobarbital. Well Phenobarbital has many side effects and had been around for decades, but it also can be a very effective anticonvulsant and proved to work well for Amanda. This, however made her even more irritable. We talked to the nurses and they said it was quite normal and it takes several weeks to adjust to the meds, but we could expect her to be back to her normal self after she had adjusted. This was a light at the end of a long tunnel for us. This evening we spoke with our Neurologist and he mentioned that one option if the seizures would not stop was surgery. He said that Miami Children's Hospital had an epilepsy ward and they were well equipped to handle surgery if it was necessary. I must add the Doctors and Nurses at Lee Memorial Healthpark were outstanding in every respect. They were very comforting and accommodating. Not much else happened today, just more medicines, more EEG monitoring and 1 seizure.

Thursday - Thursday was pretty much the same as Wednesday, except no seizures, yahoo!! They kept the medicine regiment the same and continued to monitor the EEG. Amanda was 1% better and we were praying that we had the right combination of medications.

Friday - Friday morning came with a really bad 45 minute complex partial seizure. They administered the Ativan as they had in the past, giving Amanda 3 small dosages totaling the maximum dose for her weight and when that did not work they gave her a reloading dose of Phenobarbital. This also did not seem to be effective so they decided to add Depakote. Well Depakote is especially bad for children under 2 where it has a 1 in 700 chance of causing liver failure. As they were literally walking to the IV to inject the Depakote the seizure stopped. Someone must have been looking down from heaven on us that day because I was really worried about the Depakote. Once Amanda stabilized we discussed the possibility of transferring to Miami Children's Hospital. We felt that we would be better off having her there incase surgery was necessary. We figured since they would have all her records and their own opinions and observations of Amanda's condition it would be better to transfer sooner than later. The PICU Doctor contacted MCH and came back and said that they were in the process of scheduling it. He said it may be today or it may not since it was a non-emergency transport and if the flight team had more urgent transports those patients would come first. Evening came and we had no word of when we would be transferred other than probably Monday.

Saturday - On Saturday the PICU Doctor came in and told us to get ready. Up until this point we were hoping that one of us would be able to travel in the helicopter to be there to comfort Amanda. The Doctor said it depends on the transport team, but this team does not allow it. This was tough for us to think she will be traveling by herself and we would not be there if something went wrong. The Doctor suggested that one of us start out immediately for Miami so they could get there sooner and the other parent stay with Amanda until the helicopter came to pick her up. Brandy's father was more than willing to drive Brandy over to Miami and they left shortly thereafter. The helicopter was delayed in arriving and it was necessary for them to land at a local airport instead of at the hospital because of construction at the hospital. The transport team arrived and hooked Amanda up to their monitoring equipment and strapped her down. I mentioned to them she was probably not going to be "cool" with being strapped down and when she wiggled her arm free they allowed it to stay free, which was a big comfort for me. They wheeled Amanda down and put her in the ambulance to take her to the airport and I went back to the PICU room and loaded up everything to take to the truck. Let me tell you that after spending almost a week in the hospital, you acquire a lot of stuff. I had used one of the wagons they had in PICU to haul all the stuff down and it was literally heaped up to the point that stuff was falling off. I said goodbye to the staff and headed out. On the way to Miami I called Brandy and was very pleased to find out that they had arrived before Amanda and would therefore be there when the helicopter arrived. Upon arriving in Miami the medflight crew told brandy that Amanda had worked her legs free from the restraints and they had never seen anything like it in the past.  They called her little Houdini.   They took Amanda to PICU because they wanted to monitor her for 24 hours before releasing her to the neurology wing. PICU was much more spartan in Miami, but the staff was very nice. There were "community" rooms which had about 10 patients in each and there were several isolation rooms.