Our Story - part IV

Shortly after his first admission at the hospital and starting on his new medication of Phenobarbital 4.5 ml twice a day, Sripathi had another seizure that lasted over 7 minutes and eventually re-hospitalized on the 10th of January.

This time he underwent EEG monitoring for 38 hours. During which time we were asked to press a button that was attached to the monitors if we see him seize. And the second evening while I was feeding him expressed milk I noticed he was shifting his focus to his right, doubtful I called Aravind to check and we hesitantly pressed the button. The next day the doctor called us into the EEG monitoring room to show us the EEG reading and the video and explained to us that Sripathi did have a seizure the previous evening. And that throughout the 38 hours of EEG he seized only once. She told us that we'll have to bring him back to the hospital if his seizures last over 5 minutes. Finally we were discharged on the 13th of January 2010 with an additional prescription of Topomax 25 mg. The neurologist suggested we get his eye checked by an ophthalmologist and gave us her reference.

So we though our nightmares were over. Sripathi had a hard time with the medications but at least he was not seizing any more. On the 23rd of January he suddenly was running temperature and it was 101.7. We were getting ready to give him Tylenol or acetaminophen but prior to that we wanted to confirm with the on call nurse as the fever was really high. And bad news she asked us to take him to the ER rather than we administer any medicine as the fever is very high. So we go into ER and they gave him acetaminophen and also took his urine samples. They were trying to make sure he did not have any seizures. And then after 4 hours we left and by the next day his fever was gone.

Eventually we did take him to the Ophthalmologist on the 11th of February. Not sure if it was all the dilation and extra lights or just his gradual increase in weight that he had 2 seizures since we returned from the eye exam that day. So we called our neurologist who insisted that she sees him right away. She asked us to give him a bolus shot of Phenobarbital 10 ml. And we drove 30 miles. At her office Sripathi seemed very normal. He did not seem like he just had seizure and on top of it he had extra dose of medication. Even Dr.K was surprised. But she prescribed to increase his Topomax to 37.5 mg that's 1 and a half tablet twice a day. And she wished that he does not seize anymore.

Well wishes don't always come true; he was hospitalized the same evening after a 13 minute seizure. This was his third hospitalization for seizures. By now all the nurses and doctors were overly familiar with us. It was the routine sever blood work, this time they got the special nurses who are generally caring for patients who are being air lifted for treatments. This was because they usually are very quick and efficient. Guess it was time for them to show their efficient by drawing several vials of blood from my sons neck. I took not be in that room for long. Sripathi was wailing and I was wailing with him. It was not helping him or the nurses. I had to leave the room. Aravind was with him. After what seemed like hours one of the nurses brought a frail little creature turned red and blue from crying. I nursed him and he was the least interested he just wanted to feel safe and painless for some time and eventually fell asleep.

We were discharged on the 13th of February with 2.5 ml of Phenobarbital, 37.5 mg of Topomax and a new member Keppra .8 ml. That was not all. He was supposed to get more blood work performed outside because they could not draw enough blood the other day. The list from the neurologist marked almost 11 or 13 different tests. We finally ended not doing any of those unnecessary and out dated tests for epilepsy except for 3.

Things were going on fine for a couple of days but on 17th of February he had a breakthrough seizure and it was so different from the rest he had before, it seemed like he will be out of the seizure but again his eyes used to get locked, we got a video of it and Aravind took it to the neurologist. Who on looking at the video felt that he was probably out of the seizure but to fired and seemed like he was still seizing. But again even she was puzzled. She anyways asked us to increase his Keppra to 1.6 ml. And to add to all the uncertainty that we were surviving she gave all the records pertaining to Sripathi and suggested that we take him to an Epilepsy center probably in Miami or Gainesville or any other that our Insurance could cover. She said that she was worried why Sripathi's seizures could not be controlled by three different medications and preferred that a specialist takes a look at him for a second opinion.

Our friends had contacted a specialist at Houston's Texas Children's hospital. Dr.W the epilepsy specialist was ready to take a look at Sripathi immediately and gave us an appointment with a week. In the meantime Sripathi had another seizure on the 21st of February.

On 26th we went to TCH, first Sripathi was scheduled for EEG and later in the day we met Dr.W who spent nearly 2 hours with us. First listening to all that has been happening since Sripathi's first seizure to the last one on the 21st. Then he spent sufficient time explaining to us about Sripathi's seizures. First he said his EEG was totally abnormal and he was surprised at how they missed to read his EEG properly back in Orlando. Then he said that his seizures were worse and critical. He called that cryptogenic localization-related epilepsy with (Tonic versive seizures) independent left and right hemisphere seizures. Other impressions he provided were that Sripathi has a developmental encephalopathy with global delays, prominent hypotonia, macrocephaly which suggests a neurometabolic disoeder.

He told us that Sripathi was on the wrong medications. First he wanted us to wean him off the Topomax as he said that the side effects of it were already showing up on the EEG. But he said that any change in medication needs to be gradual or otherwise it could have adverse effects on his seizures. So one change at a time. Regarding the seizures he explained that Sripathi was first be placed of antiepileptic medications to control his seizures and only if they don't seem to help at all we should go for the next treatment which usual is surgery. Unfortunately Sripathi is not a candidate for surgery for the mere reason that his seizures are originating from both the left and the right sides of his brain. So the next possible treatment would be a Ketogenic diet. Not all patients fair well with this diet which is unique to each patient. Lastly if this does not work out then we can try the Vagus nerve stimulator, which involves a surgery to place a chip in the patient’s body. But for now he sent us home with a plan to wean Sripathi off the Topomax. And for convenience sake make the Keppra 1.5 ml from 1.6 ml. He also suggested that we extend our stay at Houston and get Sripathi checked by a neuro ophthalmologist, get an MRI and a few more blood tests.

Our stay typically planned for 3 days was extended to 30 days. On the 2nd of March Sripathi was given his Polio shots and on the 5rd of March he had his MRI and the results stated that Sripathi's brain had abnormally increased subcortical and periventricular white matter volume with abnormal thickening of the corpus callosum. The following evening he had cluster of seizures and eventually taken into ER. Later discharged with prescription for Diastat.

On the 9th of March Sripathi was scheduled for an eye exam and further blood work for Alexander’s disease, Canavans, the amino acid plasma which all eventually came back negative except for the CMA - chromosomal micro array. From which they found that Sripathi has a PHF8 gene duplication on the X chromosome. So later they had taken my blood samples to test for the same. The gene that has duplicated for Sripathi has never happened to anybody before.
Sripathi had his eyes checked by a neuro ophthalmologist. And as per the specialist Dr.E his eyes are perfect, but not the neurological connectivity with the brain. So he was diagnosed with Cortical visual impairment - CVI and he will require visual therapy.

On the 11th of March Sripathi had his HIB vaccination and the next day he had a 12 minute seizure and we administered Diastat for the first time. We had informed Dr.W's nurse about Sripathi's seizure and that evening we were informed to increase his Keppra to 2.5 ml. Gradually on the 14th of March we had weaned him off Topomax which took us over 2 weeks.

On the 22nd he got his Prevnar vaccine and the following day he had several seizures and was given a diastat. When we informed about his recurring seizures after vaccines Dr.W confirmed that Sripathi still has more room for intake of Keppra and suggested that we increase his Keppra to 3 ml which we did on the 24th of March but he still continued to have a 7 minute seizure that day and we gave him a diastat. Well we had to wait because the Keppra can take time to increase in his blood to help counter the epilepsy.

On the 27th of March we finally left to Orlando. Sripathi seemed fine throughout the flight but sadly seized in the car on our way from the airport. We stopped on the Highway and gave him a diastat. He was fine for a couple of days and again seized on the 29th for like 4 minutes.

The story so far...after that things seemed to settle. But like the calm before the storm Sripathi had several breakthrough seizures from the 9th of April till the 14th. Sometimes 6 seizures in a day. But each seizure would not last more than 5 minutes so we did not have to use diastat most of the time. On the 12th Dr.W increased his Keppra to 3.5 ml. Finally the seizures were controlled after we gave him a bolus 3.5 ml of Keppra.