Garak's Trees

For the past 7 years Sheri (Jailyn's sister) and Sheri's sister-in-laws and Mother-in-law have decorated and donated a tree for the festival of trees fundraiser for Primary Children's Hospital. This year they are sweetly donating two trees in Garak's name. All the proceeds from the trees go to Primary Children's Hospital in Salt Lake City. Each tree will have a picture of Garak and a small description of Garak and all that he has been through. Jailyn is very grateful for their love and support for Garak.

Garak has been such a great blessing to all of us around him. From the challenging day to day activities that he faces, he always has such a sweet spirit to him. He makes you just want to smile when you hold him or talk to him. There is no way to explain the great love we have for this sweet boy, he truly is a gift that gives hope to everyone around him.

Meds

Garak is now 2 months old and growing bigger ever day. Below is a little information about the medicines Garak takes....

On September 20^th Garak received his first dose of Phenabarbital. On this day they gave him a ‘loading dose’ which is a big jump start dose to get his level where it needed to be, then his daily dose was 10mgs twice a day. We have gradually upped his dosage and now we give him 34.2 mgs once a day. Since his first day on this medicine we have had to check his levels every 3-7 days to make sure the dose he is receiving is within his “therapeutic level”. This is done by drawing his blood and running it through a lab for testing. The neurologists determined his therapeutic level was between 35-39. He has now been on the drug for 2 months and we are finally to a point that we are staying consistent with his levels. Before a week ago, his levels would go extremely high above 40 (which is considered ‘toxic’ for him, which means the drug can begin ruining his liver and kidneys) and then dropping very low into the mid 20s. It seemed like no matter what they gave him, his levels could not hold steady. This past week and a half his levels finally held steady. 11 days ago his level was 29.6 and when we tested him again on Saturday his level was 30.3. Since he is not having as many seizures anymore, we have decided to leave his dosage which is currently 34.2 mg once a day as is. Side Effects: the only side effect we see with Phenabarb is it makes Garak very sleepy. Since cutting down to once a day, we are giving him the medicine at night since it does make him sleepy. We also switched from liquid to pill form. The liquid was absolutely disgusting (Garak’s doctor convinced Tyson to try a little of it). The pill is a LOT easier to give Garak then the liquid, 34.2mgs is almost 5mls, which is a LOT to give an infant when it is horrible, the pill almost dissolves in his mouth.

On October 28^th we added a 2^nd seizure medicine called Keppra. We put him on 2mls twice a day, after the first 5 days we were suppose to up his dosage to 4mls twice a day and then after 5 more days up his dose to 6mls twice a day, but since his seizures decreased drastically, we kept the dosage at 2mls twice a day. This drug we don’t test his levels, the half life of this drug is 6-8 hours, so it isn’t as important to verify how much drug is in him. (The Phenabarb half life is anywhere from 50-500 hours). Side Effects: The Keppra is making Garak very fussy. There are times he cries for hours. Older children say that the Keppra gives them really bad headaches, so there are beliefs that infants also get bad headaches and therefore causes them to be fussy. Garak has also come down with a really bad rash over his face and body which is also from the Keppra. We have been putting Aquaphor on it to help, after almost a month, the rash is finally looking a little better.

At this point we are not sure which medicine is helping the most or if it is the combination of both, we are just grateful something is helping!

Photo Shoot / Weekly Update

Jailyn & Tyson took the cute boy to get his pictures taken. He was pretty good. He had one seizure during the shoot but other than that he was a natural as you can see!

Since the last update on Garak he has been doing really good. He has been on both Keppra and Phenabarb and it seems that the Keppra is working really well. He will occasionally have seizures maybe 1 every other day. The only drawback/side effect to the Keppra is it makes him more fussy. Which in turns makes it hard for him to go to sleep.

Tyson's mother Carol has been at Jailyn's and Tyson's house helping out with the kiddos almost the entire time since Garak was born. She has been a great blessing to Tyson and Jailyn during this time and we as Jailyn's sisters we want to thank her for everything she has done for them. She leaves today so please keep Jailyn in your prayers since she will be handling everything by herself now (at least during the day she will).

Garak's First Month

We (Jailyn's sisters) decided to start this blog as a way for everyone to be informed as to Garak's story and keep everyone updated as much as possible. There are so many friends and family who have expressed their love and are curious as to how Garak, Mom and Dad are doing. Also, many friends and family have inquired how they can help Jailyn and Tyson. With the numerous medical expenses arising from not only Garak's medical bills but also Lincoln's, we have set up a donate link where you can help Jailyn and Tyson.

Cute baby Garak was born at 1:59pm on September 20th, 2011. He weighed 7lbs. 10 oz. and 18” long. Delivery was perfect, and the Emmett family was so excited to welcome this new little addition to their family, and everything seemed fine and normal at first. Sadly, however, nine hours after he was born, he began having “seizure”-like episodes. These episodes would start with him crying out horribly, his body would then go very stiff, his head and eyes would deviate to the right, then he would go extremely dark red, almost a purple color and then shortly after go white and his oxygen level would go extremely low. For the first day, the doctors did not know what was happening.
They at first thought it was ‘reflux’ and ran a swallow test on him. He did have reflux but they said all babies have some reflux, and did not think that was what the “episodes” really were. As the “episodes” increased in quantity and duration, his primary pediatrician was pretty sure he was having seizures. A day and a half after the symptoms began they transported Garak to Primary Children's Hospital in Salt Lake City.


An hour and a half after arriving at Primary Children’s, Garak had another seizure. Luckily there were several Neonatologists and attendees there to see it firsthand, after watching it they all determined he was having seizures. They started Garak on Phenobarbital (Phenabarb), an anti-seizure medication, to try to control the seizures which were now occurring almost every hour. The Phenabarb sedated Garak for many days.

Garak had several tests which included 3 EEG's, a CAT scan, a MR Spectography, a MRI, ultrasounds, several spinal taps and hundreds of blood work tests. After the MR Spectography, the neurologist's concluded that Garak does have some minor brain damage in his basal ganglia area. The damage was so minor it took a 2^nd group of neurologists to find it. Since it was so minor, the doctors did not think the damage was causing the “episodes.” After the first EEG they also could not determine if he was in fact having seizures. Normally, with seizures, your brain waves would spike, Garak was not showing any spikes in is brain wave, in fact it was showing opposite, a decrease in brain waves. The neurologist thought the decrease was due to him being so sedated from the Phenabarb. At this point the doctors started testing for a lot of other things that may be causing the “episodes.” Numerous tests came back all normal or negative.

The Phenabarb knocked Garak out for a quite a few days which really worried everyone. At one point they were really concerned Garak would not wake up at all. However, around 1:00 p.m. on the 5th day, Garak woke up and Jailyn said it was one of her least stressful days. With Garak awake and almost a week after the first EEG, they decided to do a 2nd EEG on Garak. This time they concluded the decrease in brain waves was in fact Garak and not due to the medication in him. They labeled the decrease in brain waves as "burst-suppressions." Burst-suppressions are generally considered the next level of severity beyond generalized slowing. They include periodic patterns of electrocerebral inactivity. They also determined by the consistent pattern of his brain waves on the EEG right before and after each "episode," that what he was having was indeed seizures.

Since then, his Phenibarb levels have been all over the place. They have been struggling to find a maintenance dosage that will work. It's been a frustrating waiting game for Jailyn and Tyson, but amazingly, they have been troopers hanging in there. Jailyn said this is the hardest thing she has ever gone through. This is saying a lot for Jailyn because the previous six months have been a tremendous heartache for her. Lincoln, their second child, who is now 10 years old, went in for an operation to help lengthen his left leg. As Lincoln has grown up, his right lower-half of his body has grown faster than his left half. For the past 7 years, he has had to have lifts put on his left shoes to help him walk correctly. Doctor's at Shriner's Hospital and Primary Children’s Hospitals in Salt Lake City had a procedure to help lengthen Lincoln's leg. It's such a painful and scary operation, but it came to a point that Lincoln wanted it done for several reasons. It seemed like if something could go wrong, it did, and Lincoln ended up in surgery 8 times in a matter of 5 ½ months. Lincoln is at the point where he needs to go to physical therapy weekly, but with everything Jailyn and Tyson have been going through with Garak, that was postponed. We’re hoping to get Lincoln started this coming week. Back to Garak: After 3 weeks, the neurologist at Primary Children’s Hospital finally diagnosed him with Early Infantile Epileptic Encephalopathy (EIEE), also known as Ohtahara syndrome (OS). Taken from the University of Chicago website “Early Infantile Epileptic Encephalopath (EIEE) is a Severe form of epilepsy characterized by frequent tonic spasms with onset in the first few months of Life. EEG reveals suppression-burst patterns, characterized by high-voltage bursts alternating with almost falt suppression phases. Seizures are medically intractable with evolution to West Syndrome at 3-6 months of age and then Lennox-Gastaut Syndrome at 1-3 years of age.” With this very scary and sad diagnosis on hand, they are now waiting on a DNA test to try to pinpoint exactly which genetic deficiency he has, although not all cases can be lumped into the 4 EIEE deficiency brackets. These tests take 4-6 weeks so it’s been another waiting game. The term EIEE itself is pretty generic...just means seizure disorder in babies. At first when Tyson told us, he said not to Google it because you would only see bad outcomes. We have been hopeful that it was something else because not all of Garak's symptoms seem to fit EIEE. This is what Tyson said: "He does seem pretty cranky most of the time. Just wakes up to get mad, won't easily fall back to sleep. They say that is common with EIEE. Here is why I don't believe Garak's case is typical of what you may research: Most infants with EIEE have obvious brain malformations where Garak does not, besides the minor malformation in his Basal Ganglia which the doctors agree may just have been found because they looked so hard. They once told us the portion of the brain that controls thinking is just fine...yet apparently EIEE can result in mental retardation. They indicated his Basal Ganglia damage could result in motor skill issues, yet so far he seems to pass all their tests; he can move his eyes to look at whatever he pleases. So far all tests have come back negative, except for the Basal Ganglia finding, and his "severe" burst-suppression on his EEG."

The doctor's apprehensively sent Garak home after being in the hospital for 4 weeks on October 19th, 2011; but he's on oxygen and an oxygen/heart rate monitor. Later that afternoon he had 7 seizures in 2 hours. The next day he seemed to be doing a lot better, but on Friday, Saturday and Sunday, he had more seizures and was admitted back into Primary Children's on Sunday night. If they can maintain his Phenabarb level between 35-39 which is the highest they can go in infants, he doesn't seem to have as many seizures but the hard part is maintaining the Phenabarb levels since babies seem to metabolize medicines a lot quicker than others. Every 2-3 days since they left hospital, they have gone in to get Garak's Phenabarb level checked since he continues to have multiple seizures, some of which have been 10+ minuets long and where his oxygen level drops drastically. The last time he was checked, his level was at 45.5, which is way too high for an infant. However, the next day, he had 6 more seizures. At this point, the doctors felt like they should add a second anti-seizure medication, Keppra. Since Friday night he has been on both Phenabarb and Keppra and has only had 2 small seizures. We are hoping it will continue to work!! Right now I'm (Sheri) listening to him cutely hiccup.