Baby Steps

Friday, December 30, 2011

The Ketogenic diet

We were admitted to Emory this afternoon to begin the ketogenic diet for Piper. It should take about 4 days to get it started while they monitor her. We will be making monthly visits to see how she is doing. The basics of the diet are.....

The ketogenic diet is a high-fat, adequate-protein, low-carbohydrate diet that in medicine is used primarily to treat difficult-to-control epilepsy in children. The diet mimics aspects of starvation by forcing the body to burn fats rather than carbohydrates. Normally, the carbohydrates contained in food are converted into glucose, which is then transported around the body and is particularly important in fuelling brain function. However, if there is very little carbohydrate in the diet, the liver converts fat into fatty acids and ketone bodies. The ketone bodies pass into the brain and replace glucose as an energy source. An elevated level of ketone bodies in the blood, a state known as ketosis, leads to a reduction in the frequency of epileptic seizures. (thanks Wikipedia)

So what this means for Piper is that her body will need to be in ketosis to get started. This means we will be doing an overnight fasting period. Fasting with an infant is insane to even think about!! She is able to drink water and diluted sprite zero. So far she had 8oz of water and 4oz of diluted sprite...not quite fooling her on this. It's going to be a long night for us but that's just part of it. After she goes into ketosis they will form a dietary plan for her diet. The nutritionist will meet with us tomorrow to decide on her plan. They make a special a formula for the diet that we will have to order online. Since she isn't eating solid foods it will be an easy transition. Once she gets older this will be an extremely complicated diet because We will have to measure and track every single thing that goes in her mouth. We will have to find special recipes for her and there will be absolutely no mistakes allowed or she could have seizures again.

The success rate for stopping infantile spasms is better than just general epilepsy. We really hope and pray this will be effective on her. It will not be an immediate change but over 3-6 months we should see if it will work. In the meantime she is still on two of her medicines and they are weening her off the other two. Hopefully she will be in a better mood without the steroids.

I will keep everyone posted!

Wednesday, December 28, 2011

Christmas and Piper

We were able to get out of the hospital for christmas. We went to Boone and stayed in a cabin with Justin's family. My mom, dad and sister were able to drive up on Christmas day and spent the night. We are so happy both of our families were able to be with us. We had a good time and glad we didn't spend Piper's first Christmas in the hospital. We picked out a tree from a tree farm and decorated it with popcorn and home made ornaments. We went for a hike, went skiing at Beech mt, played games and tried to relax as much as possible. Justin's white elephant gift he picked out was lottery duds this year, he won $500!! He said, "awesome, I can pay off some hospital bills!!"

Piper was put on steroids at Emory to try and stop the spasms. The initial doses worked well and decreased her seizures from 200+ to about 50 a day. We got our hopes up that this was going to be the medicine that finally worked. Unfortunately they have crept back up to 200 a day again. Disappointed is an understatement. I tried to enjoy our time away as much as possible but it's hard to do so when most of your time is spent counting seizures and making sure she gets all of her four medications on time. The hardest part is the seizures really bother her now which is new. She screams and screams before, during and after she has them now. This is absolutely heart wrenching. I could handle them when she didn't seem affected but now it kills me to even watch her knowing how much it makes her cry. My patience and strength are wearing thin. She is not able to sleep through the night anymore because the seizures wake her up every 2 hours. So we get about an hour of sleep at a time. I am physically and emotionally exhausted. I can't imagine how she feels. She just can't catch a break.

We called our neurologist at Emory today and he is going to call us tomorrow to let us know what he wants to do next. We are expecting to be admitted back in the hospital this week. We are living out of suitcases at this point. Our home has become the place we are together whether that is a hospital or somewhere else. It's not getting any easier because we all are so frustrated we can't stand it. We continue to pray for her seizures to stop. I don't know how much more Justin and I can take. There is nothing more devastating than watching your baby hurt and not be able to make them feel better, especially when it is pretty much the majority of every day. Please pray that we get a second wind.

The only thing that gets us through at this point is laughter and prayer. Laughing truly is the best medicine. We like to surround ourselves with people who are uplifting and light hearted. If we are around things that add to our stress and anxiety we quickly shut those things out. We are at the maximum stress possible and don't need any unnecessary stressors. Piper picks up on people's emotions so if you are calm and peaceful she will be too. She also picks up on crying, so we don't cry around her.

We also have a hard time right now being "normal" and doing
things like we used to. Going out in public is not the same because we don't like dealing with people looking at her while she is having seizures. It just hurts when people don't understand and we get tired of explaining it. So if we are not our usual social selves don't take it personally.

I will keep you updated this week as we travel back to Emory for our next steps.
Hope everyone had a great holiday!

Sunday, December 18, 2011

Pipers diagnosis

Well we finally have a diagnosis. She has infantile spasms, this is what we have been worried about the whole time. From the MRI and EEG they found that the right side of her brain has many abnormalities and is causing the problems. The left side of her brain looks relatively normal which is extremely unusual. One of her doctors said in 20 years he has only seen one case somewhat similar but it was still not the same. He said her brain is truly fascinating. With that said the left side of her brain isn't being affected by the right due to the absence of the corpus callosum. If she continues to have
seizures her left side could be affected.If this happens there is a possibility she will have to have brain surgery to remove or disconnect the part that is causing these spasms. Please pray that we don't have to do this because it is absolutely terrifying.

The main concern we have now is that she has all of the symptoms of a very rare genetic syndrome called Aicardi syndrome. It is a genetic condition where there is an mutation on the x chromosome so it only occurs in girls. The prognosis for the syndrome varies and most cases are not so good. Genetic testing will done at some point but controlling the spasms is first priority. To control to spams steroids are used. She was started on a loading dose of steroids today. She will continue for 3 days to see if there is any improvement. The steroids increase her metabolism and decrease her immunity so we have to be very careful that she isn't around anyone who is sick. She is also being taken off one of her 3 other anticonvulsants so hopefully we will see the happy baby we are used to.


We feel very lucky to be here at Emory and will not be going back to Erlanger since these will be her primary neurologists.

Tomorrow is her 4 month birthday! Its amazing that 4 months have already gone by but at the same time the past month has taken forever. We figured out that she has spent a quarter of her life in the hospital. It's a good thing she is little and won't remember any of it.

We appreciate all of the continued prayers and support. The encouraging words do so much for us and help us continue to stay strong.

Thursday, December 15, 2011

Emory visit

We came down to Emory yesterday to meet with the epileptologist and see if they have more insight about piper's condition. We were told by the neurologist at Erlanger that all of our records, MRIs and EEGs would be sent over for review. When we got here yesterday the doctor had no idea why we were here and did not have the MRI or EEG to study. We are so frustrated with the lack of communication at Erlanger. We would have brought them ourselves but even requesting a copy takes so long that we didn't have time to get them ourselves.
The doctor decided he wanted his own EEG done to study. Apparently he specializes in reading EEGs so that is a good thing. He was very concerned that she is still having 200+ small seizures a day. He admitted us to the er and were admitted to the hospital yesterday evening.this was a huge surprise because we were told this was a consultation visit so we didn't pack a bag or bring anything. Justin went to target last night to get the basics.
They had to draw a lot of blood because they are doing every test possible. Today they will do another EEG and more testing. We will see what they say about the results and go from there.
The main reason why we thought we were coming here is to talk about the ketogenic diet. We were told they work with infants her age but the doctor said otherwise. They are adamant about not starting it until 6 months which is a huge disappoint for us.
We don't know how long we will be here or what the plan will be. The visit is already so much better than our experience at Erlanger so that helps. We will keep you posted.

Monday, December 5, 2011


We were released on Saturday to go home and monitor Piper. It got to the point at the hospital where we were really only talking to our neurologist and all of the other doctors and nurses didn't have much to say besides wait and see what the neurologist says. It was getting a little frustrating having to repeat the entire story of the past 18 days to a new doctor and nurse everyday.We felt comfortable coming home to watch her here.

Her new medication should be established by next week and we are monitoring her like we did in the hospital. Our methods of monitoring her and keeping data are pretty detailed, I guess that's what you get with an engineer and an OCD teacher. We write down everything she does so when we go to the epileptic specialist we will be prepared. She is still having small seizures but nothing compared to what we saw the first time. They seem to be a little better on this medicine but not by much. She is having them at night and it wakes her up out of her sleep. She also has her days and night confused again so she isn't sleeping very well at night. Hopefully we can get her back on schedule soon.

We go on Wednesday morning to get her blood drawn so they can do a trough on her levels. This is measuring the amount of medicine in her blood to determine if it is at a therapeutic level. If it is at a high level and she is still having seizures it means the medicine has failed and we try something else. There are not a lot of options left as far as meds go because she is so little.

We have an appointment at Emory for jan 26 but they are trying to get us in earlier. Justin will be traveling for 2 weeks in jan so hopefully we can go when he is able to. The specialist is going to help us get started on a diet that is used called the ketogenic diet. It is an intense diet and you have to measure and keep track of everything that goes into her body. From the research we have done it works well when medicine has failed. It will be a full time job for me just preparing her meals but if it works it's worth it.

She is still happy and playing as much as she can between naps. The meds make her very mellow and sleepy but after a while she should get used to them. We are happy to be home and feel very thankful that things are mostly under control.

Thursday, December 1, 2011

Day 16

This is Piper's second day on the new medicine. It doesn't seem to be doing much and she had more episodes than normal through the night. Very frustrating. We were discussing going home today if everything looked good. Since she the number of episodes has increased, we will have to stay and figure it out This is the same thing that happened last time we tried to go home :( I'm sitting here waiting on the neurologist to see what he plans on doing. I think his plan is to increase the dosage and see what happens. We will most likely have to stay here another day to see if it helps.

She is still as happy as she can be which continues to make the situation easier.

There was a Christmas parade in the lobby of the hospital and she got her first picture with Santa! She loved getting out of the room for a few minutes to see a new place. It was nice to have a change of pace.