Well, Grace is improving each day. She is an amazing child. She got settled into her new room on the Neurology floor yesterday and it did not take her long before she was asking to go to the playroom and to paint! She remembered diong that when she was there in January to start the diet. She is on contact precautions because of the pneumonia so she is not allowed in the playroom. However, she is allowed out of her room in 'her wagon'. Last night, the nurse rounded up a wagon for her and put the contact precautions signs on it. Tom has permission to take her around the hospital so long as she does not touch anything - open hallways are alright.
Grace is still on IV antibiotics and she is still getting IV fluids from time to time. It looks like she may be discharged if she can go without needing IV fluids for the rest of the day and tonight. She is very weak and still congested but she is more alert and sleeping less. She is talking a bit more - not when the doctors are present - but talking more nonetheless!
This is the ketogenic diet initiation week at CHOP for another set of families. Tom was asked to talk to the families this afternoon about our experiences with the diet. I'm sure that will be interesting!
I think Grace is ready to get home. She has been talking about Rita lots and lots. She has not seen Rita since Saturday. She is also talking about her friends and teachers at school. According to Tom, while being pulled in the wagon last night, she told him that one of her teachers is a 'slow poke' and someone else was a 'bumpy-head'. As the fog of her illness lifts, she is thinking about the people who are special to her - her sister, her teachers and her friends!
Wednesday, March 11, 2009
Tuesday, March 10, 2009
Coming out of ICU
Well, Grace is doing much better and is getting sprung from ICU this morning. Yesterday, they got her off of the dopamine and her blood pressure held steady. Last night, they got her off of the oxygen and her levels held fairly steady. She is still fighting the pneumonia so her oxygen levels are a bit lower than normal - but acceptable. They just removed her central line - the last barrier to coming out of ICU.
The diet has been a blessing and a curse. She has remained seizure free through the worst of this. She did have a few small seizures at the onset of the illness. However, given her history, everyone expected the seizures to come. The medicines to stop the seizures were at the ready should they be needed. Thankfully, this was not needed. The drawback from the diet has been her nutrition. When she was on the dopamine, she was not allowed to eat anything. Normally, the IV fluids would have dextrose in them and that would surfice. With the diet, Grace needs to have plain saline - no dextrose. With no fat coming in, she was buring whatever fat and muscle her body could find within. And she was hungry. Thankfully, she was allowed to have clear liquids last night and this morning she was allowed to have some real food for breakfast. It was with renewed appreciation that we measured out the heavy cream, oil and food this morning!
We are thankful for the doctor Friday night at St. Mary who had the sense that things were not quite right and had Grace transferred to CHOP. Then once she was admitted at CHOP, that doctor felt that things were heading downhill quickly and started some treatments immediately that really prevented this from getting worse. Several of the doctors in the ICU said that Grace would have been much worse off if we had waited to get her in. Our sweet baby has a guardian angel or two looking out for her!
Thank you for all of the prayers and good wishes. Grace bounced back quicker than they expected. We were being prepared for several days in the ICU before they expected her to stabilize on her own.
The diet has been a blessing and a curse. She has remained seizure free through the worst of this. She did have a few small seizures at the onset of the illness. However, given her history, everyone expected the seizures to come. The medicines to stop the seizures were at the ready should they be needed. Thankfully, this was not needed. The drawback from the diet has been her nutrition. When she was on the dopamine, she was not allowed to eat anything. Normally, the IV fluids would have dextrose in them and that would surfice. With the diet, Grace needs to have plain saline - no dextrose. With no fat coming in, she was buring whatever fat and muscle her body could find within. And she was hungry. Thankfully, she was allowed to have clear liquids last night and this morning she was allowed to have some real food for breakfast. It was with renewed appreciation that we measured out the heavy cream, oil and food this morning!
We are thankful for the doctor Friday night at St. Mary who had the sense that things were not quite right and had Grace transferred to CHOP. Then once she was admitted at CHOP, that doctor felt that things were heading downhill quickly and started some treatments immediately that really prevented this from getting worse. Several of the doctors in the ICU said that Grace would have been much worse off if we had waited to get her in. Our sweet baby has a guardian angel or two looking out for her!
Thank you for all of the prayers and good wishes. Grace bounced back quicker than they expected. We were being prepared for several days in the ICU before they expected her to stabilize on her own.
Sunday, March 08, 2009
Still in the hospital
Well Grace is still at CHOP. She has a confirmed viral pneumonia with a possible secondary bacterial infection. (Boy, I have been spending way too much time around medical professionals.) They started giving her some high-powered antibiotics while we await the results from the blood cultures. This will take a few days so rather than waiting to treat, they are asuming that she has the bacterial infection and treating it.
Grace has been on oxygen since Friday night. She has been having a lot of trouble keeping her blood pressure up and keeping her heart rate down. As a result, she was moved to the PICU unit Saturday night. She is being given Dopamine to help with the blood pressure. This required a higher level on monitoring than what was available on the regular wards. Prior to starting this medicine, they gave Grace lots of fluids - apparently a bit too much for her veins and her veins started to leak out some of the fluid into surrounding tissue.
The doctors decided that Grace needed a central line so she had one inserted today into her leg. This gives direct access to her arteries and gives her veins a small break. They gave her some medicine to sedate her for running the central line and also something to help her forget and block out the event. - I didn't know that was even possible. I always thought that was something just for James Bond.
As a result of everything they did for Grace, things seem to have stabalized for her. Her blood pressure is just a hare short of normal and her heart rate has fully come into the normal range. Her oxygen concentration is holding steady at 100%. This is all great news. Grace is not doing this on her own but with a lot of help from medicines and oxygen. However, we are all thrilled to see things stabalize - regardless of who or what is doing it right now! Grace will likely be here for a few days while they work to getting Grace to maintain this stabilization on her own.
Grace was very out of it on Saturday. The combination of high fever and low oxygen levels seemed to just wipe her out. As she has gained stability, she is able to concentrate more when she is awake. She has started to fight back when they try to do things to her - which everyone is happy to see. I hope they are still happy to see that when she is allowed to eat - that is usually a huge fight!
The most amazing this is that on Friday night when Tom and I were trying to decide what to do, Tom asked Grace it she wanted to go to the hospital. She actually said yes. We new at that point that she was really sick and knew it herself! We keep telling the doctors that Grace participated in this treatment decision!
Thank you to everyone who has been praying for Grace. I believe in the power of prayer so please know that it is greatly appreciated!
Marie
Grace has been on oxygen since Friday night. She has been having a lot of trouble keeping her blood pressure up and keeping her heart rate down. As a result, she was moved to the PICU unit Saturday night. She is being given Dopamine to help with the blood pressure. This required a higher level on monitoring than what was available on the regular wards. Prior to starting this medicine, they gave Grace lots of fluids - apparently a bit too much for her veins and her veins started to leak out some of the fluid into surrounding tissue.
The doctors decided that Grace needed a central line so she had one inserted today into her leg. This gives direct access to her arteries and gives her veins a small break. They gave her some medicine to sedate her for running the central line and also something to help her forget and block out the event. - I didn't know that was even possible. I always thought that was something just for James Bond.
As a result of everything they did for Grace, things seem to have stabalized for her. Her blood pressure is just a hare short of normal and her heart rate has fully come into the normal range. Her oxygen concentration is holding steady at 100%. This is all great news. Grace is not doing this on her own but with a lot of help from medicines and oxygen. However, we are all thrilled to see things stabalize - regardless of who or what is doing it right now! Grace will likely be here for a few days while they work to getting Grace to maintain this stabilization on her own.
Grace was very out of it on Saturday. The combination of high fever and low oxygen levels seemed to just wipe her out. As she has gained stability, she is able to concentrate more when she is awake. She has started to fight back when they try to do things to her - which everyone is happy to see. I hope they are still happy to see that when she is allowed to eat - that is usually a huge fight!
The most amazing this is that on Friday night when Tom and I were trying to decide what to do, Tom asked Grace it she wanted to go to the hospital. She actually said yes. We new at that point that she was really sick and knew it herself! We keep telling the doctors that Grace participated in this treatment decision!
Thank you to everyone who has been praying for Grace. I believe in the power of prayer so please know that it is greatly appreciated!
Marie
Saturday, March 07, 2009
Update on Grace
Just a quick update on the last few days. Grace is sick. She has had a few seizures over the last few days and she is running very high fevers. Friday night Grace spiked a fever of 104.5 while being o both Tylenol and Motrin. We took her to the Emergency Room. After spending the night in the ER, they transferred her to CHOP with a possible pneumonia (chest x-ray did not show it but symptoms indicated it), high fever and low oxygen saturation.
Tom went with Grace down to CHOP and I came home with Rita to take a nap and to pack up some things for the hospital - like the scale and some other things we need to support the diet. We did not think about this last night. For certain types of medicines, we are supposed to counter-balance the carb content with a fat. For instance, children's motrin has carbs for flavor. Grace is supposed to have a fat with that so that she stays in ketosis. We didn't bring any fats with us so she had to go all night without. The local hospital is not prepared to support kids on the diet like that. I'm not even sure that CHOP is prepared that way.
Rita is a trooper. We were going to drop her off at Tom's parents house last night but people are sick there and we didn't want any new germs coming home. The same is true for most people's homes that we would normally ask Rita to go. So, in the interest of containing the germs we currently have, Rita is trudging along from hospital to hospital. Thankfully, she looks at this as an adventure - and more playroom to explore. The promise of a McDonalds in the lobby holds great attraction as well !!
Well, I need to run. Rita is packing up some toys and coloring books for Grace as I type. The longer I type the bigger the pile grows.
Marie
Tom went with Grace down to CHOP and I came home with Rita to take a nap and to pack up some things for the hospital - like the scale and some other things we need to support the diet. We did not think about this last night. For certain types of medicines, we are supposed to counter-balance the carb content with a fat. For instance, children's motrin has carbs for flavor. Grace is supposed to have a fat with that so that she stays in ketosis. We didn't bring any fats with us so she had to go all night without. The local hospital is not prepared to support kids on the diet like that. I'm not even sure that CHOP is prepared that way.
Rita is a trooper. We were going to drop her off at Tom's parents house last night but people are sick there and we didn't want any new germs coming home. The same is true for most people's homes that we would normally ask Rita to go. So, in the interest of containing the germs we currently have, Rita is trudging along from hospital to hospital. Thankfully, she looks at this as an adventure - and more playroom to explore. The promise of a McDonalds in the lobby holds great attraction as well !!
Well, I need to run. Rita is packing up some toys and coloring books for Grace as I type. The longer I type the bigger the pile grows.
Marie
Tuesday, March 03, 2009
The streak resets
Well I may have tempted the fates a bit too much with my last post. Today Grace had a seizure and went to visit her friends at St. Mary. She also got a ride courtesy of our local ambulance company.
As I stopped at WaWa to pick up our heavy cream, I ran into the paramedic who treated Grace this morning. I have not seen him since he was at our house on Christmas day - 67 days ago. He stopped me to check how Grace was doing. He had some questions about the diet as well.
Grace caught whatever virus had Rita under the weather for the last five days. Rita was running a high fever and just generally felt miserable. We tried to keep the kids apart to keep the germs from spreading but they spread anyhow.
The good news is that this was a short seizure. It responded to the Diastat - an emergency rescue medicine to stop the seizures. In the past, Grace has been resistant to the Diastat and has had long seizures. In that regard, today was a victory for Grace and the diet. This was one of my measures of success when we first started this diet. We wanted the length/duration of Grace's seizures to decrease. We have been repeatedly told to expect break-through seizures.
Here is a photo of the girls from a few weeks ago. I think they look cute together. This was on Tom's camera phone - along with photos of feet from the ER today! I decided to include this one - no one wants to see feet!
Marie
Sunday, March 01, 2009
March 1st
It is hard to believe that we are at a new month already. Even harder to believe is that we made it through all of January and all of February with no Emergency Room visits!
Part of our discharge routine with the staff at St. Mary's ER would be a prediction of when we see each other next. Kind of like friends parting and deciding when we would meet up next. Of course, we were often invited to just come in to say hello or to perhaps just meet up in the grocery store. Generally, we would pick a date about a month out and say, see you then. I guess we were all resigned to the fact that Grace was a frequent visitor. As we left the ER on our last trip - Christmas Day, they joked, that they would see us in January. I told them that we were starting the diet in January so not to expect to see us until February.
Well February is over. Grace is still fighting with us about eating. But, we have not met up with our friends at St. Mary Medical Center in a while now! In December, making it one whole month seizure-free seemed liked a victory. Today is a reminder that this diet is giving Grace more seizure control than we thought possible as well as other unexpected little victories.
Tom and I have discussed sending a 'Thinking of you' card to our friends at the ER. Everyone there is great and genuinely seems to care about Gracie. They get to see her amazing transitions better than anyone else. A small little girl coming in on a stretcher just after a seizure or sometimes still seizing. They care and tend to her for few hours and usually before she leaves she is jumping on the bed! Grace often leaves the ER blowing kisses to everyone. I imagine they wonder what is going on.
I am starting to think that some of the fight Grace gives us is a matter of principle. Sometimes she seems to fight us right up until we get the sryinge up to her mouth at which point she stops fighting and takes what is given. When I look back, she did this a lot with medication as well. It is as if she feels an obligation to fight us - as if heart really is not in it. Then, of course, there are the other days when her heart is in it and the fight doesn't just stop with getting the syringe in to her mouth. I guess this is part of being a normal three-year-old.
I haven't had any photos to include lately. I let the kids use the camera to take some pictures and now I can't find it! It's time for me to check the purses and bags the kids play with. I'm sure it will show up soon. In the meantime, no photos for today!
A big thank you to all of the folks offering encouragement to us on this journey!
Marie, Tom, Rita and Grace
Part of our discharge routine with the staff at St. Mary's ER would be a prediction of when we see each other next. Kind of like friends parting and deciding when we would meet up next. Of course, we were often invited to just come in to say hello or to perhaps just meet up in the grocery store. Generally, we would pick a date about a month out and say, see you then. I guess we were all resigned to the fact that Grace was a frequent visitor. As we left the ER on our last trip - Christmas Day, they joked, that they would see us in January. I told them that we were starting the diet in January so not to expect to see us until February.
Well February is over. Grace is still fighting with us about eating. But, we have not met up with our friends at St. Mary Medical Center in a while now! In December, making it one whole month seizure-free seemed liked a victory. Today is a reminder that this diet is giving Grace more seizure control than we thought possible as well as other unexpected little victories.
Tom and I have discussed sending a 'Thinking of you' card to our friends at the ER. Everyone there is great and genuinely seems to care about Gracie. They get to see her amazing transitions better than anyone else. A small little girl coming in on a stretcher just after a seizure or sometimes still seizing. They care and tend to her for few hours and usually before she leaves she is jumping on the bed! Grace often leaves the ER blowing kisses to everyone. I imagine they wonder what is going on.
I am starting to think that some of the fight Grace gives us is a matter of principle. Sometimes she seems to fight us right up until we get the sryinge up to her mouth at which point she stops fighting and takes what is given. When I look back, she did this a lot with medication as well. It is as if she feels an obligation to fight us - as if heart really is not in it. Then, of course, there are the other days when her heart is in it and the fight doesn't just stop with getting the syringe in to her mouth. I guess this is part of being a normal three-year-old.
I haven't had any photos to include lately. I let the kids use the camera to take some pictures and now I can't find it! It's time for me to check the purses and bags the kids play with. I'm sure it will show up soon. In the meantime, no photos for today!
A big thank you to all of the folks offering encouragement to us on this journey!
Marie, Tom, Rita and Grace
Thursday, February 26, 2009
How we got here
As we struggle and fight with Gracie on a now daily basis to get her to eat, I keep needing to reassure myself that we are doing the right thing for her. In my head I know that we are but in my heart I keep questioning if we should keep this up for her. How bad would it be if we went back to where we were before the diet?
--------
Grace came home from China with us on December 23, 2006. On December 27, 2006, we took her for her first doctor appointment / physical. She was given some catch-up immunizations (4 to be exact). The next day Grace spiked a fever and had her first seizure. When the seizure hit, we didn't know what it was or what to do. At first we thought she was choking - but she had no food and her whole little body was shaking so that just didn't make sense. That was our first trip with Grace to St. Mary Medical Center. I remember the nurse explaining to us how frequently they see children who spike a fever and have a seizure. We were reassured that this was likely her first and last seizure. Most children who have a seizure never have another one in their lifetime. Grace was fully checked out - all of those routine labs were done and she had a chest x-ray. We left the ER that day thinking that we could leave all worries of seizures behind and move on.
One month later, Grace had a second seizure. She woke up early that morning and I brought her to lay with me in bed. She was snuggled up with me when the seizure hit. We now knew that it was a seizure right away. We called 911 and had her taken to the closest Emergency Room. From there Grace was transferred to CHOP - Children's Hospital of Philadelphia. As an aftermath of the seizure, Grace has these awful red dots/spots all over her face, head, arms and legs. Apparently these petechia are often indicators of some very scary and serious illnesses. When we got to CHOP, Grace was tested for everything under the sun. She had a spinal tap and an MRI, x-rays and tons of blood and urine tests. She was on all kinds of precautions and everyone who came to see her was covered head to toe and earing masks. She had consults with infectious diseases and neurology as well as general pediatrics. It turned out that she had RSV - a common respitory virus, the virus caused a fever which triggered the seizure and an unknown origin for the rash. That was the trip that got us hooked up with her present neurologist - we loved him from the start. We took Grace home with the understanding that some children are prone to febrile seizures when they spike a fever. Most children with these seizures have a few episodes during their childhood and grow out of it by age five. The neurologist discussed some medication options for seizure control but did not feel that was warranted at that point since all of her seizures had a known trigger. I was so glad that we didn't have to start any medications for Grace that she would have to take the rest of her life.
Grace continued to have the occassional seizure whenever she got sick. Fever continued to be the trigger for her seizures.
Grace had a major seizure in June 2007. This was the first time she seized with no obvious fever trigger. It lasted 50 minutes and when it was over she could not move the right side of her body. The ER doctor called me out of the room to say they thought she may have suffered a stroke in the midst of this seizure. They sent her for an emergency MRI of the brain while we waited for the CHOP transport team to come and get her. No stroke. And the best news of all - the paralysis was temporary. Her brain was tired from the seizure and took a break from sending signals to that part of her body. It was now time to start Grace on medication. Even thought this was only one non-fever triggered seizure and they ususally wait for two such events, the doctor thought it best to start her on medication then rather than wait. We were also given medication to administer at home for any seizure lasting over 5 minutes. We left the hospital with Grace on daily medications but the hope that the medications would help us to never get to that point again.
Grace continued to have seizures over the next few months both febrile and afebrile. Each time, we upped the dosage of her medicine until we hit a point that the medication seemed to be controlling her seizures. Grace went almost 4 months without a seizure. We once again relaxed - it seemed that we were past the worst of it. When the seizures started up again, they seemed to be a bit stronger and different. Grace also started to have longer and longer seizures. Once the seizure got hold of her, it was very hard to stop. We started changing medications and dosages and playing with it but nothing really gave her control again. Her neurologist recommended that we be aggressive. We wanted to stay ahead of the seizures and try to stop them before they did any lasting damage.
During the summer of 2008, Grace went into the hospital for long-term video eeg monitoring. Basically, they hook her up with an eeg machine and slowly decrease her medications to try to capture the seizures starting on the eeg. The point of this is to see if there is one area in her brain that was triggering all of her seizures and if so, was surgery an option. At the same time, we did some genetic testing on Grace. The end result, Grace was not a canidate for surgery. This was both the good and bad news - we did not have to make an agonizing decision regarding brain surgery on a two year old but it cut out one possible option to give her some hope of control. We discussed the ketogenic diet as an option for Grace at this time but we decided against it because we knew it would be difficult to keep her on the diet.
The genetic testing results came back about 6 or 7 weeks later. Grace tested positive for the SCN1A genetic mutation - she had a sodium channel mutation thus leading to Dravet's Syndrome. Once again good and bad news - we now knew exactly what we were dealing with but we didn't like it. We found a drug not yet authorized for use in the US that was found to really help kids with Dravet's. CHOP was willing to do the mountains of FDA paperwork to allow Grace to take this medicine. We were so sure that this would be the thing to give Gracie back some control. As we waited for thing to get rolling with this, Grace was having some trouble with her blood counts and the levels of the main drug she is taking and how it worked in her body.
In November of 2008, we were told that because of the problems with the blood levels of the one medication, Grace was not a canidate for the experimental drug. Our options at this point were a drug called Felbatol that has some very serious side effects or the ketogenic diet. It didn't take long for us to decide to go with the diet. For a patient to take Felbatol, you need to sign a consent form and acknowledge the risks of taking this medication. Of particular concern to us was the potential side effect of aplastic anemia - a cancer of the bone marrow. There really was no choice - Grace was starting the ketogenic diet.
So, here we are - knowing in our hearts that the ketogenic diet is right and good for Grace but feeling oh so conflicted when we need to hold her down and force her to drink heavy cream. We watch the days seizure-free counter go up and know that this diet has given Grace seizure control that she has not had in over a year and a half. And yet, we know that this is coming at a cost. On the days that she is happy and does not fight us, it is very easy to know that this diet is the right thing. But on the days when she is miserable, tired and weak, I find myself asking if we have sacrificed her quality of life for a few seizure-free days. Were the seizures really so bad? Can we just go back to where we were?
In the end, I think my ability to block out the fear and the bad memories is what is making my guilt so bad right now. I know that we are doing the right thing for Grace. I know that this is the best course of treatment for her disorder. I know that nothing will control her seizures as well as this diet is doing. I know that this diet gives her the best long-term prognosis. I just forget when she cries.
Marie
--------
Grace came home from China with us on December 23, 2006. On December 27, 2006, we took her for her first doctor appointment / physical. She was given some catch-up immunizations (4 to be exact). The next day Grace spiked a fever and had her first seizure. When the seizure hit, we didn't know what it was or what to do. At first we thought she was choking - but she had no food and her whole little body was shaking so that just didn't make sense. That was our first trip with Grace to St. Mary Medical Center. I remember the nurse explaining to us how frequently they see children who spike a fever and have a seizure. We were reassured that this was likely her first and last seizure. Most children who have a seizure never have another one in their lifetime. Grace was fully checked out - all of those routine labs were done and she had a chest x-ray. We left the ER that day thinking that we could leave all worries of seizures behind and move on.
One month later, Grace had a second seizure. She woke up early that morning and I brought her to lay with me in bed. She was snuggled up with me when the seizure hit. We now knew that it was a seizure right away. We called 911 and had her taken to the closest Emergency Room. From there Grace was transferred to CHOP - Children's Hospital of Philadelphia. As an aftermath of the seizure, Grace has these awful red dots/spots all over her face, head, arms and legs. Apparently these petechia are often indicators of some very scary and serious illnesses. When we got to CHOP, Grace was tested for everything under the sun. She had a spinal tap and an MRI, x-rays and tons of blood and urine tests. She was on all kinds of precautions and everyone who came to see her was covered head to toe and earing masks. She had consults with infectious diseases and neurology as well as general pediatrics. It turned out that she had RSV - a common respitory virus, the virus caused a fever which triggered the seizure and an unknown origin for the rash. That was the trip that got us hooked up with her present neurologist - we loved him from the start. We took Grace home with the understanding that some children are prone to febrile seizures when they spike a fever. Most children with these seizures have a few episodes during their childhood and grow out of it by age five. The neurologist discussed some medication options for seizure control but did not feel that was warranted at that point since all of her seizures had a known trigger. I was so glad that we didn't have to start any medications for Grace that she would have to take the rest of her life.
Grace continued to have the occassional seizure whenever she got sick. Fever continued to be the trigger for her seizures.
Grace had a major seizure in June 2007. This was the first time she seized with no obvious fever trigger. It lasted 50 minutes and when it was over she could not move the right side of her body. The ER doctor called me out of the room to say they thought she may have suffered a stroke in the midst of this seizure. They sent her for an emergency MRI of the brain while we waited for the CHOP transport team to come and get her. No stroke. And the best news of all - the paralysis was temporary. Her brain was tired from the seizure and took a break from sending signals to that part of her body. It was now time to start Grace on medication. Even thought this was only one non-fever triggered seizure and they ususally wait for two such events, the doctor thought it best to start her on medication then rather than wait. We were also given medication to administer at home for any seizure lasting over 5 minutes. We left the hospital with Grace on daily medications but the hope that the medications would help us to never get to that point again.
Grace continued to have seizures over the next few months both febrile and afebrile. Each time, we upped the dosage of her medicine until we hit a point that the medication seemed to be controlling her seizures. Grace went almost 4 months without a seizure. We once again relaxed - it seemed that we were past the worst of it. When the seizures started up again, they seemed to be a bit stronger and different. Grace also started to have longer and longer seizures. Once the seizure got hold of her, it was very hard to stop. We started changing medications and dosages and playing with it but nothing really gave her control again. Her neurologist recommended that we be aggressive. We wanted to stay ahead of the seizures and try to stop them before they did any lasting damage.
During the summer of 2008, Grace went into the hospital for long-term video eeg monitoring. Basically, they hook her up with an eeg machine and slowly decrease her medications to try to capture the seizures starting on the eeg. The point of this is to see if there is one area in her brain that was triggering all of her seizures and if so, was surgery an option. At the same time, we did some genetic testing on Grace. The end result, Grace was not a canidate for surgery. This was both the good and bad news - we did not have to make an agonizing decision regarding brain surgery on a two year old but it cut out one possible option to give her some hope of control. We discussed the ketogenic diet as an option for Grace at this time but we decided against it because we knew it would be difficult to keep her on the diet.
The genetic testing results came back about 6 or 7 weeks later. Grace tested positive for the SCN1A genetic mutation - she had a sodium channel mutation thus leading to Dravet's Syndrome. Once again good and bad news - we now knew exactly what we were dealing with but we didn't like it. We found a drug not yet authorized for use in the US that was found to really help kids with Dravet's. CHOP was willing to do the mountains of FDA paperwork to allow Grace to take this medicine. We were so sure that this would be the thing to give Gracie back some control. As we waited for thing to get rolling with this, Grace was having some trouble with her blood counts and the levels of the main drug she is taking and how it worked in her body.
In November of 2008, we were told that because of the problems with the blood levels of the one medication, Grace was not a canidate for the experimental drug. Our options at this point were a drug called Felbatol that has some very serious side effects or the ketogenic diet. It didn't take long for us to decide to go with the diet. For a patient to take Felbatol, you need to sign a consent form and acknowledge the risks of taking this medication. Of particular concern to us was the potential side effect of aplastic anemia - a cancer of the bone marrow. There really was no choice - Grace was starting the ketogenic diet.
So, here we are - knowing in our hearts that the ketogenic diet is right and good for Grace but feeling oh so conflicted when we need to hold her down and force her to drink heavy cream. We watch the days seizure-free counter go up and know that this diet has given Grace seizure control that she has not had in over a year and a half. And yet, we know that this is coming at a cost. On the days that she is happy and does not fight us, it is very easy to know that this diet is the right thing. But on the days when she is miserable, tired and weak, I find myself asking if we have sacrificed her quality of life for a few seizure-free days. Were the seizures really so bad? Can we just go back to where we were?
In the end, I think my ability to block out the fear and the bad memories is what is making my guilt so bad right now. I know that we are doing the right thing for Grace. I know that this is the best course of treatment for her disorder. I know that nothing will control her seizures as well as this diet is doing. I know that this diet gives her the best long-term prognosis. I just forget when she cries.
Marie
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