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.


Monday, February 23, 2009

Small Victories

We are still working on getting Grace to eat. We had some successes lately with some new recipies.

Thank you to Melinda and Mike for the KetoCal Pancake recipe. It worked like a charm! She wasn't letting go of the plate until she ate up every last crumb. We are saving that to make again on Wednesday for her lunch when the other kids get pizza. Hopefully something as enticing as the pancakes will get her to eat her lunch that day!

Tom tried some egg souflees - a few different ways. However, Grace was willing to try each one out and generally ate a good bit of it each time. He tried egg white patties. Grace called them crackers and ate them without question.

Tom is also getting very inventive with the soups. We start with heavy cream and butter and go from there. Sometimes we add cheese, seafood, bacon, chicken broth, chicken. Basically, anything we can find that we think she might eat. The soups are great because they mask the heavy cream for her.

One of the big winners of late is what Grace calls 'Pizza cheese'. We take her serving of protein as mozzarella cheese and add a dab of marinara sauce and melt. We take the cheese apart and make little balls. They harden back up rather quickly. Grace picks them up and pops them in her mouth. She usually gets a plateful of them this way. She had that for three meals over the weekend!! It doesn't do anything for the heavy cream so that is the only drawback.

Since little cheese balls were a big hit, Tom made her little meatballs one night. He ground everything up in the chopper and fried up the smallest meatballs I've ever seen. Grace got a lot of them that way.

We are also having success with french fries - eggplant sliced up like shoestring french fries and fried up and salted. Grace has accespted these as french fries and she is happy to get them. We cut up the eggplant and frooze them in little single serve portions. It is easier to take a pre-measured bag out of the freezer and cook them up.

Thank you for the prayers and the encouragement!
Marie, Tom, Rita and Gracie

Friday, February 20, 2009

"Behavioral Issues"

Well we had our follow-up appointment yesterday. Grace lost more weight - as we have been noting at home as well. Neurologically, she is doing great. Her regular neurologist as well as the epiepsy specialist who runs the program were both quite pleased with her progress.
We discussed the not eating bit. The nurtitionist and the nurse told us that the diet is fine and medically she is fine; it is all just behavioral issues at this point. They recommended that we might want to consult with a therapist. We were told that there was another child in the program like Grace who would not eat. They went to a therapist so the mother could learn how to get better control and get this child to eat.
Tom thinks I took the suggestions the wrong way but I was annoyed. I do not think that Grace not eating is a 'behavioral issue'. She is a otherwise normal three-year old who loved to eat. And, she loved to eat healthy foods. She would have happily survived on fruits and vegetables but she was always willing to try new things. We have taken away so many of her favorite foods or severely limited the quantities she is allowed to eat. I don't think it is abnormal for her to be annoyed about it. We didn;t ask her if she wanted to do this. We made this decision on her behalf. I still think we made the right choice but I think she is entitled to be annoyed about it.
Then I was annoyed at the suggestion that we, the parents, are the issue. When Grace refuses to eat, we try something else. We have been working very hard to get those calories into her. We were asked if we tried just giving her what was prepared the first time and if she doesn't eat it, let her go hungry. This just flies in the face of everything we are trying to do for her. Grace is very strong-willed (I think that is the nice way to say stubborn!!). When she refuses to eat, she could be starving and she will not give in. When we let Grace go too long with not eating, she gets low blood sugars. I guess I would rather fight her to take a syringe of oil or heavy cream than have her get a low blood sugar.
I also think that in the matter of a month, Grace has come a long way with this diet. She is back to trying some foods that she refused to eat early on. She no longer sits and cries when she sees someone eating something she can't have. She acknowledges that there are foods that she is not allowed to eat. However, on Chick-fil-A day at daycare, she smells the foods she loves and sees other kids eating this. Of course she is annoyed. Of course she refuses to eat what we send in for her. She is mad about this diet and she is letting everyone know it in her way. If she was throwing tantrums or being disruptive or destructive, I would agree that she needs help.
I am getting down off of my soap box now. On the whole, the diet is going well. We are not ready to quit or give up. We are working with her to get her to eat more. We worked with the nutritionist to increase her calories to helpfully get some weight back onto Gracie. We are working on changing her suppliments so that hopefully she will not have lingering nutritional issues. The current suppliments go into her food and when she refuses to eat, she is also not getting the right suppliments - or at least not the right amount. She is also doing better drinking. She is not yet to the target that is set ofr fluid consupmtion but she is doing better than when she first started on this diet.
Thanks for the support and kind words.
Marie, Tom, Rita and Gracie

Tuesday, February 17, 2009

Battling colds

We are all battling colds right now. Grace started with the sniffles on Friday and it progressed through the weekend. Her sleeping and her appetite are off. As I sit here typing, Tom is working to get some dinner into her.

Grace continues to be stubborn. The other morning she woke up and announced that she wanted pancakes for breakfast. Well, pancakes are not on her diet plan. Grace didn't much care, it was pancakes or nothing for her. She snubbed her nose at the porkroll with cheese that Tom made for her instead. Usually, she loves porkroll and cheese. That was one of the mornings we had to force feed her. She ate some of the porkroll but we had to put the oil and heavy cream in syringes and force them into her mouth. It's not pretty but we are willing to battle for every calorie we can get into her!

It is so tough to just not give her what she wants to eat. The puppy dog eyes and pouty lips are really hard to say no to over and over. It's even sadder when what she wants is a reasonable request. She is sick and wants orange juice. It is not on the plan. She wants more carrots with her dinner but she already ate her portion of carbs for the meal. She wants fresh fruit and we have to tell her no. Some of this just defies logic and common sense. However, the diet is working for her. So as hard as it is to tell her no, we keep with it. We are not ready to give up now.

We have our next follow up visit for the ketogenic diet on Thursday. This time we will see the nurse practitioner, the nutritionist and her regular neurologist. We are waiting to see what labs they want to do on her that morning prior to her visit. We don't like having to have her blood drawn but it is SO much easier at the hospital than at the outside lab. For starters, the folks at the hospital deal with children who have trouble getting blood drawn on a routine basis. They often have success on the first stick. Sometimes it takes a secnd stick and rarely a third try. Outside of CHOP, it usually takes 4 or 5 sticks or more to get Grace's blood drawn. Secondly, the hospital labs get results back within hours whereas the outside lab takes two weeks. (yes, hours versus 14 days!) I will be anxious to see what her regular neurologist has to say about Grace's progress on the diet.

Thanks for all of the prayers and support!
Marie, Tom, Rita and Gracie

Friday, February 13, 2009

Some we win; Some we lose

We continue to battle to get Grace to eat. In true Grace fashion, she continues to change her tastes and appetite practically daily! There are some days that we are successful and we get her to eat all of her meals. There are a few days when we are overly successful and she actually eats more than her meals. Then there are the days when she will not eat all of her meals - skipping entire meals. We fight to make these type of days not outnumber the successful days.

We keep trying to change her menu plan so that she will not get tired of foods that are working. We have tried some of the great recipies folks have shared with us. We are on the lookout for new ideas and ways to get her to drink her heavy cream and to eat her food. When we can make the heavy cream part of the food that seems to work more often that not.

Grace is holding realtively steady at the 25 pound mark. We would like to see her put on a bit more weight but we are happy that she is not losing pounds. We weigh her each morning prior to breakfast. If we can;t have continual upward progress, at least it is only fluctuating by ounces rather than pounds!

Despite the battles and struggles, we see the benefits. Grace just hit the 50 day mark for being seizure free. This is tremendous for her. The last time she had a seizure break this long was over a year and a half ago! We listen to the stories of others whose children are on this diet and we are heartened by their successes as well. This diet really works! We have seen it work for Grace already. She deserves the chances that this diet will give her. For that, it is worth the fight. Grace is worth the fight!

I have been asked a lot recently about how the diet works. Basically, the idea behind the diet is that she will use fat as her energy source rather than carbohydrates. This is called ketosis. When this happens, her body produces a chemical that supresses the seizures. She gets so little carbs to keep her body deep in ketosis. This his how her body can produce enough of this special chemical. Grace takes supplements to help provide her body with the neutrients that she is no longer getting in her food. The diet is done under the care of a team of doctors, nurses and nutritionists to ensure that no long-term damage is being done to her.

I want to give a big thank you to WaWa. We got set up on the WaWa Heavy Cream Program. This means that once a week, we get two quarts of special WaWa Heavy Cream delivered to our local WaWa - free of charge! We just show up and ask for it and they just give it away! This is a great program. The hospital has a deal with WaWa to perform this service. WaWa donates the heavy cream to any child in their delivery area who gets referred by the hospital. It sure makes me feel good about all of the times I drop into WaWa for something!!

Tuesday, February 10, 2009

The sum of the parts

Grace is adopted from China. So is her big sister Rita. Being Chinese is a part of who they are. We are quite proud of our girls and their heritage. We are so blessed that they have graced our lives. They are our daughters.

Grace is an epileptic. This is part of who she is. It means that she gets seizures. It means that she has had many, many visits to the Emergency Room - most of which start with a call to 911 and an ambulance ride. It means that she has had so many IV lines and blood draws that her veins are now so scarred that accessing them is anything but simple. It means that we watch her very closely. It means that she takes multiple medicines, multiple times a day. It means that we all struggle to keep her on this diet to hopefully prevent future seizures or to at least lessen their impact when they do hit.

However, none of these things define Grace on their own. She is a funny, vivacious, caring, stubborn, loving, imaginative child who happens to be Chinese, an adoptee and an epileptic. She is so much more than this pervasive chronic disease.

Grace's neurologist is a wonderful man. We all love him. He sees Grace as a child first, a patient and as an afterthought that she is adopted. Last summer, Grace was tested for a sodium channel mutation. The results were positive for a SCN1A genetic mutation. The diagnosis of that is Dravet's syndrome. This is a severe, hard to control with medication form of epilepsy. As her doctor likely does with most patients' parents that he delivers news of a genetic mutation, he recommended that we go for genetic testing to see if we are carriers. And then, he stopped himself, looked slightly embaressed and said nevermind. He just remembered that Grace is adopted and despite everything we share, we do not share the same genetics. I love that this was an afterthough to him. The fact that Grace is adopted does not define her to him; he doesn't even think about it.

I have had to laugh at Tom from time to time (OK a lot of times!). When we were in an emergency room in Florida one time with Grace, a doctor asked where Grace was from and Tom replied Pennsylvania. A very natural response to a common question. The doctor paused for a moment and then asked if she was adopted.

One of my biggest fears for Grace is that she will be defined by one label or another. That someone will take one facet of who she is and limit their perception of her to this one thing. Our tiny, sweet, little girls is made up of so many wonderful parts. They all combine to make her who she is. She is Grace.

Sunday, February 08, 2009


I know that I am stubborn. However, Grace gives us a run that I wouldn't think possible. I am often times amazed at just how stubborn a three-year old can be.

When Grace does not want to eat, there is no amount of cajoling or bribing that will get her to eat. Force feeding only works so far. She has learned that if she does not swallow what we push in, we can not force in any more!

Grace is stil at just over 25 pounds. We continue to struggle to get her to eat - and to make things that encourage her to eat! We are most grateful to our new on-line friends who shared some great keto recipies and suggestions. We made a run to Whole Foods and got some of the ingredients needed for the new food ideas and we started on some of them this weekend. My biggest surprise was the tofu spaghetti noodles. We thought we woud give it a try; not really sure they would work. Grace ate two bowls of them! Of course, they were coated in an alfredo sauce of heavy cream, butter and parmesean cheese! I am hoping that the scale will reflect the fact that she ate this weekend!!

On a non-diet note, Rita seems to have taken off with reading on her own this week. In kindergarten, they have been making little books in school each week. Every night she is to read one of these books to us and we read a different one to her. Up until now, she has basically been memorizing the words to her books and then reading them back. This week, Rita started taking the books she picked for us to read to her and she started reading them to us. We could really see the difference when she started reading 'Fox in Sox'. This is a big book and we watched as she sounded out the various words. It is amazing to watch her be able to piece the words together and to unlock that magic of the letters on the page. Her face lights up with the magic of being able to read the pages for herself.
All the Best!
Marie, Tom, Rita and Gracie

Wednesday, February 04, 2009

Happy Birthday - Happy Everyday

Thank goodness for birthdays and the candles! Grace was very excited prior to her birthday and had to practice - A LOT! Multiple times a day we would sing Happy Birhtday to Grace and she would pretend to blow out the candles. Since her birthday, we continue to practice but now the candles are real! It has become a great reward for completing a meal!

It is becoming harder and harder to get Grace to complete all of her meals and snacks in a day. She lost all of the weight she gained since she has been home. She is just a hair over 25 pounds right now. She is very thin and bony. We work to get every calorie into her that we can. Many times it means using a syringe to get the heavy cream and oil into her. She does not fight the oil much. However, she acts like the heavy cream is absolute poisin! Grace is refusing to eat at most meals now. Worse than refusing the whole meal, she will eat everything in one category and refuse the rest. Such as, she will eat all of the protein or carb and none of the fat or heavy cream. Since the diet is based on ratios, this does not work.

We have had some successes with the fluids. She did not like the keto-kool-aid when we started the diet. However, the other day she helped me to make some and she is drinking it. I just keep reminding her that it is the juice we made together and she will have it. She was getting tired of decaf tea, diet grape soda, diet ginger ale and water. We expended her diet soda to include caffine-free diet cola and diet birch beer. (She will not drink the diet orange soda.) I made her special hot chocolate the other day and this time she drank most of it. We have been trying once a week but she has not liked it until this week. The hot chocolate is heavy cream with sugar-free cocoa powder and some sweet-and-low sweetner drops. I also add some water to the mix. It doesn't taste so bad!

The biggest success we found this week is sugar-free jello. Grace gets 2 cups of this per protein exchange so we can usually include one cup for half an exchange. She gets 2.2 protein exchanges per meal. We have managed to whip up some of the heavy cream and get her to eat it with the jello.

We continue to work on getting her to eat. If singing happy birthday or dancing around the kitchen gets her to eat, then that is what we do! When all else fails, we force her where we can. We switch protein and carb exchanges on the fly so that if she says she will eat a food we didn't plan on, we try to accommodate her taste at the moment as much as we can. At this point, we are happy with every complete meal we can get her to eat. We need her to put that lone pound back on and hopefully a few more to go with it!

Thanks for all of the great comments and support. We appreciate it!

Marie, Tom, Rita and Gracie - the perpetual birthday girl!!

Sunday, February 01, 2009

We are having our ups and downs with the eating. Grace is getting better with some parts of the meals and worse with others. At this point, she is tired of the heavy cream. Unfortunately, the heavy cream is a staple of her diet. Every meal and snack includes heavy cream. If we were to take away the heavy cream, she would need more stand-alone fat. So, this is where we are at right now!
Gracie is back down some of the weight she was able to put on. A few days of not eating complete meals is enough to shed some weight for her. We continue to struggle to get all of her calories into her each day. It is still worth the struggle though :-)

Here are some photos of the girls. Gracie got a vanity for her birthday. Her birthday was January 3rd but we just got around to opening the box last night! Rita has taken over as the head beauty shop consultant. She instructs Gracie where to sit or stand to have her hair or make-up done. It is somewhat comical to watch them. One of these days Grace is going to stop being the one ordered about. That will be a sad day for Rita! For now, Grace just follows the orders she is given.
Marie, Tom, Rita and Gracie