Four days of steroid freedom

Marek has had a very busy week. Monday he went to get his blood ran for the Keto diet and Topamax. They also drew blood for genetic testing. Tuesday he had OT, Wednesday was PT, Today he had an appointment with his neurologists and tomorrow he has DT.

I will start with the positives. Marek has found his voice again. While on ACTH he never babbled at all. The day of his last injection his speech came back full force. We even have a new HAPPY scream that is his preferred sound right now. He isn't using any consonants but is stringing together just about every vowel, at every VOLUME. He has also had his personality and demeanor return this week. He laughs at everything and has hardly cried all week. He slept for his longest stretch in months, FOUR whole hours. I didn't even mind the fact that he was then awake for the next four hours since he entertained me from midnight until 4am with lots of "stories" and giggles.

The not so good news is that he has regressed all the way back to where he was at the beginning of January. He has very low tone in his trunk, legs and arms. He hasn't rolled over in two weeks and can no longer sit up unassisted for any length of time. I think that is what is the most painful, watching him lose all the skills he has worked SO hard to gain. It is also hard to stomach that when he was first diagnosed he was 5 months delayed, now he is nearly 8 months delayed.  He is having what we think are seizures/spasms multiple times a day now. They are not the classic spasms that we are used to but they are spasm like in the fact that his arms go up and down and they are clustered together. The neurologists feel that since he has regressed that it is a possibility that his hypsarrythmia has returned. They don't feel the need to run an EEG since it wouldn't change their course of treatment so we will never know if his hypsarrythmia has come back at full force. The doctors told us that they are going to use Marek's development as their guide for treatment. If he starts to develop again, then we will assume he is responding to the medicines he is on. It is hard for us because we want an EEG to tell us exactly what is going on in his little brain but I guess it makes sense that if he starts to develop his brain activity must be normalizing.

On Monday we started Topamax (this was the drug he was initially on that made his bicarbs drop) we stayed at a low dose all week and his bicarb levels were checked today. The good news is that they are still within the normal limits so we can continue to stay on this medication. The doctors want to titrate up the dose as fast as possible to see if he has success with it quickly. This way if he doesn't show any improvement they can take him off of it as soon as possible since he can't be on Topamax when he starts the Keto diet. We are not out of the woods with his bicarb levels though, they can still drop at any time but at least this is a better start than the first time we were on Topamax. We will also be going BACK on steroids tomorrow. Marek will be on Prednisolone. This steroid is not as powerful as ACTH but our hope is that if he responded to one type of steroid he will respond to another. Prednisolone comes with the same side effects as ACTH so we will be keeping Marek isolated as much as possible. I am keeping my fingers crossed that the irritability and insomnia won't be so bad. I also can't imagine him gaining any more weight, he is up to 24 lbs and could barely open his eyes the last week of ACTH. However, with a new treatment plan comes new HOPE. We are hopeful that maybe this new treatment will be the miracle combo of medications that will cure Marek of IS.

Our neurologists in St. Louis are now looking into seeing if Marek is a surgical candidate. They are going to do a PET scan on him in the next month to see if there are any lesions on his brain that hadn't shown on the MRI and to also see if there is a single focal point to Marek's seizure activity. This is the same thing Dr. Chugani from Detroit wants to do so we will have to see if insurance would cover two PET scans. We were told that all of Marek's labs came back a "go" for the Keto diet so he will start that if Prednisolone doesn't work.

So we are praying for these new drugs to work, that God keeps our little guy safe from their harmful side effects and that they help him to start to make gains in his development again.

Short Lived Freedom

Marek had been doing well since our trip to Detroit in regards to his spasms. We were noticing only one cluster a day and with less than 10 spasms in each cluster. Then we went a day with out spasms. As the 24 hour mark approached I could feel my anxiety rising. We hadn't gone spasm free for 24 hours in months. My mom and I knew it was approaching but we couldn't talk about it out loud to each other or anyone else. We were scared that some how we would jinx Marek and the spasms would return. 24 hours came and went with no spasms. My heartbeat must have doubled ever since then because of sheer joy as well as out of fear. I watched Marek closer than any Mom watches their child. It has become a habit that every few minutes I have to assess what he is doing with his eyes and arm movements. Well after we went 24 hrs without a cluster I was almost scared to look at him because I was scared I would see his eyes roll up and his arms go stiff. The pit in my stomach grew three sizes because I never knew what the next hour would hold for Marek. 24 hours passed by, then 48, then 72.....we were on a roll yet the fear, anxiety and tension never let up.

We went down to to one shot a day on Sunday. I told Ryan how nervous I was about this because for 7 weeks now Marek has had a dose of steroids in his system. Sunday came and went, no spasms. Monday we spent the day outside and Marek was laughing the whole day. I let myself for one minute think that maybe, just maybe, I would never have to see my son have another spasms ever again. That is when it happened. Right before midnight Marek was drinking his bottle and in the dim light out of the corner of my eye I saw him move his arms in such a way that deep down I knew what I saw was a spasms.  But I tried to stay positive, I started pleading to God to please have that be just a shadow I saw, or my mind playing tricks on me. I turned the light on and watched Marek so closely over the next few minutes, I didn't even blink in fear that I would miss something. Then it happened, his eyes rolled up and his arms flew outward and stiffened. The damn things were back. My heart literally broke into a thousand pieces and I just broke down. It was as if I got the diagnosis that Marek had IS all over again. I had to get my mom at this point so that she could help me since it took all I had to keep it together and to help me count his spasms. For 30 minutes I watched him have spasms after spasms. This was the longest cluster he had had for weeks. I don't understand why they had to come back, why to my son? After Marek has been through so much why would God let the spasms come back?

The next morning I tried to come up with some sort of explanation as to why this was happening. Ryan told me that maybe it was just a breakthrough seizure. Many parents report having spasm freedom then they see a spasm come through and they adjust the meds. I prayed all day that this was just a hiccup. I kept trying to convince myself that his spasms weren't coming back, it was a breakthrough spasms, but I was never able to fully believe that. I had learned my lesson, guard my heart so that it wouldn't break again. Tuesday night, Marek had another cluster of spasms. It never gets any easier watching him go through this. My whole world crashes down around me every time I have to stare at him and count how  many times his small body is made to do things he doesn't want it to. IT ISN'T FAIR!

My biggest fear now that the spasms are back is that they will take away all the progress that Marek has worked so hard to gain. Marek hasn't rolled over since Sunday and he is hardly picking his head off the ground when he is on his tummy. I hope that WHEN we get the spasms back under control these skills will quickly come back.

So it has been a crappy week to say the least. I don't know what this means as far as what our next treatment will be but I am eager to find out on Monday.

We ask that you please keep praying for our little guy, this is just not fair to him.

Easy as pie...or cake

Last week Sunday, we celebrated my birthday. This was Marek's favorite part of the weekend because he was able to eat cake! He loved it! Birthdays are that much more special now that I am celebrating them as a Mommy!  I loved watching how FAST he ate the frosting. We figured that we better let him enjoy cake now because if he is on the Ketogenic diet for his first birthday, cake won't be on the menu. It was a great night, however the nurse wasn't too happy Monday morning when his glucose level was high but after explaining that cake was the reason she wasn't as worried.

Monday, the Neurologist called me to tell us he had the final report from the EEG. He gave us great news that he does not have hypsarrythmia anymore. He has what they call Epileptic Encephalopathy which means he still has a chaotic and unorganized EEG but is less severe than hypsarrythmia. Encephalopathy still causes problems of cognitive development but at a less catastrophic level than hypsarrythmia. Marek has still had spasms this week and unfortunately on top of this the Neurologist now thinks that he might be having other seizure types. We started seeing him do different movements the last couple weeks but were unsure what to think of it. I finally caught it on video and sent it to the Neurologist. After reviewing the video the neurologist feels that this type of movement is seizure activity not spasm activity. The doctors won't know this for sure until these movements are captured during an EEG. Depending on what type of seizure they diagnose him with will depend on what anti-epileptic medicine we add to our fight. We are still unsure what treatment plan for IS we will do next. The doctors really want to wait until the last day of ACTH to make that decision. We could do Prednisolone (oral steroid) Zonegran/Topamax (anti-epileptic drug for IS) or the Ketogenic diet.

This past Tuesday, we went to get Marek's vision checked. He had an eye exam last month which showed that his actual eye was healthy but he was still having trouble with his vision (the problem lies with how the brain receives the image and deciphers it) which got him the diagnosis Cortical Vision Impairment. The eye doctor wanted to measure how impaired his vision was so he scheduled the eye appointment that we went to on Tuesday. They hooked Marek up to a few leads, the same ones they use during an EEG so that they can measure how fast images are received in his brain and how much activity is going on in the Occipital lobe of his brain. He got to wear a pirate patch and all! He did a great job watching all the lights and trying to follow the objects. We will get the results in the next two weeks.

On Monday, March 23rd we will be going to get blood work done at Children's. The first set of blood workup will be for the Ketogenic diet to assess if he is a candidate of the diet. The second workup will be for genetic testing. It has taken over a month to get insurance to approve this testing.  Infantile Spasms is sometimes caused by a mutation of a gene. We are keeping our fingers crossed that the genetic testing comes back normal. Please pray for good results for Marek.

Occupational therapy came for the first time Tuesday. We didn't get much accomplished because Marek was so upset. Marek is still having a hard time on the wean. We are now at the lowest dose amount twice a day, then we step down to once a day, then to every other day. His last injection is a week from Sunday. I am hoping that as the steroids get out of his system that #1 HE DOESN'T INCREASE SPASMS and #2 we quickly get rid of the irritability. Our little guy is just miserable.

At the end of last weekend, Marek cut his first tooth. We have thought that Marek has been teething for months now but we never saw or felt anything. On Saturday night,  last week we felt his lower right tooth. Our poor guy is so tough, he is fighting so hard to beat IS and is now OFFICIALLY teething.

Physical therapy has been going well. Patti his PT was very pleased by everything he was able to do this week. Marek has graduated OFF the wedge since he can now pick his head up off the ground. We are now working on having him push up to a sitting position. The PT thought that his back and left side were getting tight so we will be stretching a lot this week and really working on his tummy muscles.

Ryan attended the church service that was put on for Marek. He said it was very moving and emotional. We thank everyone that came out to pray for our peanut at church and like always we thank everyone that has continued to pray at home.

Drum roll please................. We have reached our first MILESTONE. Marek can now roll tummy to back and back to tummy. We have noticed with Marek that if he can do something once, HE HAS GOT IT! He is rolling over like crazy, easy as pie! So proud of our Mighty Man. He is a fighter and won't let anything stop him!

A Step in the Right Direction

Our Neurologist called us on Thursday and told us that they had a cancellation for Friday morning for an EEG. I immediately took it since every day seems like an eternity to wait. So our EEG got moved up from March 12th to yesterday morning. Marek was a champ at getting all the leads on his head and during the EEG. During an EEG they want you to be able to put your child to sleep for 20-30 minutes. I was nervous for this part since our little guy really doesn't sleep when we want him to, but he fell asleep 10 minutes after we started the EEG. For the past few weeks when Marek is asleep he twitches a lot and his eyes will flutter open and shut. I have always wondered if this was seizure activity or just normal baby sleep. He had a few of these twitches and eye flutters while hooked up to the leads so I was sort of relieved that these were caught on the EEG. The whole test lasted about an hour and then we were on our way. The EEG tech told us we would have our results by the end of the day Monday.

Late afternoon yesterday I received a call from our Neurologist. He told me that he was able to watch part of the EEG and had some preliminary results for us. He told us that unfortunately Marek does not have a normal EEG (this I already knew since he is still having spasms), however he did tell us that the EEG showed improvement from Marek's previous EEG. We are taking steps in the right direction! He told us that Marek has moments of normal brain waves but his background is still chaotic. He told us that this shows Marek has had some response to the steroids. We still have a very long road ahead of us but I am staying hopeful that we are on the right path to get Marek better. We are holding at the same dose of ACTH until Monday. On Monday we will be talking with our neurologist more on what our plan looks like now that his EEG looks better. We are still taking things one day at a time because we know that the rug can be pulled out from under us at any moment. We know we have to come off the ACTH because the risk for Marek to have more serious side effects are growing higher each day he is on it. We also know that there is a high risk that the spasms can get worse as we wean him off the ACTH. Although we have lots to worry about in the next few weeks I am trying to "celebrate" our step in the right direction.

One thing I am celebrating is Marek has reached another inchstone. He has been able to hold himself up with straight arm with the assistance of his boppy pillow. This is amazing since Marek is very apprehensive of putting his hands down. He is still clenching his fists while pushing up but with a little help he will let me open his hands and keep them that way for a short time. Our occupational therapist is calling me Tuesday to set up his first therapy appointment. I am hoping we can work on keeping his hands open.


Speaking of celebrating,drum roll please......................Marek has finally hit a half MILESTONE (Yes in our home we have half milestones that we celebrate!) Marek is able to roll over from his tummy to his back. He did it for the first time yesterday and I was scared that maybe it was just on accident but he did it later last night and this morning. We caught his 4th roll on video!! I am so proud of my Mighty Man. His strength and determination amazes me. I love him so much!

Go Mighty Man Marek

Trip to Detroit

We left for Detroit on Monday around 10:30 AM. Marek was PERFECT the whole way up there. We were very scared to travel 8+ hours in the car with a baby who has been EXTREMELY irritable for the last four weeks, but he did great. He slept for the first four hours and then played with his toys for the next four. We got into the hotel around 7:00 pm. Marek took a bath in the very deep sunken tub. Actually we treated it more like a mini pool and filled it up high enough that he could just float in it. He was so relaxed to lay there and not feel the weight of his body. After baths we went to sleep so we would be rested for our appointments in the morning.

We woke up to a snow/ice storm happening in Detroit (this is just our luck). Our hotel was about a mile from the hospital but it took us 30 minutes to get there. When we got to the neurology office the receptionist had me spelling our last name three times and kept asking me if I was sure I had an appointment for that day. I started to get very nervous. I told her we drove all the way from St. Louis to see Dr. Chugani and that we were told by his personal nurse that the appointment was for March 3rd. The receptionist sent me to another office where I explained everything again and re-spelled our last name for the 10th time. The receptionist at this office said she couldn't find our appointment either so they were going to contact Dr. Chugani's nurse. I am not sure what happened but after a small panic attack that maybe we drove all the way to Detroit for nothing Dr. Chugani's nurse came out to tell us that everything is OK and that they will be setting up an exam room for us.

We met with a neuro fellow first who took down all of our current information and then Dr. Chugani came in. I immediately knew that I liked him. He has great bedside manner, he is very personable and he doesn't sugar coat anything. False hope is not something I am looking for. He has seen over 900 cases of IS and written many papers on treatment plans of the disease. We could tell right away that he is very knowledgeable about IS. He explained to us that he would have administered the ACTH with a different more aggressive schedule than our doctors in St. Louis. We asked him if we could start that aggressive schedule now before Marek is completely off ACTH but he told us it's too late in the wean to do that but doing a second round of ACTH is still an option but he suggested we try the Keto diet first. He also looked over a plan that we have with our doctors down south and made some other suggestions that we will consider.

The whole time that he was discussing things with my mom and I he kept an eye on Marek and would interact with him during the visit. He then told us that Marek seems to be very high cognitively. He said that clinically Marek doesn't present like a patient that has a gene mutation or multi focal seizure activity. He told us that he couldn't be 100% sure but he strongly feels that Marek might just have one single focal point that is causing his seizures since he is very interactive, he looks from person to person when they talk and he plays with his toys when they are given to him. Dr. Chugani explained to us that if Marek has a single focal point where the seizures are occurring and that focal point is on the left hemisphere of his brain he would make a great surgical candidate. Brain surgery?!?! On my baby!?!?! I am still wrapping my mind around it. Dr. Chugani ordered a 24 hour EEG, PET scan and a follow up appointment for us. We will not know anything more until those tests are ran. We walked out of the office feeling, dare I say, a little hopeful that maybe we have another option  for fighting IS.

We started the long drive home from the hospital around 11:30am. The roads were  horrible in Detroit still. Mom did a great job driving in the icy conditions. I was a nervous wreck in the back seat. Once we reached the Indiana border it turned to rain and the roads got better. The drive back was a lot harder than the one going there. Marek didn't sleep all that much at the hotel so we were extra tired coming home. But we switched drivers half way home and stopped for some caffeine. The good thing about traveling with my mom is that we always can find things to talk about! Marek was again PERFECT all the way home. He didn't nap for more than 20 minutes during the trip but he was content playing with his toys. I am sure  his arms had to be sore,  I know Mom's and mine were from holding his toys above his carseat so he could play with them. I wouldn't have had it any other way though. I love seeing him be so interactive.

We got home around 8:00 pm. We gave Marek his medicine and then everyone headed to bed.....for 30 minutes. Yes, Marek's new sleep schedule is to wake up every 30-45 minutes. Of course he doesn't wake up smiling, he wakes up screaming and expecting food. The last couple nights we have filled up bottles with 2oz of formula so that we aren't overfeeding him. I don't know if the wean is making things worse or the increase of his other medicine, Onfi?

Thank you to everyone who has continued to pray for our Mighty Man! We love you all!!!

Inchstones and Awareness

Marek had a a really good day on Wednesday. He only had 4 spasms. Of course we got our hopes up and were really excited that maybe, just maybe this might be the turning point. Unfortunately, Thursday night he had a big seizure of 73 spasms in 45 minutes. So as of now we are just focusing on getting his strength up and working on his development. Marek has had some rough days while weaning off the ACTH. A lot of parents that I have spoken to said that their children were more worked up and irritable while weaning because their little bodies are actually going through withdrawals of the drug. On top of that Marek has had quite an upset stomach and now has thrush in his mouth (one more drug to add to the mix). Wednesday night and Thursday night he hardly slept. We were back out taking middle of the night drives to calm him down. Last night though was a great night for him. Ryan and his mom watched him so I could catch up on sleep again and he slept for 2-3 hours at a time. Hopefully tonight will be another good night for him.

As far as therapy goes, our little man is working his butt off! We are excited that he can finally hold up his head while on his stomach. Granted he is only doing this on his "wedge" which keeps him at a 45 degree angle, but its a start! It's not a milestone but its an inchstone and we are happy to celebrate all the little moments. Marek has a new motto, which is "WILL WORK FOR FOOD!" He is doing so many little things that are making him stronger as long as we reward him with a bottle. He is able to get his shoulders off the ground when he on his back reaching side to side for his bottle. Our therapist showed me how to help him with rolling on an exercise ball. He is doing a great job understanding how to start to roll but he is just not strong enough to get there by himself. He has realized this past week that he has back muscles. All he wants to do is arch his back to see what is going on behind him. Our therapist was very proud of this but now we need to work those tummy muscles! He is doing a lot of sit ups this week so we can balance out those back muscles.

Thank you again for everyone who continues to support Marek on his journey towards a seizure free life. We also want to thank everyone who has come to the house to drop off food for us. We really appreciate it since we don't have much time or energy to cook since our focus is on Mighty Man.

I started this blog as a way to raise awareness of Infantile Spasms/West Syndrome and to help families that are going through difficult times with this disease. I have learned many valuable lessons in the short time going through this ordeal with Marek. One important thing I have realized is that support goes a long way. Support from family and friends, support from other families who are going through the same fight we are, and support from organizations. Today is National Rare Disease Day. There are over 6,000 rare diseases in the world and less than 500 treatments to help cure or control them. Many diseases don't have ANY treatments. Without awareness this will continue to be a problem. Not only is my son struggling with a rare disease but so is my dad (who has sarcoidosis). For both of them I will help to spread awareness with hope that one day drug companies start to take interest in the FEW but very SPECIAL people that struggle from these diseases. I ask that you take the time to watch the video and spread the word to just a few people and ask them to talk about it with a few more people. The ripple effect is a powerful tool. The video was put together by NORD (National Organization of Rare Diseases). They do so much to support families that struggle with rare diseases.

Ryan, Marek, and I have been blessed to receive the help of a student group at Collinsville High School called Kahokstrong. This student organization helps families of students and staff that are struggling. This year they are hosting their second annual Kahokstrong 5K. The theme this year is "Who is your hero?" Participant are asked to dress up as their favorite superhero. My family will be walking  to support our little but mighty superhero Marek as well as to support the club who has done so much for so many families. Please join us to help support a great and worthy student organization. We will be wearing our Team Marek shirts!

KAHOKstrong 5K

Monday Mom and I will be heading to Detroit to see Dr. Chugani. I will update everyone when we get back from our appointment.

A New Path

Sadly the doctors feel that the ACTH is not going to be Marek's miracle drug. We are weaning him off of the steroid and looking toward our next option. It is frustrating and very discouraging that the number one drug out there to fight IS didn't cure Marek of this disease. Marek will not be completely off of ACTH until March 17th. Since ACTH is so strong and Marek is still at risk for many of its side effects we don't have many options on what we can do next until he is 100% off the drug. Although we are off of the drug mid March he will not have a full immune system for many months. The doctors told us for the six weeks after ACTH he will still have a severely compromised immune system.

We met with our neurologist and epileptologist today at Children's to map out where we go next. It is scary because we are running out of options as far as drugs go and we have already tried the "top" treatments for IS. The plan our doctors propose is to continue Marek on the Onfi which he has been on for two weeks now. We will now be a little more aggressive with titrating up the dose as we slowly go down on ACTH. Before ACTH Marek was having over 200 spasms a day, ever since we have been on his high dose of ACTH he is having between 40-80 spasms a day. We are hoping that as we come off this drug his spasms don't become worse. If they do, then we will start another less potent steroid called prednisolone. If Marek's spasms stay in the 40-80 range we will start the drug Topamax (which Marek was on before and which caused his bicarbs to drop). If Topamax doesn't eliminate the spasms or if his bicarb levels drop again (we will have to go to Children's hospital every other day to get his levels checked) we will then be starting the Ketogenic diet towards the end of April. We will have to be admitted back into the hospital to start the diet. The doctors have scheduled Marek for an EEG on March 12th to evaluate if the ACTH has cleared up any of his hypsarrythmia. We are keeping our fingers crossed that it has.

Although we love the care we have received at Children's Hospital in St. Louis I have been trying to get a second opinion from a doctor in Detroit named Dr. Chugani. He has 35 years of experience with Infantile Spasms and is well known throughout the Epilepsy Community. We finally got a call today that they have an opening so we will be leaving for Detroit Children's Hospital Monday for our appointment on Tuesday. We feel that with any major medical concern a second opinion is necessary.

We have to thank everyone again for their donations and support. We are so thankful to have such amazing friends and family willing to help us. We will be using these donations to travel to Detroit and to be under the care of Dr. Chugani who is not in our insurance network. I have spoken to many parents that I have met through Infantile Spasms support groups who have gone to Dr. Chugani and have great things to say about him.

A new path to travel down brings new hope and new fear. We hope that this will be the KEY to stopping our son's IS and yet at the same time we are fearful that he will fail another treatment. In regards to treating IS there are four main treatments. #1 ACTH/Prednisolone #2 Vigabatrin #3 Topamax and #4 The Ketogenic diet. My family and myself are big believers in signs. I was so nervous with how today's appointment was going to go because of our timeline. Dr. Chugani's office told us that he would like to run a PET scan four weeks after Marek is off of ACTH which would be mid April. In order to have a PET scan done he cannot be on steroids or the Ketogenic diet. I was scared that our doctors in St. Louis would have a plan that would not allow us to have the PET scan ran in Detroit. So after hearing that Marek wouldn't be on steroid or the Ketogenic diet at that time (granted everything goes according to plan) I was so relieved and felt that we are finally heading down the right road. Our prayers these last four weeks have basically been on repeat asking God to let the ACTH work. Tonight I will have a new prayer asking him to allow our new path to grant Marek seizure freedom.

My aunt sent us a message today that said, "It is usually the last key on the keyring that opens the door." We are coming to our last key...fingers crossed the door opens.

Below is information about the Ketogenic Diet from the Epilepsy Foundation

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• What is the ketogenic diet?

The ketogenic diet is a special high-fat, low-carbohydrate diet that helps to control seizures in some people with epilepsy. It is prescribed by a physician and carefully monitored by a dietitian. It is stricter than the modified Atkins diet, requiring careful measurements of calories, fluids, and proteins.

• The name ketogenic means that it produces ketones in the body (keto = ketone, genic = producing). Ketones are formed when the body uses fat for its source of energy.
• Usually the body uses carbohydrates (such as sugar, bread, pasta) for its fuel, but because the ketogenic diet is very low in carbohydrates, fats become the primary fuel instead.
• Ketones are not dangerous. They can be detected in the urine, blood, and breath. Ketones are one of the more likely mechanisms of action of the diet; with higher ketone levels often leading to improved seizure control. However, there are many other theories for why the diet will work.

Who will it help?

• Doctors usually recommend the ketogenic diet for children whose seizures have not responded to several different seizure medicines. It is particularly recommended for children with the Lennox-Gastaut syndrome.
• The diet is usually not recommended for adults, mostly because the restricted food choices make it hard to follow. Yet, studies done on the use of the diet in adults show that it seems to work just as well.
• The ketogenic diet has been shown in small studies (case reports and case series) to be particularly helpful for some epilepsy conditions. These include infantile spasms, Rett syndrome, tuberous sclerosis complex, Dravet syndrome, Doose syndrome, and GLUT-1 deficiency. Using a formula-only ketogenic diet for infants and gastrostomy-tube fed children may lead to better compliance and possibly even improved efficacy.
• The diet works well for children with focal seizures, but may be less likely to lead to an immediate seizure-free result.
• In general, the diet can always be considered as long as there are no clear metabolic or mitochondrial reasons not to use it.

What is it like?

• The typical ketogenic diet, called the "long-chain triglyceride diet," provides 3 to 4 grams of fat for every 1 gram of carbohydrate and protein.
• The dietician recommends a daily diet that contains 75 to 100 calories for every kilogram (2.2 pounds) of body weight and 1-2 grams of protein for every kilogram of body weight. If this sounds complicated, it is! That's why parents need a dietician's help.
• A ketogenic diet “ratio” is the ratio of fat to carbohydrate and protein grams combined. A 4:1 ratio is more strict than a 3:1 ratio, and is typically used for most children. A 3:1 ratio is typically used for infants, adolescents, and children who require higher amounts of protein or carbohydrate for some other reason.
• The kinds of foods that provide fat for the ketogenic diet are butter, heavy whipping cream, mayonnaise, and oils (e.g. canola or olive).
• Because the amount of carbohydrate and protein in the diet have to be restricted, it is very important to prepare meals carefully.
• No other sources of carbohydrates can be eaten. (Even toothpaste might have some sugar in it!).
• The ketogenic diet is supervised by a dietician who monitors the child's nutrition and can teach parents and the child what can and cannot be eaten.

What happens first?

• Typically the diet is started in the hospital. The child usually begins by fasting (except for water) under close medical supervision for 24 hours. For instance, the child might go into the hospital on Monday, start fasting at 6 p.m. and continue to have only water until 6 a.m. on Tuesday. The diet is then started, either by slowly increasing the calories or the ratio. This is the typical Hopkins protocol.