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
- 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.
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