Ketogenic Diet - Night 2

Not much to report - we finally got some sleep last night! Jenelle woke once around 4:00 a.m. acting hungry and frustrated, but went back to sleep after some cuddling with Mom. I can't imagine what she must be thinking - she was healthy coming in here, why are they treating her like this? She had blood drawn at 6:00 a.m. and slept right through it! The last seizure I saw was around 6:30 last night - that is hopeful! (and now I've probably jinxed it!)

I was planning to feed her at 7:30, but decided to let the Princess get some more sleep. With the nurse "Shift Change", she'll be waking up soon. Have a great day and I'll update when I can!

Ketogenic Diet - Day 2

This afternoon we had some visitors and I got to make Jenelle's next 4 meals all on my own! Father Reese, the priest who married us at St. Paul's in Tustin stopped by for a visit and to pray for Jenelle. He had a meeting in West LA and was in the area. We didn't get the chance to go to church on Sunday, so it made me feel better that he could pray for her. Jenelle is a regular during the "healing" part of the prayer in our Sunday services, when we do make it to church.

Later in the afternoon, a cute little dog named "Zeesee" came to visit Jenelle and sit with her in her crib - courtesy of patient services. Don't they look alike?


Jenelle & therapy dog "Zeesee" 

To give you an idea, Jenelle's meal this evening for dinner was 39 grams of Stage 1 beef (jarred baby food), 35 grams of carrots (again, Stage 2 baby food), 10 grams of "fat" (mayo) and 5 grams of olive oil, all mixed together. With that, she got to have 35 grams of "heavy" whipping cream mixed in with her 3.5 ounces of Ketocal formula, for 4 ounces total. Small portions to say the least! The nurse explained that it may take Jenelle a good 2 weeks to adjust to the smaller portions, but calorie and fat content wise, she is getting enough. She can have an unlimited amount of water (with zero calorie flavoring added) and must get at least 32 ounces of fluids a day. And of course, she can have as much of her "thumb" as she wants since it has no calories! ;)

Jenelle is tolerating the food well, but is still acting hungry, and gets quite upset at times when Mommy tries to give her water instead of her usual milk. If her blood sugar levels remain stable and her ketone level stays up, we may get to leave late tomorrow after more instruction during the day. She is still having seizures, however they are no more than usual, and no less. That too could take a few weeks to show a difference. By the way - they found me a "pull out" bed for this evening! YAY! Brett has been driving back and forth and is staying with Jack this evening. Jack spent the previous night with Grandma, who also took him to Disneyland today! He is having more fun than any of us!

Thank you for the continued prayers - we truly appreciate it!

Ketogenic Diet - Night 1 - Rough Start!

Well, we had a rough first night and literally woke every hour on the hour. Part of it is the new surroundings (hospital noises and our neighbor's monitor going off ever hour!) the other part is that Jenelle was very hungry. I am sleeping in a chair with another chair to prop my feet, so that didn't help things. I didn't explain the egg nog well - Jenelle was only allowed 2 ounces at a time, so in total, she got 6 ounces over a 6 hour period, and nothing for the rest of the night. Like I said, she did not sleep well and was so frustrated she wouldn't suck her thumb. All that helped was lying on Mommy's chest. We got through it though!

This morning we took our first ketone level and found that Jenelle actually had too many ketones. She had a little vomiting last night as well, and they believe that is from the increase of ketones. Because of this, she got one ounce of cranberry juice, and that did the trick on lowering the ketones. This is surely an exact science. So far, no other problems like low blood sugar, etc.

Brett and I had our first lesson this morning and we're choosing the "easy" course in that part of Jenelle's meals will be supplemented with a formula called "Ketocal", and the rest of her meals will be prepared with jarred baby food. Jenelle is pretty alert right now and enjoying the special time with Mom and Dad. Our next "lesson" is at 1:00, so I have to run. Will try to update when I can this evening. Thanks for the continued prayers!

Ketogenic Diet - Day 1

Thank you all for the prayers and positive thoughts this weekend, after waiting 3 1/2 hours, we got a bed at UCLA today. I may have mentioned this, but because of Jenelle's new diagnosis of Metabolic Acidosis, the team here at UCLA is approaching the Diet differently, and Jenelle did not need to "fast". Which was a good thing because Jenelle has been getting pretty hysterical these days if we are too late with her meals! She has already had her first "keto" meal of eggnog and flavored water. She'll get another serving around 8:00 p.m., and again at 10:00 p.m. if she is still awake. We're going to keep this up and will hopefully start real "keto" food later tomorrow.

Thanks for keeping us in your thoughts and prayers. I'll update again if I can tomorrow! We should be here until at least Thursday.

UCLA here we come... Again...

As you know, we met with Dr. John Menkes on Thursday afternoon. Wednesday evening, I decided to “Google” the good doctor to learn more about him. We had heard many things, especially that “he wrote the book on metabolic disease”- come to find out, he really did write the textbook "Child Neurology" (now in its 6th edition) as well as a recent novel called “The Angry Puppet Syndrome” I was particularly fond of a review I found “blogcritics.com” of his new novel by someone with the handle “bookofjoe” who wrote the following review:

“Menkes is a pediatric neurologist at UCLA, famous for his discovery of Maple Syrup Urine Disease (you can guess the main sign), also known as Menkes' Disease or Menkes' Syndrome.

I was in his clinic one afternoon during my peds rotation at UCLA Med School, where some kid whose disease was undiagnosable was brought in. Menkes walked into the room with all of us med students, interns, and residents behind him, looked at the kid, asked the mother a few questions, wiggled the kid's finger and wrists, said, "this is a case of so-and-so," and walked out of the room.

We all went and looked up the disease, and damned if the kid didn't fit the picture perfectly. Something like 17 cases reported in the world literature up to that time. The classic absent minded-professor, Menkes seemed a little baffled and bewildered, with these thick glasses and a scraggly beard, but that's a big-time brain in there.”

As of Wednesday evening, Brett and I were convinced we’d found our “Dr. House!” I wish it ended that way, but unfortunately, we left Dr. Menkes office on Thursday with Jenelle having stumped yet another one of the world’s top Neurologists. Our visit was invaluable as he really took his time to review her medical records and MRI, however he said there was really no diagnosis he felt she had. He did suggest some new genetic tests we could run, and pretty much agreed with everything Dr. Shields has been saying about Jenelle. He believes that Jenelle’s brain has “developed” abnormally, which of course is causing her problems, but why we may never know. Dr. Menkes felt the Ketogenic Diet was our best next step.

Perhaps it’s my optimistic attitude and positive outlook on life, but I truly feel that this may be for the best. As long as there is no diagnosis that limits her life or limits her development, then really there should be nothing holding her back once we control her seizures! Without a definite diagnosis, her potential remains unlimited!

Today I took Jenelle to CHOC for her upper GI and Swallow Study. The Swallow Study results were good (we will have to wait for the GI results) – Jenelle is not aspirating when she drinks, however, as the test went forward and they checked how she swallows food, I heard the Occupational Therapist say, “Wow, that is really strange!” Apparently Jenelle does not swallow her food unless it is followed by a bottle, and/or there is too much food in her mouth to force it down! This most likely is a sensory issue that can be improved with more therapy. But, it is dangerous in that she could aspirate on her food. The OT was surprised because she said that usually a child with a good “suck and swallow” like Jenelle’s with the bottle of milk, should also know how to swallow food – even pureed food! OK, so should it really surprise me anymore when medical people tell me Jenelle is strange?

I spoke with UCLA this afternoon and we are on for Monday – however we still won’t know if a bed is available until we get there. Please keep us in your thoughts and prayers next week! Thanks so much – I’ll keep you posted!

Jenelle's Insurance Case Manager Earns Her Wings!

Our insurance case manager really earned her wings today… as in she is an "Angel with wings" and a true miracle worker! OK, maybe that is a big over the top, but I was amazed today by her quick response… I'll get to the full story in a minute, but first, the latest on Jenelle…

Last Friday, I got home from work and had a message from Dr. Ben-Ezer on our answering machine (grrr - don't you hate messages on Fridays?) Dr. Ben-Ezer consulted with the Rheumatologist at CHOC about Jenelle and decided to run two more blood tests to check for certain metabolic issues. One of which is a test for Lupus. Why we are checking for Lupus, I have no idea - Jenelle certainly does not fit the symptoms I've found on the internet, but hey, why not? I called on Monday to get the details, but Dr. Ben-Ezer is out until Wednesday. I will be taking Jenelle in for this blood work tomorrow (we had to wait a week after starting her bi-carb supplement) and will try to get a hold of Dr. Ben-Ezer to get her thoughts.

Also, I contacted Dr. Menkes today, who is a well known metabolic specialist at Cedars Sinai. He is going to see Jenelle this Thursday at 2:00 p.m. He first suggested an appointment for next week, but upon hearing that Jenelle was to be admitted next week for the Ketogenic Diet at UCLA, he said he could see her this Thursday in case he saw anything that might compromise the diet. Wow - an appointment with 2 days notice? How lucky can you get! Just after making the appointment, I got a call from UCLA's financial person and she was having problems again getting our admission approved for Monday. I told her we needed to give our new "case manager" a try, and gave her the number to Kathy at Health Net, Jenelle's new case manager. In addition, I also decided to contact Kathy to see if we could get approval to see Dr. Menkes. After explaining our reasons for wanting to see Dr, Menkes, Kathy said she would pull Jenelle's file and call me back. Five minutes later, she called and "everything" was approved, including our admission to UCLA for Monday! Like I said, she certainly impressed me and earned her wings - I've now dubbed her our new "Angel"!

I spoke to UCLA this evening to let them know about our appointment with Dr. Menkes on Thursday. They are glad we are going, and want a full report on his opinion! Unfortunately, I was also informed today that we may be in for a problem again with bed availability on Monday. Please start thinking positive thoughts that we'll have a bed available at UCLA next week! I'll keep you posted after our appointment on Thursday. Thanks for the continued prayers and positive thoughts - please keep them coming! I can't help but feel we are finally getting closer to an answer for our Jenelle!

A new date for the diet!

Well, it looks like Dr. Ben-Ezer was true to her word and spoke with Dr. Shields yesterday afternoon. The nurse at UCLA called last night to inform me that Dr. Shields would like us up there on April 18 to admit Jenelle for the Ketogenic Diet! Even though Dr. Shields told me at the conference we needed to "talk", I think he would prefer to have Jenelle admitted so we can start everything right away, including any other tests he may want in light of her new metabolic issues.

UCLA warned me that they expect Jenelle to have a difficult time at first with the diet, but that it was not impossible to get her on it. Jenelle will start the diet a little differently than most patients in that they don't think they can "fast" her to get her into ketosis. What this means is that we may be there longer than usual. UCLA also warned me that she could get "very sick, very fast" and that they wanted to take some extra precautions while she is admitted like daily blood work and keeping an IV open. Remember, Jenelle already has low bi-carbs, and usually when a patient goes on the diet, their bi-carbs get even lower, so we could see some problems. Although I know of kids who had difficulties with the diet and metabolic acidosis, I think it is worth a try, especially since it may bring to light some answers to other questions about Jenelle. Also, it will be easier with insurance to just admit her so we can work with Dr. Shields, rather than wait for an authorization for a consultation, and then admit her! One funny thing - Dr. Shields said, "knowing Jenelle and her usual "opposite" reaction to things, we'll probably have an easier time with this than we think!" - even he knows she is unusual!

Please keep us in your thoughts and prayers next week as we get ready - especially that Jenelle can remain healthy so we are not delayed again! Fortunately, Jenelle has her GI tests scheduled for next Friday, so we won't have to worry about using Keto food instead! As always, I'll keep you posted!