Last week was so crazy, I'm not exactly sure where to begin...
Tuesday, the day after Valentines Day, Jenelle's teacher at Blind Children's' told me she was concerned about Jenelle because all of a sudden, it was as if she has forgotten how to suck a bottle. It took Jenelle an hour to finish a bottle, and she was practically choking herself while taking it. Grandma noticed the same thing last weekend, as have I. Of course, the biggest scare in all this is that she is regressing and losing skills - always a scare when there is no diagnosis. Her teacher thought it might be a side effect from our recent increase in Felbatol, so I told her I'd call her Neuro at UCLA.
The next day, UCLA called back to inform me Dr. Shields was out of town. The nurse who called is also in charge of the Ketogenic Diet Program, and told me they had most of Jenelle's lab work back! They had a concern in that Jenelle's "bi-carb" levels were too low indicating that she is acidotic. This can happen when a child is on the Ketogenic Diet and it is something they watch carefully. Apparently the bi-carb level (which is the level of CO2 in the blood) should be between 21 - 34, and Jenelle's was 15 - much too low for a child not on Keto already. There are two other little girls that are similar to Jenelle (Lily in Arizona being one) that were recently diagnosed with metabolic acidosis. Apparently, acidosis can causes "loss of appetite", and "loss of gross motor skills" (so this may explain the recent problem with the bottle!) When these two other girls started taking bi-carb supplements, their development really began to improve. UCLA does not want to start Jenelle on a supplement until she starts the diet, but does want her bi-carb level to increase before they will let her start. UCLA is convinced this issue is due to Jenelle taking Topamax and not the Felbatol. Ironically, we recently increased Topamax last month (remember our ER fiasco?) so now, we have been instructed to wean her back down to the previous level to see if it helps. We are still on track to start the diet March 14, assuming we don't get bumped by someone needing the bed.
As I was on the phone with UCLA, our daycare provider was trying to reach me because Jenelle was having too many long seizures again. She eventually got a hold of Brett and asked if she should use Diastat (our emergency medication to stop her seizures) - at that point, Jenelle had been seizing for 6 minutes. He told her yes, and that he was on the way. Once I was off the phone, I got Brett's voicemail about Jenelle and called him to get more details. After talking to him, I called our daycare to find out what type of seizure Jenelle was having and was told it was her usual atonic seizure type. As I was on the phone with daycare, Jenelle went into a grand mal. Our daycare provider hadn't given Jenelle her Diastat yet as she had stopped seizing after her previous 6 minute seizure. The daycare provider told me that Jenelle was seizing like she had never seen before (literally convulsing) so I told her it was a grand mal, and to give her the Diastat immediately without waiting for the seizure to last over 5 minutes. At this point, Jenelle seizures added up to almost 30 minutes of the last hour. The Diastat did its job, and Jenelle was OK the rest of the evening.
After getting off the phone with daycare, I called UCLA again to let the nurse know about the increase in seizures - she was concerned and told me to hold off on lowering the dose of Topamax until we figured out why she was so unstable. So... once I finally got home to Jenelle, I decided to take her temperature - Jenelle was running a low grade fever of 100.8, which ironically is the same it was with her last grand mal in January! Anyway - we decided she was most likely fighting off some kind of bug and decided to wait a couple of days to see if she was going to get sicker. She was fine all weekend, so I decided to lower her Topamax on Sunday- whew, back on the road to the Diet!
Oh yes, it doesn't end there. On Thursday, UCLA called to inform me that the lab neglected to run a test Dr. Shields had ordered, and they needed me to take Jenelle to draw more blood. I took her in on Friday afternoon, and she a great job, even with out our favorite Phlebotomist as she was gone on vacation (lucky her!) What an oxymoron - the use of "favorite" and "phlebotomist" in the same sentence!
Also last week, Jenelle's Pediatrician called to inform me that our referral to a GI Doctor had been approved. Poor Jenelle is very constipated from her meds, and even with daily fiber, increased fluids and the like, she will only have a BM every 3 to 5 days. Dr. Shields said this is common in children like Jenelle who have little mobility. During a conversation with our Pediatrician about the bi-carb levels, I mentioned that I had noticed that Jenelle was not urinating frequently. Remember it took 3 days just to get a sample two weeks ago? They are concerned and think we may also need a referral to a Kidney Doctor as well. This weekend we increased Jenelle's fluids, and did see a difference, so hopefully we can avoid that route. Dr. Patel mentioned that we may want the GI Doctor to run a "swallow study" to make sure Jenelle is not aspirating while taking her bottle (given her recent problems.) If she is aspirating, we may be headed towards her getting a G-Tube for feeding. All of this is just looking ahead, and hopefully we can get an appointment soon to the GI Doctor - I'm still waiting for a call back with our appointment, and would like to get this resolved before we start the diet since the diet also increases constipation - OH JOY!
And finally, this is now Tuesday. I've been drafting this update over the long weekend and spoke to UCLA again today. We could use some quick prayers as I learned today that the child who was supposed to start the Ketogenic Diet today was bumped because they did not have a hospital bed. This of course means that if they cannot get the child in tomorrow, our start date for the diet may be bumped as well. Everything for us works so well for the week of March 14 and we'd really like to start that week. Please say some prayers and send some positive thoughts that this child can start tomorrow so we will not be delayed. Jenelle is doing better this week, but we realize from our experience last week - things can change daily!
Sorry this was so long - Thanks again for your thoughts and prayers - I'll keep you posted!