Friday, March 25, 2005

Good Friday update

I'm not sure if it is a jinx, or just Murphy's Law, but usually after sending a positive update about Jenelle things change. I guess that is the way this roller coaster goes - ups and downs.

Jenelle had a seizure lasting 10 minutes yesterday morning, was given Diastat (her emergency medicine) and was fine for the rest of the day. Prior to the 10 minute seizure and within that same hour, Jenelle had many other lengthy seizures all around 4 minutes in length. I called UCLA and left a message telling the nurse that I didn’t think we could take this for another three weeks or more (assuming it takes that long to get onto the Diet.) It seems her seizures are getting longer, and I was afraid for the days ahead. I also called the Kidney Doctor from CHOC and asked to be placed on their cancellation list, explaining that Jenelle's seizures were out of control and I wasn't sure that things wouldn't get worse before our April 5 appointment.

The nurse from UCLA returned my call, and said Dr. Shields was concerned with the new seizures and wanted to increase her Topamax again to the last dose we were at to see if that stabilizes her. We both agree this sudden onset of seizures is most likely from her reduced dose of Topamax (she was literally on half the dose she was previously taking.) It is tricky because we do not want to lower her bi-carbs again, but we also do not want her going into status because her seizures are uncontrolled. After 3 days of this increase, I am to update Dr. Shields on Monday, and we'll go from there. The nurse said that there was a small possibility that if Jenelle's seizures do not improve over the weekend and continue to be like they have been this past week, Dr. Shields may want to admit her to the hospital at UCLA for some tests and observation. We are pretty sure all of this is being caused by the medication changes, but there is a small chance that "something new" could be happening. Fortunately, Dr. Shields will be around through the weekend and all next week. Usually after Jenelle is given Diastat the frequency of her seizures decrease… hopefully this last dose will work throughout the weekend while we increase her Topamax.

On a side note, we are scheduled to meet with Dr. Charlotte Dravet from France in the afternoon on Friday, April 1st (no fooling!) I have also been working with the Epilepsy Foundation to help them prepare Jenelle's "PowerPoint" presentation for the Pediatric Epilepsy Conference next weekend. Would you believe that Jenelle's medical records take up almost 3 full binders already?

And some good news. I finally got our HMO to assign a "case manager" to Jenelle. You would not believe all it took to get that. I had one customer service person take down my request for a case manager (including details on Jenelle's history), and then another rep told me the company did not have any positions like that, nor could he find my previous request! The day after I was told they didn't have "case managers", I got a call from Jenelle's "new" case manager who works for our HMO. She took down her history, and told me she would now be handling all of Jenelle's authorization requests, as well as communications between her medical group and the insurance. She explained that the purpose for having a "case manager" is so that one person is assigned to Jenelle, and that one person will know her history and thus we won't have to explain things like why we go to UCLA instead of CHOC. In theory, it sounds perfect - haven't used her yet, so the jury is still out on that one! ;)

It goes without saying - thank you for the continued prayers and positive thoughts for Jenelle. To those of you who celebrate, Happy Easter! I'll keep you posted!

Tuesday, March 22, 2005

More doctor's appointments...

We finally have some appointments lined up for Miss Jenelle with the CHOC Kidney doctor being the first set for April 5. After reviewing the documents from UCLA, the CHOC Kidney doctor felt it was OK to see Jenelle at the first available appointment rather than on an urgent basis. Luckily, we are only waiting a couple of weeks. Jenelle's GI tests are scheduled for April 15, so we had to postpone her follow up with the GI doctor to April 21. The month is already looking busy - and we haven't even factored in a date for the diet at UCLA (hopefully!) I spoke to Dr. Shields' nurse and he is pleased that Jenelle's bi-carb levels have increased, but really would feel better waiting for the approval from the Kidney doctor before we start the diet. Let's hope if the Kidney doctor orders tests, that they can be done quickly.

Since lowering her Topamax, Jenelle has started to bring back some of her old seizures we haven't seen for a while, specifically her myoclonic jerks. Unfortunately, our girl has decided to make some changes to these "old" seizure types and will now cry after a myoclonic jerk - something she has never done before. I think it is just that she is more aware of her seizures now, and how they interrupt her day, and not because of pain. Seizures may wear out a patient but are usually never painful - so we are told. Also this morning, Jenelle had a 3 minute seizure that I had never seen before and didn't quite know how to describe. It started as if it were going to be a grand mal, but then she started doing things she has never done before like lip smacking and grinding her teeth. I called UCLA to report this new stuff, and told the nurse I didn't know how to describe it - but she told me to just tell her what I saw, which is their preferred way of describing a seizure rather than me trying to naming it (grand mal, petite mal, etc.) Dr. Shields feels these new seizures are from the lower dose of Topamax, but does not want to increase it and would prefer we get her onto the diet as soon as possible! Sounds good to me!

And now for some good news. Jenelle is really recognizing people lately. I've been playing a game with her where I sit her up in my lap, and ask her to look for someone in the room (i.e.: Where is Daddy?, Where is Jack?) Would you believe that Jenelle will actually move her head and/or her eyes in the direction of each different person? What a wonderful improvement! Sometimes when I'm holding her, I ask "where is Mommy?" and she bends her head back to look up at me! We were showing this off today for her OT this morning, and Jack was on the other side of the room. When I asked Jenelle to look for Jack, she turned her head in his direction and looked directly at him. She may not be able to say our names, but she definitely knows who we are!

That is all for now. We are definitely on hold for the diet until after we see the Kidney doctor. Please say some prayers that we can get that done quickly, especially if the Kidney doctor wants to run special tests (that may require additional authorization - ugh!)

I'll keep you posted!

Friday, March 18, 2005

Our "Typical" 2 Year Old!

Well, we went to the ER last night even though I really did not want to spend St. Patrick's Day in the ER - it was packed! When Jenelle woke from her afternoon nap, she again refused her bottle only taking an ounce of fluid, and did not have a wet diaper. We arrived in the ER at 4pm, and got to leave around 9:30 - not too bad. Believe it or not... she is fine. She is NOT dehydrated, her liver function was normal and her bi-carb level is 20- almost normal and definitely within UCLA's limits for the diet! Basically, the "worst case scenario" is that we have a typical 2 year old who is being picky about wanting to drink her bottle! WOW - who would have ever thought Jenelle was just being normal!

I was in touch with UCLA while we were at the ER, and they faxed a letter and the labs to the ER to help us out. When I spoke to the nurse at UCLA just after we were discharged, she was just as shocked as we were! While at the ER, I was convinced she was dehydrated because they were unable to get a vein for an IV and she did not have tears when she cried. Apparently her labs said otherwise!

UCLA still wants her to see a kidney doctor before rescheduling the diet. We are now waiting to get an appointment with the doctor we were referred to at CHOC. Dr. Shields drafted a letter to this doctor explaining his concerns and why it was an urgent need to get her in. We should hear something soon - please pray that she gets in quickly!

We appreciate the support - I'll keep you updated!

Thursday, March 17, 2005

St. Patrick's Day Update

It has been an emotionally draining and challenging week in regards to Jenelle. It has also been a week where so much has happened, its difficult to know where to begin to update. However, if I postpone this update any longer - who knows what I will miss!

First of all and most importantly - Jenelle is fine. In the past two weeks we've seen fewer seizures than ever. She seems to be in less pain from her UTI, and we are half-way through the course of medication. Yesterday was a long, difficult day in dealing with our insurance. As I was putting her to bed last night, I leaned over the crib and put my hand to her cheek and got the biggest smile in return (smiles are as rare as laughs these days) Then after she smiled, she gave me some giggles. It was as if she knew her poor old Mom was emotionally spent, and she was trying to give me a little ray of hope to help me fight on. She is amazing.

On Monday, Dr. Shields presented Jenelle's case (including the new issue with her UTI) to the team at UCLA. It appears that the blood work Jenelle had done on March 7 indicated that her bi-carb level was still too low, even though we have reduced the drug (Topamax) that UCLA believes is causing this problem. I found out the next day that her bi-carb level was a 14, even lower than the previous level. Before re-scheduling our date to start the diet, UCLA wants Jenelle seen by a Nepherologist (Kidney Doctor). They are very concerned that Jenelle has something called Renal Tubular Acidosis which could eventually lead to kidney stones and/or kidney failure. If Jenelle's acidosis is caused by her drugs, then she will be OK to do the diet. If it is "metabolic" in nature (and thus pre-existing) she will not be able to go onto the diet.

Part of the agony of this week has been dealing with insurance and Jenelle's medical group. UCLA wants Jenelle to urgently see a kidney doctor at UCLA (they are holding an appointment for next Thursday if we can get authorization - maybe sooner.) The problem is that HMOs do not want to authorize an urgent request for a doctor outside their contracting medical group. After a couple of days figuring out who and how to request the authorization (apparently Jenelle's medical group only considers phoned in requests as urgent, not faxed ones - who knew!) we got word last night that we she is approved to see a kidney doctor at CHOC. I called last night for an urgent appointment, but apparently "the doctor" wants to review her medical records first so he can determine for himself whether or not her situation is urgent. UCLA is upset because they know their kidney doctor has experience working with kids that are on the Ketogenic Diet, and feel they may not be able to communicate easily with a doctor at CHOC than they have with their own kidney doctor. That is understandable, but unfortunately with our HMO I don't think it can be done. I'd pay out of pocket, but they are talking ultra sounds and other tests - we just can't afford it. All that said, I don't believe we'll be getting a new date for the diet anytime soon. UCLA would like us to start as soon as possible after we get these kidney questions answered. I appreciate them being so through - I feel we are in the right place.

Brett's favorite saying is "surrounded by morons, we press onward!" This couldn't be a more accurate desription about dealing with our insurance. Jenelle's doctors have been wonderful in doing all that they can to help the process. I wish the insurance process could be so much easier.

During the time it took to type this, another issue has come up. Jenelle is refusing her bottles and has not had a "wet" diaper since yesterday morning. Refusing her bottle is most likely the acidosis as it will cause you to lose your appetite. The lack of "wet" diapers could indicate dehydration. If after her afternoon nap she is still dry, our Peditrician's office has instructed us to take her to emergency (they are closed on Thursday afternoons.)

I will keep you all posted. Thank you for your continued prayers.
Oh yes, and Happy St. Patrick's Day from the Curran Family. Lets hope the luck of the Irish can help us pull through some miracles!

Monday, March 14, 2005

Change in Plans!

Yesterday morning, Jenelle started screaming and crying unconsolably. Knowing she wasn't constipated, we had no idea what was wrong. She screamed in pain when I felt around her tummy, so I took her to the ER thinking it may have been a kidney stone or worse. Turns out, Jenelle has a pretty bad urinary tract infection (UTI), most likely caused by her "clean out!" (oh joy!) They placed her on a 10 day course anti-biotic. Once we got home I called UCLA to see if this would hold up our admission.

Because of the infection, UCLA told us not to come. It is their experience that it is difficult if not impossible to get a child into Ketosis while the child is fighting off infection. We are disappointed, but a little relieved not to be wasting time driving to LA or in the hospital (trying to look at the "bright" side!) Had we not diagnosed the UTI last night, we'd have probably found it today or tomorrow anyway

The team at UCLA will discuss Jenelle today and make a decision as to when we should plan to come back. The nurse who runs the diet is hoping for this coming Monday, but is not sure what the doctors will want. Worst case scenario is we'll have to wait until she finishes the course of antibiotics (so another 9 days!)

Additionally, the nurse informed me last night that Jenelle's bloodwork from last Monday indicated that her bi-carb level is still too low. This may give us time to lower her Topamax even more, and maybe start a bi-carb supplement (which may also help the infection!) I am at home today with Jenelle. At times she seems uncomfortable, but otherwise is doing great!

I'll keep you all posted as to our new start date! Thank you for the continued thoughts and prayers!

Friday, March 11, 2005

Go Bruins! UCLA, here we come!

Just a quick update to let everyone know that everything is in place for Jenelle's admission to start the Ketogenic Diet at UCLA this Monday, March 14! This week has been crazy - I'll spare you from the details of Jenelle's "clean out" and simply say, Jenelle and Mount St. Helens have a lot in common! ;) The good news is that since her "clean out" last weekend, we've seen a completely different child in Jenelle. On Tuesday she laughed in response to Brett laughing at the TV - a laugh we haven't heard in many, many months (I can't even remember the last time I heard her laugh!) She's had fewer seizures, and is just so different! A very good way to start next week!

Both kids had a bout with Pink Eye this week …enough said about that! In addition to dealing with pink eye, my sore shoulder, work (for both of us) and shopping for Ketogenic diet supplies, the family has been busy and excited for Jenelle's Godmother's wedding this weekend in Dana Point! Jenelle's Godmother is Brett's Cousin Shelly (Brett's cousin) and we are very happy for both her and soon to be husband Alain. My Mother is in route as I type (hopefully) to watch the kids this weekend while the rest of the Brett's side of the family is celebrating the wedding. Mom will stay on through next week to be with Jack while we are at UCLA. Jack is thrilled to see his Nana again and Nana is pretty excited about it too! Nana is even thinking of taking Jack to visit my Aunt Onie sometime next week - so I'm sure he'll be spoiled rotten, and will barely notice we're gone!

So we will be out of touch for most of next week - I'll try to update if I can. Please keep Jenelle in your thoughts and prayers next week as we start the diet. After seeing her response to the "clean out", I am for once very optimistic and hope we are finally going in the right direction towards total seizure control for our girl!

Thanks again! Kelly

P.S. Jenelle will be staying at Mattel Children's Hospital on the UCLA Campus. Admission is after 12pm on Monday (assuming they have a bed!) At lot of her stay will be "education" for us on how to feed her and calculate her meals. Visiting hours are 10am to 9pm - just in case you are in the area! ;) GO BRUINS!

Thursday, March 03, 2005

Appointment with the GI DOctor

Well, as if seizures weren't enough trouble, it seems Jenelle has some other issues to address concerning her GI Track. Neurologists are only concerned with problems involving the "head up", and sometimes we are so intent on stopping her seizures, we never really address other issues for Jenelle. I'm glad we had our GI Referral today - a lot of issues that are long overdue for our girl were addressed!

As if I don't give out too much information already, to my knowledge this morning, Jenelle hadn't "pooped" since last Saturday, after a fleet enema of course! The doctor felt she was "backed up", so there was nothing to prove there. I first met with a nurse practioner who spent an hour getting Jenelle's extensive history, as well as family history. Because my shoulder is still hurting - I'll cut to the plan of action! We are going to spend the next two days "cleaning out" Jenelle - she will be taking LOTS of Milk of Magnesia, and after that cleans everything out, we'll start a daily prescriptive drug called "Miralax" (which is the one UCLA recommended as being good for the Keto Diet.) So Jenelle needs a new drug to help keep her from being constipated from the drugs she is already taking!

In addition, they want to order an Upper GI Swallow Study to see if Jenelle is aspirating while eating. Jenelle sometimes coughs when taking her bottle, spits up after a meal, and/or appears to choke once in a while, so we think with her low muscle tone, there is a danger of choking and/or pneumonia from aspirating while eating. This of course may mean we're headed for a G-Tube. They also want a study done by an OT to see if she is swallowing properly, and chewing properly. Our own OT has said Jenelle is doing this fine, but it will be good to have a second, more thorough opinion on the matter (especially as it relates to GI issues.)

And finally, they'd like us to take Jenelle off milk completely and put her on Pediasure as they are concerned that she is "failing to thrive." Jenelle lands on the bottom 20th percentile of weight for her age and we've never been concerned before because of her weak muscle tone. I just spoke to UCLA and the dietician was not concerned with Jenelle's weight, and Pediasure is not something Jenelle can take on the Ketogenic Diet. UCLA did say it was OK to give her the Pediasure until we are admitted so as to get Jenelle used to not drinking milk. The good news is that the GI Doctor felt there was nothing in his opinion as urgent that would prevent us from starting the diet. Jenelle on average "poops" every 5 days (imagine the constipation?) and they explained that once we get her pooping regularly, we may actually see a "decrease" in seizures as well, as the constipation can add stress that can lower the seizure threshold - makes sense to me!

By popular request (or rather, by popular demand) I'm attaching a couple of photos of Jenelle's new "pink" AFOs! Yes, Jenelle is really that limber! Thanks for all the prayers and support - we'll keep you posted!

Pink AFOs! Posted by Hello

Wednesday, March 02, 2005

Another trip to UCLA

Well this update will hopefully be brief as I am nursing a sore shoulder (which makes it difficult to type) which I will detail later in this update. Last Thursday, UCLA called and had a cancellation appointment with the dietician for this Monday (2/28)- so we took it! After we made that appointment, Dr. Shields decided he wanted to see Jenelle again as well, so Friday and Monday I was frantically trying to get that visit approved with our HMO!

We spent the weekend in Lake Arrowhead with a friend of mine and her son who is Jack's age so the kids could build a snow man (something Jack has been wanting to do since Christmas!) My shoulder had been bothering me prior to the weekend, as if I'd slept on it wrong, and then really started hurting when I lifted Jenelle into her car seat on Saturday evening. Remember, she is 26 pounds of "dead weight" with her disabilities - I fear this shoulder injury may not be my last! Then again, it could have been my shoveling snow? The pain was so bad on Sunday, I went to urgent care for Vicodin and Ibuprofen - they think I injured my rotator cuff! Of course, Mommy's pain takes a second to getting Jenelle to her appointments, and we drove up to UCLA Monday afternoon to meet with the dietician and Dr. Shields (with Mommy on Vicodin - fun!)

The diet will require lots of liquids for Jenelle, and some of it can still be used with her jarred baby foods! Sounds like it should not be too problematic, and she will get some variety. We are scheduled to start the diet at 9:00 a.m. on March 14 - again assuming we are not bumped by sick patients. Luckily, the family I mentioned in my last update did not get bumped, but I'm told they waited until 10:00p.m. that day to get admitted to the hospital! Thankfully they did that so as not to disrupt the schedule for anyone else (like us!) We got lots of information on what to buy before hand (real butter, real mayonnaise, Centrum vitamins, etc.), and the week she is admitted will consist of lots of teaching for us, and hopefully Jenelle will become stable quickly!

Dr. Shields saw Jenelle because he was concerned with her bi-carb levels and recent seizure activity. He explained that there is no "buffer" on the scale used to measure bi-carbs, and that if her bi-carb level was 15, it was most likely that 80-90% of her blood was acidotic. Not something to fool around with, and definitely something to watch while on the diet. He wants more blood work done next week, prior to our admission to check her levels again. We are going to come down even lower on Topamax to see if that helps! Also, remember I mentioned Jenelle's constant constipation? We have an appointment for 9:00 tomorrow to see the GI Doctor at CHOC. Dr. Shields felt that she was very backed up, and was relieved to hear we were getting in this week. He did say we should think about inserting a "G-Tube" for feedings, especially if Jenelle does well on the diet. A G-Tube is a tube inserted into her stomach where you can feed her directly. With Jenelle's inconsistent appetite, and problems swallowing, it is something to consider if the diet works well for her, so we don't have to fight her each meal. Eating all of her meals is important on the diet, as is the times in which she eats. A G-Tube is something I was hoping we could avoid, but I realize it may be necessary. Dr. Shields wanted to start a bi-carb supplement, but then agreed with the nurses that we should wait to see how she does on the diet first. Everything else looks good so far - now we just hope she stays healthy these next few weeks so we don't have to postpone our start date!

I finally got into see an Orthopedic Doctor yesterday, and an x-ray shows I have a calcium deposit on my rotator cuff (right arm of course - as I'm right handed!), that is probably causing too much inflammation. I got a cortisone shot, and am still in some pain today. I will check back with him on Friday if there is no improvement.

And finally, Jenelle got her new AFOs (ankle/leg supports) last Friday, and I got some cute photos of that with Jack. With Jenelle not meeting normal milestones for a girl her age, we have to celebrate what we can - even if it is her "first" new pair of "pink" AFOs! :)

Please keep Jenelle in your "healthy" prayers and positive thoughts for the next two weeks so we can start the diet on time! Thanks for the continued support - I'll keep you posted!