Just Found Out Emmalin Has To Get A Feeding Tube

For the last couple of months, Emmalin has slowly stopped wanting to eat and drink. It's been several things that have been/still are in the mix that got Emmalin to this point. About 3 months ago, when we were at Dr. Weisenberg's office (Emmalin's epilepsy specialist and "main" doctor), she had brought to our attention that Emmalin had not gained any weight since her birthday (October 29th). She encouraged us to meet with a dietitian (again) and set up a "game plan" for if things were to go "south" (and of course these were not her exact words). So we did that and I even added another feeding therapist "on board" that we would see once a week (so now we have feeding therapy twice a week). The additional feeding therapy is done at the Ozark Therapy Institute (http://www.ozarktherapy.com/ or you can check them out of facebook at Ozark Therapy Institute) and our therapist's name is Dan McCool, Speech-Language Pathologist . He is AMAZING!!   He is one of the very few therapist out there that treat the child that they are seeing "as a whole" instead of just treating the one area.
Anyway... we met with the dietician in Springfield, it was randomly after that things starting going south. Little by little Emmalin just stopped wanting to eat.

Emmalin and Dr. Weisenberg

Here's an example:
We went from getting Emmalin to drink around 24-28 ounces of Pediasure a day (which still isn't much) to around 16 a day (and sometimes less). She still eats some food, but it is mainly forced and it's not much.

So when we went to visit Dr. Weisenberg again, yesterday for a routine checkup, we found out that Emmalin still hasn't gained any weight and she has actually even lost a pound. We weren't surprised by this from the way she had been eating. So when Dr. Weisenberg brought up the subject of a feeding tube, as much as we were hoping she wouldn't mention it, we were not surprised by this either.

Some people have a hard time picturing things, so here's another example:
Emmalin wore 24 month clothing last summer. She was in 2T all last fall and winter/spring. When I went to get her old summer clothes out to sale from last year, not only could she wear them all, but some of them were even too big on her now. She has gone from 24 months/2T clothing to 18 months.

There are still SO many things that have to be sorted out and a lot of questions that don't have answers at this point because everything is so new. We do know that we will be getting the procedure done at the St. Louis Children's Hospital. Dr. Weisenberg has referred us to a general surgeon named Dr. Jacqueline M Saito, MD, to actually to the insertion of the tube. She is then having us follow up with a GI specialist, (we are still working on who that will be) who will then help us with the "maintenance" of the feeding tube. The GI will also be the one who will also address Emmalin's acid reflex issues (that are not major but there).

We also know that before Emmalin will have the feeding tube surgery done, we will have to do an "initial visit" with Dr. Saito and Emmalin will also have to have an upper GI done. We have been told that they do the upper GI to make sure that everything is in place/where it's supposed to be before the do the surgery. We now know that we are supposed to meet with Dr. Saito on the 20th but do not know at time or date for anything else. I will keep everyone posted as we find out more information.

Ok, so all this being said, YES Jarrad and I are totally bummed, scared, sad, nervous, etc... But honestly Emmalin is going to be getting SO many positive things from this feeding tube that Jarrad and I are also very optimistic. And these are the things that Jarrad and I have decided to focus on and we would like for you to do the same.

Ways Jarrad and I Feel Emmalin Will Benefit From Having A Feeding Tube:
1. Her body will have more energy and feel better all-around because it will be getting the nutrients that it needs
2. She will get to take her medications through her tube so she won't have to taste all of the bitter medication (3 times a day)
3. She will 100% of the time get 100% of her medication (because sometimes she will spit some of it out)
4. We think she could even sleep better because her body won't be hungry all the time (therefore restless)
5. She will do better and be stronger in therapies (because she will be well rested and have more energy)
6. If she is having a "rough day" and sleeping a lot, I can feed her through her tube while she is asleep (there have been several times where Emmalin will have a seizure right before or in the middle of eating and she doesn't eat anymore for that meal)
7. When it comes to eating, we will be able to focus on the quality of what we are trying to do instead of the quantity of calories we need to get in her
8. She will start gaining weight

 

Emmalin and Jarrad after her ERG Test Today

 I spoke with a mother of another little girl who has CDKL5 and just got a feeding tube last week. She said that her daughter started gaining weight this week and within the first week of getting the tube, she was smiling and starting to laugh again. We are hoping for the same for Emmalin :)

I think it's also important that you know that we ARE NOT GIVING UP on Emmalin eating through her mouth (I'm sure you could tell that by some of the things that I have said). We are going to be keeping her feeding therapies going and work toward some great goals (but will be very challenging for Emmalin). Through our therapists we are going to try to strengthen Emmalin's muscles in her mouth (which will help her have more control in her mouth and help her chew better and easier) and we are also hoping to get it to where she enjoys eating (because right now she doesn't) and get a bigger variety of foods that Emmalin will tolerate (flavor wise and larger textures). So we have a lot of work to do and we realize that it is not going to be easy and that it is going to take a lot of time to try to accomplish everything (or even some of these things). But I am very confident that I have the right "team" behind Emmalin to give her every tool possible to help her succeed! Yes, there is a possibility that she may never eat on her own, but there is also the possibility that she may eat completely by mouth again one day and that's what we are shooting for!!!

When it comes to Emmalin's feeding tube, we are also 100% confident that we have the right team of doctors Dr. Weisenberg has been so amazing with us and Emmalin so far and we know that whoever it is that she refers us to is on top of their game. We have been so blessed that God has always given us such an amazing team of doctors/nurses/therapist/coordinators that genuinely care for Emmalin. And we are trusting in God that it will be the same in this case too!


Like I said earlier, yes we are nervous and scared about this new journey, but we are also very optimistic about all of the possible benefits!!! As we progress in this new feeding tube journey, I will do my best to post updates to keep everyone informed on where we are at with things and how Emmalin is doing.

Emmalin trying to wake up after her ERG Test

                                               God is SO good and we are SO blessed!



HIPPOTHERAPY HAS STARTED BACK UP!

Last night Emmalin got to get back on Freckles for the first time since November. Emmalin did SO well! As expected, there was a little regression, but not as much as I thought that there would be. So will now get to see Freckles for 7 more weeks (this session anyway) and then there will be 2 more 8 week sessions following this in the summer and fall. We are hoping to be able to participate in those also.

The place that we do hippotherapy at is called Dynamic Strides. It is an amazing place that is ran as a ministry (and strictly on donations). They ask that you pay $45 a week if you can, but if you can't they say to come anyway. Their "motto", for lack of a better word, is that as long as they can keep the horses fed and the lights on they are going to keep going!

If anyone out there has an extra hour or two and are wanting to get out there and participate something that is rewarding and will leave you smiling each week, think about volunteering at this Dynamic Strides. It's so wonderful!!! They will take any volunteer for as long as they are willing to be there (even if it's just an hour).

If you are interested in learning more about Dynamic Strides, making a donation, or volunteering, check out their website at: www.dynamicstrides.org

You can also follow them on Facebook and Twitter!

Pictures of Emmalin on Freckles will be coming soon!!!

1 YEAR WITH A DIAGNOSIS!!

It was around this time last year that I got a call telling me that Emmalin had tested positive for the CDKL5 Gene Deficiency.  Yes, when we got the call our lives were shattered! The hopes of Emmalin being able to be “fixed” were gone forever!  But you see, before we had this diagnosis, we had been told that we had like an 80% chance of never finding out what was causing all of Emmalin's seizures (and she was only 17 months old). We had been told that it was probably something that was metabolic or genetic, and that there were literally millions of tests that they can run for those. So our game plan was to test for one thing, get it sent off to the Mayo Clinic (or where ever the one place in the US was that would run the test) and then wait for the results to come back(which normally took anywhere from 6 or 8 weeks). Once we found out we could rule that out, we would then draw more blood or do another spinal tap or whatever, just run the next set of tests. Emmalin was getting poked SO much and SO often!!! It is for this reason alone that I am SO incredibly thankful that Emmalin has a diagnosis!
Since she has tested positive for CDKL5, we have been able to take out the guessing on some of the options, medicine wise, and have been able to narrow in on what we think will work best. We have also been able to join a support group that is based out of the UK that is just for people who have a loved one with CDKL5 (which has been a HUGE blessing). Through this support group, I have been able to ask questions and read blogs about other children who have CDKL5 and it has helped me have a better understanding on where we are headed with Emmalin. I guess the biggest difference is just that, we now have a lot better understanding on where we are headed and what to possibly expect (at least a little bit) and that has made all the difference in the world!!! It just makes us not feel so lost with Emmalin and I FINALLY "feel like my head is starting to come above the water"! Getting this diagnosis changed our lives!!! And even though we would have loved for it to not have been something like CDKL5, like I keep saying, the fact that we now have an idea of the direction we are headed has truly changed our lives, in a good way, forever!


God is SO good!!

ROUGH AFTERNOON WITH SEIZURES

Today Emmalin had 4 of her "big" seizures. I hate it when we have days like this! Yes, there are MANY people who have it a lot worse, seizure wise, than Emmalin, but for us, this was a hard day! You see, if we have 3 days in a row where Emmalin has seizures (the same amount in a day or the same times in a day) at the same time, in some way, and then I consider it our "norm". I know this doesn't seem like a long time before it's our "norm", but with Emmalin, her seizure types and amounts are constantly changing! It just comes with the territory of CDKL5 (at least for us anyway). Here lately Emmalin had only been having 2 "hard ones" a day and we have been pretty okay with that. Emmalin also has head drops and sudden jerks that are seizures, but as bad as it sounds, I don't even count those in her "grand total" at the end of the day. I know this may sound bad, but it's just the way it is.

Anyway...Emmalin had her 1st seizure today around 6:30am and then her 2nd one ended up being around 11:00. This one was a surprise because she had been only having 2 a day, one in the evening and one around 2:00pm or 3:00pm. Emmalin had to sleep a lot after her second one and that's very typical right now. So because of that seizure, we Emmalin had to eat lunch around 2:00pm (because of her sleeping so much), but she wouldn't really drink anything, which isn't normal for her. I should have realized that she wasn't feeling well right then, but played it off being from her molar that she is cutting and went on with our day.  We then had to go run some more errands and then when we came back, she had her 3rd seizure around 5:15pm. Every time Emmalin has more than 2 seizures in a day, or if she's had 2 pretty close together, Emmalin doesn't normally feel good for the rest of the day (unless she has like 4 hours or so to "sleep it off"). Well, after Emmalin's 3rd seizure, she just didn't feel good, and her body kept having a lot of big jerks (poor thing). So Jarrad and I "through our plans out the window" for the evening and I just held her. She would try to relax (and go to sleep) and then she would jerk again and it would just make her wide awake again. I felt so bad for her! Then around 7:00, Emmalin had her 4th "big" seizure. Sometimes I wonder if she can tell that another "big seizure" is coming. I know today she just didn't feel good after her 3rd one (but who would) and this is pretty normal after a third. At that point, whatever it is that we have planned for the rest of the day we have to cancel and get Emmalin back to our house so that she can sleep or just be held and rocked the rest of the day. You just feel so bad for them as they have to go through all of this! Again, I realize that there are many people out there who have it worse, but knowing that still doesn’t' make me feel any better while I have to watch my baby have these bad afternoons. I HATE SEIZURES!!! After her 4th seizure, I got her jammies on and then Jarrad and Emmalin cuddled and fell asleep in the recliner. The best part of my entire day was watching the two of them together asleep in the chair. I know that may sound strange, but seeing them like that, made my day a little better.  That's Emmalin and Jarrad's thing, getting their cuddle time in after dinner and Em's bath. I have to say that Emmalin is very lucky to have a daddy that loves her so much!

I feel like I need to say, as rough as an afternoon/evening that we had, we have definitely had worse!!! And when I go to bed, I will always thank God for Emmalin only having 4 seizures (or however many she had that day) instead of 5. Like I always say, it could ALWAYS be worse, we've had worse days!!! And I feel very blessed to be at the point where we have a diagnosis and Emmalin's seizures are down to 4 big ones on a hard day.
God is SO good!!

ROUND 2 OF ABM THERAPY

We are soon going to be headed to St. Charles again for another round of ABM Therapy. We are very excited to see what exciting things my take place this time with Emmalin! Jarrad is getting to go this time (instead of my mom). It will be nice for Jarrad to get to see it in person instead of through the video camera!! I still having figured out how to get my video of the ABM sessions to post, so I am going to have Jarrad do the video this time and see if he can figure it out. Keep your fingers crossed!!!  When we get back, and actually a little bit while we are there, I will post Emmalin's progress and keep you all posted on how she is doing!

While we are up there, we will also be seeing Dr. Weisenberg, Emmalin's epilepsy specialist. It is just for a regular update, but it will be nice to go back over the "game plan" for the rest of the Clonazepam wean. I will also post information about how this appointment goes when we get back! I'm hoping for it to be very uneventful!!

CLONAZEPAM

Clonazepam...this is one of the four seizure medications that Emmalin is on at this time. Out of the four, this is the one that Emmalin has been on the longest. From the time that we started going to St. Louis to see Dr. Weisenberg, (Em's epilepsy specialist-who we LOVE), she has said that she only wanted Emmalin on 2 medications or less at a time (she was then on 4). This is also what Jarrad and I would prefer. So we have been trying to take Emmalin off some of these, but for one reason or another, Emmalin has managed to stay on 4 seizure medications. We have tried to get her off of her Clonazepam 3 other times so far. This is one that Emmalin's body REALLY likes (her body is addicted to it). Every time we get down so far, Emmalin starts having more seizures, and that is how her body has withdrawals (poor baby). So we end up putting her back on the original dose and go on. Well, this is attempt #4. Our game plan this time, is to reintroduce Topamax, which she has been on before and her body responds pretty well to (at least so far) and when she starts having withdrawals, increase the Topamax so that we can continue the decrease of Clonazepam. Once Emmalin's body had the opportunity to get familiar (again) with the Topamax then we started taking away the Clonazepam. We only take her Clonazepam down 1/2 a tablet every 2 weeks, so this is going to take a while (to say the least). We are also hoping that Emmalin's body won't have as many withdrawals because we are taking it so slow. We are now down from 5 pills a day to 3 pills a day. We have had to increase the Topamax as expected (and she's now on a pretty high dose of it) and Emmalin is still having some withdrawals, but we are making it through it. So far, Emmalin has been having some jerk and head drop seizures, some days more than others, and her larger (or as I call them her "big seizures”) seizures range from 1 to 4 a day (but mainly around 2). I realize that this amount of seizures in a day is not amazing, but believe me, it could be A LOT worse! I am keeping a very close eye on it and adjusting Emmalin's Topamax (under Dr. Weisenberg's supervision) accordingly. Next week we are supposed to bring it down another 1/2 a pill. I just hope and pray that things continue to go as smoothly as they have been throughout this process!

I'll keep you posted!

HIPS AND SPINE

I think everyone would agree that, in terms of mobility, a "typical" 2 year old walks and runs all over the place. Considering Emmalin is still working on sitting up and head control, running and walking are obviously things that Emmalin cannot do yet. So, back in September of last year, Emmalin's doctor decided to do some x-rays on Emmalin's hips (both of them) and spine to get a baseline on how her body was developing as it was growing. When they came back, Emmalin's spine x-ray was great, but her hip x-rays showed that they were not forming the way they were supposed to be forming. We were told that this is very typical of children who have developmental delays and who are not walking (which means not weight bearing and that’s what the hips need to form correctly). Dr. Weisenberg thought that it would be a good idea to get an orthopedist "on board" so that we can start monitoring Emmalin's hips and spine more closely. So, back in January, we were referred to a doctor named Eric Gordon, an orthopedic doctor that also works at the St. Louis Children's Hospital. When we got in to see Dr. Gordon, and he said that Emmalin's hips were angled at 21 degrees and 23 degrees. A “typical” 2 year olds hips should be angled at 20 degrees. He also told us that given Emmalin's circumstances (developmental delays and not walking), he was not overly concerned at this time and that we will keep updating her hip x-rays every 6 months. I then asked him we can be doing at home to try to help the hips get a little more weigh bearing so they had an opportunity to form correctly. He said to make sure that we are doing exercises (that he showed us), keep up all the therapy, and to increase her time in her Super Stand (a piece of equipment we have at our house that literally stander her up).

As for her spine, Dr. Gordon looked at that x-ray and confirmed (again) for us that Emmalin's spine looked great. But he did say that now that she is 2 and still not walking, we will now need to start keeping an eye out for scoliosis. This is a normal thing that they start watching for in any child that is not walking on their own. Dr. Gordon said that we will repeat a spine x-ray once a year, as long as the spine continues to stay straight.

So for now, this is where we stand on these two things. We go see Dr. Gordon at the end of June again to follow up on everything again. We will be hoping that Emmalin's hips have formed a little more and that her spine continues to stay straight! I will keep you posted!!!