Finding peace in a world full of "what ifs?"

Lots of things have happened since my last post way back in May. At that time Sophia's seizures were really bad and we were running out of medicines to try. We decided to go ahead and get the VNS implant in hopes that it will control the seizures and hopefully stop some of the medications and their side effects. The VNS couldn't be turned on right away and when it was on the doctor turned it up very slowing making sure that Sophia was able to tolerate the feeling in her throat.

 It has been five months since her surgery and I'm happy to report that getting the VNS implant has worked great! Sophia is down to only two medications and she is as happy as always. She is not seizure free as we knew the implant was not a cure, but it has definitely giving my girl some well deserved quality to her life and ours. 

This past year was really hard on all of us. Of course it was hard on Sophia since she is the one who couldn't catch a break but it was also very hard on Eric and me. It was also really hard on my mom who was here helping me when I needed it the most which I am so grateful for. The seizures took a toll on Sophia and all the progress she had made in the last couple of years, the set back is big and although she is slowly getting her strength back I can see some areas where she is going a little backwards and... well, that sucks! During Sophia's first few years I always pride myself in focusing on her abilities instead of her disabilities but this year I've had a really hard time with it. I've found myself wondering about a lot of "what ifs": what if she could walk?", "what if she didn't have a syndrome?", "what if she didn't have epilepsy?", "what if she could sleep through the night?", "what if Sophia could have sleep overs?", "what if... we were typical?". And every time I would stop and get mad at myself for feeling that way, then I'd feel sad and angry that this was the hand that was dealt to us. It was becoming a vicious cycle that I needed to end. 

The truth is this is the hand we were dealt and I can't change that but I can change how I feel about it, at least most of the time. All those "what ifs" are not really a possibility so there's not much sense on wasting all my energy on feeling sorry for myself. Now, I'm not saying I won't ever wonder what if this and that, I'm just going to try to go there as seldom as possible and when I do I'll do my best to forgive myself and go back to enjoy the life I get to live. 

Sophia is doing great, her seizures are mostly under control and she is walking with her walker again, she is playing with her toys and she is back to be the curious girl we all know. She is laughing out loud a lot again and she is enjoying the things she used to before the seizures took over. She is going to school all five days of almost every week and I get great observations from her teacher every single day. I want to choose  to forget about the "what ifs?" and concentrate on the "she is" and "she can", for Sophia... for us.

The amazing Sophia.

"A sneak peek into the unique world of the girl that amazes me every day", that's the description of this blog and Sophia proved that statement once more today. It has been a really rough few months for her dealing with lots of seizures and side effects from the medicines she is on. Sophia had to deal with many stays in the hospital for overnight EEGs, lots of changes in her medications doses, several stay home from school days and countless missed social events and celebrations. Her quality of life has been really affected by this disease and it was clear that we were not getting anywhere with medications and that a different path was much needed. Our doctor suggested one of two paths: a keto diet or a VNS implant. We did our research and decided for the latter. A VNS is a device similar to a pacemaker but for the brain, it goes on her chest just underneath the skin and it has a wire that goes to the vagus nerve on the neck. The implant stimulates the nerve and sends pulses to the brain to stop the electrical discharges causing the seizures. It's pretty safe and it could potentially reduce the amount and the frequency of the seizures, has almost no significant side effects other than a little cough and hoarseness which happen in the beginning. The only downside is that it doesn't start working right away but is for a good reason: it needs to be turned on and programmed little by little to cause the least amount of discomfort and studies show that the longer it takes the better results you get.
 Based on all this we decided the VNS is the right path to take but while we were waiting for the surgery day, Sophia's seizures were still uncontrollable; happening day and night, we needed something sooner and the doctor decided to put her on steroids. That was a treatment she didn't want to have to prescribe because of the side effects but the benefits definitely outweighed the risks.  Fortunately it worked! The hunger, the irritability and the mood change are there for sure but so is the being more awake and alert as well as decrease in the number of seizures. Sophia is definitely not back to being herself but it's so nice to see a glimpse of the resilient girl she is.
As I said, today she amazed me once more! Today was her VNS surgery and I had all kinds feelings about it. I was nervous, scared, hopeful, mad and anxious. Surgery means anesthesia and Sophia has had problems with nausea and low oxygen  in the past. It's supposed to be a day surgery but I was scared that she was going to have problems and we were going to have to stay and I was mad (still am a little bit) that my girl had to go through yet another procedure, mad that she has epilepsy hard to control and mad that this implant, although promising, is not a guaranteed fix and we still need to wait and hope that it at least helps some.
Yes, I was a nervous wreck but not Sophia. My sweet girl was as happy as always when the doctors took her away, when she woke up she did cry for a few minutes while she was waking up but then I saw the beautiful smile and the curiosity and the personality we all know. She ate very good with no signs of nausea and in no time we were discharged. She was very happy on the ride home and hearing her laugh in the back seat made me forget, at least for a while, what she has been through. At home she was very hungry which I was very happy to be able to fix and then she fell asleep in her abueliita's arms. It was almost a perfect day despite the surgery and it's all because of Sophia, people often tell me how strong I am but the real hero here is this girl! I am strong because she is strong, I am brave because she is brave and if she can put a smile on her face after having a day like today and keep on going then I can too.

Side effects.

Epilepsy sucks! It really does. It's stubborn and it's tricky, when you think it's under control and you start to relax, the seizures start again.
Sophia was diagnosed with epilepsy about 5 years ago and it was pretty manageable for 4 of them. She was on one medication that needed to be increased every year as she got older and bigger. About a year ago after we increased that medicine Sophia started having more seizures, her neurologist said that we had maxed out the dosage for the medicine she had always been on so we added a new one. That medicine came with its side effects: loss of appetite and risk of kidney stones which helped to finally make the decision to get a feeding tube. Things got better for a period of time until Sophia started showing new symptoms: she was slouching towards the left when sitting down, sometimes she could catch herself but sometimes she couldn't and would fall on the floor. She was very shaky and weak and we increased the medication several times with little improvement. Last November Sophia had what was supposed to be a 48 hour EEG at the hospital but she ended up staying there for a week because the test showed that her brain was in constant seizure activity even in her sleep, you can read more about it here. At that time, we made a lot of different changes in her medications and found a treatment that seemed to control the seizures enough to be sent home. Sophia was so drowsy the first few weeks due to the side effects of the meds that she would take several naps during the day and still slept all night. Little by little she was more awake and alert but then she started having seizures again so we kept increasing the dose of some of the medications but with them the sides effects increased too. The sleepiness and the drowsiness came back and she also started losing her beautiful hair. It's funny how you can be strong facing difficult situations but then, something little sneaks up on you and you lose it... I was able to manage the stress of having to watch Sophia like a hawk to make sure to catch and tape any seizures to show the doctor, trying to remember all the dosage of all the medications and seeing my daughter exhausted all the time. But when I saw the incredible amount of hair in her brush and the bold spots in her beautiful head I lost it! I felt so powerless and so helpless... Her hair loss was like an imminent proof of the toll the strong medications that try to control this horrible disease have taken on my daughter.
The last dose increase helped for a few weeks which seemed to be the pattern with every medicine we try. Sophia showed the same symptoms she had last year: tremors, shakiness, absent and full blown seizures and extreme tiredness. An EEG showed she was seizing constantly again, day and night non stop. Her brain is in constant activity, when she sleeps she doesn't enter into a deep sleep so she never gets to really rest. That's why she is so tired all the time!
The medicine is clearly not working, all it is doing is adding to the sleepiness and causing her hair loss. Right now the plan is to go back on a medication Sophia was on before which helps a little but she can't be on it for a long period of time but enough to make a decision as to what path we should take. The doctor gave us two options to think about: a ketogenic diet (a very strict diet high in fat, low in carbohydrates) or a VNS therapy (vagus nerve stimulation) which is an implant that sends pulses to the brain to control the seizures, this therapy is used when seizure drugs are not effective and surgery is not an option. Both have their pros and cons and we clearly have a lot to think about. I am not sure which path we are going to follow but we have to do something soon! Sophia wants to be the happy, energetic, strong, active, she wants to learn and advance, she wants to play and walk but her body is trapped in a cobweb made with drug side effects and a fog caused by the seizure activity in her brain, and I am going to do everything and anything I can to help her break it and be the girl she is meant to be and we all know.

I can see clearly now.

Sophia sees a lot of doctors. She is followed by about 6 specialists and one of them is an eye doctor who has seen her since she was born. One of the first things we noticed when he saw Sophia as a new born was her left eye being smaller than the other one as well as her left pupil. When she was about 2 years old, Sophia had a surgery where the doctor opened up her tear duct and saw that everything looked normal inside despite looking different on the outside. She had follow up every six months or so and Sophia seemed to see ok but because she can't tell us we were never really sure. A few weeks ago, the doctor examined her eyes with her pupils dilated and saw that Sophia has astigmatism and since she is also turning her left eye out at times he wants to give eyeglasses a try. He wasn't sure how she was going to react and I didn't either. We didn't know if the glasses were going to be too overwhelming for her or if she was going to try to take them off all the time and I was not looking forward adding another stress to her life. But we decided to give the whole thing a chance and I am so glad we did! When the glasses were ready to be picked up, I sat on a chair with Sophia on my lap, the guy helping us was having a little bit of a hard time fitting the strap around Sophia's neck but she didn't care at all! She was too busy being amazed by the world around her! Her eyes wide open and a "wow" look on her face were priceless.
 It has been a week since Sophia tried her glasses for the first time and she hasn't tried to take them off, not even once!! Once again I underestimated my daughter and once again she shows me that she can figure things out, I guess I tend to transfer my own insecurities and fears into her and assume she feels the same. I know that doesn't make me a bad mother but I do want to assume that she is capable of doing amazing things if given the opportunity. I was not sure the glasses were going to work and I was already leery about them even before we got them! Sophia is smiling more when we read her books and noticing little things like prints on my clothes and details on her toys. At school she is looking a things as if it was for the first time. Her teacher said that Sophia is more attentive since she is wearing her glasses!
We gave her the chance and here she is re discovering the world around her, the same world she had seen before a little blurry and distorted,  now sharp, bright and full of life!

And she looks great too!

The blessings of having a feeding tube.

This past week was feeding tube awareness week, and I wanted to take a moment to reflect on what this journey has been for us and also on why it is important to create awareness. When some doctors and feeding specialists had suggested a feeding tube for Sophia, I refused to do it because I was convinced that I just needed to work harder and insist on making her drink enough. I thought I needed to keep trying out different sippy cups,  different flavors... There was no way I was going to open a hole in my baby's belly! Turns out I didn't really know a whole lot about tube feeding and I was afraid of the unknown. I thought that a feeding tube would be the result of me failing my daughter, crazy I know but that's what I was feeling at the time because I knew so little about the amazing possibilities that this procedure would give us.
Today, 6 months after her surgery, the tube not only became second nature to us but it also came to our lives at the right moment. In November, Sophia's seizure activity increased considerably and we had to add medications to control the seizures. She is now taking 4 different meds and I know that without the tube it would be a nightmare to try to give her the 6.5 mls she takes in the morning and the 18 mls  she takes at night. Sophia went from drinking, with a lot of difficulty, 2-3 ounces of liquids a day to getting 24-26! For that reason alone the feeding tube has been a life saver. Another benefit from having this tool is the possibility of getting the nutrition Sophia needs whenever she can't get it from eating by mouth. When we started trying new medications for her seizures, her body was having a hard time adjusting to them and she was extremely drowsy and sometimes she wouldn't wake up to eat. Other times it would be one of the medications causing her to lose appetite. Whatever the reason is for Sophia to have a hard time eating, we have now the possibility of feeding her through her tube and that not only alleviates the frustration but it truly gives me a great peace of mind.
Having a feeding tube hasn't stopped Sophia from doing the things she loves. She can take baths and play in the water like she always has and she is not going to stop going to the beach in the summer because of it. We still go to restaurants, playgrounds and visit family. The feeding tube is part of Sophia, it's no more than an extension of her digestive system and a tool for feeding just like a spoon is.
There's dozens of reasons why kids and adults have feeding tubes. Some have a lot allergies so they can't get the proper nutrition, others have a hard time swallowing, many aspirate food and liquids. A feeding tube is the reason why so many people can get the nutrients, medicine and fluids that their bodies need to thrive and reach their potential.
I didn't know all of this until Sophia got her feeding tube, I wish I had been more informed long before we made the decision to get it. The transition would have been less traumatic and I honestly think I would have done it before we did because it would have saved us a few trips to the ER for dehydration. That's why it's so important to create awareness about feeding tubes, people that are considering it need to know that it's not as terrible as it sounds and having one saves lives. The community in general also need to get informed so there's less judgement and more understanding. So here it is to the Feeding Tube Awareness Week, a picture of my Sophia being saved by one.

Medicine too thick and yucky? No problem!

Too tired to eat? No problem!

Mommy and daughter day left me exhausted. But that's a great thing!

Today Sophia and I had a busy day. The day started right before 4:00 am when she woke up happy and ready to go. This has been her waking up time for the last few weeks, not sure why but she is not upset at all. I can see her in the monitor having a great time laughing and babbling. Yes, it's way too early for me but who can get mad at that silly happy girl really?!

First was our orthopedist appointment which went excellent. Everything with Sophia's hip looks great as well as with her feet. The AFOs are doing their job and three hip surgeries seemed to do the trick to fix her hip dysplasia. The doctor wants to see Sophia in 6 months for a follow up and if everything looks good, we can see him only once a year, which could really help us a lot.

Sophia also had an audiology appointment but not until early afternoon, so we went to the mall and it was great! we had the playground for ourselves and Sophia wanted to walk from wall to wall holding hands. She didn't want to crawl, she wanted to walk! After a good workout walking, we went to the restroom and even though her diaper was very wet, she sat and peed! My big girl!
Then we went to the hospital where we had the appointment, had lunch and with still one hour to go, Sophia wanted to spend it walking everywhere and exploring everything. She was loud, alert and very happy. The hearing test went well, she was very interested and amused by the sounds but after a little while she decided she would rather walk than sit and listen to the same over and over again. The audiologist was very pleased to see Sophia so grown up and so not like a baby anymore.
By the time we were finally on our way home, she was so tired that she fell asleep in the car which is so not like her. Sophia has fallen asleep in the car about 3 or 4 times in her whole life! We got home and she was still sleeping so I put her down,  I sat next to her feeling exhausted but oh so very happy! I looked at my sleeping daughter and thought about the kind of day today was, everything Sophia did today, the walking, the exploring, the going to the bathroom, the interest in being a big girl, how proud she was of herself, all those things are nothing but the living proof of how far she has come. Despite all the surgeries hospitalizations and set backs, Sophia always finds her way back to where she was before and more.
There's still a lot of uncertainties, a lot of unknown territory and probably many more battles to fight, and we will fight them and we will face them over and over again as many times as we need to just to have more days like today.

 

 

On a brighter note...

I needed to write this type of post for a long time, one in which I talk about the wonderful things Sophia is doing and the progress she is making. The last few weeks have been clouded by the seizures episodes and the ESES (electrical status epilepticus in sleep) which haven't stopped but we are working along with the neurologist until we find the magical combination of medicines that will keep the under control for a significant period of time.
Last year I talked about Sophia's communication system the PODD, (you can read about it here). It's a very robust system and Sophia is making good progress with it. She understands that the PODD is part of her and accepts it in her personal space. She started to notice that if she wants something she can get it by asking for help with the book. Sophia has tapped on the picture of the item she wants a handful of times, has said yes or no by putting her hand on the symbol more than a few times. It's a very long process but there is progress, although slow it has been steady.  Sophia is a smart and stubborn little girl, all her life we have been giving her what she wants and anticipated her needs. She hasn't had to work for them until now, we know what she wants but we are showing her how to use to book to ask for it. I am very fortunate to have a great teacher who works so hard to make sure Sophia has everything she needs to succeed in communicating her wants and needs. And she will succeed, I believe she will.
Another exciting and certainly unexpected area of progress is Sophia's potty training. I have to confess I thought we were going to be changing diapers for the rest of Sophia's life. I thought potty training was something way too complicated for Sophia to understand. Well, did I under estimate my daughter! We started this process about 3 weeks ago along with Sophia's teacher and it has been amazing! She definitely understands the purpose of sitting on the potty and is so proud of herself when she goes. Sophia is staying dry all night long and for long periods of time during the day so the wet diapers are less. Her teacher is amazing and has helped me so much with this process giving me tips, recording data to find a pattern of Sophia's trips to the bathroom and keeping excellent communication between us so we are in the same page always.
Potty training Sophia has given me a new look at Sophia's future. Sophia has been our baby for seven years, literally. The fact that eventually she could use the bathroom has given me hope. I was so worried about going out to a public restroom when Sophia was too tall and too heavy to be on a changing table  (technically, she already is). Now, seeing her so motivated, I can see her being more independent and confident using the big girl bathroom and leaving behind the baby diapers. It's funny how excited I was buying flushable wipes and emptying the potty seats and cleaning them, it made me feel a little like a typical mom. I felt like we are slowly making the transition from having a baby to having a toddler. It made me see once more that my daughter is going at her own pace and I can't and shouldn't rush her. She'll get there, when she is ready she will.

Baby Sophia

Big girl Sophia

Trying to get back to "normal"

Hello, it's been a while since I last wrote a post but here I am. I left off when Sophia was finally able to go back to school after a few weeks of being drowsy from all the new seizure medications. She was back to the routine and I was ready to deal with the sadness and stress those weeks left on my shoulders and we were going to go back to our "normal,"  Well, we haven't gotten there yet.
Sophia had a couple of good days at school and still needing naps at home and sleeping all night. The medications seemed to be working fine, she was still sleepy at times but nothing we couldn't handle. Then she started with this very dry cough waking her up at night wheezing, we gave her nebulizer treatments but then she had low fevers so the doctor gave her antibiotics. When she finally was getting better and trying to get back to the routine, she got a bad ear infection and since we just had finished a round of antibiotics,  had to start a stronger kind which caused diarrhea. My poor baby had a nasty diaper rash and as if that was not enough, she also got a yeast infection. It was painful for me to see her like that, I can't imagine how painful and uncomfortable it was for her to have it. That took several weeks to clear up but it finally did. Sophia was being more awake, not taking as many naps and everything seemed to start getting better.
No such luck.
For the last two weeks Sophia has been waking up very early. I try to get her back to sleep, sometimes she does and many others she doesn't. When she does go back to sleep next to me or when she takes a nap in the afternoon, I noticed Sophia having short seizures. Seizures! Again!
I understand my daughter has epilepsy and always will. I know that. I know she will always needs medication to control it and I am ok with that. What I am not ok with, is the fact that we don't seem to find the medication that would control it longer than just a few weeks. I am also not ok with adding more medications and dealing with the side effects. I am not ok with having EEGs and seeing my little girl go through the pain and discomfort of putting on and taking off of the leads. But, I have to be ok with all of it. I need to keep until we find the right medication and the right dosage that will control the seizures for a significant amount of time. I want to get excited that Sophia wants to walk everywhere using her walker or holding our hands, I want to enjoy the fact that she is interested in getting potty trained and has succeeded a handful of times. I want to go back to our "normal" and I want it for a period of time longer than a week. Is that too much to ask?
So here we are, already increasing the dosage of one of the three medications we started in November and waiting to be schedule for an ambulatory EEG to see how Sophia is doing at night. And here I am, trying to stay positive and trying to not let this condition get the best of Sophia, of me, of us.