EEG Results are In......Sort Of.

Bexley had her EEG on Friday morning. The one positive is that the EEG process itself went much smoother on Friday than it did in the hospital. In the hospital, they had to glue approximately 30 electrodes to her head, which she hated and screamed through the entire process of putting them on and taking them off. When I woke up Friday, I was dreading having to watch her go through that process again. Thankfully, this time they were able to use a gel and I could hold her the entire time (I think she is starting to realize being laid down on a doctor's office table/bed means something uncomfortable is about to happen). She was pretty relaxed through the entire process and ended up falling asleep. I was so relieved because she has been through so much that I was dreading having to watch her be "tortured" again. If more EEGs are in her future, hopefully, the rest of them will go as smoothly.

We finally got the results back from her EEG at the end of the day today.  These results seemed to have left us with more questions than answers. However, we are really just trying to focus on the positive news. The great news is that her EEG is currently showing that her Hypsarrhythmia is considered resolved at this point, which means that she is responding to the ACTH injections. We hope this will continue as we wean her off the medication. 

The nurse also reported focal abnormalities were noted in her EEG. When asked what that means, she said it tends to suggest epileptic potential.  We know that kids that suffer from Infantile Spasms are at increased risk to develop different Epileptic Disorders. At this point, we are unsure if the abnormalities noted are typically seen in all kids who have recently had Infantile Spasms or if those abnormalities mean that Bexley has an underlying Epileptic Disorder.  We are going to try to call the doctor's office tomorrow to see if they can shed a little more light on these EEG results, but we have a feeling that we will be told that we will just have to wait for our neurology follow-up appointment in August. 

As far as little Miss Bexley goes, the side effects to the medicines are still there, but seem to be lessening a little bit. We have started to notice her personality coming back more and more over the last week. She is giggling and smiling more as well as "talking" up a storm. I hope and pray everyday that she keeps those smiles coming because they brighten up everyday for us and continue to help us have the patience to wait for answers as well as the strength to face whatever challenges that may be in our future.  

Let me cross your T's and dot your I's for you........

Today Bex was scheduled for her two-week follow-up EEG. As Chris mentioned in the Side Effects posts, the ACTH appears to be stopping the spasms, but the real test is the EEG to determine if her brain waves are normalizing and no longer showing hypsarrhythmia (the abnormal brain wave pattern associated with Infantile Spasms). We were told to be at the neurologist's office at 8:15. Surprisingly, we were right on time, which is impressive considering we had to get her fed, give her oral meds and her injection as well as get ourselves ready and battle Charlotte rush hour traffic. When I went to check her in, the receptionist looked at me like I was crazy when I said we were checking in for Bex's EEG. Long story short.....but this appointment was scheduled by the dispatch coordinator at the hospital. They scheduled us a neurology follow-up appointment...not an EEG. The office that we were at doesn't even do EEGs. Chris and I were beyond frustrated - a) because this morning was a complete waste of our time and b) it means more stress/worry waiting to find out the results of this EEG. 

One thing that we are definitely learning throughout this process is that we really need to double check things when it comes to communication between different doctor's offices as well as doctor's offices and insurance. For example, we were sent 3 vials of the ACTH gel for her injections. The doctor's office told us that would be enough for the full 8 weeks of injections. When we went through the first vial in a week, we knew that couldn't be right. After some investigating and phone calls on our part, we figured out that insurance only sent us 30 days worth of the medicine. Thank goodness we caught that early enough because if we ran out of the ACTH gel, Bex would have to be readmitted to the hospital until more could be delivered to our house. 

After some calling around by the neurologist's office, they were able to squeeze Bex in for an EEG tomorrow morning at 10:30. We are hoping to have the results tomorrow by the end of the day, so we don't have to wait all weekend. 

In other news.....Bex did have her weekly check-up with the pediatrician yesterday. Her blood pressure was good. She was showing no signs of infection. Today we got the results from her blood work that they do bi-weekly to look at liver functioning, kidney functioning, etc. and everything was looking good with that. We continue to be thankful that she is not showing any serious side effects at this point! She did gain an impressive pound and a half in a week due her increased appetite, but this is the one time in her life that extra chubby cheeks are irresistible!

Bulletin Board Material...Motivation

I am a big believer in visualization.  For example, when I used to play baseball in high school and college I put up motivational signs or goals to look at every day.  This helped remind me of my objectives and visualize a successful outcome.  I even do this today regarding my yearly performance metrics or business targets.  Some may think this is silly, but I really do believe it can help encourage you to reach your goals.

So I have taken this practice and applied it to what Lindsey, Bexley, and I are facing with her disorder and treatment.  I have the following checklist up in Bex’s nursery to help push us to keep working with her to prevent spasms and improve her developmental areas:  gross motor skills, fine motor skills, etc.

I know this is “sports” themed, but being a competitive person and former ballplayer this resonates with me.  I can explain each bullet point to help illustrate how it applies to Bex if that helps...

Pregame Prayer – Before you take the field, or before we take on the day, we should pray for the strength to do our best.  Bex deserves nothing but the best and we need certainly need help from above.

Stay Within Yourself – Don’t try to do too much!  Basically, don’t press or push yourself.  Right now we can’t cure Infantile Spasms…but we can work on certain things with Bex that can limit or even prevent damage/delay.

Compete on Every Play – Never give up, even when you hit a dribbler to the pitcher. You run hard and never assume you’re automatically out.  The same can be said with our effort with Bex.  Even if it’s a simple task like helping her play with toy rings to work on transferring…don’t take it for granted because this could be critical for helping her develop.

Pick Each Other Up – You are part of a team and sometimes you have a rough day at the plate.  That is when you need your teammates to pick you up.  Lindsey and I are a team and we know there are going to be days where one of us will struggle, but that is when the other needs to step up and provide support.  There is no “I” in Team Bex.

Learn from the Losses – There will be days we lose…whether it’s a day Bex doesn’t want to cooperate with exercises/play time or a doctor’s appointment that isn’t the most optimistic.  You take those losses and you figure out how to do better the next time.  We shouldn't be discouraged.  We just have to be realistic and learn from the experience so we can hopefully have a better outcome the next time.

Celebrate the Wins… - And most importantly, when Bex has a great day or hits a milestone or we get great news from a Dr’s appointment, we need to party like its 1999.  There is nothing wrong with enjoying victory and when you put in hard work you deserve that celebration.  However, you also need to quickly look at the next opponent…or milestone to go after!

I don’t mean to be the prototypical jock with this list, but I do think it works for me...and Lindsey agreed it works for her too!  That is one thing I love about my wife...she is just as competitive as me.  In the end, if it helps keep us motivated then that is all that matters.  Besides, I always loved playing sports because I was part of a team.  I can’t imagine a better team to be on than Team Bex!

#gobexgo

Side Effects may include…a Mad Monch!

Lindsey and I don’t want to get our hopes up too much, but it seems that Bex is responding to the ACTH injections because the spasms are either so mild we don’t notice them or they aren't happening at all right now.  However, we are only feeling cautiously optimistic and aren’t waving the victory flag just yet because she is still on a high dose and we are aware that it is possible for her to relapse as we start tapering down her meds.

While the meds appear to be working, we are also dealing with the side effects.  Now I will preface this by saying we aren’t experiencing any of the serious health side effects as of yet – such as high blood pressure, increased sugar in the urine, gastrointestinal bleeding, etc.  We have a weekly appointment with the pediatrician to check for these issues as well as draw blood every other week for additional tests (electrolytes, kidney function, etc).  He is also checking for any signs of infection because her immune system is weakened right now. So far so good with these results and we hope it remains this way for the remainder of the time she is receiving the ACTH injections.

The side effects we are dealing with are regarding her temperament and behavior.   These are increased appetite, frequent urination, change in sleep habits, and irritability.  The bigger appetite and frequent tinkling (yea I used that word) aren’t necessarily a major deal.  Although, a hungrier Bex does mean Lindsey has to up her game with milk production...and we have had to start tapping into the freezer reserves of milk. We also spent our Saturday night making sweet potatoes, green beans, carrots, pears, and prunes to stock our freezer up with food to keep up with her increased appetite.  The real downer for us is her mood change.  She just seems to be less happy and not our normal Bex.   We can do normal activities with her, but it just seems like she isn’t enjoying them like she used to and it is harder to get smiles and giggles out of her.  She is not constantly fussy or crying by any means…but we just know she is not herself.

We were made aware that this could happen and I can’t necessarily blame our little Monch (long story....but one of our nicknames for her is after the 90’s dolls named Monchichis).  We are pumping her full of a synthetic steroid and also giving her an anti-seizure medicine.  I have read how these types of drugs can affect adults…I can’t imagine the impact it is having on an 8 month old 16 lb baby.  We are trying not to complain, because again our goal for the short term is to stop the spasms and so far the meds appear to be helping with that.  The big test to see if the meds are working is if her EEG shows improvement on Thursday.

We just get a little sad thinking that these meds are keeping her from enjoying herself.  However, we know this won’t be a long term issue.  We will be tapering down the Achtar gel doses throughout the weeks and that should hopefully help her normal personality to return.  Plus, there are brief moments where she is herself and we get one of her famous Monch smiles that could brighten a room…so in a way we know the old Bex is still in there.  We just have to be patient and she will be back full time eventually!

You'd Like Me To Tell You About Infantile Spasms...Don't Mind If I Do!

To say last week was a bit stressful and hectic would be an understatement.  In a way, our world was turned a bit upside down with Bex’s diagnosis of Infantile Spasms.  One of the main reasons we felt so helpless at first was because we didn’t know what we were up against.  Even though IS/West’s Syndrome was discovered back in the 1840’s it’s still a relatively unfamiliar disorder…it only affects roughly 1 in 3,000 kids.

Even though we are still roughly “new” to IS, Lindsey and I feel we can serve a purpose with spreading awareness.  We are hitting the books, looking up articles online, enlisting friends/family in research, and reaching out to medical experts to gain a better understanding of this serious disorder.  One of the websites that has been helpful is The Child Neurology Foundation website on Infantile Spasms Awareness.  There is a great video that explains Infantile Spasms (see link below) and I ask that you please take the time to watch it (about 25 min long).  I have also posted this video on the “About West Syndrome” page, along with other informative videos.

Understanding Infantile Spasms

I believe we have been given an opportunity to be advocates for children and families suffering from IS.  To put it in perspective, when the neurologist confirmed Bex had IS, I felt a wave of emotions – sadness, anger, & fear…all at the same time.  The biggest kick to the gut was the fear…not knowing how to help my baby girl.  I would never want another parent to feel that way.  Ever. 

We have a lot of friends and family members who are new parents or on their way to becoming parents.  My intent isn’t to scare them with our message.  They shouldn’t necessarily have to worry themselves over the possibility of their child being diagnosed with infantile spasms.  The odds are 99.97% of kids won’t be affected by IS…but what about the 0.03% that will be? 

Emerson said, “Knowledge is the antidote to fear.”  Truer words were never spoken.  Hopefully Bex’s journey will spread awareness and help new parents gain knowledge so they can 1) understand and identify symptoms of IS accurately and quickly, 2) receive treatment ASAP and reduce the chance of more damage, 3) develop a game plan for addressing any type of developmental delays and improve their child’s quality of life, and most importantly 4) be FEARLESS when taking on IS…like my little girl.

#gobexgo

A Social Worker, an Occupational Therapist, and a Psychologist discover a Bexley on a couch...

This morning we had a social worker, occupational therapist, and psychologist out to the house to evaluate Bex and determine if she is eligible for North Carolina's Infant-Toddler Early Intervention Program. Since Bex is only 7 1/2 months old, the majority of the evaluation involved Chris and I talking about what she can and cannot do. Bex was in a great mood throughout the evaluation and worked hard to show off some of her skills for them.

The good news is that she is on track in 3 out of the 6 areas that they evaluated her for: social, communication, and adaptive behavior. As we suspected, her gross motor skills are her greatest area of weakness. Since all areas of development build off each other, her gross motor skills are also impacting her fine motor skills as well as her cognitive development. Cognitive development was a little surprising. However, they explained that babies mainly show their ability to problem solve through their motor skills, so then that made sense. They did comment that she has a great foundation of skills.  The key is getting her to use those foundational skills more consistently. Hopefully, with the right interventions in place, we can get those gross motor skills clicking, which will help the other two areas start to fall into place.

In the hospital, I had mentioned to the Occupational Therapist that I spent the last 4 years working in Special Education, so I was familiar with how the evaluation process works. She commented that it was awesome I was so willing to get Bex evaluated, since sometimes people who have worked in Special Education are resistant to that idea.  I will admit that I never thought I would find myself sitting on the other side of the table. However, my experience as an IEP Coordinator showed me how important early intervention is, so I am more than willing to accept all the help that is needed to help Bex reach her full potential.  When I initially set up this evaluation, I thought that hearing Bex qualified for services might be difficult or make me sad, but after our meeting today, I actually felt a great sense of relief for two reasons. 1) I no longer have to worry about what milestones that a typical baby is accomplishing and worrying that she is falling further and further behind. Instead, I can focus on the individualized goals and milestones that we develop for Bex. 2) I will finally have resources to help me figure out how to best help her instead of guessing and trying to figure it out on my own, so I will no longer have to second guess if what I am doing is "right."

Next Monday, they are coming back to work with me on how to integrate all kinds of exercises and activities into our daily routines to help Bex start improving her gross motor skills. The only real disappointment of today is that I have to wait a full week for them to come back to get the plan in place!

Happy Father's Day!!

Bex is one lucky little girl to have Chris as her daddy! Not sure if it is a fluke or a side effect of the ACTH, but she was awake all day today. Thankfully, she was in a good mood, despite refusing to take a nap, so Chris was able get a lot of good quality time with her.

After our week in the hospital last week, we couldn't have given him a better Father's Day gift.

She is a pretty charming little girl because in the last 7 and a half months, she has managed to wrap her dad as well as her grandfathers and great-grandfathers around her little finger. 

Happy Father's Day Pappy, Great-Gampa, Buelo, and Pawpaw!!! 

She also has one special great-grandfather watching over and cheering her on from above!!

‘Nother Round of Shots!

So today was the first day of Bex’s ACTH injections without any assistance from a trained medical professional…yup, on our own!  To say it was a bit stressful would be an understatement.   Actually, giving the shot wasn’t that bad.  It was trying to measure out the exact dosage that was the complicated part!  Our doctor wrote down the dosage in units of the medication and the syringes are in mLs.  So we are supposed to be giving Bex 60 units of the ACTH, but we didn’t know how that translated to mls.   We spent about an hour with the hospital trying to make sure we knew the exact amount to give her.  

The funny part is after they explained how to measure the correct amount of medicine I nearly laughed out loud.  On the box it provides the ratio of the units to mls (400 units = 5 mls or 80/1).  Welp, there is your answer!  If we are supposed to give her 60 units then it’s just 60/80 to get .75 mls.  It was my own “come on man” moment.  Not to toot my own horn, but I am a pretty smart guy.  However, when the situation is giving your 7 month old daughter a steroid injection you tend to worry a bit more and error on the side of caution.

After giving Bex the shots Lindsey and I went to work on researching more about IS/West Syndrome.  It’s time to be proactive vs. reactive and we have already submitted several requests to get in touch with resources and support networks to get more help on how to work with Bex (and manage our lives) with this challenge.  More to come as we continue to update the “About West Syndrome” page…

-Chris