Deacon Update

Medical updates:

1) Labwork: The doctor called with results from the Methymalonic Acid Test, which gives a better indication of his B12 level. The highest end of the normal range is at 318, his was 321.

Guess what this means??

Inconclusive... What the heck! Can we not get a straight forward result for ANYTHING!?!

She spoke with the Geneticist again and they said abnormal usually is in the 1,000's, so they don't think it is the cause for the seizures. But, they want to do the test again.

2) 24 hour EEG: On the bright side, the hospital called yesterday and there was a cancellation, so they are now able to admit him a WEEK early for his 24 hour EEG. A week may not sound like much, but I could possibly be THE most impatient person in the entire world, so 168 hours sooner is fantastic! Not only that, but I am a teacher and the other dates we had scheduled fell on TAKS (state standardized test), and those are the worst days to miss.

We will also get his labwork re-done while admitted and b/c we will be in-patients, the hospital does the labs themselves, getting us the results much sooner.

Deacon updates:

Deacon has been in a great mood these past few weeks with lots of smiling, and some laughing and eye contact too. His coordination has grown leaps and bounds. He stumbles very little now and has improved at getting around obstacles.

I am trying to be an optimist in all this, but don't want to paint a false picture of how everything is effecting him.

You know how babies faces light up when they hear or see their parents? We very rarely get that. Most of the time there is no recognition in his eyes when he looks at us. It is like he is seeing us for the first time every day. He will wander around our house aimlessly and doesn't seem to notice or care whether or not we are there with him. He will let us hold him as long as we like, but doesn't initiate contact. He turns his head when we lean in to kiss him.

It is hard to see some of the milestones he previously hit disappear. Deacon was saying "mama", "dada", and "bubba" at 5 months and now is at least starting to be vocal again, but will mostly hum or make an "a" sound. He is still having trouble holding his bottle while upright, and wants nothing to do with a sippy cup (I have an upcoming blog dedicated to this topic alone).

The medicine has greatly reduced the spasms, but they are still happening up to about 10 times a day. Half the time he catches himself before he falls, but the other half he doesn't. He has constant cuts, bumps, and bruises on his face. Because of this we are looking into getting him a helmet. I know it sounds so terrible on my part as a parent, but I have really been delaying getting him one. Everyone who knows about his medical issues and then meet him are so surprised on how "normal" he looks. I don't want that to change. I don't want him to look "different." I don't want kids at the daycare to stare at him or ask Carson what is wrong with his brother. But, it is getting to be a safety issue, and I will choose that over something as stupid as vanity any day. And, he would wear it mostly just at daycare, where he typically gets injured more often because of the amount of cribs in his room to be bumped into.

Some days all of this can be a little tough to swallow.

But other days we catch a glimmer. The recognition. A laugh. Leaning his forehead against us as we kiss him. Snuggling tighter or laying his head on our shoulder when we hold him. That is what we are fighting so hard for. He is in there and he is relying on us to find an answer.

Deacon's Labwork

I left a message with Deacon's Epileptologist this afternoon to see if anything had come back regarding his labwork. A couple of hours later the doctor called with some of the results.

His Organic Acid level was elevated. Normal level ranges from 0-6; his was 14. She said that this is the second time he has had this test, the first time it came back at the top of the normal range of a 6. The high level indicates a metabolic issue that causes an enzyme deficiency preventing him from absorbing the vitamin his B12. This very well could be the culprit for the seizures and developmental delays.

Our doctor said that labwork could have errors from a number of things, for example the length of time it took for it to be processed or the sample drawn, so wanted us to get more testing done to check the validity of the results. We went TODAY for more bloodwork and the lab told us it will take 3-5 business days to hear back.

We are praying the deficiency is the cause. If so, we will be referred to a Geneticist and Deacon will be given something either to help him absorb the B12, or injections of the vitamin itself. This could mean that his struggles with Infantile Spasms may disappear!

I am trying not to get overly excited because I know that the results could come back with no metabolic connection or that this could mean a whole other set of issues. But I am hopeful that this could be the answers to our prayers.

ECI Update

While we wait for Deacon's 24 hour EEG (scheduled April 4th), we are definitely utilizing his Early Childhood Intervention services! The picture above shows some of the goodies they have given us for Deacon to use. The item around his neck is called a chewy tube and is basically a baby chew toy to keep him from gnawing on his clothes while he is teething. On his hands are thumb splints. One of the things we have learned is that many children with neurological issues have a habit of tucking their thumbs inside their fists, which interferes with fine motor skills like pinching and grasping items. The splints keep him from tucking his thumbs in, but allow him to hold stuff. He is supposed to wear these at least 8 hours a day, but he actually likes them (he must think they are another item to chew on, and with 2 new teeth arriving this week they have come in handy). We have already seen improvement in him holding cookies and his bottle.

Deacon's newest service, Vision, is provided by our school district, but they keep in close contact with ECI. We were given district enrollment paperwork and it feels so strange to be filling it out for our 1 year old, instead of our 5 year old!

I have heard fabulous things about his VI teacher, who services some children at Deacon's daycare, including the directors grandson. She already knew who Deacon was, and has peeked in on him a couple of times before he was officially added to her caseload. Last week she did her formal observation and sent me an email about her first impressions:

Wanted to let you know that I worked with Mr. Deacon this morning. First of all, he is so precious! I worked with him in the "barn" and noticed some great things. I will, of course, write up a report that I will send to you, but wanted to give you some quick things that I noticed. First, I noticed that peripheral vision on left side seems somewhat delayed which could account for the bumps on the head most often on that side. [It amazes me that she said the same thing the neurologist told us about the spasms happening in the right side of the occipital lobe, where left vision is effected.]

Next, he appeared to be most interested in toys with lights when the overhead lights were off. In this unfamiliar environment of "the barn" he was not quite as willing to walk around like in his familiar classroom. That's okay and can work towards his benefit. Since he's not sure of new environments, this behavior will keep him safe....that smart boy has figured that out.

Also, I noticed a sudden "drop down" where he was standing and got on almost all fours in a very quick motion. I don't feel like this is balance, but him trying to figure out where he is in space and feeling most comfortable with this constant contact (think of being on a cliff and looking over, you would drop to all fours to keep a better sense of where you are...I'll explain this one in person).

When she called, she said that Cortical Visual Impairment is a spectrum and he appears to be on the better end, especially in that he is walking and many kids with this don't. She also told us that with intervention other parts of his brain can learn to compensate for his vision and we will see a big improvement.

She will also be referring Deacon to the Orientation and Mobility Specialist, who helps Deacon learn to move around safely. If he qualifies, he will be up to FOUR visitors each week! I can't even begin to say how completely grateful we have been to this program and the information and suggestions they have given us already.

Ladies & Lil Gents

Although our blog may appear like Carson has disappeared, I assure you he has not. :) To be perfectly honest, I don't know what I would do without him. I really believe that I would be institutionalised from the stress surrounding Deacon's future, if I didn't need to be strong for Carson. God plans accordingly for these types of things. I have no doubt that this is one of the millions of reasons God gave Carson to us: so that this momma didn't lose her mind when times get tough, because baths still need to be given, lunches need to be made, and laughter needs to be heard.

Last weekend Carson and I had a date that rocked, and we are still laughing about the fun we had.

Our city held a "Ladies & Lil Gents" dinner for moms and sons. My friend, Care (I put a link to her blog in hopes that she decides to EVER update it...) had posted on her facebook that she was going, and it looked like so much fun, I decided to get Carson and I tickets at the last minute too!


It was a western theme this year, and you would think that b/c we live in Texas, we should just have boots and cowboy hats sitting in our closet waiting to be worn...well think again. We improvised with what we had (ie. I wore a dress, and Carson had a button-down with cargo shorts and flip flops...).

Not only did the event have dinner and a dj, they also had a TON of activities geared towards the little guys! The frame pictured above was actually built by us!

They had a huge haystack filled with prizes to be found:

Carson dug up a sheriff's badge:

Popping balloons to find what team they were on for the next game:

...followed by a relay race around haystacks in cowboy attire:

and Limbo:

Carson's favorite activity was dancing!! From the moment we walked in the door, he was getting his groove on! And he has got some moves, too!

He was such a gentleman all night long and even opened doors for me! When we left he said that was the best date ever! (I will remind him of this when he is 16...)

2nd Opinion

What a difference a doctor makes! We didn't find out news that was any more optimistic, but we at least felt heard, informed, and not rushed.

Our new Epileptologist gave us insight on the reasoning behind decisions that have been made, and also had some completely different answers to some of our questions than the previous Epileptologist did.

First, they aren't 100% sure that it is Infantile Spasms. They appear to be because of the way his body startles when he has one, and the multi-focal spikes on the EEG, but they have not caught an actual spasm on the EEG. (We didn't even know this!) The muli-focal spikes are how his brain normally looks when not having the spasms (a pattern for "normal" brains are just waves, not the spikes). But, spasms typically come in clusters, not single spasms like he has, and usually show hypsarrhythmia on the EEG, which his hasn't (maybe b/c they haven't caught a spasm on the EEG??). She said that if it is Infantile Spasms, it isn't a typical case, and doesn't necessarily mean better or worse news...

If it isn't IS, it may be Myoclonic Seizures/Epilepsy, which from what she said, seems to have a similar prognosis as Infantile Spasms. She said the seizure's outward appearance is very similar to to IS, but the EEG looks different. (I tried to do research on this, but there are so many different types, it is hard to find definitive information for the general term...)

So, as of now, our main goal is to get a definite diagnosis from an EEG that actually catches a spasm in action. To do this, they will admit Deacon into the hospital for an EEG lasting 24 hours. It looks like the first opening they have is in April, but they will call this week to confirm a definite date.

Depending on the diagnosis, we will address treatment options. The reason they did not immediately put him on ACTH, is b/c of the absence of the hypsarrhythmia (hyps). She said it is protocol to use that only when hyps are present due to the small success rate in cases w/ no hyps compared to the terrible side effects from using the medicine. (Could the other dr. not have told us this??) For now, she is comfortable with the Topamax prescription we are on, and agrees with the increase in dosage.

She also ordered more labwork to be completed to look at his metabolism and make sure it is working properly; Quest told us it takes 4-6 weeks to get these results, as well as 6-8 weeks to get the genetic results we already had done.

The Epileptologist gave us more information on treatment options. She said about 33% of people with epilepsy do not respond to medication, and usually after 3 different medications are tried with no seizure elimination, the success rate is about 1% for additional prescription trials. Because Deacon took such a small dosage of the Keppra, she said that won't count as a trial, so the Topamax he is on now will be considered his first.

If we get to the point where we have tried 3 prescriptions that don't work, we will then discuss brain surgery. The majority of this problems are coming from the right part of the occipital lobe, so brain surgery would most likely entail removing that part of the brain. The removal would cause his left vision to be eliminated (right side of brain controls left side of body). Both eyes would still work, but he would have no vision in the left direction. The other 2nd-line treatment option for the seizures is the Ketogenic Diet (very similar to Adkins, but everything must be measured, weighed, and there is NO cheating for it to work). This is basically the last resort, if all else fails. It has a decent success rate, but is hard to follow, especially in small children due to no flexibility in meal options.

We already feel much better to be working with someone who is on the same page as us, and Deacon liked her too! Our next appointment with her depends completely on the results from all of the tests we have coming in.

See Us Back in 2 Months?!?

I feel like today's appointment was a waste of time...

The results from Deacon's 2nd EEG weren't even in the system, and very few results were back from his bloodwork. The doctor asked us the exact same questions the neurologist did at the last appointment regarding milestones and pregnancy history. Most questions we had were answered with "it is so different with every child," "I really can't say," or "we'll need to see test results."

The epileptologist said that b/c we have seen a decrease in spasms on the Topamax, that he would like us to increase his dosage from 30 mg/day to 60 mg/day over the next two weeks and see us back in 2 months!?!

Am I crazy, or does this seem too far??

I was thinking he would say "see you back in 2 weeks" not 2 MONTHS! When I questioned the length of time he told me that basically his schedule is full, and that if it is cryptogenic, as long as we can get the spasms down to 1 every few days, that they usually go away on there own anyways... That contradicts everything I have read online! I thought the whole goal was to get the spasms to STOP COMPLETELY!?!

He said if the Topamax doesn't work that in 2 months we will consider ACTH.

I don't feel like I am hard to please or demanding anything more than what another parent in my shoes would, but I am at a loss...I feel like we have been rushed through every appointment, and that there is no sense of urgency by the doctors to eliminate the spasms. I don't even know where to go from here?? I am totally okay with increasing the dosage to see if it works, but what if it doesn't?? We have to sit around and wait 2 months to try something else??

More Appointments

Whew! What an exhausting day! Brian, Deacon, and I spent the entire day at the hospital appointment-hopping from neuro-opthamologist, EEG, and lab work.

First appointment was with the eye specialist. They dilated Deacon's eyes and sent us to the waiting room, where we sat for almost 2 hours. Finally we were called back to see the doctor.

The good news: Deacon does NOT need glasses. Seriously. Deacon's prescription is +1.75 and she said that she typically doesn't even CONSIDER glasses for anything less than +3. She told us the near-sighted/far-sighted prescription he was given is a pretty typical one for children his age, just because the eye/vision hasn't completely developed for small children. Looks like I will be calling the eye doctor tomorrow for a refund on the $500 glasses.

The bad news: (At the moment I don't know if this is BAD news, but because it is not good news, it is going in the "bad" category.) Deacon was diagnosed with Cortical Visual Impairment due to his Infantile Spasms. This means that his eyes are healthy and work exactly as they should, but the brain (occipital lobe where we already knew there were issues) doesn't know how to process what is being seen.

Are you ready for your lesson of the day?? Here is more info regarding CVI:

Children with CVI frequently have fluctuating vision where their vision may be different at various times of the day. Their central vision may have spotted blind spots and their vision may be analogous to looking through Swiss cheese. Consequently, they often do not make eye contact and their eye hand coordination may be poor. Children with CVI often have many interesting behaviors. Aside from not making eye contact, they are highly stimulated by high contrast, rotating, and moving objects. They frequently look at ceiling fans, the scrolling credits on videos, and some stare at blinking and flashing lights. A small percentage are extremely sensitive to glare and bright light and will keep their eyes closed or turn away and stare into a corner of the room. Children with CVI frequently have very sensitive peripheral vision and they can become overwhelmed when they are in crowded places that have a lot of visual noise. Their visual attention fluctuates tremendously and they may demonstrate short periods where they use their central vision to focus and identify objects but their attention quickly weakens and they then glance at objects with their peripheral vision. During these times, they appear totally blind and use their hands to interact with their environment rather than using their vision to guide their hands.

The doctor gave us the impression that if we get his spasms under control, his vision should improve significantly. I think this is a little comforting to us just because he doesn't have a bunch of separate issues to tackle, but just one BIG obstacle for us to battle and overcome. And with spasm elimination Deacon's life will change a million times over. But for now, this diagnosis means he qualifies for visual therapy through Early Childhood Intervention, bringing him to a total of 3 therapy sessions each week.

Next came the EEG. Deacon was ready for a nap by this point, so it was perfect timing for his testing.

Deacon with all of his wires

his poor hair after plugs were taken off (the red spots are from the marker they used to mark wire placement)

After the EEG was bloodwork where they attempted to draw blood and finally got it right on the 3rd try. We didn't receive any results today for the lab work or EEG, but will hopefully find out a lot more on Monday with his Epileptologist appointment.

Deacon was amazing the entire day and Brian and I enjoyed spending the time with him and seeing the big improvements he has already made since being on his new medication. He is laughing, smiling, and making eye contact so much more, and is having about 4-7 spams a day! We are hoping that maybe just by increasing his current prescription he will be spasm free and won't need to try ACTH.