I cannot believe it is the end 2012.  This has been an eventful year.  It honestly feels like 5 years have passed since William’s diagnosis instead of 7 months.  

We had the time of our lives this month in Orlando for our Make-a-wish trip.  We flew and visited all parks including  Disney World, Sea World and Universal.  We were going non-stop, and William was such a trooper!  He was always ready to go and take everything in.  It was a great family bonding experience that we will never forget, and we have tons of pictures to remind us as well.

We had a great Christmas and went to Macon and Columbus to see family. We want to make use of every opportunity we have to visit with family and let everyone see William.

Christmas or any holiday/birthday is hard for us because even though we try, we can’t help but think about next year and how our family will be faring.  It is also hard because we don’t fit into the normal mold. William doesn’t play with toys or understand a lot, and when people haven’t seen him or don’t know the full situation, it is hard to see their expressions.  He changes so much if you haven’t seen him in a month, he has, more than likely, lost a skill.  I would describe it as he is returning to infancy.  A baby grows and develops so much during the first year and gains so many skills.  Well, William is losing his skills as quickly as most children gain them. This is the most painful part of the disease as those who love him have to sit by and watch our highly spirited boy lose parts of his personality and motor skills.  Every step down or every skill lost triggers a grieving response.

It is really hard for anyone to understand what our lives are like on a daily basis unless they are living it. With this disease, it strips the child of every motor skill they have until they are just living in a vegetative state.  We may not have a lot now, but things will get worse, and we need to be grateful that William is still able to see a little and is still trying to walk.  We are also grateful for every smile he gives.

This has given new meaning to the expression, “Live life one day at time for tomorrow has enough troubles of its own.”  It is our motto and our goal.

I didn’t want this post to be a depressing one, but an honest one, and wanted to let you in on a little of what we are dealing with in the physical and emotional realm of this disease.

Some of you have been asking how you can help. An account has been created to help with William's medical expenses, and this is an easy way to contribute using PayPal or credit card.

Update from St. Louis

My emotions are raw right now so I probably shouldn’t be writing this.  We had our 48-hour EEG at St. Louis Children’s Hospital , but it was stopped at 30 hours. The doctors saw some break-through seizures, but they were not significant enough to keep us the entire 48 hours. The EEG showed William having seizures without any outward signs of distress.  Meaning he is having them without anyone knowing.

We were told that his vision has decline significantly.  He could lose his vision in three to six months.  We were also told that he may not regain his ability to walk. The progression of the disease is variable, and on average, children at this stage go down quickly with regards to mobility.  This was a very hard trip!!  

I went to St. Louis hoping the doctors would give us something to help the tremors and improve his mobility.  I was not prepared for the disease to be striking us so quickly.  William was just diagnosed in May.   Seven months later, he is growing immobile and losing his truck support, meaning he is losing the ability to sit up by himself.

I am glad we went ahead and placed the gastric tube.  At the time I thought we were being proactive, but probably, we would have to make that decision sooner rather than later.   The tube has simplified giving him his medications which before would have taken up the majority of the day.

I am very grateful that we are going to Disney next week, especially after hearing this news about William’s vision.  Time is not on our side.

We are gaining strength through all the prayers and concerns people have been sending.  They touch our hearts so much.  I also know that there are many other Batten parents out there doing the same thing, and if they can manage, so can I.

God will give me the strength to handle this situation.

November has really been a trying month.  William IS sleeping better these days, and that’s a blessing.

 

William now has a g-tube (gastric tube).  He is still eating, but is getting his medications and extra nutrients through the tube.  It sure is convenient.  He underwent surgery on Monday and was out of the hospital Tuesday afternoon.  The doctor cleared us to travel “over the mountain and through the woods to Grandmother’s house” Wednesday.  William slept almost the entire time we were in Mississippi.  As he wasn’t getting up much, he became really congested.  We were concerned it might turn into pneumonia, but once he started sitting up, the congestion got better. 

The doctors told us that if he was weak going into the surgery, it would take longer to get back to normal afterwards.  I would say William was a little weak before surgery.  He had a virus which lasted 1 ½ weeks, then an ear infection, and was really shaky which we thought was increasing seizures.  He is getting better slowly.  He went to school today for the first time in about 2 weeks.  He is awake more and is smiling more and more.  His primary care physician gave him an all clear on his ear and the congestion, but he is still not walking.  The longer he is off his feet, the weaker his legs are getting.  We need to make sure he is feeling good for his upcoming trip to Florida.

William has been approved by Make a Wish, and our family will be going to Orlando in December.  We will be visiting Disney World, Sea World, Universal Studios and staying at Give Kids the World Village (http://www.gktw.org/).  I know we are going to have fun, but just getting everything together is going to be a job.  It will be William’s first flight, and I pray we won’t have a 5+ hour layover! 

In January, we will head back to St. Louis for another 48-hour videoed EEG.  We are hoping to get his seizures under control.  But, in the meantime, we will be trying different drugs and combinations.  Hopefully, something will work better than the others.

God has a plan and we are along for the ride. 

(Just an unrelated tidbit, William has been great at the doctor's offices and hospital lately.  He doesn't cry or throw a fit, etc.  He let the nurse take out his stiches and didn't even whimper.  This is a big departure from his behavior a couple of months ago.  I'm not sure if this is bad or good, but it sure is less stressful!)

 For the past week, William has been a little more unstable than usual, and it’s apparent that his seizure activity is increasing.  This was expected because, over time, his body becomes adjusted to one of his current medications.  His St. Louis neurologist prescribed a new drug that has been approved in the U.S. since January.  Evidently, the dosage prescribed was too high for him because it really knocked him for a loop.  Trying to get the right combinations and amounts is really tricky, especially on the phone, and William has been having a hard time adjusting (his parents too!).   He is doing better, we think, but still there is more tweaking to do.

William was scheduled for surgery today, but it was postponed because the surgeon had a family emergency.  We are getting a Mic-Key button, a low profile skin-level feeding tube inserted.   It’s mainly for active lifestyles allowing children to be more mobile while ensuring their nutritional health.  At this point, William can eat and swallow, but he isn’t getting the nutrition or fluids he needs.  With the tube, he will get what he needs in addition to eating what he wants.  Hopefully, he will gain some weight and strength.  The surgery is rescheduled for Monday, November 19. 

It’s funny how things work out.  William woke up today with sinus drainage and acted like he felt bad.  His primary care doctor diagnosed an ear infection and gave him antibiotics.  Now I’m glad he didn’t have surgery today.  Hopefully, he will be better by next week. 

All in all, this has been a week.  It’s been hard to see William not being able to walk at all on his own, but not wanting to slow down.  In that state, he requires 100% attention.   I’m ready for “normal,” whatever that is anymore.

Your concern, thoughts and prayers really do help.  Thank you!

The moment we admit our weakness and need for him God begins to move on our behalf by opening doors of hope. If we become fixated on our own feelings, then we are unable to comfort our children when they most need us. But, especially as Christians, we must get our eyes off of ourselves and onto God. Life’s sorrows and challenges always provide opportunities for us to grow and mature, not just emotionally, but spiritually as well.

John (9:3 2)   “Rabbi who sinned, this man or his parents, that he would be born blind? Jesus answered neither, but it was so that the works of God might be displayed in him.”

When we focus on Him instead of on our circumstances, He is glorified, and we are strengthened so we can endure the painful journey we have been called to bear. God has a plan for the adversity we face. He will carry us through this adversity or difficulty, and we do not have to be fearful. There are times that God will call on us to endure hardships beyond a point that we think is necessary or effective. He is the person who wants to accomplish in our lives. He calls us to trust him even if our circumstances do not make sense.

When Your Children Hurt

Charles Stanley



William is not a sickly child, but he got the virus that was going around last week and wouldn't eat or drink.  He had to get an IV at the hospital, but from there he recovered quickly.  He has had a "swallow test," and it showed he was swallowing normally - which was good.  However, he is a stubborn little bugger and will only eat and drink when it suits him.  You can't get it down him if he doesn't want it.

He finding life funny these days.  You just wonder what's going on in his head.  He will just be sitting down and will start laughing.  His laughter is very contagious and everyone laughs back.  You would think that was his motivation, but he finds things funny when he is alone too.  It's so good to hear him laugh! 

William attends ACCESS in Little Rock.  The facility and staff are excellent, and he LOVES it.   We are lucky to have him there.  For a child who doesn't talk, he sure can communicate.  He lets his wishes be known!  During the last week, he started climbing on things and taking an interest in things like he hasn't done in months.  Once he climbs up, he can't get down, so you have to stay diligent to keep him protected.

Just about time you think you have everything worked out, things change.  Just part of the game I guess.  His meds are always changing and being tweeked.  Seems like we are giving him something all the time.  As long the meds control his symptoms, we are OK.

An update on William and our family:

William is maintaining these days which is good news. Currently, his vision seems the worst impacted which we are getting tested later this month. Funny how things change: I am not concerned with William gaining skills anymore. Now I am concentrated on his not losing the skills he currently does have. We can still walk for which I am grateful.  He is unsteady and falls quite easily but still walks and usually wants to hold your hand when you are outside of the home. I am not sure if it is his vision, balance or both that cause him to feel unsure but it is a change from the child who would escape from the house every chance he got.

There are a lot of things swirling around this head of mine. God’s will has been a big one. I do not believe that God wishes evil to occur. God does not will the death of innocent children, but in a world where suffering and tragedy will occur, He uses these things when they are placed in His hands. “All things work together for good for those who love God, who are called according to his purpose.” (Romans 8:28)

God not only promises to walk with us, but God promises to walk through us to come to the aid of others in need. God uses people to accomplish his goal. We are his hands and feet. If we listen to the nudges of God’s spirit and pay attention, we will find others being used by God to answer our prayers. Maybe we will also become aware of the ways God seeks to use us to answer the prayers of others. The key is paying attention and listening. I pray that I will be aware of things and that this PAIN will not be wasted but will glorify his name.

Continuing with Batten

We enjoyed a family vacation last month.   It was William's first trip to the beach, and he enjoyed it.

The results of the his St. Louis eye exam (ERG) indicated moderate rod and cone dystrophy, a part of the Batten disorder. We were hoping that the sight loss would be mild, but I suppose moderate is better than severe.   

William has been accepted by Make a Wish Foundation for a trip to Disney World. We will be visited soon by the Little Rock representative.

Also, we are facing having to make a major decision, and need peace that we are making the “right” choice. We need your prayers! Thanks!!

Here is an update for those wanting to know how things are going for the Braddocks.  We are hanging in there.  William is still the same.  Sleep is almost non-existant, and we are trying different medicines for his increase in tremors and seizures.

There are two things that we know for sure about life. There will always be change. And…there will always be hope. Sometimes it can be anticipated and even controlled, and often it cannot. Sometimes we are startled by its suddenness, and other times it is so gradual that it takes a lifetime to see.

Hope is innate. Whether we realize it or not, we evaluate change and we either comfortably or uncomfortably embrace it and, once we do, almost immediately hope enters the picture. We pull ourselves up by the bootstraps. We reinvent ourselves.

We catch ourselves mulling over the ‘what ifs,’ and the current status of it all just kind of gets us.  However, we are trying to live in the present as each day is truly a NEW day, and we’re going to live it to the fullest!  We realize that we are truly blessed in so many ways.

Hope is the anchor of the soul.      Hebrews 6: 19

Thank you for prayers.

There is nothing more to report except we are struggling to cope with this diagnosis.  We are uplifted and touched by the number of people praying for us.  Thank you and please continue.

Diagnosis

This is a hard blog to write, but here it goes.

We got a diagnosis from our St. Louis doctors, and it’s about as bad as we could imagine. William has Neuronal Ceroid Lipofuscinoses or more commonly known as Late Infantile Batten Disease, http://www.bdsra.org/.   He is getting genetic testing to verify this diagnosis Friday, but the results won’t be back for several weeks. We have little hope that it’s a misdiagnosis because we feel the doctors wouldn’t have given us this devastating news without being sure.  

There are about 200-300 children in the U.S. with this horrendous disease and 1,200 in the world. It’s classified as an “orphan disease” because it’s not economically feasible to find a cure for only 1,200 kids. There is a clinical trial at Cornell University Hospital in New York that is an option, but we have time to think about that because they need the genetic testing results to accept a patient. The research involves injecting stem cells into the brain, and isn’t a cure.   It might slow down the progression somewhat, but that’s all.

We really appreciate your concern and prayers! As you can imagine, we are having trouble dealing with this, and find it hard to talk, or even think, about it without crying.   We are trying to take this one day at a time and even that seems overwhelming!

We will keep you updated through William’s journey.  Please keep us in your prayers!!

William's First Trip to St. Louis Children's Hospital

William is an active 3- year old with some developmental delay.  At 2 ½ years we got a diagnosis of mild PDD-NOS (on the Autism Spectrum).  He isn’t talking much (maybe 10 words), but has always been above average on physical milestones (until lately).  William also has a sleep disorder that makes the household tired and sleepy!  He has been going to sleep with little trouble, but he rarely sleeps longer than 5 hours at a time.  Usually, at about 1am, he is up for the night!  He has been in therapies since he was about a year and a half, but they haven’t seemed to help.

We started noticing tremors in his hands while he was trying use a spoon within the past 3-4 months. Then it progressed to his legs.

Not long after that, William stopped walking all together. He would just crawl and we took him to the ER for evaluation.  Nothing was found to cause his inability to walk, but it lasted about three days.  After this episode, William progressively got worse with balance, falling, tremors, possible seizures. 

He was prescribed Keppra (an anti-seizure med).  It doesn’t take away the tremors but it may help contain them?  Tremors and jerkiness with unsteady gait and unbalanced motor skills are worse when tired or trying to mentally accomplish a task, for example stacking blocks. And then there are many times his legs just give and he falls.  This is progressing while he is at rest. 

William’s pediatric neurologist in Arkansas suggested a motion disorder specialist at St. Louis Children’s Hospital, Dr. Barry Schlagger.  Dr. Schlagger gave us an appointment but wanted William to have an in-patient 48-hour videoed EEG.  The doctor wanted the test results before the scheduled office visit at the end of May. 

William was admitted to St. Louis Children’s Hospital on Monday, March 26.  The leads were attached to his head (not fun!) and the wires ran into a backpack which was attached with a line to the wall.  It wasn’t as bad as we expected, as William got used to his head being wrapped and the test lasted only a little over 24 hours.  They got the information they needed and stopped the test.  An MRI was scheduled on Tuesday.  It was a feat to get this scheduled so quickly and required some string- pulling, but Dr. Schlagger made it happen.  The MRI showed he was having electrical spikes in his brain, but these weren’t related to the episodes of falling.

While he was sedated for the MRI, they did a lumbar puncture and took a good amount of blood.  We really appreciated that they put him out with gas before inserting the IV!  They sent some of the testing to Seattle, some to Emory, and some to other places that I can’t remember.  They are doing a broad spectrum of tests and maximizing the hospitalization and trip. 

They brought a team of physicians together to review/evaluate William.  This team approach reminded me of “House,” but thankfully Dr. Schlagger is nicer!  The head of many departments had their “go” at William.  The epilepsy neurologist ruled out that he was having seizures, but said he might or might not have some in the future.  (We thought that was definitive!  NOT!)  The Ophthalmology Department did an eye exam while William was sedated and thought they found something.  We met with the head of that department and scheduled another test in May when we are there to see Dr. Schlagger. 

I can’t really count the number of doctors who saw William, but I was thankful for every one of them.  Someone cared enough to arrange all this!!  You just can’t imagine how much that means to us. 

They put William on Klonopin in addition to Keppra.  It does seem to help with his tremors/shakiness, and they tried to get the dosage regulated before we left the hospital.  The new medication also helped him sleep through the night, and there was a glimmer of hope.  However, the doctors busted the bubble when they said his body would get used to the new med, and it wouldn’t affect his sleeping before long.

 I don’t think we are going to get a definite diagnosis before all the tests come back, but we did get a bunch of stuff ruled out.  That’s good.  This is a process, but for once we feel we are on the right track. 

We are on our way back to Arkansas with a good feeling about the experience!

Please keep us in your prayers.

 William and Braley right after he got the leads on his head.  He was TRAUMATIZED!!

He got used to the situation before long, though.  PawPaw really came through as a distraction!