Wednesday, March 23, 2011

Be Very Careful...

This little tiny vial is a ten day supply of Acthar Gel (ACTH).  Our nurse handed this to Dan and said  "Be very careful with this, it cost $30,000."  WHAT!?!?!?!  We knew this was expensive, but actually holding this little vial between your thumb and index finger and hearing that is worth more than our cars was still a ridiculous thought.  There are two more vials arriving at our house tomorrow, which will complete a thirty day supply.  For those of you doing the math, that is $90,000 per month, and Will is going to be on this medicine for anywhere between two to five months.  Thank God - Thank God -  for good health insurance, because as we mentioned in a previous post our copay will be $40 per month, although we were warned that if we drop the vial (did we mention it is glass??) we have to pay for it ourselves.  Yikes!  Now, before anyone decides to break into our house to steal the ACTH, don't bother, there is no black market for a nasty injectable steroid that is pretty much only used to treat infantile spasms.

We checked into the Clinic again on Monday morning, 3/21.  Gabby (we love you!) rearranged her schedule so that she could be the one to wire Will up for the Video EEG.  Next, Will had to have an EKG to test if his heart would tolerate the ACTH.  He cried too much during the first one so they ended up having to repeat it a second time.  We practiced giving shots on a teddy bear, and then we got to practice giving each other a shot of saline solution in the arm so we could both know what it feels like to be on the giving end and on the receiving end.  Not so bad.  Actually, spend a few days in a tiny hospital room with your spouse and an active 20 month old who is wired to the wall...jabbing your spouse in the arm doesn't seem like a bad idea!  Just kidding...

By evening we were all going stir crazy, so we asked begged the Resident to give us a special pass to disconnect the main wire on the V-EEG so we could leave the room for a few minutes.  He agreed, so we took Will up to the Rooftop Pavilion and took in a pretty awesome view of downtown Cleveland.  Will was so happy to be able to run around up there and it was great that he got to burn off some energy.  We came back to the room and settled down for the night.

Tuesday morning, Will was literally awoken to a needle going into his arm to get a blood sample.  Not a good start to the day.  After being delayed from the last visit because he was sick, we finally went forward with the plan to begin the ACTH treatment.  After saying a few prayers, Dan gave the first shot, and everyone - parents and child - did pretty well.  Some of the side effects that have been worrying us include:  hypertension, suppressed immune system, severe weight gain, irritability, and what a lot of other parents who have been through this describe as "roid rage."  We didn't expect to see changes very quickly, but within a few hours, he started acting pretty hyper, walking around (as much as his ten foot cord would allow) and babbling more than usual.  On the bright side, he was very smiley and really gave us some good belly laughs when being tickled.  We got a visit from a medical student who plays music for the kids in the hospital, and Will enjoyed having someone in his room that didn't want to poke him or put a blood pressure cuff on him.  We also got a visit from Chaplain Laura and her seeing eye dog, which was a nice comfort to all of us, and again had the effect of putting Will right to sleep.

Kelly gave the shot on Wednesday 3/23, and all went well.  We were told we could go home a day early, so we started packing up, and they removed Will's EEG leads.  Of course, Dan was out taking the first load of things to the car when our nurse practitioner came into our room to let us know that they had found an "abnormality" on the EKG, and Will would need to have an Echocardiogram, which is an ultrasound of his heart, to find out more.  He was to be sedated, but miraculously, he fell asleep right before it was to be done, and somehow stayed asleep the entire time so there was no need for sedation.  Luckily, about an hour and a lot of prayers later the results came back that his heart is completely normal, which was a huge relief.  We're not sure we could handle having to worry about two major organs, worrying about his little brain is enough.

We finally got discharged and made it home around 7:00 tonight (Weds. 3/23), and Will was really happy to be back home to see Jack and Duke.  Jack learned some new jokes while we were gone from his grandparents, and couldn't want to tell them to us.   Tomorrow we have a home health aide who will come to our house to check Will's blood pressure, and she will be coming every 2 days for the duration of this treatment.  So far, we aren't seeing any results from the ACTH, just side effects.  In fact, if anything, he seems to be having more seizures and they seem to be getting more intense, but we were told that although sometimes results are seen fairly quickly, it could take up to 2 weeks to know if it is going to work.

Saturday, March 19, 2011

Counting Blessings

By the time we got home from the hospital last weekend, Will was pretty sick and had lost almost 3 pounds...which, when you consider he only weighs about 23 pounds to begin with, that is a lot!  Of course, soon after we got home, he was smiling and acting significantly better.  We aren't sure if he had a virus, or if he was just extremely stressed by life in a hospital.  Either way, he is back to normal, and we are going to be admitted again on Monday for approximately 3 days.  Let's hope our third try will be the charm for starting ACTH.  There is a large part of us that wonders why we want to do this, as the side effects and extreme risks of ACTH are terrifying.  Don't Google it.

We have had a lot of ups and downs this week, although that is starting to become par for the course around here.  Will continues to have an average of 5-6 clusters of seizures per day.  Some days are better than others.  This morning he had one in the bathtub, and although obviously we would never leave a 20 month old unattended in the tub with or without IS, it was pretty scary to watch his head plunge forward into the water.

As expected, the Keppra has done nothing to lessen Will's seizures, and Will's doctors aren't very optimistic that the ACTH will work either.  We find ourselves already trying to evaluate the next steps, even though we haven't even started this round of treatment yet.  We met with a pediatric opthalmologist on Tuesday, who explained to us the very real risks to Will's vision from Vigabatrin (the next drug option if ACTH fails) as well as from brain surgery because of the location of the optical nerve.  There are no easy solutions here, and the options we are given are forcing us to make choices between the lesser of 2 evils.  We simply don't have an option that isn't without significant risk, although when given the choice between potential damage to Will's vision (or possible brain atrophy from ACTH) compared to the strong likelihood of severe mental retardation if we can't get the seizures under control, the answer becomes pretty clear.  We have no choice but to do everything possible to stop the seizures. When we first read about so many of the treatment options for IS, especially ACTH, our initial reaction was "no, way, not our child", but as we come to grips with the seriousness of Will's condition, seemingly impossible choices become easier to make than you might think.

We can honestly say that we are hanging on by an absolute thread, but somehow we are still holding it together despite it all.  Life keeps going whether you feel like you want to continue on the ride or not...our children need us to maintain a normal, happy life for them, we have jobs that demand our attention and bills to pay.  Sometimes we amaze ourselves by talking to someone about Will's condition all matter-of-factly as if we are completely in control. Other days, it is hard to even put one foot in front of the other. We met with a specialist in pediatric neurodevelopmental disabilities this week who is going to help coordinate Will's therapies, and she said "This is your personal tsunami" - she clearly understood what we are going through.

Will wanted to type this blog update himself
Of course, we know things could be so much worse.  Spending a few days in a pediatric hospital makes you intensely aware of this.  We are blessed in so many ways.  We know we have said this before, but since the beginning of all of this, we have been absolutely blown away by the amazing generosity of our family and friends.  We have the most amazing friends and family!  Our list of thank you notes to be written has gotten overwhelming, and we are not sure that we are going to get to all of them in light of everything.  To everyone who has dropped off a meal for us, or even just offered to make one, helped take care of Jack during Will's many appointments, helped get Jack to and from school, neighbors who have plowed our driveway, friends who treated me to a spa day, those who have given special treats and presents for the boys, the ladies prayer group who knitted blankets for Jack and Will, co-workers who chipped in and got us gift cards for parking and meals at the Clinic, friends who sent us a note or card or even just a text, those who called to say they care, friends and strangers who have said a prayer for Will, or even those who just followed along on our blog...we are so grateful.  We are truly blessed to have such an amazing support network with us on this journey.

Saturday, March 12, 2011


The past few days have not gone exactly according to plan.  After Will was sedated on Thursday, he had a hard time recovering.  We think it was mostly because he hadn't had anything to eat or drink for so long, and his little body really got shocked from it.  Somehow in all of this, he picked up some sort of a virus, which has now put his treatment on hold once again.  We are packing up and getting ready to go home today, but we have not started ACTH, and we will likely be back at the hospital again in a about a week.

Will mostly cuddled and whimpered all day Friday, and just wasn't himself.  He was also very shaky, so much that he was unable to stand up, although the IV in his foot wasn't helping matters.  He was a generally unhappy little boy, nothing like the smiley little Buster Beans we are used to.  The doctors decided to postpone starting ACTH until Saturday, so we waited.  They did discuss the results of the PET scan with us, which basically confirmed the malformation that had been found on the MRI.  This was somewhat of a relief, as it means that he is still a candidate for surgery, and they didn't find anything unexpected.  The Dr. described the malformation as a "fuzzy spot" without clearly defined edges, making it difficult to know exactly where it starts and stops.  We don't know what this means in terms of how much of his brain might eventually have to be removed, as a lot of additional testing will have to be done by that time.

We got a visit yesterday from a woman who is a chaplain here at the Clinic.  She was blind and had a seeing eye dog with her, and thank God it was a black lab - our dog at home, Duke, is a black lab, and Will adores him.  When she came in, Dan was sitting on the floor with Will trying to get him to play with his toys, comfort him, get him to sleep, ANYTHING to get him happy.  The Lab came over and laid down next to Will on the floor.  Will must have felt like he was at home with Duke because he touched the dog's back a few times, grabbed a few handfuls of fur, then curled up on Dan's lap and was asleep within 5 minutes. The chaplain then sat and talked with us for about 20 minutes and she was great, very friendly and comforting.  She closed with a small prayer for us.  What a great visit!  We hope to see her and her dog next time we are here.

Dan's parents came down Friday night and brought dinner.  Will finally perked up while they were here and put down a lot of food, and we felt relieved that he was getting back to normal.  However, shortly after they left, he threw up everything he had eaten, and became miserable again.  He finally went to sleep after midnight.  At this point, we knew that beginning ACTH was not an option, and the doctors confirmed this during rounds this morning.

So, we are going home today.  As much as Dan and I love each other and our little Buster, we can't wait to not be in a 12x12 room tripping over each other, and we can't wait to see Jack.  We are just feeling completely exhausted and somewhat defeated.  Regardless, as much as we want to get on with this, the time is not right, and for some reason we aren't meant to start ACTH right now.  They are going to use this opportunity to try another drug, Keppra, in addition to the Topamax.  Keppra is administered orally in a liquid form, so it's an easy one to give him, and has relatively few side effects.  Right now, we are praying for patience, and for health for our little Buster so that we will be able to continue his treatment  soon.

Thursday, March 10, 2011

Necessary Steps

WOW, was today a rough one.  We tried to mentally prepare ourselves for everything that we were going to go through today, but we are completely drained.  Will slept pretty well last night, considering he was wired up to an EEG and in an unfamiliar environment.  Each room in this unit is supposed to press a button if the patient is having a seizure which sounds an alarm in the hallway so the staff can properly record and respond to the seizure.  Periodically throughout the night a loud BEEP BEEP BEEP would come from the hallway, followed by some commotion and the alarm would eventually silence, so sleep is challenging.

Will was awoken at 5:00 AM for his medication, then fell back asleep. At this point, Will had been on the EEG recording machine since we arrived yesterday afternoon, and still hadn't had a seizure.  Finally he had a minor one (about two minutes) around 9AM, then a pretty severe one (about seven minutes) at about 10:30.  Both were recorded and we got good data.  You never want to hope your child has a seizure, but we were glad that they were able to record the data before starting the ACTH.

At 11:00 he was taken to have an IV put in for the PET Scan.  He was not allowed to eat or drink anything (not even water) today so by this time he had been fasting for 13 hours, and was getting a little edgy.  They tried to get an IV into a vein and had a terrible time finding a good one.  After two failed attempts in his arm, they resorted to his left foot and got it, about a 30 minute process.  By the time they were done, he was terribly agitated, his voice was hoarse from screaming and his face very red and splotchy from fighting the pain.

Next, he had to have a short, 25 minute EEG study while remaining relatively still.  They then took him to administer the anesthesia, and conducted the PET scan, lumbar puncture (spinal tap), and blood draw.  We were taken to his room and were there when he woke up.  The look on his face was heart wrenching, but our tough little guy had done what had to be done.

Will under sedation
We returned to our room and Will finally got to eat and drink at about 4:00, 21 hours after his last meal.  Lots of visitors followed from the Clinic to make sure that Will was doing alright, and that WE were doing alright as well.  At around 6, Will was given the Pyridoxine injection, which took about 20 minutes.  By that point, he had pretty much had it, and he finally fell asleep for a little while.

Then, Grandpa Dan and Grammy came to see us, and Will was happy to see some familiar faces.  Even better - they weren't wearing labcoats or scrubs, as he is quickly realizing what it means when he sees a white coat.

Tomorrow should be a much slower day.  The nurses will give Will his first ACTH shot tomorrow, and then on Saturday we will have to give the injection ourselves.  Today we had to practice giving the shot on an orange...Kelly's hands were trembling even just doing that, but we know we need to be strong for Will.  Once we are comfortable with it, we will be discharged, which we think will be Saturday.

We should know within the next 2-3 weeks whether the ACTH is working or not.  If it does work we have five to six months of treatment, then step down to another drug, and so on.  If it doesn't work, we have two months of weaning from the initial shot, then move to the next option.  We still have a long road ahead of us.

We knew today would be a tough one, and now we are back in our room and Will is resting in his Mommy's arms.  Plenty of great people are watching out for us here at the Clinic, and we are getting constant support from our family and friends.  Thank you all!

Wednesday, March 9, 2011

Separated at Birth?

Will and Indiana Jones...separated at birth??

We think the resemblance is undeniable!

A Lot to be Positive About

It has already been a long week, and we're only halfway through!  Will was admitted at the Clinic earlier today to complete additional testing and to begin ACTH.  We will probably be here until Saturday.  As soon as we got checked in, Will was hooked up to the EEG lines.  It was actually a bit easier this time because at least we knew what to expect, and because we have an awesome nurse helping us (thanks Gabby - you are unbelievable!).  The EEG is recording his brain waves 24 hours a day, as well as taking an audio and video recording.  We have to be on our best behavior, as we are being taped too!  I told Dan, no picking, no scratching, and please, no breaking wind.  It is actually sort of crazy to watch the EEG waves change with Will's every movement.  We have a red button that we are to press each time he has a seizure, so that the nurses can be sure to see it on the screen.  Will is basically tethered to the wall, and he keeps trying to pull the gauze off his head which has caused them to have to re-wrap him.  As you can imagine, keeping a 19 month old in front of a camera and more or less in one place is a bit of a challenge!

Will eating his dinner
Tomorrow we start the day at 4AM for Will to get his medicine, and then he will not be able to eat or drink again until 1PM when he will get sedated for his PET scan and for the lumbar puncture (spinal tap) and they are going to do some more blood testing.  He will also get an injection of Pyridoxine (Vitamin B6), which has been used as a treatment for IS, with limited success.  The chances of it doing anything to help control Will's spasms are very low, but it is also very low risk so definitely worth trying.  Will is also going to get his first injection of ACTH tomorrow, and then they will teach us how to do it ourselves as he will need to get the injections daily once we are discharged.  When I look at Will's chubby little thigh, it breaks my heart to think of jamming a needle in it, but I know it is what we have to do to try to help him.

We have met with numerous doctors, nurses, and nurse practitioners who have all reviewed Will's chart, and it is very apparent that we are in the right place, where they have a great deal of experience in treating children with IS.  We have been told repeatedly that because Will's spasms are caused by an identified area of the brain, the likelihood that we will be able to stop his seizures with surgery is very promising, which is typically not an option for children, unlike Will, that have more generalized seizures.  That, coupled with the late the age of onset of Will's seizures and his relatively normal development thus far, we are trying to remain hopeful for the future.  One nurse practitioner told us "As devastating as his diagnosis is, you have a lot to be positive about."

Jack spent the weekend visiting his cousins a few weeks ago, and when he got back, I asked him if he missed me.  He said "I tried really hard to miss you mommy, but it just didn't work."  When we left today, he told me "This time, I am going to miss you mommy."  We are going to miss him too.  Luckily, he has lots of grandparents, aunts and uncles who are going to spoil him over the next few days!

I now have the blog set up for email subscriptions, so if you want to receive emails of updates, you just need to put your address into the "Subscribe by Email" box at the right of the blog.  You will receive an email that you need to click on to confirm your subscription, and that should be it.  Bear with me, I am very new to the world of "blogging" and not quite sure how all of this works!

It sounds like we have a very long day ahead of us tomorrow, please keep little Will in your prayers.

Wednesday, March 2, 2011

Searching for Direction

Last week we woke up to see all of the trees outside covered in the most beautiful blanket of ice.  Dan took this picture of the weathervane on the roof of our barn.

We have been amazed by the support we have received from our family, friends, and even total strangers.  Within days after creating this site, Buster's Blog had logged almost 800 hits, and this is from a non-searchable blog!  Hopefully, everyone can excuse the typos, and misspelling of medical terms.  It is crazy for us to think that so many people might be reading this.

Will came down with a virus over the weekend.  He was a pretty sick little boy, but finally turned the corner yesterday.  Judging by the kids at Jack's preschool, we think just about every child in town has been sick lately!  Unfortunately for Will, that is causing some delays in his treatment.

The Dr. made the determination that Will has failed Topamax as there isn't really any change in his episodes, and we are going to be beginning ACTH.  He was to be admitted to start the tests and treatment yet this week, but because of the virus we need to wait until he has been healthy for at least a week as the ACTH will suppress his immune system.  Thus, for now, Will is still taking the Topamax for another week.  We were hoping to be able to get together with some friends this weekend before we have to go into semi-isolation, but we had to cancel as we need to do our best to keep him healthy so that his treatment doesn't have to get delayed any further.

ACTH, or adrenocorticotropic hormone, is a steroid injection treatment (it is kind of similar to Prednisone) which is sometimes effective for treatment of IS for reasons that doctors are not able to explain.  This side effects are daunting, but we have read posts from many parents referring to ACTH as a "Miracle Drug".  The cost of a course of treatment (approximately 2-4 months) is close to $100,000.  No, that is NOT a typo.  Because of its extreme cost, it is one of only 12 medications in existence for which the National Organization of Rare Diseases provides medical assistance.  Amazingly, we have been told that our co-pay for this will only be $40 per month.  Thank God for good health insurance!

We thought it might be helpful to post a little more information about Infantile Spasms (IS).  It is ironic because the seizures seem so mild and the name "Infantile Spasms" is so benign.  Yet, it is one of, if not the worst type of epilepsy that a child can have because of the negative effects IS can have on a child's development.  IS is a rare type of childhood epilepsy, affecting approximately 1 in every 5000 children.  Almost all cases of IS (90%) begin prior to 12 months of age, and most (but not all!) children who suffer from IS will have developmental delays or mental retardation later in life.  Will actually has what is termed late-onset Infantile Spasms, because he did not have his first cluster until he was almost 18 months old.  In fact, Will was developing completely normally until around 15-16 months, when we started to worry because although he babbles he still hadn't said his first word.  The prognosis is significantly better if a child was developing normally before the onset of the seizures, and we have to believe that since Will had almost a year and a half of normal development his chances are better than most.  Unfortunately, the one question we so desperately want an answer to - "Is our child going to be OK?" - is the one question that no one can answer for us right now.