Sunday, April 24, 2011

One Strong Little Spirit

Will didn't have to get a shot yesterday!  Since we are starting the ACTH wean, we have switched to every other day dosing on that medication.  This is awesome because it means that we are one step closer to being done with ACTH, which has some nasty side effects.  We will slowly wean over the next 3 weeks, but at least for that medication, there is an end in sight.

This also meant that we were able to enjoy a much needed night out without the kids.  My birthday is coming up at the end of the month, so Dan surprised me by arranging for my parents to watch the boys overnight so we could go out to dinner and have a night off.  Since Will didn't need to get his shot or blood pressure read in the morning, we were able to just pack up all his medications and let the boys spend the night with Grandma and Grandpa.  We felt a little guilty leaving our boys behind given all that is going on right now, but it was really refreshing to get away, and the two of us really needed to escape for a little while.  The last time we went out without the kids was on December 31, a few days before Will had his first seizure.

This was all on the heels of a difficult week.  On Tuesday (4/19) we took Will for a routine eye exam, which is required within 30 days of starting Vigabatrin, to get a baseline so that we can evaluate if he suffers any vision loss from the medication.  Will just had a full eye exam in March, so we really weren't at all concerned about this appointment.  We even brought Jack with us.  As the doctor examined Will, the doctor paused for a moment and said that he noticed a slight paleness or atrophy to Will's right optic nerve (on a scale of 1 to 10 he said it was about a 2 or 3).  What we thought would be a quick appointment ended up lasting over 3 hours.  Another doctor was brought in, and he concurred.  We were shocked to find this out, as Vigabatrin causes retinal damage, but this was optic nerve damage, therefore not related to the medicine, but perhaps somehow related to the seizures.  His is mild, but if it progresses the result would be total blindness.  Thankfully, right now it is only in one eye.  We will be going back to the Clinic to meet with our metabolic neurologist soon for additional testing to see if we can figure out why this happened, and if we can prevent further damage.

We got a special phone call earlier in the week.  The pastor at our church asked if Will would be one of the "disciples" at the Holy Thursday services, and get his feet washed.  We were a bit nervous because of his immune suppression, but Will waited in the car with his grandparents until it was time, and then we just took him straight up to the front of the church for the feet washing, not letting him touch anything, and whisked him back out to the car as soon as the feet washing was over.  It was awesome to get to be a part of the service, and to feel the blessing of the entire congregation upon Will!  Jack got to come up to the altar with us, which was great because we didn't want him to feel left out.

So far, we are not seeing any real reduction in seizures from the Vigabatrin, they have actually been back in full force for a little while now.  He just reached his target dose, so we won't really know if it is working for another week or two.  He is extremely hyper right now, just chasing him around the house is exhausting!  The amazing thing is that despite the seizures we are seeing some positive changes in Will.  Since starting the Vigabatrin, we are seeing glimpses of our happy little boy and there is often a sparkle in his eyes that we haven't seen in a while.  This is truly awesome to see!  Although he is still in a bit of a fog, he is interacting with us again, and responding to simple things like his name and the word "no" - of course, only when he is in the mood, as he is a toddler after all!  It is great to hear the sound of his laughter again.  When we look at the list of anti-epileptic medications Will is on - Topamax, Keppra, ACTH and Vigabatrin - it is hard to believe that he can even be alert.  His little spirit is so amazingly strong.  We hope that you and your families enjoyed a blessed Easter!

Thursday, April 14, 2011

A Delicate Balance

Will at the Infinity Fountain outside the Clinic
We spent most of this week in the hospital with Will...again.  Initially, the reason for the admission was to evaluate the effects of the ACTH.  It seemed to be working, although there was still some seizure activity, so the plan upon admission was to monitor Will's brain wave activity and to look at increasing the dose of ACTH in an effort to achieve a normal EEG.  However, once test results came back, Will's treatment plan took a detour.

Will was admitted on Monday (4/11) for additional testing and video EEG monitoring following his neuropsychological evaluation.  During rounds the next morning, the doctors discussed the plan to increase the ACTH back to his previous dose, and in anticipation of this, Will was scheduled to have a renal ultrasound, another EKG and echocardiogram on Wednesday.  We added another specialist to our team, a nephrologist which is a specialist in kidney function, in addition to our neurologist and cardiologist.  Will's blood pressure ran dangerously high at the previous dose of ACTH, and deciding to go back to that dose was a scary proposition.  We needed to be sure that his kidneys and heart looked good, and as long as we could get everyone on board the plan was to increase the dose of ACTH with extremely careful monitoring and with the addition of medicine to control his blood pressure.  This meant that we would likely be inpatient for several more days.  Another delicate balance of risk and reward.

The renal ultrasound went well, and we got word that Will's kidney function looked great.  Next, we needed to get the tests run on his heart.  We were waiting for transport to take us down to the where the scans would take place, when we got word that those tests had been cancelled, and we were given a brochure on the next drug option, Vigabatrin.  Our hearts absolutely sunk. We felt physically ill to realize that he had failed yet another drug, especially one that we thought was working.

We were told that the data collected from the video EEG revealed a few things.  First, ACTH was not actually working, but was basically just masking his symptoms.  The activity we have been seeing was definitely seizures, and not just any type of seizures, but the dreaded infantile spasms.  Second, the focus of Will's seizures seems to be slowing.  Essentially, what this means is that a portion of the seizures seem to be originating from multiple areas of Will's brain, and not just from the left rear quadrant.  This also means that brain surgery to remove a portion of Will's brain, while it may improve the spasms, is less likely to eliminate the seizures than we had originally thought.  With infantile spasms, unless the seizures are completely eliminated, the prognosis for mental development is not very good, so the goal is total elimination and not just improvement.  Will's surgical review board is scheduled for next month, so we will know more as to the Clinic's recommendation on surgery at that time.  We are also hoping that getting the 2nd opinion in Detroit (and if necessary, a third or even fourth opinion elsewhere...) will help us to make the right decision.

We are, as always, happy to be home again.  Jack, at age 4, is struggling to deal with all of this, and we miss him terribly when we are away.  As we tucked him in to bed last week, he asked, "Mommy...what is ep-i-lep-sy?"  No matter how hard we try to protect him from all that is happening, he is more aware of everything that is going on than we think.

Tomorrow we are starting Will's first dose of Vigabatrin.  We have been uncomfortable with the idea of trying this drug because it carries with it a rather significant risk of permanent loss of peripheral vision.  The drug facts list the risk as 1 in 3, although anecdotal evidence shows the risk to be less in children.  We are running low on options, and we don't really have a choice.  At this point, Vigabatrin is less of a risk for him than ACTH, and the doctors feel that the chances of Vigabatrin working is almost equal to ACTH, and we can go to a higher dose with less risk.  For a few weeks, he will be on 4 very strong medications - ACTH, Vigabatrin, Keppra and Topamax, all of which have significant side effects.  When you combine this with the effects of the infantile spasms, Will's brain development has basically been on pause for months.  Although, we can be thankful that his brain development has not regressed very much either. 

Thankfully, we will be weaning ACTH and Topamax in about a month.  Even though we have only been dealing with immune suppression for about a month, living in isolation and not being able to go anywhere, the constant blood pressure checks, and the needle in his little thigh every morning is definitely wearing on us all physically and emotionally.   In this respect we will be glad when Will is done with ACTH.  We are also hoping that weaning some of the medications will help to get our little boy's personality back, although Will has amazed us with the way his spirit still shines through despite all of his medications and his disorder.

Saturday, April 9, 2011

At the Crossroad

Spring has arrived, and for what seems like the first time since this began, we have some good news to share!  Will's EEG (a test which measures brain wave activity) on Thursday showed "significant improvement." It still doesn't look normal, but it looks much better than what it looked like prior to beginning ACTH.  None of the doctors expected to see this, and Will's neurologist told us that at this moment, she was excited.  Since his dose of ACTH was decreased last week, we are also starting to see his smile again, which is a wonderful thing.

Will is still having what we believe is a modified type of seizure, but since the EEG on Thursday was only for 75 minutes, they weren't able to detect any of these episodes during the test.  So, Will is going to be readmitted to the hospital on Monday for what will likely be two nights, where he will be connected to the 24/7 video EEG so that they can continue to monitor him and hopefully provide us with more answers as to what is going on.  As much as we hate being inpatient at the hospital, we are actually glad that they are going to be gathering more data.  Plus, we have been there almost every day this week anyway, may as well have a room to call our own.

We are nervous to even type this for fear it won't stick, but Will has not had a "head drop" flexor spasm in 6 days.  He is still not "seizure free", but we will take any blessings we can get.  We are basically standing at a crossroad right now.  There is a chance that the seizure activity and abnormalities in his brain waves we are still seeing right now will continue to improve from the ACTH.  However, this is also a chance it won't especially because we had to reduce the dose because of his blood pressure.

We have been joking that Will is on the prayer list at every church in the area, which we love because it is awesome to know that so many people of all religions are praying for our son.  If you believe in the power of prayer, keep them coming!  Since ACTH is a drug that a child cannot remain on indefinitely, we need prayers that the spasms will not come back when we wean the drug.  We need prayers that Will's blood pressure, which continues to run very high, won't spike again so he can finish this course of medication.  We need prayers that his EEG brain wave pattern will continue to show improvement to more closely resemble a normal EEG.  We need prayers that the modified seizures we are seeing right now will go away.  Finally, we need prayers that we have the patience to survive 3 more days inpatient with Will hooked up to the wall again...would it be out of line to ask if they allow alcoholic beverages in the epilepsy monitoring unit?

Tuesday, April 5, 2011

The Sweetest Smiles

Things have been a little tough around here, and yet, we know that things could also be so much worse.  We are now 14 days into Will's ACTH treatment, which means we are beyond the time frame for when we should be seeing results. So far, the ACTH doesn't seem to be working although it is actually difficult for us to tell.  We are seeing a reduction in the "head-drops" associated with his spasms, but he is still having seizure type episodes several times a day.  We think that only his next EEG will tell if there is any real improvement.

Will has not tolerated the side effects of the ACTH very well.  He is extremely irritable, and his smiles are becoming few and far between, although when we do get one of his precious smiles, it makes it all the more sweet.  His little legs are starting to look like pincushions.  Almost immediately after we got home from the hospital after beginning treatment, Will began having extreme intestinal distress and wasn't able to keep anything down, which meant for a lot of long days and sleepless nights.  That started to improve after a few days, which was a relief.  Then, over the past few days, his blood pressure began to spike.  The first elevated reading was on Friday, and it has pretty much gotten worse from there.  High blood pressure is a common side effect of ACTH, but his went higher than expected.  We had another reading taken today, and after consultation with Will's neurologist, cardiologist, and our amazing pediatrician (we are assembling quite the team of specialists) the decision was made to decrease his dose of ACTH starting tomorrow.  For now, his little heart just can't handle it.

At the same time, the doctors are preparing for Will's epilepsy review board panel.  Prior to any pediatric brain surgery at the Clinic, each patient's case is first presented to a panel of neurologists, neurosurgeons, and others at the Clinic to make sure that the best course of action is being taken.  We were told that for Will's review board there will be close to 40 people in the room.  It is awesome to think that so much brain power will be focusing on helping our son!

We have also made the decision to travel to Detroit later this month for a second opinion with a doctor there who is well known for treatment of infantile spasms, particularly for surgical candidates.  We seem to be getting close to making choices and decisions on brain surgery, and we want to make sure that we are doing all that we can for little Buster.  It is sort of ironic because prior to Will's diagnosis, we were in the process of trying to plan a romantic getaway for our upcoming 10th anniversary, but now it looks like our exotic vacation will instead be a trip to a hospital in Detroit...why can't the leading experts on infantile spasms practice on some tropical island somewhere?

Will has a lot of appointments scheduled in the next week.  He now has a standing appointment at his pediatrician's office every morning to check his vital signs.  They let us in through a side entrance and take us directly to a well child exam room because of his suppressed immune system, although we are hoping to be able to go back to the home health visits once they get his blood pressure under control.  Over the next few days, Will also has another MRI scheduled with sedation, an EEG, and a neuropsychological exam.  They are also going to do some additional DNA/chromosomal testing as well.  We are hoping that with all these tests, we will have more answers soon.