Great Leaps - and Steps!

Seth completed his last dosage of Vigrabatrin on Monday, October 20th. It has only been two days off the medication, but he's doing great. Our neurologist seemed fairly confident that he would not regress to having more seizures after weaning him. While I know deep down that this is true, it doesn't keep the anxious mommy at bay. I have found myself getting more and more nervous each day that any little tic he has might be the return of the spasms. I am guessing that this will continue for a few months before I finally come to the conclusion that everything is going to be fine.

Seth has made a few leaps in his developmental milestones since the last post. He is now walking (up to 16 steps at a time!), can chew soft solid foods like bananas and carrots, and likes to make a "h-sh" sound.

As mentioned previously, Seth's neurologist referred us to ECI (Early Childhood Interventions) to see if he would qualify for any services. We met last week with our case manager to answer background questions. Today our case manager and physical therapist came out to do an evaluation on him. The evaluation encompasses a wide range of skills and takes about an hour and a half (at least in our case) to complete. The areas Seth was tested on today included: personal-social, adaptive, fine and gross motor, communication, and cognitive abilities. Our case manager and physical therapist ran through a battery of tests with him and asked us questions to decide his score in each area. As there were quite a lot of areas tested, I had a hard time keeping up with some of the scoring, but I will do my best to share what I retained.

In personal-social skills, Seth scored at 13 months. We were told that he is interacting quite well with those around him, so there was no deficit in this area. Fine and gross motor skills were averaged together for a total score of 11 months. He had a slight deficit in fine motor skills due to his "messy" pincer grasp. Since he is already walking, that put him ahead of the curve in gross motor skills. However, he will be receiving PT services to help with his balance and strength while standing and crawling. We have been given a goal to help Seth learn to crawl rather than hop. The other two goals were for him to be able to stand up without pulling up onto anything and to walk at least ten feet. Both of these goals should be met by January.

Seth scored at 9 1/2 months in communication. His receptive communication was above average at 11 months, but his expressive communication scored at 8 months. Again, both were averaged together. Since Seth understands certain commands such as "no; stop; come here", he scored high in receptive communication. He still does not copy sounds that we make, so this brought his expressive score down. He has a goal to begin producing consonant sounds and waving hi and bye. For his cognitive abilities, he scored at 8 months simply because he has not mastered object permanence yet. Josh and I have been working on this skill since he was six months old with no success, so we are anxious to be given ideas to help him master this skill. He scored right on target at 10 months in the adaptive area. I really don't remember what this area entails, but he has no deficit in this area.

We will begin meeting once a week beginning next Tuesday to address his PT and communication/cognitive needs. Everything should be reassessed in January. Most of the services he will be receiving seem to be preventive rather than greatly needed. Since Seth has the infantile spasm diagnosis, he automatically qualifies for the services. Our case manager wanted to go ahead and sign him up for services to keep him on track. She doesn't want to risk him regressing backwards. Josh and I are anxious to begin learning what we can do at home to help him meet these goals. We are both excited to be learning ways to help him!

All the kind gestures, comments, and thoughts over the last few months have been greatly appreciated. Josh and I feel that your support has been integral in helping us to stay positive. Thank you so much!

Terrific Results

Seth returned to the hospital today for his updated EEG. He has been on the Sabril/Vigabatrin for about six weeks now with no seizure activity since July 30th.

We knew ahead of time that this day would not be enjoyable. EEGs are never enjoyable for Seth. As expected, he screamed throughout the preparation for the EEG and fought me when it was time for the sleeping portion. I was able to get a clear view of the screen today and was upset to see that several large spikes were still appearing on the screen at random times.

As we left to head to his neurology appointment down the hall, I prepared myself for the inevitable. We were going to be told that his EEG still wasn't clear. When his neurologist walked into the room to see us, I was shocked to see a smile on her face. We were told that his EEG was FINALLY clear after all! I obviously don't know what to look for during the test. :)

She said there are still a couple of "sharps" occurring when he falls asleep, but everything was normal other than that. The sharps were explained as being much smaller than spikes and not a huge worry.

She asked about developmental milestones, and we mentioned that he still seems to be struggling with speech. She told us that while she thought everything would turn out fine, a reference to ECI (Early Childhood Intervention) would probably be in his best interest. That should be set up later this week.

We celebrated his clear EEG today with family. It was wonderful to be able to share his good news with the people we love most!

Our other great news is that Seth began weaning from the Vigabatrin tonight. In four weeks, he should be off it completely! We have been told to return for his next neurology checkup in January. He will not need another EEG at that appointment. We are so excited that our little superhero is doing so well! Thank you for all your continued prayers and support!

ERG? What's that?

Seth went in today to have an ERG (electroretinography) done on his eyes. Josh and I had never heard of an ERG prior to Seth's first eye appointment in August, so today was quite a learning experience for us. We were given instructions to not feed him after midnight as he would be under anesthesia for the duration of the procedure. As always, with a child as young as Seth, this made us nervous. He had been under full anesthesia prior to this in June when he had his MRI and did well, but this procedure would be done in a doctor's office that was not connected to a hospital. Luckily, Seth was only mildly sedated and was awake the whole time.

We were told that Seth's ERG would give a baseline of whether or not his retinas had changed due to the Vigabatrin. Unfortunately, it's not a very fun test to watch. He first had to be given the anesthesia while we were seated in a dark room. After that had kicked in, Seth and I were moved to another dark room where his eyes were held open using some metal spectrums and a kind of contact lens was placed directly on each of his eyes to monitor the electrical activity in his retinas. The doctor then used a tool that flashed several lights into his eyes. The test was then repeated with the lights on in the room.

Seth handled the test much better than I expected. He squirmed a little at the beginning of the test, but then just "talked" to myself, the anesthesiologist, and the doctor during the remainder of the test. When the test was over, the doctor told us that his results were normal for a baby in his age group, and that there was one large discrepancy between the data on the eyes during one test. However, she was not concerned about this difference as she notated that it was probably due to the fact that the makeshift contact lens did not fit that eye properly. Unfortunately, there are only two sizes in the lenses for babies and neither size truly fit his right eye, so she had to work with what she had been given.

His test results were faxed over to his eye doctor, so I'm sure we will hear more about his results when we return to the regular eye doctor in November. We are definitely glad that no vision issues have come up while using the Vigabatrin!

Since my last post, Seth has learned how to pull up on everything in sight and has produced a "d" consonant sound a few times. He has also given up almost completely on crawling and has chosen his preferred method of movement to be hopping everywhere. He's gotten quite adventurous and has hopped all the way from the living room down the hall to his room so far. We are really starting to do some serious baby proofing!

Seth will go in for his next EEG at the end of September, so we are hoping for even better news after that appointment. For now, things are going really well. Seth has been seizure free since July 30th, so we have finally hit one month seizure free! He is completely off the ACTH and prednisone at this point and is solely using the Vigabatrin. We will get a better idea when he will be weaned from that medicine after his EEG at the end of the month. Keep praying for our little fighter. It is doing wonders!

Miracle Medicine

I know many of you have been waiting for an update on Seth. I had been waiting until the Vigabatrin/Sabril had been in his system for a full week before choosing to update. To recap from previous posts, Seth had been placed on Vigabatrin to help lessen his seizures. The neurologist had told us that he could become seizure free as soon as 24 hours after beginning the new medicine.

Josh and I are VERY pleased to announce that Seth has been seizure free since Wednesday, July 30th! The Vigabatrin definitely seems to be working. We will go in to a pediatric eye specialist on Monday to check on his peripheral vision. These visits will continue every three months.

Seth has now started attempting to stand from a sitting position without pulling up on anything. He isn't successful yet, but he's getting close. He looks quite funny hopping on his bottom repeatedly in a failed attempt to stand! I've been told that he reminds many of Tigger when he does this. Josh and I are loving watching him grow and learn new skills!

The ACTH has finally given Seth a "moon face". I'm not used to seeing so much roundness to my little boy's face. It's going to take some getting used to on my part!

Pray that Seth's eye appointment is uneventful. We are hopeful that the new medicine does not end up affecting his vision. Thank you so much for your continued prayers and concern!

Joy and Anguish

The past week and a half have been an eventful and emotional roller coaster for our family. Seth experienced one glorious seizure free day right after we came home from the hospital, but this joyous occasion did not last. Instead, Seth has had one cluster a day (a cluster being full of multiple seizures in a small amount of time) ever since. Even more unfortunate is that each seizure has been worse than the last. Each seizure that has occurred within the past week and a half has sent me spiraling into tears.

The Saturday after we returned home, he experienced a cluster that lasted fifteen minutes. None of his clusters had ever lasted that long, so I called in to the neurology department to find out what to do next. On Monday, we were given a prescription for a rescue medicine that would stop a long lasting seizure. I took the medicine home feeling apprehensive and relieved at the same time. It was great to know that we now had a way to stop a long seizure, but I prayed I would never have to use it.

Seth experienced one of his most rapid clusters on Friday with seventy one seizures occurring in a seven minute time span. That particular episode was especially difficult to watch.  I had been instructed to call in every episode as they completed so it could be added to his chart. On Saturday, I finally asked the on call neurologist what the plan was to stop his seizures.

I was told that he would more than likely be given a second medicine on top of the ACTH. When I asked what medicine, the probable answer was to start Seth on Vigabatrin (the oral medicine). Vigabatrin is an antiepileptic drug. Since there is always a down side to any medication, we will have to be on our guard with this medicine as it can cause peripheral vision loss. Even with this serious side effect, I felt a huge sense of relief when I heard this was the next step in his recovery. Something just felt right about this choice, and for the first time in months, I felt at peace with Seth's IS.

On the flip side, Josh and I seem to have noticed an odd, unexplained link between Seth's seizures and his developmental milestones. With Seth experiencing the seizures, his brain should be slowing or regressing his development. In Seth's case,  however, he has not only been progressing each time a spasm occurs, but progressing rapidly. When he began having the clusters again that sent us back to the hospital, he attempted to crawl for the first time. He is now so close to truly crawling that I'm guessing he will be setting out to explore our house in roughly a week. He has also learned how to hold a sippy cup and can sit up from his belly all on his own. Tonight Josh was able to get Seth to stand on his own for about two or three seconds. While we are grateful that the spasms have not succeeded in slowing his development, we are both quite confused as to how this is possible.

Today I spoke to the neurologist about the next
step in Seth's treatment. We were given three options to choose from: Sabril/Vigabatrin, Topamax, and Onsi. Topamax can cause language delays and weight loss, so we quickly marked that off the list as he really can't afford to lose any weight. The Onsi could cause extreme emotional highs and lows. The Vigabatrin not only came highly recommended by the neurologist, but also just felt right to us. I can't explain the feeling - we just felt at peace with our choice.

He will begin treatment with the Vigabatrin hopefully on Wednesday. We are praying that our gut feeling is correct and that medicine will finally heal our baby. We are anxious to put this behind us as quickly as possible. Pray for good
results. If we see no improvement ten days after beginning this treatment, we will have to try yet another medicine.

Super Seth

We got released from the hospital yesterday afternoon. Since no one really ever gets a restful sleep in a hospital room, all three of us came home and crashed.

When Seth's neurologist made her rounds yesterday morning, we received news on his latest EEG and were told what his new round of treatment would become. She said that his EEG looks surprisingly better than it did at his follow up four weeks ago. I don't fully understand how this is possible since he wasn't experiencing any seizures at the follow up appointment, but I'm not a neurologist. So while it was confusing to hear this update, it was also very reassuring.

Since his hypsarrhythmia appears to be lessening, she said she had decided that the best course of treatment in Seth's case would be to raise the levels of ACTH dosages and taper off slower than we had before. She said that everything suggests that the ACTH was working before he began having the clusters again and is hesitant to jump to another drug without giving his body a second chance with the current medicine.

I am hopeful that another round of the steroid will stop the spasms once and for all, but a tiny part of me fears that it won't be enough. If the spasms recur, we will have to return to the hospital for another twenty four hour observation before switching up medicines. I would like to avoid another day long hospital stay if at all possible, so hopefully this will be it.

Seth did have another very large cluster this afternoon. Forty two spasms occurred in a fifteen minute period. This was his largest cluster since we began the first round of ACTH back in June. To make things even worse, he cried for the first time during the episode. Josh and I have decided to grit our teeth and bear with the spasms until Tuesday. If things do not improve, we will be calling to find out what to do next.

A major positive in all of this (minus the decrease in his hypsarrhythmia) is that Seth managed to pull his way all the way across a blanket on the floor on Thursday. When I shared this with the neurologist, she was very pleased. She said to take it as a good sign that he is not only staying on target with his milestones, but is actually ahead! Josh and I are celebrating every milestone he hits and are so excited to see what he will be able to accomplish next. He may not have his own cape, but he is definitely a real superhero in our eyes!

Hopefully I will not be updating this blog for quite some time. I would like to not write that we had to return to the hospital. Keep praying that no new issues will arise for the next six weeks.

Update 7/22:


Yesterday was a good day for Seth. He had a completely seizure free day AND almoooooost crawled. He is using a combination of ways to get to his desired toy (the pacifier). It's fun watching him rock back and forth on all fours, army crawl, pull his way across a blanket, and roll log style in his effort to learn. He's getting fast, too! So proud of our little superhero!

Carried

The last few days have been emotional for myself and my family. After Seth's episode on Monday, he had another on Wednesday. I had been directed to call the neurologist if it occurred again so we could be admitted for a 24 hour video observation. I made the call yesterday morning, and we checked in shortly after 1 pm.

There was one small condition - before further treatment could be decided, the neurology department had to catch Seth having an episode on camera. I was wary of this happening in the short amount of time we had been given since he had only had two episodes in the last week.

I will never forget the feeling I experienced at 4:42 am today. Seth had woken up and began to cycle through the seizures. My husband alerted the nurses, and his spasms were caught on camera. I have never felt such an odd mixture of emotions.

I felt a deep sense of relief that Seth had been able to have another seizure on camera. At the same time, however, I could feel my heart just hurting. I didn't really want him to have any more seizures. I couldn't stand this idea that I had to sit back and watch him seize through those cold hospital bars on his crib. All I wanted to do was pick him up and hold him close. After all, isn't it a mommy's job to help their child feel better? Making the situation even more heartbreaking was watching as he turned large, clearly terrified eyes on me and kept reaching his tiny arms toward me in desperation. How do you watch that as a mother and not feel immense guilt?

I am praying with every fiber of my being that now we can finally get a treatment that will stop these nasty episodes from ever returning. I am hopeful that Seth's wonderful neurologist will find a way to cure him. Josh and I are finding small bits of comfort in leaning heavily on family and friends as we sort through all of this.

I can't help but think of one of my favorite poems during this time as it's already given Josh and myself more strength to face this demon than I ever thought possible. I know He's there through it all!

Thank you all for your support, encouragement, and prayers. Even the smallest "I'm here" has meant the world to us. We are so grateful that Seth has so many people pulling for him to get past this!

Twenty nine

I had fully intended on writing a celebratory post today as Seth would be seizure free for one month today. Unfortunately, this celebration did not go as planned.

Seth had just woken up from a nap today when it happened. Seven involuntary head bobs in one minute. Seven times that my recurring nightmare crossed over from dream land to real life. Seven little knives tearing my heart into pieces in rapid succession. And then it stopped as quickly as it began.

Thousands of thoughts raced through my head during that one minute. Why isn't the ACTH working? Where do we go from here? Why can't he just get past this? Did I do something wrong? Is he ever going to fully recover from this? And the most selfish question of all - why my baby?


There is a flip side to this disappointment. My silver lining that I have to cling to is knowing that while Seth may have regressed a bit today, he DID make it a whole twenty nine days without having any clusters. Twenty nine glorious,  wonderful seizure free days have occurred. That means it can happen again. I know there is hope, and I have to cling to the reality of twenty nine consecutive seizure free days.

Now I just have to dry my tears, hold my son close, and celebrate...because twenty nine is a great number to celebrate!

Update 7/16:

Seth had another episode this afternoon. The movements were extremely small but more occurred today than on Monday. This time, he had twelve spasms in a couple of minutes. I was able to hold myself together for a longer period of time before my emotions got the better of me today.

We will be calling the neurologist in the morning to see when they can get it cleared with insurance to have him be readmitted to the hospital for observation. Josh and I are trying to keep our spirits high, but it's getting harder to stay positive as the spasms become more frequent. We simply want our little boy to be free of any neurological complications. I feel selfish saying this when I know that things could be far worse, but it's what we want. It's all we've wanted since this all began. Is that so much to ask?

EEG Results

Yesterday Seth had his follow up EEG. I think it's safe to say that it was not an enjoyable experience. Seth had to be sleep deprived for four to six hours prior to the procedure. He screamed throughout the entire prep and clean up. Josh and I were unable to see much of the screen, so we had no ideas as to what his results were this time around.

This was Seth after his EEG. He wasn't the happiest baby yesterday!

Today we met with Seth's neurologist to hear the results of yesterday's EEG. His EEG is still showing abnormalities, but there are far less spikes in his brain waves than when he was diagnosed. According to the neurologist, this is considered normal. She said she did not expect his EEG to be completely normal so soon because he is so young. It was explained to us that the brain is still developing, so it will take some time for the spikes to completely disappear.

The major positive is that Seth's EEG from yesterday was easier to read. His first EEG showed so many spikes that the neurologist had to zoom out to see the complete wave. She was able to view the entire wave from yesterday without any problems.

We are supposed to begin weaning Seth off the ACTH today because things are expected to get better over time. We go back for a follow up EEG in three months to see if the spikes in his brain waves are continuing to get better. While today's news wasn't the absolute best news we were hoping for, it is reassuring to know that Seth's EEG is creeping a little closer to normal.

Our second set of positive news today was that we have tentatively been given permission to slowly return to crowds. Josh and I have decided to tackle a grocery store visit with Seth before attempting anything else, but it's so nice to know that we can begin to return to normal activities. We are still very cautious to let anyone other than myself or Josh hold him due to unknown germ exposure, but it's a small victory!

We are hopeful that Seth will continue to respond well to the ACTH and develop normally. We are pleased with today's results and are praying for his EEGs to progress to normal with time. God has a plan for our little fighter, and I know He is with us through this journey in Seth's health! Thank you again to everyone who has kept our little boy in their thoughts and prayers! Stay tuned for more updates!

Waiting

Seth has remained seizure free since Saturday, June 14th. Josh and I no longer feel the need to watch in anticipation for an unexpected round of seizures to hit his little body. Seth is still doing well with the shots.

We have started to notice a couple of side effects from the ACTH. Since it is a steroid, Seth is hungrier than ever. At one point last week, our little porker downed twelve ounces in one feeding! We have upped his intake of baby food in an effort to quell the sudden desire for extra formula. So far, that seems to be working. Due to his increased appetite, Seth has started to get a bit chubbier. He's always been a thin and active baby, so he looks more like a "normal" baby now. We have been told that the puffiness will decrease once he has been weaned off the ACTH.

Several people have asked when he can be weaned off the medicine. The answer will largely depend on what Seth's follow up EEG shows on Thursday. If his EEG comes back clear, we can begin to wean him. If not, we regroup to discuss how to proceed next.  Josh and I are anxious to hear the EEG results on Friday. We are praying that everything comes back normal.

I should also make it known (for those who have asked), that we have been directed by the neurologist to keep Seth away from large crowds until further notice. The ACTH inhibits his immune system, so even seemingly insignificant illnesses are hazardous to him. Josh and I have gone through three full bottles of soap just within the last week! We do not mind visitors provided everyone visiting is currently feeling well and does not mind a few extra hand washings!

I will update again after our meeting with the neurologist on Friday. Thank you to everyone who has been sending well wishes and prayers. We are so grateful for your support!

A Wonderful Gift

Seth has now received five shots in all. The process has not gotten any easier with time. It still feels like my heart is being broken into millions of pieces when the needle goes through his fragile skin. He handles it like a true champion - with a few tears and a brave, sniffling smile.

The first two full days after being given the shot were hard on Josh and myself. Seth not only had more episodes each day, but each one also seemed to be growing stronger. After one particularly nasty episode (46 seizures in a six minute period), I panicked and asked the doctor if this was normal. I was told that it would take a few days before the medicine would start working. I remember thinking, "I'm supposed to just sit back and knowingly do NOTHING as my child seizes?! Are you insane?!"

But slowly, the medicine began to work. By Thursday, Seth had only experienced six seizures. It was a far cry from the nine seizures he endured on Wednesday. By Friday, only three seizures occurred within his tiny body.

Then we got a small glimpse of our own personal heaven. On Saturday, June 14, Seth remained seizure free for an entire 24 hours. It felt too good to be true. I was afraid to utter this observation aloud. What if the seizures returned full force? When I finally dared to whisper it to Josh, we had a small celebration with Seth and braced ourselves for a relapse.

As if Saturday's events weren't wonderful enough, we were somehow blessed with another seizure free day. I am proud to say that Seth has been seizure free for 56 hours now!  Josh and I are holding our breath in anticipation of what tomorrow will bring, but we are finally (and cautiously) optimistic about his progress.

God is so good! We owe it all to Him for carrying us through this. He is amazing!

A Difficult Diagnosis

Monday, June 9th started off like any other day in the Hill household. My husband, Josh, and my son, Seth, were up early and ready to head to work and Mimi's house. Josh was taking the morning off work to take Seth to the doctor while I went to work, but we planned to enjoy our evening as a family like normal. Our plans, however, got changed.

One week prior to this, Josh had noticed Seth nodding his head a few times in a row. It looked like nothing to me, so when Josh mentioned it as a concern, I pushed it to the side. As the week wore on, Seth's head nodding became more violent. By Thursday, he was folding his whole body in half and popping back into an upright position in rapid succession. These odd bowing moments started to occur in clusters and increased in frequency.

By Sunday, I was finally concerned. I still figured we were both overreacting as first time parents tend to do. I googled his symptoms and came across a site dedicated to something called "infantile spasms". I had never heard of this disease before. After reading more about the disease, I discovered it was a rare seizure  disorder that presents itself in infants between the ages of 3 and 8 months. The website stated that if the disorder went untreated, the child would lose the developmental milestones they had already reached and severe mental retardation could ensue.

As a teacher, I was terrified by the prospect that my baby could lose everything due to my ignorance. I didn't want to know that I chose to keep him from reaching his full potential. Josh had taken Seth to the doctor that morning to see what her thoughts were on his strange symptoms. She suggested an EEG to rule out seizures as a possibility. We weren't scheduled until Wednesday, but I received a call a few hours later telling us that some strings had been pulled and we needed to head to Cook's right away. So we left, fully expecting to return home later in the day.

What happened next was a blur of events and emotions. During Seth's EEG, the tech got up and told us that she was going to get a neurologist in to speak to us. I knew then what his diagnosis would ultimately become. Every person we came in contact with would bring me to tears that night. He was admitted as a patient right away. All I remember thinking from that point forward was, "Why us? Why our baby? Did we do something wrong?"

When we recieved his diagnosis, we were told that an MRI would be done to rule out any abnormalities of the brain. Waiting for those results were the longest moments of my life. Luckily in our case, Seth's MRI came back clean, and we were allowed to begin treatment.

Seth was to begin receiving a daily shot of a steroid named ACTH. ACTH is an anti-seizure medication and not guaranteed to work, but it was our best option at giving Seth a normal life. The clencher? Josh and I had to be the ones to give him the shots. I don't know about you, but I had never been able to handle watching someone get a shot, much less give one myself. I didn't think I could do it. Voluntarily hurt my child? I don't think so. However, I ended up surprising myself and gave him the shots. I have learned that I can do anything for the health of my child.

Right now, we are still waiting to see if the medication is going to work on Seth.  It's only been four days since treatment began, but we are hopeful that the ACTH will do its job and stop the seizures. God has been carrying our little family from the beginning, and I have faith that He will carry us through this as well.

This is our story of infantile spasms. Please feel free to check back on his progress. Thank you all for your prayers and support!

Megan, Josh, and Seth