November 26, 2013: All Clear!

November 26, 3013:  We are back in Rochester for the follow-up EEG after one month.  Last month they saw activity consistent with a focal seizure disorder, and the past few weeks have been like a roller coaster. Chase was doing really great and we noticed improvements in his development- even throwing temper tantrums just like his sister did at this age!  Then last week, he caught a virus at daycare and was really "off".  This is an example of how this diagnosis has put fear in us forever.   He would wake up from naps dizzy and fall over. He was miserable especially when he woke up and would not smile at us or focus on anything.  (This reminded us of when his brain activity was abnormal.)  We spent too many hours crying last week and did not sleep, anxious to get back here for his test, just sure that they were going to see something on this EEG.

Funny picture of the boys- Luckily the crying did not last long!

It turns out, Chase was just acting like a miserable child with a virus and we will forever be paranoid :).  We found out today that Chase had his first all-clear EEG and we do not have to return for 3 months for another test!!  He will stay on the topiramate for at least one year with clear EEG's before they do any experimentation of taking him off medication.  He has a very high chance of developing other seizures later in life, so we will always be on guard but he has had one of the best turnouts that we could ever wish for.  The doctors discussed how Chase has a lot of work to do to catch up, but we feel good that we are already doing everything we can possibly do at this point to help him.

2 of the 3 Pediatric Neurologists we have been working with at Mayo,
Drs. Wong and Perumpillichira

My new mission is to share our success story to help other families facing this devastating diagnosis. There are many terrible outcome stories online as we personally discovered, which added to our stress. Of course every situation is different, but I would like to share our positive story.  I have successfully contacted the marketing lead for Infantile Spasms at Questcor, the manufacturer of the Acthar gel (ACTH) which stopped Chase's spasms.  During this Thanksgiving week, she is planning to share out story with the team at Questcor to remind them of why they work hard and do what they do every day.  I have also contacted other Infantile Spasms support groups and neurology organizations.

This Thanksgiving, we are most thankful for Chase's success.  He is an amazingly strong and resilient little man and we tell him every day he will do great things in his future.

November 18, 2013:   The past few weeks we have all been sleeping again and life is getting back to normal.  Ahhh.  Although I still randomly freak out, and my husband is patient and loves me.  (He made me write that.)  Chase's gigantic cheeks have gone down a bit, and his appetite has come back after adjusting to the new medication.  About 2 weeks ago, there was a noticeable change in Chase and he started being able to focus better, even stopping to look us in the face.   His little brain has been so overwhelmed healing and learning so much, so fast.  He is now 14 months and had been evaluated by Early Intervention at 7 or 8 month old levels for everything other than gross motor skills, so we have some work to do.  Basically most of those skills froze in their development when the spasms started.

Early Intervention has begun working with Chase weekly at daycare, and he has upcoming consultations with occupational therapists for his eating issues and speech delays.  However, yesterday he really started babbling so that was very exciting for us!  Although Chase's new hobby of biting wasn't going over well at home or daycare, it didn't seem out of anger but him just wanting to chew.  The daycare has been reporting recently that they are noticing improvements, like he will actually sit and play with a toy for a minute and that he has clapped his hands together (although we have to see it to believe it!)

Chase's first Halloween- Witch Anna & Lion

Chase won't eat textured food-
EXCEPT Twizzler's?

I have also been connected with another mother in the area with a son about 4 months older than Chase who had Infantile Spasms.  Although their story is different as their son had a stroke in utero and began having seizures the day he was born which then turned into IS, it was SO, so wonderful to talk with someone who truly understood the diagnosis and had been through the same things.  I hope to see more of her and meet the rest of her family.

Tom and I have now had time to sit back and reflect on what has happened the past several months and it has brought the most emotion and tears.  We had literally been running on autopilot (and fumes), just getting done what needed to be done.  We are so grateful for how Chase is doing now, realizing the odds and how badly this could have gone.  We have been amazed the most at the generosity and kindness of people we barely know from our church.  Just yesterday we received a pile of Target and grocery gift cards in the mail from the Pastor at our church, and a sweet card for Chase made by a religious education student.  This week a new friend from the church is coming over to meet Chase and bring a quilt they made for him.  Tom and I are looking forward to helping others in the church in some way who have gone through similar situations, when things slow down a bit for us.

The Next Bump in the Road - New Focal Seizure Activity

On October 22, we went back to the Mayo Clinic for another EEG and meeting with the neurologists. We were feeling wonderful because we knew the spasms had stopped and Chase was already changing so much. That day, Chase had blood work done to test his cortisol levels while we were there (since ACTH suppresses cortisol production and they had to be sure this was rising since tapering from the ACTH so that his body could fight infection again). We also had to meet with the nephrology (kidney) doctor since Chase had consistent high blood pressure while on the ACTH. The cortisol was low so needed monitoring, but his blood pressure was going back to normal. Chase also had to have a vision exam after taking Sabril, but he passed that with flying colors.  Whew.

A few hours after the EEG we met with the Neurologists and they gave us some news we weren't expecting. Chase did sleep for this test (unlike last time) and they were able to see he had activity consistent with a focal or generalized seizure disorder. The good news is that these type of seizures were obviously not as destructive to the brain as infantile spasms, and they could pinpoint exactly where in the brain this was occurring so it should be easier to get under control with medication. But this was not what we wanted to hear, so soon. We wanted to hear that he was free and clear of any problems and this was the end of our journey and everything would return back to normal. "We just do not know how his development will be", is what they said. "We will do everything to give him the best shot at life that HE can have." The conversation about where he was at with his development and his future was not sounding so positive anymore, and Tom and I were back on the next drop of our roller coaster ride. Chase would remain on the Topomax and we would return in one month for another EEG and evaluation.

Chase at Mayo Clinic October 22, 2013

EEG's were no big deal for Chase anymore

University Square in downtown Rochester from the 16th Floor of the Gonda Building

Happy swimming at the hotel pool

Tapering off ACTH and Feeling MUCH Better

October 4, 2013: Chase was feeling so much better after a few days of decreased amounts of the ACTH in his injections and was so happy – but now he would NOT sleep.  He would stay awake during the day for 16 hours, without one nap.  He just stared around in amazement, like he was seeing the world for the first time.  It seemed like he was overstimulated and suddenly aware of his surroundings like he had not been for a long time. He must have been walking around in a fog before. It was so wonderful to see him alert (albeit overly) and showing interest in toys by picking them up. Throughout the next few weeks, Chase didn't even notice his injections any more.  We realized what a tough little guy he had become. 

The next few weeks were still hard, but exciting.  Chase now had started the Topomax, so almost immediately his appetite decreased.  We could barely get him to take his bottle with medications!  He was now hyperactive- darting from one thing to another.  We could not get his attention, which was a little disturbing but we were so glad he was moving and interested in things around him.  I would call Tom crying and excited that Chase got into the garbage or the toilet, everything around that we had become so used to not worrying about because he was not interested in/did not notice ANYTHING before.  Suddenly these things you take for granted (and can be frustrating when you have kids) were the most important events in our days! Chase went back to daycare at the Goddard School in Fargo and things were still pretty wild in his little brain. They were so wonderful and accommodating, as Chase had never had a real sleeping or eating schedule like all of the other children. In the next few weeks it was now exciting to see him start to follow somewhat of a normal schedule!  

Chase (obviously) feeling better.  He just loves being outside!

Great News- ACTH stopped the Infantile Spasms!

October 3, 2013: Two weeks later we had just begun tapering the ACTH and were back at Mayo Clinic in the Gonda Building 8th Floor we had become so very familiar with.  It was the first EEG where Chase sat completely still and didn't even flinch as they scratched his head to attach the leads under the infant cap.  It was almost eerie.  We were so nervous during that EEG, we watched the computer monitor trying to decipher the brain activity but did not know what we were looking at but somehow it did not look good.  We left the room sick to our stomachs and had to wait over 2 hours to meet with the Neurologists to go over the results. It felt like days.  I knew the moment Dr. Joseph walked in with the biggest smile I had ever seen on his face that we were wrong. 

Chase ready for his EEG- hoping for good news

Chase and his new belly and cheeks at Mayo Clinic October 3rd

The doctor reported great news – Chase’s brain activity was back at a normal level for a boy his age!!  They were a little upset that he did not sleep during the EEG but his brain activity awake looked great. The plan now was to continue the taper off of the ACTH, but also start on the Topamax as an extra precaution.  They were hitting it hard.  They needed to monitor him very closely in the coming weeks because these seizures like to come back, so we were scheduled to come back in another 2 weeks for another EEG.  We were told that he had a very high chance of developing other types of seizures later in life, so unfortunately we will always be on guard.  That night, we celebrated with a great meal at our new favorite Rochester restaurant, Chester’s.  Chase just sat with us and didn't make a sound but looked around. 

Chase's EEG while he was awake was back to normal brain activity for a boy his age!

Tom (originally from Illinois) vowed he would become a
MN fan if a MN hospital saved our little boy.
Here is the new MN Fan!

Thumbs up after hearing the great news!

Going Home with High Hopes- 1 Month of ACTH

Chase on September 16th

Chase on October 2nd 

Sunday, September 22nd- On day 6 of the ACTH, Chase had his first day with no spasms! Our hopes were as high as the sky!

Chase was now immunodeficient so he could not go to daycare and risk getting sick or infection during the duration of the ACTH injections. Tom and I began our shifts of each going to work for half a day. Tom owns his own lighting business and luckily my employer Spectrum Aeromed was completely supportive and flexible.  

The next 2 weeks of the full dosage I would say were the hardest we had ever been through as parents. Chase was just miserable, crying all day and all night long. It was so hard to see him in so much pain and it was tons of work feeding, diapering, doing laundry all day long. Chase would drink an 8 ounce bottle of formula every hour and ate 3-4 full meals of baby food each day and snacks all day in between. This was quite significant since he would barely eat baby food prior to this!  He would wake up all night long screaming, miserable and hungry. He gained 30% of his body weight within a week and could barely walk around.  His stomach bloated out. And the diapers…  We went through diapers, formula and food like you have never seen.  Our daughter Anna would walk around with her hands over her ears and would run and hide in a closet when it was time for the injections.  

Chase paced aimlessly and cried like this every waking minute for 2 weeks...

Morning and night, Tom would hold Chase down, and I would give the injections (the nurses told me I would get used to it but it never got easier).  Chase would scream in terror each time he saw us coming with the needle.  It was the hardest thing we have ever done, but each day there were no more spasms we just focused on that fact and it made it easier.  We had to visit his local pediatrician 3x a week during this time, who reported his blood pressure and blood test results to the doctors at Mayo.  

Obviously by now, our beliefs had turned around. Although I did not know it at the time, Tom later told me that he took Chase to church every morning and prayed for him and had a priest bless him there. We had a priest from the parish I grew up in come to the house and bless Chase as well. He was on the prayer chain at both churches we attended and we started sending our daughter to Sunday school at this time. Ironically, during her first week the lesson was on how Jesus heals the sick.

Chase's stomach was so hard & bloated it looked
and felt like it would explode!

Not a happy boy

Hospitalization- Beginning the ACTH Injections to Treat Infantile Spasms

September 17, 2013: I took my mom (Grandma Judy) on the second trip to the Mayo Clinic. Tom and I had already missed so much work and we just figured they would give us the new medication and we would return home. Although I knew things were not going well, I was shocked when the neurologists came in the room in a panic at Chase's EEG test results from earlier that day. They hypsarrythmia (the abnormal brain pattern between seizures consisting of high amplitude and irregular waves and spikes in a background of chaotic and disorganized activity which helped the doctors diagnose his condition), was no longer intermittent but constant. Basically he no longer had any normal brain activity. The 2 weeks between the last EEG showed remarkable progression of his condition. 

The spinal tap and all of the other tests results came back unremarkable, so there was no known cause for the Infantile Spams. I was told this was the best circumstance to have. However, I was told we had a VERY small window of time to get these seizures to stop for Chase to have any chance of leading a somewhat normal life. They said we needed to start the ACTH steroid injections immediately and Chase would need to be admitted to the hospital for monitoring the first few days, as there are very serious side effects. In addition, we needed to be taught how to give the injections at home. I agreed without even calling Tom. I knew he would want me to do anything possible to help Chase.  

Chase's EEG on September 17

It was almost 6:00 pm and the Mayo Clinic offices were closing and we needed to go over to St. Mary's Hospital. The regular hospital admissions were closed for the day, so they had us report to the Emergency Room to be admitted to the hospital. The next few hours is a completely different story for another time, but I did NOT need to see what I did in the ER after learning this bad news! Whew!! I am still trying to erase those visuals from my head. 

Chase had his first injection that night. He did not sleep well after that, but I had no clue what was about to happen next with him. The stay in the hospital was the most insane time. I was there by myself (Grandma Judy had a bad cold and had to stay at the hotel), and on top of Chase not sleeping more than 1 or 2 hours at a time, a different doctor, resident or nurse from several different departments, social worker, hospital chaplain, arts and crafts person (not kidding), or any other person you could possible think of came walking in the room literally every 8 minutes. It is a teaching hospital so there were a couple of times there were literally (I would count as they surrounded Chase and I) 10+ people in the room at one time. It was overwhelming. My cell phone rang every half hour, from Tom, our church, our daycare, Early Intervention in Fargo, to the medication manufacturer, to Walgreen's Infusion Center trying to line up the shipment of the drugs to our home (which was a complete mess.)   The next few days in the hospital were miserable.  

The hour or 2 I could have spent sleeping at night, I lay in my hospital cot crying and screaming to God to do something! I talked to my mom about our anger with God. Over a lot of tears, she gave me the best advice I could have ever received. She told me- NOW IS NOT THE TIME TO TURN YOUR BACK ON GOD. I got it. I turned it around and started praying. I promised God I would do ANYTHING if he saved my boy's brain.

Chase became a "wild man" just as they said would happen. He did not stop moving and I was afraid he would hurt himself on the hard floor and sharp edges in the hospital room but he pulled my hair and bit me when I picked him up. I put him in the crib often and he would bang on the sides of the crib like a maniac He ate nonstop. They said he would be hungry and gain weight but I did not anticipate to what extreme this would happen. He was put on a number of other preventative medications due to the side effects of the ACTH: Zantac to protect from stomach bleeding and ulcers, Bactrim to prevent infection, pyridoxine (Vitamin B) to help with seizures, and he was still tapering off of the anti-epileptics Sabril and Keppra. Chase's blood pressure was extremely high, so he was put on Diurine. The nephrology team had to keep checking on him and were concerned with possible kidney damage.  The ACTH (Acthar Gel) was being shipped to Minneapolis and took time to get insurance approval. Once he had started the injections he could not stop, so even after Chase was released from the hospital 3 days later we had to move to a hotel and go in for injection appointments at the infusion center of the hospital morning and night. Tom had arrived the day we were being discharged from the hospital because he was losing his mind not being there with us.  

Chase passed out long enough for us to enjoy lunch at Mac's in Rochester,
the day after we were discharged from the hospital  

Chase's blood pressure still did not lower so he needed a one-time dose of isradapine and they held us for hours the morning we thought we could leave to go home. The dose of Diurine was then doubled going forward and they finally let us go. The discharge plan was that Chase would receive the full ACTH dose for 2 weeks and we would return to Mayo for an EEG. If there was no longer the hypsarrythmia we would begin tapering him off of the ACTH for 2 weeks after that and he would be done. It was very dangerous to take this any longer, as long-term use caused many health complications and even diseases. His immune system was compromised so he could not go to daycare during the time he was receiving injections. Plan B was to add another medication, Topiramate, to his treatment if the spasms did not decrease within one week.

We received the call that morning that the Acthar Gel was in Minneapolis at the Walgreen's Infusion Center, so we could pick it up on the way home. We were informed there that we needed to be VERY careful with the medicine, as each injection dose was just under $7,000. At 2 injections, that was $14,000 a day, for a month over $400,000!! Luckily, my health insurance covered every penny.  We arrived home on Saturday, September 21st and were on our own to juggle this new complicated schedule of injections and medications, which was very scary.

What a relief! Picking up the medication that was
our last chance of saving Chase's brain!