The Beginning of the End of Steroids

Dear Infantile Spasms,

 You suck! You have taken away so much from our son and our family. You have brought fear, sorrow and anger to a new level, a level that I could never have imagined existed. Although you have won many of the battles these last few months, I promise you that we will fight until the end and we will win this war against epilepsy. 

With Sincere Hatred,

Krysten

This last week and a half has been yet again a roller coaster. As Marek was getting over RSV his spasms got worse. We were down to one cluster of spasms every other day but then they picked up again and he was having multiple clusters a day. We went up on the steroid, Prednisolone, on April 16th to 40 mg a day. Since Marek was still having spasms the doctor let us increase his steroid on Saturday, April 25th to 80 mg a day but with the understanding that in three days if he doesn't show a response he would start to come off all steroids.

We feel that Marek is starting to respond to the steroids. FINGERS CROSSED, KNOCK ON WOOD & PRAY. I met with his doctors today to discuss where we go from here. They told me that even though Marek might be responding to the Prednisolone they feel that it is in his best interest to start weaning him off of them. They dropped his dose to 60 mg this evening and he will be weaning off of them over the next TWO MONTHS. (We have been on steroids for so long that I think roid rage, sleepless nights, insatiable appetites, and extremely chubby cheeks is typical Marek. I honestly have a hard time remembering what our little man is like off of steroids)

Marek's original PET scan was scheduled for last Tuesday in St. Louis but since he was still getting over RSV they rescheduled the scan to May 19th. After talking with the doctors in St. Louis they feel that we should go to Detroit and have Dr. Chugani run the scans and they will work along side of him depending on what they find. The catch is Dr. Chugani will not run a PET scan until Marek is off of steroids for four weeks. So we most likely won't be traveling back to Detroit until the end of July. After they run the PET scan in Detroit we would come back to St. Louis and be admitted to Children's to start the Keto Diet.

What we are hoping for is that even though they are decreasing the dose of his steroids that he continues to respond to the medicine throughout the entire wean. Towards the end of the wean St. Louis will be doing an EEG to see how much the steroids helped and to also give us a starting point for the diet. I know many of you have continually been praying for Marek. Please continue to do so, and maybe say a few extra. This is the last medicine on his list of Anti-spasm drugs. Next step is either the diet or surgery. Yikes, just typing that makes my stomach turn.


Thank you to all our friends and family who have put so much time and effort to help fundraise for Marek.

Go Away Germs

I am not quite sure how our germ free bubble got so infected but it wiped us out. Ryan, my mom and I all tested positive for strep and on top of that, I am pretty sure we got Marek's little virus that presented like a cold in my mom and myself. We have been feeling like crap all week so I can only imagine what our little guy feels like having a virus plus seizures plus being on steroids. I am amazed at how good of a mood he has been in the last couple of days.

Since Marek tested positive for RSV the hospital originally cancelled his PET scan for Tuesday and then the next day called me back to tell me they will still go forward with the test as long as he is not wheezing or has a productive cough. Since they have gone back and forth with whether to perform the procedure, I am taking Marek back to the pediatricians on Monday so that the doctor can listen to him and either give me the all clear or let me know I should reschedule his procedure. They said that sedating him while he is still congested would be putting him at risk and we don't want that.

Marek did go on high dose predisolone on Thursday. We are hoping, praying, pleading and begging that this is going to do the trick. We just need to get rid of these spasms. When this all started back in January I had no idea we would still be struggling for control months later, I guess I was being naive thinking that maybe the first one or two medicines would work. His bicarb level has slowly been going up therefore, we have been able to continually increase the Topamax. In order for Topamax to be at a therapeutic level in the blood his topiramate level needs to be at a 10, and then the doctor would keep him on Topamax for a few weeks at that level to see if it is effective. When we checked his topiramate level he was at 8.4, so we are almost there. I am hoping that if his level can reach 10 while he is on high dose predisolone that this could be the magic combo that kicks these monster seizures out for good.

We got some good news this week, Marek was approved for OT for an entire year. Now we are just waiting to hear about PT. I am glad OT is back! She hadn't seen Marek in three weeks. She told us she felt that his skills have regressed since she had last seen him, which his PT also put in her report. We are hoping that this is due to him being sick the last week and a half. His vision teacher came on Thursday and evaluated him. With Cortical Vision Impairment they score children's vision on a scale of 0-10, zero being that a child can only see light and ten being that they can basically see how neuro-typical children see. Marek scored a 5.2 on his evaluation. She said he did pretty well with tracking but his hand-eye coordination is very delayed. His teacher will be coming every other week to help Marek exercise his eyes and to teach Ryan and I exercises that we can do with Marek to help improve his vision. She told me that with a lot of practice many children increase their vision over the years.

RSV and Genetic Testing

Last week was a tough week. Marek was diagnosed with RSV on Wednesday. It made us mad because we have been so careful about keeping the germs out of our house. It hit him pretty hard, he was wheezing and coughing so hard that we took him back to the doctor on Friday. There isn't anything the doctor could prescribe to help him get rid of RSV since it is a virus however, the doctor did prescribe albuterol to help him breath a little better. So we have been giving him breathing treatments every four hours since Wednesday. We are so lucky to go to St. Clair Pediatrics, everyone there treats Marek and us like family and goes above and beyond to make sure Marek is safe and taken care of. We went back to the doctor today and were told that Marek sounds a little better. His wheezing is not as pronounced and he seems to be coughing a little less. We haven't completely beaten the virus but I think we are on our way to getting him better.

Since Marek has been fighting RSV his neurologist didn't feel that he could safely up his dose of Prednisolone since it would make him more immune suppressed. So that has been put on the back burner until he has a clean bill of health. We are hoping he will get over the virus soon so he can start the high dose of steroids. He has had more seizures this week than last. I am not sure if there is any truth to this but some say that when little ones are sick they are more prone to seizures. Hopefully that is the only reason he has had so many.

We got Marek's genetic testing back today. We were hoping for normal results but unfortunately that wasn't the case. His results show that he has an extra extension on the arm of chromosome 7. I have no idea what this means and our neurologist didn't either so he is sending us to a genetic specialist. We are waiting for a call to set up an appointment. It is so scary to think what this means. We are not sure if this is the cause of Marek's IS or if it might cause other health problems. We are stuck WAITING once again for answers. I am hoping and praying for the best while trying to keep my mind from thinking about the worst.

We have a busy week with PT, OT and Marek's vision teacher. Hoping that he feels better so we can make some progress with his development.

Please keep our Mighty Man in your thoughts and prayers as we continue on this journey.

"Hopping" for Seizure Freedom

With Easter being this past weekend time got away from me and I wasn't able to update Marek's blog.  Let's see, where to begin.

My mom went home last weekend (she needed to bring home all her winter clothes and bring back all her spring clothes) and we gave Ryan's mom the weekend off so we could be our own little family again. It was nice just being the three of us. We got Marek a bike trailer and took him for a ride which he loved! We spent lots of time playing with our peanut, visiting with Gigi and Grandpa (which was the first time Ryan's dad was able to see Marek in months), doing some Easter crafts and enjoying Marek's good mood. That mood changed Sunday night. I think the new steroids finally got in his system. Although Ryan was staying here at the house I was on my own at night from Sunday to Wednesday (that is when my mom was getting back). I cannot explain how hard it was to take care of our steroid pumped baby by myself. He is back to waking up every hour with this high pitched scream wanting to eat, be rocked, or walked around. Then after a sleepless night we had therapies in the morning and doctor appointments in the afternoon. I think I called my mom every few hours begging her to come back sooner. We made it through though and my mom AND dad came down on Wednesday. Its the first time that my dad was able to see Marek for months. I enjoyed watching BOTH my parents spending time with their grandson.

Ryan unfortunately had to go back to his parent's house Wednesday night for the rest of the week because he started to get sick. He was prescribed medicine and was able to come back Friday night but wore a mask while he was home.

We celebrated Easter on Saturday. It was nice to have BOTH sets of grandparents with us. Marek enjoyed having a house full of faces. He loves to be a ham and make people laugh. The Easter bunny came on Sunday and brought Marek lots of goodies. His favorite gift though came from his Auntie Kourtney. She got him a door bouncer. Although he doesn't "bounce" in it he is starting to use his legs ever so slightly. Marek hasn't even wanted his feet on the ground for the last 8 months so the fact that he is trying to straighten his legs while in the bouncer is wonderful. THANKS NINI!

The sad news about therapy is that our OT is not coming to our home right now because our insurance hasn't authorized any more visits. I am so fed up with insurance!!!! Marek is so vulnerable to regressing with his skills that it is crucial he gets as much therapy as possible. I am praying that insurance gets figured out this week so that OT can come back and more importantly that we can keep PT coming. Our poor physical therapist has yet to be paid for her services and is still coming over just because she is AMAZING and is hopeful that insuranc will get figured out soon.

As far as Marek's development is going, it's a SLOW process but we think (fingers crossed) we might be back on the right road. This week he is able to sit up much better. Last week we referred to him as "Linguini" because he was just a limp noodle when trying to do anything. This week he seems to have better tone through his trunk and is able to sit up on his own for short periods of time.

He isn't rolling over again yet which still makes us so sad. His PT brought a new wedge for him to do tummy time on. It isn't as steep as his first wedge but it gives him a little assistance since he isn't really able to hold his head up still while on the flat ground.  We are hoping that maybe these small steps in the right direction are a sign that the medicine might be starting to work.

Last week we were slowly increasing his dose of Topamax. We got his bicarbs checked last Monday. We found out that his bicarbs dropped to 20 (normal limit is 20-27, when we started Topamax this time his bicarbs started at 26). Since it was still within normal limits his doctors increased his dose but we had to go back to the hospital last Thursday to get his levels checked again. On Friday we got some good news. His bicarb level actually increased, it was at 21!  Although  not a huge increase, the doctors told us that typically when your bicarbs increase, even slightly, it means your body is now able to tolerate the medicine, so they increased his dose again on Friday. We will continue to increase his Topamax dose every three days until we reach a "therapeutic level". Marek is only about half way to reaching a full dose. Since his bicarbs have now stabilized, the doctors are now able to increase his steroid dose from low dose to high dose. This will happen this coming Thursday.  Our fingers are crossed that once he gets on high dose Prednisolone this will be his miracle combination and we can get rid of the monster seizures once and for all.  We need lots of prayers please.

Marek is still having spasms/seizures. He is having a "big" cluster every other day or so which lasts about 20 minutes. He is also having several small clusters or single spasms throughout the day. We are hoping to get seizure control soon. Our neurologists scheduled an EEG and a PET scan for April 24th to see if Marek has a focal point where his seizures are originating from which would make him a surgical candidate.

This coming week is a pretty low key week for us. Usually we have multiple therapies and appointments to go to, but this week we only have PT on Wednesday. We had to say goodbye to Papa (my dad) this morning. He has to get back to work.  Marek will miss him and so will I.  He was such a BIG help. He took the 4 am shift every morning and had all Marek's bottles washed and put away before I woke up each morning. He also put up Marek's swing which he loves. THANKS DAD!