ACTH Day 3

Well he had 3 clusters of spasms during day 3.  One at night and 2 during the day.  Usually he has 8-10 clusters a day!  We have even seen improvements in the twitching.  He definitely is getting hungrier and is telling us!  He gets fussy 45 minutes prior to feeding and sucks on his pacifier like crazy!  It is loud and hard smacking...think Maggie Simpson...and when nothing comes out of it, he gets frustrated!  He is bringing his fists up to his chest during his feeds too!  All this is very exciting and we hope it only continues to get better!  As far as the side effects go...we have seen an increase in appetite and he is irritable.  He seems to be irritable more during the evening.  His fussiness is fairly constant until we hook up his drip feed at night.  

It is good to know he is hungry!

  

ACTH Day 2

Jake gave the second shot.  Nicolas seemed to cry a little more today.  Probably because his mommy is such a natural!  HA! Honestly, daddy did great.  We were discharged shortly after. The Neurologist stopped in before we left and mentioned Nicolas' blood pressure was a little elevated.  On Monday we are scheduled to go back to Riley for lab work for our 1 week follow-up and to have his blood pressure checked again.  Nicolas was wide awake from about 8:30 to 10:30 this morning.  He seems relaxed so far.  The first spasm we saw today was at noon on the way home.  Here is a picture of him today.  We will attach a new picture to every post so that you can see the weight gain. 

ACTH

On Monday last week we contacted the Riley Neurology Department to inform them that Topamax was not working. On Tuesday we were told ACTH was our next move. So the nurse began making the arrangements through insurance and with a pharmacy that carries ACTH. I then took Nicolas to Parkview North so that he could have a Chest x-ray, blood and urine test. In order for Nicolas to take ACTH we had to prove he was a good candidate (i.e. not a diabetic, does not have high blood pressure, does not have an infection). Then yesterday I had to wait for UPS to deliver the medicine...apparently insurance wants you to sign for $84,000... This will last us three weeks. We will know if it works within 1-2 weeks. ACTH is a medicine given by injection (shot) in the child's leg. It is given at home once a day. If it does work, Nicolas will be on it for 9-10 weeks. Nicolas will be taking a high dose and along with that comes the following side effects...

• Increased chance for infection
• Thrush of mouth
• Diaper rash
• Increased appetite
• Water retention ("chubby cheeks"...as if he needed help in this department)
• Stomach irritation (He is back on Zantac for this reason)
• Increased blood pressure
• Fussy or irritable
• Rash on face
• Electrolyte imbalance

Because of the side effects, once a week Nicolas will have lab work. The 1st week is at Riley, 2nd and 3rd week can be done in Fort Wayne, and then the 4th week (if ACTH is working) Nicolas will have another eeg along with the lab work at Riley. As of today Jake, Nicolas, and I checked into Riley to be taught how to give the shot. Nicolas had been really good about getting his immunizations so I thought...piece of cake, right!?! Well I just gave him his first dose. He did great...I cried. No turning back now! We pray for his health and healing.

Lord, hear our prayers...
Felisa

Hello!  I know it has been a while...my apologies!  Well...last week we visited Riley (again) to talk with the Neurology department.  We still have no diagnosis.  What results we do have back from the spinal tap and blood draws have came back negative...but we are still waiting on a few tests!  What we do know is that Nicolas has infantile spasms which are a form of seizures.  He will outgrow them sometime between now and three years of age.  There is limited treatment options for these and they rarely work.  Once he outgrows infantile spasms a new form of seizures will take the place of them...at that point we will need to do another EEG to determine what kind and then more medications will be available with a higher success rate.  Right now he is currently taking Topamax for the spasms.  In 14 days we will know if it is working or not.   Once we get the brain to stop seizing Nicolas will have more opportunities to develop...However through our three therapy sessions a week we have already began to see progress.  It has been fun to watch!  We have appointments scheduled at Riley once a month through December.  I'll keep you informed once we return from each visit!

Thank you for your continued thoughts and prayers!

Felisa

Riley Updates from Emily Vlk

Thursday Night...

These are from my friend Emily who lives in Indy...she sent them out this week...Thanks Emily! I'll update you when possible...we should be coming home soon...maybe tomorrow...some corrections (which were my fault)...spinal tap and blood test results will be back in 3-4 weeks....10 -14 days refers to how soon we'll know if some of the medications work or not...my bad. Sleep study went great with good results...he just had an echo cardiogram...not sure about that yet. We have an appointment in August scheduled with Neurology and Dr. Hainline to discuss results and treatment plan. We currently are trying some medications that are to treat his symptoms...not diagnosed yet.
Love to all!
Felisa

Thursday Night...

Hello,

Nicolas had a big day yesterday. He had some blood work done as well as a spinal tap. The results can take up to 10 - 14 days to come back to the hospital. I need a little more clarification and I will get it soon but they think Nicolas's brain has lots of information to tell his muscles and organs but that information is not getting there as well as we would like via his nerves. Another busy day at Camp Riley. Nicolas is glad that his daddy comes home today from his business trip. He was glad Grandma Stein came and visited yesterday. Susie is visiting tomorrow.

I found this link on the Riley website if anyone was interested.
http://www.rileyhospital.org/information-desk/online-postcard.jsp

Love you!
Emily

Wednesday Night...

Good Morning -

I haven't spoken with Lis this morning yet, but they had a good day yesterday. They were moved to a different room with private bath. Now Nicolas won't have a stinky mommy. He also saw an eye doctor and....good news....no vision issues to report!!! Another point on the chalkboard!! I will keep you posted on updates.

Love, Em

Tuesday Night...

Baby Nicolas has made lots of new doctor friends. He had an EEG this morning. He has met with a hearing specialist and.....good news!! His ears are in perfect working order. He mustn't not want to hear what we have to say. :-) He will be doing a sleep study later this week. Some dental doctor is giving him some medicine that is supposed to help with his spit issue. Her sorority sisters are bringing her lunch and dinner. I guess that means I am cleaning tonight. I will keep you posted!

love you!
Emily M. Vlk


Monday Night...

Last night Joe and I took Felisa dinner at Riley. They are currently in a single, but may have to move to a double if a super sick infant comes in that needs it. They are hoping to be moved to one of the long term rooms that has a private bath. Eventually, Lis will be staying at the Ronald McDonald House if this is a long long term stay. When they first arrived, they were in a double room with some rude/unkind roommates, so the upgrade was nice and appreciated.

They met with the Developmental Team and they started the discussions on what testing they wanted to do and what each doctor was looking for. They are going to be meeting an eye doctor and hearing specialist to make sure Nicolas does both. Other testing is required but I don't remember it all. I should know more today.

Lis seems to like her nurses and is as happy as I can be to be at Riley. She believes this could be a long stint but we will see. There are laundry facilities on her floor so I am going to pickup a small bottle of detergent for her. She is packed well. A week's worth of clothes in the room, another week in the car trunk and a third week at home already to go. I am creating a downtown guide for her with restaurant/shopping/entertainment options for her. Garrett will come down maybe Monday. His school starts like August 12th so early. I am picking up my apartment tomorrow in case of visitors.

I am going there tonight too, unless some of her sorority sisters are there. I will keep you posted. Anything you want me to ask?

Love - Em
Emily M. Vlk

We had another appointment at Riley this past Wednesday, this time to meet with both the pediatric gastroenterologist and a developmentalist. The GI said Nico's esophagus and stomach look good. The nisson looks good and doesn't appear to be letting stomach contents back up into the esophagus. There are no signs of inflammation and food appears to be moving normally through the digestive tract. Though it is not likely, it is possible Nicolas is having allergic reactions to the food though there is no evidence to suggest that at this time. Nico's retching, which has become more frequent and much more uncomfortable for him over the last few weeks, might be caused by intermittent problems with his stomach emptying into the intestines. Again, there is no evidence of this except the retching.

Now onto the heavier stuff. The developmentalist spent a couple of hours with us reviewing Nico's history and observing him. She was able to see him retch and see his spasms, both video and live. She also spoke with the metabolic geneticist who had seen Nico last month. Because her goal is to help Nico feel better and to determine what is causing his symptoms, she feels he needs a neurological follow up as well as to try different treatments e.g. feeding changes and prescription changes. She also thinks that experts in infant development, pediatric neurology and metabolic neurology should observe Nicolas on a regular basis and be able to observe him as a team. Therefore, she recommended we admit Nicolas as an inpatient at Riley. So, back to the hospital we go. We are taking the family truckster to Indy on Monday and will be there until they can help our little angel feel better. As difficult as it is to go back to a hospital, this time 150 miles from home, we know that our prayers lately have been to provide him with comfort and to help us understand what is happening to him. Hopefully, this is the beginning of an answer to our prayers. Thanks to everyone for your continued thoughts, prayers and support. Though we don't have the opportunity to tell you thank you every day, please know that we think of you all daily and our hearts are full of gratitude that you are part of our lives. Until the next update....Jake and Felisa

Happy 4th of July!

This past weekend we had a wonderful time with our family and extended family up at Papa's lake house to celebrate the 4Th of July.

Here are our photos...

Dad dancing with me at the lake...

Our sweet little Nicolas is growing bigger and bigger every day...12 1/2 pounds!

We will be starting therapy with First Steps this coming week. There will be three different types of therapist working with him a total of three hours each week. Then later this month we will be meeting with a new team of doctors - Behavioral Development Doctors at Riley.

The Doctors are still stumped with Nicolas case and all past and recent tests all have come back negative. We are all looking forward to the day we get some answers for Nicolas.

Thanks for all your prayers and please continue to pray for us. We are in Gods hands and we are waiting on His timing - easier said then done some days.

Much LOVE...

The Stein Family