Posts Tagged ‘Niemann-Pick Type C Disease’

Recap of Week and IEP Meeting Outcome

Did we say how nice it was to be actually home all week and not traveling? This past Monday Brisan was able to access his port-a-cath for the first time since surgery on April 7, 2010. It was a calm week besides all the work Jennifer put into getting ready for the IEP meeting for Brisan, Parker, and Duncan on Friday the 7th.

Brisan's first usage of his port-a-cath

Brisan's first usage of his port-a-cath

The meeting overall went well. Since Brisan is moving into Kindergarten next year, we needed to have the discussion on what was next for him. It was an emotionally charged atmosphere at times. We all know with Niemann-Pick Type C Disease that the outcome isn't pretty with the grim truth that there might not be an abundance of schools years left.  As a family we had many concerns if GV school district could accommodate us.

Brisan will essentially be going from 8:30 to noon, which isn't much different from now. He will participate in the regular class with his peers and also eat lunch with them. They agreed to hire an LPN for Brisan and starting Monday they will have an additional Para in the class for both of them for the remainder of the school year and beyond. Next year Brisan and Parker will both be in different buildings which poses a challenge. It probably is unlikely they will hire two LPN's.

The IEP isn't final until they formally write it up and give us our 10 days to review. We also spoke about Duncan starting next January. Parker's IEP will be talked about in this upcoming week.

Overall the stark truth was very sad and disheartening as we listened to all his inabilities. This is the time reality sets in and gives you a smack across the cheek. Just over the course of a year Brisan has regressed in a lot of his abilities. For others who are outside our home it might be hard to completely see this digression.

We will write more later! Honestly there is way too much to write about. If you have any questions please feel free to email Jennifer or myself .

Below are some pictures of Brisan graduating preschool on Thursday May 6, 2010.

Brisan's Graduation from PreSchool 5-6-10 Brisan getting his dipolma 5-6-10Brisan 5-6-10Brisan and Parker drinking juice 5-6-0

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Upcoming week

It's been a week ago tomorrow evening (Monday) that we were finally able to return home after Parker's crazy situation while in DC. Nothing new has transcribed since we've been back with our doctors. You almost get the feeling that with Brisan's sinus polyps and Parker's blood redirection or loss that no one has an answer.  Leaves you wondering what shall happen next?  Brisan's sinus situation is so bad that it is causing his right eye to matte shut and consistently be weepy. Our new nickname for Brisan is “Pirate Pete”. That is what he looks like! Hopefully we can get somewhere!

Friday  we have an important IEP meeting with the Grain Valley School District regarding the future of the boys. Jennifer will be busy gathering information and coordinating up until then. The meeting will speak to all points hopefully on how they can and cannot fulfill the needs of Brisan and Parker. We also will speak about Duncan going next January to school. . There will be probably upwards of 20 people in attendance to discuss this matter.

Brisan is suppose to start Kindergarten in the fall. After three hours in the morning now he is completely wore out! There is no way in the world the little man can go all day. They would allow him to go a half a day from previous conversations, eat lunch at his own leisure, and allow additional attention to his needs. Many things besides what some would call “minor decisions” for us. The important issue here is safety, the schools funding of the special needs program, and if they can provide for our children. No hard feelings if they cannot.

Some would say that his progress is indeterminable because he has been absent from school a lot. Regardless, Niemann-Pick Type C is already taking it's toll on him and Parker. All we've seen from my recent memory is digression in all areas from quarterly updates.

We feel that there is a better alternative for both boys out there. With a state representative telling us that our school district will be losing  $400,000  in funding in the upcoming school year, this doesn't point to a sign that says they can fulfill the basic needs for our sons.

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Parker not doing well

Yesterday was our last day for the NAC drug trial but unfortunately isn't our last day here. Early yesterday morning both boys started throwing up. They took turns every 15 minutes it felt like. After 3 sheet changes we made it into phlebotomy for their blood work.

We were essentially done after eating breakfast; well the boys didn't eat or show any interest. While in the lobby of the PED's clinic talking with other Niemann-Pick Type C (NPC) families Parker had a nice projectile vomit! After that moment he just became more pale than he normally is. Dr. Porter saw him and he was instantly concerned. We brought him back to an examination room where they think he had some type of seizure we didn't catch that made him unresponsive to what they were doing with him. We also found out we were suppose to leave Friday evening not Saturday.

During that time Jennifer's family (Uncle Jim's sister Tammy and family) drove up to visit but I was in the lobby with them during that time when Parker was not Parker. I do remember glancing in the room without knowing what was happening and thinking that Parker didn't look right. We all know Parker how if he thinks your going to touch him he responds!!!!!!!!!

Parker before ICU 4-23-10

Dr. Porter and Dr. Holmes felt that the floor they had him on where Dr. Porter does his protocol work for NPC, wasn't suitable to watch him properly. So they moved him up to ICU on the 3rd floor. At this point it was about 9 pm EST and poor Brisan hadn't even taken a real nap all day. He also was vomiting but he didn't act like Parker did (Brisan seems to be doing well this morning as I write this).

The biggest concern after getting Parker into a room for testing was why his hemoglobin was so low. It was a level 6 which in adults we would be pretty lethargic and non responsive.  His heart rate and pulse were up and his blood pressure was low. The question that remains is where is all the blood going? Jennifer updated me this morning that they gave him a blood transfusion and he is now up to level 9. She said he isn't a 100% but appears to be improving from the low points over the last 24hrs.

We have a scheduled plane ride tonight at 6 pm EST but we are a 100% sure that isn't going to happen. I would suspect maybe Monday we could come home (???). Thank you for all your support and prayers! Truly it is your support that makes things better because alone we are not capable of producing such comfort on our own.

Isaiah 41:10 ESV
fear not, for I am with you;
be not dismayed, for I am your God;
I will strengthen you, I will help you,
I will uphold you with my righteous right hand.

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Trip Update 4-22-10

Today started off pretty early at 8 am EST in the PED's clinic. We did our basic “overview” of the last eight weeks with Nicole (Dr. Porter's nurse). Infectious Disease came up and spoke with us twice.

Still a mystery with Brisan's sinus issues but they wanted to do a few work ups that haven't been performed yet to rule out any additional possibilities from previous conclusions (maybe lack of???). After a long day we finally were able to get a nap inn and polish off the evening eating some Chinese food!

Tomorrow starts off at 8 am EST again to do a tanker size blood draw. Hopefully we can get through that without a hitch. We should be able to meet some other Niemann-Pick Type C patients tomorrow as they are having a noon lunch/ meeting here at the Children's Inn.

Jennifer & Parker at aiport 4-21-10

Parker thinking at airport 4-21-10

Brisan eating dinner 4-22-10

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Today we are heading East to Bethesda!

In about an hour we are leaving to head towards the airport for our last trip for the NAC Drug Trial at the National Institutes of Health in Bethesda, MD. The trial started at the beginning of December 2009.

We'll have updates as we go along but please pray that we have a safe trip today and the boys are able to be comfortable enough to last through the trip.

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Next week we’re heading to the NIH

Parker and Brisan are doing better. Parker seems to be walking in an improved state.  Wednesday Duncan decided to jump on the couch and broke Jennifer's nose. She isn't feeling well and has an appointment with the ENT on Monday to see what can be done to resolve the issue. She posted the x-rays on her Facebook profile page.

April 21-24 we will have our last visit to the NIH for the NAC drug trial. That Thursday will be used to provide insight into Brisan's sinuses and polyps along with the finishing touches of paperwork / examinations they will perform. Nothing major expect 14 tubes of blood! In May we will start Zavesca back up but I am sure we will talk with Dr. Porter more about it to get his insight.

Brisan and Parker's port-a-cath sites are looking good. Monday will be the first time they can access Parker's site for his infusions. They also took Parker off AMICAR and gave him another drug in its place.

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