Brisan and Parker Stults | Living with Niemann-Pick Type C

Archive for the ‘Drug Trials’ Category

If all goes well we should be coming home tomorrow afternoon. That of course is a bold statement but Parker appears to be improving. His hemoglobin level is 9.7 and his hemocratic level is 30 (H&H). When he was at his low point his hemoglobin was like 6. They also have him on antibiotics which they need to observe for 48 hours before they can release us if he is doing better.

They are moving Parker out of ICU to the PED's floor which is more comfortable than were they are at now.

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!!!!!!!!!

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.

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.

We arrived safely in DC yesterday afternoon on a beautiful rainy day (not really). It seems every time we come it is either raining or snowing. Today's weather will be in the 60's so that should be good!

Overnight Parker had a bloody nose. We love leaving DNA behind when we come to visit. Today's mission is to see if they can help us with a solution with Brisan's sinus issues. Most of you that have seen him can see that his right eye is becoming severely infected because of the polyps in his sinus cavities.

We also get to meet some other NPC families which will be exciting!

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.

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.