Progress

He's made some progress in beating this flair up!  Kyin's levels have been so great (platelets 400k, and hemoglobin 12.2 on Tuesday the 4th) that Dr. Kurt has taken him off the Prednisone! He also gets to decrease his blood draws to once a week and infusions every 3 weeks instead of every 2 weeks. This last trip to DeVos went fairly well.  Kyin didn't fight too awful hard when getting his IV.  He also picked out a poke prize and didn't complain too much.  He played Scribblenauts his whole infusion, and even had Miss. Jen's help for a little bit.  

He's definitely 100% back to himself.  He's decided that he wants to play soccer again this spring.  I'm hoping he will enjoy it, and doesn't have a flair up get in the way. 

Going Strong!

I was going to wait until tomorrow to write this blog post, but I can hear Dixon coughing and chattering up in my room so going to bed isn't an option right now.  Kyin's labs came back excellent.  Which is great because he had a slight bloody nose this morning, so that had me a little worried.  He did have to get poked twice today, his left arm just didn't want to give up any blood. He was SO not a happy camper after that.  The lab wasn't very busy so we had lots of helper in the room. One of which was my cousin, Catherine. We work in the same building but never see each other, so it was nice to see her and for Kyin and Soph to meet her.  

Rebecca called when we were on our way home today around 5:00.  She said she was walking out to her car and turned around to call me because she forgot.  She's so awesome.  Because his levels are so great Kyin gets to drop his Pred down to 5mg every other day, instead of every day.

Platelets          387,000

Hemoglobin       12.9

WBC                8,500

He is still getting labs done twice a week, and we're still planning on doing plasma infusions every 2 weeks.  So far, so good!

Plasma Infusion #2...done!

Kyin had his second infusion on Thursday (1/23).  He was a trooper!  I wasn't thinking and put his numbing cream on the insides of his elbows, like I do when he get's his blood drawn-not on the tops of his hands where he would get his IV. They ended up placing it where I put the cream, but his nurse had to do both arms (the IV tube came over half way out of his vein before she got it taped on his arm, and couldn't get it back in).  Miss. Jen, the child life specialist in the Hemat/Onc/BMT area, came in to help hold his arm still. After an enthralling conversation with Kyin about poop and farts (he was giggling uncontrollably-she's very good at her job) she promised him a big poke prize for being so brave and getting two pokes (he maintains he wasn't brave).  He ended up getting his second Lego set from her.  

While waiting for Dr. Smith to come in (Dr. Kurt was out that day), Kyin's social worker at the hospital came in.  She ended up submitting paper work to sign Kyin up for Children's Special Healthcare Services.  It's a co-pay assistance program, she has not doubt that his diagnosis would qualify him for this program, which as insurance EOBs are starting to come in, will be a lifesaver!

His appointment with Dr. Smith was uneventful.  His labs came back a little worrisome for me, although Rebecca (his fantastical nurse) assured me that they were in the normal range.

                                                 Tuesday (1/21)                     Thursday (1/23)

WBC                                              7,800                                    12,370

Hemoglobin                                     11.8                                       11.9

Platelets                                        215,000                                 167,000

I guess it worries me that his platelets dropped so much in just 2 days.  I'm ecstatic that his hemoglobin is staying steady, considering we dropped his Pred down to 10mg a day.  His spike in WBCs with the decrease in Pred also had me a little concerned, but he did get the sniffles yesterday, so I guess he could be fighting something off.

The infusion itself was pretty easy, a lot more relaxed without Max there this time.  The infusion area was super busy, but we were able to find a station.  It happened to be right next to the nurses station. Kyin ended up throwing sticky snowflakes at the window to the nurses station with one of the nurses.  They gave him a little box lunch and his slushy and he sat and played with his Nook for a while, until Miss. Jen gave him his Lego City set.  Then he started putting together his Lego's. 

 Even though we were there for over 3 hours it didn't feel like that, thankfully.  He goes back again for another infusion in 2 weeks.  At that time he will also have an exam with Dr. Kurt again.  I think I might talk to her about having a port placed for him.  If it's an option, I'll let him decide on if it's something he'll want to do.  He just absolutely hates getting his blood drawn and IVs placed, so I think it would take a lot of stress off him.

Jerrad got some great news on Friday-he got his separation date from the Navy!  He will be a veteran of the USN after April 27th!  He should be back up in the mitten on April 29th- just in time to celebrate his 32nd birthday!

Found These Little Gems

Considering it's so hard finding anything on TTP, I was super happy to find these!  A woman on the TTP Support Facebook Group is selling these.  $3 each with .50 from each going to TTP research.

Party of 2..

Yesterday, I got the call. Max has Congenital TTP too.  Not that Jerrad and I were at all surprised about the test results, we honestly would have been surprised if they would have came back negative.  I was however, surprised at how fast the test results came back!  So the plan for Max is yearly exams at DeVos, and a standing order at the local lab if I ever feel the need for him to have his levels checked.  Sophie and Dixon will be tested in the next week or two, but I honestly don't think they have it.

Kyin had his second set of labs today, again they looked great!  Platelets are up to 238,000, Hemoglobin dropped to 10.8 (but he did decrease his prednisone, so that could be why), and his WBCs also dropped to 11,180.  So over all, he's looking great!  He'll still be getting his fresh frozen plasma next week, hopefully that will be all he needs to kick him into full blown remission.  

And I have to say, thank you God that we have medical insurance.  Medical bills are starting to roll in...holy geez. That's all I have to say about those!  I have joined a TTP support group on Facebook, majority of the members have the acquired type though.  Treatment for those flair ups consists of week-two week long in patient stays with plasma daily.  They were talking about their bills for a flair up-$200,000-$300,000!! Talk about sticker shock.  We're very thankful that Jerrad is being medically retired from the Navy and we get to keep Tricare.  

Speaking of Jerrad, he received his numbers from the VA-he got 90%!  That's great, considering we thought he would maybe get 60% and we'd have to fight after he was out to get what we thought he deserved.  We were pleasantly surprised when he got word that he got 90%.  So he'll be home no later than April!

Today's Lab Results

Today was the first day of Kyin's biweekly lab draws that we do here in Muskegon.  Dr. Kurt had written the order with a request that the labs be ran STAT and the results faxed to her at DeVos. I didn't realize how fast lab results can come in! I brought Kyin to the lab in the building my office is at at 8:40 am. His nurse, Rebecca from DeVos called with the results before 11am!  His labs were GREAT!  His nurse seemed just as excited about it as I was!  Have I mentioned how much I love the hematology crew DeVos!?

Anyway, here are his labs:

WBC:  Last week 23,840 Today 17,300- these are still high, but that's to be expected from the Prednisone. 

Hemoglobin: Last Week 8.9  Today 11.1 - That's almost normal folks!

Platelets: Last Week 20,000 Today 206,000- Now, I expected this to be higher because of the infusion

She also said his creatine was better and his sugars were normal!  Prednisone can make the patient's sugars sky rocket if they're not careful.  Luckily Kyin has wanted to eat healthy lately and has limited his sweets intake on his own.

The game plan as of today is that he will go in on Thursday for his second set of labs for the week, then again on Tuesday next week, and then plasma infusion on Thursday.  He gets to drop his prednisone intake to 20mg once a day, instead of twice a day.  Rebecca had said that if his labs keep looking great then they might just postpone the second dose of plasma.  Fingers crossed that it stays looking this way!  

Max's ADAMTS13 enzyme level test isn't in yet. They're expecting it to be in by Tuesday of next week, possibly sooner.  

1.09.2014. The day that it was made official.

Yesterday Kyin and Max had an appointment with Dr. Kurt at DeVos.  I love her and I'm so happy that she is Kyin's hematologist.  It was made official. Kyin does have Congenital TTP.  They ran his labs, along with Max's.  Max's came back great. Platelets and hemoglobin were both where they should be.  She sent out a sample for the test to determine if Max also has TTP.  Now, Kyin's labs were less than desirable.  Pretty much everything was lower yesterday than it was on Friday.  Hemoglobin, platelets, whole RBCs..everything.  So the decision was made to give Kyin his first fresh frozen plasma infusion.  Because his platelets were back down to 20,000 they also gave him a platelet infusion.  He has to have labs drawn on Tuesday, and he goes back to DeVos for more plasma in 2 weeks. 

Game Plan:
Kyin will be monitored via his weekly blood draws, Dr. Kurt has requested to be paged when his results are in to DeVos.  That way she is fully monitoring the situation.  He will be going back in 2 weeks for more plasma.  She is hoping that will kick him into remission.  They'll gage when he goes into remission by his platelets being his and his LDH being low, on it's own.  When that happens he'll be in remission.  Once in remission she's going to administer plasma once a month, and see if that will keep him in remission.  There is a more invasive procedure called Plasma Exchange that involves a central line being place and it would work almost like dialysis, as the blood comes out, gets filtered (plasma out) and then is put back in with new plasma.  We would like to avoid that treatment if at all possible.  I would rather him not have a central line, and I'm sure he wouldn't like to have one either.  Once in remission, he can live his life like a normal 7 year old boy.

One of the first questions I get asked when I talk to people about what Kyin has been diagnosed with is "how are you handling this?" or "how are you?".  I'm okay.  Kyin's okay.  Obviously this isn't something Jerrad and I would ever wish for.  But it's the hand we were dealt.  I am, however, grateful that he has been diagnosed with something treatable.  I'm grateful that it was caught early and he didn't sustain any neurological or kidney damage.  I joined a TTP group on facebook, and to read their stories...it's scary.  Most of these people were diagnosed with the acquired form.  Their prognosis and sometimes treatment is a little different than Congenital TTP.  But, diagnosis is still the same.  Some of these people it took them days, if not weeks for a doctor to take them seriously and to dig deeper.  Because this is such a rare diagnosis many people go in a flair up too long and sustain more permanent damage.  The key to this disorder is to obtain treatment ASAP for a flair up.  This diagnosis is deadly, if not treated.  Even when treated there still is a 20% mortality rate.  Now, that is not something I focus on or dwell over.  Kyin will live his life as much like a normal kid as possible. 
He doesn't know the extent of what's going on, and I don't plan on telling him until he can actually comprehend it.  For now, he knows that he's sick and that he has to go in frequently and get pokes.  He absolutely hates getting poked, but I tell him it's the only way to make him better.

Until next week...