1st BMT – Journal Updates DAY +201 – DAY +250

Twitter Updates: December4-5, 2011

Posted on December 6, 2011 by Nana

Kristy’s Posts: I love waking up to mornings like this!

Just watched Water for Elephants. What a beautiful movie I loved it. A hard journey that led to a happy ending. The passion is inspiring! 

Sleep would be so good!

Liam’s Chimerism Results

Posted on December 6, 2011 by Nana
Mysticlimber13
Liams graft results aren’t what we wanted:( CD3 Tcell 76% donor CD33 10% donor CD56 NK function 40% donor CD19 8% nothing seems 2 make sense 

I have so much anger right now. I’m paralyzed with fear as the reality of this horrible disease really sinks in. U try 2 become emotionless

DAY+ 200 – 11/29/11        DNA Chimerism Analysis – Blood

(CD3+) T Cell                       76% donor origin (24% host)

(CD33+) Myeloid Cells           10% donor origin (90% host)

(CD56+) NK Cells               40% donor origin (60% host)

(CD19+) B Cells                        8% donor origin (92% host)

DAY+ 138 – 09/27/11        DNA Chimerism Analysis – Blood

(CD3+) T Cell                       62% donor origin (38% host)

(CD33+) Myeloid Cells           23% donor origin (77% host)

(CD56+) NK Cells               71% donor origin (29% host)

(CD19+) B Cells                      22% donor origin (78% host)

DAY +28 – 06/14/11        DNA Chimerism Analysis

(CD3+) T Cell                       59% donor origin (41% host)

(CD33+) Myeloid Cells           100% donor origin (0% host)

(CD56+) NK Cells               100% donor origin (0% host)

(CD19+) B Cells                      100% donor origin (0% host)

Chimerism analysis is important for monitoring post-transplant outcome and for determining appropriate therapy after transplantation. Since it is the basis for providing early treatment decisions, chimerism analysis requires sensitive and reliable techniques to detect the presence of donor leukocytes in the hosts’ peripheral blood or bone marrow. As lymphoid and myeloid engraftment is asynchronous, lineage-specific chimerism analysis is necessary. This requires the specific enrichment of myeloid and lymphoid cells prior to performing chimerism analysis (e.g. by PCR-based detection of donor- or recipient-specific mini-satellite DNA sequences).http://www.stemcell.com/en/Products/Area-of-Interest/Chimerism.aspx

Chimerism in nonmalignant diseases

http://www.nature.com/bmt/journal/v35/n2/full/1704715a.html

Allogeneic stem cell transplantation is the only curative treatment option for many patients with inherited or acquired nonmalignant diseases as thalassemia, sickle cell disease, immunodeficiency diseases, osteopetrosis, storage diseases, severe aplastic anemia, bone marrow failure syndromes and others (for a review, see Section IV in Thomas et al34). The aim of the procedure in these diseases is to achieve sustained engraftment to (i) improve the hematopoietic function, to (ii) correct the immune competence and/or to (iii) increase or normalize the respective enzyme shortage. Therefore a priori, it is not necessary to replace the recipient hematopoietic system completely. The implementation of a state of mixed chimerism is mostly sufficient to substantially improve the patient’s well being. Thus, to reduce toxic side effects, most conditioning regimens are less myeloablative and thereby mixed chimerism is more likely.60,61,62,63 As a consequence, graft rejection or nonengraftment remained the major causes of treatment failures in this diseases. Sensitization to minor histocompatibility antigens by prior blood product transfusion might increase this danger. The rapid development of complete chimerism in NK and T cells seems to play an important role in achieving sustained engraftment especially in patients who were treated with a dose reduced conditioning regimen.64,65,66,67

http://www.nature.com/bmt/journal/v35/n2/fig_tab/1704715t1.html#figure-title

 Pediatric Transplants

Increasing mixed chimerism and the risk of graft loss in children undergoing allogeneic hematopoietic stem cell transplantation for non-malignant disorders

http://www.nature.com/bmt/journal/v42/n2/full/bmt200889a.html

http://www.nature.com/bmt/journal/v42/n2/pdf/bmt200889a.pdf

Please say a prayer for Little Ryan’s Family

Posted on December 6, 2011 by Nana

Terri Smith posted in Liam’s Journey – HLH Warrior.
10:27am Dec 6

Our hearts are broken over the loss of Ryan and our hearts and prayers go out to his mommy and daddy , Holly and Dusty-xoxo He had just turned one, on the 9th and was 127+ post BMT, very unexpected, he had surgery to replace his hickman line yesterday due to infection. Not sure exactly what happened but know prayers are much needed. His family is from Oregon and need our prayers. You can write a note to his family on his caringbridge site.

http://www.caringbridge.org/visit/ryandavis
CaringBridge / Ryan Davis / Welcome

♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥  ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ ♥ Our hearts are broken, I’m in such shock. Holly Davis we are sending our love and prayers. ♥ ♥ RIP Ryan

Baby Ryan earned his Angel Wings last night…. 12/5/11 ;’(
Ryan had HLH, and had his BMT on July 29th, 2011
http://www.facebook.com/pages/Team-Ryan/134303483323180?sk=info
by Holly Davis (mommy)

DAY +209

Posted on December 12, 2011 by Nana

Kristy’s Twitter Update: I spoke with Liam’s dr in Seattle & he said not be worried that his t-cell has went up & that is most important. They will check again n Jan     

DAY +211 – Happy 15 months to Liam!

Posted on December 12, 2011 by Nana

Update from Kristy – Saturday at 10:16pm – December 10, 2011

Happy 15 months to Liam!

You amaze me every day and feel my heart with so much happiness.

      Wearing Papa’s hat!

DAY +218

Posted on December 19, 2011 by Nana

FB Update from Kristina: Saturday, December 17th, 2011
Liams next appointment is on the 20th. We will discuss removing his Hickman when we go to Seattle. They will draw an IGG level to see if he will need IVIG if he does he may not get it removed. His level has been holding it’s own for a couple months so that is good!
Today he broke another tooth through it was on the top and it was bleeding pretty bad which scared me. I don’t remember the other boys having such a hard time with teething but maybe it’s due to all he has been through.
There is nothing in this house he won’t climb! Im really considering removing all furniture!!!
Liam and Ryder are quite the little partners in crime. They are best friends as Ryder says and are so excited to be together.
Liam loves to watch Noa and Hunter from the window as they ride circles around the house on there dirt bikes. He gets so excited he shakes. I can’t wait until they can all play together outside.

DAY +221

Posted on December 21, 2011 by Nana
Kristina Smith posted in Liam’s Journey – HLH Warrior – Facebook Group.
http://www.facebook.com/n/?profile.php&id=1299260376&mid=55b7ecbG21d5b705G781a8abG96&bcode=sTizGCWN&n_m=marcar1980@aol.com
10:50pm Dec 20, 2011
Liam weighs 21lbs 4.6oz so he is still going up in weight :) he is now in the 10% which is great because before he wasn’t even on the chart. His labs looked good but his ALT level fluctuates up and down I wish it would just stay in the normal range. Maybe with time it will.

DAY +223

Posted on December 23, 2011 by Nana

Mysticlimber13

Liam is scheduled 2 have his hickman out on Jan.17 I’m so nervous but excited @ the same time keep him in your prayers 4 no complications

DAY +237 The Hickman has a crack in the line

Posted on January 4, 2012 by Nana

Update from Kristy – 3 January 2012
So I went 2 flush Liam’s line & it squirted saline on my hand. He has a crack n his line & I’m panicked we go n 2morrow pray 4 no infection

 

Update from Kristy – Wednesday, January 4, 2012
I just talked to Seattle they said they recommend that Portland takes Liams Hickman out. So now just waiting 2 hear from Portland.

The scary thing with a hickman is that it leads straight to there heart so if any bacteria was in or on the crack when I started to flush it could be a very big problem for someone who has had a BMT or is on immune suppression. So needless to say I didn’t get much sleep last night. Checking Liam for a fever. He has not got a fever yet. But like they said there could be something growing in the line. I pray to God that’s not happening. A little boy in Seattle had a BMT for HLH and got a crack in his line and it took his life so it is scary. Please pray for no infection and no complications.

January 4, 2012, 11:05 AM
So we are headed in I guess they r going 2 repair it today & take it out tomorrow I wish it could just b done today

      Our Little Warrior heading to the Clinic today

3:26 PM 1/4/2012 Update:

I am so pissed we sat and had to hold Liam down for two hours so they could repair his line. They repaired his line and while repairing it, it broke again. I ask can’t you just call and get it out. They him and ha then we get sent to the surgery office and wait in the waiting room forever and then get put in a room while waiting I call Seattle they say to me the line needs to come out now. Have the dr call us so we can talk to them. So the dr comes in and I tell her Seattle wants her to call them and she refuses and tells us that Liam will get this line out within the week. I am about ready to get up and walk out and tell them that if Liam gets an infection because he has to wait to get surgery. The nurse trying to repair the line was like he’s going to need antibiotics and was very concerned about him getting an infection. The medical system is so screws up it’s not fair that you as a patent can’t call the shots they just refuse and basically tell you to bad. Wonder what would happen if it was there child????

DAY +238 Hickman Line removed today

Posted on January 5, 2012 by Nana

Kristy’s updated her FB status: 1/5/2012 3:25:30 P.M. Pacific Standard Time
“We are on our way home! Liam is doing good. I’m very excited to see him with no line he’s had something hanging out of him since he was 28 days old so it is nice. I hope he never needs another one. Thank you for all the support it sure does help going through this to know so many care.”

DAY +243 Clinic Apt. today

Posted on January 10, 2012 by Nana

Kristina updated her status: Monday, January 9, 2012

Can’t sleep don’t know if it is cause Liam will get poked tomorrow or because they are testing his graft? I pray all is well HLH sucks!!

 Kristina updated her status: Tuesday, January 10, 2012

Liam is 16 mons old 2day What a rough way 2 start life but what a warrior 2 still b here he inspires me everyday & I’m so blessed 2 have him

Liam is 21lbs 14oz still behind kids that are younger than him but I think he looks really good!

Blood draw was no fun :( but he did good!

Kristina Smith updated her status: January 10, 2012, 2:01 PM

“Labs looked good! Now we just wait for the results of his chimerism test (graft). Sun is shinning life is good.”

DAY +245 Happy 8 months post BMT

Posted on January 12, 2012 by Nana

Kristina updated her FB status: “Happy 8months post BMT 2 my little warrior Liam I am so proud of him His strength & courage inspire me & his brothers selflessness melts my heart. I’m one lucky momma!!”

DAY +246 Liam driving…

Posted on January 14, 2012 by Nana

DAY +246

Posted on January 14, 2012 by Nana

Kristy’s FB Update: Liam fell asleep early tonight & all I can do is worry I think why did he fall asleep early is he sick or losing his graft that’s a BMT mom

DAY +249 Liam looking at the snow

Posted on January 17, 2012 by Nana

Best friends, Ryder and Liam

Posted on January 17, 2012 by Nana

 

 

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