Friday, August 27, 2010

Jackson and his port have parted ways....

It's true after 12 months together, 48 chemo infusions, (1x4=4 4x12=48 48x4=192)  a lot of accesses, (math is hard), and no infections, (good job port and Jax)  Jackson and his port have decided go their separate ways.  I'm o.k. with it.  We went to Seattle last night, ran into rock blasting on the pass(of course), and got to the Hospital early this morning.  They did surprise us by announcing they would include a spinal tap in his port removal surgery.  We have wanted this procedure because Dr. McClain is finding clinical ways to track the progress of ND CNS LCH by analyzing the cerbrospinal fluid.  So he had his port out, spinal tap done, and came home.  We had been warned by another histio Mom to move slowly and stay flat after the spinal tap and we did, Jackson was pretty much flat for 6 hours today, and no spinal headache.  Worth it, from what I have heard you do NOT want a spinal headache.
Jackson looking like we woke him up at 5am, which we did.
Back from the recovery room.

Michael and I can always tell they haven't replaced him with a clone by the way he itches his nose when he has been under anesthesia!
Tiffany and Bailey (neighbors and friends) brought Jax Royal Knight balloons and a football which they had the team all sign--I am sure that took plenty of time and effort--these kind acts always bring me to tears.  And the flowers are for little ole' me ,courtesy of the Tiffany & Bailey team too. 
Grateful today for:
1.  Ports to deliver my son medicine necessary to prolong his life and quality of life.
2.  Doctors and nurses who are willing to study and go to school for years, and then work long hours to save lives and improve medical techniques.
3.  Friends who don't forget about us month after month after month
4.  Family who are always there to pick up the pieces-and the children. 


Thursday, August 26, 2010

The final farewell to summer vacation---AND The first day of school 2010 style

If my blog is like a book, today's book will read like an illustrated children's stories.  Heavy on pictures, light on text.  And Brenda I don't know why some pictures are lit right and some are not, they were all taken within seconds of each other from the same spot.  Maybe teenagers have a natural darkness about them---ohh I'm spooking myself out now!




The day before school started there was one last "pond day".  Lunch and swimming with friends was on the schedule for this soiree.  This is a picture of  Gracee, Zoe, and McKayla---now SECOND graders.  I remember when all these babies were born.

The kiddos had some game going, I never quite caught on to it--sometimes you sit on the pipe, sometimes you jump in, sometimes your thrown in.  Oh well, as long as they understand.
When we got home from the pond party it was showers and pre-first-day-of-school-spa-day.  Zoe obviously NEEDED this firming mask, especially on her lips!
And I think Sierra was getting her hair done did while Zoe firmed her 7 year old wrinkles.
Then it was time for the back to school dinner.  We used to just have a special dinner then Michael would give the kids blessings.  But I got an idea from another blog last year of having a family theme for the school year--something to focus and work on.  Last years was "We can do hard things".  I think we did too! I know Heaven helped us, usually through many of you.  Now looking back makes me realize what strength and depth my children have.  I'm a lucky Momma.  
Bless you Dollar Store.
Can you guess this years theme?  I'm not telling!
Do me a favor, next time you see Jackson, or Sammi, or Sierra, or Zoe, or Carter ask them what their family theme is this school year!
Babies first.  (He can't read -- next year I probably won't be able to put baby safely by his name!)  This sad looking little Carter was up at 6:30 am grabbing my face asking how many more Spongebob's till Kindergarten starts.  I sure hope Mrs. Mueller teaches him to tell time---and not by T.V. shows--- real time.
Now for the baby girl (I'm safe she likes to be called baby girl).  Zoe is beginning second grade today.  She even let me curl her hair, I hope Red Rock enjoyed it while they had it.
When I grow up I want to have the style Sierra has always had.  She is starting fourth grade today.  A few more weeks and this chicky will be 10!
Ummm Sammi has a certain "style" about herself today too.  Top of the Totem pole this year--Eighth grade.  Wish the lighting was better she is giving you all quite the look.
His Royal Highschoolness, Jackson.  All hail the freshman.
Looking like nice normal Noftle's--in the shadows of course, I might want/need photography lessons for Christmas.
More children in the dusk.  I just added this one to make Brenda sigh/cry--hey don't judge me, she was all braggy about being at Disneyland today.  People stuck in real life, taking poor quality pictures of their kids, need some revenge.
I thought it was cute how Carter's backpack took up a large percentage of his body! I am easily amused.
Our neighbor/Sammisitter Bailey and Sammi--these two didn't spend very many days this summer apart.
The always popular Red Rock sign photo op. 
Carter and his Kindergarten teacher, Mrs. Mueller.  This smile showed up several times today--is this his "schoolboy look"?
Zoe and the wonderful Mrs. Vermeer.  Can't wait to see if  they are both smiling nine months from now!
Sierra and Miss. Hill. We are experimenting with nepotism this year.  Miss. Hill is Grandpa Kents' cousins daughter---I'm not sure Sierra knows that though.  Hope there isn't a family tree test soon.
Another popular photo op for the Noftle children--Grandma's desk.  That cool schoolboy smile on Carter again.
Kindergarten orientation.  Or, mini MTC, as Michael calls it.   Cool smile still there?? Check.
After we parents were "oriented" we got to sneak into the Kindergarten classrooms to see they hadn't corrupted the offspring yet.  Carter was cracking up at the book, "Don't eat the teacher".  Words to live by--you see he is learning life skills already.
Let's end the day with some cutie wooties--Sierra and Abigail,  and don't miss their flower headbands!

Everyone came home happy and excited.  Mr. Kindergarten himself,  Carter, is spending the night at Matt and Courtneys', the girls with Mom, and Michael and I are heading to Seattle with Mr. High school--tomorrow's big soiree is Jackson's port removal surgery, one party after another around here!

Saturday, August 21, 2010

Houston trip, the unabreviated edition!

We are so grateful for the prayers, good wishes, and genuine happiness over Jackson's latest news---Thank you.  We are planning a big "Chemo done, time for fun" party and if you read the blog, or brought a meal, or watched a kid, or sent a happy thought, lit a candle, prayed and prayer, painted a house, gave a hug  whatever, then you are invited!! (date and time pending upon Royal Football schedule!!)
 We got to Houston Saturday night and the three of us stayed up until 2am Texas time, just too wound up to sleep I guess.  The next day we slept in and looked forward to seeing Sister Katelyn Christensen and her companion.  I used to babysit Katelyn and we are neighbors with her family so it was fun to kidnap her and her companion for a couple of hours.  I've seen her, hugged her, and can honestly report she is doing great.  She seems so happy and appears to be thriving and loving her mission.  Sunday night Michael and Jax were snoring soundly and I was enjoying some understandable insomnia.  We had to wait at Dr. McClain's office for almost an hour before he came in.  However, when he is with us we feel like he is in no rush and can stay with us as long as we want---I don't know how he does it.  I've already told you all that Jax is done with Chemo, just yearly MRI's, BAER's, and Spinal Tap's for now.  In the last 12 months since we last saw him they have discovered that with active ND CNS LCH there is demylanazation of the mylan sheath that covers the nerves connections (I reserve the right to bad spelling and medical terminology) and that this in turn shows up as extra protein in the cerebrospinal fluid.  The tricky part is at different ages of life the protein in the cerebrospinal fluid fluctuates so there has been some figuring out  what the normal ranges are for the different ages.  Potentially this could be very helpful in Jackson's future.  A yearly spinal tap could possibly detect the disease is becoming active again before he becomes symptomatic and more damage is done.  Already our Doctor in Seattle is warning us we will have a fight on our hands to get the spinal taps done---funsies.  Dr. McClain and Dr. Allen have also published recently in the journal of immunology about all of this and I haven't taken the time to look up the article yet, but I doubt it will have cartoon drawings so I may just have to sleep with it under my pillow in order to gain a better understanding of what is happening!  But for now Jackson is happily enjoying 2 a day football practice, has sore muscles and is anxiously awaiting the port removal next Friday (the 27th) so he can get in there and hit someone! He is tired from his Chemo last week, but so excited to be looking at a Chemo free 9th grade year that he doesn't even complain about the fatigue.  He did mention tonight that he wished he could have his port out before school starts, but the Hospital couldn't squeeze him in before Friday.
Smiling and hoping this was the last Chemo
The 48th dose of Cytarabine---man that sounds like a lot.
Jackson in the Houston airport Aug. 2010
(if you like there is a picture of Jax from Aug. 2009 in front of the same cow it's in the Sept. 09 file I couldn't figure out how to paste it in here!)
With the wonderful Dr. McClain--by the time we got home we already had an e-mail from him saying how much he enjoyed seeing Jackson again and reminding us of how to contact him if we had further questions or concerns.  It's the great customer service in Houston that keeps us coming back!!
I thought it was funny how both of them wandered over to Bessies behind to make their calls!
See--wrong end of the cow again, like father like son.


Here is something from the Texas Children's Cancer Center site that explains a little about Dr. McClain and Dr. Allens' research.


Principal Investigators

Kenneth McClain, M.D., Ph.D. and Carl E. Allen, M.D., Ph.D.



The Research Challenge

Langerhans Cell Histiocytosis (LCH) is a rare disease of white blood cells in which an overabundance of abnormal dendritic cells causes damage to the skin, bone and other organs. The disease affects both children and adults, with peak incidence occurring in children between 5 and 10 years old. The incidence in children is approximately five per million, and in adults, approximately half that. Because LCH is rare, few physicians have experience in evaluating and treating it; patients and their families must often see physicians who do not give timely or proper evaluations and treatment.



For those affected by it, LCH is an exceptionally frightening and sometimes disabling experience. It can manifest in an alarming variety of ways: as a scaly rash that does not respond to treatment; as bone pain, headaches and severe leg pain; as severe gastrointestinal pain, vomiting, diarrhea, bleeding from the esophagus, weight loss and failure to thrive; as swelling and inflammation in the mouth, face, ears, scalp and lymph nodes; and even as mental deterioration. Although most patients with bone, skin or lymph node involvement survive, they can suffer multiple recurrences and face long-term disability.



Unlike other rare white blood cell disorders, such as acute myelogenous leukemia or lymphoma, the cell of origin and causes of LCH remain unknown. Despite clinical trials conducted by the Histiocyte Society, many fundamental questions remain unanswered regarding the biology and treatment of LCH. Few significant scientific studies have even attempted to identify basic biologic characteristics of LCH. Most importantly, there have been no systematic studies of LCH that provide the fundamental knowledge essential to developing effective targeted therapies. Additionally, we lack the knowledge necessary to identify patients who, at the time of diagnosis, might require more intensive treatments.



One of the most important challenges we face is identifying those genes responsible for the development, growth and dissemination of LCH. Over the past 20 years, only a limited number of genes have been studied for their role in LCH. Most of these studies used techniques that, in retrospect, could not have yielded accurate results.



Now, using newly available, advanced technologies, we have the capability of assessing gene expression in LCH in a highly sophisticated and comprehensive manner. Data, obtained over the past two years from ongoing studies of cell-specific expression of more than 45,000 genes, have significantly changed our view of the biology of LCH. We have identified new genes important to the pathogenesis of LCH that regulate lymphocyte movement and other molecular pathways not previously associated with LCH. We are poised to use this data to further understand the genetic abnormalities that cause LCH and thus to better identify effective targets for treatment.



Advances in our understanding of LCH will help us understand the biology and treatment of the other histiocytic diseases.



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The Histocytosis Research Program at TXCCC/HS

Despite decades of research, the origin of LCH remains speculative, and survival of patients with severe disease remains poor. The overall goal of the LCH Research Program at Texas Children’s Cancer Center is to comprehensively identify essential genes critical to the growth and development of LCH. By knowing the genes, we can understand the key proteins that regulate cell function and the pathways they control. This knowledge will lead to a complete understanding of how LCH lesions are formed and how they progress and, ultimately, to better treatments and a cure for this disease.



Our LCH Research Program has four main areas of endeavor:



Comprehensive Analysis of Cell-Specific Gene Expression in LCH Tumors:

This project is the core of our research program. It is based on the idea that we cannot improve treatment of patients with LCH until we understand the fundamental nature of the cells that comprise LCH lesions. These lesions can arise in almost any organ system, and their severity can range from a rash to systemic disease that obstinately resists chemotherapy.



Data from our cell-specific microarray experiments do not support the current concept that LCH arises from clonal proliferation of activated-immature Langerhans’ cells (LCs) from the skin. We propose an alternative hypothesis that LCH tumors arise from pathologic myeloid dendritic cells that affect lymphocyte activation, migration and accumulation.



Developing a Comprehensive Genomic Database:

We also plan to develop a comprehensive genomic database for LCH, JXG, RDD, ECD, and malignant histiocytosis that will be used by Texas Children’s investigators and also by collaborators from various institutions in the United States and around the world. This genomic database will help identify novel therapeutic targets based on the genetic changes unique to histiocytic diseases.



As the largest LCH clinic in the world, and as the tumor bank for a consortium of LCH clinics performing basic biologic studies in the histiocytic diseases, Texas Children’s Cancer Center is the logical choice to lead this project. We intend to recruit an additional investigator to help us more rapidly achieve our research goals.



Developing Unique Models for Preclinical Drug Screening:

A significant obstacle in developing novel therapies for LCH has been the lack of a preclinical model that accurately mimics LCH in humans to predict clinical response. The large number of samples acquired by our laboratory now makes it possible to attempt development cell culture and animal models that may be used to study disease progression and test therapies.



Implementing High Throughput Therapeutic Drug Validation Strategies:

Once we have identified potential drug targets using the gene profiling studies described above, we will perform high-throughput studies to validate these possible targets. For this crucial step, we have the special advantage of being able to use highly sophisticated technologies now available to us through the recent establishment of the John S. Dunn Gulf Coast Consortium for Chemical Genomics. Using this state-of-the-art facility, we propose to use siRNA approaches to independently validate the function of the candidate genes and pathways by either targeted inactivation or by using whole genome siRNA libraries.



We will also use the chemical genomics approach as part of the target validation process. The compounds identified at this stage could be used both as chemical probes to further dissect the pathways and as potential lead compounds in the subsequent drug development phase of the project.



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Participate in Histiocytosis Research

The TXCCC Histiocytosis Team is dedicated to studying Histiocytic Diseases in order to identify the causes and discover cures. If you or your child is undergoing procedures (surgical biopsy, blood draw, lumbar puncture) for clinical purposes, we invite you to participate in Histiocytosis Research. This research requires tissue samples from patients with Histiocytic Diseases including Langerhans Cell Histiocytosis (sometimes called Eosinophilic Granuloma), Juvenile Xanthogranuloma, Rosai-Dorfman Disease, Erdheim-Chester Disease, Hemophagocytic Lymphohistiocytosis, and Malignant Histiocytosis. These tissue samples provide vital information that will assist the TXCCC Histiocytosis Team in understanding the basic causes of histiocytosis which hopefully will lead to more effective treatments.



What is needed for this research?

Surgical biopsies The current research is focusing on an effort to isolate pure populations of cells fresh or frozen biopsy specimens to discover which specific genes are causing Langerhans Cell Histiocytosis and related diseases.

Blood samples White blood cells can be isolated and analyzed in order to determine which circulating cells and genes contribute to Histiocytic Diseases. The plasma fluid in blood can also be analyzed to determine which proteins are important in diagnosing and treating Histiocytic Diseases.

Cerebrospinal fluid CSF is the fluid that surrounds the brain and spinal cord. The Histiocytosis Team studying proteins that are involved in Histiocytosis-related nerve problems.

Medical information Medical details associated with the biology samples will help determine the clinical significance the genes, proteins and cells identified in the Histiocytosis research studies.

Thursday, August 19, 2010

Houston trip=No More Chemo!

That's right after 48 Chemo treatments Jackson is done!  Just like he wanted, starting High School with no monthly Chemo distractions.  I am thrilled for him and will give a complete Houston/Doctor/Katelyn update soon.  But right now I have some children who haven't seen me in 6 days, and I am demanding their attention.  And big doings tomorrow---school clothes shopping with Sammi and some other brave souls!  If I survive, then for sure this weekend will see me catching the  blog up!  Thanks for the good wishes and prayers.

Friday, August 13, 2010

Chemo round 12 day 3

Today Sierra and I were thwarted in our attempt to get our nails done did.  But after a promise of a couple more minutes that stretched into 60 minutes of waiting we left "welcome nails" grabbed Jackson from the hotel and headed over to "Sea Top" nails where we barely had time for Sierra to be fancied up.  She was grinning so I feel it was time well spent, despite what her Dad says about throwing money away!  Next we went to Chemo.  Jax and Sierra were able to claim the x-box today (first time in 47 chemo's!) in our infusion room and they had fun with that.  Then we took Sierra to the sibling play room and headed over to audiology.  We discovered that our oncology scheduler and the audiology department had miscommunicated and audiology did NOT have the time to do a BAER test today.   But he did the preliminary 1 hour hearing test and if Dr. McClain wants us to get another BAER we can schedule one when we get home.  This put us on the road at 4:30 which was better than we thought, but with weekend traffic we still only made it to Cle Elum by 7:15.  We only waited a few minutes before Ryan and Lucy brought Michael to us and took Sierra from us---thanks Ryan!  We are back in our room now, we just medicated Jackson so we should be in for a quiet evening.  Good thing -- I am long overdue typing up the primary program!  Tomorrow may be our LAST chemo--I'm saving the medical bracelets just in case!!  We won't be taking the laptop to Houston but we will post on Tuesday to let you all know what Dr. McClain said.  Feel free to call us if you just can't take the suspense!  As always please keep our boy in your prayers--Thanks.
Sierra getting treated right--finally!
(That was a dig at YOU Welcome Nails)

Blurry but this is Jackson during his hearing test--he passed!

Thursday, August 12, 2010

Chemo round 12 day 2

Enjoy the pictures knowing your not drunk but that my camera is struggling--at least the cars all work! (knock on wood please)

This morning Sierra and I went to Target and wrapped up her school clothes shopping while Jackson rested at the hotel.  Then we drove the looooong way to chemo, 45th street still under construction, and per Sierra's request we ate lunch at the hospital cafeteria which Jax barely tolerates.  He is so over the cafeteria in the hospital, but the other kids think it is so fun to carry their trays around picking and choosing from the plethera of options.  We went back to our room for a few minutes then headed to Bainbridge Island (Poulsbo) to see my friend Julia who just moved there with her family.  We were supposed to catch the 4:45ferry over to the Island and we were there at 4:15 but the ferry filled up before we got even close.  So we cooled our heals and read until the 5:30 ferry (which meant 5:50 in real time!!  Ferrys' don't tell time very well) actually Jax and I read ,Sierra bounced.  It felt like Tigger was in the back seat.  We enjoyed the ferry ride and the drive over the Island to Julia's house is gorgeous.  Their home is on the Liberty bay and we ate outside by the water---I'm newly inspired to fix up my yard--(how do I add the ocean view?)!  I forgot to take any pictures at Julia's;  oh well, I'll just have to go back someday soon, maybe when she makes another blackberry pie!  We caught the 9:45pm ferry (10:00 real time), but we thought the ferry was fun, so no complaints here. Then once back to Seattle it was home to the hotel.  Sierra never stopped talking they WHOLE day, I remember Zoe doing the same thing when she went on her 'chemo" trip---maybe it's having almost undivided attention (Jackson certainly doesn't interrupt) from me that makes them want to use up the rare chance to have no competition for my listening skills.  I really feel like i am available at home whenever they want but by the way the  both unload maybe I need to make some concerted effort at special one on one days in the future.  That being said---It is not surprising I am losing my hearing sheeesh!  They have added a BAER test tomorrow afternoon 3:15 and it's a 3 hour test so it will be later that I trade Sierra for Michael tomorrow, Sierra is thrilled though to get more time at the hospital.
Ferry riders, we felt pretty "big city" catching the ferry at 10pm.
Camera is getting worse--but pretend it isn't blurry and you can see the space needle all lit up and pretty.
Sierra wanted me to take this one of a ferry going the other direction. 

Wednesday, August 11, 2010

Chemo round 12 day 1

Round 12, ROund 12, ROUnd 12, ROUNd 12, ROUND 12!!!  It is possibly our last round of chemo, I'm really trying (ignore the first fragmented sentence) not to think that we are done, I don't want to be disappointed and especially don't want Jackson to be disappointed but the facts are there is a chance that this is our last round.  His ANC was sky high today (not abnormally high--just Jax high) 2449 WBC 4.7 PLT 336 and HCT 37.6.  So Sierra, who is this months winner of a trip to Chemo, enjoyed the sibling playroom and Jax got his treatment.  Then we fought traffic bravely and met my Aunt DeeAnn, Kyle, Heidi, Kate, Preston, and baby Lindsey for dinner at Olive Garden.  Yummy!  I'm referring to the baby, of course, she is 1 week old and soooooo cute.  Sierra and I were in a tug of war over her.  I took some pictures of us, but I took them with my fancy, new, too hard for Becky's brain, phone.  I sent them to my email and they got lost "somewhere out there".  If they ever come home I will post them!
However I will post the pictures I did just find, not sure who the photographer(s)are but they are pretty good!  Plus dolls really freak Michael out....
Something on the ground has their attention.
Sisterly love.
Have you met Rebecca?
Rebecca enjoys gardening,
daydreaming under a shady tree,
playing hide and go seek with her BFF's,
hosting the coolest slumber parties,
and entertaining Sierra and Kathryn.
Rebecca is also a loving friend,
and apparently quite the jokester.
Aha--I found them from "somewhere out there"! 
All of the crew at dinner.
You might not be able to smell it through the computer, but that Lindsey smells so good and newbabyish.