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.
Jackson looking like we woke him up at 5am, which we did.
