March 2007 Archives
Dear Charlotte and Roy,
We want to express our heartfelt sympathy. May the love of family and friends comfort you.
Paulina & I wanted you to know that we are grieving with you and thinking of you always.
Jack's battle against the NEMO primary immune deficiency has inspired many and will inspire many more.
We want you to know that we are here for you.
Sincerely,
Andres & Paulina Treviño
Please say a prayer for Jack.
Jack had his first transplant a year ago. More about Jack's story here and here.
This from Jack's mom blog last Saturday:
He is still having breathing problems and is still on oxygen. This pneumonia is really kicking his butt. The doctors have been giving him doses of Albumin (protein) with Lasix (diuretic) chasers to help reduce the fluid in his lungs as well as any other place that he really doesn't need it. By mid week we found out that the peumonia is not bacteria but virual and was identified as Para Influenza, The FLU!!!! Not for anything, but when Jack gets something he has to get it good. It's been a little scarey at times watching his respitory counts (breaths per minute). Normal respitory rate is about 20 breaths per minute and Jack has been as high as 80 which is not good.
And this is from Jack's Mom blog yesterday:
Roy and I are asking once again for everyone to keep Jack in their prayers and to even say an extra one. Yesterday marked a year from the first transplant and unfortunately he was moved into the Intensive Care Unit for assistance in breathing. We are praying very hard for God's healing touch and to give us all strength.
www.cure4jack.org
www.vic4andy.com
Good morning lads hope all is well after your 21 miles yesterday i completed it and it was a lot easier than i had expected or should i say that it was not easy but it was not as difficult as i had feared being that i have had a bad year training for this years run but i am glad that i will have both of you helping me in my times of difficulty as the three musketeers would say one for all and all for one we have all the hard part done now and we are on the last legs of the 2007 Boston marathon and all is left is that little run from Hopkington to Boston on April 16 we will definitely have a blast i am looking forward to meeting in copley and we will be all smiles and delighted to have been part of this great experience together see you all soonMark
www.mark4andy.com
Thirty five times around the sun and this is the first time that I run (and also walk for a little while) for 21 miles.
Twenty one miles. T w e n t y o n e m i l e s !
Unbelievable.
Thats 33.79 km. Thirty three point seventy nine kilometers.
I managed the patelar tendonitis and my left knee did ok.. I changed shoes from Nike to Brooks and that helped. I had 6 packets of GU. And I finished in a little less than 4 hours.
Here is the route.
Here is the map:
Very interesting article at Nature about NEMO and GI problems:
Epithelial NEMO links innate immunity to chronic intestinal inflammation
Deregulation of intestinal immune responses seems to have a principal function in the pathogenesis of inflammatory bowel disease1, 2, 3, 4. The gut epithelium is critically involved in the maintenance of intestinal immune homeostasis—acting as a physical barrier separating luminal bacteria and immune cells, and also expressing antimicrobial peptides3, 5, 6. However, the molecular mechanisms that control this function of gut epithelial cells are poorly understood. Here we show that the transcription factor NF-B, a master regulator of pro-inflammatory responses7, 8, functions in gut epithelial cells to control epithelial integrity and the interaction between the mucosal immune system and gut microflora. Intestinal epithelial-cell-specific inhibition of NF-B through conditional ablation of NEMO (also called IB kinase- (IKK)) or both IKK1 (IKK) and IKK2 (IKK)—IKK subunits essential for NF-B activation7, 8, 9—spontaneously caused severe chronic intestinal inflammation in mice. NF-B deficiency led to apoptosis of colonic epithelial cells, impaired expression of antimicrobial peptides and translocation of bacteria into the mucosa. Concurrently, this epithelial defect triggered a chronic inflammatory response in the colon, initially dominated by innate immune cells but later also involving T lymphocytes. Deficiency of the gene encoding the adaptor protein MyD88 prevented the development of intestinal inflammation, demonstrating that Toll-like receptor activation by intestinal bacteria is essential for disease pathogenesis in this mouse model. Furthermore, NEMO deficiency sensitized epithelial cells to tumour-necrosis factor (TNF)-induced apoptosis, whereas TNF receptor-1 inactivation inhibited intestinal inflammation, demonstrating that TNF receptor-1 signalling is crucial for disease induction. These findings demonstrate that a primary NF-B signalling defect in intestinal epithelial cells disrupts immune homeostasis in the gastrointestinal tract, causing an inflammatory-bowel-disease-like phenotype. Our results identify NF-B signalling in the gut epithelium as a critical regulator of epithelial integrity and intestinal immune homeostasis, and have important implications for understanding the mechanisms controlling the pathogenesis of human inflammatory bowel disease.
This from Simon's mom:
We thank God everyday for the engraftment of 99% and keep praying it holds strong and steady.
We had some of Simons vomitting issues come on strong the last two days with vomitting to the point of dry gagging while I hold him over the sink first thing in the morning.
.....
We are so thankful to be at the place we are with Simon. He is doing well with numbers and things and very spirited and busy, running all around and keeping up with the girls and chattering alot. Its so nice to see him so alive. We pray for continued healing and that we may soon be able to venture out into the world and let him see faces besides our own. We continue to pray, for Simon and all the boys who we have come to know and their families.
>
We're hoping for no fevers for JT and the calcium issue to resolve soon.
This from cure4treebeard.org:
Tomorrow is another big day. We need for JT to tolerate his feeds and be completely fever-free. We also need the calcium numbers to stay down on their own. If all of that happens, we may possibly be discharged on Wednesday to care for him back at the apartment and they’ll follow him on an outpatient basis. However, if he spikes a fever tonight, we’ll be back at square one. We’re both tired. Please pray for strength and perseverance for JT, that he may continue to run his race well.
This from Jack's Mom blog:
Jack has pneumonia. This is not a result of the transplant however his medication is suppressing his immune system and that could be part of the reason why the cough ended up in pneumonia. Because of the snow storm on Friday Jack was unable to be transported down here to Philly so he was kept in the PICU in St. Barnabas Friday night and Helicopter Transport came Saturday morning to pick him up and this time Roy got to take the flight down here to Philly.
I copied this from Giovanni's dad progress notes:
When I entered Giovanni's room I walked to his side and laid my cheek against his as he soundly slept softly breathing. As my cheek lay against his puffy cherib cheeks feeling his warmth I selfishly fed myself comfort from this touch me a mere weak mortal to his constant strength.
........
The first thing I saw this morning when I awoke a 4am was a bottle of scotch on my dresser and thought I should take a slug from it to dull my senses as Christina and I began our 100 mile journey to the side of Giovanni for the first day of the rest of his life.
........
This morning we were very worried about Giovanni and his declining condition. He's now stable but no longer conscious or playful like yesterday. He's slept most of the day, his body exhausted. His vitals have stabilized, but his oxygen levels are erratic.
........
A doctor came in late moring and told me they identified Giovanni's infection: Citrobacter (Sit-row-back-tur)– Genus belonging to the family Enterobacteriaceae.
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This from Kevin, Rhys father,
More at www.cure4rhys.org
Me and my brother Ralph wanted to do something for Rhys together, as skydiving is not everyones cup of tea. We thought we would do a cycle ride, I thought mmmmm cycling the Uk has been done and does not tell a story.
So in my infinite wisdom I said why dont we cycle to Paris to meet Proffessor Jean Laurant Casanova, the leading person on NEMO in europe and ask a few questions about Rhys and promote the charity and raise awareness.
After Ralph picked himself off the floor he agreed to the cycle, not only that but his wife Liz also voluntered.
As usual all the die hard lovers of Rhys and the cause came forward to the mark!!
Of course Deb Shord teacher of the Deaf at Hollybush School and all around super woman thought the dkydive was to tame! Steve Britton a former Mr Gt Britain, Mr Wales bodybuilding champion.
Steve also done the skydive, but said that he won his bodybuilding titles when he was 40 years of age, this year is another big land mark (I will not divulge as he could bend me in two) and his birthday is during the ride!!! www.stevebritton.co.uk , he has also been our
trainer and ride planner!
Mike Cadugan, Steves training partner Big Mike!! also kindly voluntered as he is soon off to sunny Australia but has a young family and is touched with our dilemna.
Debs son Sebastian has also put his best foot forward, he is only 15 so I guess he will put the old men like us to shame!! However a good weapon to chat up any French women for rooms and directions!
Sarah Steves girlfriend and her daughter Libby are coming as support in the vehicles to muck in!!! However i think Sarah is good enough to cycle.
We will also be filmed by a couple of guys so the DVD will be out in all good stores soon after, so our journey will be in our memory forever!
Before we go to Necker hospital to see Jean laurant we hope to see some of our French rugby playing friends at their ground at Stade Francias Paris.
This all starts on the 1st of April from the Liberty Stadium the home of our Heroes the Osprey Rugby team, 1st stop will be Newport, 2nd Warminster 3rd Portsmouth these are 3 hard grinding days but steves birthday is the 3rd so want to celebrate on the boat!!
Le Harvre through to Paris again 3 days Arrive in Beautiful Paris on the 6th if all goes well.
This is going to be tough but as I say
THE STRENGTH OF THE CHILD IS WITHIN THE FAMILY, AND THE STRENGTH OF THE FAMILY IS WITHIN CHILD
And these guys are now family and that is all the motivation we need.
Rhys we love you!!
Three years old.
¡Hola!
¡Solo faltan 5 semanas para el Maratón de Boston!
Ya aparecemos en la base de datos de competidores:
www.bostonmarathon.org/2007/cf/Public/EntryLists.cfm
Llevamos en lo individual $15,375 dólares, vamos por $4,625 más para llegar a $20,000. El equipo “Miles for Miracles” vamos llegando a los $700,000 dólares.
El entrenamiento va muy bien; el fin de semana pasado corrí 3 horas y 10 minutos del Desierto de los Leones casi hasta “La Pila” con alto grado de dificultad:
- Mucha subida
- Por un camino con grava suelta
- A más de 3,000 metros de altura
No sé que distancia fue pero habiendo corrido más de 3 horas y en esas condiciones me siento, por primera vez, seguro de poder terminar el maratón.
Agradezco a mis cuñados Antonio, Juan y Carlos (cuñado honorario) por su apoyo moral y logístico en esta corrida, aunque me quedaron a deber las quesadillas.
Este fin de semana corrí una carrera de 7km en Santa Fe, en 44 minutos, también con muchas subidas. Los resultados están en www.delcorredor.com
La próxima semana toca otra vez correr 30km y la que sigue voy a correr la carrera “Imagen Banamex” de 10km.
Muchísimas gracias a todos. Los sigo informando.
Saludos
Victor
We made our reservations to the IDF national conference in St. Louis, MO. June 28-30, 2007.
Dr. Jordan Orange will give a talk about NEMO Friday June 29th. from 10:45 am to 11:45 am at the specific diagnosis sessions. These specific diagnosis sessions will provide individuals and families affected by each particular disease a better understanding of the disease, treatment options, research advances, and management of associated complications.
Other talks include:
Bone Marrow and stem cell transplantation
Managing Gastrointestinal Complications of Primary Immune Deficiency Diseases
The Latest Advances in Gene Therapy
General Care of the Primary Immune Deficient Patient
The Immune Deficiency Foundation is the national patient organization dedicated to improving the diagnosis and treatment of patients with primary immune deficiency diseases through research, education and advocacy. Founded in 1980 by parents of children with primary immune deficiency diseases and their physicians, IDF is governed by a Board of Trustees, has an active Medical Advisory Committee of leading clinical immunologists and a grassroots network of volunteers across the country.
