06/21/2008
Mission accomplished: Jayme Green completes 357-mile Ride for Granton
By: Charlotte Marsch, BHFP editor charlottem@cpimo.com

Jayme Green arrived at Wellspring Baptist Church at about 7:30 p.m. Friday, June 20, to complete his Ride for Granton to raise money for his 8-month-old cousin, Granton Bayless and his family.
In five days, Jayme Green logged 357 miles on his bike, leaving Topeka, Kan., Monday, June 16, and arriving in Bolivar at about 7:30 p.m. Friday, June 20. It wasn’t just a joy ride. He had a mission: Raising money for his cousin Jenni Bayless and her husband, Daniel and 8-month-old son, Granton.

Green was greeted by family members and friends of the Baylesses at Wellspring Baptist Fellowship at the end of his ride Friday evening. About 10 children brought their bicycles and rode a victory lap with Green, Jenni’s cousin, after he arrived.

As of Friday evening, the fundraising total was at more than $8,000 with more donations still expected. On Friday, Green’s story went out on nationally syndicated Christian radio, so he expects that to bring in more money, too.

Granton has been hospitalized at Children’s Mercy Hospital in Kansas City since late March when he was diagnosed with severe combined immunodeficiency syndrome, commonly referred to as “bubble boy disease.” Granton was born without an immune system but has survived several infections and viruses. He received a transplant of umbilical cord blood stem cells Tuesday, June 10.

“He’s holding his own right now,” Daniel said Friday evening. “He’s on Day 10. They say the first 30 days are the most critical.”

Daniel and Jenni are posting daily updates at grantonbayless.blogspot.com.

Daniel took sick leave for the remainder of last school year, but the Bolivar High School teacher and track coach has returned to Bolivar the past three weeks to teach summer school. Jenni’s visit Friday evening was her first trip home since Granton went to Kansas City, where he likely will stay for at least another three months.

Daniel said the community has supported them well during the last three months.
“It’s just amazing,” Daniel said, “just the way people have really come out to help out Granton. But I’ve learned in Bolivar we take care of our own, and people have been so amazing for us.”

Granton has been featured on three TV stations and in the Kansas City Star, an article that focused on how the people of Bolivar have rallied around the Baylesses.

The ride

The bike Green rode was donated by Sunshine Bike Shop in Springfield. Green sent an e-mail to the store telling what he was planning to do and why he needed a bike donated.
“We were just touched by the story and offered to donate the bike,” said Kay Meeker, who owns the store, along with her twin granddaughters Jenna and Jerra Umlauf. They attended the party Friday night.

The bicycle was then given away Friday night, with the drawing for the bike raising $1,200. Signs to Go in Topeka, Kan., donated lettering on the bicycle that reads, “Ride for Granton June 16-20, 2008,” “Romans 12:12″ and “Isaiah 40:31.”

Toby, the Support and Gear Man, drove alongside Green throughout the ride.

“It was great,” Jayme said just minutes after completing the ride. “You could really tell that God was behind it.”

Along the way, Green raised awareness for Granton’s story, as well as raising money for the Bayless family.

“I met a lot of great people along the way,” he said. “Every town I stopped in was wonderful.”

Read his blog at rideforgranton.blogspot.com and you’ll find out what he’s talking about:

* The first night, they were hosted by First Christian Church of Burlington, Kan., where he was greeted by the local newspaper and the chamber of commerce, served dinner by the Young Adults Sunday school class and served a large breakfast the next morning.

* The second evening they arrived at Daniel’s alma mater, Caney Valley High School, where he was greeted by a reporter from the local newspaper. They then went to the Bayless farm, where Daniel’s parents met them with a “massive feast.”

* When they arrived in Stockton at 1 p.m. Thursday, they were greeted by students from Stockton Elementary School, where Jenni taught art until Granton was born. Pat Steinmuller, Jenni’s former boss and the principal at Stockton Elementary, treated them to lunch, and then they pitched a tent for the night.

They spent most of the day Friday at Stockton, speaking to summer school students at Stockton Elementary School.

“They were awesome,” Green said. “The whole lawn of the school was filled with kids, and a lot of the teachers and students had already donated.”

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Everybody Wins
Marc Ringel

GREELEY, CO (2008-06-16)
The medical community is full costly and often times unnecessary diagnostic tests that may not yield conclusive results. But KUNC Commentator Dr. Marc Ringel says some tests are worth taking.

One of the premier principles of screening for a disease is that there has to be a treatment for the illness or else it’s not worth testing for. There are, for example, tests (not very well validated yet) that can suggest if a person is susceptible to developing Alzheimer’s Disease. Given that there is no known treatment for this horrible malady, nor any reliable way to stave off its appearance, what good could it do to know you’re likely get it? I can only see a downside, based on the panic that expectation of Alzheimer’s might inflict with every forgotten face or misplaced word. Another basic principle of health screening is that the disease being looked for needs to be prevalent enough that sufficient cases will be turned up. Otherwise, you might spend hundreds of thousands with little prospect of uncovering even one person to help. So, how come the State of Wisconsin has just invested half a million dollars for medical laboratory equipment and will spend another $400 thousand a year on supplies to add to its newborn blood screening panel a test for an illness that afflicts one in 65,000 newborns? In 2005 there were just 68,532 live births reported in that state. In other words, not even counting startup costs, Wisconsin would spend nearly half a million dollars to find just one case. The screening does makes sense when you consider the disease they’re looking for. It’s called Severe Combined Immunodeficiency Disorder (SCID), an affliction that cripples the immune system, leading, from early in life, to an existence fraught with suffering brought on by overwhelming repeated infections. Medical expenses for a person with SCID are estimated at $2 million, from cradle to premature grave. Testing for this affliction does meet the first principle of screening, that there be an effective therapy for the disease uncovered. In fact, SCID meets that criterion so well as to make it worth looking for, no matter how rare. Bone marrow transplantation is successful in curing 96% of infants of this immune disorder if accomplished before the age of 3-1/2 months. Since they already have a program that tests all newborns for other congenital diseases, any of which can cause untold misery if not diagnosed early, it’s easy enough for Wisconsin to add one more test to the panel. Thanks to amazing recent advances in biotechnology, the cost of an ultra-sensitive test for SCID is under $10 per individual. Admittedly, this program will not affect a lot of kids, about one per year in the whole state of Wisconsin. But everything about it is still right. That one baby is likely to live a long healthy life, instead of a brief miserable one. Imagine the benefits to this kid’s family too, having a well child instead of a chronically ill one in the house. And society is saved a whole lot of money. I can’t think of a better deal for the public at large, not to mention for the child and his family. It’s a great deal for us doctors too, at least psychologically, because, even in the dense thicket of disease screening and health economics, for once we have a clear-cut right answer.

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Neonatal bone marrow transplantation of ADA-deficient SCID mice results in immunologic reconstitution despite low levels of engraftment and an absence of selective donor T lymphoid expansion

Denise A. Carbonaro^1,2, Xiangyang Jin¹, Daniel Cotoi¹, Tiejuan Mi³, Xiao-Jin Yu¹, Dianne C. Skelton¹, Frederick Dorey², Rodney E. Kellems³, Michael R. Blackburn³, and Donald B. Kohn^1,2

¹ Division of Research Immunology/Bone Marrow Transplantation, Childrens Hospital, Los Angeles, CA; ² Department of Pediatrics, University of Southern California Keck School of Medicine, Los Angeles; and ³ Department of Biochemistry and Molecular Biology, University of Texas-Houston Medical School

Adenosine deaminase (ADA)–deficient severe combined immune deficiency (SCID) may be treated by allogeneic hematopoietic stem cell transplantation without prior cytoreductive conditioning, although the mechanism of immune reconstitution is unclear. We studied this process in a murine gene knockout model of ADA-deficient SCID. Newborn ADA-deficient pups received transplants of intravenous infusion of normal congenic bone marrow, without prior cytoreductive conditioning, which resulted in long-term survival, multisystem correction, and nearly normal lymphocyte numbers and mitogenic proliferative responses. Only 1% to 3% of lymphocytes and myeloid cells were of donor origin without a selective expansion of donor-derived lymphocytes; immune reconstitution was by endogenous, host-derived ADA-deficient lymphocytes. Preconditioning of neonates with 100 to 400 cGy of total body irradiation before normal donor marrow transplant increased the levels of engrafted donor cells in a radiation dose–dependent manner, but the chimerism levels were similar for lymphoid and myeloid cells. The absence of selective reconstitution by donor T lymphocytes in the ADA-deficient mice indicates that restoration of immune function occurred by rescue of endogenous ADA-deficient lymphocytes through cross-correction from the engrafted ADA-replete donor cells. Thus, ADA-deficient SCID is unique in its responses to nonmyeloablative bone marrow transplantation, which has implications for clinical bone marrow transplantation or gene therapy.

linkback url: http://bloodjournal.hematologylibrary.org/cgi/content/short/111/12/5745?rss=1

Baby with rare condition with get a cord-blood transplant

The Bolivar, Mo., baby who inspired more than 1,700 people in three Midwest towns to register as potential bone marrow donors is scheduled for a cord-blood transplant today.

Eight-month-old Granton Bayless is in Children’s Mercy Hospital with a rare condition called severe combined immunodefiency. A cord-blood transplant is his best option, said his aunt, Denise Bayless.

It is too early to know whether last month’s donor drives have produced a bone marrow transplant match. For more information, go to http://granton bayless.blogspot.com.

| Debra Skodack, dskodack@kcstar.com

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Last Call !!! June 4, 2008

Melody’s transplant joy

PROUD parents Scott Davies and Lauren Travis have hailed their “champion” baby girl who has bravely battled through chemotherapy and a bone marrow transplant.
The Express has been following the story of little Melody Davies who was born four months ago without an immune system, which meant a common cold - and a kiss - could kill her.
The tot was diagnosed with the rare genetic disorder severe combined immunodeficiency (SCID) and sparked a worldwide hunt for a bone marrow donor to save her life.
A donor was found just as time was running out and Melody had to undergo chemotherapy before the transplant at a Newcastle hospital three weeks ago.
Dad Scott, 28, of Briggs Avenue, Castleford, said: “Melody’s really on the mend now - she’s a champion. She’s a completely different child. She doesn’t look as sad and is much more happy and settled.
“She’s flown through the chemotherapy and she sailed through the bone marrow transplant too.
“It was a nice moment for us as parents, knowing that when we pushed that button for the transplant, we were giving her the start of the rest of her life.
“We have a chart on the wall which counts the cells which are developing her immune system back up and it’s slowly gathering pace.
“We hope that next month we might be able to take her around the hospital in a push chair. That would be great. Just to do the things you take for granted - like kissing her.
“We just want some normality as soon as we can.”
The family had a set back two weeks ago when Melody developed an infection and blood clot in her Hickman line - a tube which goes into a vein in her chest.

Scott said: “That was probably the worst week yet. I had just come home to Castleford and had to rush back. They were worried that the clot could have moved to her lungs which would have been fatal. But she got through that as well.
“I’m very proud of her. I have never known anyone be as brave as my daughter for what she has gone through in her short life. I could never suffer as much pain as she has had, she’s so strong.”
Melody is now in a critical period, which lasts 100 days after the transplant, in which she could reject the donor cells.
She is also awaiting results of a test which will determine how many cells from the donor have developed in her system.
Scott said they will be allowed to find out the identity of the donor in five years’ time and intend to do that.
He said: “We will definitely find him. We owe Melody’s life to him as well as all the people who donate blood - she has had so many blood transfusions. Every bit of help we have had from people has been brilliant.”
linkback url: http://www.pontefractandcastlefordexpress.co.uk/news/
Melody39s-transplant-joy.4128112.jp

05/27/2008

Charity run caps season

Aside from Run The Woodlands 5K, which is held year round on the second and fourth Saturdays at Barbara Bush Elementary, this Saturday’s David’s Dream 5K at HEB Market Street in The Woodlands will complete the county’s 2007-2008 running calendar.

The annual event raises money for the David Center at Texas Children’s Hospital in Houston.

The Center, which is part of the Allergy and Immunology Clinic at the hospital, was established after the death, in 1984 at the age of 12, of a little boy who touched millions of hearts around the globe without ever leaving his sterile, plastic bubble.

That young boy was known as David, the Bubble Boy.

David, who lived with his family in Shenandoah, was born with a genetic immune disorder known as SCID, or severe combined immune deficiency, which robbed him of any germ-fighting ability.

David Elementary School in The Woodlands is named after the race’s namesake.

Online registration is available at the event’s web siteat http://www.davidsdreamrun.org/, through Thursday evening. The registration fee is $22 for the 5K and a kids 1-mile. A family can register for $65 and receive up to four T-shirts.

Race day registration begins at 7:30 a.m. on Saturday with the race to set to begin at 8 a.m.

The race also serves as the final leg of the spring version of the Montgomery County Triple.

Twenty-one of the 98 finishers of Saturday’s Run The Woodlands 5K had also finished the Conroe Family YMCA Trail Run 5K two weeks before.

TWHS senior Bryce Wolfe won Saturday’s event in a time of 16:54 as he beat Spring’s Jacob Mazone and Conroe High School’s Ricky Lugo to the finish line. Mazone, who was second at last year’s David’s Dream Run was second with a time of 17:12 while Lugo finished the race in 17:26.

The Woodlands’ Sarah Broyles won the women’s field in 22:19 followed by a pair of fellow The Woodlands natives in Ann Leoni and Cheryl Pilgrim in 24:15 and 25:10, respectively.

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Bubble Boy Disease means certain death if left untreated

Bubble Boy Disease means certain death if left untreated

Problem with diagnoses is that symptoms vary for the more than 140 forms of the disease

May 22, 2008 12:32 PM
MARIA TZAVARAS

The boy in the bubble was popularized in the 1976 TV movie The Boy in the Plastic Bubble starring John Travolta and recently in shows such as The Family Guy and Seinfeld.But what you may not know is that these “bubble children” actually have a form of PID or primary immunodeficiency disease, a disease that for many can mean certain death if left undiagnosed.

According to Richard Thompson, executive director of the Canadian Immunodeficiency Society, there are 140 different forms of PID, each ranging in levels of severity.

“PID is a defect in the immune system, it’s something you’re born with. It can affect anyone in any culture and doesn’t have to be a previous family history,” he said.

Most forms of PID can be maintained with a proper diagnosis and medication, except for the worst form, severe combined immunodeficiency (SCID).

SCID, more commonly known as Bubble Boy Disease, means a child is born without an immune system. There is an 85 per cent survival rate but children with this disease must have a bone marrow transplant.

Sick Children’s Hospital in downtown Toronto houses the PID clinic, the leading centre of PID treatment and bone marrow transplants in Canada.

It’s estimated there are 13,000 people affected by PID in Canada, and Thompson said 50 per cent of those with SCID are not diagnosed, or misdiagnosed, due to doctors not recognizing the problem.

“It has a lot to do with the symptoms, they vary from individual and it’s also because of the different forms,” he said. “Out of 140 different disorders, the symptoms vary and general practitioners are often not looking for PID.”

For example, Thompson said it’s common for babies and young children to get ear or sinus infections so the physician will treat the infection and not look for anything further, let alone something immune-system related.

“So part of our main effort is to educate physicians on the warning signs and on primary immunodeficiency,” he said.

Lori Peters and her husband Jason know first-hand the heartache of PID being misdiagnosed.

Their second child, Brooklyn, died in 2002 at five months old after continuously being in and out of hospitals.

Peters said Brooklyn was born a healthy baby girl on Oct. 29, 2001. At three months she got a clear runny nose and a cough, but doctors said it was nothing to worry about.

“But the cough kept continuing. It didn’t seem to be bothering her but it was weird to me why she still had it,” Peters said.

After another thorough examination, the doctor prescribed Dimetapp, which made Brooklyn cranky so Peters took her off of it. The cough persisted.

Brooklyn’s sickness came to a head on a trip to Florida that was spent almost entirely in hospital.

Besides the cough, she soon had a multitude of symptoms including diarrhea, rapid breathing and vomiting. After doing every imaginable test, doctors still couldn’t figure out what was wrong with the little girl.

“Even through all this she was the happiest little thing, she was such a sweetheart, and that was what was so strange, how happy she was,” Peters said.

By this point not only were they frustrated and exhausted, but they also had to return home. Accompanied by a nurse, they immediately went to their local hospital.

“The doctor checked her out, didn’t really do much and sent us home, and about every three to four days after that I went to my family doctor and my concern at this point was that she wasn’t eating,” Peters said.

Four days later the doctor saw a drastic change in Brooklyn, and after several more visits to the doctor, she was finally admitted to hospital with a violent cough and diagnosed with double pneumonia.

“They admitted her, put her on antibiotics and an IV and told us we should see an improvement within 48 hours,” she said.

Getting worse, Brooklyn was finally rushed to McMaster Hospital and put on a ventilator. Brooklyn died a few days later on April 9, 2002.

The reason was a mystery, according to doctors.

After her death, autopsy reports showed she had SCID, which was devastating news.

However, if there was a blessing in it all, Peters said when her son Ethan was born in 2005 he was tested and treated immediately once shown to also have SCID.

At three days old, he was admitted to Sick Kids Hospital in the bone marrow unit to await a transplant. Five months later, he received a transplant and at eight months old was discharged. Today he’s healthy, 100 per cent cured and not on any medication.

Ethan’s case is testimony to the fact that if diagnosed and treated properly, SCID does not have to result in death.

Peters said it’s a tragedy that Brooklyn wasn’t properly diagnosed.

“Especially when you see our son Ethan now and see how awesome he’s doing and he had the same thing,” she said.

“It was devastating not knowing why she was dying, she never even had a chance to fight, which was really hard for us. If we had gotten to Sick Kids she may have had a chance.”

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Corinth Boy Suffering From Rare Disease Gets Lung Transplant

Web Editor: Rhonda Erskine, Online Content Producer
Created: 5/14/2008 6:13:58 PM
Updated: 5/15/2008 11:25:28 AM

CORINTH (NEWS CENTER) — Last fall, we brought you the story about a 10-year-old boy from Corinth who needed a lung transplant. Wednesday, Zachary Nickerson returned home after getting the procedure he desperately needed.

Zachary has SCIDS, Severe Combined Immuno-Defficiency Syndrome. It is often called “bubble boy disease.”

Zachary’s immune system is so weak that even the slightest cold can land him in the the hospital.

Zachary got a special ride home from the Corinth Fire Department Wednesday. An excited group of friends and family were there to greet him.

“This is pretty awesome,” said Zachary’s mother, Michele Nickerson. “Zachary is so excited. He’s been anxious to get home. There’s been times in that hospital when he would wonder if he would ever come home.”

Zachary has lived three years of his life in the hospital and has had more than two dozen operations, including two bone marrow transplants. Despite his problems, his family says he has an amazingly positive attitude.

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{WASHINGTON, D.C., May 8, 2008} – The Immune Deficiency Foundation (IDF) applauds Senators John Kerry (D-Mass.), Lamar Alexander (R-Tenn.) and Debbie Stabenow (D-Mich.) for introducing S. 2990, “The Medicare IVIG Access Act.” The bill seeks adequate reimbursement for Intravenous Immunoglobulin (IVIG) therapy and accompanying care in the management of patients with primary immunodeficiency diseases (PIDD) and other serious conditions that require IVIG therapy.

“For our patients with primary immunodeficiency diseases who need antibody replacement, IVIG is their only treatment option,” said Marcia Boyle, IDF President and Founder. “Senators Kerry, Alexander and Stabenow have stepped forward to ensure that individuals whose lives depend on access to IVIG will not be put at risk.”

Presently, Medicare beneficiaries who require IVIG are experiencing serious access problems – an unintended result of the way Medicare determines the payment for IVIG. Many doctors and hospitals cannot purchase the products at Medicare reimbursed rates, and as a result, some patients are not getting this life saving treatment.

“The Medicare IVIG Access Act” would fix these current problems by ensuring adequate reimbursement for IVIG. It also allows for home treatments for Medicare beneficiaries with PIDD by covering related items and services excluded from the current home infusion benefit. These changes will allow these patients to lead healthy and productive lives.

Multiple patient, professional and industry organizations have joined IDF in support of this legislation.

Primary immunodeficiency diseases are a group of over 150 rare disorders in which part of the body’s immune system is missing or does not function properly. In contrast to secondary immune deficiency disease in which the immune system is compromised by factors outside the immune system, PIDD are caused by intrinsic or genetic defects in the immune system. Approximately 250,000 people in the United States are diagnosed with PIDD and between 35,000 and 50,000 of them depend on IVIG as their life-saving treatment. Untreated primary immunodeficiencies may be characterized by frequent life-threatening infections and debilitating illnesses.

The Immune Deficiency Foundation (IDF), founded in 1980, is the national non-profit patient organization dedicated to improving the diagnosis, treatment and quality of life of persons with primary immunodeficiency diseases through advocacy, education and research.

To learn more about IDF visit www.primaryimmune.org