Susan's Transplant Video is on You Tube

Susan Burroughs, the Founder of Cystic Fibrosis-Reaching Out Foundation has now put her transplant video on You Tube. To see the video please go to and search "Susan Burroughs".

Sunday, November 30, 2008

Cystic fibrosis: FAQs on the disease of '65 roses'

Cystic fibrosis: FAQs on the disease of '65 roses'

Last Updated: Thursday, November 27, 2008 |CBC News

Roses are a symbol for cystic fibrosis. Cystic fibrosis, or CF, is sometimes called 65 roses. The name came about after a boy overheard his mother talking about the condition on the phone and thought she said "65 roses." A single rose is often used as a symbol for the disease.

A child with cystic fibrosis may cough frequently and need to have a parent clap his or her back every morning to make it easier to breathe.

Cystic fibrosis is an inherited disease of the mucus and sweat glands. Unlike in healthy lungs, where mucus protects the airways, people with CF have mucus that becomes thick and sticky. When the substance builds up in the lungs, bacterial infections may occur.

Mucous-producing cells in the digestive system — including the pancreas, liver and intestines — and respiratory system are the major problem areas for people with CF.

What causes CF?
Jake Szatmary was sick and malnourished for the first three months of his life, before cystic fibrosis was diagnosed. (CBC)People with CF inherit two copies of an abnormal gene, one from each parent. Those with one copy are carriers who don't have the disease or show any symptoms but may pass it on to their children. About one in 25 Canadians carries a defective version of the gene responsible for CF, according to the Canadian Cystic Fibrosis Foundation.

Canadian researchers discovered the gene responsible for CF, called cystic fibrosis transmembrane conductance regulator, or CFTR, in 1989. More than 1,000 mutations of the gene have been associated with the disease.

Who is affected?
An estimated one in every 3,600 children born in Canada has CF. The severity of the disease varies, and both sexes are affected with a similar frequency.

The risk of being a carrier varies by ethnicity, according to

Ethnicity Carrier risk
Caucasian 1 in 25
Ashkenazi Jew 1 in 25
Hispanic 1 in 46
Black 1 in 65
The disease mainly affects Caucasians, which includes Europeans, Indians and people from the Middle East, said Nadine Imbleau Redman, a spokeswoman for the Canadian Cystic Fibrosis Foundation.

How is it diagnosed?
Doctors may suspect CF in babies who cough a lot and get many lung infections. A simple and painless collection of sweat is done, since people with CF have extremely salty sweat. Chest X-rays, sinus X-rays, lung function tests, analysis of sputum and stool and genetic tests on blood may also be done to help in the diagnosis. Prenatal genetic tests show if a fetus is likely to have CF.

What are the common symptoms?
Frequent coughing with phlegm.
Frequent infections such as bronchitis and pneumonia.
Salty-tasting skin.
Excessive appetite with weight loss.
Bulky bowel movements.
Poor growth.
Infertility, mostly in men.
How is it treated?
The goal is to keep the lungs clear of mucus to avoid infection. Physical therapy, such as clapping on the chest and back, breathing exercises, wearing a positive pressure expiratory mask and drinking a lot of fluids helps to loosen mucus.

Pancreatic enzymes are taken with meals to aid digestion since mucus in the pancreas makes it difficult to digest food and absorb nutrients. A vitamin and mineral supplement may be needed for the same reason. Drugs that thin the mucus, reduce inflammation in the airways, and antibiotics, taken in pill, intravenous or inhaled form, may be prescribed, according to the U.S. National Heart Lung and Blood Institute.

Eating a healthy diet, avoiding tobacco smoke, exercising frequently, and frequent hand washing are also important.

How is daily life affected?
In 2006, for the first time, more than half of all Canadians with CF were 18 years or older, the Canadian Cystic Fibrosis Foundation said. Half of Canadians with CF are now expected to live into their late 30s or longer.

When lung disease limits daily life, then lung transplants may be an option.

In 2004, a 21-year-old woman with CF received a rare lung transplant from two living donors, her mother and aunt. Such transplants are complex since both donors and the recipient are at risk.

"The CF had clogged up my lungs," Jessie McQuitty of Edmonton said at the time. "I was unable to breathe, I was unable to work, unable to school, everything."

In 2008, Canadian researchers discovered a genetic variant that can increase the severity of lung deterioration in children with CF.

These patients "experience a vicious cycle of infection and inflammation that destroys lung tissue, inhibits lung function, and erodes quality of life," said Dr. Julian Zielenski, an associate scientist in the genetics and genome biology program at the Hospital for Sick Children and a lead author of the study.

People who have the variant produce less of a protein that defends against bacterial infections. The researchers hope to one day develop a genetic test that can predict the severity of a patient's lung disease.

Wednesday, November 26, 2008

The Amazing David Adkins AGE 53

This is David Adkins..(pictured with Barbara Crews of Scottish Rite) In his own words: Well, I'm an old man (53 - born 1955). I run three days a week 52 weeks a year come hell or high water. I have completed 4 marathons (1 Atlanta 3 New York), at least 7 or 8 half marathons (and counting). I'm a strong advocate for aerobic exercise for CFers and I'm always willing to promote and encourage CFers (really everyone, but particularly CF patients) to exercise. I attribute my good health to a combination of exercise, good heath care, positive attitude, and a happy home life (in no particular order). I have been married to the love of my life, Betti, for 15 years. We have no kids, but two cats, Bogie and Maggie. I work full time, and plan to work full time until retirement (which keeps moving forward as the market moves downward).

Thursday, November 13, 2008

Update on the Adult Program of the Emory CF Center

From left to right: Welela Berhanu, RN, Arlene Stecenko, MD, Viranuj Sueblinvong, MD, Jessica Enders, RD, Lysandra Brown, MSW, Vin Tangpricha, MD, PhD, Lindy Wolfenden, MD. Photo taken at NACFC 2008.

In 2005, under the guidance of Dr. Arlene Stecenko, a CF physician with extensive experience in the care of adults with CF, a separate adult program was created at the Emory CF Center. Dr. Stecenko was joined in 2006 by Dr. Linda Wolfenden, who became the Director of the Adult Program. The adult program was initially run with significant support from Children’s Healthcare of Atlanta as well as Emory Children’s Center pediatric staff and physicians. In 2007 the Emory CF Center was visited by members of the Cystic Fibrosis Foundation’s Center Committee, the organization which accredits all CF Care Centers
in North America. That group, which consists of experts who set the national CF standards of care, made a strong recommendation that the adult program become independent of the pediatric program and rely on staff and physician providers trained in adult care. Given the solid rationale behind these recommendations and our desire to ensure that we deliver the highest quality care to our patients, the CF center staff carefully reviewed these recommendations and met with administrative and medical leaders at Emory University, Emory Healthcare, and CHOA to come up with a plan.

We are pleased that Emory Healthcare has given the adult CF program
an unprecedented level of support to meet and exceed the recommendations of the CF Foundation. With its full backing and the support of the Emory University Department of Medicine, we are now prepared to advance the care we offer to adult CF patients to a higher level. Our goal is to provide our patients with exceptional care. To that end, we are pleased to announce a number of changes to our adult CF program. Some of these are already apparent; some will become apparent in the coming months:

1. The adult CF program is now fully integrated within the Department of Medicine at Emory. Adult CF clinics take place on four days weekly at The Emory Clinic, 1365 Clifton Road, Building A, 4th floor, Atlanta, GA 30324. The Emory Clinic is about a half-mile down the road from the Emory CF Center. The phone number for appointments is 404-778-3261.

2. When required, all adult patients followed by the Emory program will be hospitalized on a specific ward (6G) at Emory University Hospital.

2. The adult CF program has expanded its staff to include many new faces, and well as keeping familiar ones. Our core physicians include
Lindy Wolfenden, MD--Adult CF Program Director
Viranuj Sueblinvong, MD--Adult CF Program Associate Director
Arlene Stecenko, MD-Chief, Division of Pediatric Pulmonary, Allergy, CF and Sleep
Vin Tangpricha, MD, PhD-Director of Adult CF Endocrinology

In addition to these physicians, others with expertise in adult specialties such as gastroenterology, surgery, and otolaryngology will be involved in the care of our patients. Hospitalized patients will also meet other members of the Emory Division of Pulmonary and Critical Care Medicine and the Division of Hospital Medicine.

Other staff includes
Welela Berhanu, RN-Clinical Nurse Coordinator
Lysandra Brown, MSW-Adult CF Social Worker
Jessica Enders, RD-Adult CF Registered Dietician
Laura-Beth Straight, MA-Research Coordinator
Deneek Hubbard-Green, NP- Nurse Practioner (starting December, 2008)

In addition, we are currently recruiting a dedicated adult CF Respiratory Therapist.

Clearly, there are a number of changes that have occurred in the adult CF program. We recognize that any type of change, even “good change” (such as marriage or becoming a parent), can be stressful and challenging at times. However, we are confident that these changes are “good changes” and that they will enable us at Emory to provide our patients with superior care. We look forward to working with you to provide you the exceptional care you deserve.

Michael S. Schechter, MD, MPH
Emory CF Center Director

New! CF Family Connection

Introducing the new CF Family Connection …
an opportunity to network with other CF family members through educational programs and social gatherings!

2009 Calendar of Events

JAN 15 Educational program
MAR 19 Social gathering
MAY 21 Educational program
JUL 16 Social gathering
SEP 17 Educational program
NOV 12 Social gathering

For more information or to be added to our email list, please contact
Lynda Ratmeyer-Fleming at or 770-716-2639

Sponsored by: CF Family Advisor Council
Childrens Healthcare of Atlanta

Wednesday, November 12, 2008

New Treatment For Patients With Cystic Fibrosis

New Treatment For Patients With Cystic Fibrosis

Kellye Lynn BALTIMORE (WJZ) ― There's a change in treatment for patients with cystic fibrosis.

Healthwatch reporter Kellye Lynn says many children with CF are not getting enough of an important vitamin.

That vitamin is Vitamin D, which is essential for bone health.

Last summer, Justin Homassel, 10, leaped off his porch backwards and broke his arm.

"I jumped and caught myself with my arm and I broke it," he said.

Broken bones are more common among children like Justin who have cystic fibrosis, a genetic disorder that causes sticky mucous to build up in the body and affects about 30,000 Americans.

"The pancreas doesn't make enzymes to digest food appropriately, especially fat so they don't absorb fat, and therefore fat soluble vitamins, the way they should," said Dr. Peter Mogayzel, Johns Hopkins Children's Center.

The result is low levels of Vitamin D, which can increase the risk of rickets and osteoporosis.

A new study out of Johns Hopkins Children's Center shows current recommendations for treating Vitamin D deficiency don't get far enough.

In the study, giving the standard 50,000 international units of Vitamin D for eight weeks was effective in only 33% of patients. It's a surprising finding and prompted Hopkins doctors to send a letter to each of their CF patients, urging an increase of Vitamin D.

The new recommendation is an extra 1,000 international units of Vitamin D a day.

"I think it's important for patients to take more Vitamin D and we believe the CF Foundation has to reevaluate this to have more appropriate recommendations for therapy," said Dr. Mogayzel.

Justin is now following that advice. His mom hopes doing so will keep looking as good as the rest of his body.

CF patients can also increase Vitamin D levels by spending 15 minutes a day in the sun without sunscreen. Doctors also recommend weight-bearing exercise.
(© MMVIII, CBS Broadcasting Inc. All Rights Reserved.)

Saturday, November 1, 2008

Low vitamin D common in kids with cystic fibrosis

By Megan Rauscher– Fri Oct 31, 9:17 am ETNEW YORK (Reuters Health) – Vitamin D deficiency is prevalent in children with cystic fibrosis (CF) and current treatment recommendations for correcting the deficiency are "inadequate," conclude clinicians from The Johns Hopkins Medical Institutions in Baltimore, Maryland.

They reviewed the charts of 262 children with CF patients treated at Johns Hopkins between 2003 and 2006 and found that vitamin D deficiency has declined but is still high; the prevalence was 86 percent in 2003 and 46 percent in 2006.

Many vitamin D deficient children remained deficient, despite getting "restorative" doses of vitamin D equal to or higher than the recommendations set by the Cystic Fibrosis Foundation, Dr. Deanna Green and colleagues report in the Journal of Pediatrics.

CF is a genetic disorder marked by the body's inability to transport chloride in and out of cells, causing mild to life-threatening complications, including recurrent and severe lung infections and delayed growth. Growing children with CF are especially vulnerable to vitamin D deficiency because a hallmark of the disorder is poor absorption of nutrients and malnutrition.

The recommended treatment dose for vitamin D deficiency is 50,000 international units (IU) of ergocalciferol (a form of vitamin D) once per week for 8 weeks for children 5 years of age or older and 12,000 IU once per week for 8 weeks for children younger than age 5 years.

But the Hopkins team that 50,000 IU per week of ergocalciferol for 8 weeks was effective in only 33 percent of the children with vitamin D deficiency, while increasing the dose to twice a week was effective in only 26 percent of children. Delivering the same dose 3 times a week corrected the deficiency in just 43 percent of children.

As a result of the findings, Johns Hopkins has amended its treatment protocol and now treats both adult and pediatric CF patients who have vitamin D deficiency with 50,000 IU ergocalciferol everyday for 4 weeks, according to a university-issued statement.

"These findings are a big wake-up call not only because they show that many children with CF are lacking vitamin D, but also because the deficiency persists even in those children who are treated with weekly doses twice or 3 times as high as the current recommendations," Green said.

"Clearly there is an urgent need to find more effective ways to restore healthy vitamin D levels," she concluded.

SOURCE: The Journal of Pediatrics, October 2008.