Akron Children's Lewis H. Walker, M.D., Cystic Fibrosis Center provides diagnosis and treatment to children and adults with CF, including respiratory therapy, physical therapy, genetic counseling and nutrition counseling. The center also actively participates in clinical trials to research new drug therapies to manage cystic fibrosis. Read More...
Akron Children’s cystic fibrosis center is one of six in the state that provides comprehensive care, including respiratory therapy, physical therapy, genetic counseling and nutrition counseling.
Cystic fibrosis treatment depends on the stage of the disease and which organs are involved.
Most therapies are designed to clear the lungs of the thick mucus to make breathing easier, minimize the bacteria that cause respiratory infections and provide the missing digestive enzymes for food absorption.
Children with CF must eat a healthy, high-calorie diet supplemented with vitamins and, usually, medications that contain pancreatic enzymes to help aid food absorption.
Many children with CF also require daily respiratory therapy, where a parent or caretaker is trained to perform vigorous percussions on the back and chest to dislodge thick mucus from the lungs.
Lung and liver transplants, stronger antibiotics and medications that thin mucus have contributed to the increased life of CF patients.
If you or a family member has cystic fibrosis, we recommend:
Akron Children's Lewis H. Walker Cystic Fibrosis Center actively participates in clinical trials to research new drug therapies to manage CF. Our goal is to improve treatment and quality of life for children and adults affected by cystic fibrosis.
The drug pipeline at the Cystic Fibrosis Foundation has more than 2 dozen potential therapies in development. One of the categories generating the most excitement and hope in the CF community is CFTR Modulation. These therapies are designed to correct the function of the defective CFTR protein made by the CF gene, which would allow chloride and sodium to properly move in and out of the cells that line the lungs and other vital organs.
In February 2011, the CF Foundation and Vertex Pharmaceuticals announced that VX-770 showed promising results in a Phase 3 clinical trial for patients 12 and older who carry at least one copy of a CF mutation G551D. Compared to placebo, patients who took the study drug showed a marked improvement in lung function, as well as a reduced likelihood of having a pulmonary exacerbation, decreased respiratory symptoms and weight gain.
A Phase 3 trial in children ages 6 to 11 with one G551D mutation also demonstrated positive results. These children showed marked improvement in lung function through 24 weeks of the 48-week trial, compared to children who received a placebo.
Vertex and the CF Foundation announced promising results in June 2011, for patients who have 2 copies of the Delta F508 mutation, the most common CF mutation. In CF patients with 2 copies of the Delta F508 mutation, the CFTR protein does not move to its proper place at the cell surface.
In this ongoing Phase 2 trial, patients are given a combination of VX-770 and VX-809. VX-809 is designed to help the protein reach the cell surface. VX-770 aims to help the protein function more normally once it's at the cell surface.
In the first phase of the trial, adult patients who took the drugs in combination showed a decrease in sweat chloride levels. Akron Children's center will be participating in the second phase, and we hope to enroll patients in fall 2011.
The basic entry criteria are: 2 copies of the Delta F508 mutation; 18 years or older; FEV1≥40% predicted; and in stable health at the start of the trial.
We are also enrolling patients in 2 other clinical trials evaluating the safety and efficacy of MP-376, an inhaled form of the antibiotic levofloxacin. Mpex 207 is a 10-week trial comparing 28 days of MP-376 to placebo in patients 12 years and older who have a positive culture for Pseudomonas aeruginosa.
In the Mpex 209 trial, patients with the same entry criteria will receive 3 cycles of the MP-376 or TOBI®. The objective is to compare the safety and efficacy of the new inhaled antibiotic to TOBI® in treating Pseudomonas aeruginosa.
We will also be recruiting for a new study in September 2011 called the ALPINE (Aztreonam Lysine for Pseudomonas Infection Eradication) study. The study drug is Aztreonam Lysine for inhalation, called Cayston®. The study population is children ages 3 months to less than 18 years with CF and newly detected Pa pulmonary infection.
To learn more about any of these trials, contact Debbie Ouellette, CF research nurse coordinator, at 330-543-3089.
We hope you find these articles and links helpful.
What to expect when coming to Akron Children's
For healthcare providers and nurses
Residency & Fellowships, Medical Students, Nursing and Allied Health
For prospective employees and career-seekers
Our online community that provides inspirational stories and helpful information.