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The report raises awareness of the huge impact of chronic respiratory diseases worldwide, and highlights
the risk factors as well as ways to prevent and... Noncommunicable diseases (NCDs), also known as chronic diseases, tend to be of long duration and are the result of a combination of genetic, physiological,...
These tools of the WHO Package of Essential Noncommunicable Diseases Interventions (WHO PEN) support implementation of very cost effective interventions...
The objectives of the WHO strategy on chronic respiratory diseases are:Better surveillance to map the magnitude of chronic respiratory diseases and analyse... Cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs. There are close to 40,000 children and adults living with cystic fibrosis in the United States (and an estimated 105,000 people have been diagnosed with CF across 94 countries), and CF can affect people of every racial and ethnic group. In people with CF,
mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to
the cell surface, the mucus in various organs becomes thick and sticky. In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, avoiding germs is a top concern for people with CF. In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children. Learn how the cystic fibrosis transmembrane conductance regulator affects the digestive system.Understand how the cystic fibrosis transmembrane conductance regulator (CFTR) affects the GI system. Today, because of improved medical treatments and care, more than half of people with CF are age 18 or older. Many people with CF can expect to live healthy, fulfilling lives into their 30s, 40s, and beyond. Read the Foundation's Patient Registry Reports. People with CF can have a variety of symptoms, including:
Learn more about CF — from diagnosis to living with the disease as an adult — in "An Introduction to Cystic Fibrosis: For Patients and Their Families," or watch the video series. Jay, a 6-year-old with CF Listen to CF clinicians explain:
Cystic fibrosis is a genetic disease. People with CF have inherited two copies of the defective CF gene — one copy from each parent. Both parents must have at least one copy of the defective gene. People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are:
The defective CF gene contains a slight abnormality called a mutation. There are more than 1,700 known mutations of the disease. Most genetic tests only screen for the most common CF mutations. Therefore, the test results may indicate a person who is a carrier of the CF gene is not a carrier. Diagnosing cystic fibrosis is a multistep process, and should include a:
Although most people are diagnosed with CF by the age of 2, some are diagnosed as adults. A CF specialist can order a sweat test and recommend additional testing to confirm a CF diagnosis. Read the CF Foundation’s clinical care guidelines for diagnosing CF. I grew up wondering why I felt sick every day. As doctors suggested unlikely diseases, such as hormonal disorders, kidney disease, lupus, and depression, I felt I was further from an answer. Then, my ENT suggested CF, a disease I had never heard of. As he described what he knew about CF, it matched all of my symptoms and promised the answer I had been looking for my whole life.” — Katie K., an adult with CF, from the Community Blog According to the Cystic Fibrosis Foundation Patient Registry, in the United States:
Did you know?More than half of the cystic fibrosis population is over 18. Cystic fibrosis is a complex disease. The types of symptoms and how severe they are can differ widely from person to person. Many different factors can affect a person's health and the course the disease runs, including your age when you are diagnosed. The OutlookTremendous advancements in specialized CF care have added years and improve the quality of the lives of people with cystic fibrosis. During the 1950s, a child with CF rarely lived long enough to attend elementary school. Today, many people with CF achieving their dreams of attending college, pursuing careers, getting married, and having kids. Watch this video to see how we "count our success in lives" as we continue writing the next chapter in CF together.Although there has been significant progress in treating this disease, there is still no cure and too many lives are cut far too short. The types of CF symptoms and how severe they are can differ widely from person to person. Therefore, although treatment plans can contain many of the same elements, they are tailored to each person's unique needs. Tré, a 24-year-old with CF, wearing his vest. People with CF and their families have expertise in how the disease affects them and how their daily lives affect the way they approach their care. By acknowledging each other's expertise, people with CF, their families, and clinical care teams can work together to develop treatment plans that align personal life goals with health goals. “My doctor and I decided to come up with a plan that would work for me. We were able to negotiate a deal so that I was doing more treatments than I had been, but I wasn’t just sitting at home hooked up to machines.” — Betsy Sullivan, a teenager with CF, from the CF Community Blog Each day, people with CF complete a combination of the following therapies:
Support From the CF FoundationThe CF Foundation supports people with CF by: Accrediting more than 130 care centers. These centers are staffed by dedicated health care professionals who provide expert CF care and specialized disease management. When a group of parents started the Cystic Fibrosis Foundation in 1955, there were no treatments for cystic fibrosis. These parents set their sights high, to:
In the following years, the fundraising and commitment of the CF community has enabled the Foundation to support fundamental research in the laboratory that has led to groundbreaking discoveries, including identifying the gene and protein responsible for cystic fibrosis. By expanding our knowledge of the underlying biology of the disease and its effect on the body, researchers have paved the way for creating new treatments. The Foundation's steadfast commitment to advancing CF research has helped enable more than a dozen new treatments for the disease. We have made incredible progress, including the approvals by the U.S. Food and Drug Administration (FDA) of Kalydeco® (ivacaftor), Orkambi® (lumacaftor/ivacaftor), Symdeko® (tezacaftor/ivacaftor), Trikafta® (elexacaftor/tezacaftor/ivacaftor), Cayston® (aztreonam), and TOBI® (tobramycin). Watch this video to see how clinical research has made a difference in the lives of people with CF. Research by dedicated scientists and clinicians from a wide range of disciplines advances our understanding of cystic fibrosis every day, helping to shape clinical care practices for people living with the disease for years to come. These include studies conducted using patient data in the CF Foundation's Patient Registry, which are helping us identify trends and track the effectiveness of treatments. From bench to bedside, the Foundation is supporting the best research here and abroad to improve the quality of life of people with CF today and increase the speed of innovative research and drug development to add tomorrows. Two major initiatives will help us in this mission. Since the launch of the Infection Research Initiative in 2018, the Foundation has funded more than $100 million to this comprehensive effort to improve the detection, diagnosis, prevention, treatment, and outcomes of CF-related infections. The Foundation also is actively pursuing and funding a broad portfolio of new treatments for other complications of the disease, such as inflammation, excessive mucus, gastrointestinal problems, and cystic fibrosis-related diabetes. The second major initiative is the Path to a Cure, an ambitious research agenda to deliver treatments for the underlying cause of the disease and a cure for every person with CF. The Foundation is challenging potential collaborators to submit proposals that will accelerate the pace of progress in CF drug discovery and development and intends to allocate $500 million to the effort through 2025. The Path to a Cure centers around two core strategies to address the underlying cause of CF: restoring CFTR protein when none exists and fixing or replacing the underlying genetic mutation to address the root cause of CF. By pursuing these bold strategies and others, the CF Foundation continues to build a robust pipeline of potential new therapies that fight the disease from every angle. Learn more about the CF Foundation's key research programs:
What is an inherited disease of the respiratory system?Cystic fibrosis is an inherited disease characterized by an abnormality in the glands that produce sweat and mucus. Cystic fibrosis affects various organ systems in children and young adults, including the respiratory system, digestive system and reproductive system.
What is the most common genetic lung disorder?Cystic fibrosis is one of the most common genetic disorders in white people in the United States, occurring in one of every 3,200 live births. It is less common in African Americans (1 in 17,000), Asian Americans (1 in 31,000) and Native Americans.
Which of the following is a genetic disorder affecting the lungs?What Is Cystic Fibrosis? Cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs.
Is pneumonia inherited disease?Genetic factors can increase the risk of respiratory infections, including acute bronchitis and pneumonia. Most often, genetic polymorphisms underlie vulnerability to recurrent infections, but in rare cases monogenic defects are responsible (table 1).
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