Promising research news for Treating Cystic Fibrosis Lung Disease

Encouraging  research news for Treating Cystic Fibrosis Lung Disease

Before finding more information about this new research, let us see what is Cystic Fibrosis Lung Disease.

What is Cystic Fibrosis Lung Disease (CF)?

 

Cystic fibrosis (CF) is an inherited disease that causes thick, sticky mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, causing lung damage and making it hard to breathe. In the pancreas, it clogs the pathways leading to the digestive system, interfering with proper digestion.

Cystic fibrosis is the second most common inherited disorder occurring in childhood in the United States, behind sickle cell anemia. More than 10 million Americans carry the defective cystic fibrosis gene without knowing it.

Approximately 30,000 people in the United States have CF. About 1,000 new cases of cystic fibrosis are diagnosed each year.

 What Causes Cystic Fibrosis?

 

A person must inherit two defective CF genes—one from each parent—to have CF.

Each time two carriers of the defective gene conceive, there is a:

25 percent chance that their child will have cystic fibrosis

50 percent chance that the child will inherit one defective gene and be a carrier but not have the disease

25 percent chance that the child will not have the gene at all

What are the Symptoms of Cystic Fibrosis?

The most common symptoms of CF are:

Very salty-tasting skin

Persistent coughing, wheezing or shortness of breath

Excessive appetite but poor weight gain

Greasy, bulky stools

How is Cystic Fibrosis Detected?

CF is diagnosed through the sweat test, which measures the amount of salt in the sweat. A high salt level indicates that a person has CF.

CF also can be identified before birth through prenatal screening and after birth through newborn screening.

How is Cystic Fibrosis Treated?

Treatment for CF depends on the stage of the disease and the organs involved. It may include:

Airway clearance techniques to clear mucus from the lungs

One technique is called postural drainage and percussion. The person with CF sits, stands or lies in a position that helps free up mucus. The chest and back are pounded and clapped to loosen the mucus. A person with CF may use a mechanical vest or blow into a device that shakes the mucus loose.

Inhaled medicines

These may be taken with a nebulizer, a machine that changes liquid medicine into a fine mist which makes it easier to inhale deep into the lungs. Some medicines can also be breathed in through a metered dose inhaler (MDI). Inhaled medicines for CF include:

TOBI, an aerosolized antibiotic used to treat lung infections

Pulmozyme, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function

Bronchodilator medicines to help open the airways

Hypertonic saline to help draw more water into the airways to thin mucus

Other treatments for CF

Azithromycin, an antibiotic that fights bacteria in the lungs

Anti-inflammatory medicines such as ibuprofen to help reduce swelling in the airways

Lung transplantation may be an option in some severe cases of CF.

New Research- Potential Treatment for Cystic Fibrosis Lung Disease

The cellular pathway that causes lung-damaging inflammation in cystic fibrosis (CF) has not only been identified, but scientists have also discovered that decreasing the pathway's activity can cause the inflammation to reduce. A previous report indicated that researchers were able to prevent cystic fibrosis lung disease in a mouse model by spraying amiloride into the animal's lungs. The current finding came from Vancouver experts who published the findings in the Journal of Immunology and believe that their research can aid to the development of a new drug target for the treatment of CF lung disease. This is significant, because many CF patients develop this illness and experience death because of it. "Developing new drugs that target lung inflammation would be a big step forward," said research leader Dr. Stuart Turvey, director of clinical research, and senior clinician scientist at the Child & Family Research Institute and a pediatric immunologist at BC Children's Hospital.

 The team analyzed the difference between normal lung cells' immune response with the immune response of CF lung cells, after exposing both to bacteria. When healthy cells are exposed to bacteria, it causes the cell to secrete special molecules that pull immune cells to protect against infection. The unfolded protein response, which is a succession of molecular events, is more highly activated when the CF lung cells are exposed to bacteria, according to the authors. This results in the secretion of more molecules that attract a significant amount of immune cells, eventually leading to more inflammation. When using a special chemical to treat the CF cells, the unfolded protein response went back to normal and the cells' immune response became stable.

 Cystic Fibrosis is the most prevalent genetic disease in Canadian youths. In Canada, one in every 3,600 kids are born with CF. Unfortunately, there is no treatment to cure the disease. Individuals with CF become vulnerable to bacterial lung infections when there is a build-up of mucus in the lungs, which causes inflammation and swelling. Since infections are constantly developing, over time, the lungs become extremely damaged and may lead to the need for a transplant. Lung inflammation can currently only be treated with steroids and anti-inflammatory drugs, which have serious negative effects. The authors plan to conduct more research with a larger number of lung cell samples from CF patients in order to confirm their findings.

(Source-Journal of Immunology)