Life expectancy increases, general health improves. In cystic fibrosis, hope is at the rendezvous according to the results of a Canadian study.
The 36 37 call centers are reopening their lines for the 2014 Telethon. All weekend long, the French will be able to support research against rare genetic diseases. It is also an opportunity to announce good news. In cystic fibrosis, which mainly affects digestive and respiratory functions, the prognosis of patients has improved for 20 years, according to a study published in theEuropean Respiratory Journal.
Improved overall health
The authors of the article used a Canadian registry that followed 5,700 patients with cystic fibrosis over 5-year periods between 1990 and 2012. They conclude that the overall prognosis has changed in the right direction. “The life expectancy and average age at death of patients with cystic fibrosis has increased significantly in 20 years. Life expectancy rose from around 31 to 49 years between 1990 and 2012, ”explains Isabelle Durieu, internist at the CHU de Lyon (Rhône) and president of the French CF Society. “The median age at death has gone from 21 to 32 years old. Life expectancy for the most recent cohorts will soon reach around 50 years, which is described in most Western countries. “
These good results can be explained by the fact that the patients are in better general health. The maximum expiratory volume in one second, which is used to measure breathing capacity, is higher than twenty years ago. In addition, the nutritional status of patients has changed, and we notice a little more overweight patients. “The improvement in the prognosis is probably linked to the fact that the nutritional status of patients has improved and that their respiratory function deteriorates less quickly”, analyzes Isabelle Durieu. “The whole helps to improve life expectancy. “
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A disease with multiple manifestations Cystic fibrosis is a genetic disease that affects 200 newborns per year. Of genetic origin, it is characterized by a mutation in the gene encoding the CFTR protein, present in cells of different mucous membranes. This is why the disease has respiratory symptoms (the best known) but also digestive symptoms. Most often, mucus clogs the bronchi and causes chronic obstructive pulmonary disease (COPD) and ultimately respiratory failure. This mucus also promotes infections. At the gastrointestinal level, the majority of patients suffer from pancreatic insufficiency. The mucus present in the digestive system promotes the stops of transit, and therefore the alternations of diarrhea / constipation, malabsorption of nutrients and vitamins. Other symptoms may appear (cirrhosis of the liver, diabetes, heart disease). |
Diabetes increases the risk of death
If the general state of health of patients improves, certain comorbidities are associated with a high risk of death. Pulmonary exacerbations (increased coughing, congestion, volume and purulence of sputum, or difficulty breathing) quadruple this risk. The presence of malnutrition doubles the likelihood of the patient dying, as does a diagnosis of diabetes in patients.
For the moment, cystic fibrosis does not have a cure. Different therapeutic approaches, however, make it possible to soothe those affected by it: respiratory physiotherapy allows capacity to be rehabilitated. Inhalation treatments are available, and many antibiotics help manage chest infections. Nutrition is included in the management of patients, as a rich and balanced diet is important to maintain a stable state of health.
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