Doha: Life expectancy of individuals in Qatar with cystic fibrosis, a genetic disorder, has reached equal to global standards due to advanced care and treatment methods available at the Hamad Medical Corporation (HMC).
HMC provides all latest medications and treatment methods to individuals with cystic fibrosis.
“Although cystic fibrosis requires daily care, people with the condition are usually able to attend school and work, and often have a better quality of life than people with cystic fibrosis had in previous decades,” said Dr. Mona Allangawi, Senior Consultant Pulmonary at HMC.
“Progress in early diagnosis and treatments lead to improvements in life expectancy. Therefore, life expectancy for people with cystic fibrosis nowadays is between 40s and 50s years of age,” she added.
Cystic fibrosis is an inherited life-threatening disorder that damages the lungs and digestive system including pancreas, liver, and intestines.
“Cystic fibrosis in Qatar is not rare, we have 77 patients with the condition receiving treatment. Among them 62 are Qataris, and 45 are children below the age of 18 years, ” said Dr Allangawi.
“Consanguineous marriages are one of the main reasons for cystic fibrosis in Qatar. If both parents are carrier for cystic fibrosis gene, with every pregnancy they will have a 25% chance in having an affected child and a 25% chance in having a child carrying the disease, while 50% chance of having a healthy child,” she added.
Dr Allangawi has urged people who undergo premarital screening to follow advice given by genetic counselors.
“The condition arises from mutations in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR), which leads to the abnormal transport of chloride and sodium across the epithelium. This makes the body secretions thick in the lungs and the digestive system, leading to recurrent chest infections, recurrent pancreatitis, pancreatic Insufficiency, intestinal obstruction and malabsorption,” said Dr Allangawi.
Bacterial changes begin in the first few years of life, very thick secretion leading to obstruction in the airway makes children repeatedly prone to bacterial infections and inflammation and shortens their lifespan. Therapies to break up mucus could postpone the increase in bacterial burden as well as might offer route to a longer life for such patients.
“People with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system and digestive system. However, adults diagnosed with cystic fibrosis are more likely to have atypical symptoms, such as recurring bouts of inflamed pancreas (pancreatitis), infertility and recurring pneumonia,” said Dr Allangawi.
“Lung symptoms in children with cystic fibrosis are likely due to an increased burden of bacteria. This implies there’s an opportunity for early intervention that could dramatically increase the quality of life for these children,” she added.
There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications.
“Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. Even in the same person, symptoms may worsen or improve as time passes. Close monitoring and early, aggressive intervention is recommended. Managing cystic fibrosis is complex, so consider obtaining treatment at a place staffed by doctors and other staff trained in cystic fibrosis. At HMC a multidisciplinary team of doctors and medical professionals trained in cystic fibrosis to evaluate and treat the condition,” said Dr Allangawi.
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