Dr. Ruth assured that there are 14 patients with this disease in the province, all of whom are being followed in consultation with a multidisciplinary team.
“In the past, cystic fibrosis was a fatal childhood disease, but thanks to our understanding of it, early diagnosis and preventive treatment of the respiratory and nutritional complications of this rare disease, it is now considered a chronic disease. In adulthood”, this is the first question that comes to mind when Dr. Ruth Rubán Nápoles, Director of the Provincial Cystic Fibrosis Committee, spoke to Venceremos.
The Master of Integrated Child Care also noted that “there are 14 patients in Guantanamo Province, 5 children and 8 adults; the situation in the country is similar.
Doctors emphasize that all patients receive consultation from a multidisciplinary team (pulmonologists, gastroenterologists, endocrinologists, psychologists, nutritionists, physiotherapists, etc.) and that their prognosis depends on early diagnosis and treatment in specialized centers. Strict treatment management. “.
Professor of Pediatrics, defining cystic fibrosis or myxomycosis, states, “It is a genetic, inherited disease that causes abnormalities in certain glands such as the eccrine glands (which produce sweat, tears, saliva, milk, and digestive juices) produces secretions.Thick, all of which can lead to organ and tissue damage, especially to the lungs and digestive tract, as well as inadequate function of the liver, bile ducts, gallbladder, and pancreas.
Regarding signs and symptoms, he explains, “Pulmonary or chronic infections associated with colds are recurring, persistent coughs and thick mucus (phlegm); nasal congestion, sinusitis, vomiting, sleep disturbances, pneumonia… As the illness progresses With the development of intercostal retraction, respiratory muscle use, barrel chest, cyanosis and decreased exercise tolerance are common phenomena.
“Pulmonary complications may be caused by non-tuberculous mycobacterial infection … and right heart failure secondary to pulmonary hypertension,” he added.
“If you have gastrointestinal symptoms Experts warn that meconium ileus (the first appearance of thick, dense, and sticky stool) due to obstruction may be the first sign of this condition in a newborn; bloating, vomiting, etc.
“Older patients,” he went on to explain, “may experience constipation or recurrent episodes of partial or complete obstruction of the small or large intestine, accompanied by colic-like abdominal pain, changes in bowel movements, decreased appetite… .
“Generally,” he emphasized, “pancreatic insufficiency manifests clinically early in life and may be progressive. Manifestations include frequent passage of large, foul-smelling stools; abdominal distension, muscle weakness despite a normal or voracious appetite Mass loss… Secondary clinical manifestations may occur, such as rectal prolapse in untreated infants and young children and gastroesophageal reflux in older infants and adults.
How cystic fibrosis is diagnosed and the importance of getting it early details “through genetic analysis of a blood sample (the disease is caused by a defective gene that causes abnormal production of mucus) or a sweat test to measure the presence of high salt levels, Indicates the presence of disease.
“After cystic fibrosis may be diagnosed in the first study, other tests are then performed, ranging from pancreatic tests to respiratory evaluations.”
The head of the Provincial Committee for Disease Control and Prevention emphasized the value of treating patients, “It must be done as soon as possible. Although a cure has not yet been found, symptoms can be alleviated, complications reduced, and the condition improved.” “The quality of life, so family members are guided and trained, So that they can comply with the requirements we indicate in professional consultations and follow-up with family doctors.”
Rubán Nápoles stressed the need to control lung infections and avoid intestinal obstruction in patients; that they receive proper nutrition; he added that when antibiotics, anti-inflammatory drugs are required, they must comply with drug therapy to stop genetic mutations, which is also urgent.
He noted, “Due to the characteristics of the disease, people with cystic fibrosis may have difficulty maintaining adequate weight, which is why we recommend families strengthen their diets with more fruits and vegetables.; Protein and calories in the diet (all types of meat, fish, shellfish, eggs, sweets, cheese…), and ensure a complete diet… Improve your immune system“.
The truth is, “balancing school and social life with the intensive medical care required by someone with cystic fibrosis can be a little overwhelming, and it can be even more complicated for family members to manage the nutrition they need during these difficult times,” However, their lives become more bearable when they know they will not lack medication or the commitment of health staff who work every day to improve their survival and quality of life,” Dr. Ruth concluded.