Cystic fibrosis is an extremely rare condition that affects around one hundred thousand people worldwide.
What is cystic fibrosis? Fibrosis is a process in which healthy tissue that makes up an organ begins to be replaced by connective tissue, leading to impaired function and the manifestation of various symptoms.
Connective tissue is structurally firmer than the usual tissue of an organ, and reduced elasticity results in a range of complications.
Cystic fibrosis is often genetically conditioned, an inherited disease. However, fibrosis can also occur as a result of various injuries, chronic infections, or autoimmune diseases.
Cystic fibrosis is an exceptionally rare disease. Nevertheless, it is considered the most common rare disease among people of Caucasian descent.
Statistically, one out of 2,500 to 3,500 babies is born with cystic fibrosis.
Fibrosis most commonly affects the lungs, but it can occur in other organs, including:
- Skin fibrosis
- Bone fibrosis
- Muscle fibrosis
- Heart fibrosis
- Liver fibrosis
- Kidney fibrosis
- Fibrosis due to lymphedema
Cystic fibrosis symptoms and their severity in adults vary depending on the stage of the disease and the individual's overall health.
Liver fibrosis is most commonly the result of liver cirrhosis, where normal liver tissue is gradually replaced by scar tissue. Heart fibrosis can be the consequence of damage to the heart muscle.
Cystic fibrosis is a chronic condition that requires continuous therapy to control the progression of the disease. Until recently, the foundation of treatment focused on alleviating the symptoms.
However, cystic fibrosis treatment and therapeutic approaches have continuously improved, and today there is a drug that can lead to the complete resolution of symptoms.
Therapy involves prescribed antibiotics, medications that aid digestion, and medications that expand airways, along with daily inhalation.
Fibrosis as a Result of Lymphedema
Untreated lymphedemas in advanced stages can lead to the development of fibrosis. Lymphedema is a condition that occurs when lymph, the fluid circulating through the lymphatic system, cannot flow normally through the tissue.
Flow obstruction leads to fluid accumulation and swelling in the affected parts of the body, typically the extremities.
If lymphedema is not adequately treated, and its progression is not controlled, prolonged tissue damage can occur, leading to fibrosis.
When fibrosis develops in areas previously affected by lymphedema, soft tissue becomes hard scar tissue, losing its initial elasticity and function. In addition, fibrosis complicates lymphedema treatment and increases the risk of numerous complications. The most common complications include reduced limb mobility, the onset of infection, and potential pain.
Early recognition and treatment of lymphedema prevent the development of fibrosis and other complications.
In cases where pulmonary fibrosis is genetically conditioned, symptoms can be observed in the earliest stages of life, shortly after birth.
What is fibrosis, and what are its symptoms in infants? A symptom of fibrosis in newborns may be the absence of the first stool within the first 24 hours after birth.
Cystic fibrosis in infants can be diagnosed early, and the content of the intestines in these babies is denser than usual, leading to bowel blockages. Additionally, greenish vomiting and a swollen abdomen may occur.
Various genetic tests allow the diagnosis of cystic fibrosis in newborns. Moreover, an increased concentration of chloride ions in sweat suggests cystic fibrosis.
Affected children in adulthood may develop diabetes, chronic liver disease, and fertility problems, conditions that cystic fibrosis can encourage.
What is the Life Like for Children with Cystic Fibrosis?
Cystic fibrosis of the lungs causes thick mucus to accumulate in the lungs. The accumulated mucus is the reason for difficult breathing and frequent respiratory infections.
Children with cystic fibrosis experience frequent bouts of characteristic, intermittent coughing. Therefore, inhalation therapy is a part of their daily routine.
In addition to respiratory issues, cystic fibrosis affects the digestion of food and the absorption of nutrients. As a result, children with cystic fibrosis often struggle with underweight despite proper nutrition and having a normal appetite.
Occasional hospitalizations are often part of the lives of children with cystic fibrosis, especially if there are severe respiratory infections or complications.
Symptoms of lung fibrosis and their intensity vary depending on the child's age, overall health, and the stage of the disease.
Although children with cystic fibrosis can attend school and regular activities, they may frequently miss school.
It is advisable for the child to maintain distance from other individuals who currently have respiratory infections, leading to occasional absences from school, especially during the flu season.
Treatment of Cystic Fibrosis and Innovative Drug for Pulmonary Fibrosis
A revolutionary drug for pulmonary fibrosis has recently become available in Serbia and has already changed the lives of many patients with cystic fibrosis.
The drug is intended for individuals older than six years. This innovative drug allows individuals with cystic fibrosis to alleviate symptoms and lead a normal life.
The bodies of those treated become significantly stronger, capable of withstanding more exertion. Coughing diminishes, and patients generally gain weight if they had previously experienced a lack of kilograms.
Thanks to this innovative drug, people with cystic fibrosis can continue their lives without symptoms of this progressive and challenging disease.
SCHEDULE A PHYSIATRIST'S EXAMINATION
Dr Jelena Kluz-Đurđević, a specialist in physical medicine
After a conversation with the patient, Dr. Jelena will conduct an examination to obtain a more comprehensive view of your health status, helping to determine whether it's an injury or a diagnosis.
The examination lasts for 30 minutes and includes:
Schedule a specialist examination by calling 011 2100 588 for the cost of 4,000 RSD.