Cystic Fibrosis

Cystic Fibrosis

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Cystic fibrosis is a hereditary disease that causes severe damage to the digestive system, lungs, and other organs in the body. It affects sweat, mucus, and digestive juices-producing cells. Normally slippery and thin, the faulty gene causes these fluids to become sticky and thick in people with Mucoviscidosis. These fluids essentially clog the ducts, tubes, and passageways between the lungs and pancreas without performing their lubricating function. The condition progresses over time and you need to take care of your own health.

Fibrosis

Causes of cystic fibrosis

A mutation in a gene changes a protein responsible for controlling the movement of salts in and out of cells and causes Mucoviscidosis. The gene is the Mucoviscidosis transmembrane conductance regulator (CFTR) gene. Due to this transformation, sweat increases with salt, and mucus in the digestive, respiratory, and reproductive systems becomes thick and sticky. Gene defects can vary. The extent of the condition is determined by the specific kind of gene mutation that occurs. Children with only one copy of the gene are carriers of the disease and pass on their genes to their children.

Symptoms of cystic fibrosis

Signs and symptoms of the disease differ according to the severity of the condition. Some people may not experience any symptoms until adolescence or young adulthood, while others may experience symptoms from childhood. People diagnosed with the condition at a young age have symptoms such as infertility, pancreatitis (inflamed pancreas), and recurrent pneumonia. Their sweat contains more than normal levels of salt. Most of the symptoms of this disease affect the digestive and respiratory systems.

Digestive symptoms: Thickened mucus blocks the tubes that carry digestive enzymes from the pancreas to the small intestine. Without these digestive enzymes, your gut cannot fully absorb the nutrients from the food you eat. It causes sticky, foul-smelling stools, bowel obstruction, weight loss, chronic or severe constipation, and poor growth.

Respiratory symptoms: Cystic fibrosis causes sticky and thick mucus to choke the tubes that carry air from your lungs.

Signs and symptoms caused by this are:

  • Practice intolerance
  • Stuffy nose
  • Frequent cough with thick mucus or sputum
  • Recurrent lung infections
  • Recurrent sinusitis
  • Wheezing
  • Inflamed nasal passages
  • Diagnosis of cystic fibrosis

Your physician will perform a thorough physical assessment, conduct a series of tests, and carefully examine your symptoms to diagnose Mucoviscidosis.

Screening tests for newborns

Doctors in many places perform screening tests to diagnose cystic fibrosis in newborns. Due to early diagnosis, treatment can be started immediately. In a blood sample, as part of a screening test, doctors check for higher than normal levels of IRT (immunoreactive trypsinogen), a chemical released from the pancreas. However, levels of the chemical may be higher in babies born prematurely or with stressful deliveries. This gives rise to the need for other tests to diagnose the presence of cystic fibrosis.

Doctors may also perform a sweat test once a child is two years old to evaluate for cystic fibrosis. He or she will apply a sweat-inducing chemical to a small area of skin. After that, the doctor will collect sweat to test it and check the salinity. Doctors may also want to do genetic testing that identifies specific gene defects that cause cyst fibrosis.

Screening tests for adults

Doctors will recommend screening tests to children and adults who have not had a screening test at birth. If you have an inflamed pancreas, chronic sinus or lung infections, nasal polyps and recurrent bronchiectasis; Your doctor will then ask you to take genetic and sweat tests to confirm cystic fibrosis.

Treatment options for cystic fibrosis

Although there is no perfect cure for this disease, treatment can help relieve symptoms and reduce complications. Early diagnosis with regular monitoring slows disease progression and improves quality of life.

Medicine

Cystic

Your doctor will prescribe medications that target the gene mutation and treat the one that causes cystic fibrosis. He may ask you to take antibiotics to prevent and/or treat lung infections. Other medicines besides these are:

  • Bronchodilators (breathing medicines) to keep the air passages open by relaxing the muscles around the bronchial tubes.
  • Anti-inflammatory medicines to reduce swelling in the airways in your lungs.
  • Stool softeners which prevent intestinal obstruction and constipation.
  • Mucus thinners such as hypertonic saline help improve lung function.
  • Certain medicines for liver disease or diabetes.
  • Digestive aids in the form of oral pancreatic enzymes can assist your digestive system in effectively absorbing essential nutrients.
  • Acid-reducing drugs for proper functioning of pancreatic enzymes.

Gene-targeted drugs

If you have a gene mutation, your doctor may recommend cystic fibrosis transmembrane conductance regulator (CFTR) modulators. The drug improves the function of the defective CTFR protein. It reduces salt content in sweat and improves lung function. Your pharmacist may perform an eye exam and liver function test before prescribing these medications. Regular follow-ups help monitor the disease while you take these medications.

Airway clearance techniques

Also known as chest physical therapy (CPT), these techniques relieve mucus blockages to reduce inflammation and infection in the airways. They loosen thick mucus in the lungs and circulate several times a day. Breathing and coughing techniques help loosen mucus.

Pulmonary rehabilitation

Pulmonary rehabilitation includes nutritional advice, physical exercise, education about your condition, breathing exercises to improve breathing, and advice and support.

Surgery and other treatments

Different surgical procedures for cystic fibrosis are:

Nose and Sinus Surgery

Clearing nasal polyps, which can impede your breathing and address persistent sinusitis (inflammation in the sinus), is a proceis aimed at enhancing your respiratory well-being.

Oxygen therapy

Your doctor may ask you to breathe pure oxygen to prevent high blood pressure in the lungs.

Feeding tube

It helps in providing extra nutrients. This is a temporary tube inserted into your nose and guided up to your stomach. It helps in giving extra calories during the day or night.

Noninvasive ventilation

Noninvasive ventilation provides positive pressure to your airways and lungs when you breathe. It is usually combined with oxygen therapy to increase air exchange in the lungs and maintain airway clearance.

Abdominal surgery

Surgery removes the intestinal obstruction.

Liver transplant

For severe cases of the disease, a liver transplant is the right option.

Lung transplant

Lung transplantation is an ideal choice for severe respiratory distress, increased resistance to antibiotics for lung infections, and life-threatening lung complications. There is no chance of cystic fibrosis recurring in your body after a lung transplant.

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