Cystic Fibrosis Treatment in India

What is Cystic fibrosis (CF)?

Cystic fibrosis (CF) is a genetic condition that causes severe damage to the respiratory system, digestive system and reproductive system. The most commonly affected organs include the:

  • Lungs
  • Pancreas
  • Liver
  • Intestines

Cystic fibrosis is an inherited life-threatening disease and it affects many organs. It causes changes in the electrolyte transport system and causes cells to absorb too much sodium and water. Cystic fibrosis is recognised by problems with the glands that make sweat and mucus. It affects the cells that produce thick and sticky mucus, sweat and digestive enzymes. Early diagnosis and treatment are critical for improving quality of life and lengthening the expected lifespan. Although cystic fibrosis is progressive and requires daily care, people can attend school and work.

What Are the Symptoms of Cystic Fibrosis? 

The symptoms of cystic fibrosis can vary depending on the severity of the disease. As time passes, the symptoms associated with the disease may get better or worse. One of the first signs of cystic fibrosis is a strong salty taste to the skin. However, people may experience symptoms differently, and the following are the most common symptoms of Cystic fibrosis (CF):

  • Thick mucus that blocks organs, such as the lungs, intestines and pancreas that cause malnutrition, poor growth, breathing problems, frequent respiratory infections, and chronic lung disease.
  • Infertility in males
  • Cystic fibrosis-related diabetes (CFRD)
  • Gastroesophageal reflux disease (GERD): In this case, stomach contents rise into the esophagus and causes serious damage.

The symptoms of Cystic fibrosis (CF) differs for each person and some Infants born with CF show symptoms by age 2, while some children may not show symptoms until later in life. The following are the common symptoms for infants:

  • Diarrhea that does not go away
  • Foul-smelling stools
  • Greasy stools
  • Frequent wheezing
  • Frequent pneumonia or other lung infections
  • Persistent cough
  • Skin that tastes like salt
  • Poor growth despite having a good appetite

Important Note: A lung transplant is necessary for people with the end-stage cystic disease.

What are the respiratory symptoms of Cystic Fibrosis?

The thick and sticky mucus with cystic fibrosis blocks the tubes that carry air in and out of your lungs. This can cause the following symptoms:

  • A persistent cough producing thick mucus (sputum)
  • Wheezing
  • Recurrent lung infections
  • a stuffy nose
  • Recurrent sinusitis

What are the Digestive symptoms of Cystic Fibrosis?

The thick mucus can also block tubes that carry digestive enzymes produced by the pancreas to the small intestine. Without these digestive enzymes, the intestines cannot absorb the nutrients from the food. This results in:

  • greasy, foul-smelling stools
  • nausea
  • loss of appetite
  • poor weight gain in children
  • Intestinal blockage, particularly in newborns
  • Chronic or severe constipation, which causes part of the rectum to protrude outside the anus (rectal prolapse)

Cystic fibrosis requires consistent, regular follow-up with your physician. You can contact your physician if you experience symptoms, such as more mucus than usual or a change in the mucus colour, lack of energy, weight loss, or severe constipation.

How Is Cystic Fibrosis Diagnosed?

All newborns are screened for cystic fibrosis and the physician uses a genetic test or blood test to check for signs of the disease. The genetic test will help you to detect whether your baby has a defective CFTR gene. The blood test determines whether a baby’s pancreas and liver are working correctly. The other diagnostic tests may include:

  • Immunoreactive Trypsinogen (IRT) Test
  • Sweat Chloride Test
  • Sputum Test
  • Pulmonary Function Tests (PFTs)
  • Chest X-rays, ultrasound, and CT scans
  • Stool tests

How Is Cystic Fibrosis Treated?

Although there is no cure for cystic fibrosis, there are various treatments available that may help to relieve symptoms and reduce the risk of complications.

  • Medications – Antibiotics may be prescribed to get rid of a lung infection and to prevent another infection from occurring in the future. Mucus-thinning medications make the mucus thinner and less sticky which improves lung function. Nonsteroidal anti-inflammatory drugs (NSAIDs) may help to reduce any pain and fever associated with cystic fibrosis. Bronchodilators relax the muscles around the tubes that carry air to the lungs helps to increase airflow. This medication is taken through an inhaler or a nebulizer.
  • Lung Transplant A lung transplant surgery may be necessary when someone with cystic fibrosis has severe breathing problems. In some cases, both lungs may need to be replaced. This can lead to serious complications after surgery, including pneumonia.

Diet and nutrition for Cystic fibrosis:

A person with Cystic fibrosis has trouble absorbing proteins, fats, and vitamins A, D, E, and K and physicians may recommend antacids, multivitamins, and a diet high in fibre and salt. You must do the following if you have cystic fibrosis:

  • Drink plenty of fluids because it can help you to thin the mucus in the lungs.
  • Exercise regularly to help loosen mucus in the airways. Some exercise includes walking, biking, and swimming.
  • Avoid smoke, pollen, and mould whenever possible. These irritants can make symptoms worse.
  • Get influenza and pneumonia vaccinations regularly.

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