Management Of Cystic Fibrosis

Cystic fibrosis is an inherited disorder that causes thick, sticky mucus to build up in the lungs, digestive tract, and other areas of the body. It is one of the most common chronic lung diseases in children and young adults. It is one of the life-threatening disorder.

As cystic fibrosis leads to complex and multisystemic involvement patients need care by specialists, treatment and follow-up care at specialty centers with multidisciplinary care teams.

Although there is no cure for cystic fibrosis there are several treatment methods available to control the symptoms and to prevent complications.

The primary goals of CF treatment include the following:

• Maintaining lung function as near to normal as possible by controlling respiratory infection and clearing airways of mucus.
• Administering nutritional therapy (ie, enzyme supplements, multivitamin and mineral supplements) to maintain adequate growth.
• Managing complications.

Pharmacological treatment

• Antibiotics: to treat and prevent lung infections
• Mucus-thinning drugs: to help you cough up the mucus, which improves lung function
• Bronchodilators: to help keep your airways open by relaxing the muscles around bronchial tubes
• Oral pancreatic enzymes: to help digestive tract absorb nutrients.

Chest physical therapy
Loosening the thick mucus in the lungs makes it easier to cough up. Chest physical therapy helps loosen mucus and is usually done from one to four times a day. A common technique is clapping with cupped hands on the front and back of the chest.

Changing position can also make it easier to remove mucus from lungs. This is known as postural drainage.

Nutritional Counselling
In general, a normal diet with additional energy and unrestricted fat intake is recommended. A high-energy and high-fat diet, in addition to supplemental vitamins (especially fat soluble) and minerals, is recommended to compensate for malabsorption and the increased energy demand of chronic inflammation.

In children, because of various physical activities and eating habits, they might need more energy based on growth and weight gain. Special consideration is given to female patients with a potential for delayed puberty because of malnutrition, patients with diabetes mellitus, and patients with liver disease.

Nutritional supplements in the form of either high-energy oral preparations or enteral feeds (eg, elemental formulas, high-fat mixtures) via nasogastric tube or gastrostomy may be indicated in some patients.

For patients with cystic fibrosis, getting the right nutrition is very important. They also need pancreatic enzymes to help absorb fats and protein as well as vitamin supplements, especially vitamins A, D, E, and K.

Oxygen therapy
Sometimes if blood oxygen level declines oxygen inhalation is helpful to prevent pulmonary hypertension.

Surgical Management
Surgical options are used in certain conditions like:

• Development of Meconium ileus or Intussuception as a complication.
• Removal of nasal polyps.
• Chest tube insertion if pneumothorax develops.

Lung transplantation
Lung transplantation is indicated for end stage lung disease.

Home remedies

• Avoiding smoke, dust, dirt, fumes, household chemicals, fireplace smoke, and mold or mildew
• Clearing or bringing up mucus or secretions from the airways. This must be done one to fours times each day. Patients, families, and caregivers must learn about doing chest percussion and postural drainage to help keep the airways clear
• Drinking plenty of fluids. This is particularly true for infants, children, in hot weather, when there is diarrhea or loose stools, or during extra physical activity
• Exercising two or three times each week. Swimming, jogging, and cycling are good options.