An inherited condition that causes severe damage to the lungs, digestive system, and various other body organs is called cystic fibrosis (CF). Cells that produce digestive juices, mucus, and sweat are affected by cystic fibrosis. Normally, these secreted fluids are slippery and thin. In people having cystic fibrosis, a defective gene that makes secretions thick and sticky.  Usually, these secretions work as lubricants, but in the case of cystic fibrosis, these secretions accumulate in ducts and tubes, and passageways, commonly in the pancreas and lungs. 

Cystic fibrosis is a progressive disease and daily care is required for it. People having cystic fibrosis can attend work and school. Nowadays, individuals suffering from cystic fibrosis have better life quality than cystic fibrosis patients in the previous decade. Screening and care changes show that individuals with CF may now live in their mid-to-late 30s or 40s, and some live in their 50s.

 

Symptoms of cystic fibrosis 

Before the development of symptoms, cystic fibrosis can be diagnosed within the initial month of life due to newborn screening in the U.S. However, before the availability of newborn screening people born before may not be diagnosed until the sign and symptoms of CF appear. 

The signs and symptoms of cystic fibrosis depend on the severity of the disease. With the passage of time, the signs and symptoms may get worse or improve in the same individual. Symptoms may appear in teenagers or adulthood. If this disease is not diagnosed till adulthood, then it may be due to mild disease and have atypical symptoms, such as recurring pneumonia and infertility. The salt level in the sweat of cystic fibrosis patients is more than normal individuals. The following are the signs and symptoms of cystic fibrosis-related to the digestive system and respiratory system. 

 

Respiratory signs and symptoms

Clogging of the tubes that carry air in and out of your lungs due to the sticky and thick mucus in cystic fibrosis. The following are the signs and symptoms related to the respiratory system. 

• Production of thick mucus or sputum along with persistent cough
• Wheezing sound 
• Severe and repeated lung infections 
• Recurrent sinusitis 
• Stuffy nose and inflamed nasal passages 
• Exercise intolerance 

 

Digestive signs and symptoms

Tubes that hold digestive enzymes from your pancreas to your small intestine can even obstruct the thick mucus. Your intestines are not able to properly consume the nutrients in the food you eat without these digestive enzymes. Sometimes, the effect is: 

• Greasy stools 
• Foul-smelling 
• Poor weight gain 
• Poor growth 
• In newborns, occurrence of intestinal blockage 
• Frequent straining while passing stools
• Severe or chronic constipation¹ 

 

Causes of cystic fibrosis

The defect in cystic fibrosis transmembrane conductance regulator gene or CFTR gene will cause cystic fibrosis. The movement of salt and water in and out of your body cells is controlled by this gene. An abrupt change or mutation in the CFTR gene leads to thickening and sticking of mucus. The accumulation of abnormal mucus in various organs of the body, including 

 

• Lungs 
• Intestine 
• Liver 
• Pancreas 

 

The amount of salt in your sweat is also increased by this CFTR gene. The CFTR gene may be affected by several different defects. The type of mutation is consistent with cystic fibrosis severity. The defective gene is passed down from the parents to the infant.

A kid must inherit one copy of the gene from each parent in order to have cystic fibrosis. They won't develop the disorder if they just inherit one copy of the gene. They would, however, be a carrier of the faulty gene, which ensures they will pass the gene down to their own offspring².

 

Treatment of Cystic Fibrosis

There is no remedy for cystic fibrosis, but complications can be relieved with drugs and other interventions.

 

Medicines:

Your doctor can prescribe you medicines that open the airways, make your mucus thin, prevent infections, and help your body get nutrients from food.

 

Antibiotics:

They assist your lungs to perform better as they prevent or treat lung infections. Antibiotics can be used in many forms like shot, pills or as an inhaler. 

 

Anti-inflammatory medicines:

For cystic fibrosis, anti-inflammatory medicines are also used including corticosteroids and ibuprofen. 

 

Bronchodilators:

They can be used as inhalers to open and relax the airways. 

Mucus thinners:

They are also used in the form of inhaler and get the gunk out of your airways. 

CFTR modulators:

They mimic the CFTR and assist you to gain weight and improve the functioning of your lungs. 

 

Airways clearance techniques: 

You can get rid of thick and sticky mucus by using various airway clearance methods such as 

Percussion or chest therapy: clasping or tapping on your back or chest to clear mucus from your lungs. 

Oscillating devices: Breathing into a special oscillating device that vibrates your airways. Your mucus can easily cough up due to thinning or loosening of mucus³. 

 

Nutritional therapy for digestive symptoms

 

It is better to consult your doctor in case of cystic fibrosis as it affects the absorption of nutrients and digestive functions. You must discuss your diet with your doctor. The digestive systems can be managed by taking help from a dietitian and nutritionist. 

To balance the absorption of nutrients, different foods and supplements can be added to the daily routine including vitamins, salt, pancreatic enzymes that balance the nutrient absorption. The body's growth can also be affected by cystic fibrosis. In children with CF, a high-fat diet and high-calorie diet are required for normal growth⁴. 

 

 

References

 

1. Cystic fibrosis. [internet]. [cited 2021 February 21]; Available from  https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
2. Cystic fibrosis. [internet]. [cited 2021 February 21]; Available from  https://www.healthline.com/health/cystic-fibrosis#causes
3. Cystic fibrosis. [internet]. [cited 2021 February 21]; Available from  https://www.webmd.com/children/what-is-cystic-fibrosis
4. Everything you need to know about cystic fibrosis. [internet]. [cited 2021 February 21]; Available from  https://www.medicalnewstoday.com/articles/147960#causes

 

 

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