Cystic Fibrosis – A Life Threatening Disease

Cystic Fibrosis – A Life Threatening Disease

The beginning of the twenty-first century saw the rise of different diseases across the world. Many of these either came with the modernization period or were not that prevalent as they are today in the older times.

Genetic disorders are also a part of these diseases. In comparison with the past, inherited diseases were not as common or either were the ones that are manageable today.

Over fifteen million people alone in the United States carry the gene for one of the most dangerous diseases called cystic fibrosis which affects more than thirty thousand children and young adults.

Despite its commonness, a majority of the people have no clue what cystic fibrosis is. Cystic fibrosis is a genetic disorder in which the fundamental functions of the epithelial cells are disturbed in the body of the patient.

Epithelial cells are the necessary building blocks of the body that are needed to form and line passageways of all of the organs in the body including the heart and the circulatory system, kidneys, liver, lungs and respiratory system and reproductive organs.

The patients with cystic fibrosis have a defective gene which is responsible for impairing the function of the epithelial cells. This leads to amalgamation of sticky mucus in the body resulting in a chronic cough and lung damage.

This disturbance of epithelial cells affected the body severely. The patients experience difficulties in breathing and filtering air, digestion, and absorption of nutrients. In addition, since epithelial cells play in the whole of the body, the severity can spread throughout it.

Cystic fibrosis is one of the lifelong diseases and should be taken seriously with proper management though according to Cystic Fibrosis Foundation, it is currently not curable.

However, the foundation has also highlighted that digestive enzymes, specific dietary changes, supplements, and lifestyle changes can aid cystic fibrosis symptoms especially if it is diagnosed at early stages.

Secondly, the treatments today are much more advanced and have greatly provided a number of options on how to manage cystic fibrosis symptoms. Many patients having the disorder are now able to live into their 30s, 40s and even 50s.

Studies conducted on cystic fibrosis have shown that early diagnosis can greatly help in preventing further developments of the disease and the patient might be able to avoid many of the complications that come with it.

 

What Causes Cystic Fibrosis?

Cystic fibrosis is an inherited disorder that affects the body’s production of a type of protein known as cystic fibrosis transmembrane conductance regulator (CFTR). This protein is found in the epithelial cells found in the body.

Epithelial cells are present in the highest quantity in the digestive tract, sweat glands, lungs, and genitourinary system.

CFTR’s main role is to help the epithelial cells manage the levels of Chloride from sodium chloride. This is why when they are not working properly the levels of fluids including mucus and their consistency are disturbed.

RELATED: How To Get Rid Of Mucus In The Chest 

The balance between sodium and fluid is crucial in maintaining the covering of mucus in the organs. When the balance is disrupted, a thick layer of mucus gradually forms initially in the passageways and lungs.

Mucus plays a far more important role in the lungs than in the other organs. It stops germs, bacteria, fungus, and viruses from damaging the respiratory system and entering the body.

In people with healthy respiratory systems, the mucus is thin and easy to remove from the tracts and passageways. This is the way the bacteria, fungus or viruses are cleared from the lungs and removed from the body.

Cystic fibrosis patients, on the other hand, face difficulty in removing the mucus containing dangerous foreign invaders from the lungs because of the thick consistency of the mucus. This is why cystic fibrosis is said to cause the most damage to the lungs.

Secondly, cystic fibrosis can develop and cause harm to other organs as well. For example, a patient with this disorder will most likely experience a digestive impairment.

Similarly, the thick consistency of the mucus can halt the production of fundamental enzymes in the pancreas and intestines. The body is unable to digest and absorb nutrients properly.

Hence, people with cystic fibrosis tend to have a lot of nutritional deficiencies and slow development.

What Are The Symptoms of Cystic Fibrosis?

People with cystic fibrosis can start having symptoms from a very early age. The most visible are:

  • Developmental issues (in children and infants)
  • Wheezing
  • A chronic cough and symptoms similar to asthma
  • Nasal congestion
  • Production of thick, discolored mucus, and heavy mucus upon coughing
  • Lung infections (including pneumonia and bronchitis)
  • Repeated sinus
  • Digestive issues such as abdominal pain, chronic diarrhea, bloating, and gas
  • Weight loss
  • Nutritional deficiencies (most of the fat-soluble vitamins like vitamin K, E, A, and D)
  • Bulky and oily stools
  • Electrolyte imbalance and dehydration
  • Chronic Fatigue
  • Frosting on the skin (caused by an imbalance between salt and fluids in the skin)
  • Long-term conditions that can come along with cystic fibrosis include bronchiectasis, rectal prolapse, sinusitis, diabetes, pancreatitis, pneumothorax (lungs collapse), low bone density, nasal blockages, and gallstones.

Most of the cases of cystic fibrosis are diagnosed in children and infants, therefore life-threatening complications and developmental issues are some of the biggest concerns when it comes to the disease.

Many of the people with cystic fibrosis have weight dangerously lower than their need. New-born babies do not gain the expected weight since the mucus buildup does not allow the pancreatic enzymes to play their role in digestion and break down the needed fats and proteins.

The organs with the highest risk of infection are the lungs which are at a constant threat of getting dangerous infections such as bronchitis and pneumonia. Most of the children and infants are not able to fight off such infections.

 

What Are The Treatments For Cystic Fibrosis?

Most of the treatments for cystic fibrosis and its main effects on the lungs and digestive tract are used for the improvement of the respiratory system, absorption, and digestion of good or nutritional a nutritional boost.

Non-medication pulmonary therapy is the most commonly used method which can serve as a lifesaver for many cystic fibrosis patients.

Devices such as Bronchodilator or oscillatory positive expiratory pressure are used with mucus medication, salt solution, and physical therapy to effectively clear the passageways. These solutions can also help with preventing infections, inflammations, and increasing airflow into the lungs.

Dietary interventions are also suggested by most of the doctors. Patients with cystic fibrosis are suggested to have a diet high in calories and fat. This, however, does not mean adding a lot of processed foods in the diet.

Where these foods might be high in calories they provide no nutrients and may increase inflammation. The recommended diet to avoiding malnutrition includes vitamins A, K, D, E, unsaturated fats, antioxidants, and omega-3 fatty acids.

Sometimes the patients are also given high-quality supplements for deficiencies including probiotic and antioxidant supplements along with multivitamins. Pancreatic enzymes are also given for better digestion and absorption.

Other approaches to the treatment of cystic fibrosis are some natural ways to help manage the symptoms including physical therapy, cupping therapy, and breathing exercises.

Though there is still debate on whether these ways are useful or not, a study published in American Journal of Respiratory and Critical Care Medicine observed several similar treatments for cystic fibrosis including exercise, forced expiratory technique, autogenic drainage, and standard physical therapy.

The study showed that most of the techniques worked fairly well on the patients especially when they were in combination with easy, normal exercises and appropriate diet.

 

 

 

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As a graduate of Public Health and Policy, Andrea developed an interest in disease development, food and safety and the latest advancements in health. She is a Freelance writer who had affiliations with multiple blogs. Andrea is now pursuing her post-doctorate in Behavioral Sciences.

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