Cystic Fibrosis Definition

According to the cystic fibrosis definition, it is a genetic disorder that affects the exocrine glands, causing abnormal production of thick and sticky mucus, which blocks the pancreatic ducts, intestines, and bronchi leading to respiratory infection.

What is Cystic Fibrosis?

Cystic fibrosis is a serious genetic condition that affects the production of a protein that controls the flow of salt from in and out of the cells resulting in the thicker and stickier production of mucus. This thick mucus blocks the organs like – lungs, pancreas, liver and the intestines leading to damages to the respiratory and digestive systems and life-threatening conditions like; infections, respiratory failure, and malnutrition among others.

Types of Cystic Fibrosis

Cystic fibrosis is the result of CFTR gene mutations and more than 1,700 types of such mutations have been detected by researchers as the cystic fibrosis causes. The CFTR gene mutations have been grouped into various classes according to the problems they cause in the production of the CFTR protein. The various types of cystic fibrosis mutation classification are;

Protein production mutations (Class 1)

Protein processing mutations (Class 2)

Gating mutations (Class 3)

Conduction mutations (Class 4)

Insufficient protein mutations (Class 5)

Cystic Fibrosis Pathophysiology:

In cystic fibrosis genetics play a huge role and the signs and symptoms vary from person to person depending on the severity of the disease. The cystic fibrosis pathophysiology or damages caused by cystic fibrosis are classified in 6 classes which are as follows;

Absence of the CFTR protein synthesis

Defective protein maturation and rapid degradation

Disordered regulation

Defective chloride conductance or channel gating

Diminished transcription due to promoter or splicing abnormality

Accelerated channel turnover from the cell surface

 

Besides the above damages the damages resulting from cystic fibrosis are as follows –

Respiratory damages

A persistent cough with thick mucus

Wheezing

Breathlessness

Exercise intolerance

Repeated lung infections

Inflamed nasal passages or stuffy nose

Digestive damages

Foul-smelling, greasy stools

Poor weight gain and growth

Intestinal blockage

Severe constipation

Cystic fibrosis diagnosis :

Cystic fibrosis diagnosis is done with the help of various screening tests and examinations like Immunoreactive Trypsinogen Test (IRT),  Sweat Chloride Test, Sputum Test, Chest X – ray, CT scan and Pulmonary Function Tests (PFTs). People having cystic fibrosis genetic strain should get themselves screened at the time of pregnancy to prevent the child from being affected by cystic fibrosis.

Cystic Fibrosis Causes

Cystic fibrosis is the result of a defect in the CFTR gene. Mutation in the CFTR gene leads to the production of thicker and stickier mucus, which instead of lubricating various organs, causes blockages in them leading to various health conditions and infections. Several different types of defects can affect the CFTR gene and each type of defect is related to the severity of cystic fibrosis.

Cystic fibrosis treatment:

There is no cure for cystic fibrosis, but there are various Cystic fibrosis treatments available that help in relieving the symptoms and reducing any complications related to it. The varied Cystic fibrosis treatments made available to the patient of cystic fibrosis are;

Medications – Medication forms the first line of defence against the flaring up of the symptoms of cystic fibrosis. The varied medications prescribed for the control of cystic fibrosis include;

Antibiotics

Mucus-thinning medications.

Nonsteroidal anti-inflammatory drugs

Bronchodilators

Chest physiotherapy – Chest physiotherapy better known as cystic fibrosis physical therapy is integral to the management of the cystic fibrosis symptoms. Doctors often recommend cystic fibrosis physiotherapy in conjunction with medication to help in the effective control and better quality of life for the patients of cystic fibrosis. A physiotherapist assesses the cystic fibrosis symptoms and condition of the patients and designs a specific set of cystic fibrosis exercises to suit the needs of the particular patient.

The main aim of cystic fibrosis physiotherapy is help in loosening the thick mucus in the lungs, making it easier to eliminate it from the body by coughing it out. Cystic fibrosis physical therapy is usually performed one to four times per day for effective respiratory function.

Surgical Procedures – Surgery is usually seen as the last resort in the Cystic fibrosis treatment, when none of the other Cystic fibrosis treatment plans or cystic fibrosis remedies works in bringing relief in the cystic fibrosis symptoms. The various surgical procedures that can be administered to clear the organs of mucus blockages are;

Bowel surgery

Lung transplant

Pulmonary surgery

Thoracic surgery

Gastrointestinal surgery

Cystic Fibrosis Home Treatment:

Although there is no cure for cystic fibrosis we can undertake certain precautions and cystic fibrosis remedies to relief the symptoms. The various cystic fibrosis home treatments are as follows;

Drink lot of liquids to help in thinning the mucus in the lungs.

Undertake various types of cystic fibrosis exercises to help in loosening the mucus in the respiratory pathways like, aerobics, walking and biking.

Try and avoid going in to overly polluted areas and avoid smoke, pollen, and mold as they can lead to the worsening of the symptoms.

Get vaccinated for influenza and pneumonia regularly.

How can we help?

Patients affected with cystic fibrosis are at a lot of risk in a polluted environment, which is almost everywhere in India and as such patients need cystic fibrosis treatment at home. To cater to the needs of such patients who also need constant monitoring and care Portea offers quality at home healthcare Cystic fibrosis treatment plan in conjunction with highly experienced doctors, nurses and physiotherapists. Since the symptoms of cystic fibrosis vary from patient to patient, Portea’s team of efficient healthcare professionals’ device a specific cystic fibrosis treatment at home plan to provide the best care and medical assistance needed by the patient. Portea also offers post surgical rehabilitation care and services, which is very much needed in cases of lung transplant and other surgeries in cystic fibrosis.

 

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References:

https://en.wikipedia.org/wiki/Cystic_fibrosis

https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700

https://www.cysticfibrosis.org.au/

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