Cystic fibrosis is one of the many genetic disorder diseases and the most common fatal genetic disease affecting children and young adults. 

It is a genetic disease that mostly cause effect on the body.  It affects the internal organs such as the pancreas, liver, lungs,  intestine and sex organ. 

 
Cystic fibrosis (CF) is basically an inherited disease of the secretory gland, which means the disease is passed from parents to children through genes. Secretory glands include glands that make mucus and sweat. The name cystic fibrosis is derived from the formation of scarring (fibrosis) and cyst within pancreas. 

According to MedicalNewsToday, there is currently no cure for CF. It affects some 30,000 people in the United States (U.S.) with around 1,000 new cases diagnosed each year.

Seventy-five percent of these new diagnoses are made in children under the age of 2 years.

Don’t forget to read: Four things parents can do to keep their kids kidney healthy

 

What causes cystic fibrosis? 

  The cause of this disease is as a result of mutation in the gene for protein named cystic fibrosis transmembrane conductance regulator (CFTR).


 This gene makes a protein that controls the movement of salt and water in and out of your body’s cells. 


Every person inherits two CFTR genes one from each parent. If each parent has a normal CFTR gene and a faulty CFTR gene, each child has a 25 percent chance of inheriting two normal genes, a 50 percent chance of inheriting one normal gene and one faulty gene, and a 25 percent chance of inheriting two faulty genes.
However, children who inherit a faulty gene from each parent will have cystic fibrosis.


  A defect in the CFTR gene causes cystic fibrosis (CF).
In people who have CF, the gene makes a protein that doesn’t work well. This causes thick, sticky mucus and very salty sweat.

Mucus is a substance made by tissues that line some organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected.

Cystic fibrosis (CF) is a life-threatening genetic disease. A child with CF has a faulty gene that affects the movement of sodium chloride (salt) in and out of certain cells. Research suggests that the CFTR protein also affects the body in other ways. 
What happens to your body when you have this condition 
If you have CF, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways. (Airways are tubes that carry air in and out of your lungs), which leads to persistent cough with productive thick mucous, Wheezing and shortness of breath
 
The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections such as pneumonia,  these infections can severely damage your lungs over time, .

The thick, sticky mucus also can block tubes, or ducts, in your pancreas (an organ in your abdomen). As a result, the digestive enzymes that your pancreas makes can’t reach your small intestine.

 
These enzymes help break down food. Without them, your intestines can’t fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients pass through your body without being used. You also may have bowel disturbance such as frequent oily stools, a swollen belly from severe constipation and discomfort.

CF can as well make your sweat to become very salty. Thus, when you sweat, you lose large amounts of salt. This can upset the balance of minerals in your blood and cause many health problems. Examples of these problems include dehydration dehydration (a lack of fluid in your body), increase heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke (excessive sweating which can lead to fainting), and, rarely, death.
Other symptoms includes 
Weight loss or failure to gain weight despite increased appetite

Infertility (men) and decreased fertility (women)

Inflamed nasal passages or a stuffy nose


Symptoms like bowel obstruction due to meconium ileus mostly appears in a new born baby or in early childhood.

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Complication of cystic fibrosis 

There are typical complication caused by cystic fibrosis which includes the following:

  • Progressive lung damage from chronic infection.
  • Difficulty in digesting fats and protein
  • Inability of the body to absorb nutrients thus leading to malnutrition and vitamin deficiency. 

How can cystic fibrosis be treated? 


Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years.  Depending on the severity of CF, you or your child may be treated in a hospital.


Antibiotics and inhalation medications are the most necessary medicines for the patients of Cystic Fibrosis. Many Cystic Fibrosis Patients mostly remains on one or more antibiotics. 

If you or your child has symptoms of cystic fibrosis or if someone in your family has cystic fibrosis talk with your doctor about testing for the disease.


People with CF need to work closely with their medical professionals and families to create individualized treatment plans. 

What you can do to reduce risk of lung infection 

People with CF can also help reduce their risk of lung by following these few steps:

  • Practice frequent washing the hands frequently
  • Ensure you get a flu shot every year
  • Do not smoke 
  • Try avoiding second-hand smoke.
  • Avoid unnecessary contact with people who have colds or other contagious illnesses
 
NB: Seek immediate medical care if you or your child has difficulty breathing.

 

 

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