Cystic Fibrosis in Children: Causes, Symptoms, Diagnosis, and Treatment

Cystic Fibrosis

What Is Cystic Fibrosis?

Cystic Fibrosis harms the cells producing sweat, digestive fluids, and mucus and it is a genetic disorder. Nearly 30,000 young adults and children deal with Cystic Fibrosis in the United States.

Currently there is no cure for Cystic Fibrosis; however, children who are diagnosed early and receive early treatment can have a longer lifespan. Unfortunately if the disorder isn’t treated in time, it can lead to complications like infertility, nutritional deficiencies, and chronic lung infections.

What Causes Cystic Fibrosis (CF)?

A mutation in the gene Cystic Fibrosis Transmembrane Connductance Regulator or CFTR causes this disorder. Medical clinics in Nashville, TN, and elsewhere have found more than 1000 mutations are responsible for CF.

In order for a child to have CF, they must inherit a mutated non-function CF gene from both parents.  At which point there is a 1 in 4 chance they will have CF.

What are the Common Symptoms?

Generally children with CF starts to show symptoms at a very early age. Depending upon the severity and type the symptoms can vary. In some cases CF patients may not show symptoms until they are an adult.

Some common symptoms:

  • Excessive sweating
  • Salty-tasty skin
  • Asthma-like symptoms with continuous sneezing and coughing
  • Sinus or lung infections happening recurrently
  • Coughing up thick mucus or blood
  • Shortness of breath
  • Delayed growth
  • Problem in certain nutrient absorption
  • Pain in abdomen
  • Heart enlargement
  • Greasy or smelly stools
  • Enlarged and round toes and fingers
  • Nasal polyps
  • Diabetes
  • Problem in liver
  • Rectal prolapsed

CF primarily affects the respiratory and digestive systems.

Getting tested

The general standard test for Cystic Fibrosis (CF) is a sweat test for high sweat chloride to see if you have high levels of chloride in your sweat.

Infants with a positive prenatal testing for cystic fibrosis will be further tested after birth to confirm the diagnosis of cystic fibrosis. A basic blood test is all that is needed to determine CF.

In addition, to find out the extent of your Cystic Fibrosis some additional tests may be performed, such as:

  • Chest x-rays
  • Pulmonary function test
  • Various blood tests to determine your nutrient levels
  • Tests to check the presence of bacteria in your lungs
  • Tests to check pancreas activity

What Treatment Options are Available?

Unfortunately, at this time, the main objective of CF treatment is to manage the symptoms, prevent the occurrence of complications, and to extend your lifespan; there is no known cure at the moment.

In addition to medical treatment, a balanced diet containing digestive enzymes, minerals and vitamins are necessary to allow for proper development and growth of a child and to continue to lead as normal a life as possible.

Because of this disease, the following is also recommended:

  • Medications: Sometimes medications are needed to improve lung function. These medicines can also help alleviate inflammation and reduce or remove infection. Medications like:
  • Medicine to thin mucus
  • Insulin shots to improve the absorption of nutrients
  • Bronchodilators to open the airways
  • Anti-inflammatory medicines to reduce airway inflammation
  • Antibiotics to treat the infection of lungs
  • Immunizations: CF patients are more likely to develop infections; therefore parents should get their children vaccinated on time, especially vaccinations for pneumococcal and influenza. Clinica Hispana La Paz can help you with vaccination services in Nashville.
  • Chest Physical Therapy: Physical therapy can be helpful in removing mucus from the lungs. By thinning out the mucus it is easier to cough it up and clear the airway.
  • Surgery: As a last resort, surgeries could be performed, if necessary, to alleviate the CF symptoms. Surgery can be used to:
  • Remove nasal polyps that were obstructing breathing
  • Treat problems related to bowel movements
  • Lung transplant surgery
  • Liver transplant surgery

Getting help

While there is no current cure for Cystic Fibrosis (CF), there are steps you can take to lead a reasonably normal life. Stay strong, eat right, exercise and one day we will find a way to help you even more.