According to the Cystic Fibrosis Foundation (CFF), more than 30,000 children and adults in the United States and about 70,000 children and adults worldwide have cystic fibrosis (CF). It is estimated that around 1,000 new cases are diagnosed each year in the US. Of these new diagnoses, about 75 percent occur in children under the age of 2 years. CF is a chronic, life-threatening disease that primarily affects the lungs, digestive system and other organs of the body. Every year, the month of May is observed as “National Cystic Fibrosis Awareness Month” in the United States to educate people about the symptoms of CF and encourage people to take the right steps to prevent the risks associated with the condition. During the CF awareness campaign, the community comes together to help others learn about the disease, share personal stories, and unite behind the mission of finding a cure for all people with CF. Cystic fibrosis (CF) is a chronic, life-threatening disease that primarily affects the lungs, digestive system and other organs. The cells that produce mucus are affected. Sweat and other secreted fluids normally appear thin and slippery. However, in people with CF, a defective gene causes the secretions to become sticky and thick. The secretions cause blocks in tubes, ducts and passageways, particularly in the pancreas and lungs. This can lead to life-threatening problems such as severe infections, respiratory failure and malnutrition. Physicians offering diagnosis and treatment for cystic fibrosis need to have correct knowledge about the related medical codes and payer regulations to submit claims and get paid. Partnering with a reputable medical billing company is important to report CF diagnosis and screening accurately.

Cystic fibrosis is a hereditary condition that occurs in a child when both parents have the defective gene. Many different defects can occur in the gene. In CF, a defect (mutation) in a gene – the cystic fibrosis transmembrane conductance regulator (CFTR) gene – changes a protein that regulates the movement of salt in and out of cells. This increases the salt content in sweat and creates thick, sticky mucus in the respiratory, digestive and reproductive systems.

Symptoms and Risk Factors

One of the initial symptoms of cystic fibrosis is a strong salty taste to the skin. The symptoms of cystic fibrosis can vary depending on the severity of the condition and age at which these symptoms occur. Symptoms may normally appear at infancy, but for some children, symptoms may not begin until after puberty or even later in life. As time passes, the symptoms of the disease may get better or worse. Common symptoms include –

  • Persistent coughing
  • Shortness of breath
  • Wheezing
  • Repeated lung infections
  • Recurrent sinusitis
  • Poor weight gain in spite of excessive appetite
  • Nasal polyps, or small, fleshy growths found in the nose
  • Intestinal blockage, particularly in newborns (meconium ileus)
  • Inflamed nasal passages or a stuffy nose
  • Exercise intolerance
  • Chronic or severe constipation

As cystic fibrosis is an inherited disorder, it runs in families, so family history is a common risk factor. Complications of this condition can affect the respiratory, digestive and reproductive systems, as well as other organs. These include – nasal polyps, damaged airways (bronchiectasis), coughing up blood (hemoptysis), nutritional deficiencies, intestinal obstruction, diabetes, and liver disease.

Diagnosis and Treatment Modalities for CF

To diagnose cystic fibrosis, physicians normally perform a physical exam, symptoms review and conduct various tests. Early diagnosis means treatment can be initiated immediately. Blood sample tests may be performed to check for higher than normal levels of a chemical called immunoreactive trypsinogen (IRT), which is released by the pancreas. For an infant, physicians may conduct a sweat test (when the infant is 2 weeks old). A sweat-producing chemical is applied to a small area of skin. Then the sweat is collected to test it, and see if it is saltier than normal. In addition, genetic tests may also be performed to check for specific defects of the gene responsible for cystic fibrosis.

There is no specific cure for cystic fibrosis, but correct treatment interventions can ease symptoms, reduce complications and improve the patient’s quality of life. Treatment options include – medications (medications that target gene mutations, anti-inflammatory medications, mucus-thinning drugs, inhaled medications, oral pancreatic enzymes, acid-reducing medications and other drugs for diabetes or liver disease), airway clearance techniques (called chest physical therapy (CPT), and pulmonary rehabilitation. If any of these treatment options does not give the desired results, surgical options like nasal and sinus surgery, oxygen therapy, bowel surgery, noninvasive ventilation and lung or liver transplantation may be recommended.

The ICD-10 codes for Cystic Fibrosis come under the E84 category. Physicians must ensure highly specific clinical documentation so that medical coding companies can choose the right codes. ICD-10 codes for Cystic fibrosis (CF) include –

  • E84 – Cystic fibrosis
  • E84.0 – Cystic fibrosis with pulmonary manifestations
  • E84.1 – Cystic fibrosis with intestinal manifestations
  • E84.11 – Meconium ileus in cystic fibrosis
  • E84.19 – Cystic fibrosis with other intestinal manifestations
  • E84.8 – Cystic fibrosis with other manifestations
  • E84.9 – Cystic fibrosis, unspecified

Cystic fibrosis cannot be prevented. However, people can reduce the risk of complications by following several home remedies like – drinking plenty of fluids (as it can help thin the mucus in the lungs), doing regular exercise, avoiding smoke, pollen, and mold whenever possible, and getting influenza and pneumonia vaccinations regularly. In addition, genetic testing also needs to be performed for couples who have cystic fibrosis or who have relatives with the disease. These tests can determine a child’s risk for cystic fibrosis by testing samples of blood or saliva from each parent.

Billing and coding for cystic fibrosis can be challenging. Partnering with a reliable and experienced medical coding company can help healthcare providers ensure complete and accurate documentation and code assignment.