Cystic Fibrosis – Symptoms, Diagnosis, Treatment Options and ICD-10 Codes

0:01 – Hello Everyone and Welcome to our podcast series.  My name is Natalie Tornese and I am a Senior Group Manager for Outsource Strategies International.  I wanted to discuss a little bit about cystic fibrosis.

0:13 – Cystic Fibrosis – Key Statistics

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 cases, about 75 percent occur in children under the age of 2.

0:35 – What is CF?

Cystic fibrosis (CF) is a chronic, life-threatening disease that primarily affects the lungs, digestive system and other organs of the body. The cells that produce mucus are affected. Sweat and other secreted fluids normally appear thin and slippery. However, in people with cystic fibrosis, a defective gene causes the secretions to become sticky and thick. These secretions cause blocks in tubes, ducts and passageways, particularly in the pancreas and the lungs. This can lead to life-threatening problems such as severe infections, respiratory failure and malnutrition.

1:12 – What Causes Cystic Fibrosis?

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 cystic fibrosis, a defect (mutation) in 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.

1:40 – 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 and sometimes even later in life. As time passes, the symptoms of the disease may get better or worse. Common symptoms are 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, inflamed nasal passages or a stuffy nose, exercise intolerance and chronic or severe constipation.

As this is an inherited condition, it can run 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.

2:58 – Diagnosing 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.

3:43 – Treatment Options for CF

There is no 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 (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, non-invasive ventilation and lung or liver transplantation may be recommended.

I will include a transcript along with this podcast outlining all associated ICD-10 codes related to this condition

4:37 – How to Prevent CF?

Although cystic fibrosis cannot be prevented, 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 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.

I hope this helps, but always remember that documentation and a thorough knowledge of payer regulations and guidelines is critical to ensure accurate reimbursement for the procedures performed.