New ICD-9 Codes for Cystic Fibrosis

Vol. 12 •Issue 25 • Page 19
Coding Outlook

New ICD-9 Codes for Cystic Fibrosis

Have you ever wondered where the phrase 65 roses came from? A little boy with cystic fibrosis was listening to his mother making phone calls seeking support for cystic fibrosis research. When he asked her what she was doing she told him that she was working. The little boy then told his mother that she was working for 65 roses. It seems that this phrase is much easier for children with the disease to say and it sounds like cystic fibrosis, which is a genetic disease that affects nearly 30,000 children and adults in the United States. In this article we will discuss cystic fibrosis and the new diagnostic codes, which went into effect on Oct. 1, 2002.

What is Cystic Fibrosis?

Cystic fibrosis is a genetic disease that causes the body to produce abnormally thick, sticky mucus because of faulty transport of sodium chloride within cells lining the organs. The disease was first known as cystic fibrosis of the pancreas. However, due to the affects on other body organs the disease is simply called cystic fibrosis. The disease affects the patient’s pancreas, pulmonary system, cardiac system, biliary system and reproductive system. One common complication of the disease is meconium ileus, which often occurs in the first few days of life. The patient’s pancreas does not produce the appropriate amount of secretions needed, which causes the stool to become thick and sticky and lodged in the intestines.

The respiratory system is also affected due to an increase in buildup of thick secretions. This can cause breathing difficulties, chronic lung disease, pulmonary hypertension and cor pulmonale. Biliary tract obstruction may also occur in patients with cystic fibrosis causing biliary cirrhosis. Women with cystic fibrosis may be fertile if growth and development have been normal, whereas men are usually infertile because of obstructive azoospermia.

Diagnosis and Treatment

The most common test for cystic fibrosis is the sweat test. The sweat test measures the amount of sodium chloride in the sweat. Sweat is collected and analyzed, and patients with higher than normal amounts of sodium chloride are determined to have cystic fibrosis. The sweat test will not always work in newborns and another test, the IRT test, may be utilized. Blood is drawn several days after birth and analyzed for the protein trypsinogen. A positive IRT test is then confirmed by a sweat test and other tests. Some patients may have a normal sweat test and can only be diagnosed by a chemical test that will note the presence of the mutated gene. Some other tests include chest X-rays, pulmonary function tests and sputum cultures.

The stage of the disease determines the treatment plan for a patient with cystic fibrosis. A patient with a meconium ileus may be given gastrografin enemas to avoid surgery. However, if this treatment fails, surgery is required. A patient with pulmonary complications may receive chest therapy to break up the secretions. Antibiotics are used to treat lung infections. To help improve breathing, a patient may use bronchodilators, mucolytics and decongestants.

Coding Cystic Fibrosis

There have been three new diagnostic codes added for cystic fibrosis, effective Oct. 1, 2002. The new codes are as follows:

277.02 Cystic Fibrosis with pulmonary manifestations

277.03 Cystic Fibrosis with gastrointestinal manifestations

277.09 Cystic Fibrosis with other manifestations

The existing ICD-9 codes are as follows:

277.00 Cystic Fibrosis without mention of meconium ileus

277.01 Cystic Fibrosis with meconium ileus

The DRGs for the newly created cystic fibrosis codes are:

277.02: DRG 79, 80 or 81 Respiratory Infection and Inflammation

277.03: DRG 188, 189 or 190 Other Digestive System Diagnoses

277.09: DRG 296, 297 or 298 Nutritional and Metabolic Disease

Appropriate documentation and diagnostic coding is not only essential in assisting with ongoing research for cystic fibrosis, but also in receiving appropriate reimbursement for your facility. The new ICD-9 codes allow us to show, with greater specificity, the complications that the patients are presenting with and the resources required to treat these patients.

Tammy Cook is a coding consultant with HP3’s Just Coding Group.