Patient Registry Report (Part 4)

Preventing Exacerbations and Prolonging Lung Health

Preventive maintenance is an important part of living well with cystic fibrosis. Over the last two decades, research has opened the doors to many new medications and therapies for the treatment of CF. As a result, the number of adults with severe lung disease is decreases. In fact, the number of patients with severe lung disease in 2005 is only half of what it was in 1985. Adherence to a preventive treatment regime enables more patients to reach adulthood with only moderate lung disease.

Frequently prescribed medications that are used to prevent exacerbations include:

-TOBI
-Pulmozyme
-Zithromax
-Ibuprofen

Each of these has been proven effective in promoting lung health and reducing the risk of potentially damaging infections.

TOBI is a formulation of the antibiotic, tobramycin. Nebulizing this medication instead of receiving it as an infusion helps direct more of the drug directly to the site where the infection is occurring. Patients who culture P. aeruginosa (PA) and have moderate to severe lung damage are those who are likely to benefit from TOBI inhalation solution. Nearly 58% of patients included in the 2005 CF Patient Registry are using TOBI.

Another preventive antibiotic used to fight P. aeruginosa is Zithromax. In the mainstream medical environment, this drug is prescribed as a powerful 3 day course of oral antibiotics. Patients with CF are prescribed this antibiotic on an ongoing basis.

Pulmozyme is a mucolytic (i.e. mucus cutting) medication. Although some patients have difficulty tolerating it, approximately 70% of patients with moderate to severe lung disease use it.

Ibuprofen is an over-the-counter medication called NSAID (non-steroidal anti-inflammatory drug.) CF researchers have studied the benefits of prescribing high doses of ibuprofen for pediatric patients aged 6-12 with CF. The goal is to prevent infection that often follows lung inflammation. A mere 5% of patients use ibuprofen as part of their treatment.

The goal of these therapies is to reduce the number of exacerbations patients experience each year. Each exacerbation has the potential to cause irreversible lung damage. The longer a patient can go without an infection, the better the chances of reaching adulthood with only mild to moderate disease severity. As treatment options and preventive care progresses, more and more patients are able to stay infection free. In 2005 nearly 65% of CF patients reported not succumbing to a lung infection. This is a growing trend, and one that the CFF hopes will continue.

The following is the list of recommendations by the CF Foundation for avoiding infection:

1) Getting a flu shot every fall for you/your child and everyone living in the house;
2) Regular exercise to strengthen your muscles;
3) Doing airway clearance to keep the lungs as clear as possible of mucus;
4) Taking the medicines as prescribed by your CF care center;
5) Telling your CF care center early when you or your child do not feel well; and
6) Avoiding germs by using good hand hygiene and properly cleaning and disinfecting equipment



Related Links:

Use of TOBI (Tobramycin) inhalation solution
TOBI Reduces Occurrence of Hospitalizations for CF Patients
Azithromycin (Zithromax) in CF Patients
Ibuprofen instead of Corticosteroids for CF
Effectiveness of Pulmozyme in Infants Being Studied
The Importance of Being Compliant (blog entry)