Cystic Fibrosis Information for Parents

What is Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited disease that causes glands throughout the body to make abnormally thick, sticky mucus. The most frequently affected organs include the lungs and pancreas. The intestines, liver, sweat glands and reproductive organs also may be affected.

About 30,000 people in the United States are affected with the disease, and about 1,000 babies are born with it each year. Cystic fibrosis is a complicated illness that requires clinical care by a physician or other health care professional. More information about cystic fibrosis can be found on the following sites.

Children’s Hospital Child Health Library

UPMC Health A to Z

Infection Control

UPMC Children's Hospital of Pittsburgh’s Cystic Fibrosis (CF) Center has established guidelines for controlling the spread of infection in clinic and in the hospital for the protection of our cystic fibrosis patients. Please review these guidelines at the link below:

Infection Control Guidelines

Additional information about infection control is available through the Cystic Fibrosis Foundation.

For questions or more information, please contact Children’s CF Center at 412-692-5630.

Quality Improvement Initiative

The Cystic Fibrosis Foundation (CFF) is committed to providing every Cystic Fibrosis Foundation–accredited care center throughout the United States with the opportunity to improve the quality of care delivered to patients with Cystic Fibrosis (CF) and their families. A standardized training program is being offered to assist centers in looking at how their center can accelerate the rate of improvement in CF care. The Cystic Fibrosis Center at UPMC Children’s Hospital applied to and was accepted to be part of the 5th CFF Learning and Leadership Collaborative, and spent two days in 2007 learning with experts in quality improvement in Baltimore, MD.

Children’s CF Center has developed a Quality Improvement (QI) Lead Team for our pediatric and adult programs. The team consists of representatives from each of the disciplines including administration, physicians, nurses, dietitians, respiratory therapists and social workers as well as three parents from the pediatric clinic and two patients from the adult clinic. The pediatric and adult teams meet every two weeks to discuss quality improvement initiatives.

The mission of the Center’s QI Lead Team is to improve outcomes by assessing operations and developing goals and projects. We have chosen to focus our efforts on improving pulmonary function in our patients. Several specific projects are under way, including efforts to improve knowledge of airway clearance techniques, to improve adherence to regularly scheduled follow-up appointments, increased efforts to avoid exposure to secondhand tobacco smoke, and efforts to optimize nutritional status.

Pharmacy News

Insurance coverage may dictate pharmacy providers. Falk Pharmacy in Oakland, will mail prescriptions to patients. If you choose to use Falk Pharmacy, hours and location are as follows:

7:30 a.m.–5:30 p.m., Monday through Friday 

Falk Pharmacy, Falk Medical Building

3601 Fifth Avenue, Room 211

Pittsburgh, PA 15213 

Ph: 412-473-7427 (PHAR)

Fax: 412-648-1838

A Note Regarding Refills

When calling to request refills, remember to give yourself ample time so that you do not run out of medication. We recommend a two-week turnaround, as many insurance companies now require prior authorization for many medications.

Learn more about Falk Pharmacy.

Tips from Our Parents

From M. Wiggins:

When my daughter was diagnosed with cystic fibrosis, my husband and I took a look at our home to figure out what we could do to help prevent the spread of germs and keep her healthy. Some things that we implemented are listed below:

  • We placed antibacterial gel by the front door so that visitors could clean their hands before entering.
  • Every day, we wipe down surfaces (for example, doorknobs, light switches, phones and other “frequently touched” areas) with antibacterial wipes. It only takes about five to 10 minutes to do the whole house. We do this more frequently if someone in our house is sick.
  • We put antibacterial gel in our cars and always clean our hands with it when we leave a store and before coming home.
  • We stopped using sponges, dish cloths and towels in the kitchen and switched to paper towels to dry hands and antibacterial wipes to clean counters, the high chair, etc.

When we were told our daughter had pseudomonas at the age of 7 months it made us take a look once again at what we could do differently around the house to protect her from this pesky bacterium. We realized we had bath toys that retained water even after we drained the tub (for example, squeeze toys). Anywhere water sits is a possible breeding ground for bacteria. We made sure to buy toys that can completely dry out between baths. We stopped using the “wipes warmer” we had, since that always stays wet. Obviously we cannot protect completely against these bacteria, but we feel better knowing we are taking precautions where we can.

These are just some of the things we do to help keep the germs and bacteria at bay. What are some of the things that you do?

Additional Resources

Cystic Fibrosis Foundation

The mission of the Cystic Fibrosis Foundation, a nonprofit donor-supported organization, is to assure the development of the means to cure and control cystic fibrosis and to improve the quality of life for those with the disease.

CF Voice

CF Voice is an online community for people of all ages living with cystic fibrosis (CF). It is a place for motivation, inspiration and connection to the CF community.

Muriel's Breath of Life

A nonprofit, family-led initiative administered in partnership with Children's Hospital of Pittsburgh Foundation, Muriel’s Breath of Life raises funds that directly benefit children and adults with cystic fibrosis who are under the care of the Antonio J. and Janet Palumbo Cystic Fibrosis Center at UPMC Children's Hospital of Pittsburgh and UPMC's Comprehensive Lung Center.


Schedule Your SuperVisit Today!

The “SuperVisit” is a clinic visit that allows patients with CF to get all of their yearly tests done in one day. Monitoring for certain health issues is very important in CF. You may also know that there can be a lot of different tests that are used to monitor health.

The “SuperVisit” includes:

  • annual laboratory studies
  • Oral Glucose Tolerance Test
  • chest x-ray
  • complete pulmonary function tests
  • cardiopulmonary exercise testing

SuperVisits are for patients age 10 and older and occur primarily in Lawrenceville in the morning (lab work is done after fasting overnight). It is possible to combine all this testing (approximately 4 hours) with your clinic visit, or you may prefer to do this on a separate day. It is possible to schedule your clinic visit in your usual clinic location after this testing.

To schedule a SuperVisit, please contact our Super Schedulers at 412-692-5630. For any questions about the testing, please speak to our Super Staff in clinic.

Flu Shots

Ask your primary care doctor or cystic fibrosis team about getting immunized against influenza. Vaccines usually are available in September.