Cystic Fibrosis Center

Harris County's Only Accredited CF Center

The Maconda Brown O'Connor, Ph.D., Adult Cystic Fibrosis Center at Baylor Medicine in Houston, Texas, provides the highest quality care in a compassionate, patient-centered setting. Our center, which is accredited by the Cystic Fibrosis Foundation, is one of the largest in the United States and is the only accredited CF center in Harris and the surrounding counties.

Offering comprehensive, multidisciplinary care to adult CF patients and their families, we pride ourselves on the evidence-based, state-of-the-art care we provide. Our center follows strict CF Foundation infection control guidelines for your protection.

Additionally, our center has been designated a Therapeutic Development Network site by the CF Foundation, promoting the conduct of cutting-edge clinical research and providing a forum for collaboration with other organizations to advance the care of cystic fibrosis. For more information or to make an appointment call (713) 798-2400.

Cystic fibrosis is caused by mutations of the CFTR gene – cystic fibrosis transmembrance conductance regulator – which was discovered by Francis Collins in 1989. Since that time, over 1900 defects in this gene have been identified.

The normal CFTR protein functions as a channel allowing the passage of salt and water into and out of the cell appropriately. This channel is defective in CF patients preventing the normal passage of salt and water and leading to thick, sticky mucus throughout the body. This mucus blocks airways, intestines, gallbladder and many other tubes in the body. This eventually results in difficulty breathing and absorbing nutrients.

Cystic Fibrosis is a recessive disease, meaning that both copies of the gene (one from the mother and one from the father) must be defective in order to be diagnosed with CF. Defects in the CFTR is the most common genetic mutation found in the Caucasian population, about 1 in 25 people have one defective gene. Having one defective gene does not give you CF. Each time two carriers of the defective gene conceive, there is a:

• 1 in 4 chance that their child will not have the gene at all
• 1 in 2 chance that their child will inherit one defective gene and be a carrier but not have the disease
• 1 in 4 chance that their child will have cystic fibrosis

The most prevalent indicator of cystic fibrosis is the overabundance of thick, sticky mucus in the airways. Normally, in the lungs, mucus performs several functions including being a protective barrier helping to prevent infection and transporting dust and other particles out of the bronchial tubes. Symptoms include:

• Persistent cough
• Frequent lung infections
• Wheezing
• Thick, sticky phlegm
• Shortness of breath

Gastrointestinal Symptoms

• Pancreatic insufficiency - abdominal pain, diarrhea, bloating
• Pancreatitis
• Acid reflux
• Malabsorption - weight loss, diarrhea
• Bowel obstruction
• Meconium ileus - present at birth

Other Symptoms

• Salty sweat or skin
• Clubbing of the fingers
• Fatigue
• Nasal polyps
• Sinusitis
• Poor growth, weight loss
• Male infertility

A person who has a family history of cystic fibrosis or exhibits symptoms of the disease may undergo a sweat test, which measures the concentration of salt in a person’s sweat. Simple and painless, the test should be administered by a Cystic Fibrosis Foundation-accredited care center where strict guidelines help ensure the accuracy of the results.

All 50 states have protocols in place to test every newborn for certain diseases in which early intervention is beneficial. This is accomplished by a simple blood sample obtained from a heel stick prior to discharge from the hospital. Cystic fibrosis is part of this protocol in every state as of 2009.

The Maconda Brown O'Connor, Ph.D., Adult Cystic Fibrosis Center at Baylor College of Medicine, a Cystic Fibrosis Foundation-accredited center, offers a full range of services to CF patients and their families.

Our specialty services include:

• Pulmonary care
• Allergy and sinus disease management
• Respiratory therapy
• Nutrition
• CF-related GI disorders
• CF-related diabetes clinic
• Pregnancy and family planning
• Medical genetics consultation and counseling
• Psychosocial and adjustment counseling referrals

The Therapeutic Development Network is a designation given to clinical centers by the Cystic Fibrosis Foundation for expertise in clinical trial research. We are one of the 77 in the United States which has received this designation.

Center patients benefit from centralized training, standardized procedures and access to the newest clinical trials because of the TDN designation. Our center, along with other TDCs, uses quality improvement tools and strategies on an ongoing basis, and our performance in clinical studies is monitored to encourage continued quality and efficiency without compromising safety.

The Adult CF Center offers a rich environment for trainees. The Internal Medicine and Medicine-Pediatrics residents rotate through our clinic monthly, as well as the Pulmonary Critical Care residents and fellows.