Online Health Chat with Elliot Dasenbrook, MD, and Silvia Cardenas Zegarra, MD
May 12, 2016
Cystic fibrosis (CF) is a genetic disease that causes sticky, thick mucus to build up in organs including the lungs and the pancreas. In a healthy person, mucus that lines organs and body cavities, such as the lungs and the nose, is slippery and watery. In people with CF, thick mucus clogs the airways, making breathing troublesome. Its blockage of the ducts in the pancreas causes problems with digesting food. Babies and children with CF may not be able to absorb enough nutrients from food.
About the Speakers
Elliott Dasenbrook, MD, is a staff member in the Respiratory Institute at Cleveland Clinic's main campus. Prior to that appointment, Dr. Dasenbrook completed his fellowship at Johns Hopkins University School of Medicine and attended medical school at Loyola University Stritch School of Medicine.
Dr. Dasenbrook’s clinical interests include adult cystic fibrosis, bronchiectasis, nontuberculous mycobacterial pulmonary disease, primary ciliary dysfunction and allergic bronchopulmonary aspergillosis.
Silvia Cardenas Zegarra, MD, is a staff member of Cleveland Clinic Children’s Center for Pediatric Pulmonary Medicine. Prior to that appointment, Dr. Cardenas Zegarra completed her fellowship at Jackson Memorial Hospital and attended medical school at Cayetano Heredia University School of Medicine.
Dr. Cardenas Zegarra’s clinical interests include cystic fibrosis, asthma, chronic lung disease and BPD.
Let’s Chat About Cystic Fibrosis
CFheart: How will I know if I am a carrier of the gene that causes cystic fibrosis?
Elliott_Dasenbrook,_MD: Approximately one out of 25 Caucasians in the United States are carriers of genes that can cause cystic fibrosis (CF). Most CF carriers do not have any symptoms; however, some carriers may have issues with their sinuses, pancreas or infertility (males).
desrtl: Can adults be diagnosed with cystic fibrosis or is it only diagnosed in children?
Silvia_Cardenas_Zegarra,_MD: Yes. Cystic fibrosis can be diagnosed in adult patients. Some patients may not present with typical symptoms early in life, and, therefore, are diagnosed later. Typically, these patients have milder forms of CF (milder mutations). Currently, thanks to the use of newborn screening, we are identifying CF earlier and treating patients early, which we know has impacted the outcome of the disease in a positive way.
Elliott_Dasenbrook,_MD: Absolutely. Approximately five percent of CF patients are actually diagnosed as adults.
jsmith: How are people diagnosed with cystic fibrosis?
Silvia_Cardenas_Zegarra,_MD: The most important test for diagnosing cystic fibrosis is the sweat chloride test. This test is performed in patients who are presenting with symptoms that are suspicious for the disease, such as poor weight gain, recurrent pneumonia and recurrent sinus infections. The test is very simple; it does not involve needles. It consists of collecting sweat from the arm of your child (or adult patient), which is sent to the lab to measure electrolytes, specifically chloride. If this is elevated, we can make a diagnosis of CF.
frankling8: Are babies screened for cystic fibrosis at the time of birth? Or, does it happen while they are in the womb?
Silvia_Cardenas_Zegarra,_MD: Babies are screened for cystic fibrosis at birth. Actually, the screening for cystic fibrosis is included in the newborn screening test that all babies are given before they leave the nursery. Typically, babies are not screened in the womb, but certainly, parents can be screened and counseled if they are carriers of cystic fibrosis. Then they can learn the risks and probabilities of having a child with cystic fibrosis.
Life with Cystic Fibrosis
gretayo: Can my infant with CF go to daycare? Is that advisable?
Silvia_Cardenas_Zegarra,_MD: We want and strive for children with CF to have healthy and normal lives. Patients with CF do not have an increased risk of getting infections. However, when they do get common viral infections, they could get sicker than children who do not have any underlying disease. With close follow up, we can help these young patients deal better with viral infections.
JoCar: Does cystic fibrosis affect fertility?
Elliott_Dasenbrook,_MD: Ninety-nine percent of men with CF have issues with fertility. The sperm is normal; however, the CF results in some of the tubes (the vas deferens) being blocked. Therefore, working with fertility specialists, men with CF are able to have children. Women with CF are able to have children at a similar rate as women without CF.
GrannyG: Our doctor said that diabetes is a common complication with CF. Is there a way to prevent it (diet, etc.)?
Elliott_Dasenbrook,_MD: Yes, the prevalence of CF-related diabetes increases as patients get older. Approximately 1/3 of adults with cystic fibrosis will develop CF-related diabetes. Unfortunately, at this time we do not know how to prevent it. The treatment of CF diabetes is very different from Type 2 diabetes. For example, given the importance of nutrition in CF, there are no dietary restrictions with CF-related diabetes. In addition, if CF diabetes requires treatment, we use insulin and not pills.
jfsiok: How long can you live with cystic fibrosis? What is the usual life expectancy?
Elliott_Dasenbrook,_MD: In the United States, the median age of survival is approximately 40 years, but every person is different. I work with adults living with CF, and some of my patients are in their 70s! There are many new therapies available, and many more are being developed. Therefore, we expect the life expectancy to continue to substantially increase.
Care and Treatments
uplift: What surgery options do I have for CF? Would a lung transplant cure it?
Elliott_Dasenbrook,_MD: Since cystic fibrosis involves the entire body (the lungs, the pancreas, the skin, the gut, etc.), a lung transplant will not cure it. For CF patients who have advanced-stage lung disease (for example, requiring oxygen), a lung transplant is an option to improve how you feel and your activity level.
valeriegof: How do you continue treatment into adulthood? Are there specialists who will take over care after pediatric treatment?
Elliott_Dasenbrook,_MD: In the United States, there are now more adults with cystic fibrosis than there are children with cystic fibrosis. Fortunately, there are many adult-trained pulmonologists who have expertise in caring for adults living with cystic fibrosis (I am one). The adult CF team works closely with the pediatric CF team to ensure a smooth transition between the centers.
GrannyG: Does the climate you live in make a difference for CF outcomes? I have heard some people suggest living in a dry climate (like Arizona) rather than somewhere cold or humid.
Elliott_Dasenbrook,_MD: In general, there has not been an association between where a patient lives and outcomes related to CF. However, for individual patients, I have had CF patients who developed asthma from the cold weather. The asthma gets better during the summer. Alternatively, I have had CF patients who moved to the Southwestern US and developed allergies. The allergies improved when they moved back to Ohio.
GrannyG: Is antibiotic resistance a concern for people with CF lung infections?
Elliott_Dasenbrook,_MD: Antibiotic resistance is a minor concern for people with CF lung infections. We are fortunate to have many antibiotics that can be used to treat different infections.
GrannyG: How do you choose which airway clearance technique is best for a CF child? Is it trial and error?
Silvia_Cardenas_Zegarra,_MD: There is no data showing that certain airway clearance techniques are better than others. Since the lungs are one of the most important organs affected by cystic fibrosis, we definitely recommend airway clearance in all patients. My recommendation is that patients use the technique that they feel fits them better or the one they are more motivated to do so they are able to do it consistently.
Cher02ry: What types of things should cystic fibrosis patients do to keep their lungs healthy? Do you have any lifestyle tips?
Elliott_Dasenbrook,_MD: Exercise is one of the most important things CF patients can do to keep their lungs healthy. Exercise helps clear out mucus, strengthens your bones and improves your mood. Exercise can consist of brisk walking, Yoga, weight lifting, swimming and/or bike riding. Exercise is in addition to the airway clearance regimen (i.e., albuterol, hypertonic saline, VEST and/or dornase alfa) that you will develop with your CF team.
Signs and Symptoms
goinggone: Dr. Dasenbrook, you mentioned that CF affects the gut. Can you explain what kind of conditions/complications might be expected?
Elliott_Dasenbrook,_MD: One of the most common gut conditions that CF patients have is pancreatic insufficiency. This can result in bloating, intermittent episodes of crampy abdominal pain and frequent bowel movements. CF can also be associated with acid reflux, gallbladder disease, liver disease and intermittent episodes of constipation.
MayaAnne: What are the early symptoms of cystic fibrosis?
Silvia_Cardenas_Zegarra,_MD: One of the most common manifestations of cystic fibrosis is pancreatic insufficiency. Patients with this issue do not have the pancreatic enzymes that are needed to absorb fat, and therefore can have difficulty gaining weight, despite eating many calories. Pancreatic insufficiency (fat malabsorption) is present in 85 percent of CF patients. Fortunately, this can be treated by giving special enzymes (in the form of pills) to the patients. Lack or insufficiency of pancreatic enzymes can cause other symptoms, including loose or greasy stools, bloating and abdominal pain. All of these will improve with enzyme treatment.
MaryJoeee: What are the respiratory symptoms with CF?
Silvia_Cardenas_Zegarra,_MD: Patients with CF present with multiple respiratory symptoms such as chronic cough, recurrent wheezing, recurrent pneumonias, recurrent sinus infections and shortness of breath. These may be present since very early in life.
goinggone: Does Cleveland Clinic do lung stem cell research for CF?
Elliott_Dasenbrook,_MD: Stem cell research is in the very early stages in cystic fibrosis. The current research that is being done is making sure that it is safe for CF patients and finding which type of stem cells may be the most beneficial for CF.
hope9: Is there new CF research you are aware of for treatments or a possible cure?
Elliott_Dasenbrook,_MD: The CF genetic defect results in a chloride channel that does not work. There are two new exciting treatments that help improve the function of the chloride channel. The first new treatment is called ivacaftor and is indicated in CF patients with specific mutations (about 10 percent of CF patients in the US have these mutations). The second medication is lumacaftor and ivacaftor. This medication is indicated in CF patients that have two copies of the F508del mutation. Both of these medications are pills that patients take twice daily. They have been associated with improved lung function as well as fewer lung infections requiring antibiotics. The entire CF community is working toward a cure.
waverunner9: Does Cleveland Clinic have any support groups for CF families, or do you know of any groups in the area?
Elliott_Dasenbrook,_MD: We do not have specific support groups (other than the CF team). However, the CF Foundation is a great resource for patient and family support. Check out cff.org.
That is all the time we have for questions today. Thank you, Dr. Dasenbrook and Dr. Cardenas Zegarra, for taking time to educate us about cystic fibrosis.
On behalf of Cleveland Clinic, we want to thank you for attending our online health chat. We hope you found it to be helpful and informative. If you would like to learn more about the benefits of choosing Cleveland Clinic for your health concerns, please visit us online at my.clevelandclinic.org.
To make an appointment with Elliott Dasenbrook, MD, or any of the other specialists in Cleveland Clinic’s Respiratory Institute, please call 216.444.6503 or toll-free at 800.223.2273 (ext. 46503) or visit us at clevelandclinic.org/respiratory for more information.
To make an appointment with Silvia Cardenas Zegarra, MD, or any of the other Cleveland Clinic Children’s specialists please call 216.444.KIDS or visit us at clevelandclinicchildrens.org for more information.
For More Information
At Cleveland Clinic's Respiratory Institute, the No. 3 ranked pulmonology program in the United States, we provide world-class patient care by combining our strengths in clinical expertise, research and education. Serving nearly 90,000 patients annually, the Respiratory Institute’s unsurpassed expertise and experience attracts patients from all over the world who seek a definitive diagnosis and cutting-edge treatment, often for a rare or complex lung disorder. With more than 60 pulmonologists, allergists/immunologists and critical care specialists, the Respiratory Institute diagnoses and treats a wide range of lung, allergy and breathing-related conditions.
Children's Hospital's Pulmonology program is consistently ranked in U.S. News & World Report's “Best Children’s Hospitals.” Our pediatric pulmonologists, critical care specialists and allergists have unparalleled expertise, attracting national and international patients seeking diagnosis or advanced treatment, often for rare or complex disorders.
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