Tuesday, March 21st, 5 - 6 p.m., E.S.T. | | |  | | | | | | Everything You Wanted to Know About Cystic Fibrosis and the Johns Hopkins CF Center Who
Pulmonologist Peter J. Mogayzel,
Director of the Cystic Fibrosis Center,
conducted this chat. | | | | | | | | | |
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Dr. Mogayzel Hi, I am Peter Mogayzel and I direct the Cystic Fibrosis Center at Johns Hopkins. Welcome to the first online chat at www.hopkins.org. The CF center at Johns Hopkins cares for approximately 425 patients. Approximately 1/3 of the patients are adults. We have an extensive research program that spans basic science to the development of new therapeutics. Johns Hopkins is one of a group of CF centers around the country that is part of the CF Foundation’s Therapeutics Development Network which conducts multi-center drug trials. We are very fortunate to have an excellent adult team with 3 full-time pulmonologists and a full multi-disciplinary team. I am very excited about this opportunity to chat with you about CF.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients:Are there any imported fruits and vegetables that we should avoid because of potential bacteria? We were once told that the black stuff on onion skins is cepacia, so of course we stopped buying them (an easy thing to do). We always wonder, though, what else could be harmful?
Dr. Mogayzel No. You do not need to worry about any fruits or vegetables. You are correct in that the bacteria Burkholderia cepacia does in fact cause onion rot. In fact cepacia is derived from the Latin word for onion. However, it is unclear how much risk is posed by household onions. Clearly onion fields are an issue, but onions at home may be OK. You probably can go back to eating onions too as long as you are careful not to let them rot. I also have a patient that buys onions and peels them and puts them in frige in plastic bags to prevent any problems.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients: Are you using hypertonic saline at Johns Hopkins?
Dr. Mogayzel Yes, we have been using it in selected patients for about a year. The studies that were recently published in the New England Journal of Medicine suggest that using hypertonic saline twice a day will improve lung function slightly. But more importantly, this therapy will decrease the number of infections that occur. It is unclear who will benefit from hypertonic saline. We typically use it in those patients that have declining PFTs or frequent pulmonary exacerbations.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients:Can my child go swimming in a pool?
Dr. Mogayzel Chlorinated swimming pools are fine. Pseudomonas tends to be found around water. Therefore, I would stay away from standing fresh water and swampy areas. Also stay away from hot tubs since they can be a large reservoir for pseudomonas.
Cindy F. Your 2 yr old patient, Amelia, is very excited to see your face on the computer. Is that Dr. Mogayzel, she asks.
Dr. Mogayzel I'm glad she's having fun chatting.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients: A while ago I read about a center that was testing a new form of technology. It enabled parents to record and somehow transmit a child's coughing to the Center so the medical team could listen and as a result, get a better sense of the nature of the cough. Do you know if this was successful? If so, is it something you'll consider at JH?
Dr. Mogayzel This is a new way of doing something we've done for years. We have parents hold the phone up to their children while they cough. It sometimes helps, but usually it's not that useful. I am not familiar with the new technology, but it may be useful at some point in the future. We do not have any plans to introduce it into the Hopkins CF Center.
Cindy F. Do you know when the ABC documentary Hopkins 24/7 will air?
Dr. Mogayzel Hopkins 24/7 was made several years ago. It repeats on Discovery Health every now and then. We could show parts of it at one of our family support group meetings.
Eric What about hypertonic saline? Is that the next big "thing"?
Dr. Mogayzel We have started using hypertonic saline in selected patients at Hopkins over the past year. I have been impressed with its effect in some patients. Over the next year its use will become more widespread.
Eric Who is a good patient?
Dr. Mogayzel It is not clear who will benefit the most from hypertonic saline. We typically use it in those patients who have declining PFTs or frequent pulmonary exacerbations.
KPT How often do you have family support group meetings?
Dr. Mogayzel We typically hold them once a month. Our last meeting addressed adolescent issues.
Bob Should I be worried about catching germs in clinic?
Dr. Mogayzel We know that bacteria can be passed from one CF patient to another. We make every effort to prevent the spread of bacteria between CF patients.
Moderator While the doctor is responding, we just wanted to remind everyone that transcripts will be available on our website tomorrow!
Dr. Mogayzel The best protection is good handwashing. CF patients should also keep at least 3 feet away from other CF patients in clinic. We clean all the surfaces to prevent any spread of germs. We discuss this issue in more detail on our website www.hopkinscf.org.
Cindy F. Can you please explain exactly what an exacerbation is?
Dr. Mogayzel There is no perfect definition, but typically an exacerbation can be defined as having more respiratory symptoms. This can occure acutely (quickly) or slowly over time. An exacerbation usually requires treatment with antibiotics because it is due to the overgrowth of bacteria in the lung . Typical symptoms include increased cough or sputum production, fatigue and weight loss.
Eric What can be done to motivate patients with CF to do their therapies?
Dr. Mogayzel It is best to try and explain the importance of therapies in preventing lung damage and gaining weight. Making the child a part of the treatment regime is very important. They must believe that these therapies are useful.
Ethan I've heard bisphosphanates help build bone strength in CF patients. How do they work?
Dr. Mogayzel Bisphosphanates work by preventing the normal remodeling of bone that occurs. These can be very helpful in patinets with osteopenia or osteoporsis. We discuss this issue in more detail in this website.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients: We were just told by a doctor at another CF center that we can give enzymes with banana pudding. Is this true? We were always told to stick with applesauce, ketchup or jelly.
Dr. Mogayzel You are correct. Pancreatic enzymes must be given in an acidic food such as apple sauce. Pancreatic enzymes are destroyed by stomach acid so they are manufactured into little beads with an enteric coating. This coating protects them from stomach acid and breaks down in the alkaline environment of the small intestine. If you put them in dairy products like yogurt or pudding, the coating will breakdown and the enzymes will be destroyed by stomach acid. This will make the enzymes less effective.
Cindy F. We know of a 2-year-old who has cancer, and doctors are harvesting stem cells for him to use in the future. Is this a possible benefit for CF patients?
Dr. Mogayzel There is active research on the use of stem cells in CF. At this point it is unclear whether this therapy will be helpful in CF. There may be a time when this therapy will be available, but I would not harvest any cells now.
Bob Is it better for a patient to see the same CF physician at each visit, or several different CF physicians over the course of a year?
Dr. Mogayzel We believe that it is useful for patients to see different CF physicians when they come to CF clinic. This will allow families to become familiar with all the physicians at Johns Hopkins. Our physicians have a very similar practice style and it is important for parents and children to feel comfortable with any of the physicians in our Center.
Sebastian Where do you see the major thrust in CF research today?
Dr. Mogayzel There are a number of therapies that are directed at the basic defect in CF. This is very exciting because up until now therapies have been directed at the symptoms of CF. There are several drugs in the pipeline that will improve salt transport in cells, the best therapy for CF. However, the next drugs to be approved will most likely be a new inhaled antibiotic (Aztreonam) and a new pancreatic enzyme. The next generation of drugs will be available in the next 5-10 years.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients: I just heard of a plastic cup sold that has a place to put pills and make it easier for young children to learn how to swallow. Do you know where I can find this?
Dr. Mogayzel These cups have a lip on the edge of the cup that holds the pill so that when you take a drink you swallow the pill along with the liquid. Pill taker cups can be purchased from a number of sources. www.mileskimball.com has one for $3.99. Another trick to swallowing pills is to have kids use mini M&M’s to practice. Once they have mastered these then you can try small-dose enzyme capsules.
Eric Are clinical trails available at Hopkins for CF patients?
Dr. Mogayzel There are many ongoing clinical trials at Hopkins. More inforamation can be found at http://www.hopkinscf.org/main/research/clinical.html. It is very importnt for CF patients to participate in clinical research. The only way to know if a drug will work is to try it in CF patients. Clinical trials often take hundreds of patients at many centers. If patients do not participate, the rate of drug development will slow.
Moderator The following is a question submitted prior to the event by one of Dr. Mogayzel’s patients: Approximately how long do you think it will be until the Pseud. vaccine is available?
Dr. Mogayzel I think it will be several years before a vaccine against pseudomonas will be available in the United States. There has been a version available in Europe as part of ongoing research trials; however, large-scale efficiency studies have not been carried out. There are several strategies to develop a different vaccine for use in the U.S., but these are in the earliest stages of development.
Sylvia How would you describe the support from the federal government, NIH, for research into CF? Are you optimistic about the development of new treatments in the next decade?
Dr. Mogayzel The NIH does support CF research. However, the most important commitment towards research is from the CF Foundation. The CF Foundation has a very large research program directed at developing new CF therapies. I am very optimistic about new therapies coming along. However, these therapies will not reverse lung damage so it is vitally important that we do everything to protect the lungs of CF patients so that they can benefit from the new therapies when they arrive.
Eric Do animals get CF?
Dr. Mogayzel Thee are no known animals in the wild with CF. However, researchers have created mice that lack the CFTR gene. These animals have severe GI problems but not much lung disease.
Elizabeth I understand that women with CF who choose to have children cannot take antibiotics for pulmonary problems during pregnancy? What are their options?
Dr. Mogayzel There are a number of therapies that can be used during pregancy to keep mothers and their unborn children healthy. There are antibiotics that are safe to use in pregancy. To have a healthy baby the mother must stay healthy as well.
Steve Do I really have to come to clinic every 3 months?
Dr. Mogayzel Yes, you should come to clinic every 3 months. This allows the CF team the opportunity of keeping you or your child as healthy as possible. By coming to clinic often we are able to detect problems early, which allows us to fix problems early.
Moderator Thank you for attending our live online event. We look forward to seeing you next time! The event is now over.
Dr. Mogayzel I would like to thank everyone for participating. If you have addition questions please feel free to e-mail us at hopkinscf@jhmi.edu. 
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