How Does Cystic Fibrosis (Cystic Fibrosis) Affect Children?

Cystic fibrosis is an inherited disorder that causes severe damage to the lungs and digestive system. Cystic fibrosis requires daily care to manage, but improvements in screening and treatments have meant that people with Cystic Fibrosis now have a better quality of life than in previous decades. The Cystic Fibrosis team at MemorialCare Miller Children’s & Women's Hospital Long Beach have dedicated their careers to treating and improving the lives of children and young adults with Cystic Fibrosis.
How Does Cystic Fibrosis (Cystic Fibrosis) Affect Children?
Featured Speaker:
Cyrus Shahriary, MD
Cyrus Shahriary, M.D., received his medical degree from Ghazvin University of Medical Science in Ghazvin, Iran, and completed his residency in pediatrics at Ali Asghar Children’s Hospital in Tehran, Iran. During his time at Ali Asghar, he was appointed chief resident.

Following his residency, he received additional training in pediatrics at the Maimonides Infants and Children’s Hospital of Brooklyn and completed a fellowship in pediatric pulmonology at Miller Children's & Women's Hospital Long Beach. Board-certified in pediatrics, Dr. Shahriary serves as a general pediatric hospitalist and pediatric pulmonary/cystic fibrosis attending for Miller Children's & Women's. He also serves as director of the Pediatric Pulmonary Function Laboratory and associate director of the Cystic Fibrosis Center.

He is a fellow of the American Academy of Pediatrics and an active member of the Iran Society of Pediatrics and the Iran Medical Council.
Transcription:
How Does Cystic Fibrosis (Cystic Fibrosis) Affect Children?

Deborah Howell (Host): Welcome to the show. I'm Deborah Howell. And today, we'll be asking the question, does cystic fibrosis affect children? Our guest is Dr. Cyrus Shahriary, a pediatric board-certified pulmonologist and Associate Director at Children's Pulmonary Institute and Associate Director of the Cystic Fibrosis Center at MemorialCare Miller Children's and Women's Hospital Long Beach. Welcome, Dr. Shahriary, or as your patients call you, Dr. Cyrus.

Dr Cyrus Shahriary: Hi, good morning. How are you doing?

Deborah Howell (Host): Wonderful. Glad to have you. What is Cystic Fibrosis, also known as cystic fibrosis with a capital C and a capital F?

Dr Cyrus Shahriary: Cystic Fibrosis is a rare disorder that is a genetic disease, that every patient will get one gene from each of their parents. It's a rare disorder. There's around 40,000 patients around the United States. And worldwide, there are around 105,000 to 110,000 patients.

Deborah Howell (Host): Do we know the causes of cystic fibrosis?

Dr Cyrus Shahriary: So, as I said, it's a genetic disorder when we have two defective genes, one came from mother, one came from father. And our patients will have some defects in the process of washing off the open tubes in the body difficult. We can say it as easy as there are open tubes in the body that always have to be cleaned out. Because of this defective gene, the mucus in this tubes just plug up and close down those tubes, and that's where the problem starts. So, it can affect the places that has open tubes, most likely the lungs of the patients who get plugged with mucus. Also, it'll affect the pancreas which is needed to provide enzymes to digest food, and the tubes in the pancreas gets plugged. Also, there are few tubes in the liver, in the reproductive center, especially in males, that all of them will be affected and eventually will cause some problems in our patients.

Deborah Howell (Host): Okay. And how do we diagnose it?

Dr Cyrus Shahriary: So, thanks to the past 10, 15 years efforts from national like Cystic Fibrosis Foundation, now there is a newborn screen going nationally. And in California, we have had it since 2007. So as babies are born, all the babies around the country will give few drops of their blood that is taken from the heel stick, and it's sent to a center that screens for multiple, multiple diseases, one of them being cystic fibrosis. And after the blood is screened, mostly 95% of the patients are picked up during those screening efforts and it will be notified to the pediatrician who will contact the parents and discuss the positive findings of the newborn screening.

Deborah Howell (Host): Now, doctor, since cystic fibrosis affects so many systems in the body, how does Miller Children's and Women's help manage and treat it?

Dr Cyrus Shahriary: So as Miller Children's Hospital has been dealing with cystic fibrosis patients in the past 30 and 40 years, it has then progressed significantly, and we are one of the most aggressive and conservative centers on managing our cystic fibrosis patients. We usually will diagnose the patients after they're born. The pediatricians or other centers refer the patients to our center. And because we are affiliated with the Long Beach Memorial Center, our patients can spend the whole duration of their treatment from birth to long life that they now can have throughout the adult center at our center.

Our center deals with these patients from usually first weeks of their life, we have a great setting in our center. We have a genetic counselor that usually talks in detail with the parents. As you know, if a parent hears that their child has a rare disorder, the first thing that they will get is anxiety. They get upset, they get frustrated, that why my kid has this disorder. And so for the first few visits, along with our great team that we have, we have a social worker, we have a very caring nurse manager, we have our genetic counselor and the whole physician team try to go through this process with the parents and discuss what's the outcome of the patients and how the managements and the treatments will work for the patients.

Deborah Howell (Host): I'm glad you said these treatments are working because those with cystic fibrosis are improving outcomes and are living longer than ever before. What do you feel helped contribute to that change?

Dr Cyrus Shahriary: If you look at it around 20, 30 years ago, cystic fibrosis patients had not had that much quality of life, and they didn't look into getting even to the mid adulthood. But thankfully, in the past 20 years, and especially in the past five to 10 years, with all the treatments that are available, now more than 50% of the patients with cystic fibrosis are actually adults above 18. And the quality of life have significantly improved, especially with the recent discovery of what we call CF modulators. that has been on market for the past five to seven years. And recently, in the past two years, many effective new modulators have combined and has been very effective in treating these patients.

Deborah Howell (Host): Can you tell us a little bit about those modulators?

Dr Cyrus Shahriary: So, I've noticed these modulators are not cure, so the patients have to take them every single day. But it tries to modify the product of these defective genes. So, at least 10% of the protein that has to be made in the body gets to the surface of the cells that are defective, and now the cells are working properly.

Deborah Howell (Host): That is absolutely wonderful. Can you explain how Miller Children's is a regional leader in the management and treatment of cystic fibrosis?

Dr Cyrus Shahriary: We are one of the largest centers in the West Coast, specifically in the south coast and south California. We have nearly a hundred of our pediatric patients and over a hundred adult patients that are transitioned to Adult Memorial Center. We provide wide range of national and local research studies that has helped actually with finding these discoveries. And we are one of the centers that directly works with the CF Foundation research centers. And we have been participating in national research in finding the treatments. And hopefully in the new future, we will work towards finding a cure for the rest of the patients that around maybe 10% to 20% in our community are still not qualified for the modifiers.

Also, I can say that we provide our patients with up-to-date and state-of-the-art studies, including our complete pulmonary function lab and, along with our new cystic fibrosis village, a coordinated care along with a GI, nutritionist, social worker, genetic counselor, nurse manager, respiratory technicians. We provide a full and complete coverage for the cystic fibrosis patients, so they don't have to go to different centers and different clinics for their care to be managed.

Deborah Howell (Host): That is fantastic. Now, what are some of the things parents can do to support their child with cystic fibrosis?

Dr Cyrus Shahriary: After the initial shock of the diagnosis, usually the parents will take it a little bit harder on themselves on dealing with these patients. They have to make sure that first of all, the clinic, our CF center is behind them and they will go over every step that they take for the patients. The second thing to know is cystic fibrosis needs daily and progressive support for the patients because, during the first few years of life, they will receive at least two to three times a day breathing treatments along with other medication that they have to take for their GI system to work and is able to digest and absorb the food.

As they grow up, these continuous treatments on daily basis, the patients, especially our younger patients, get frustrated that why they have to sit down for 30 minutes to get a breathing treatment, why they have to take pills with their lunch, with their dinner, why do they have coughing in front of their friends. So, they have to go along with their child, support them and provide them with psychological support for them to go through this difficult times. Also, we have to know that as the child grows up, because this is a disease that will stay with the patient, thankfully, again, as I said, with the modifiers, and we are now having better outcome for them, but they still need to take pills, they still need to do their breathing treatments. And as they grow up into the teenagers, most of our patients will be very upset that they have the disorder. So, this is where the support of the family really needs to kick in and help them to go through these difficult times and understanding and being able to manage their disorders.

Deborah Howell (Host): Yes, absolutely. Teenage years are rough, even just if you're normal and don't have cystic fibrosis. So, maybe you can explain how parents can help bring a sense of normalcy to children living with cystic fibrosis, you know, including playing sports and attending school, et cetera.

Dr Cyrus Shahriary: Actually the cystic fibrosis patients, if the parents can set up a good schedule for the patients, that, "Okay, you have to do these treatments twice a day," usually they last 20 to 30 minutes of the breathing treatments, so they have a set schedule for them in the morning before they go to school. And in the evening, maybe before their dinner time, they will sit down watching tv, playing games, and some other activities and they do their treatments. If they have a of schedules for their treatments, then most of our patients actually are very active in different social activities. All of them, we encourage them to participate in different sports. We had many good football players, soccer players. We have very, very successful swimmers, surfers, water polo players. So, there is no limitation to what they do if they stick to their treatments, and especially if they stick to their schedule and they will have no limitations except for little bit of bumps on the way as they grow up because they may get infections, they may have a little more cough and during those exacerbations are the times that they will feel, "Okay, this is a real deal and I need to take more medication," sometimes they need to get hospitalized and take the treatments in the hospital. But other than that, we have many, many successful patients in sports. And many of our patients go on into very, very prestigious colleges and universities. And as I said, because of the lifespan has improved, they are now growing to become fathers, mothers, and very successful personnel in the community.

Deborah Howell (Host): Wonderful. It's such good news. Where can people go to learn more about cystic fibrosis, Dr. Cyrus?

Dr Cyrus Shahriary: Something that everybody should know, you cannot go online and Google, "Okay. Cystic fibrosis." Because as soon as they Google cystic fibrosis, as we know, many of the bad misinformation pops up on the top of the Google page. The best place to look for cystic fibrosis information is Cystic Fibrosis Foundation, cff.org. So, that's the foundation that supports the CF research, supports CF treatments, and most of the patients are under their care and they can get great information within that website. And also if any patient, any parent has any questions, they can reach us through the Memorial Care Communication Center through the Cystic Fibrosis Clinic, and we will be back to them with information if they need any.

Deborah Howell (Host): Perfect. Once again, cff.org. Thank you so much, Dr. Shahriary, for your time and your expertise today. We really, really enjoyed having you on the show.

Dr Cyrus Shahriary: Thanks a lot for having me. And hopefully, the information will be helpful for our patients and their parents.

Deborah Howell (Host): I'm sure it will. And for more information or to listen to a podcast of this show, please visit memorialcare.org. That's memorialcare.org. That's all for this time. I'm Deborah Howell. Have yourself a terrific day.