Nationally Recognized Cystic Fibrosis Treatment
UH Rainbow Babies & Children’s Hospital is home to one of the largest cystic fibrosis centers in Ohio - and one of the first Cystic Fibrosis Foundation-accredited care centers in the nation. For more than 50 years, our comprehensive and compassionate treatment of patients with cystic fibrosis has been nationally recognized.
As an academic medical center, our board-certified physicians are experts in the field of cystic fibrosis who are also training the next generation of specialists. Through our work, we help patients from across the region who need world-class cystic fibrosis treatment options. From routine to the most complex care, including innovative clinical trials, our patients receive the most advanced cystic fibrosis care available. We are passionate about improving the quality of life for our patients - and helping them thrive well into adulthood.
Zoe’s Wish for Cystic Fibrosis Patients
Comprehensive Care for Better Long-Term Outcomes
Cystic fibrosis (CF) is an inherited or genetic disorder that causes the body’s natural mucus to become thick and sticky in the lungs and other organs. Instead of lubricating organs as they normally would, this mucus coats and clogs up important passageways in the body. Cystic fibrosis is particularly damaging to the lungs and pancreas.
A cystic fibrosis diagnosis is devastating as it is chronic, progressive and no current cure exists. However, recent research and treatment breakthroughs have greatly improved the longevity and quality of life for those living with cystic fibrosis. Patients with CF now live into their mid- to late-thirties on average, and some survive into their 50s and beyond. We’re honored to help children and adults with a CF diagnosis live happy, full and longer lives. This also gives us special expertise in the care of older CF patients, whose cases may be more complicated.
Thanks to our comprehensive, multidisciplinary approach, we can achieve better outcomes. We’ve embedded a wide range of specialists into our CF care team so we can bring a vast depth of expertise to our patients under one roof. Our multidisciplinary team includes experts in:
- Pediatric pulmonology
- Pediatric endocrinology
- Pediatric gastroenterology
- Internal medicine
- Pediatric psychology
- Adult gastroenterology
- Adult pulmonology and critical care
- Hospice and palliative care medicine
In addition to our primary CF physicians, we have access to a consulting staff of more than 40 adult and pediatric specialists who are knowledgeable, experienced and readily available for our cystic fibrosis patients, including:
- Transplant surgeons
- ENT specialists
- Critical care specialists
Advanced Diagnostic and Treatment Options for Cystic Fibrosis
Having a child diagnosed with cystic fibrosis can be overwhelming. At UH Rainbow Babies & Children’s Hospital, we’ve invested in the latest technologies and advanced cystic fibrosis treatment options so our patients receive the best care available. We offer a wide range of services to guide the process from diagnosis to treatment to long-term disease management. These services may include:
- Newborn screenings: CF is a required screening for every newborn. If your child’s screen comes back positive for CF, you may be referred to UH Rainbow Babies & Children’s for additional testing.
- Diagnostic sweat testing: To confirm a cystic fibrosis diagnosis, your child will need a sweat test. A chemical is applied to the arm to cause the sweat glands to become more active. We collect the sweat and test for salt levels. The process takes about 60 minutes. It is not painful, but will cause a tingling feeling in the arm.
- Specialized nasal laboratory: We test for measurements of nasal potential differences, which are indicators of CF.
- Genetic evaluations: Babies who do not have cystic fibrosis, but who tested positive on the first newborn screening, are often carriers of the CF gene. This means that although they are not diagnosed with CF, they might pass on the disease to their future children. A cystic fibrosis genetics evaluations can confirm future risk of having a child with CF.
- Lung function testing: These diagnostic lung function tests measure how well the lungs can exchange oxygen and carbon dioxide. This determines severity of a problem in lung function, and monitors the effectiveness of treatment. We perform this testing in children, adolescents and adults.
- Bronchoscopy: Using a scope, we can examine the throat and airways to monitor for any abnormality or infection. This also helps our pulmonary specialists see and test material deep within the lungs.
- Nutrition monitoring: It’s important for patients with CF to eat foods that are high in protein and calories throughout the day. This helps CF patients maintain a normal weight, because the disease makes it harder for bodies to digest and absorb proteins and fats.
- Radiology services: Our radiology services include bronchial artery embolization (used to treat bleeding from the lungs) and provide difficult line placement for long-term medication treatment.
- Full laboratory services: Our lab work is specialized for CF care, including microbiology, biochemistry, hematology and immunology.
- Counseling and support groups: We offer support groups for patients and their families who are fighting CF.
Dedicated Research to Advance CF Treatment
With dedicated research efforts, our team has achieved scientific discoveries that are now a mainstay of CF treatment. For example, in partnership with Case Western Reserve University School of Medicine, UH Rainbow Babies & Children’s Hospital was one of the leading research sites for the development of the renowned CF drug Ivacaftor. Our team was the highest contributor to the research conducted to develop this new and life-changing medication. Our team was also part of the world’s first stem cell trial for adults with CF. The trial offers pulmonary specialists a first look at how stem cell treatment could improve quality of life for people with this difficult diagnosis.
Our patients also have access to clinical trials, which means they can participate in new and innovative therapies before they are widely available. We are deeply committed to research and innovation that leads to better outcomes – and ultimately finding a cure – for children and adults diagnosed with CF.
Convenient Inpatient & Outpatient Care
Our CF clinic for both children and adults is held on the main campus of University Hospitals Rainbow Babies & Children’s Hospital. This unique care model, with pediatric and adult specialists working side by side, brings the entire spectrum of CF care into one setting and allows for outstanding continuity of care. In addition to the primary CF Physicians, our dedicated team of nurses, respiratory therapists, dietitians, pharmacist and social workers are available at each of your clinic visits.
We also offer a state-of-the-art inpatient facility. When hospitalization is required, pediatric patients are admitted at University Hospitals Rainbow Babies & Children’s Hospital in a specialized unit dedicated to the care of pediatric pulmonary patients. Adult patients are admitted to a 10-bed unit devoted exclusively to the care of patients with cystic fibrosis over age 18.
The adult unit has age-appropriate accommodations and activities for patients with all stages of disease. CF patients are placed in private rooms and the infection control precautions recommended by the Cystic Fibrosis Foundation are strictly followed.