Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
These preschool guidelines provide clinical care guidelines for people with cystic fibrosis between 2 and 5 years. It includes 53 recommendations intended to help make informed decisions in the care of preschool-aged children with CF.
Lahiri T, Hempstead SE, Brady C, Cannon CL, Clark K, Condren ME, Guill MF, Guillerman RP, Leone CG, Maguiness K, Monchil L, Powers SW, Rosenfeld M, Schwarzenberg SJ, Tompkins CL, Zemanick ET, Davis SD. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics. 2016;137(4):e20151784. Epub 2016 mar 16. PMID: 27009033
In 2014, a multidisciplinary committee convened to develop preschool guidelines to fill in the gap between existing clinical care guidelines for infants 2 years and younger and individuals 6 years and older. Fifty-three recommendations were included on the following topics:
The purpose of these guidelines is to help make informed decisions in the care of preschool-aged children with CF. Research is needed to improve the strength of many of the recommendations in the preschool guidelines.
Since these guidelines were published, guidelines for enteral tube feeding in individuals with CF have been published that provide more detailed recommendations on the process of enteral tube placement and feeding.
Fifty-three recommendations were approved by 80 percent or more of the committee. Reflecting the limited evidence in this age group, the majority of recommendations were made based on consensus only, and only three recommendations met the U.S. Preventive Services Task Force (USPSTF) Grade A criteria. Find additional information about USPSTF grading definitions.
The guidelines recommend the following for children with CF, ages 2 to 5 years:
Routine well-child care according to American Academy of Pediatrics (AAP) guidelines, including routine immunizations.
Children, family members, and caregivers should receive annual influenza vaccinations.
The first dose of pneumococcal polysaccharide vaccine (PPSV23) should be administered.
Parents and CF health care professionals should review treatment goals and care plans quarterly.
Spirometry should be attempted as early as 3 years old and be used to monitor for exacerbations and response to therapy.
Chest X-rays should be obtained at least every other year.
A chest computed tomography (CT) scan may be considered every two to three years, to replace chest X-rays, using the lowest possible radiation dose.
Oropharyngeal cultures should be performed at least quarterly to monitor airway microbiology.
Daily airway clearance is recommended.
For children who are pancreatic insufficient, the maximum pancreatic enzyme replacement therapy dose is 2,500 lipase units/kilogram (kg)/meal and 10,000 lipase units/kg/day.
Hypertonic saline and dornase alfa may be selectively offered based on individual circumstances.
Children persistently infected with Pseudomonas aeruginosa should be treated chronically with alternate-month inhaled antipseudomonal antibiotics.
Ivacaftor should be used for children with specific gating mutations and considered for children with a confirmed diagnosis of CF and an R117H mutation.
For pulmonary exacerbations:
Weight-for-age ≥10th percentile should be maintained.
Use body mass index (BMI) percentile to assess weight-for-stature and maintain BMI ≥50th percentile.
CF team members should work with the family to set energy-intake goals and assess progress regularly.
For those meeting optimal nutrition thresholds, calorie intake should be ≥90-110 kcal/kg/day, and protein intake should be ≥13g protein/day in 2- to 3-year olds and ≥19 g protein/day in 4- to 5-year olds.
For children at nutritional risk who are not responding:
Routine pulse oximetry, assessment of bronchodilator responsiveness, or multiple breath washout
Chronic inhaled bronchodilators, high-dose ibuprofen, leukotriene modifiers, or azithromycin
Attempts to eradicate Staphylococcus aureus (S. aureus), including methicillin-resistant S. aureus (MRSA), in asymptomatic patients
Chronic use of oral antistaphylococcal antibiotics in children persistently infected with S. aureus
Oral antistaphylococcal antibiotics
The authors note that additional research is needed in the following areas for preschool-aged children with CF:
Schwarzenberg SJ, Hempstead SE, McDonald CM, Powers SW, Wooldridge J, Blair S, Freedman S, Harrington E, Murphy PJ, Palmer L, Schroeder AE, Shiel K, Sullivan J, Wallentine M, Marshall BC, Leonard AR. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016 Nov;15(6):724-35. Epub2016 Sep 3. PMID: 27599607. This article provides more guidance on the process of enteral tube placement and enteral feeding in individuals with CF.
Don B. Sanders, M.D., M.S. and Stephanie D. Davis, M.D. (Indiana University School of Medicine)
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