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.
Cell models have been critical to enhancing our understanding of the physiological, biochemical, and genetic mechanisms underlying cystic fibrosis and to developing therapeutic strategies. The Cystic Fibrosis Foundation provides funding and support to make several cell model systems available to researchers.
Although primary human bronchial epithelial (HBE) cells are the “gold-standard” for drug discovery in cystic fibrosis research, scientists are limited in the genotypes available because of how they are collected. For this reason, a number of other cell models have been developed to fill the gap.
Below is a listing of some of cell models available through the Cystic Fibrosis Foundation or other sources to advance CF research.
Fischer Rat Thyroid (FRT) cells have been used extensively for studies of cystic fibrosis transmembrane conductance regulator (CFTR) protein for more than 20 years and have been a ”workhorse” for drug discovery in the disease.1 The cells have been used by the U.S. Food and Drug Administration (FDA) as relevant to expanding drug label and therapeutic access for individuals with very rare forms of cystic fibrosis.2,3
FRT cells expressing CFTR are established using Flp-In™ technology.4 All cells are based on the background of a clonal FRT Flp baseline (cells bearing a single FRT insertion site), FRT Flp4, to generate CFTR variant lines. FRT Flp4 cells are clonal and selected based on zeocin and β-galactosidase activities expressed from the base construct (pFRT/lacZeo).4-6 FRT Flp4 base cells contain a single copy of lacZ-Zeocin fusion gene as shown by Southern blot and copy number variance analysis (Applied Biosystems).5 FRT wild-type and mutant CFTR cells express CFTR on an isogenic background generated using FLP-mediated recombination between pairs of FRT sites.5 All of the listed FRT Flp-CFTR cells are clonal and hygromycin resistant and selected based on very similar mRNA expression levels.5 FRT cells are commonly used in the following types of studies:
To request FRT cells, please contact:
Jeong S. Hong, PhD, Andras Rab, PhD, or Eric J. Sorscher, MD
Immortalized human CF bronchial epithelial cells (CFBE41o- generated by D. Gruenert, UCSF) were modified using a single Flp recombination target to express CFTR cDNA to generate CF8Flp cells (as reported in Gottschalk et al. 2016). CF8 cells are commonly used in the following types of studies:
To request CF8Flp cells, please contact:
CFTR2 Cell Center
PI: Garry Cutting, MD
Johns Hopkins School of Medicine
Immortalized, CFTR wild-type expressing 16HBE14o- cells (generated by D. Gruenert, UCSF) were gene edited at the endogenous CFTR locus using CRISPR/Cas9 to create isogenic cell lines (16HBEge). During the immortalization of the parental cells, SV40 sequence was inserted into one of the two CFTR alleles in these cells, rendering these cells monoallelic with respect to functional CFTR expression. (Full SV40 sequence information and whole genome sequencing data for parental cell line available upon request.) These cells have the native CFTR gene construct and are commonly used in the following types of studies:
Cells can be requested from the Cystic Fibrosis Foundation Therapeutics Lab (CFFT) by filling out a Material Transfer Request form.
For questions related to these cells:
Hillary Valley, PhD
A large number of human respiratory cell lines, originally collected and distributed by Dr. Dieter Gruenert, have been used to facilitate research for academic and non-commercial research purposes. This collection includes the 16HBE14o- and CFBE41o- parental cell lines as well as a number of other CF lines from different respiratory tissue locations, such as the nasal passages, submucosal glands, and tracheal tissues. In addition to the respiratory cells, the collection also includes a number of other cell lines, such as the CF pancreatic ductal cells (CFPAC), and induced pluripotent stem cells.
The USCF Cell and Genome Editing Core recently began housing and distributing these cells for researchers. Cells are still being processed into the system and will be added to inventory list as that process is completed. However, if you would like to request cells that do not appear on the list, please contact firstname.lastname@example.org to inquire about availability.
To request cells, please follow the process outlined here. If you run into any issues, please contact:
USCF Cell and Genome Editing Core
HBE cells are collected from explanted lungs after transplant. These cells are considered the “gold standard” for evaluating CFTR function in a cell type representative of the CF lung. When cultured at air-liquid interface (ALI) conditions on permeable filters, the cells resemble a pseudostratified airway epithelium as seen in the airways.
Ideal for the following types of studies:
This list of mutations available in HBE cells changes regularly based on availability of cells and is not inclusive of all mutations available through the CF Foundation. If you are interested in a mutation that is not on the list, you can inquire about its availability when filling out the Material Transfer Request form by clicking on “other” and indicating the mutation of interest in the text box.
As HBE cells are a limited resource, please note that all requests will undergo a review process to determine scientific merit of the study before requests will be granted.
Human nasal epithelial (HNE) cells can be collected by nasal brush or curettage and expanded for evaluation in 2D planar or 3D spheroid culture. When cultured at air-liquid interface (ALI) conditions on permeable filters, the cells form a pseudostratified respiratory epithelium.
Ideal for the following types of studies:
G551D Observational Study (GOAL) and PROSPECT Prospective Biomarkers Study HNE Cells can be requested through the CF Foundation Biorepository.
HNE cells collected as part of the RARE Study can be requested from the Cystic Fibrosis Foundation Therapeutics Lab by filling out a Material Transfer Request form. The current collection of HNE cells available through the CFFT Lab all have genotypes with two nonsense mutations. This list of mutations available in HNE cells changes regularly based on availability of cells and is not inclusive of all mutations available through the CF Foundation. If you are interested in requesting cells, you can inquire about their availability when filling out the Material Transfer Request form by clicking on “other” and indicating the mutation of interest in the text box.
To request HNE cells collected through the CF Canada-SickKids Program for Individual CF Therapy (CFIT), please contact:
Paul Eckford, PhD
The Hospital for Sick Children (SickKids)email@example.com
Induced pluripotent stem (iPS) cells are stem cells generated from adult cells (non-embryonic) by introducing specific transcription factors that reprogram the cells to become pluripotent. iPS cells do not express CFTR in the undifferentiated state but can be differentiated into different cell types (including those that express CFTR) by using various differentiation protocols.
iPS cells can be requested from the CFFT Lab by filling out a Material Transfer Request form.
To request iPS cells collected through the CF Canada-SickKids Program for Individual CF Therapy (CFIT), please contact:
Paul Eckford, PhD
The Hospital for Sick Children (SickKids)
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