Cystic Fibrosis (CF)


Cystic fibrosis (CF) is a genetic condition that affects multiple organs.1 Education on this disease involves awareness not only by the patient, but also of their caregiver(s), on several topics, including lung health, prevention of infections, and nutrition.2 Patients and caregivers may find information through a variety of resources. For younger patients, parents, and caregivers, medical staff and medical literature are primary sources of information. Information available on the internet is also an important resource, representing 35% to 73% of the resources consulted by patients.2

Lung Health and Infection Prevention in Cystic Fibrosis

The abnormal mucus produced by the body in patients with CF accumulates within the airways, causing difficulty breathing and frequent infections.1 There are several measures that patients may follow to implement and improve infection prevention. These recommendations are described in a group of infection prevention and control clinical care guidelines, which are delineated by an interdisciplinary team that reviews and evaluates available evidence for each recommendation.3 Measures to prevent infections include social distancing from other patients with CF to reduce the risk of transmission of different CF pathogens and washing the hands regularly with alcohol-based hand sanitizer or water and antimicrobial soap to reduce potential contamination.4 The Cystic Fibrosis Foundation is a donor-funded, nonprofit foundation focused in funding CF research and providing support and care to patients with CF and their caregivers.5 The organization provides an Infection Prevention and Control (IPC) Passport Card with educational information directed at care providers that can be printed and carried by patients with CF.6 Patients may also find resources on the same website regarding information about lung transplantation.7

Nutrition in Cystic Fibrosis

Treating CF requires an optimization of the patient’s nutritional status, as patients with CF have different requirements than other people. Adding protein and calories to the diet should be performed under the supervision of a healthcare provider since the patient’s nutritional needs may vary during the disease progress.8,9 A balanced diet that often includes pancreatic enzymes is therefore crucial for CF management. When these enzymes are prescribed, their ingestion should occur as instructed by the dietitian, with meals and snacks. Other supplements for increasing levels of vitamins A, D, E, and K may be required and prescribed.8

Caring for Someone With Cystic Fibrosis and Mental Health

Caring for a patient with CF can be stressful and emotional, as responsibilities such as managing drug administration or aiding in airway clearance techniques build up. Parents of a child with CF may end up neglecting their own care and nurture states of anxiety or depression, which may ultimately impact the care given. Caregivers who experience signs such as difficulty sleeping, headaches, anxiety, frustration, or sadness are encouraged to talk to a primary care provider or the CF care team. Talking to other parents living with similar feelings may also help reduce stress. Establishing a daily routine for respiratory treatments and nutrition can help manage time and decrease feelings of anxiety.10 A guide for parents and caregivers to help them cope with the disease is available through the Cystic Fibrosis Foundation website.11

Reproduction in Cystic Fibrosis

In addition to affecting both the respiratory and digestive systems, CF can impact the reproductive capability for some women. Most men with CF are affected due to the blockage or absence of the vas deferens. The use of assisted reproductive technology (ART) can help these patients have biological children. ART includes in vitro fertilization (IVF) and intrauterine insemination (IUI). Using donor sperm, eggs, or embryos are also options to consider that can be discussed with the care team.12

Support Network

Several organizations provide information on CF and potentially related conditions, the treatments available, and how to live with the disease. The Cystic Fibrosis Foundation provides complete information and several resources to guide patients and caregivers in understanding and managing the disease.5 These materials may be accessed and requested through a CF Center Portal or by email.6 The foundation also provides educational videos on infection prevention, nutrition, and mental health through a YouTube channel.6 Other organizations providing educational resources include the Cystic Fibrosis Research Institute,13 the Lung Transplant Foundation,14 the Cystic Fibrosis Trust,15 Cystic Fibrosis Worldwide,16 the Genetic and Rare Disease (GARD) Information Center,17 and the American Lung Association.18

References

1. Cystic fibrosis. National Organization for Rare Disorders (NORD). Accessed January 20, 2022.

2. Lonabaugh KP, O’Neal KS, McIntosh H, Condren M. Cystic fibrosis-related education: are we meeting patient and caregiver expectations? Patient Educ Couns. 2018;101(10):1865-1870. doi:10.1016/j.pec.2018.06.004

3. Infection prevention and control clinical care guidelines. Cystic Fibrosis Foundation. Accessed January 20, 2022.

4. What is IPC? Infection prevention and control guidelines. Cystic Fibrosis Foundation. Accessed January 20, 2022.

5. Our mission. Cystic Fibrosis Foundation. Accessed January 20, 2022.

6. Patient education materials available. Cystic Fibrosis Foundation. Accessed January 20, 2022.

7. The journey through lung transplant. Cystic Fibrosis Foundation. Accessed January 20, 2022.

8. Cystic fibrosis – nutrition. MedlinePlus. Updated January 12, 2022. Accessed January 20, 2022.

9. Hollander FM, de Roos NM, Heijerman HGM. The optimal approach to nutrition and cystic fibrosis: latest evidence and recommendations. Curr Opin Pulm Med. 2017;23(6):556-561. doi:10.1097/MCP.0000000000000430

10. Coping while caring for someone with cystic fibrosis. Cystic Fibrosis Foundation. Accessed January 20, 2022.

11. What about siblings? Helping my family cope: a guide for parents and caregivers. Cystic Fibrosis Foundation. Accessed January 20, 2022.

12. Assisted reproduction and CF. Cystic Fibrosis Foundation. Accessed January 20, 2022.

13. About us. Cystic Fibrosis Research Institute. Accessed January 20, 2022.

14. Our purpose. Lung Transplant Foundation. Accessed January 20, 2022.

15. About us. Cystic Fibrosis Trust. Accessed January 20, 2022.

16. About us. Cystic Fibrosis Worldwide. Accessed 20 January, 2022

17. About GARD. Genetic and Rare Disease Information Center. Accessed January 20, 2022.

18. About us. American Lung Association. Accessed January 20, 2022.

Reviewed by Kyle Habet, MD, on 1/25/2022.