Quittner, A. L., Saez-Flores, E., & Barton, J. D. (2016). The psychological burden of cystic fibrosis. Current Opinion in Pulmonary Medicine, 22(2), 187–191. https://doi.org/10.1097/mcp.0000000000000244

Cystic fibrosis is an inherited disorder that affects cells that produce mucus, sweat and digestive juices. In affected individuals, these secretions are much thicker than they should be, causing blockages in passageways of the body.

Cystic Fibrosis

Symptoms vary between affected individuals, and can change over a person's lifetime. Those with milder symptoms are sometimes not diagnosed until teenage or adulthood, and commonly experience atypical symptoms.

Cystic Fibrosis Symptoms

Cystic fibrosis is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resulting protein is not able to regulate the movement of salt in and out of cells as well, causing thick mucus in the respiratory, digestive, and reproductive symptoms, and increased salt levels in sweat. The different ways a mutation can develop will affect the severity of the symptoms that present. Children need to inherit the mutation from both parents in order to have cystic fibrosis; if they only inherit from one parent, they will be asymptomatic carriers.

Cystic Fibrosis Causes

Cystic fibrosis has no cure, but can be treated to manage the effects of presenting symptoms. Medications can be used to thin mucus and control respiratory and digestive symptoms. Newer medications can target the mutated genes, allowing for better performance of inhibited proteins, lessening the severity of symptoms. Other treatments include airway clearance techniques, pulmonary rehabilitation, and certain surgeries.

Cystic Fibrosis Treatment

Vaeth, S. V., & Martins, L. M. (n.d.). Teens, cystic fibrosis and emotional health. The Cystic Fibrosis Center at Stanford. https://med.stanford.edu/cfcenter/teens/CysticFibrosisandEmotionalHealth_000.html

reference for cystic fibrosis and teen mental health

reference for the psychological burden of cystic fibrosis

Often times, treatments for adults may not be as efficient as treatments for adolescents and vice versa. As such, the lived experiences of patients of different age demographics with the same chronic condition can vary widely. So, the need to examine the realities and burdens faced by both groups separately is important in understanding the overall psychological burden of cystic fibrosis.

Learn Before

Related