Cystic Fibrosis and its Effects on Quality of Life

One of the most challenging symptoms of having cystic fibrosis CF is labored breathing because of mucus obstruction and opportunistic infections. CF has been characterized as a multisystem genetic disease that ranges in severity and affects the quality of life (QOL) in children and adolescents (Kianifar, Bakhshoodeh, Hebrani, & Behdani, 2013). In addition to lung function, it affects the reproductive organs and the pancreas, which can lead to CF- related diabetes (Kianifar et al., 2013). In addition to these biological challenges, older adolescents and adults have psychological burdens, such as concern about obtaining and maintaining a job, having a normal family life, and life expectancy (Kianifar et al., 2013).

CF is the cause of significant emotional stress on patients and families (Hegarty, MacDonald, Watter, and Wilson, 2008). Daily health maintenance routines are time consuming, but necessary, and can interfere with maintaining a normal life. Treatments with medications, nutritional demands, and sufficient exercise can create demanding lifestyles, although such a lifestyle is necessary for health maintenance (Tibosch et al., 2011). There seems to be a paucity of research on the emotional QOL of children, and there is some disagreement between parents' perceptions and the perception of the child with CF, with parents rating emotional QOL lower than the children rate their own emotional QOL.

In Laureate Education, Inc. (2012), Chelsey found it daunting to be told she had to be accountable for a number of tasks related to her CF. In addition, children and especially adolescents, face the challenges of self-management, identity development, intimacy, and family planning (Tibosch et al., 2011). Additionally, because of the increased awareness of the effects of infection, segregating children and adolescents with CF in schools has the potential to exacerbate stigma and social isolation (Quittner, Barker, Marciel, & Grimley, 2009), which may be well-developed in the CF population because of unusual and time-consuming daily routines (Tibosch et al., 2011).

                                            Two Approaches to Enhance QOL

Family dynamics play an important role in the lives of children, and meal time is an experience wherein family members have the opportunity to interact and communicate (Janicke, Mitchell, Quittner, Piazza-Waggoner, & Stark, 2008). Positive interactions at the dinner table have the potential to promote emotional health and facilitate a positive environment for eating and digestion (Janicke et al., 2008). These health factors are more important in families of children with CF, since they need to eat more than the average child. One such intervention, Be In Charge, encourages giving praise to appropriate behaviors and not giving attention to the disruptive or inappropriate behaviors (Janicke et al., 2008). In addition, the approach fosters setting goals and rewarding children for good work. This behavioral intervention teaches parents to be consistent with rules and expectations and fosters healthy family interactions at the dinner table. Further, this program improved adherence to special diets and was successful in increased weight gain (Janicke et al., 2008). Weight gain is a result of dietary adherence, which is an important part of the overall management of CF (Janicke, et al., 2008; Stark et al., 2003). Overall management of the disease would increase QOL.

A second approach is a psychological intervention that utilizes social support to relieve maternal anxiety (Drotar, 2006). Pairing mothers of children with CF, diabetes, and asthma not only helped the parents cope, but it helped the children as well. Creating a family-to-family support network relieved stress and anxiety in mothers, and reduced anxiety and depression in the children. There is a paucity of research on this approach with families with CF, however, and further research is warranted (Drotar, 2006).

To maintain a good quality of life in children and adolescents with CF, families need to work with a team that includes pediatricians, pediatric psychologists, and social service workers. Although this approach is often ignored, it has been associated with positive health outcomes for children and adolescents with CF (Kianifar et al., 2013).

References

Drotar, D. (2006). Psychological Interventions: Cystic Fibrosis. In , Psychological interventions in childhood chronic illness (pp. 203-219). American Psychological Association. doi:10.1037/11412-010

Quittner, A. L., Barker, D. H., Marciel, K. K., & Grimley, M. E. (2009). Cystic fibrosis: A model for drug discovery and patient care. In M. C. Roberts & R. G. Steele (Eds.), Handbook of pediatric p sychology (4th ed., pp. 271–286). New York, NY: Guilford.

Hegarty, M., MacDonald, J., Watter, P., & Wilson, C. (2009). Quality of life in young people with cystic fibrosis: effects of hospitalization, age and gender, and differences in parent/child perceptions. Child: Care, Health & Development, 35(4), 462-468. doi:10.1111/j.1365-2214.2008.00900.x

Janicke, D. M., Mitchell, M. J., Quittner, A. L., Piazza-Waggoner, C., & Stark, L. (2008). The impact of behavioral intervention on family interactions at mealtime in pediatric cystic fibrosis. Children’s Health Care, 37(1), 49–66.

Kianifar, H., Bakhshoodeh, B., Hebrani, P., & Behdani, F. (2013). Quality of Life in Cystic Fibrosis Children. Iranian Journal Of Pediatrics, 23(2), 149-153.

Laureate Education, Inc. (Executive Producer). (2012). Child and adolescent health: Living with cystic fibrosis. Baltimore, MD: Author.

Stark, L. J., Bowen, A. M., Tyc, V. L., Evans, S., & Passero, M. A. (1990). A behavioral approach to increasing calorie consumption in children with cystic fibrosis. Journal of Pediatric Psychology, 15(3), 309–326.

Tibosch, M., Sintnicolaas, C., Peters, J., Merkus, P., Yntema, J., Verhaak, C., & Vercoulen, J. (2011). How about your peers? Cystic fibrosis questionnaire data from healthy children and adolescents. BMC Pediatrics, 1186. doi:10.1186/1471-2431-11-86