CYSTIC FIBROSIS

Cystic Fibrosis



Cystic Fibrosis

Introduction

Cystic fibrosis (CF) is a genetically transmitted disease that affects 1 in 3,000 children born in the United States every year. Children are typically diagnosed within the first two years of life and have a mean life expectancy of 32.2 years (Stark, Mackner, Patton, & Acton, 2003). The disease affects the exocrine glands of several major organs, such as the lungs, liver, and pancreas, as well as in the skin. Due to thick mucus production, the patient is susceptible to lung infections and chronic progressive pulmonary disease. Further, due to pancreatic insufficiency, patients with CF suffer from nutritional deficits and poor growth as a result of their inability to absorb nutrients (Hankard, Munck, & Navarro, 2002).

Background

As a result of recent advances in treatment therapies (e.g., antibiotics, vest airway clearance), the mean life expectancy of CF patients has increased dramatically by about 6 years over the past two decades. Individuals with CF can now expect to live into adulthood.

However, CF remains one of the most challenging pediatric illnesses for families to manage due to the rigorous demands of CF treatments and nutritional requirements. Patients are required to complete daily chest physiotherapy to loosen mucus from the lungs, which often requires wearing a high frequency compression vest several times a day. Families of children with CF must balance the daily respiratory therapies to clear the child's airways with the need for their child to eat a diet high in protein and fat (Mitchell, Powers, Byars, Dickstein, & Stark, 2004). Research suggests that it can take several hours daily for the child and family to comply fully with the complex CF regimen (Quittner, Espelage, Ievers-Landis, & Drotar, 2000). As a child moves into adolescence, treatment adherence declines regardless of the specific chronic illness (Rapoff, 1999). Daily illness management is often complicated by the adolescent's desire to become autonomous and assume more of the disease management responsibilities that were previously delegated to parents. Children and adolescents who do not comply with their treatment regimen are likely to suffer from symptom exacerbation and experience a decline in overall health and psychosocial functioning. Treatment demands can also leave the family feeling overwhelmed and burdened by the child's disease, prompting a decline in the overall functioning of the family. On the other hand, the reverse scenario may also be true. Tension and conflict in the family may lead to a decline in adherence behaviors, causing a worsening of symptoms and, in the case of CF, a progression in lung disease.

CF treatment adherence

Evidence suggests that across a range of diseases, children with a chronic illness adhere to their treatment regimens 50% of the time (Rapoff, 1999), with estimates suggesting adherence in CF is even lower (Modi & Quittner, 2006; Quittner et al., 2000). As CF is a progressive disease, noncompliance to treatment protocols can severely jeopardize a child's health and prognosis (Stark et al., 2003). Lung infection, malnutrition, osteoporosis, diabetes, and liver disease are all possible complications of ...