Advances in the Management of Cystic Fibrosis

Cystic Fibrosis: Background

Cystic fibrosis (CF) is a hereditary illness that affects the lungs and digestive system (Gibson, Burns, & Ramsey, 2003). Individuals with this illness have an exocrine system dysfunction (essential in production of saliva, sweat, tears and mucus). Affected people produce an abnormally large amount of thick, sticky mucus in their digestive tract, lungs, and airways (Jones, 2008). This mucus may cause a blockage, interfering with the pancreas' digesting function. Mucus in the lungs can attract recurrent infections by trapping bacteria, resulting in gradual damage. Lung failure is the leading cause of mortality in Cystic Fibrosis patients (Cystic Fibrosis Australia, 2017). Currently, the disease has no cure. Therefore, management is mainly palliative to ensure patient comfort and improved quality of life. The intensity of management however depends on the trajectory level of the condition (Ratjen, 2009). In stable phase as in the case of Jordon, management is mainly aimed at preventing exacerbations while maintaining quality life for the patient.

Early Diagnosis and Family History

The fact that Cystic Fibrosis is diagnosed as early as in a newborn is a positive note because management can be started early enough to prevent exacerbations and mortality. The case in reference relates to a four year old female patient who was diagnosed with Cystic Fibrosis as early as seven weeks of age. The family, devastated, received the news only to learn later that a first cousin to the child died at 12 years from the same. This indicates the genetic link but the family was determined to take care of the baby despite the fact that prognosis of Cystic Fibrosis is quite poor.

Management of Cystic Fibrosis

Management of cystic fibrosis requires close monitoring, early and aggressive intervention. Reassurance and patient counseling are also necessary to ensure effective care (Ratjen, 2009). A multidisciplinary team of specialized doctors and nurses come up with both a pharmaceutical and non-pharmaceutical care plan for the individual patient aimed at meeting goals of care which include: Controlling exacerbations, removal of mucus from the lungs, supply of adequate nutrition, prevention and treatment of intestinal blockage, and building exercise tolerance (Mayo Clinic Staff, 2016).

Care Plan for Jordon

While implementing the care plan for Jordon, age should be taken as an important consideration because it has great influence on compliance. A child at some point may not understand the necessity of the several measures in management of cystic fibrosis, for instance limited interactions, therefore the case need be handled delicately with a lot of patience. The strategies and medications used should also be child-friendly. An effective care plan should implemented to include the following:

Drug therapy

Mucolytic agents that lyse mucus strands and helps cough up phlegm thus improving lung function

Bronchodilators to ensure airway patency

Jordon also has a malfunctioning pancreas and thus needs oral pancreatic enzymes to help the digestive tract absorb nutrients

Antibiotics to prevent and treat lung infections thus preventing exacerbations

Anti-inflammatory agents to curb airway inflammation and swelling.

(Mayo Clinic Staff, 2016; Gibson, Burns, & Ramsey, 2003)

Physical therapy

Daily physiotherapy but with less exertion would be suitable for the child to help clear the chest and build inner strength. This should be done delicately while considering the extent the child can handle. A well trained physiotherapist or a nurse can help achieve this by portraying it as a fun activity to the little patient. Family members could join in at times to give moral support (Mayo Clinic Staff, 2016; Ratjen, 2009).

Dietary requirements

A high calorie nutritious diet, low in animal fat alongside salt and vitamin supplements is a great recommendation for a cystic fibrosis patient. Fresh fruits and vegetables are also necessary to help boost immunity as well as aid digestion by providing dietary fiber (Ratjen, 2009)

Pulmonary rehabilitation

This entails a long-term program aimed at improving lung function and overall wellness. Strategies to achieve this include aerosol mist inhalations to clear airways, physical exercise, nutritional support and avoidance of allergens and any other factors that may trigger worsening of the condition (Mayo Clinic Staff, 2016). For instance, as stated by Jordon’s sister in the video, the family protects her from any member with an infection, even flu.

Vest therapy

For a childlike Jordon in whom physical therapy to clear lungs may not be so easy to achieve, this strategy may be quite useful. A mechanical device in form of a vibrating vest worn by the child could help loosen mucus in the lungs and even decrease need for frequent doses of mucolytic agents (Mayo Clinic Staff, 2016).

Challenges in Managing Cystic Fibrosis in Children

Unlike dealing with adults, a healthcare provider managing a child with cystic fibrosis may experience quite an array of challenges because a child has more delicate and special needs than an adult does. Therefore, as a palliative caregiver of any kind to a child one should be ready to embrace and deal with the challenges patiently (Centers for Disease Control and Prevention, 2015). The physiological challenges in children with Cystic Fibrosis start early in life. Lack of appropriate enzymes for digestion may interfere with absorption of essential nutrients necessary for healthy growth and development. Retarded growth, malnutrition, diarrhea, constipation, foul smelling stools and inability to maintain a healthy weight are some of the challenges a healthcare giver, family and professional counselors may come across. Other physiological challenges include side effects from long-term use of several medications, decreased mobility and lung capacity with time, and progressive debilitation (Storlie & Baltrinic, 2017)

Psychosocial challenges come about as depression and hopelessness associated with the chronic illness and the intense and demanding management strategies. Children may feel alienated as caregivers try to shield them from so much exposure and this may interfere with psychosocial development and family system functioning. To effectively deal with these issues, professional counselors need to be well updated on mental health research related to this special group of patients as well as their family. They need to be well versed in handling conditions such as negative body image, anxiety, stress and internalization of behaviors especially with female children (Storlie & Baltrinic, 2017)

Social-Ecological Model for Cystic Fibrosis

This is a system that gives a summary of the bands of influence involved in management of cystic fibrosis at different levels. It serves as a guide to ensure all parties affected fulfill their roles to ensure a smooth flow of affairs. Implementation of the health management strategies at each level ensures synergism of efforts thus maximizing outcomes (Centers for Disease Control and Prevention, 2015).

Individual Level

The innermost band represents this level and it relates to the person affected by the disease. In this case, Jordon is the individual of interest. This level serves to enhance the patient’s knowledge of their condition, to influence their attitude and outlines the role they need to play to ensure management of their condition runs smoothly (Centers for Disease Control and Prevention, 2015). In our case, it is necessary to explain to the patient, however young they may be, the importance of all the medications, diet, the exercise and even routine screening. The benefits of all the strategies, the risks associated with lack of such as well as importance of compliance are also key factors to mention. It is important that the child is shown their importance in the entire journey and encouraged as much as possible to comply and stay positive.

Interpersonal Level

Activities at this level facilitate individual behavior by enhancing social and interpersonal relationships. This is where the role of family, friends, community health providers, and health workers in management of cystic fibrosis is outlined. Interventions relevant to this level include moral, emotional, and financial support from family, reminders of taking medications, nutritional support, providing company during hospital visits, and reassurance (Centers for Disease Control and Prevention, 2015).

Organizational Level

The activities aimed at promoting management of cystic fibrosis are implemented at the organizational level. Employers, healthcare plans and systems, local health departments, and professional organizations are the key providers of support at this level. Organizations such as the Cystic Fibrosis (CF) Foundation are responsible for refining the quality of life for cystic fibrosis patients by supporting innovative research and comprehensive care (Lubsch, 2007). Activities at this level aimed towards the promotion of healthy living among Cystic Fibrosis patients include promotion of screening and preventive procedures, assessment of patient progress, patient education, and development of policies and regulations that govern prevention and management.

Community Level

Activities at this level are aimed towards enhancing individual behavior change by pooling resources and participation of community-based institutions such as the media, community advocacy groups, tribal health departments, and community health movements. Improved survival for cystic fibrosis patients has been shown to increase over the past four decades because of cooperation between Cystic Fibrosis nurses and community health-care professionals in support of patients and their caregivers (Cottrell & Burrows, 2009). Useful interventions at this level include the creation of awareness, expansion of resources to manage cystic fibrosis especially for the less privileged patients, collaboration with health organizations in campaigns and education, and grants and donations (Centers for Disease Control and Prevention, 2015).

Policy Level

This is the widest band and it represents the highest level of interventions useful in the management of Cystic Fibrosis. At this level, decisions are made, that in one way or another influence the kind of interventions to be undertaken at lower levels. This level involves big authorities and agencies such as the federal state, government and non-governmental agencies, tribal government agencies as well as some international agencies such as the World Health Organization (WHO) and the Cystic Fibrosis Foundation. These agencies create policies and regulatory strategies that help create a harmonized and standardized way of handling Cystic Fibrosis in addition to other health conditions. The guidelines provided by such agents may also outline management strategies and handling of complications in some special populations such as pediatrics, adults, geriatrics, pregnant women, and any vulnerable population. Relevant interventions that could be adopted at this level include making policies regarding effective management of CF, communicating policies to the public, translating local policies to the community, and funding research and preventive programs e.g. screening.

References

Centers for Disease Control and Prevention. (2015, October 27). Social-Ecological Model. Retrieved April 4, 2017, from CDC: https://www.cdc.gov/cancer/crccp/sem.htm

Cottrell, J., & Burrows, E. (2009). Community-based care in cystic fibrosis: role of the cystic fibrosis nurse specialist and implications for patients and families. Disability and Rehabilitation, 254-261.

Cystic Fibrosis Australia. (2017). About Cystic Fibrosis. Retrieved April 4, 2017, from Cystic Fibrosis Australia: http://www.cysticfibrosis.org.au/all/learn/

Gibson, R. L., Burns, J. L., & Ramsey, B. W. (2003). Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis. American Journal of Respiratory and Critical Care Medicine, 168, 918-951.

Jones, A. C. (2008). Ototoxicity monitoring of Adult Patients with Cystic Fibrosis. San Diego: San Diego State University.

Lubsch, L. (2007, July 13). Advances in the Management of Cystic Fibrosis. U.S Pharmacist.

Mayo Clinic Staff. (2016, October 13). Cystic Fibrosis. Mayo Clinic.

Ratjen, F. A. (2009). Cystic Fibrosis: Pathogenesis and Future Treatment Strategies. Respiratory Care, 54(5), 595-605.

Storlie, C. A., & Baltrinic, E. R. (2017). Counselling Children With Cystic Fibrosis: Recommendations for Practice and Counselor Self-Care. The Professional Counselor.