Cystic Fibrosis treatments
Cystic fibrosis (CF) is a multisystem disease that requires a holistic approach to care by a
multidisciplinary team of CF specialist health professionals.
The nurse
A nurse has a lot of tasks when it comes to treating patients with CF. The main tasks
includes advocacy, clinical management, support, advice, education, research and
management (Bush et al. 2006). One of the most important roles, is to take care of not
only the patient, but also the family. Because the disease is inherited, the family can feel
guilt and blame. The nurse can help them with this feeling (Dyer 1997, p. 21). Other
tasks for the nurse are to teach and supervise the patient, so he knows more about his
disease. In that way, the nurse can help him to deal with the disease of cystic fibrosis,
and encourage him to take responsibility. As a nurse you can become a clinical nurse
specialist in cystic fibrosis, and this role is defined by the Royal College of Nursing, who
says:
“The key function of the nurse specialist is to provide direct patient care and
to influence other nurses doing so. The role demands that the specialist be a
resource for colleagues and an advocate for the patient.” (ibid. p. 21)
There are different areas, where the nurse can work. This include diagnosis, inpatient and
outpatient care, care in the community, adolescence and young adulthood, family
planning, transition from pediatric to adult care. In your work as a nurse, there are
overlaps with other disciplines, like physiotherapy, dietetics and psychology. These groups
often work together in a close partnership (Bush et al. 2006).
Physiotherapist
Physical therapy has a big effect in CF: Airway clearance techniques are an important part
of the respiratory management in people with cystic fibrosis (De Boeck et al 2008).
Airway clearance techniques (ACTs) may significantly improve mucociliary clearance and
gas exchange, thereby being of clinical benefit in reducing pulmonary complications in CF
patients. (Pisi & Chetta,2009). Airway clearance therapy should be a routine activity for all
CF patients. (Wagener & Headley,2003). The conventional techniques include postural
drainage and thoracic percussion in different anatomical positions, so as to facilitate the
removal of secretions using the force of gravity. Such techniques include the following:
autogenic drainage, modified autogenic drainage, active cycle of breathing, forced
expiration, positive expiratory pressure using a mask, use of oral oscillatory devices, high
frequency thoracic compressions and intrapulmonary percussive ventilation. The patient
should be guided in the choice of techniques or combinations of techniques and should be
instructed in the correct performance of the maneuvers. The frequency and duration of
the treatment should be individualized. (Dalcin & Silva,2008).
“Aerosol therapy plays an essential role in the treatment of chronically infected CF
airways providing topical delivery of the drug. Correct inhalation techniques in
conjunction with physiotherapy may improve drug deposition thereby optimizing
clinical efficacy” (Prasad et al 2000).
The dietitian
The dietitian must try to ensure a good nutritional status in patients. They offer advice
and support in a number of different areas. The expanded role of the dietitian includes
feeding problems, CF-related diabetes, distal intestinal obstructive syndrome (DIOS) and
other gastrointestinal problems, enteral feeding and pregnancy (Hodson & Margaret,
2007). Because of the pancreas insufficienty maldigestion and malabsorption of food,
especially fats, can be caused. To avoid these problems, dietitians can advice them to
take pancreatic enzyme pills. The main functions of these pills are to digest carbohydrate,
protein and fat, to help with weight gane and to promote nutrient absorption (Maguiness
et al, 2002). Since food is not as well absorbed in patients with CF, the body lacks the
proper amounts of essential nutrients and energy needed for normal growth and
maintenance of weight. That’s why it is important to advice CF patients to have a higher
intake of energy. Also malabsorption of fat-soluble vitamins is common in CF patients. All
patients should receive supplements of the fat-soluble vitamins and annual monitoring of
serum vitamin levels is essential (Korneluk et al, 1996).