Physiotherapy management of newly diagnosed Cystic fibrosis (CF)

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Introduction

The diagnosis of cystic fibrosis has been enhanced in recent times. Hunt & Geddes (1985) noted that, in the past, many children succumbed to the condition in less than a year after birth. In addition, very few victims lived for over a decade after diagnosis.

However, great strides have been achieved in the management of the condition and over three quarters of the affected victims can now live up to adulthood with the condition. In recent times, treatment of cystic fibrosis has been advanced where both antibiotics and physiotherapy treatment are used to manage the condition.

Maffessanti, Candusso, Brizzi, & Piovesana (1996) noted that the diagnosis of cystic fibrosis is common among children with very few cases reported among those in middle and late adulthood.

This paper discusses the issues that one would need to consider in the physiotherapy management of the patient newly diagnosed with cystic fibrosis (CF).

According to Di Sant’Agnese & Davis (1976), individuals diagnosed with Cystic fibrosis (CF) should have physiotherapy procedures as part of their treatment regimen. The procedures used, and the treatment program should be specific to each person.

Stern (1997) noted that the procedures should encompass the airway clearance techniques, exercise, and sustenance of the thoracic and general mobility. Airway clearance techniques focus on reducing the blockage of the airway and enhancing ventilation.

This is meant to postpone the progression of the disease. Exercise strives to increase cardiovascular and respiratory capability and strength within the lower and upper muscle areas. This improves endurance, functioning of the lungs, and general health.

The maintenance of the general and thoracic mobility and body posture use certain stretching and strengthening routines that maintain or re-establish healthy body posture.

They avert or treat an inflexible thoracic spine, as well as the costovertebral joints. Thus, the exercise facilitates efficient, painless rib excursion.

The implications of cystic fibrosis (CF) for patients and their careers

Cystic fibrosis (CF) is a complex condition that is challenging to manage. The therapeutic schedule is tedious and delicate for most patients. In addition, the therapeutic program is quite expensive and may strain the patient’s and/or family resources.

Baumann, et al (2003) noted that the cost of the management rises with the age of the patient. In some countries, segregation is taken as a measure of preventing infection.

This has a devastating impact on the social welfare of the patients. The segregation measures are most common among CF centers in some European countries.

Though studies have revealed comparable educational levels between those suffering from CF and the general population, it has to be noted that CF patients experience delay in their education. Studies have also indicated that CF patients can be employed.

However, according to Burker, Sedway & Carone (2004), the patients’ condition seems to play a great part in influencing their careers. Another effect of CF is on the fertility of the adult patients. Male patients are normally infertile.

This is due to the lack of vas deference in their genitalia. On the other hand, female patients may be fertile. However, they can expose themselves to great risks if they get pregnant.

The age of the patient at diagnosis

Diagnosis of CF has been advanced in modern days. According to Lannefors, Button & McIlwaine (2004), most countries have embraced the screening of newborn infants. This has made it possible to diagnose CF in children within the first two months of birth.

Initially, it was difficult to diagnose CF among the children. In this case, the diagnosis was based on symptoms that appeared when the victim was much older.

During the initial stages, CF is asymptomatic and thus there are no signs of this condition. Symptoms begin to show after several months or year of birth.

Tepper (1998) observed that CF is commonly diagnosed among infants and toddlers than in adults. However, this does not indicate that CF is unheard of in adults. As denoted by Hunt & Geddes (1985), there are isolated cases in which adults have been diagnosed with the condition.

In addition, Gershman, Mehta, Infeld & Budev (2006) noted that CF cannot be considered solely as a pediatric disease. The Cystic Fibrosis Foundation (2003) noted that close to 80 percent of the CF patients are able to attain adult age when the condition is properly managed.

Indeed, Yankaskas, Marshall, Sufian, Simon & Rodman (2004) observed that those patients who were born with the condition starting from the 1990s are set to live for an average of forty years. This has been attributed to the enhanced management regimens in the modern world.

The role of the respiratory physiotherapist as part of a holistic, MDT approach to treatment management

People should seek the services of a physiotherapist immediately after having been diagnosed with cystic fibrosis. The patients should start training in the anatomy and physiological treatment techniques, as well as the relevant regime.

Stern (1997) observed that patients should be taught how to detect an increase in respiratory symptoms, when to increase the incidence, and duration of treatment and the appropriate time to seek further medical advice.

According to Kulich, Rosenfeld, Goss & Wilmott (2003), education and training in cystic fibrosis and its treatment should be a continuous process of care. In addition, it should form a section of each review. The quantity of information communicated at this stage is determined by each family’s needs.

Written information should be cross checked accordingly. It is essential to introduce early dialogue about the exercise being an integral part of the patient’s lifestyle.

Peebles (2005) observed that, the family should be encouraged to think and develop activities that allow all members to participate. Children from highly active families have a likelihood of participating in sports more than any other activity.

As denoted by Bush (2006), it is essential for parents and the entire family of the patient to be actively involved in the management program of the patient at an early stage to integrate physiotherapy into their daily routine.

During the initial period, it is essential for the physiotherapist to build a strong rapport with the family. This will enable the physiotherapist to reassure, encourage, and obtain a clear understanding of how each family member copes with information about the diagnosis and the potential burden of caring for the patient.

Substantial levels of rapport are fundamental in achieving an acceptable and efficient treatment strategy.

According to Davis (2006) and Doull (2001), all patients should be evaluated and treated by a qualified physiotherapist within the first 24 hours after admission. This treatment regime should be sustained at an appropriate level with constant reassessment of the patient’s progress.

Whenever feasible, the patient’s family should not be left to conduct treatment on their own. When a patient is hospitalized, it should serve as an opportunity to relieve the family from the burden of care.

Horsley, Cunningham, & Innes (2010) observed that, clearing of lung fields is the cornerstone of physiotherapy management procedures for a patient diagnosed with cystic fibrosis.

The main causes of mortality and morbidity are known to be bronchiectasis and obstructive pulmonary illness. However, the latter accounts for a considerable share of all known deaths.

The interrelationships between those involved

According to Henley & Hill (1990) cystic fibrosis is a condition that requires team management. In this case, the physiotherapist, the patient, and the family members have to participate in the management program.

Essentially, the disease management team may include a dietician, a physician, a physiotherapist, a CF coordinator, a community nurse, a genetic counselor, and a CF pharmacist. Members of the disease management team are critical as each has a vital role to play.

This enables the patient to live as comfortable as possible. According to Jaffé & Bush (2001), the family members of the patient should be summoned to the hospital immediately after the patient has been diagnosed with the condition.

Hough (2001) and West (2011) acknowledged that, this is done to ensure the physiotherapist discusses the implications of the condition with the relevant stakeholders.

In most cases, such discussions are overseen by the respiratory physicians who will manage the patient’s condition. The physician may be assisted by a cystic fibrosis coordinator and a counselor. A respiratory fellow of registrar might also be present.

Notably, Horsley, Cunningham & Innes (2010) noted that cystic fibrosis is a complicated disorder and its diagnosis can be a big blow to the families of the newly diagnosed patients. Therefore, it is important for a thorough education program to be initiated immediately a patient is diagnosed with this condition.

Given that the diagnosis may have a strong emotional effect on the family members, they may not be able to comprehend the new information on how to manage the disease.

According to Ranganathan (2006), the delivery of information should be a repetitive and continuous process to enhance comprehension. In addition, the aspect of staying positive and acknowledging the gravity of the condition should be emphasized.

Conclusion

It can be acknowledged that cystic fibrosis is an extremely complex disease. The condition is common among children. However, isolated incidences of adult being diagnosed with the disease have been noted.

Nonetheless, with the enhanced technologies in the modern world coupled with the adoption of the policy requiring the screening of the newborns, CF is diagnosed early among children. In fact, it has been noted that the condition can be diagnosed within the first two months of birth.

The management of the condition can be tedious and time consuming. However, with determination and commitment from the patient and team members, the condition can be managed successfully. Individuals who have been diagnosed with the disease can live up to an average of forty years.

It is extremely essential for the physiotherapist to establish a strong rapport with the patient and the patient’s family. This enhances effective communication about the diagnosis and the subsequent treatment regimen.

All members of a family should help the patient recover by providing all the necessary support. In essence, the therapy management team is critical to effective disease management.

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