Life-Threatening Disease: Understanding Cystic Fibrosis (CF) - Research Paper

Introduction

Cystic Fibrosis (CF) is a disease that affects the lungs and the digestive system. It is a single disease that is characterized by a wide range of symptoms such as tissue distribution. The affected person experiences difficulties in breathing as time progresses (Chuang et al., 2014). According to Tiddens and Rosenow (2014), a patient with CF experiences chronic infection in the lungs at an early age. The disease is hereditary and can be life-threatening. The genetic disease occurs to people whose parents have defective genes. In the white population, the disease has been found to be a lethal and common genetic disease. Due to its high prevalence and complications, individuals suffering from the disease possess a short-lived lifespan. A child suffering from CF develops respiratory problems linked to pulmonary infections, both of which requires diagnosis and examination using radiations such as Computed Tomography (CT), MRI and x-ray among others which can be dangerous to them. Despite advanced technology having brought new assessment methods, use of radiation affects children compared to ultrasound which is non-ionizing and has less associated dangerous effects on children.

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Radiation That Affects Children

Major deaths of individuals suffering from CF occur due to respiratory degradation associated with pulmonary infections (Munck et al., 2015). Due to the presence of pulmonary infections, CF patients require to undergo frequent antibiotic treatments to reduce its prevalence. Also, due to the infection that the disease causes to the lungs, the child is exposed to CT and ultrasound among other advanced technologies. Earlier disease detection has been found to reduce disease prevalence over the years. In addition, the improved and better understanding of the disease has necessitated better management and new approaches to treatment. Also, therapeutic advances have led to longer life expectancy. An example of such suitable technology is the CT which according to Leung et al. (2015), is a very significant imaging tool reliably used to identify lung disease at an early stage hence leading to an increase in the survival rate of an individual, albeit, the dose has to be administered in a low dose. It is expected that with advances in technology, life expectancy will continue to increase further.

Some challenges have been encountered as a result of technological tools used in the detection of the various diseases for patients with CF (Leung et al., 2015). For instance; patients with CF encounter limitations during the identification and classification of liver disease. According to Leung et al. (2015), the limitation is as a result of unreliable sensitive and specific diagnostic markers of liver involvement in CF. In another study, the use of CT in CF children was associated with irreversible structural lung damage that reduces the lifespan of the patient (Triantopoulou & Tsapaki, 2017). CF is associated with high radiation exposure and this can further reduce the lifespan of a patient. Moreover, children with the highest exposure levels of CT examinations are more susceptible to develop malignancies. This makes it difficult to induce low dose levels of CT since the malignancies have to be reduced through continuous high cumulative doses. The high doses of CT have been found to be linked to higher levels of cancer-mortality risk and incidence (Triantopoulou & Tsapaki, 2017). In this regard, exposing a child to a CT radiation of about 518 mSv increases the risk of cancer prevalence by five times compared to the children who have not been exposed to radiation. Also, exposure to CT leads to irreversible damage known as bronchiectasis. Tiddens and Rosenow (2014) further stated that even after a CF patient undergoes the present current treatment, they will portray progressive bronchiectasis and mild airways disease. Despite the various problems experienced in the identification of diseases, ultrasound has been suggested for use in patients with CF.

Ultrasound for Pediatric Cystic Fibrosis

Techniques such as CT have been found to have serious implications on the CF patients. There is hence the need to identify and use better techniques that will have less or no impeding severe effects on patients especially children. Ultrasound is a better technique in the diagnosis and assessment of lung disease, heart problems and pneumonia infections in CF patients. According to Mueller-Abt et al. (2008), ultrasound is a technique that is widely used in the evaluation of liver diseases. The technique is suitable especially for children because it is non-ionizing and therefore, does not expose children to severe effects of ionizing radiations (McLario & Sivitz, 2015). A CF patient can be exposed to ultrasound during diagnoses of other diseases such as lung infections and pneumonia. The lung ultrasound diagnostic technique is specifically a feasible point of care device that facilitates the assessment of the lung (Yadav, Awasthi and Parihar, 2017). Ultrasound technique has recently gained attention from the paediatricians for critical decision making and procedural processes (McLario & Sivitz, 2015). This is because the technique is feasible, non-ionizing and highly preferred for use in fetus and children. For a fetus with CF, ultrasound can be used for easy detection of the condition even at an early stage of 20 weeks (Terlizzi, Sciarrone, Cook, Botta & Chiappa, 2016). In their study, McLario and Sivitz (2015) found that ultrasound allows sound-wave penetration and image resolution making it suitable for children whose small internal organs are inaccessible. The technique allows for detection of the organs that most of the techniques cannot detect. In their study, Mueller-Abt et al(2008) were able to evaluate the echotexture and contour of the entire liver and compared it to the echogenicity of the kidney.

Imperatively, other uses and applications of ultrasound include detecting the contributing factors to CF, assessing the severity of CF such as pulmonary thickening and B lines, predicts the adverse outcomes such as early ARDS, response to treatment and for comparison of pulmonary function test with ultrasound assessment. McLario and Sivitz (2015) posit that ultrasound is ideal for assessing the presence of free fluid present in the peritoneal cavity. Moreover, it can be used in the evaluation of the heart and hence making it possible to understand the contractility and pericardial effusion.

According to McLario and Sivitz (2015), ultrasound is an effective technique that has found use in the visualization of the infections taking place in the soft tissue of the skin. With ultrasound, it is possible to detect the cellulitis. It has been established that when used to distinguish the abscess from cellulitis, point-of-care ultrasound has a sensitivity ranging from 90% to 97% and a specificity of between 69% and 83% (McLario and Sivitz, 2015). When compared to the clinical suspicion whose specificity and sensitivity range from 66% to 80% and from 75% to 78% respectively, point-of-care ultrasound is to a large extent better technique (McLario and Sivitz, 2015).

Ultrasound performed at the musculoskeletal section allows the visualization of the cortical discontinuity of long bone fractures (McLario and Sivitz, 2015). This visualization allows evaluation, diagnosis and early immobilization prior to conventional radiography. As stipulated by Yadav, Awasthi and Parihar (2017), the technique achieves the assessment with much higher sensitivity, accuracy, repeatability and specificity. Better assessment of small body tissues can be achieved by combining ultrasound with MRI, however, MRI has ionizing radiation that makes the combination unsuitable. As a result of the various significances, applications and benefits of ultrasound, the technique needs to be taught to the nurses so that they can perform the scans. In this way, the nurses will be able to detect the lung diseases and any other complication early in fetuses and children

Conclusion

The radiation techniques such as the use of MRI, CT, X-Ray and among others, have been found to have adverse effects on children with CF. In addition, their sensitivity and specificity in assessing some small parts are low. Conversely, ultrasound is a highly sensitive and specific technique for the assessment of the pediatric cystic fibrosis. The technique helps the paediatricians to establish the CF's contributing factors, severity, response to treatment and in predicting the adverse outcome. Also, ultrasound is useful in the detection of the infections at an early stage and accordingly necessitates earlier treatment. Some of the minute problems that could not be detected can now be detected using the technique and a good example is the visualization of the soft tissue and the impending infection. The many benefits of ultrasound compared to other techniques such as CT has led to the reduction in mortality and morbidity rate of CF children.

References

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