Cystic Fibrosis in the Modern

Cystic Fibrosis in the Modern Era disease that steals a childhood, adolescence and adulthood in an assault on the lungs and other vital organs that cause every living action to revolve around the diagnosis, care, and treatment of the condition; it is Cystic Fibrosis (CF). In the United Kingdom, CF has been identified by the Cystic Fibrosis Trust as the nation's most common inherited life-threatening disease (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).According to information on the Trust's web site, more than 8,000 people in the UK suffer from the disease, and more than 2,000,000 people carry the gene identified as the defective CF gene; that is 1 in 25 people (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).It is an unrelenting condition that usually claims the life of its victim anytime between birth and early adulthood, but seldom does the victim live beyond his or her early thirties (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).

Millions of dollars are donated to the Cystic Fibrosis Trust each year in an effort to fund research for new and innovative life-expanding treatment, and a cure (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).The work of the Trust and others who devote their lives to research and to finding a cure for CF, are now working in an era of incredible technological advancement and information exchange, which holds great promise for research and for eliminating CF in the future. This brief essay examines the history in the pathology of the disease, diagnosis, and research in the advances in treatment and increasing the life expectancy of individuals who confront the challenges of the disease in their daily lives as science explores the vast complexities of human genetics in unraveling the mysteries of cystic fibrosis.

The following study begins with a literature review, introducing some of the significant sources of research material that make up the bulk of this essay. The literature review is followed by five informative chapters: Chapter One, the anatomy and physiology of the disease; Chapters Two, the imaging modalities; Chapter Three, the unique tool represented in the MRI, as well as its drawbacks; Chapter Four, the drawbacks of the other imaging modalities, and the future of imaging technology in the diagnosis and treatments for CF; and, Chapter Five, the summary and conclusion of the overall information contained in the essay.

Literature Review

Research of the past two decades has been especially prolific in the production of books and peer reviewed journal articles that speak to the diagnosis, treatment, and advancement of research in the study of CF. Using keywords: cystic fibrosis, cystic fibrosis pathology, CF radiology, CF lungs, CF pancreas, CF digestive yielded thousands of books, journal articles, magazine articles, and newspaper articles. For purposes of this study, non-peer reviewed magazine or newspaper articles were eliminated, as were most sources that were older than the past ten years, with the exception of a few books and journal articles that helped to demonstrate the history in the progress of the diagnosis, treatment, and progress in transplants and in the overall research and treatment of the CF.

Of the remaining works, the field was narrowed down to those works that contributed to the most significant understanding and demonstration of the research and progress in the work to increase the quality of life for individuals afflicted with the disease, and works which inform this essay in that direction.

A.R. Col n's and P.A. Col n's (1999, pp. 250, 255, 260, 263) Nurturing Children: A History of Pediatrics, discusses the early advancements in diagnosing CF because of an innovative new tool: radiology. While radiology advanced diagnostics, it helped the key researchers of the era to understand and arrive at conclusions about CF.

Identification and Assessment of Ongoing Stressors in Adolescents With a Chronic Illness: An Application of the Behavior-Analytic Model' is a journal article written by Ann M. Digirolamo and Alexandra L. Quittner highlighting the relationship between stress and chronic diseases (pp. 53-). The study participants involved 45 adolescents, age 12-17, with CF. The data yield and the findings have informed this essay.

000) Chris Bennett and Daniel Peckham. August 2002. The genetics of cystic fibrosis [online]. Leeds University Teaching Hospitals, Leeds, UK. Available from: http://www.cysticfibrosismedicine.com, serves to provide some input on the genetics of CF. The studies and research conducted by these researchers on CF contributes diagrams and statistical information on genetics to this essay.

001)'Magnetic Resonance Imaging of the Lung in Cystic Fibrosis', like radiology advanced the research and understanding of CF. This article by Talissa a. Altes, Monika Eichinger and Michael Puderbach (2007) explores the giant leaps MRI has meant to body of knowledge on CF by imaging the changing condition as it impacts the vital organs at the different stages of the disease (pp. 321-327).

002)'Computed Tomography and Cystic Fibrosis: Promises and Problems,' ZA Aziz, JC Davies, EW Alton, AU Wells, DM Geddes, DM Hansel (pp. 181-186), is yet another peer reviewed contribution to the study here, and discusses the contribution in the progress of CF through modern technology. The CT scan, as it has been referred to in some medical circles, is an amazing technological advancement that has contributed much to the understanding of CF and other diseases. This group of authors look at the technology in terms of how it has advanced the understanding of CF.

003) the availability of modern technologies does not constitute a carelessness in the use of those technologies. For people suffering chronic illness, like CF, there are risks associated with being subjected to the modern technologies, and for that reason it is with a cautious progress that physicians use those technologies. Cystic Fibrosis: When Should High-Resolution Computed Tomography of the Cest Be Obtained? Is the question that F. Santamaria, G. Grillo, G. Guidi, a. Rotondo, V. Raia, G. de Ritis, P. Sarnelli, M. Caterino, and L. Greco (pp. 908-913).

Cystic Fibrosis in Children and Adults: The Leeds Method of Management, S.P. Conway, J.M. Littlewood, K.G. Brownlee, and D.G. Peckham (2003), all members of the Leeds Regional CF Units at St. James and Seacroft University Hospitals, have provided an informative and useful insight in the experiences of the CF patient at these hospitals. It serves well to inform the understanding of the research here from the patients' perspectives, the hospital staff and teams of ancillary specialists. There is much that goes into the care and treatment of a chronic illness, and this book recognizes those staff positions and career paths that have vital roles in helping patients to achieve and maintain a quality of life for as long as possible in the face of this daunting illness.

Teresa Berrocal, Manuel Parron Pajares, and Amelia Fernandez Zubillaga (2004, pp. 305-309), talk about Pancreatic Cystosis in Children and Young Adults with Cystic Fibrosis: Sonographic, CT, and MRI Findings, that work in support of the previously mentioned authors and bodies of research on the subject. Clearly, modern technology and the ability of professional staff and physicians to be able to interpret these technologies has been the basis for vast amounts of work and study to be analyzed and discussed from the peer reviewed perspective, and in ways that furthers other researchers and physicians in the progress of their own research.

John C. Avise (2004) talks about the Hope, Hype and Reality of Genetic Engineering as it relates to CF. Many people have come to rely on their expectations that research in genetic engineering has advanced to a place in time where it will prove possible to save the lives of those victims of CF who might be in approaching vital points in the progression of the disease. While that is a goal of genetic researchers, and it is the hope of many patients and their families and the medical community; the question of whether or not it is a reality for the near or even distant future is discussed in this book. The information is the difficult but necessary reality that needs to be part of the discussion of this essay.

Much has been investigated for purposes of this research on the existing technology like CT, MRI and radiological services in the advancement of CF diagnosis and treatments. To what extent, though, 'New Tools, New Dilemmas: Genetic Frontiers' exist is discussed by Kathleen Nolan and Sara Swenson in 1988, as we look at the progress made since that discussion two decades ago. Nolan and Swenson contribute to the historical understanding in the advancement of technology, and in putting into perspective those technologies which never manifested themselves as useful, or which have not yet been invented. What are we looking at in the way of 'New Tools, New Dilemmas?'

Other works related the study of genetic research as it pertains to CF will be introduced, and works that focus on CF through sociological studies will be introduced as they come up in the chapter discussions. The official web sites of the Cystic Fibrosis Trust, located online at: http://www.cftrust.org.uk/;the National Institutes of Health, found online at: http://www.nih.gov/;and the American Cystic Foundation, found online at http://www.cff.org/will each be the source of information and professional peer reviewed articles will be cited from these sources and identified by source as they cited.

There is a wealth of available information, data and studies on CF. What it all means to the patients who suffer from this debilitating and life-threatening disease will be understood as this essay proceeds.

Chapter One

Diagnosis and the Anatomy and Physiology of a Life Threatening Disease: Cystic Fibrosis child is born in the UK and, since 2007 are tested for CF in this country where cystic fibrosis is the most common inherited life-threatening disease prevalent amongst Caucasians who at a ratio of 1 in 25 people carry the faulty gene that causes CF (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).

While the UK's Cystic Fibrosis Trust cites the average life expectancy of a person with CF as 31; information on the site also says that children born with the disease today might expect to live even longer; presumably because of the advances in diagnosing treating CF (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).It is towards this goal in increasing the quality and length of life expectancy that the Trust works toward, because, it says, the cure appear to be a long way off (Cystic Fibrosis Trust, 2008, available at: (http://www.cftrust.org.uk/aboutcf/whatiscf/).

Discovery of the genetic disposition of CF has lead to current abilities to diagnose the condition prior to birth through a process called amniocentesis (Conway, Littlewood, Brownlee, and Peckham, 2003, p. 10). Amniotic fluid is aspirated from the fluid surrounding the baby (p. 10). The amniocentesis is an invasive procedure, which carries with it the risk of miscarriage following the procedure (p. 10). Chorionic biopsy is an embryonic testing method which has a "small but definite risk to the baby," and should only be performed with a decision to terminate the pregnancy upon discovery of a CF positive diagnosis (p. 10).

After birth, CF can be identified by way of a fairly simple Guthrie test, which involves pricking the infant's heel (a test that reveals other information too) (Cystic Fibrosis Trust, 'How is Cystic Fibrosis Diagnosed, 2008, NPG).

Modern technology now allows for the disease to be detected even prior to conception for couples who have a family history of the disease (Conway, Littlewood, Brownlee, and Peckham, 2003, p. 10).

What Science Looks for in the 1930s, Dr. Dorothy Andersen, a pathologist at Columbia University, noted the pathological similarities in the cases of certain children who were just days old, to as much as several years old (Clark, 1997, p. 27). In 1938 Andersen published a paper identifying the disease that would come to be known as cystic fibrosis, opening the doors for research that would, by the 21st century, identify the genetic mutation that responsible for the disease, and see the accumulation of a vast body of research and work around the diagnostics and physiology of he disease (p. 27).

CF is an autosomal recessive disease that has been identified by its genetic footprint of a defective cystic fibrosis transmembrane conductance regulator (CFTR) (Berrocal, et al., 2004, p. 1305). This genetic defect of the CFTR results in the body's inability to transport chloride across the membrane of the epithelial cells that are the expression of CFTR (p. 1305). This result is abnormally thick secretions that impact every vital organ in the body: the pancreas, the liver, the lungs, paranasal sinus tract, and even the reproductive organs (p. 1305). It is the resultant organ dysfunction and inability to deal with the abnormally thick secretions that eventually brings about death, usually as a result of respiratory disease and failure because of the CF (p. 1305). However, if an individual suffering CF did not die as a result of breathing disorders and problems, the other impacted vital organs would eventually bring about death too (p. 1305). Unfortunately, there is at this point little good news in the way of prognosis; except that the average life expectancy of individuals suffering from CF has gone from about five years old in the 1960s, to an average of 31 years old today (Cystic Fibrosis Trust, 'How is Cystic Fibrosis Diagnosed, 2008, NPG).

The complexities of the disease are much greater than just this notion of CFTR. It is an inherited disease passed on when both parents, usually of Caucasian European ancestry (it is seldom found in Asians or people of Black African descent (Berrocal, et al., 2004, p. 1305)) (Lewis, 1993, p. 22). When it is passed on to the carrier, either sex of the children of the couple whom each carry a copy of the mutated CFTR; then the child it is passed on to has a copy of the mutated gene, which can cause the illness, and a copy of a normal gene, which can prevent the illness (p. 22). A child of carrier parents has a 1 in 4 chance of being free of the disease, and a 1 in 2 chance of being a carrier like the parents (p. 22).

The diagram below demonstrates the autosomal inheritance chain (006 gif):

While CF is a very physical disease, a painful disease, it has a psychosocial element too.

Social researcher and physician, Dr. John S. Rolland (1994), says that for those families into which a child stricken with CF is born, it requires the family to define itself in a new way that is consistent with the chronic disability, which will one day probably result in their loved one's death (p. 19). Rolland says the family needs a way of describing systemic illness in a way that recasts the biological disease in psychosocial terms (p. 19). Only in this way can the family achieve the relevant psychosocial schema in which it needs to form its disabled identity and to develop the metalanguage that allows movement and discourse between the world of the family's and their stricken loved one and the world around the family that is not stricken (p. 19).

Even though there is a social aspect to CF, as acknowledged by the St. James's and Seacroft University Hospitals' 'Leeds Method of Management (2003),' an informative book put together directed towards facilitating the psychosocial aspect of CF; Simon J. Williams, Lynda Birke, and Gillian a. Bendelow (2003) conclude that there remains a disjoint between the biologists and sociologists that causes the biologists to ignore for the most part the social aspect of disease and to concentrate solely on the "proximal" causes of the disease (p. 16). It is, after all, unraveling the mysteries of the biological, the molecular, and the genetics of the disease which will one day in the future yield the cure.

Complex Physiology

To begin understanding the physiology of CF, consider first that the body has a vast network of ducts lined with cells (p. 28). These are the epithelial ducts, serving as internal "tunnels" between the body's internal organs in the delivery of substances (p. 28). Not the least of these substances are nutritional substances that sustain the overall bodily ability to convert substance to energy and to thrive (p. 28). Through the epithelial ducts the body is able to move substances without threat of contamination or spillage (like an oil spill on the pristine Alaskan waters) (p. 28). The ducts assist in the functional priority of each of the body's vital organs; the pancreas and the liver produce and send digestive enzymes along epithelial duct routes to the intestines, without which the body could not digest food and convert to energy the necessary substances to fuel the bodily functions (p. 28).

Epithelial ducts line the lungs as well, and the reproductive organs (p. 28). The cells that line the epithelial ducts, like most human cells, have a nucleus containing 46 chromosomes (Bennett and Peckham, 2002, NPG). The chromosomes are long coils of double stranded DNA (NPG).

Forty-four of these chromosomes are matched into twenty-two pairs and are numbered from 1 to 22 (autosomes). The last pair makes up the sex chromosomes, X or Y. Females receive two X chromosomes while males receive an X chromosome from their mother and a Y from their father (NPG)."

The chromosomes contain some 35,000 genes that are made up of segments of DNA (NPG). The DNA segments are the body's map, dictating the body's production of protein (NPG). It is this production of protein, or lack thereof, wherein is the problem that is known as cystic fibrosis (NPG).

In 1985 the gene was localised to 7q21-34 on the long arm of chromosome 7 (Wainwright et al., 1985) and this was soon followed by identification of the gene sequence (Rommens et al., 1989; Riordan et al., 1989, Kerem et al., 1989). The gene encodes a 1480 amino acid protein, which has been named the cystic fibrosis transmembrane conductance regulator or, for short, CFTR (NPG)."

The diagram below provides a visual of the above description (007 gif):

CFTR is a protein that is found in various cell types, including lung epithelium, submucosal glands, pancreas, liver, sweat ducts and reproductive tract. It comprises two membrane-spanning domains and two nucleotide-binding domains separated by a regulatory R. domain. The two membrane-spanning domains form a low-conductance chloride channel pore (figure 2). This is regulated by the binding and hydrolysis of ATP at the nucleotide-binding domains following initial phosphorylation of the R. domain. CFTR also regulates ion transport. It has inhibitory effects on apical sodium permeability across epithelial surfaces and activates non-CFTR chloride channels (NPG)."

This process helps put into perspective the CFTR throughout the body, and it is logical, then, that CF is a multi-systemic disease because the protein is being transported throughout each of the body's vital organs and interactive systems (NPG).

Mucus serves an important function in the body, an when the epithelial ducts are blocked, the mucous is blocked from performing its bodily function and becomes clogged in the ducts (Clark, 1997, p. 28). It no longer performs its function in the gut as a lubricant as food passes and the very process of food intake becomes a painful process (p. 28). The epithelial cells are no longer protected by ingested food and bones, seeds, and even food texture can become a painful or even life threatening event (p. 28).

In the nose, throat, and lungs the mucus serves to trap microbes and foreign particles that are then pushed along and out by the cilia beneath the mucus and the flow out of the system of the foreign or microscopic particles continues much like a flowing river; except when the individual is a victim of CF (p. 28). Then, these functions are impaired, and backup in the body's system activating the body's natural response to the condition, which is usually one of inflammation and swelling, only further complicating the patient's condition (pp. 28-29).

The difficult and painfully desperate life of the individual suffering from CF becomes clearer as the discussion of the multiple ways in which the body's systems are adversely impacted by the disease that monitoring of the vital systems and consistency in therapeutic treatments and of the importance of pharmacological regimes are essential to the quality of life for victims CF victims. So, too, is the ability to have visual images of the body's systems in order to understand how those systems are responding to the progression of the disease, and to the treatment regimes.

Chapter Two

Computerized, Tomography as a Tool in Treatment Approach for Cystic Fibrosis

In a study conducted by Thomas H. Helbich, MD, Gertraud Heinz-Peer, MD, Irmgard Eichler, MD, Patrick Wunderbaldinger, MD, Manfred Gtz, MD, Claudia Wojnarowski, MD, Robert C. Brasch, MD and Christian J. Herold, MD involving 117 CF patients for purposes of.".. CT was performed as part of an examination in anticipation of lung transplantation in 10 patients, as part of a DNA treatment protocol in 29 patients, or as part of our prospective CT evaluation in 78 patients (Helbich, et al., 1999, found online at: (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).The scoring results produced by the study are shown below:

TABLE 1. Criteria of Cystic Fibrosis CT Scoring System

Score

Category

Severity of bronchiectasis

Absent

Mild (luminal diameter slightly greater than diameter of adjacent blood vessel)

Moderate (lumen two to three times the diameter of the vessel)

Severe (lumen more than three times the diameter of the vessel)

Severity of peribronchial wall thickening

Absent

Mild (wall thickness equal to diameter of adjacent vessel)

Moderate (wall thickness greater than and up to twice the diameter of adjacent vessel)

Severe (wall thickness more than two times the diameter of adjacent vessel)

Extent of bronchiectasis*

Absent

More than 9

Extent of mucous plugging*

Absent

More than 9

Extent of sacculations or abscesses*

Absent

More than 9

Generations of bronchial divisions involved (bronchiectasis or plugging)

Absent

Up to the fourth generation

Up to the fifth generation

Up to the sixth generation and distal

Severity of bullae

Absent

Unilateral (not more than 4)

Bilateral (not more than 4)

More than 4

Severity of emphysema*

Absent

More than 5

http://radiology.rsnajnls.org/math/dagger.gif

Severity of mosaic perfusion*

Absent

More than 5

http://radiology.rsnajnls.org/math/dagger.gif

Severity of collapse or consolidation

Absent

Subsegmental

Segmental or lobar http://radiology.rsnajnls.org/math/dagger.gif

Numbers are the number of bronchopulmonary segments.

NA = not applicable.

Helbich, MD, Heinz-Peer, MD, Eichler, MD, Wunderbaldinger, MD, Gtz, MD, Wojnarowski, MD, Brasch, MD and Herold, MD, 1999, p. 537-544, found online at: (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).

The study yielded the following data, which proved not just significant in the study of the 117 patients, but also supports the CT scan as a very viable tool, a superior tool, in the diagnosis, study, and ongoing care of CF patients (at: (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).

That support is demonstrated by the data and information yield of the study and as compared to the data and information yield obtained by radiological examinations:

Study evaluation included CT in all 117 patients, clinical examination with the Shwachman-Kulczycki scoring system (

) and immunoglobulin, or IgG, assays in 104 patients, pulmonary function test in 90 patients, and genotypic evaluation in 77 patients. All examinations of each patient were completed within 2 weeks. To assess the influence of age on disease status, patients were divided into three age groups: group 1 (age range, 0-5 years; mean age, 2.4 years a± 1.5; n = 20; seven girls, 13 boys), group 2 (age range, 6-16 years; mean age 9.4 years a± 3.1; n = 61; 31 girls, 30 boys), and group 3 (age range, 17 years and older; mean age, 22.1 years a± 4.1; n = 36; 19 women, 17 men).

CT Evaluation

Scanning was performed with either a TCT 900S (Toshiba, Tokyo, Japan) or Tomoscan SR 7000 (Philips, Best, the Netherlands) CT unit. CT studies consisted of scans of 2-mm-thick (Toshiba unit) or 1.5-mm-thick (Philips unit) sections at 10-mm intervals, extending from the lung apices to below the costophrenic angles and obtained with the use of a 350-mm field of view and a 512 x 512 reconstruction matrix. Examinations were performed at 137 kV and 250 mA (Toshiba unit) or 140 kV and 175 mA (Philips unit) with a 1-second scanning time. Images were reconstructed with a high-spatial- frequency algorithm and with window settings appropriate for assessment of pulmonary parenchyma (level, -450 and -700 HU; width, 1,500 and 1,000 HU). Scans at the level of the hilus were additionally reconstructed with window settings suitable for evaluation of the mediastinum (level, 35 HU; width, 400 HU). Six images were transferred to each 14 x 17-inch film hard copy.

CT images were acquired at suspended end-inspiratory volume in older children who could cooperate. In children unable to cooperate with breathing, images were obtained during quiet breathing. Scans were obtained with the patient in the supine position, and additional scans were obtained with the patient in the prone position when the reversibility of dependent areas of increased attenuation were assessed.

Morphologic Analysis of CT Studies

Two radiologists (T.H.H., G.H.P.) together reviewed the studies in random order and arrived at a consensus opinion. Both radiologists were blinded to the results of the clinical scores (Shwachman-Kulczycki scoring system), the immunoglobulin assay results, the pulmonary function test results, and the genotype. The radiologists determined the grade and the anatomic distribution of each morphologic sign listed in Table 1. The morphologic changes were scored with respect to severity and extent by using a modification of the system proposed by Bhalla and co-workers (

). As in the Bhalla scoring system, the severity and extent of bronchiectasis, peribronchial wall thickening, mucous plugging, sacculations, bullae, emphysema, and collapse or consolidation were evaluated (1999, online at (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).

This study demonstrates the superiority of the CT scan, however, and for reasons that remain unclear, radiological examinations of CF patients continue to be opted over CT scans (1999, online at: (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).The ease with which the progression of the disease can be observed on CT scans is obvious, and it yields itself to a better informed approach in the decision making processes of ongoing patient care. The following images were produced as a result of the above study, but support the superiority of the CT scan as a tool in the imaging of CF.

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Figure 1. CT scan at the level of the upper lobes in a 4-year-old boy (group 1 [0-5 years]) demonstrates mild signs of bronchiectasis (short arrows), bronchial wall thickening (arrowhead), and mosaic perfusion. The overall scoring of bronchiectasis was based on the most frequently identified severity in accordance with Bhalla et al. (19). Thus, the moderate signs of bronchiectasis (long arrow) did not affect the overall score.

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Figure 2. CT scan at the level of the upper lobes in a 9-year-old boy (group 2 [6-16 years]) demonstrates mild to severe signs of bronchiectasis (curved arrows) and mild to moderate signs of bronchial wall thickening. In addition, CT scan shows mucous plugging (straight arrows) and mosaic perfusion (*).

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Figure 3. CT scan at the level of the upper lobes in an 18-year-old man (group 3 [17 years and older]) demonstrates severe signs of bronchiectasis partly filled with mucus, moderate signs of bronchial wall thickening, multiple areas of consolidation (arrows) with air bronchogram, and emphysema (arrowheads).

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Figure 4. CT scan at the level of the upper lobes in a 26-year-old woman (group 3 [17 years and older]) demonstrates mild to moderate signs of bronchiectasis and peribronchial wall thickening. Mosaic perfusion, bullae (straight arrows), emphysema (*), and an area of consolidation (curved arrow) are also seen.

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Figure 5. CT scan at the level of the lower lung zones in a 26-year-old woman (group 3 [17 years and older]). In contrast to the usual severe findings in this age group, this CT scan demonstrates mild signs of bronchiectasis, mosaic perfusion (*), and a small area of consolidation (arrow).

Helbich, MD, Heinz-Peer, MD, Eichler, MD, Wunderbaldinger, MD, Gtz, MD, Wojnarowski, MD, Brasch, MD and Herold, MD, 1999, p. 537-544, found online at: (http://radiology.rsnajnls.org/cgi/content/full/213/2/537).

Chapter Three

MRI and Cystic Fibrosis

Magnetic Resonance Imaging (MRI) is yet another tool that is available in diagnosing and for use in follow the progression of CF and other debilitative and chronic illnesses. The principle upon which MRI works is explained by Dr. Joseph P. Hornak (2007, found online at: http://www.cis.rit.edu/htbooks/mri/inside.htm, Ch 1, p. 1). Hornak, a specialist in MRI imaging, explains MRI this way:

Magnetic resonance imaging (MRI) is an imaging technique used primarily in medical settings to produce high quality images of the inside of the human body. MRI is based on the principles of nuclear magnetic resonance (NMR), a spectroscopic technique used by scientists to obtain microscopic chemical and physical information about molecules. The technique was called magnetic resonance imaging rather than nuclear magnetic resonance imaging (NMRI) because of the negative connotations associated with the word nuclear in the late 1970's. MRI started out as a tomographic imaging technique, that is it produced an image of the NMR signal in a thin slice through the human body. MRI has advanced beyond a tomographic imaging technique to a volume imaging technique (2007, Chapter 1, p. 1)."

The image that is produced is one that has been described as "slices" of the designated area of the anatomy (Ch 1 p. 1). Each "slice" has a thickness, almost like a 3-D image, and the slice can be viewed and analyzed by the experts to gain a sense of dimensional conditions of a tumor, or lesion, or, in the case of CF, the inflammation, or the thickness of the blockage in the lungs, the throat, the pancreas and the other vital organs and systems affected by the disease. MRI actually is a technology that is capable of presenting the clinician, researcher, or physicians the images of the multiple small cysts that are a manifestation of CF in a way that each "slice" can be examined as a structure within the system or arising out of the organ tissue.

Physician researchers SB Fiel, AC Friedman, DF Caroline, PD Radecki, E Faerber and K. Grumbach (2007) say that it is difficult to rely on the yearly chest roentgenograms (CXR) for making treatment decisions for young adult patienhts suffering with CF (p. 1). The CXR does not provide the level of detail about the patient's condition as does the MRI (p. 1). The physicians studied 16 young adults with CF, utilizing the information yield from CXR and MRI (p. 1). Evaluations of the pancreas, spleen, liver and gallbladder were done (p. 1). Researchers found the MRI to be "superior" in the quality and detail of useful information produced (p. 1).

The team says that the MRI has proved itself useful in the study of the progression of the illness with respect to the various stages of the physiology of the illness, and especially in examining and understanding the fatty infiltrations of vital organs like the pancreas that is an indication of the progression of the disease (p. 1).

The MRI and CXR result demonstrate the lack of information available to the physician when relying solely on CXR. The clarity, the detail and the imagery of the MRI is far superior, showing the condition of the patient's lungs as they are impacted by the progressing CF. While the CXR, flat in presentation and limited by the extent of the radiological processes, fails to show the disease in its stage of progression and could therefore make it difficult for the physician to relate to the stage of illness impacting the patient's health. The CXR results are not conducive to forming the best treatment plans utilizing the information that modern technology is capable of producing because of the technological limitations of the CXR.