Treatment History Of Cystic Fibrosis The field of pediatric nursing is a particularly large one in which its members may find themselves having to counsel both young patients and their families suffering from a myriad of diseases. Cystic fibrosis (CF), in particular, is a debilitating disease that strikes many young people. In the first years following its discovery, many children could not expect to make it to their teen years. Today, with advances in the management and care of this disease, patients are living into their 40s. It is even possible that there will one day be a cure.

This paper will look at CF from a historical perspective. The timeline of its discovery and research will be briefly presented, as will a few of the significant "milestones" in the history of the treatment of CF and what these mean for people battling this disease.

References to cystic fibrosis were made as early as 1705, although it was two centuries before the actual name "cystic fibrosis" was coined. A folk saying from the early 1700s proclaimed, "Woe to that child which when kissed on the forehead tastes salty. He is bewitched and must die." People with CF lose a higher amount of salt than normal in their sweat, which leads to their skin having a salty taste to it. In fact, it is this salty taste on the skin of newborns that often leads parents to the indication that something is seriously wrong.

One of the foremost pioneers in the field of CF research (and the woman who gave it its name) was Dorothy Andersen of Columbia University. In 1938, she published the first comprehensive description of CF as a specific disorder. She gave the disease its name because the pancreases in people who have it are riddled with fibrous scar tissue that surrounds cyst-like, fluid-filled spaces. Prior to this, it was not understood that the symptoms of CF were related to a specific disease per se.

In 1946, Andersen recommended treating affected children with a high-calorie, high-protein, low-fat diet, supplemented with digestive enzymes from animal pancreases. In 1948, antibiotics were developed specifically aimed at killing the Staphylococcus bacteria, which is responsible for the severe, chronic chest infections that, prior to this, often killed children with CF before they reached the age of seven.

In the late '40s, physicians discovered that a defect in mucus secretion throughout the body was responsible for many of the symptoms of CF. However, it was years before scientists determined that a defective channel protein that controls the flow of chloride ions was responsible for producing this mucus. In 1946, another pioneer in the study of CF, Paul di Sant-Agnese, reported using antibiotics successfully to treat lung infections caused by CF.

In 1952, a heat wave in New York City led scientists, di Sant-Agnese among them, to the understanding that people with CF became dehydrated faster than those without it (given the aforementioned larger-than-normal salt loss, this shouldn't be surprising). Consequently, di Sant-Agnese and his colleagues realized that people with CF have especially salty sweat (at last the connection with those folk sayings from the seventeenth and eighteenth centuries was made). As a result, Lewis Gibson and Robert Cooke of the Johns Hopkins University Medical School developed an effective method for collecting sweat, and doctors began using this "sweat test" to diagnose CF. In 1955, the first review of the use of pancreatic enzymes was released, further adding to the body of knowledge surrounding this disease.

In 1964, Doershuk, Matthews, and their colleagues described a modern comprehensive treatment program similar to Andersen's. In 1978, doctors first used enteric-coated pancreatic enzymes to help treat CF, and in 1981-1983, scientists Knowles and Quinton and their colleagues introduced a description of electrolyte-transport abnormalities to the field.

Heart and lung transplants have also proved effective in combating this disease, although the cost of the cure may outweigh the disease itself. By 1996, this procedure was perfected and had been performed in a few hundred people with CF. Because of the difficulty and expense in caring...

In 1979. The test was used to detect levels of a substance found to be elevated in the blood of neonates with CF. The most recent research in this area has been done in Australia and New Zealand, where newborn screening for CF is implemented on a nationwide level.
Since then, both Colorado and Wisconsin have made newborn screening for CF mandatory by law. It is felt that with early identification and treatment, improvements in nutritional and respiratory outcomes may appear. However, although the intent behind newborn screenings is beneficial, it can have unintended disastrous results, including "the issue of false-positive and false-negative results, misconceptions and miscommunication of results, and potential stigmatization" (Baroni, 1997). As a result, Wisconsin introduced a two-tier screening process in 1994 in which the number of false-positives has declined and the infants referred for further testing are determined to be definite carriers of the CF gene.

It is in the area of counseling patients and their families when this occurs that pediatric nurses can perhaps be especially effective. Numerous studies demonstrate that the effect this has on parents is nothing short of devastating. For example, psychological reactions of parents are characterized by overprotective behaviors and chronic anxiety regarding their child's health. Such long-lasting effects may have a direct impact on parents' ability to establish that special bond with their child that is crucial to the child's healthy growth and development (Baroni, 1997).

Despite screenings, scientists still maintain that "the only definitive diagnostic test is the quantitative pilocarpine iontophoresis sweat test, which cannot be done until 3-4 weeks of age when adequate sweat collection is possible" (Baroni, 1997). The test works by pharmacologically stimulating localized sweating and then collecting the amount of sweat secreted;

measurements of electrolytes are determined and NA or Cl values > 60 mEq/L confirms the diagnosis given a suggestive clinical picture or positive family history. It is estimated that less than 1:1000 patients with CF will have a sweat chloride less than 50 mEq/L (Phillips, 2002).

The biggest leap forward in understanding and treating CF, aside from Andersen's work in the '40s, occurred in 1989 when Tsui, Riordan, and Collins discovered the gene that was actually responsible for CF. In 1990, the correction of the chloride transport defect in CF cells in culture by an adenovirus-mediated gene transfer was announced, and 1992 saw the first trials of gene transfer in living people with the disease.

Beginning in the '80s and carrying on through the '90s and into today is the concept of holistic healing. This one-time alternative method of treatment is now seen as a widely accepted aspect of treating the patient as a whole rather than just treating the disease. The holistic approach to treating CF integrates not just traditional scientific methods and approaches, but also is designed to address a patient's mental, spiritual, social, and even economical health and well-being.

A recent study examining this approach was based on van Manen's techniques and set in Watson's theory of human caring.

Watson views the art of nursing as a creative focus on human interrelationships, not just on disease and germs...[it] is demonstrated in the nurse-client relationship, in which empathy and transpersonal caring predominate. Caring and curing are now meshing as healing forces (Tracy, 1997).

Van Manen suggests that phenomenologic research "borrows" other people's reflections and actual experiences to "elucidate a deeper significance of human events in the context of the whole person. As various situations are selected, structures or themes that make up that experience emerge, bring focus, and simplify the phenomenon one is trying to understand. Furthermore, nurses need to reflect on what matters to individuals who grow up with a…