Gendered embodiment and survival for young people with cystic fibrosis

Abstract

In the context of improvements in both longevity and the quality of life for people with chronic disease, this paper takes as its sense of problem the differential life expectancy for young men as against young women suffering from cystic fibrosis. From a qualitative study of the transition to adulthood for young people with the disease, a theory of gendered embodiment is proposed to explain this differential. The social construction of masculinity and femininity as social practices resulted in the former being more conducive to survival than the latter in this case. There were marked differences between the young women and young men in attitudes to: the meaning of life, death, career and body image; all of which affected adherence to medical regimen.

Introduction

Since the Second World War, significant advances in medical technology have improved the diagnosis and management of chronic illness. Both earlier diagnosis and improvements in drug technologies have revolutionised the treatment and management of a wide range of chronic illnesses. Survival chances have improved significantly especially by controlling secondary infections such as pneumonia which previously had resulted in the death of many patients with weakened defenses against illness.

The improvements have had several implications. The prevalence of these chronic conditions in the community has increased as more are diagnosed, and the life expectancy has improved dramatically. As with many other areas of ill health, the focus has changed somewhat from aiming primarily at keeping the patient alive, to attempting to enhance quality of life for people with these conditions. Quality of life, as a number of authors have argued, has become a legitimate aim of medical attention (see Charmaz, 1987; Gerhardt, 1990). In order to maximise this quality of life, it is important to know the effects of the disease on the lived experience of patients by the provision of detailed information on the social impact of the disease on the person concerned and the effects of medical interventions on the lives of chronically ill people.

One of the most dramatic areas of improvement has been with cystic fibrosis (CF): the most common single gene disorder as well as the most common fatal hereditary disorder affecting Caucasians. Estimates of its incidence vary from one in 2500 Caucasians to one in 3300 (NIH, 1997). It is the most common cause of chronic lung disease in children and young adults. In countries where Caucasians predominate, it is the most common lethal genetic disease of childhood. In the largest of these, the United States, approximately 25,000 have been diagnosed with CF at the rate of new diagnoses of approximately 850 per yr (NIH, 1997). CF affects the exocrine system in the body; producing mucus that is abnormally thick and sticky and causing major problems involving the lungs, pancreas and bowel. Mucus blocking the pancreatic ducts results in pancreatic insufficiency (the absence of digestive enzymes for the absorption of protein, fat and carbohydrate), as well as inadequate absorption of nutrients. This can result in a voracious appetite but inadequate weight gain. Mucus that is thick and sticky sits in the lungs and if not removed, results in over-inflation of the air sacs causing reduced elasticity. Lung damage is affected also by continuous infection of the bronchi leading to gradual weakening of the bronchi walls, deterioration of the lungs, and death. (see Hopkin, 1998; Orenstein, 1997).

As a disease, CF is highly variable in its presentation, severity and course. Some people with Cystic Fibrosis (PWCF) are hardly affected and may not present until they are in their second or third decade of life. Most, however, are more severely affected and may experience gastrointestinal and/or pulmonary complications from the outset. The main determinant of both quality of life and longevity appears to be the severity of lung disease as 90% of PWCF die as a result of these pulmonary complications (NIH, 1997, p. 5). Treatment regimens are complicated and extensive. They involve physiotherapy and/or exercise daily to clear mucus from the lungs, combined with antibiotics to reduce lung infection. To maintain an adequate weight, a high-calorie diet is necessary, and to help the absorption of nutrients PWCF must take digestive enzymes with meals. Regular exercise, physiotherapy and careful nutrition are all conducive to survival.

Prognosis has improved markedly with the improvements in the medical technologies of diagnosis and treatment: physiotherapy, digestive enzymes, antibiotics and nutrition. Before 1939, Jackson (1989, p. 9) reports, most PWCF died before the age of one year. Life expectancy varies slightly from country to country but has broadly improved from a median of about seven years in the early 1960s, 14 yr by the early 1970s to almost 30 yr in 1995 (NIH,1997, p. 5). Many young people with CF (YPWCF) can look forward to a decade or more of adult life. A few PWCF have lived out a near- normal life span.

Despite the apparent gains, improvement in survival and quality of life has been uneven. The problem taken for investigation in this paper is the gender differential in mortality amongst PWCF. Why do males experience this disease with lower morbidity and mortality rates then females? Furthermore, should the differential be considered an issue of the premature mortality of young women or the extended life expectancy of young men?

Firstly, however, it is necessary to establish that the gender differential in mortality is a real phenomenon. Evidence collected from patient data registries in various countries with a high incidence of CF, confirm the phenomenon. The largest disparity we have found is in the Australian state of Victoria with its centre in Melbourne. Data from the Australian Cystic Fibrosis Data Registry (ACFA, 1994, p. 30) for that state, shows that while the median survival rate for all young people with CF has doubled since 1969, the prognosis is better for males, with a median survival rate of 41 yr, compared to only 27 yr for young women; a massive 14 yr gap in life expectancy. In the Canadian context the median survival rate difference in 1985–1989 was 36.7 for males and 27.8 yr for women, a difference of 8.9 yr. Indeed the differential had increased over time: in 1970–1979 it was only 6.4 yr (Corey & Farewell, 1996). Most recent Canadian data suggest that the gap has been shrinking (Median survival age for 1992–1996 was 30.0 for females and 33.4 for males) but now appears to have stabilized. (CCFF,1996, p. 2). In the United States, the median survival rate is 28.4 yr for young men and 25.3 yr for young women, a differential of 3.1 yr (Rosenfeld, Davis, FitzSimmons, Pepe, & Ramsey, 1997). In the United Kingdom, Jackson (1989, p. 9) reports “in most reports the survival rate is slightly higher for boys than for girls.” (See also Dodge et al., 1993).

In a detailed analysis of the gender gap, Rosenfeld et al. (1997) used a methodological tool called the Cox proportional hazards regression analysis to compare age-specific mortality rates and to identify risk factors that may explain the gender-related difference in survival. They found that among PWCF of 1–20 yr of age, females were 60% more likely to die than males, but beyond 20 yr of age, the survival rates were not significantly different. So it seems the main difference to focus on is apparent in childhood and teenage years.

What biomedical factors have researchers found that might explain this gender differential? Jackson (1989, p. 9) argues that the reason for the differential has not been explained. Corey and Farewell (1996) argue that “poorer survival in females was associated with poorer weight, but the interrelation of declining pulmonary function, weight maintenance, sex and mortality requires further study”. Rosenfeld et al. (1997) go further:

Nutritional status, pulmonary function and airway microbiology at a given age were strong predictors of mortality at subsequent ages. Nonetheless, differences in the genders in these parameters as well as pancreatic insufficiency, age at diagnosis, mode of presentation and race could not account for the poorer survival among females. Even after adjustment for all these potential risk factors, females in the age 1–20 yr remained at greater risk for death.¹

Another study considered whether earlier chronic infection with the bacteria pseudomonas aeruginosa (PA) in young women with CF might explain the differential. (Demko, Byard, & Davis, 1995). After eliminating both puberty-related hormonal influences and nutritional status as explanatory variables, the researchers found that the median age for both initial positive culture for PA, and for chronic PA infection was lower for young women than young men, but “this does not account entirely for the three-year age in survival according to gender” (Demko et al., 1995, p. 1046). They conclude “it appears that the gender gap in survival for CF may be explained in part by earlier acquisition of a more noxious form of bacteria by females, but not by greater impact of this organism on their lung disease… other unknown factors may contribute to gender differences in the progression of CF lung disease” (Demko et al., 1995, p. 1046).

In this paper, we aim to shed light on this medical mystery by arguing for a more socially based explanation. Based on research on the transition to adulthood for YPWCF, we attempt an explanation in terms of gendered embodiment that goes some way to explaining this phenomenon. Our research into the experience of YPWCF focused on understanding how they negotiate the transition to adulthood. Researchers of youth have established that the processes of transition to adulthood are increasingly complex. As the traditional ‘markers’ of adult status (such as marriage, leaving home and establishing a career) have become increasingly ambiguous, youth researchers are asking how we define adult status (Wyn & White, 1997). Young people with a chronic life-threatening illness are faced with a more complex experience of transition, depending on the degree of severity of their condition. As a result, we chose to focus our research on how the YPWCF resemble and differ from their peers. Our findings suggest that the differences between the males and females’ morbidity and mortality rates are related to their patterns of adherence to the normative prescriptions of feminine and masculine behaviour appropriate to young people in general. To facilitate this analysis, we have drawn upon the concept of gendered embodiment, the explanation of which is considered in the next section.