Cystic fibrosis through a female perspective: Psychosocial issues and information concerning puberty and motherhood

Abstract

The purpose of the study was to investigate psychosocial issues concerning puberty and motherhood among CF adult females, to see how they had obtained and conceived information on these matters and how they would like information to be given. Fourteen adult CF females were interviewed. The majority of the women felt socially accepted and did not remember being ashamed over their delayed puberty. Thirteen of the women had been or were living in stable sexual relationships. However, the study revealed problems with destructive behaviour during puberty due to thoughts about premature death, secret worries over delayed puberty, poorly received information about puberty and fertility, avoidance of close relationships with the opposite sex during adolescence and concerns about being a mother with a chronic illness. Information about puberty and fertility should be given individually and in small discussion groups with teenage girls combined with thorough medical and psychological guidance concerning motherhood.

Introduction

Cystic fibrosis (CF) is the most common autosomal recessive hereditary lethal disease among the Caucasian population. Previously most CF-patients died at a very young age due to progressive lung damage [1]. Due to intensified symptomatic treatment and emphasis on nutritional aspects, a majority of the patients now go through puberty and reach adulthood [2]. With new methods available such as organ transplantation [3], and hope for gene-therapy [4], CF is viewed more positively than before.

CF patients have previously shown a delayed puberty. Malnutrition has been considered the main ethiological factor 5, 6. In a retrospective study of 17 females with CF, we showed that delayed puberty also existed among well nourished female CF patients with good lung function [7]. Whether CF primarily can affect sexual maturation is yet not known, but it is known that female patients have decreased fertility rate. This has been thought to be secondary to thick impermeable cervical mucus [8]. Artificial insemination has become an option for females with CF [9]. Today the risk of deterioration during pregnancy for females with CF are considered small if good medical care are provided and the women are in a stable good condition [10].

Even with this positive view in mind, it has to be remembered that patients with CF are living with a life-threatening disease. Earlier psychosocial studies among adolescents and young adults with CF have shown different emotional problems such as low self-esteem, social dependency, distorted body images due to altered physical appearance, denial of sexuality with a delay in intimacy, isolation and increased awareness of the future and death 11, 12, 13, 14, 15, 16. In three of the studies, CF-females showed more emotional disturbances than the male CF-patients 12, 15, 16. CF-females appeared to be more prone to denial of the illness compared to the males who more readily integrated their chronic illness into their self-concept.

We know from previous studies that healthy infertile women normally go through a life crisis before accepting the medical facts concerning their infertility 17, 18, 19, 20. A previous interview study of 22 females with Turner's syndrome showed that during the years prior to diagnosis and hormonal replacement therapy, the women often isolated themselves as they felt different from their peers. Infertility had affected the women very deeply, and many felt depressed because of this [21]. Most other chronic diseases, such as insulin-dependent diabetes mellitus (IDDM) or multipel sclerosis (MS), are unusually detected later in life and lack the complexity of CF including delayed puberty and decreased fertility. Women with IDDM might be compared with CF-females if the sole issue is medical care during pregnancy. There is one epidemiological study of women with IDDM which showed that the women were negative toward motherhood due to their illness [22]. On the contrary, it appears as if pregnancy improves the prognosis of MS [23]; therefore the issue of motherhood is viewed completely different for these women. There is no other disease that can be compared with CF with all its complexity. To be born with an incurable disease with a very unpredictable life span is unique for CF.

In two recent studies, it has been shown that patients with CF have limited knowledge about reproductive issues 24, 25. There are no previous studies focusing on the psychosocial aspect of decreased fertility among CF females.

The main aim of the study was to investigate how CF is connected with psychosocial issues concerning puberty and motherhood among CF adult females. We were interested to find out if and how they had obtained information about pubertal development and fertility, how it had been conceived by them and how it had influenced them later in life. Further on we wanted to know how the patients would prefer this information to be given. Our hopes were that the outcome of the investigation would help us to optimize the care for women with CF.