Published ahead of print on November 16, 2006, doi:10.1164/rccm.200605-598OE
© 2007 American Thoracic Society doi: 10.1164/rccm.200605-598OE
Pregnancy and Chronic Progressive Pulmonary Disease1 Department of Pediatrics, Hadassah University Hospital, Mount Scopus, Hebrew University Hadassah Medical School, Jerusalem, Israel; 2 Department of Women's and Children's Health, Uppsala University, Uppsala University Hospital, Uppsala, Sweden; 3 Adult Cystic Fibrosis Unit, Northern General Hospital, Sheffield, United Kingdom; and 4 CF Legal Information Hotline, Houston, Texas Correspondence and requests for reprints should be addressed to Eitan Kerem, M.D., Director, Department of Pediatrics and CF Center, Hadassah University Hospital—Mount Scopus Campus, Hadassah Hebrew University Medical Center, Jerusalem 91240, Israel. E-mail: kerem{at}hadassah.org.il ABSTRACT Progressive pulmonary disease may preclude the option of pregnancy for a number of women in their child-bearing years due to the severity of the disease. For a subset of women with chronic lung disease including cystic fibrosis, pregnancy is possible, but can have a devastating effect both on the prospective mother and fetus. The potential hazards of pregnancy in cystic fibrosis or other progressive pulmonary diseases may trigger a moral conflict between physician and patient. The female patient may argue that her autonomy cannot be circumscribed and that the physician is obliged to assist her reproductive efforts. The physician can counter that his/her participation in potentially harmful interventions is not consistent with professional norms requiring adherence to the principles of beneficence and nonmaleficence. Whenever possible, the ethical conflict between physician and patient should be resolved before initiation of pregnancy. We propose that this best be done through structured negotiations between physician and patient with the goal of constructing an ethical framework for reducing the moral tension between the two. Steps in the negotiating process include defining the therapeutic alliance, information exchange, dialog, and deliberation. As part of the information exchange, it is important to discuss alternatives to pregnancy such as adoption and surrogacy, especially when there are strong contraindications to pregnancy. If negotiations reach a satisfactory conclusion for both sides, there should be a well-delineated consensual agreement to commence the pregnancy with the full support of the medical team.
Key Words: pulmonary disease • cystic fibrosis • pregnancy • patient autonomy • beneficence It is relatively unusual for women of child-bearing age to be afflicted with respiratory diseases of such severity that pregnancy may be counseled against or contraindicated. In many respiratory disorders seen commonly in women of child-bearing age, pregnancy is well tolerated, and pulmonary status may improve or remain unchanged. Even in those cases in which deterioration does occur, it is often reversible at the conclusion of pregnancy and hardly ever life-threatening (1). Although poorly controlled asthma during pregnancy may be associated with increased morbidity for the newborn, the slight increase in adverse effects such as prematurity and low birth weight can be avoided with optimal care (2). Hence, it is rare in these cases for a moral conflict to develop between patient and physician with regard to initiating and maintaining a pregnancy. The case is often different for women during their child-bearing years who suffer from chronic progressive pulmonary disease. The hypoxia, pulmonary hypertension (e.g., systemic lupus erythematosus, Eisenmenger syndrome), and/or hormonal changes (e.g., high estrogen effects on lymphangioleiomyomatosis) may adversely affect a pregnancy, and be a relative or strong contraindication to pregnancy (3–6). In these situations, the patient's desire to reproduce may place the physician and patient in a morally difficult position as they both must contend with conflicting ethical principles such as autonomy, beneficience, and nonmaleficence as well as their own personal feelings and desires. Cystic fibrosis (CF) is an example of a chronic progressive pulmonary disorder in which the issue of pregnancy looms large both in terms of the medical management and the ethical challenges that it presents. CF is a life-shortening inherited disorder in which pulmonary manifestations begin in early childhood and respiratory failure is the most common cause of death. CF is a multisystem disorder, and those with CF must contend with growth and nutritional problems, increased frequency of diabetes, liver disease, osteoporosis, and/or impaired fertility. Thus more than other chronic respiratory diseases, CF lends itself to consideration of the moral dilemmas facing a woman considering pregnancy. Forty years ago, the outcome of pregnancy for women with CF was not encouraging, with half the women having adverse events related to the pregnancy (7). Fortunately, improved treatment protocols with early preventative care, better pharmacotherapy, intensified nutrition, and physiotherapy support have extended life expectancy from less than one year in the 1950s to a median of 33.4 yr in 2001 (8, 9). Most women with CF are now reaching reproductive age in a relatively healthy state and are weighing the option of having a child. Nearly two-thirds of women with CF can become pregnant spontaneously (10, 11), and the subset of women with fertility problems can often be treated effectively with assisted fertilization techniques (11–15). Those who become pregnant require significant assistance from a multidisciplinary CF team to sustain the pregnancy, maintain optimal pulmonary status, and manage pregnancy/CF-related complications (16–22). From an epidemiologic perspective, outcomes for the woman with CF and her fetus have been improving over time (16, 20, 22–31). A carefully planned pregnancy is well tolerated if good medical care is provided and the patient is in stable condition. There is even a trend for women with poor pulmonary status to become pregnant, and many of these women have carried to term without adverse effects (17, 28). However, women with moderate to severe lung disease (FEV1 < 50%), colonization with Burkholderia cepacia, poor nutrition, and diabetes mellitus produce more preterm infants and do worse themselves (16, 22–31). Even relatively healthy women with CF are found to have an increased frequency of pulmonary exacerbations and a greater number of hospitalizations during pregnancy (31). The prospect of a woman with CF becoming pregnant and giving birth presents significant challenges with which the physician and patient must contend. The potential deleterious consequences that a pregnancy may have on the health of the mother and the possible damage to the fetus secondary to CF-related complications or administered drugs must be considered (16, 24, 25). Despite improved survival, the possibility that a woman with CF might die during the pregnancy or shortly thereafter is still a reality. This is especially true for the subset of women with increased risk factors for poor outcome. The potential hazards of a pregnancy may set the patient with CF and her physician at odds regarding the advisability of a pregnancy. Without appropriate resolution of this conflict, patient care may be compromised either because the patient refuses to comply with treatments recommended by the medical team or health professionals are unwilling to participate in a process that they believe to be detrimental to the patient. Competing principles of medical ethics exacerbate the difficulty in resolving moral problems associated with a CF pregnancy. Physicians are obliged to respect a patient's autonomy, and that right to autonomy allows for an individual to make decisions even if they result in a negative outcome. This is especially true for reproductive choices, which have significant ramifications for women on many different levels. Beyond the biological and psychological drives to reproduce, there are often religious imperatives, cultural directives, and social/familial pressures that make childbearing a crucial life event that far outweighs other concerns. For these reasons, Western society has been very wary with regard to curbing individual freedom when it relates to decisions about fertility and pregnancy, that is, procreative liberty (32–34). There has been minimal effort on the part of governments or health care systems to prevent mothers with medical conditions such as diabetes, advanced maternal age, or even human immunodeficiency virus from entering into pregnancy even though there may be negative consequences for the health and well-being of the mother (35–38). From this perspective, there is little basis for telling a woman that she cannot become pregnant or take on child-rearing responsibilities simply because she has CF. At most, physicians can provide counseling with regard to the implications of a CF pregnancy (39). From a different perspective, active physician participation in facilitating and managing a pregnancy in a woman with CF is morally problematic. The norms of the profession dictate that physicians do no harm to their patients (nonmaleficence) and provide them with medical care that is in their best interest (beneficence). In the case of CF, the physician, by facilitating the pregnancy, may ultimately cause physical harm to the patient. Even when the patient argues that the pregnancy is in her best interests, the physician may not see that as sufficient justification for compromising the principle of "primum non nocere." The physician's position is further complicated by moral obligations that he/she may have to the fetus. A therapeutic intervention may have a "double effect" by which the intervention is beneficial for the mother, but may have negative repercussions for the yet-to-be-born child. For instance, the interests of the mother and fetus are in conflict if the mother requires a drug that is potentially teratogenic. In this situation, the physician must decide whether the relationship that he/she has with the mother precludes any consideration of the infant's best interests (40–42). RESOLVING THE MORAL TENSION: DEFINING THE PHYSICIAN–PATIENT RELATIONSHIP The tension between the nearly unlimited reproductive autonomy afforded to a woman and the physician's adherence to the norms of nonmaleficence and beneficence must be resolved before embarking on a CF pregnancy. Without resolution, a potential mother may fall victim to a paternalistic medical system that de facto does not allow her to realize her reproductive aspirations. From the other side, the physician's blind support of the patient's preferences may mean forfeiture of the physician's professional autonomy and compromise of moral principles upon which the medical profession is based (33, 43). Several methods have been proposed for resolving ethical conflicts between patient and physician in similar circumstances (44–49). One method is to identify the medical principles at issue in the conflict between patient and physician and assign primacy to one of the principles. Most often this is patient autonomy, and in the case of a woman with CF who wishes to conceive, the physician would be expected to defer to the patient. A second method involves harmonization of principles that are at variance using a synthetic approach for redefining principles so as to resolve the conflict. For example, the principles of beneficence and nonmaleficence can be redefined so that facilitating patient autonomy is the best and least damaging course of action for the physician, as this maximizes benefit from the patient's perspective. Both of these approaches tend to bolster the position of patient self-determination. However, as they both involve a priori ethical constructs, they may not resolve the intellectual and emotional difficulties that a physician has in performing procedures that he/she believes are harmful to the patient. A more pluralistic and practical approach would be to concede that the patient and physician have a different set of interests. When there is conflict, it is best for both parties to seek resolution within the therapeutic relationship. This is best done by negotiation between the two parties (45, 50). There are several forms of negotiation. Negotiations can be a one-sided dialog with either the patient or physician dictating the course of treatment, and the other side acquiescing. Alternatively, the physician can adopt the model of "nondirective counseling" widely used by genetic counselors in which the physician imparts information ("information transfer") with the goal of improving the decision-making capabilities of the patient, but without offering any direction (51). The advantage of the nondirective approach is that it recognizes that physician and patient often have different values, and this form of counseling minimizes the possibility that a physician may coercively force his/her value system on the patient (52). For each of the above approaches, there is the drawback that one side passively concedes decision-making authority to the other without necessarily relieving the moral tension between patient and physician (53–55). An alternative approach, which has been extensively discussed by Charles and associates, is the "shared treatment decision-making model." In this paradigm, patient autonomy is enhanced by a model based on shared information between physician and patient followed by both sides expressing their treatment preferences (56–58). Armed with this information, the physician and patient enter into a process of deliberation and negotiation culminating in a mutually agreed upon treatment plan. This model works best in situations in which there is general agreement about treatment goals, but where there exist different treatment options for achieving the goals without one option being clearly superior to the others. However, the model may be less effective in situations in which the interests of the physician and patient are diametrically opposed, as may occur in CF-related fertility issues. Despite this drawback, the model of shared decision-making, with some modifications, can be appropriate for resolving the inherent conflict between physician and patient's interests in CF and pregnancy. In the modified model that we are proposing, there would be a staged approach that structures the dialog between patient and physician (Table 1). The first stage of the patient–physician encounter would be devoted to defining the parameters of the relationship between the two sides. The goal during this stage is to formulate a therapeutic alliance with both the physician and patient expressing their interests from the outset. For the physician, this may be providing the best patient care possible that allows for an extended lifespan and an optimal quality of life. For the patient, the objective could be attaining the empowerment that allows for control of her destiny, including having a child. Aware of each other's position, the patient and physician can begin exploring the practical differences between their positions and identify the areas in which there may be conflict. The physician needs to delineate the boundaries defining how far he/she is willing to cooperate with the patient's choices when they potentially conflict with the physician's concept of beneficence and nonmaleficence. To make this point, the physician can present hypothetical scenarios in which the patient's choice may not be acceptable. For example, the physician, if he/she is to be an active participant in facilitating the pregnancy, may find it unacceptable if the patient reserves for herself the right not to take a specific medication during pregnancy that the physician considers life-sustaining.
The physician also has to present his/her perspective regarding the responsibility to the mother vis-à-vis the fetus. Will the physician give absolute primacy to the mother or balance the interests of the fetus against those of the mother? For her part, the prospective mother must make clear to the physician how willing she is to accept the counsel of others including the physician regarding the advisability of pregnancy. Are there health situations in which she will countenance termination of pregnancy? Having articulated their views, both the physician and patient need to modify their positions if they are to bridge the gap between them. The physician may have to change his/her perspective and accept that for this patient, the greatest level of beneficence is to facilitate the pregnancy, even with a potentially negative outcome, providing that all measures are taken to prevent CF-related complications. The patient must demonstrate flexibility by accepting that the physician cannot countenance certain actions that may endanger her welfare. She must be willing to accept limitations on her autonomy, and recognize from the outset that certain choices on her part are not acceptable to the physician and that the latter is not under obligation to implement those decisions. INFORMATION EXCHANGE: OBJECTIVELY REVIEWING THE BENEFITS AND RISKS OF PREGNANCY IN CF Once the physician and patient come to an agreement regarding the therapeutic alliance, the next stage involving information exchange can be started. This step can either be initiated by the patient or the physician. The woman can explain her desire for a pregnancy, provide the physician with medical information that he/she may not be aware of, and discuss the resources available to her in terms of family and social support. The physician needs to inform the patient about the general statistics involving pregnancy in women with CF, how those statistics apply to her, and the unique aspects of her medical condition that may positively or negatively impact on the pregnancy or her medical status (30). A discussion of the risk factors associated with pregnancy in women with lung disease may be seen by some physicians as an infringement of a patient's reproductive rights and autonomy. Physicians should view such discussions not as a dictate regarding the appropriate course of action, but as the provision of information about the health risks associated with pregnancy. Just as a physician will discuss the risks of smoking to a patient with lung disease, so too the physician should discuss the risks associated with pregnancy in the presence of CF. The physician, in the course of discussion, should also propose alternatives to pregnancy such as adoption. The option of becoming a parent through adoption should be discussed with patients, especially those who may be at increased risk during pregnancy. Adoption allows women with CF to become mothers without any of the risks associated with pregnancy. In most situations there should be no obstacle to adoption, providing that a physician certifies that the health of the mother will not impair her ability to serve as a parent. In fact, numerous adults with CF, both female and male, have adopted children and are successfully raising those children. The availability of different adoption options provides flexibility to prospective parents, as they can adopt older infants or children, which will free the mother from the rigorous demands associated with newborn care. It is important for the physician to be well-versed in the local regulations governing adoption so that when discussing this option, he or she does not to misinform the patient. Some women may be interested in using a surrogate mother, and this option could be explored as well. Discussions of the risks of pregnancy should also include information about the demands of motherhood. Many women with CF make the decision to become pregnant with very little information about the actual time needed to care for a newborn. Treatment burden should not be overlooked in the discussion of pregnancy. A patient who has relatively good pulmonary function but spends 2 to 3 hours a day performing medical treatments may have trouble maintaining her treatment regime after the birth of a child. While treatment burden should not prevent a woman from opting to become pregnant, it should be considered when assessing the risks associated with pregnancy and the need to both care for oneself and an infant. The health of a parent with CF may decline after the child is born because the person is not able to devote enough time to CF self-care. Women with CF may feel they have to "show everyone" that they can be a good parent and may not ask for help or may take on too much responsibility in caring for their babies. The physician may suggest that the woman with CF spend a weekend helping a friend or family member who has a newborn, which can be an eye-opening experience. Only through the provision of information about care issues after a child is born can a woman with CF make an educated decision about pregnancy and parenthood. The physician is also obliged to discuss the issue of the patient dying prematurely, leaving the child without a mother. The impact on other family members also needs to be considered, especially as they may have to shoulder greater responsibility if the condition of the mother deteriorates (59, 60). DIALOG AND DELIBERATION At the completion of the information exchange stage, the process of dialog and deliberation on specific issues relating to the pregnancy can begin. The physician needs to assess whether the patient's ideas regarding pregnancy are rooted in reality, and if the physician can rely upon the patient to maintain the therapeutic alliance as agreed upon. For example, the physician may be wary of continuing the relationship if the patient states that she knows for sure that nothing bad will happen to her during the pregnancy, and that issues concerning damage to her and the fetus will never arise. Such magical thinking should be a warning to the physician that ensuing events may place him/her in a difficult position if complications develop during the pregnancy and the patient reneges on previously agreed upon therapeutic interventions. Given the information that the patient has received from the physician, she must decide whether a pregnancy is realistic, and whether the parameters set forth by the physician are acceptable. At this point, it may be appropriate to involve others in the discussion. The prospective mother's partner should be thoroughly informed about the risk to a woman with CF during pregnancy. He should be made aware that a mother with CF is likely to require more support than a healthy woman both during the pregnancy and subsequent child-rearing. It is also quite probable that he might become a single parent while the child is still young. If there is no father, then the same discussion should be held with the patient's parents or other family members about who will have a role in caring for the child. The physician should also involve members of the multidisciplinary CF team, since all of them will be in close contact with the patient during the pregnancy, and the ethical dilemmas facing the physician will apply to them as well (61). CONSENSUAL AGREEMENT TO PROCEED: THE GAME PLAN The final stage in negotiations is reaching a consensual agreement to proceed and developing a game plan. Inherent to any game plan should be an established mechanism for resolving conflict between the physician and patient. This should be done in the framework of the accepted first principles of the therapeutic alliance. PRACTICAL CONSIDERATIONS The process outlined above is complex and time-consuming. It need not be done at one session, and unless the positions of the physician and patient are relatively close, the discussions may be protracted. Members of the CF team should initiate a dialog with their patients regarding fertility early on. The initial discussion of issues surrounding pregnancy should occur in the late teen years. The physician can objectively provide information regarding the benefits and risks of pregnancy. This information can serve as the framework for women with CF as they begin the process of formulating their personal objectives regarding fertility and child-rearing issues. The patient who has general discussions about pregnancy with her physician at this age will be able to grow in her understanding of the risks associated with pregnancy in women who have CF. It is often quite difficult to convince a woman of the risks associated with pregnancy if she is only presented with such information after she has decided to become pregnant. It is possible that neither the personality of the physician nor the patient is conducive to fruitful negotiations. For meaningful dialog to take place between two parties, both sides must exhibit specific characteristics, including (1) mutual respect, (2) honesty, (3) truthfulness, (4) open-mindedness, (5) empathy, and (6) a willingness to alter beliefs (50). In the case of CF and pregnancy, the emphasis needs to be placed on both the physician and patient being forthright and flexible if a consensual agreement is to be reached. There is also the chance of unilateral action on the part of one of the sides. This can be especially troubling for the physician. Even if the patient, after becoming pregnant, reneges on previously agreed principles (e.g., taking specific medications), the physician and health team are morally obliged to provide her with medical services unless a suitable and equivalent alternative can be found. This is a risk, but it is a smaller risk if the physician and patient have built a strong relationship before embarking on the mutual venture of a high-risk pregnancy. Similarly, a woman with CF may already have initiated a pregnancy without prior consultation. In this case, the position of the physician and CF team is radically altered. If the pregnancy is highly risky and inadvisable, the physician may recommend termination to preserve the health of the mother. If the mother refuses, the physician and CF team may have no alternative but to fully cooperate with the mother, as lack of cooperation is a form of abandonment that can result in an even worse outcome. Still, it should be stressed to the prospective mother that the model presented above is still preferable to unilateral action, as adequate planning and prior agreement on a treatment plan will best ensure the safety and well-being of both mother and infant. For the future, it will also be important to keep updating our knowledge of pregnancy in women with CF. Several countries already have registries, and these registries should be expanded and integrated. As more is known about the consequences of pregnancy, information exchange between patient and physician will becomes more detailed and less ambiguous. With more precise information about outcomes, both the physician and patient can make decisions that are based on fact rather than supposition. Hopefully, as treatment for CF and CF pregnancy improves, the risk of pregnancy in CF may become sufficiently minimal as to preclude any moral dilemma for the physician or patient.
CONCLUSION FOOTNOTES Originally Published in Press as DOI: 10.1164/rccm.200605-598OE on November 16, 2006 Conflict of Interest Statement: None of the authors has a financial relationship with a commercial entity that has an interest in the subject of this manuscript. Received in original form May 3, 2006; accepted in final form November 16, 2006 REFERENCES
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