The Anticipatory Politics of Improving Childhood Survival for Sickle Cell Disease

Abstract

Crediting scientific discovery for prolonging life is pervasive in biomedical histories of the genetic blood disorder, sickle cell disease. This includes the preventive strategies, such as newborn screening, that have underwritten the success of its life-extending interventions. Newborn screening is a technology that relies not only upon intact health infrastructures but also expertise and enhanced vigilance on the part of caregivers to anticipate complications while they are still open to circumvention. This paper posits that even after overcoming institutional barriers to make newborn screening equitably available, care and vigilance are resources that are themselves subject to what i term anticipatory politics, where structural conditions also stratify expectations for the future, including the affective appeal of medical innovations. This paper elaborates the paradigm of anticipatory politics through an ethnographic examination of newborn screening to connect the comprehensive care practices that have improved survival for sickle cell disease, and as the burden of mortality shifts to young adulthood, to expose how those who are resourced to care for these futures preferentially stand to benefit from preventive interventions.

The biggest challenge for families going forward is this sense of hope. Families want to know that their child… when I see the 1-month-old or 2-month-old who has just been diagnosed… they want to know that their 18-year-old is going to finish high school and that they are going to have a normal life…. I look to that future for every one of those new borns. … I look the parents in the eye and say, “Your child, as a result of medical advances, research that has been done by others prior to your child’s birth has greatly increased the chances of your child reaching her 18th birthday, and I hope to be able to celebrate that day with you when it comes.” DeBaun (2013)

Scientific and clinical innovations have transformed sickle cell disease from a genetic disease that had been largely fatal in childhood to a chronic, but still life-shortening condition.¹ As recently as fifty years ago, the majority of children born with sickle cell disease in the US were not expected to reach adulthood. Today, in high-income countries where public health systems have implemented newborn screening and disease-specific comprehensive care practices, the likelihood of surviving to age eighteen with sickle cell disease has never been better. Epidemiologic studies of US children born since the 1980s report upward of 95 percent surviving to age eighteen (e.g., Quinn et al. 2010). Centers in the United Kingdom and elsewhere in Europe have cited survival to age eighteen that approaches 98–100 percent (Telfer et al. 2007).

Unique to sickle cell disease is that its prevalence has predominated among groups that are also disadvantaged minorities in high-income countries such as the US and France, where life chances are already stratified by race, ethnicity, immigration status, and class. An estimated 100,000 people have sickle cell disease in the US, and this population is largely composed of black and Hispanic-identified individuals (Hassell 2010).² Interpersonal and interinstitutional power dynamics, as well as broader social and economic structures, inevitably have coproduced clinical outcomes for these populations.³ These social contexts generate human capabilities⁴ to remake worlds and to envision viable futures. Among these imagined or anticipated futures are the world-making possibilities that adults conjure on behalf of children. In turn, expectations for the future shape what kinds of treatments, research, and clinical decision-making are being proposed and practiced on behalf of the patients.

I offer anticipatory politics as an analytic framework to conceptualize these clinical and experimental spaces as they have been informed by stratified expectations, in this case, for the future lives of children. To this end, this paper presents an historical ethnography of the knowledge production of newborn screening to discern how comprehensive care practices for sickle cell disease became accepted as clinical standards and operationalized as national health policy. Biomedical efforts to extend and improve upon patients’ life chances are fraught with overt and implicit assumptions about researchers, health-care providers, and families’ intentions when seeking care and pursuing treatment options. Yet even conflicted and contradictory desires among these stakeholders ultimately align with modes of care to define and justify who is eligible for treatment interventions and under what circumstances.

Given that blood travels throughout the body, the biomedical assumption is that the sequelae of sickling red blood cells occur everywhere. These effects become cumulative when unmitigated by disease-modifying therapies. Because sickle cell anemia provokes incremental changes to the body, identifying who is at risk of complications involves recognizing and defining evidence of disease progression, ideally, prior to the appearance of overt symptoms. Currently, no treatment outside of hematopoietic cell transplantation halts the progression of sickle cell disease.⁵ Transplant practitioners have noted that the extensive diagnostic testing that precedes a bone marrow transplant can lead to “discovering” previously undetected signs of organ dysfunction. Learning to anticipate these signals of future pathology while they are still amenable to circumvention has underwritten the success of life-prolonging interventions for sickle cell disease.

Nonetheless, predicting the future health of people with sickle cell disease continues to defy scientific explanation and clinical expertise. Even when they share the same genetic mutation, patients’ symptom presentation and clinical course can vary. For some, the red blood cells’ predisposition to distort and accumulate in blood vessels manifests predominantly as painful crises or life-threatening acute chest syndrome. For others, strokes or leg ulcers present as the primary complications of the body’s long-term exposure to the contents of ruptured blood cells due to chronic hemolytic anemia. A subset of patients may experience relatively quiescent symptoms as children until the effects of long-term organ damage become apparent in adolescence or young adulthood. Outward signs of suffering—and the lack thereof—during childhood are imperfect forecasters of disability and early mortality in adult survivors.

Much clinical knowledge is operationalized today as practice-based prognostication. This blending of anticipation, care, and practice has a specific name in Anglophone Western medicine: “anticipatory guidance.” As performed in medical contexts, anticipatory guidance denotes a range of predominantly—though not exclusively—preparatory strategies. Anticipatory guidance is a form of care that is inherently oriented to the future. As such, anticipatory guidance is especially prominent in the parlance and practice of pediatric health care. The latitude for defining what counts as performing anticipatory guidance ranges from the specific and self-limited preparation of a patient for a one-time procedure to capacious forms of support—informational, material, emotional—that implicate open-ended futures.⁶ Seeped in potentiality, anticipatory guidance transfers knowledge and expertise for an ever-widening breadth of conditions. While anticipatory guidance can assume secondary and tertiary forms, its role in preventive medicine and child development exemplifies the pediatrician’s primary care orientation, where the imaginable future for a child holds an inverse temporal relationship to the life course.

In conceptualizing anticipatory politics, I incorporate the affective elements of medical innovation, where expectations for improving upon mere survival empower “speculation the authority to act in the present” (Adams, Murphy, and Clarke 2009, 249). Building upon Ahmed’s (2004) theory of affective economies, these future-looking, non-individuated emotions that are subsumed in care work and research expertise surface to underwrite social and political projects. In order to articulate the “politics” of anticipatory politics, this case study employs an analysis of sickle cell disease medicine that is conversant with the historical contexts and political conditions of its knowledge production. Practicing anticipatory guidance not only generates new matters of clinical and experimental concern, these practices also instill affective states of preparedness, “a strategy that must continually keep uncertainty on the table” (Adams, Murphy, and Clarke 2009, 250). That is, even as stakeholders implement anticipatory guidance with the objective to mitigate future risk and forestall complications, this form of expertise remains oriented toward inherently uncertain futures. Tacking between the practice and affect of anticipatory guidance, this paper considers how patients, parents, and health-care providers are actively entangled in “affective economies of fear, hope, salvation and precariousness oriented temporally towards futures already made ‘real’ in the present” (Adams, Murphy, and Clarke 2009, 260). In this way, the clinical interventions that shape lives in the present also relocate expectations for the future.

In assembling the case, I draw methodologically from Mol (2008) and Puig de la Bellacasa (2011) in their efforts to apprehend the sciences by attending to their practices and politics as “matters of care.”⁷ In bracketing praxis and care, I write against reducing the critique of clinical decision making to categories of scientific versus nonscientific expertise, such as in the way the “art and science of medicine” tends to dichotomize value-laden interpersonal relationships vis-à-vis value-neutral scientific knowledge. To this end, I utilize oral histories and in-depth interviews, participant observation in clinical settings and research and advocacy meetings, and document analysis of the medical literature.⁸ Using these research methods, I elucidate how newborn screening was deemed scientifically valid and therefore actionable as social policy. With this brief history of sickle cell disease medicine, I also consider how the conditions that improved childhood survival have made the proximate risk and deferred promise of hematopoietic cell transplantation a plausible research and treatment proposition. To employ the paradigm of anticipatory politics is to recognize how orientations toward the future, such as in the clinical care work of anticipatory guidance, are subject to structural relationships and power dynamics as key determinants for whether and how scientific knowledge becomes empowered to act upon these futures.

Blackboxing Childhood Survival: The Success of Newborn Screening

In 2014, the American Society of Hematology conferred special recognition to Dr. Michael DeBaun for his career achievements in clinical research for sickle cell disease. This honor includes giving the Ernst Buetler memorial lecture at the Society’s annual meeting, a conference that regularly convenes over 25,000 international attendees. Among the colleagues DeBaun commended during his lecture was Dr. Elliott Vichinsky, whom he described as “a pioneer that initiated newborn screening for sickle cell disease.” DeBaun cited Vichinsky’s findings, now three decades old, involving a cohort of eighty-one children in Northern California whose sickle cell disease was identified through newborn screening, and how they experienced 1.8 percent mortality over seven years of follow-up. By comparison, the sixty-four children who had not undergone newborn screening and attended the same comprehensive sickle cell center experienced 8 percent mortality (Vichinsky et al. 1988).

The children in the second group received their diagnosis of sickle cell disease after three months of age: on average, by twenty-one months. Of the five children who died in the second group, two were diagnosed only at the time of their fatal septic illness, the culmination of a precipitous and overwhelming bacterial infection. Vichinsky’s paper quietly proffered that both groups from their center fared better than the much higher mortality of 15–30 percent reported in other study cohorts and geographic regions (Vichinsky et al. 1988, 753). I asked Vichinsky what explained the difference in mortality between the study groups, apart from the timing of their diagnosis, given that the same sickle cell program had followed all families. He noted that teaching parents how to provide anticipatory care operated differently when their children’s sickle cell disease was detected later in life, and particularly when in extremis. For families who were proximate to a child’s life-threatening crisis at the time of their diagnosis, attempts to transfer preventive knowledge seemed less successful than with the family of an infant who had not yet manifested signs of illness (Vichinsky, pers. comm., 2015).

The benefits of newborn screening to individuals and populations affected by sickle cell disease are no longer subject to dispute. This claim is made with confidence by physician leaders in sickle cell disease medicine, including Debaun and Vichinsky. Newborn screening programs have been credited for increasing survival for children with sickle cell disease in the US, and these results also have been replicated worldwide. Because fetal hemoglobin is still present at birth, newborns are temporarily protected against the effects of sickling red blood cells. From delivery onward, the sickle hemoglobin varieties begin replacing the residual fetal circulation in earnest. Sickling red blood cells predominate by four to six months of age, when children may begin to display signs of illness, depending on the severity of their disease. To obtain a diagnosis of sickle cell disease in early infancy within the purview of an intact public health system provides families the occasion to receive expert evaluation and counseling, ideally well in advance of the emergence of symptoms and complications. Among the key interventions of newborn screening is the retention of families to health-care institutions specialized in the practice of sickle cell disease comprehensive care, where future risk is managed and contained through enhanced forms of vigilance, attentiveness, and care.

My framework for analyzing newborn screening for sickle cell disease draws from Latour’s (1999) important observations on blackbox⁹ processes and “the way scientific and technical work is made invisible by its own success”:

When a machine runs efficiently, when a matter of fact is settled, one need focus only on its inputs and outputs and not on its internal complexity. Thus, paradoxically, the more science and technology succeed, the more opaque and obscure they become. (p. 304)

In an analogous fashion, newborn screening for sickle cell disease assembled new matrices of care and practice that were vital to its success as a screening technology. Here, I explore further how public health systems recruited families to health-care institutions that would in turn enlist these caregivers’ expertise to act as first responders to potentially lethal complications of sickle cell disease in childhood.

The term “newborn screening” implies a diagnostic technology to test an infant at birth for an existing and often (as of yet) unapparent condition. Newborn screening is in fact the accepted shorthand for an assemblage of coordinated practices that operate across multiple institutions, within a specific period of time, and under a particular health policy mandate. The newborn screening test is performed on blood drawn from a pinprick to the infant’s heel, blotted to a filter paper card, dried, and sent on to a laboratory facility to detect hematologic markers of sickle cell and certain other diseases. Taken alone, however, the heel prick test is insufficient to comprise an efficacious newborn screening program.

This limitation was substantiated when newborn screening for sickle cell disease was first implemented in 1975 in New York State. The initial rollout of newborn screening in New York City was “dismal” (Grover 1989, 819), as nearly half of the positive filter paper tests never underwent confirmatory testing, leading to numerous missed diagnoses. In a case-by-case analysis of the eight children known to have died of sickle cell disease in New York City between 1976 and 1978, all had succumbed after fewer than forty-eight hours of infectious illness, and none was receiving regular medical care. Five of the families were unaware of their children’s underlying condition. Four of the children who died had undergone screening for sickle at birth, but only one had their initial abnormal test confirmed. These findings strengthened the conclusion that “accurate screening of newborns in the absence of a comprehensive follow-up program is of limited value” (Grover 1989, 821). Not until additional federal funding was secured in 1978 did newborn screening in New York begin to function as intended. Hence, a successful newborn screening program is comprised of interlocking practices that include reliable initial and confirmatory testing, effective conveyance of results to families, and efficient linkage of affected children to health-care institutions capable of providing disease-specific care.

Two anticipatory strategies linked to new born screening remain unchanged since the 1970s. Both entail redirecting parents’ and health care providers’ threshold for evaluating and treating episodic illness. The first approach induces caregivers to detect and respond to fever more aggressively than they would for an otherwise healthy child. With each instance of fever, parents are coached to immediately bring their children to a health-care institution for urgent evaluation. The second strategy teaches parents how to palpate the abdomen to detect an enlarged spleen, whose sudden appearance can indicate a precipitous pooling of blood in the organ, a life-threatening condition. These practices do not necessarily reduce the occurrence of complications; rather, they compel families to seek care in the earlier stages of an acute illness, when still responsive to antibiotics or blood transfusion. This heightened vigilance coupled with more assertive attention from the health-care system ultimately reduces the risk of a fatal outcome.

The role of anticipatory guidance in preventive medicine and child development exemplifies the pediatrician’s primary care orientation, where providing “well baby” and “well child” care are hallmarks of the practice of general pediatric medicine. In this way, anticipatory guidance assumes greatest import during the earliest stages of a child’s life. This is also the case when the trajectory of disability or chronic illness overlays the expected development of a child. Dr. Kwaku Ohene-Frempong¹⁰ pointed to how newborn screening allowed for health-care workers not only to provide anticipatory guidance for children before they showed symptoms but also to reimagine the natural history of sickle cell disease:

… I think health workers were also learning that this disease was not necessarily a death sentence … [E]ven when we didn’t have any major innovative therapies, taking good care of them in an ordinary sense, and anticipating their problems—good “well baby” care, good “well child” care, making sure they get all their immunizations—were saving lives. (Ohene-Frempong with author, April 16, 2013)

In a similar vein, Serjeant (2013) indicates that the instrumental interventions linked to newborn screening are also an adjunct to the new knowledge that comes with diagnosis, when “[t]he mother [sic] also receives an important psychological message that although her child has a currently incurable disease, much can be done by close observation and simple interventions to improve its outcome”¹¹ (p. 3). Articulating newborn screening as the assemblage of anticipatory care practices not only resists blackboxing an efficacious medical technology but also considers its structural politics. Moreover, in the paradigm of anticipatory politics, the capacity to provide enhanced vigilance became a “matter of care” (Puig de Bellacasa 2011), an affective practice, and a vital resource that was coproductive with—not merely adjunct to—the distribution of economic and social capital required for newborn screening’s success. This affective appeal to the expectations of families and health-care practitioners instilled the capability to imagine and nurture alternate futures, thereby undoing prior assumptions of what sickle cell disease should look like.

While early reports of significantly improved child survival were being ascribed to newborn screening, as the initial experience in New York demonstrated, these gains were not uniform. As of 1981, the Cooperative Study of Sickle Cell Disease, a nationwide multicenter observational study organized and funded by the US National Institutes of Health (NIH), had not observed a decline in the (still unacceptable) 30 percent case fatality rate for sepsis among the children in their sample who were born during these earlier years of newborn screening (Gaston et al. 1986, 1594). While a handful of US states had adopted newborn screening, another technology was being added to the armamentarium for pediatric sickle cell disease: prophylactic penicillin. Penicillin does not modify the effects of sickling red blood cells to the body; rather, it attenuates infection from certain bacteria.¹² Although penicillin was no longer a new drug in the 1980s, some practitioners began prescribing it as a daily medication for infants and children with sickle cell disease.

The Cooperative Study itself held no intervention component, but one of the Study’s organizers, pediatrician Marilyn Gaston, pushed leadership at the National Heart Lung and Blood Institute (NHLBI) to fund the Prophylactic Penicillin Study (PROPS), a multicenter randomized, blinded, placebo-controlled trial of penicillin prophylaxis for children with sickle cell disease (Vichinsky with author, April 15, 2013). The 215 participants were drawn from a national sample of twenty-three centers across the US. After fifteen months of follow-up, the results of PROPS were compelling enough to end the study prematurely: thirteen episodes of sepsis due to pneumococcal infection occurred in the placebo arm, compared with two cases among children in the prophylactic penicillin arm (Gaston et al. 1986).

Vichinsky stressed the underlying importance of experimentally demonstrating both morbidity and survival benefit among the children who were assigned twice daily doses of penicillin. As he explained in our interview:

[N]ewborn screening is the critical thing, and penicillin was the tool that allowed public health departments to accept it, because you had a pill to give them. What [families] really needed was not a pill. What they really needed was counseling, education, [knowing] when to come in [to the hospital], how to manage pain, how to feel for the spleen. … They needed comprehensive care, and no one wanted to support that as an indication for newborn screening. It was too kind of touchy-feely stuff, so … no one would support it from the traditional schools. (Vichinsky with author, April 15, 2013)

Experimentally demonstrating the effectiveness of penicillin in reducing fatal sepsis recursively justified the presence of the newborn screening programs already in existence and added impetus for their expansion elsewhere. It could no longer be argued, especially by those who dismissed the “touchy-feely”-ness of comprehensive care practices, that sickle cell disease was untreatable and therefore unworthy of an early diagnosis. In response to the PROPS findings, an NIH Consensus Conference recommended universal newborn screening for sickle cell disease (NIH, Office of Medical Applications of Research 1987). Congress approved additional funds to support state-sponsored newborn screening programs during the same year.

Penicillin’s historic contribution to sickle cell disease care and practice exceeded its direct antimicrobial effects. Prior to PROPS, only five states had required newborn screening for sickle cell disease. By 1990, newborn screening programs expanded to twenty states; within a decade of the NIH Consensus Conference, forty states required universal newborn screening¹³ (Benson and Therrell 2010). The clinical study of penicillin provided the policy justification to broadly implement newborn screening programs across the US, and it was through newborn screening processes that children with sickle cell disease obtained the anticipatory care they needed.

Newborn screening’s import as the vehicle to comprehensive care became evident to me even outside my more narrowly defined field sites. While waiting for my son to finish his dance class, another parent, pointing to the conference bag I was carrying, approached me. The mother explained that her younger son had sickle cell disease, and he was “doing great.” She mentioned that her family had refused vaccinations and adhered to a strict diet to maintain his health, but still endorsed their close relationship to the large hospital-based sickle cell center, where her son was first referred as an infant. “Because he is doing so well, they leave us alone,” she interpreted as the doctors’ response to their unorthodox treatment choices. Before we parted, she said, “I just wanted to ask, do you know Dr. H?” In fact, Dr. H was among my study participants, though he no longer worked at the sickle cell center they attended. She was delighted. “He was the doctor who first explained sickle cell disease to us. He taught us how to feel for the spleen. We would come back [for our follow up visits], and he would make sure we knew how to do it right. We’ll never forget him.” In an evocation of Mol’s (2008) “logic of care,” I pose this as an example where the “medical home”¹⁴ was successfully maintained in the presence of conflicting stake holder positions, where the health-care institution continued to care for the patient, even though his family had decided to opt-out of some of the recommended preventive care practices. As Mol (2008) explains, “… even in moments that leave a lot to be desired, health-care professional do not write people off as bad investments. … In the logic of care, patients are not a target group, but crucial members of the care team” (p. 26).

Ambiguities of Improving Longevity: Childhood Survival, Adult Mortalities

The NHLBI of the NIH has promoted the narrative of progress through biomedical discovery in an oft-cited slide of life expectancy with sickle cell disease (see Figure 1). The time line begins in 1910, when the first clinical case report for sickle cell disease was published in the US (Herrick 1910) and ends in 2000. The graph depicts a modest rise in life expectancy until 1970, when the curve angles sharply upward. Annotated near the base of this surge is the National Sickle Cell Disease Control Act. Signed into law by President Nixon in 1972, the Act directed federal funds to health services and medical research specifically for sickle cell disease. The remainder of the time line is punctuated by interventions asserted as effective by clinical trials. Yet newborn screening, which gained ground in urban centers in the 1970s and reached nearly half of states by 1990, is curiously absent from the Figure.

Figure 1.

Sickle cell life expectancy (National Heart, Lung, and Blood Institute; US Department of Health and Human Services). Newborn screening (NBS) prevalence annotation added.

Also notable is the relentlessly smooth rise in the life expectancy curve, whose slope predates any of the scientific proofs—for penicillin, hydroxyurea, or chronic transfusions—that the graph means to highlight. These interventions scarcely register as inflection points against their time line; yet this portion of the life expectancy curve does hew to the expansion of newborn screening and comprehensive care programs for sickle cell disease. When I queried Ohene-Frempong about the NHLBI slide, which he had used in his own presentations, he readily acknowledged the inadequacy of representing the impact of scientific discovery in this way:

[T]he “smooth rising thing,” some of it is not justified, because we haven’t done something beyond newborn screening and chronic transfusions that has in a major way affected the potential for dying for young adults with sickle cell disease…. They’re living longer with many, many challenges. (Ohene-Frempong with author, April 16, 2013)

Carlton Haywood, Jr., PhD, was born with sickle cell disease in the 1970s. Haywood, now in his fifth decade, recalled desperately seeking reassurance from his parents as a child, after happening upon pamphlets that informed him the life span of a person with sickle cell disease was eighteen years (Haywood with author, April 11, 2015). The life expectancy cited in the 1970s proved to be a moving target, and Dr. Haywood and others of his generation found themselves crossing over this epidemiological line. Since 2000, however, median life expectancy remained stubbornly in the forties. These statistics never sit well for the generation of patients who first benefited from early childhood interventions but now find themselves catching up to the life expectancy curve.

The available epidemiologic data indicate that childhood mortality for sickle cell disease has shifted to young adulthood (e.g., Quinn et al. 2010; Lanzkron et al. 2013). In this way, the achievement of a child surviving to adulthood and the tragedy of an adult life foreshortened became “two sides of the same coin.”¹⁵ Whereas death certificate data demonstrate a consistent annual reduction in childhood mortality from 1979 to 2005, adult mortality due to sickle cell disease in the US has steadily increased compared to the general population, with an average age at death of forty-two years among females and thirty-eight years for males¹⁶ (Lanzkron et al. 2013). Today, sickle cell disease mortality is regularly invoked to justify research objectives or advocate for improving health-care resources for adults. At scientific meetings, projected life expectancy statistics often inhabit a presenter’s background slide. Halima, who had recently entered her fifth decade with sickle cell disease, mentioned to me as we left one of these sessions together, “I always tear up a little bit, when they mention mortality.”

Improved outcomes in children have shifted advocacy discourse to the disparities between pediatric sickle cell disease patients and their own prospects for accessing appropriate care as adults. Improving the survival of pediatric patients makes the public health system a victim of its own success, when these systems are unequipped to provide appropriate care for their adult population. Comprehensive care programs and disease-specific specialists are often readily available to pediatric populations in the US; but even in geographic regions without overall physician shortages, obtaining optimal care for adults remains a challenge. This is partly due to a lack of dedicated providers and institutional support,¹⁷ but also a result of losing federally mandated health insurance. Depending on the state, loss of Title V coverage for children with sickle cell disease and other special health care needs contributes to lapses in health-care access in adulthood.¹⁸ A study of emergency department discharge data in California grimly observed that young adults with sickle cell disease became high utilizers of emergency room services, upon losing their pediatric health insurance (Wolfson et al. 2011).

The NIH Consensus Conference that had endorsed newborn screening for sickle cell disease produced rights-based language to advocate for the appropriate clinical management of children: “Comprehensive specialized care should be the right of every child who is affected by a clinically significant hemoglobinopathy. Economic, social, cultural, or geographic concerns should not limit access to this care but should be taken into account when structuring a follow-up program” (p. 1207) (NIH, Office of Medical Applications of Research 1987). This rights-based language was not extended to the adult population thirty years ago. Today, the need to maintain disease-specific care in adulthood—a time when the long-term effects of sickling red blood cells manifest as damage to kidneys, lungs, bones, and brain—is greater than ever. The Affordable Care Act of 2010 may benefit adults with sickle cell disease in accessing health insurance in certain states; its impact, especially given the current administration’s attacks to this legislation, is as of yet unknown. Conspicuously, comprehensive care is not merely a clinical concern, but a political proposition that depends upon social commitments to systems of care that promote health equity.

Elliott Vichinsky has bemoaned the “too rosy picture” of sickle cell disease that pediatric hematologists can present to patients and families today. If a perception has been advanced that sickle cell disease is relatively benign or even “gets better” in adulthood,¹⁹ I suggest that this is less an artifact of pediatricians painting an untroubled future than the current state of affairs: childhoods with sickle cell disease have become more manageable and predictable, and the existing care systems that have produced these outcomes concomitantly obscure knowledge of the possibility of decline in adulthood. Even pediatricians with more intimate knowledge of the historical legacy of sickle cell disease or of the challenges ahead in the life course will eventually relinquish their role as their charges’ primary health-care providers. This is among the reasons that Vichinsky advocates for improving upon anticipatory care practices for the adult population, akin to those performed in the pediatric setting,²⁰ and promotes his center’s model for a comprehensive sickle cell program that serves children and adults.

Anticipatory politics provides an analytic response to a call in the study of science for “greater temporal and spatial reflexivity… that… move[s] away from normative futures and … sees expectations [as] rooted in particular times and places” (Brown 2003, 18). Translating scientific knowledge to clinical practice (and its study, dubbed today as “implementation science”²¹) is rarely a linear process. Nor do scientific questions emerge outside of the soupy milieu of ongoing practices, embedded in their own social and material contexts and affective concerns for the future. In organizing scientific knowledge production as constitutive of practices and affective economies, anticipatory politics provides a methodological grounding to articulate abstract and contingent hopes for the future, as they become located in the present and the past. Groundbreaking interventions for sickle cell disease, such as newborn screening, prophylactic penicillin, and other comprehensive care practices, also are preventive strategies whose success is mediated by expertise in vigilance and care. Care and vigilance require, and are themselves, resources that are subject to social and economic stratification. These paradigm shifts are made explicit through the analytic framework of anticipatory politics.

This paper employs anticipatory politics to unpack the Latourian blackbox of research priorities, clinical practices, and public health policies that transformed sickle cell disease from a fatal disease of childhood to a chronic condition. With a diminishing hazard of dying in childhood, families and caregivers, patients and advocates, and researchers and health-care providers are redirecting concerns and expectations to the longer term, including the deferred potential for children to thrive as adults. The very likelihood that children with sickle cell disease reach adulthood is proliferating uncertainty for the longevity and health quality of adult lives—uncertainties that also produce grounds for considering novel and risky interventions. When the first bone marrow transplant was performed for a child with sickle cell disease in 1983, the procedure also treated an otherwise fatal leukemia, thus providing its ethical justification (Johnson et al. 1984).²² Today, the urgency of a more pressing affliction is no longer required for patients with sickle cell disease to assume the risk and hardship of transplant. Understanding why health-care providers have proposed that children undergo a procedure that exposes them to the short- and long-term risks of chemotherapy, prolonged hospitalization, and months of social isolation—and why families agree to undertake it—takes on new valences when the goal of treatment is not an express effort to stave off a more proximate death, but the hope and expectation for a durable cure. To opt for a child to undergo transplant in an effort to circumvent morbidities in adulthood is to extend the politics of anticipation to its logical conclusion. The appeal of embracing the risk of hematopoietic cell transplantation is not to improve upon survival in childhood but to imagine a life without sickle cell disease.

Biography

Gina Jae is a doctoral candidate of medical anthropology in the Department of Sociomedical Sciences, Mailman School of Public Health, Columbia University.