Nurses are the largest health care workforce in the United States, but in our patients’ eyes, we may not have much force at all. In 2010, the Institute of Medicine (IOM) published a report on the Future of Nursingand made recommendations that just as easily could have come from patients with sickle cell disease (SCD), had we listened.
During the 2011 faculty retreat at the University of Illinois at Chicago College of Nursing, we deliberated on the IOM recommendations. Recently, as I read anecdotes of patients with SCD from my research on perceived injustice—a context-bound unfairness of treatment that a person receives from important others, such as health care providers—I noticed striking similarities with the IOM recommendations.1,2
SCD is an inherited blood disorder affecting approximately 80,000–100,000 people in the United States, mostly of African descent; it’s among the most common fatal inherited diseases. Pain crisis, its hallmark and most disabling complication, is severe and recurrent. Patients with SCD often interact with nurses within the health care system asking for help to control their pain.
As I read the research anecdotes from patients with SCD about their interactions with nurses, the patients’ message was clear: nurses lack enough training about SCD to provide competent pain care. They echoed the IOM’s recommendations:
- Implement nurse residency programs
Some nurses recognize inadequacies in their education but blame their incompetency in caring for patients with SCD on nursing education programs. One patient wrote about a nurses’ comment to her, “When you attend school to become an RN, the [nursing] school [doesn’t] go into a lot of details on SCD.” Nurse residency programs could provide nurses with opportunities for exposure to diverse patient populations and engender necessary confidence for clinical practice. One may contend that lack of funding is a barrier for residency programs to implement this education. But it is costlier to the U.S. health care system not to do so. In 2006 alone, the cost of acute health care visits for patients with SCD was $2.4 billion.3 If nurse residencies could help reduce the 33.4% and 22.4% of patients with SCD re-admitted within 30 days and 14 days, respectively, for pain crisis, there would be possible substantial cost savings.4
- Ensure that nurses engage in lifelong learning
Patients with SCD say that nurses need lifelong learning to champion their care. One patient asserted that “there needs to be more training on [SCD] and more understanding of why it is necessary to treat [SCD] pain crisis with narcotics ASAP to help patients get control of the pain and move toward ending the painful crisis.” Another said most of her problems come “from ignorant nurses.” Lack of time could factor as a deterrent for continuous learning. But with advancement in technology and Internet access, nurses can search and learn about a disease in no time and with little cost.
- Prepare and enable nurses to lead change to advance health
Patients with SCD, particularly those who are knowledgeable about their conditions, want a partnership with nurses for better control of their pain. Nurses are well positioned to be their champions, but need personal and professional growth. A patient with SCD applauded a nurse who embodied this recommendation when she wrote, “Few nurses took training from Dr. X, and my nurse came to me and said that she now understood more on our pain and that she [learned] a lot about SCD that she didn’t know.” This statement gives me hope for our profession. We need more efforts to sustain it and advance health for those with SCD.
It is in nurses’ best interests to engage in personal and professional development. The consequence of inaction could be detrimental. We may be the largest health care workforce, but without much force we lose patients’ trust in our competency, profession, and ability to ease their suffering. The IOM report is a “dawn of a new day” for nurses, and we are in an excellent position to advance health care. We should take advantage of its recommendations and this opportunity to improve the nursing profession so that all patients maintain the trust they have bestowed upon us.
The work cited in this publication was made possible by funding from the NIH Basic and Translational Research Program (1 U54 HL090513) and the Computerized PAINRelieveIt for Adult Sickle Cell Disease (R01 HL078536). The author thanks the patients with sickle cell disease for their study participation; the staff at the Comprehensive Sickle Cell Center for their support of the work; her colleagues (Diana Wilkie, Ph.D., R.N., F.A.A.N.; Robert Molokie, M.D.; and Crystal Patil, Ph.D.); and the other members of the research team for their assistance with study implementation (Marie Suarez, Ph.D.), data management (Young Ok Kim, Dr.P.H., R.N., C.H.E.), and data collection (Harriett Wittert, B.S.N.; Jesus Carrasco, B.A.; and Veronica Angulo, B.A.).
- J.A. Colquitt, “On the Dimensionality of Organizational Justice: A Construct Validation of a Measure,” Journal of Applied Psychology, 86 (2001), 386–400.
- M. Elovainio, J.E. Ferrie, D. Gimeno, R. De Vogli, M. Shipley, E.J. Brunner, and M. Kivimaki, “Organizational justice ans sleeping problems: The Whitehall II study,” Psychosomatic Medicine, 71 (2009), 334–340.
- S. Lanzkron, C.P. Carroll, and C. Haywood, Jr., “The burden of emergency departments use for sickle-cell disease: an analysis of the national emergency department sample database,” American Journal of Hematology, 85 (2010), 797–799.
- D.C. Brousseau, P.L. Owens, A.L. Mosso, J.A. Panepinto, and C.A. Steiner, “Acute care utilization and rehospitalizations for sickle cell disease,” Journal of American Medical Association, 303 (2010), 1288–1294.
Response From Nnenna Ukachi, Samantha VanHorn, Revathy Sundaram
This case raises many ethical issues. Jehovah’s Witnesses are widely known in the medical community for their refusal of blood and blood products. According to multiple biblical texts, blood is regarded as sacred, representing life, and a gift from God. As a result, blood is acceptable only when used for the atonement of sins, and may not be ingested or transfused in any form. A Jehovah’s Witness who accepts a blood transfusion essentially dissociates him/herself from the religion and is shunned by other members.1 TH cited religious conflicts and made her refusal of transfusions apparent as soon as she was legally able to do so. For members of the clinical team who have cared for TH from infancy, this may have been a difficult decision to accept. In addition, she appeared to be acutely ill and in impending cardiac failure from the description of this crisis episode. The first step would be to maintain her hemodynamic stability and then transfer TH to the ICU for close monitoring with fluid hydration and aggressive pain control. Prevention and treatment of other causes of anemia and diminishing iatrogenic blood loss would be essential. Although not emergent, the use of hormonal contraceptives should be considered to minimize iron deficiency from menstrual loss.
To improve oxygenation, the risks and benefits of alternate and experimental therapies, which have been used in adult patients who refuse the use of blood, should be discussed with TH. Although experience with these therapies is limited in pediatrics, they should be offered to a patient in this situation. It would also be helpful to discuss the case with a physician from an established “bloodless” program. Such centers have experience in the treatment of Jehovah’s Witness patients who refuse transfusions. A consultation with an internist-hematologist might be helpful. In addition to offering medical advice for management, an adult hematologist might also assist the pediatric team in gaining appreciation for an adult-oriented approach. Erythropoietin analogs and nonblood oxygen carriers should be offered to the patient.
It is also important to determine if the patient is psychologically and physically capable of making decisions. For TH, her newly empowered status as a legal adult and the confrontation with a difficult and potentially life-altering decision such as this may be overwhelming, but the care team should ensure that her decisions are not made under duress or pressure from family or friends, which could potentially invalidate them. In addition to clinical support, she and her family would require continued psychosocial and emotional support. It would be crucial to educate TH and her family on the risks and benefits of the treatment options available, determine the validity of her decision, and to provide optimal supportive care regardless of the decision and its outcome. Involving the Hospital Ethics Committee is crucial, but more important is identifying an individual who TH respects, trusts, and with whom she is comfortable speaking. Typically such an individual is from the medical team and has an established therapeutic relationship with TH. Examples might include a social worker or child life specialist. Such an individual can often advocate for the patient/family and serve as a liaison between the patient/family and the medical team, helping to bridge differences in a nonconfrontational manner.
Pediatric providers seldom face situations in which their decisions to save a life are questioned or not accepted. When the patient is a minor, they usually can seek and obtain a court order for treatment. When the patient is a young adult, everything changes. Pediatricians then feel anger and helplessness. Their personal beliefs and the lifelong training direct them to provide treatment. Legal restraints demand that they stand by and let a patient die. Education and posttreatment support sessions with the pediatric staff are important to address the anxiety and feelings of guilt and confusion. It would also serve to remind them that they were not alone and that resources were available to help them during a period of crisis.
Response From Wynne Morrison
In the United States, adults with decision-making capacity have a right to refuse medical therapy, even if the therapy is thought to be life-saving.2 This standard applies to Jehovah’s Witnesses who would rather die than receive blood transfusions. A medical team may challenge a refusal only if they think that the patient does not understand the therapy, alternatives, or consequences of refusal or is otherwise impaired (eg, suicidally depressed).
In pediatrics, however, it has become relatively common practice to transfuse children whose parents are Jehovah’s Witnesses when such transfusions are absolutely necessary. This practice is not universal or without controversy,3–6 but many pediatricians obtain court orders to override parental refusals when all alternatives to transfusion have failed. A central argument for overriding parents is that the child has not yet made a mature choice to be a member of a particular religious community and to follow that community’s doctrines.7 We justify transfusing the child because there is a chance the child will decide transfusions are acceptable as he or she grows older. An emotional argument buttresses the philosophical one: clinicians are distressed at the possibility of letting a child die for lack of what is seen as a relatively routine therapy.
We recognize that decision-making capacity emerges over time as an adolescent matures. A 16-year-old patient’s informed opinion may, therefore, deserve more respect and consideration than that of a 12-year-old,5,8 depending on the maturity of the child, experience with decision-making, and the implications of the decision. The legal cutoff for adulthood marks an arbitrary, abrupt transition, but it exists nonetheless.9
Pediatricians are sometimes unprepared for a patient’s transition to legal adulthood at age 18. We may forget to ask about advance directives, may habitually look to parents to continue to make decisions, and may become accustomed to using the courts to transfuse a patient without a family’s consent. For TH’s physicians in the vignette described, it must be very distressing to realize that they may be legally required to accept her refusal. Reaching the age of legal maturity may seem like a death sentence. A clinical ethics consultant in this case would want to explore how strenuous TH’s objections had been in the past, what her relationship with her religious community is like, and how well she understands the consequences of refusal. TH should be privately offered the opportunity to accept a transfusion without revealing to her family or community leaders that it took place. Any possible additional therapies to support her without transfusion should be aggressively pursued.
Some authors claim that many competent adult Jehovah’s Witnesses accept transfusion as long as they do not have to explicitly agree to it.10,11 But how is a clinician to distinguish between, on the one hand, a patient with a principled determination to die rather than receive a blood transfusion and, on the other, a patient who would not mind being transfused as long as he or she did not have to agree to it? There is no good way.
Sometimes, ethics crises arise that could, perhaps, have been prevented. It would certainly be best for a thorough exploration of these issues to begin before the patient’s 18th birthday and before the patient is in an acute, decompensated situation. If TH and her long-term physicians had agreed on a working plan before the crisis occurs, all would have been more comfortable accepting it.
If given an opportunity to plan in advance, TH’s physician might have said to her: “I understand that you do not wish to accept blood transfusions and that this issue is very important to you. I have been caring for you as your physician for years and am committed to continuing to do so. Many times in the past, I have given you blood, and I am convinced that I have saved your life or helped treat your pain when I did. You are about to turn 18, and when you do, it will be really hard for me if avoiding transfusions puts your life at risk. I therefore want to let you know that I will give you transfusions if I think they are absolutely necessary, but I will not make you sign a consent form and will not let anyone in your family or your community know that you were given blood. If imagining being in such a situation is absolutely horrifying to you, then I need you to send me a letter in writing now saying that you do not want me to ever transfuse you. I would then not give it to you even if the situation became life-threatening. If you were dying because of not getting blood, I would make sure you were comfortable. I also need to let you know that if you go to another physician who has never transfused you before, chances are that doctor will honor your wishes as an adult to refuse blood. I hope that you do not choose this option, but understand if you do.”
Such a strategy forces the patient to take action (writing a letter or finding another doctor) to “actively” refuse blood transfusions. It allows the patient to passively accept blood by simply not writing a letter and continuing to follow up with the same physician. The physician would need to make sure this is a legally tenable option both at the institution and in the state where he or she is practicing by talking with the hospital attorneys and planning how to best document the conversation. Perhaps a judge will have to be identified who is willing to confidentially support such a plan. The need for legal clarity may make such a nuanced approach too difficult to manage.
In this case, if the medical team, support staff, and ethics consultants (who are hopefully involved) are convinced that she understands the consequences of her decisions and is expressing her true wishes, then they should respect her refusal. They should talk with her about what other options make sense in light of her refusal of blood. Should the care at this point focus on her comfort rather than invasive measures like intubation?
It may be difficult for the medical and nursing team if TH dies. They should be offered support. If possible, any who are uncomfortable caring for her given the circumstances should be able to change assignments.
This case reminds us that we should avoid becoming too comfortable overriding our patients’ wishes when we disagree with their decisions, just because we are sometimes able to do so. It also reminds us to be proactive in anticipating the legal and ethical shifts that occur when our patients turn 18, no matter what the clinical circumstances. And, as always, we should continue to care for and about our patients, even when forced to acknowledge that we disagree.12 If TH dies in this case, it will be because she chose to die for her beliefs.