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Breathing exercises for inpatients with sickle cell disease.

Sickle cell disease (SCD) is a complex medical condition characterized by acute painful exacerbations often requiring hospitalization. The pain is the result of a gene mutation which causes an abnormal hemoglobin that results in "sickle-shaped" red blood cells, promoting vaso-occlusion and leading to both acute and chronic vascular inflammation. Mousa and colleagues (2010) noted an acute painful crisis, or pain episode, is characterized by a sudden onset of pain that might start in any part of the body, including the back, long bones, and chest. The pain ranges from mild to severe, and may be excruciating, deep pain felt in the bones and soft tissues. In Figure 1, SCD pain is illustrated by artist Hertz Nazaire (personal communication, June 20, 2012).

Significance of Research

Pain episodes, which can last from hours to days, are the primary reason for hospitalization of individuals with SCD. The average hospital length of stay for a diagnosis of sickle cell pain episode is 6 days in the United States, but the pain can last much longer (Brandow, Brousseau, & Panepinto, 2009).

During a patient's hospitalization for an acute pain episode associated with SCD, opioid analgesics are a significant part of the pain management protocol. Their administration requires frequent reassessment to provide optimal pain management in a timely manner (Brown, 2012). Further, opioids are problematic for some individuals; in particular, side effects may be intolerable. Additionally, use of opioids can lead to physiological problems, such as hyperalgesia or increased sensitivity to pain, physical dependence, respiratory depression, and withdrawal-related pain (Manchikanti, Benyamin, Datta, Vallejo, & Smith, 2010). Because of the pain of SCD and issues related to pain management, nonpharmacologic options for pain management should be part of the inpatient treatment plan.


The purpose of this study was to explore use of breathing exercises by individuals with SCD. The role of medical-surgical nurses in teaching breathing exercises as a complementary pain management strategy for inpatients with SCD also is discussed.

Literature Review

PubMed and CINAHL were searched for 2009-2014, with studies limited to participants age 19 or older, and published in English. Search terms included sickle cell, pain, breathing exercises, and nursing. These searches did not return any results. Notably, all studies were published more than 5 years ago or pertained to individuals younger than age 19. The lack of current literature on this topic further supports the need for this pilot study.

Yusef, Atrash, Grosse, Parker, and Grant (2010) explored characteristics of emergency department visits made nationally by patients with SCD. Although pain was the overwhelming reason for care-seeking, they found 5% of visits also included complaints of shortness of breath, breathing problems, or cough. In another study wherein all the participants had a chief complaint of pain, 29% also reported respiratory symptoms, 59% reported back pain, and 42% reported chest pain (Tanabe et al., 2010). Miller and colleagues (2012) reported 77% of all patients with SCD seeking care complained of chest or back pain that could impact the process of breathing. Furthermore, in individuals with SCD, recurrent respiratory symptoms (namely wheezing) were associated with increased occurrence of pain (Cohen et al., 2011), increased frequency of emergency department visits for pain (Glassberg et al., 2012), and increased morbidity.

Support of breathing is an important aspect of care for inpatients with SCD. Given the potential for central nervous system depression with opioid use, inpatients receiving opioids must have frequent assessments of blood pressure, pulse, and respirations before medication administration and periodically during use (Brown, 2012).

Hypoventilation, a significant concern for patients with SCD during opioid use, may contribute to acute chest syndrome (ACS) (Miller & Gladwin, 2012). Acute chest syndrome is defined as a new infiltrate on the chest x-ray that involves at least one lung segment. This syndrome may occur when the lungs are deprived of oxygen during a pain crisis. Although significant symptoms are not exhibited by all patients, ACS-associated signs and symptoms generally include a productive cough, hemoptysis, shortness of breath, hypoxia, chest pain, and chills (Livesay & Ruppert, 2012). Acute chest syndrome is second only to episodes of pain as the reason for hospitalization (Miller & Gladwin, 2012). Moreover, the highest mortality rates in this population are attributed to ACS and pulmonary hypertension. Consequently, incentive spirometry often is recommended for inpatients requiring opioid analgesia (Miller et al., 2012). The incentive spirometer supports goal-oriented inhalation that may prevent pulmonary complications, such as atelectasis and ACS, in individuals with SCD. Miller and colleagues reported the use of incentive spirometry at 10 puffs every 2 hours while patients were awake reduces the risk of ACS significantly; however, only 56% of their patients received protocol-recommended spirometry, although it was described as a safe and inexpensive intervention.

While opioids are an important part of the treatment plan for patients with SCD, their side effects and potential contribution to lung complications suggest a need for complementary pain management options. One such option is mind-body medicine therapy. According to the National Center for Complementary and Alternative Medicine (2012), mind-body medicine therapies are focused on the "interactions among the brain, mind, body, and behavior," with the purpose of using the mind to "affect physical functioning and promote health" (para. 1). Mind-body medicine therapy uses and increases the mind's ability to be alert and self-regulate symptoms. The literature has documented the effectiveness of mind-body medicine therapy interventions in helping to manage the pain and emotional distress associated with a variety of medical conditions, including SCD (Hassed, 2013; Majumdar, Thompson, Ahmad, Gordon, & Addison, 2013; Morone, Rollman, Moore, Qin, & Weiner, 2009; Thompson & Eriator, 2014). Yet to be tested, however, is the application of mind-body medicine therapy in patients with SCD in acute crisis in the hospital setting.


Design and Sample

For this descriptive pilot study, a convenience sample of adults with SCD (N=84) was recruited from the adult outpatient sickle cell clinic affiliated with the Comprehensive Sickle Cell Program at the University of North Carolina at Chapel Hill. Inclusion criteria were a diagnosis of SCD, the ability to understand English, and age of at least 18.


Demographics. The demographics questionnaire requested information to describe the sample by age, sex, education, and questions specific to SCD, such as number of crises per year that require hospitalization and the SCD genotype.

Use of complementary strategies survey. This survey was developed by the third and fourth authors along with one other expert in SCD, providing content validity. The survey was used deliberately to assess patients' current use of as well as the likelihood they would use complementary strategies (e.g., prayer, meditation, guided imagery, breathing exercises, sound/music, gentle yoga) to manage pain. Participants also were asked if they discussed these strategies with their health care providers or if their providers offered information about these strategies. The discussion will focus on results related to breathing exercises because it may be the complementary strategy that has the most effect on the pulmonary complications of SCD.


All eligible participants were approached for enrollment by a member of the study team. After providing written informed consent, participants completed the demographic form and the Use of Complementary Strategies Survey. Data collection took place in a private area of the clinic. University institutional review board approval was obtained from The University of North Carolina at Chapel Hill prior to the start of the study.


The sample was 63.1% female (n=53) and 36.9% male (n=31). The average age of respondents was 37 (range 20-74). Participants reported an average of 2.9 crises per year (SD=3.8) requiring hospitalization; they said they had pain requiring medication or interfering with activities at least four times per week (SD=4.1). Of the sickle cell-specific demographic variables, a significant correlation existed between education and number of SCD crises (r=-0.35, p=<0.001). For the 84 participants, 65.5% reported their SCD type as sickle cell anemia, 11.9% as sickle cell C disease, and 9.5% beta thalassemia; 13.1% did not know their SCD type.

Regarding breathing exercises, approximately 36% (n=30) of the surveyed adults with SCD already used breathing exercises for pain management. Twenty-three percent (n=19) indicated they would try breathing exercises for pain management. Only one provider stated they would never try breathing exercises. In addition, only 4.8% (n=4) of respondents stated they had discussed breathing exercises with their health care providers and only one provider stated a health care provider had taught them to use breathing exercises.


Although results may not lead to care-seeking requiring hospitalization, findings are consistent with existing literature indicating pain interference is a significant issue for adults living with SCD (Darbari, Balias, & Clauw, 2014; Smith et al., 2008; Thompson & Eriator, 2014). Additionally, literature indicates young adults tend to try to treat pain at home and avoid the health care system due to past treatment (Jenerette, Brewer, & Ataga, 2014). With pain interfering with activities an average of four times per week and requiring medication, perhaps respondents use breathing exercises and other self-taught strategies to avoid the health care system whenever possible. Moreover, some individuals with SCD already use breathing exercises to manage pain without the benefit of training by a health care provider. Individuals with SCD are willing to learn breathing exercises to help manage the pain of SCD.


Although this pilot study provides insight into the use of complementary strategies for pain management in individuals with SCD, respondents are from one comprehensive program that serves a large geographic area. Responses from this convenience sample may be different than a larger random sample of adults with SCD; therefore, the results cannot be generalized. Additionally, the data are all self-reported as no demographic or sickle cell-related information was confirmed from medical records. Finally, respondents in this study were a convenience sample of adults with SCD, ages 20-74. The experience of care-seeking may be different for other adults with SCD who may have developed different skills.

Nursing Implications

Because many of the participants already use breathing exercises, some without the benefit of instruction by a health care provider, medical-surgical nurses could assess and teach a few modalities of breathing techniques during an inpatient hospitalization. Moreover, nurses could encourage patients to engage in a daily practice so they are able to incorporate these techniques as a part of their self-care management routine to manage stress or pain, or reduce the intensity of painful crises.

Recommendations for Future Research

This pilot study may be a first step in uncovering the potential for use of complementary strategies such as breathing exercises by individuals with SCD. A need exists for researchers to uncover additional information about use of complementary strategies in adults with SCD. Individual interviews or focus groups may be approaches to gathering additional information. Because breathing exercises have the potential to mitigate pain and pulmonary complications in adults with SCD, research needs to discern how to integrate breathing exercises effectively into the nursing care of inpatients with SCD.


Sickle cell disease is a complex chronic disease with acute, painful exacerbations. Although opioids are a prominent part of the management of painful crises, complementary therapies such as breathing exercises also may play an important role. In addition to encouraging the use of incentive spirometry, medical-surgical nurses can teach breathing exercises for individuals to use anywhere and without any equipment. Breathing exercises as a relaxation technique may be an important self-care management tool for individuals with SCD. Self-care is a vital aspect of care in chronic illnesses such as SCD (jenerette, Brewer, & Leak, 2011).

Acknowledgement: This project was supported by Award Number UL1RR025747 from the National Center for Research Resources. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center for Research Resources or the National Institutes of Health. This work was also supported by a NIH/NINR postdoctoral fellowship (T32NR007091) to the first author.


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Nadine Matthie, PhD, RN, CNL, is Postdoctoral Fellow, The University of North Carolina at Chapel Hill, Chapel Hill, NC.

Cheryl A. Brewer, PhD, RN, is Manager, Clinical Trials Operations & Project Management, Duke Cancer Care Research Program/Center for Learning Health Care, Duke Clinical Research Institute, Durham, NC.

Vera L. Moura, MD, is Adjunct Instructor, The University of North Carolina at Chapel Hill, Chapel Hill, NC.

Coretta M. Jenerette, PhD, RN, CNE, is Associate Professor, The University of North Carolina at Chapel Hill, Chapel Hill, NC.
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Title Annotation:Patient Education
Author:Matthie, Nadine; Brewer, Cheryl A.; Moura, Vera L.; Jenerette, Coretta M.
Publication:MedSurg Nursing
Article Type:Report
Geographic Code:1USA
Date:Jan 1, 2015
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