A Novel Approach To Pain Management in Persons With Sickle Cell Disease

Mary Myers Ellen J. Eckes

ickle cell disease (SCD) is an inherited genetic blood disorder that affects red blood cells (RBCs). Neonates have more fetal hemoglobin (HgbF) than the normal adult who has HgbA (the normal adult component of hemoglobin). Normal infant blood screen reflects HgbFA, while an infant with the sickle cell trait or disease will reflect HgbFS. Sickle cell hemoglobin will appear as HgbAS for a patient who carries the trait for SCD, meaning he or she could transmit the genetic trait to any children. An individual carr)/ing the trait for SCD may or may not have symptoms; this would depend on additional genetic make-up of their hemoglobin. An individual with HgbSS has received the genetic trait for SCD from both parents, and has the disease. This is significant because it is the hemoglobin in the RBCs that is responsible for carrying oxygen. The life span of a RBC in sickle cell disease is only 1020 days, compared to 120 days of a normal RBC (National Heart, Lung & Blood Institute, 2008).

Sickle cell disease (SCD) is an illness that affects red blood cells. Patients with SCD can have chronic pain or acute pain episodes, which must be managed with medical therapy Although many options are available for pain management, utilization of subcutaneous patient-controlled analgesia for pain management has positive outcomes for patients in both pain management and satisfaction.

Hemoglobin S typical of SCD does not perform in a normal way, leading to the distortion of RBCs into a characteristic sickle shape similar to the letter C. Such malformed RBCs cannot pass easily through blood vessels and capillaries (see Figure 1). This not only decreases the amount of oxygen that can reach the body's tissues, but causes blood vessel occlusion that restricts blood flow and contributes to ischemia. Additionally, sickle cells attach to the endothelium and trigger an inflammatory cascade that leads to further

tissue damage. This is known as a vaso-occlusive crisis or vaso-occlusive event (VOE). It is particularly significant when these events involve microvasculature (e.g., in bone marrow) or macrovasculature (e.g., in organs) (Ballas, 2007; Ellison & Shaw, 2007). VOE can cause additional sequences secondary to tissue damage. These changes involve biochemical, neurologic, and electrochemical changes in a process known as nociception. Nociception causes the patient to perceive acute pain (Ballas, 2007). Patients with SCD can have acute or chronic pain, or a mixture of both. Onset of sudden and acute pain leads to an acute crisis and usually necessitates immediate hospital management. An acute crisis may also be precipitated by dehydration, infection, cold weather, extreme temperature change, asthma, or increased emotional stress (Howard, Thomas, & Rawle, 2009; Johnson, 2008). Involvement of the bone marrow can precipitate fat embolization to the lungs, creating a cascade

of symptoms, such as severe pain, fever, cough, chest pain, leukocytosis, pulmonary inflltrates, tachycardia, and tachypnea. This constellation of symptoms is called aaite chest syndrome (ACS) and is a medical emergency (Ellison & Shaw, 2007; Rees, Williams, & Gladwin, 2010). Treatment involves pain management, antibiotics, respiratory therapy (including bronchodilators), oxygen, hydration, and diagnostic testing to establish other underlying or coexisting causes (Ellison & Shaw, 2007; Hernandez & Patterson, 2009). If the hemoglobin decreases significantly during ACS, a transfusion is given or in severe cases, an exchange transfusion is performed (Rees et al., 2010). Due to the constant hemolysis of blood cells, there is an increased incidence of vasculopathy "characterized by systemic and pulmonary hypertension, endothelial dysfunction and proliferative changes in the intima and smooth muscle of blood vessels" (Rees et al., 2010, p. 2019). When hemolysis occurs, hemoglo-

Mary Myers, MSN, RN, PCCN, is Senior Clinical Research Nurse III, Medical/Surgical/Telemetry Unit, National Institutes of Health Clinical Center, Bethesda, MD. Ellen J. Eckes, MSN, ARNP, FNP-BC, CCRN, is Clinical Nurse Specialist, Nursing and Patient Care Services, National Institutes of Health Clinical Center, Bethesda, MD.

MEDSURG

September-October 2012 Vol. 21/No. S

293

Ciinical Practice
FIGURE 1. Malformed RBCs Cannot Pass Easily through Blood Vessels and Capillaries

Q Normal red blood e ^ rtoi c8li(RBC)

RBCs Sow f f ^ wtthinblooves!!

Abnormal, s k k M , nKt blood eelte (sickle cells) SicktoceSs y^

rces-sactron of siMe am

naproxen [Naprosyn]; ketorolae [Toradol]; adjuvant medieations (antihistamines, antidepressants, antiemeties, laxatives), and other nonmedieine treatment modalities, sueh as physical therapy, transeutaneous electrical stimulation, heat therapy, acupuncture, massage, biofeedbaek, and relaxation exercises, can be utilized (Bailas, 2007). An acute pain crisis is an emergent medical condition requiring timely treatment strategies. Pharmacologie approaches to pain management include oral (PO), intramuscular (IM), eontinuous intravenous (IV) infusion, and IV or subeufaneous (SQ) patient-eontrolled analgesia (PCA) (Solomon, 2010). All approaches are utilized at the National Institutes of Health Clinical Center (NIHCC) (2011). In addirion to pain management, patients should receive adequate hydration, oxygen therapy, and treatment with concurrent therapies, such as antibiotics, blood products, iron chelation (for iron overload due to multiple transfusions), and symptom management (Ballas, 2007; Ellison & Shaw, 2007; Montalembert, 2009).

Abnormal hemoglobin formstians thatcauM sickle shatM

Incidence, Prevalence, and Barriers for Appropriate Treatment

In Africa, approximately 200,000 babies are born with SCD annually, and 60,000 to 100,000 worldwide (Hayword et al, 2009). In the United States, 72,000 individuals have SCD (Ballas, 2010). Individuals with SCD often are stereotyped with: behaviors of drug dependency, as "frequenf flyers" to emergency departments, unemployed, undereducated, and underinsured (Ballas, 2010). To combat these negafive perceptions, health eare providers should receive education regarding disease process, life-threatening complications, and above all pain management (Hayword et al, 2009). Additionally, health eare providers should aecept patients with SCD with an open mind, be receptive to the individual's specific needs, and use compassion to guide subsequent acfions (Howard et al., 2009; Solomon, 2010).

Source: National Heart, Lung & Blood Institute, 2011

bin is released into the plasma and may inhibit nitric oxide production. Low levels of nitric oxide may contribute to hypercoagulability and lead to additional complications such as avascular necrosis (AVN), leg uleers, venous clots, chronic renal failure, and stroke (Howard et al., 2009; Rees et al, 2010). Treatment of chronic pain is accomplished by fargefing a known cause or initiating generalized medical management (Johnson, 2008). For example, if a patient's chronic pain is related to leg ulcers, treat-

ment is centered on wound care. Treatment for pain associated with AVN may involve additional health care specialties (e.g., orthopedics, surgery, physical rehabilitation) and medications such as non-steroidal anti-inflammatory drugs and/or gabapentin (Neurontin) (Howard et al., 2009; Johnson, 2008). Chronie pain is treated with long-aeting opioids for extended management and short-acting opioids to address breakthrough pain (Howard et al., 2009). In addition, non-opioid (acetaminophen [Tylenol]); ibuprofen [Motrin];
September-October 2012

294

Vol. 21/No. 5 MEDSURG

A Novel Approach to Pain Management in Persons with Sickle Cell Disease

Literature Search
A literature search was performed utilizing the following key words: subcutaneous PCA, sickle cell anemia AND subcutaneous PCA, sickle cell patient AND subcutaneous PCA, and sickle cell patient AND PCA. Databases searched included Puh Med, emBase, CINALH Plus, and Scopus. There was no parameter set on years for the search. Search findings yielded many articles written in the late 1990s and early 2000s. A great deal of the information regarding fhe use of subcutaneous PCA reflected treatment in postoperative patients, burn patients, end-of-life, and oncology patients. The salient features of this research are reported in brief detail to document scientific evidence for best practice. PCA pain management is equal fo IV injection or continuous infusion (Doyle, Morton, & McNichol, 1994; Moulin, Kreeft, Murray-Parsons, & Bouquillon, 1991; White, 1990). Van Beers and co-authors (2007) conducted a randomized, controlled study (N=19) to compare efficacy of PCA administration wifh continuous infusion of opioids and noted dose requirements, as well as the duration of use, were less in fhe PCA group than the group with continuous infusion. The mean daily pain scores were comparable and side effects of nausea and constipation were improved in the PCA group. Ellison and Shaw (2007) discussed the assessment of VOE with suggested pharmacological management, including opioid administration via oral, subcutaneous, intravenous, transdermal, and rectal routes. These authors suggested opioids could be administered effectively via continuous infravenous drip, intermittent intravenous bolus, and PCA device. Schein, Hicks, Nelson, Sikirica, and Doyle (2009) conducted a meta-analysis of 55 randomized controlled trials involving use of PCA for pain managemenf and concluded PCA provided better

pain control and enhanced patient satisfaction. Urquhart, Klapp, and White (1988) compared the efficacy of SQ-PCA fo convenfional IVPCA. They concluded no differences existed in analgesic scores or overall side effects between the two groups, but dose requirements were higher for SQ than IV. Doyle and co-authors (1994) compared IV PCA and SQ PCA administration of morphine in pafients following appendectomy, reporting SQ PCA was as effective and safe as IV PCA administration. SQ opioid administration is equigesic with IM opioid administration (Semple, Upton, Macinfyre, Runciman, & Mafher, 1997; White, 1990). Comparing pharmacokinefics of morphine IM and SQ authors found blood concenfrations of the drug were comparable. Solomon (2010) discussed one barrier to the utilization of SC administration for pain management as a lack of physician and patient education. This form of treatment was recommended to be utilized in the future, even if IV access was established. Although some of the references discussed in the literature search are dated, they provide evidence for best practice. A current literature search did not reveal recent studies, although Johnson (2008) did describe three international case studies outlining the effecfive use of subcutaneous PCA administration for pain management. The lack of available information demonstrates the need to disperse information regarding this pain management technique and potential future research in this area.

DVT formation from clumped sickle cells in fhe vessel, placement of peripherally inserfed central lines may be difficulf and further limit vascular accessibility NIHCC (2011) suggests utilization of SQ PCA for pain management. Benefits include the following: (a) decreased patient dependence on IV access before receiving pain medication; (b) IV access is available for administration of blood products, iron chelating agents, antibiotics, and other medications that may not be compatible with opioid therapy; (c) minimal fo no risk of infiltration when compared wifh IV administration; and (d) increased patient satisfaction due to timeliness of intervention.

PCA PrograiTiming and Administration

PCAs are programmed in a variety of ways: basal rate (the patient receives a set dose as a continuous infusion) and/or bolus dose (the patient requests a prescribed amount of medication in a set time by pressing a button) (Ellison & Shaw, 2007). For example, if a patient is faking long-acting opioids and the medical team wants to continue the dose without interruption, the bolus-only option may be chosen to manage breakthrough pain and treat the acute crisis. Pain management is multifaceted. To administer opioids effectively by SQ PCA, the nurse must perform continual assessment of the patient's pain, satisfaction with therapy, vital signs, and infusion site. Assessing the patient's pain and devising a suitable treatment plan to achieve patient satisfaction are essential. A patient's ability to perform bolus dosing with SQ PCAS is critical and should be evaluated by the nurse. Once the decision is made to begin treatment with SQ PCA, the drug primarily utilized at NIHCC (2011) is hydromorphone (Dilaudid), but occasionally fentanyl or morphine is used as well. Supplies include a pump, batteries, documentation sheet, appropriate medication, tubing, SQ needle, chlorohexidine gluconaate (Chloraprep), and dressing supplies. The PCA pump settings are

Clinical Practice
Patients with SCD have problems with vascular access (Johnson, 2008). These problems are due to veins that are hard to cannulate, dehydration, decreased blood flow, and multiple accesses over time (Ellison & Shaw, 2007). As a result of

MEDSURG

September-October 2012 Vol. 21/No. S

295

Clinical Practice
FIGURE 2. A Subcutaneous Injection into the Fatty Layer of Tissue (pinched up to give the injection) Under the Skin FIGURE 3. injection Sites

Source: NIHCC, 2012

Source: NIHCC, 2012

checked independently by two nurses against the written medical order, validating the Five Rights and the carrier solution (NIHCC, 2011). For comfort and ease of mobilify, opioids administered via SQ PCA for SCD pain crisis are given via the abdomen, though other sites such as the back, arms, and thighs can be utilized as well (see Figures 2 & 3). When assessing the patient for an acceptable site for SQ administration, the nurse should avoid painful or ecchymosed areas, enlist patient participation in site selection, and reassess the site per hospital policy (Justad, 2009). SQ sites may remain
FiGURE 4. PCA Supplies

accessed, with assessment, dressing care, and management based on the same criteria as an IV site (see Figures 4 & 5) (NIHCC, 2011). At NIH, the policy for PCA management via IV or SQ is comprehensive. Upon initiation of PCA, assessments are performed every 30 minutes for 1 hour, gradually titrating to every 4 hours (NIHCC, 2011). More frequent assessments should occur when there is a change in patient condition, an increase in dose administered, or other care requirements that necessitate close observation. Assessment of patients receiving SQ PCA includes respiratory rate, oxygen saturation, sedation level, blood pressure, and heart rate. The patient's pain is assessed by rating his or her pain intensity on a 0-10 Visual Analog Scale, reviewing the PCA program, documenting the amount of use and demand by the

patient, and determining if the goals of pain management, safety, and satisfaction are being met. In addition, the SQ insertion site is assessed for irritation, ecchymosis, and dressing intactness. All these interventions and assessments are documented on the PCA flow sheet and within the electronic medical record (see Figure 6) (NIHCC, 2011). Successfijl pain management is achieved when the patient identifies satisfactory resolution or control of pain and the nursing assessment indicates the patient is oriented with stable vital signs. Complications may occur with SQ PCA that include oversedation, infection, pruritus (local and systemic), and equipment failure. The nurse must be aware of these potential complications, assess for any possible problems, and be ready fo intervene appropriately. Many complications can be avoided with accu-

FIGURE 5. Site Utilization for PCA

296

isrR TJ n September-October 2012 Vol. 21/No. S M E D S U G

s I isr Gt.

A Novel Approach to Pain Management In Persons with Sickle Cell Disease FIGURE 6. PCA Flow Sheet
Drug/Concentration Route: D Peripheral (IV) n Central (IV) G Epidural Location: n Epineural Location: D SC Loci< Level: 3. Container D 4. Full Lock D

|o

I
S

ig

II
oo ma <E

um E
Yes No Yes No Yes No Yes No Yes No Yes No Yes No

If

'Indicators RA = Routine Assessment BC = Bag Change PC = Program Change c e = Change of Caregiver

Level of Sedation (LOS):

1 =
2 = Drowsy/Dosing intermitteniy 3 = Sleep/Easily Aroused 4 = Sleeping/Difficult to Arouse 5 = Confused/Hallucinating 6 = Unable to Arouse

"'Motor and Sensory Assessment: Details documented in electronic medical record (CRIS) or other approved medical record form.

COMMENTS:

Source: NIHCC, 2011

rate patient assessment, vital signs monitoring, competence with all equipment, and identification of potential problems. Insertion sites that are reddened should be rotated and all sites monitored for further signs of infection. Locally occurring pruritus may be managed by changing the site, while systemic pruritus may require medical interventions such as diphenhydramine (Benadryl), lotions, or low-dose IV naloxone (Narcan) drip. Many patients with SCD admitted to the NIH for the first time have not had the experience of SQ PCA. However, with appropriate education and trial of this altemative pain management process, many patients ac-

cept this form of treatment and have a positive outcome.

Conclusion
Effective pain management and positive patient satisfaction can be achieved with the use of SQ PCA for SCD pain crisis. While there are some disadvantages and potential complications, there are many more advantages to using this form of therapy. The need for continued evaluation and research of this mode of pain management is acknowledged, specifically in patients with

REFERENCES Ballas, S.K. (2007). Current issues in sickle ceil pain and its management American Society of Hematology Education Program Book. Retrieved from http:// asheducationbook.hematologylibrary.org /contenV2007/1/97.long Ballas, S.K. (2010). Self-management of sickle cell disease: A new frontier. Journai of the Medicai Association, 702(11), 10421043. Doyle, E., Morton, N., & McNichol, L. (1994). Comparison ot patient-controlled analgesia in children by IV and SC routes of administration. British Journai of Anaesthesia, 72, 533-536. Ellison, A.M., & Shaw, K. (2007). Management of vasoocclusive pain events in sickle cell disease. Pdiatrie Emergency Care, 23(11), 832-841.

MEDSURG

September-October 2012 Vol. 21/No. S

297

Clinical Practice
Haywood, C, Beach, M.C., Lanzkron, S., Strouse, J.J., Wilson, R., Park, H., ... Segal, J.B. (2009). A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease. Journal of the National Medical Association, 10^^Q), 10221033. Hernandez, S., & Patterson, GE. (2009). What you need to know about acute chest syndrome. Nursing2009, 6, 42-45. Howard, J., Thomas, V.J., & Rawle, H.M. (2009). Pain management and quality of life in sickle cell disease. Expert Review Pharmacoeconomics Outcomes Research, 9(4), 347-352. Johnson, L. (2008). Management of pain due to sickle cell disease. Journai of Pain and Paliiative Care Pharmacotherapy, 22('\), 51-54. Justad, M. (2009). Continuous subcutaneous intusion: An efficacious, cost-effective analgesia alternative at the end of life. Home Healthcare Nurse, 27(3), 140-147. Montalembert, M. (2009). Current strategies for the management of children with sickle cell disease. Expert Reviews Hematotogy, 2(4), 455-463. Moulin, D.E., Kreeft, J.H., Murray-Parsons, N., & Bouquillon, A.I. (1991). Comparison of continuous subcutaneous and intravenous hydromorphone infusions for management of cancer pain. Lancet, 337(8739), 465-468. National Heart, Lung & Blood Institute. (2008). Sickie celi anemia. Washington, DC. Retrieved from hftp://www.nhlbi.nih.gov/ health/dci/Diseases/Sca/SCA.Whatls. html National Heart, Lung & Blood Institute. (2011 ). What is sickie ceil anemia? Retrieved from http://www.nhlbi.nih.gov/health/ health-topics/topics/sca/ National Institutes of Health Clinical Center (NIHCC). (2012). Giving a subcutaneous injection. Retrieved from http://www.cc. nih.gov/ccc/patient_education/pepubs/ subq.pdf National Institutes of Health Clinical Center (NIHCC). (2011). Standard of practice: Care of the patients receiving continuous and patient controlled analgesic infusions. Retrieved from hftp://intranet.cc. nih.gov/nursing/practicedocs/SOP_PCA. PDF Rees, D.C, Williams, TN., & Gladwin, M.T. (2010). Sickle cell disease. Lancet, 376, 2018-2031. Schein, J.R., Hicks, R.W., Nelson, W.W., Sikirica, V, & Doyle, D.J. (2009). Patientcontrolled analgesia-related medication errors in the postoperative period: Causes and prevention. Drug Safety, 32(1), 549-559. Semple, T, Upton, R., Macintyre, P., Runciman, W., & Mather L (1997). Morphine blood concentrations in elderly postoperative patients following administration via an indwelling subcutaneous cannula. Anaesthesia, 52, 318-323. Solomon, L.R. (2010). Pain management in adults with sickle cell disease in a medical center emergency department. Journal of the National Medical Association, ?02(11), 1025-1032. Urquhart, M., Klapp, K., & White, P (1998). Patient-controlled analgesia: A comparison of intravenous versus subcutaneous hydromorphone. Anesthesioiogy, 69, 428-432. van Beers, E. J., van Tujin, C.F.J., Nieuwkerk, P.T., Friederich, PW., Vranken, J.H., & Biemond, B.J. (2007). Patient-controlled analgesia versus continuous infusion of morphine during vaso-occlusive crisis in sickle cell disease randomized controlled trial. American Journai of Hematoiogy, 82, 955-960. White, P (1990). Subcutaneous-PCA: An alternative to IV-PCA for postoperative pain management. The Clinicai Journal of Pain, 6, 297-300. ADDITIONAL READINGS Hopkins, D., Shipton E., Potgieter, D., Van derMerwe, C, Boon, J., De Wet, C, & Murphy, J. (1998). Comparison of tramadol and morphine via subcutaneous PCA following major orthopaedic surgery. Canadian Journai of Anaesthesia, 45(5), 435-442. National Heart, Lung & Blood Institute (NHLBI). (2004.). The management of sickle cell disease (No .04-2117; 4th ed.). Bethesda, MD: NHLBI Health Information Center. Sharpiro, B.S., Cohen, D.E., & Howe, C.J. (1993). Patient-controlled analgesia for sickle cell related pain. Journal of Pain and Symptom Management, 8(^), 22-28.

Various Factors Influence the Adoption of Ergonomics in Health Care Organizations

MEDSURC Nursing welcomes readers' comments and invites readers to share information with their colleagues through Letters to the Editor. Submission of a letter constitutes permission for its copyright and publication in MEDSURC Nursing. Letters are subject to editing. Please address your correspondence to: MEDSURC Nursing, East Holly Avenue Box 56, Pitman, Nj 08071; E-mail: msjrnl@ajj.com. Include name, address, and contact information with your letter.

Several barriers can challenge the facilitation of human factors science (ergonomics) (HFE) in health care organizations. If the HFE intervention or innovation is too complex or employees do not understand the benefits of HFE, adoption can be hindered. When employees can actually experience the positive impact of HFE on their day-to-day tasks (e.g., improved working conditions), it is more likely that these innovations will be accepted and used to their fullest over the long haul. Having individuals who champion the cause at the local user level, such as a head nurse, can influence the way other employees become early and lasting adopters. For details, see Carayon, P. (2010). Human factors in patient safety as an innovation. AppUed Ergonomics, 41(5), 657-665.

New Resource Helps Hospitals Get Up to Speed on Safety Culture

Hospitals working to improve the safety culture of their organizations have a new Web-based resource that provides practical information on the patient safety dimensions used in the Agency for Healthcare Research and Quality's Hospital Survey on Patient Safety Culture, available at http://www.ahrq.gov/qual/patientsafetyculture/hospsurvindex.

MELSURG
IST Tjr D E ^ s u asr o ,
298

isrR t jG II September-October 2012 Vol. 21/No. 5 M E D S U

s iisr C,

Copyright of MEDSURG Nursing is the property of Jannetti Publications, Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.