A Novel ApproachTo Pain Management in Persons

With Sickle Cell Disease

Mary MyersEllen J. Eckes

Sickle cell disease (SCD) is aninherited genetic blood dis-order that affects red bloodcells (RBCs). Neonates have

more fetal hemoglobin (HgbF) thanthe normal adult who has HgbA (thenormal adult component of hemo-globin). Normal infant blood screenreflects HgbFA, while an infant withthe sickle cell trait or disease willreflect HgbFS. Sickle cell hemoglobinwill appear as HgbAS for a patientwho carries the trait for SCD, mean-ing he or she could transmit thegenetic trait to any children. An indi-vidual carr)/ing the trait for SCD mayor may not have symptoms; thiswould depend on additional geneticmake-up of their hemoglobin. Anindividual with HgbSS has receivedthe genetic trait for SCD from bothparents, and has the disease. This issignificant because it is the hemoglo-bin in the RBCs that is responsiblefor carrying oxygen. The life span ofa RBC in sickle cell disease is only 10-20 days, compared to 120 days of anormal RBC (National Heart, Lung &Blood Institute, 2008).

Hemoglobin S typical of SCD doesnot perform in a normal way, lead-ing to the distortion of RBCs into acharacteristic sickle shape similar tothe letter C. Such malformed RBCscannot pass easily through bloodvessels and capillaries (see Figure 1).This not only decreases the amountof oxygen that can reach the body'stissues, but causes blood vessel occlu-sion that restricts blood flow andcontributes to ischemia. Addi-tionally, sickle cells attach to theendothelium and trigger an inflam-matory cascade that leads to further

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 manyoptions are available for pain management, utilization of subcuta-neous patient-controlled analgesia for pain management has positiveoutcomes for patients in both pain management and satisfaction.

tissue damage. This is known as avaso-occlusive crisis or vaso-occlu-sive event (VOE). It is particularlysignificant when these eventsinvolve microvasculature (e.g., inbone marrow) or macrovasculature(e.g., in organs) (Ballas, 2007; Ellison& Shaw, 2007).

VOE can cause additional se-quences secondary to tissue damage.These changes involve biochemical,neurologic, and electrochemicalchanges in a process known as noci-ception. Nociception causes thepatient to perceive acute pain (Ballas,2007). Patients with SCD can haveacute or chronic pain, or a mixtureof both. Onset of sudden and acutepain leads to an acute crisis and usu-ally necessitates immediate hospitalmanagement. An acute crisis mayalso be precipitated by dehydration,infection, cold weather, extremetemperature change, asthma, orincreased emotional stress (Howard,Thomas, & Rawle, 2009; Johnson,2008). Involvement of the bonemarrow can precipitate fat emboliza-tion to the lungs, creating a cascade

of symptoms, such as severe pain,fever, cough, chest pain, leukocyto-sis, pulmonary inflltrates, tachycar-dia, and tachypnea. This constella-tion of symptoms is called aaite chestsyndrome (ACS) and is a medicalemergency (Ellison & Shaw, 2007;Rees, Williams, & Gladwin, 2010).Treatment involves pain manage-ment, antibiotics, respiratory thera-py (including bronchodilators), oxy-gen, hydration, and diagnostic test-ing to establish other underlying orcoexisting causes (Ellison & Shaw,2007; Hernandez & Patterson, 2009).If the hemoglobin decreases signifi-cantly during ACS, a transfusion isgiven or in severe cases, an exchangetransfusion is performed (Rees et al.,2010).

Due to the constant hemolysis ofblood cells, there is an increased inci-dence of vasculopathy "character-ized by systemic and pulmonaryhypertension, endothelial dysfunc-tion and proliferative changes in theintima and smooth muscle of bloodvessels" (Rees et al., 2010, p. 2019).When hemolysis occurs, hemoglo-

Mary Myers, MSN, RN, PCCN, is Senior Clinical Research Nurse III, Medical/Surgical/TelemetryUnit, National Institutes of Health Clinical Center, Bethesda, MD.

Ellen J. Eckes, MSN, ARNP, FNP-BC, CCRN, is Clinical Nurse Specialist, Nursing and PatientCare Services, National Institutes of Health Clinical Center, Bethesda, MD.

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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 » ^wtthinblooäves!«!

©Abnormal, s k k M , nKt blood eelte(sickle cells)

SicktoceSs y^

rces-sactron of siMe am

AbnormalhemoglobinformstianásthatcauMsickle shatM

Source: National Heart, Lung & Blood Institute, 2011

bin is released into the plasma andmay inhibit nitric oxide production.Low levels of nitric oxide may con-tribute to hypercoagulability andlead to additional complicationssuch as avascular necrosis (AVN), leguleers, venous clots, chronic renalfailure, and stroke (Howard et al.,2009; Rees et al, 2010).

Treatment of chronic pain isaccomplished by fargefing a knowncause or initiating generalized med-ical management (Johnson, 2008).For example, if a patient's chronicpain is related to leg ulcers, treat-

ment is centered on wound care.Treatment for pain associated withAVN may involve additional healthcare specialties (e.g., orthopedics, sur-gery, physical rehabilitation) andmedications such as non-steroidalanti-inflammatory drugs and/or gaba-pentin (Neurontin®) (Howard et al.,2009; Johnson, 2008). Chronie pain istreated with long-aeting opioids forextended management and short-act-ing opioids to address breakthroughpain (Howard et al., 2009). In addi-tion, non-opioid (acetaminophen[Tylenol®]); ibuprofen [Motrin®];

naproxen [Naprosyn®]; ketorolae[Toradol®]; adjuvant medieations(antihistamines, antidepressants, anti-emeties, laxatives), and other non-medieine treatment modalities, suehas physical therapy, transeutaneouselectrical stimulation, heat therapy,acupuncture, massage, biofeedbaek,and relaxation exercises, can be uti-lized (Bailas, 2007).

An acute pain crisis is an emer-gent medical condition requiringtimely treatment strategies. Pharma-cologie approaches to pain manage-ment include oral (PO), intramuscu-lar (IM), eontinuous intravenous(IV) infusion, and IV or subeufa-neous (SQ) patient-eontrolled anal-gesia (PCA) (Solomon, 2010). All ap-proaches are utilized at the NationalInstitutes of Health Clinical Center(NIHCC) (2011). In addirion to painmanagement, patients should re-ceive adequate hydration, oxygentherapy, and treatment with concur-rent therapies, such as antibiotics,blood products, iron chelation (foriron overload due to multiple trans-fusions), and symptom manage-ment (Ballas, 2007; Ellison & Shaw,2007; Montalembert, 2009).

Incidence, Prevalence, andBarriers for AppropriateTreatment

In Africa, approximately 200,000babies are born with SCD annually,and 60,000 to 100,000 worldwide(Hayword et al, 2009). In the UnitedStates, 72,000 individuals have SCD(Ballas, 2010).

Individuals with SCD often arestereotyped with: behaviors of drugdependency, as "frequenf flyers" toemergency departments, unemployed,undereducated, and underinsured(Ballas, 2010). To combat these nega-five perceptions, health eare providersshould receive education regardingdisease process, life-threatening com-plications, and above all pain manage-ment (Hayword et al, 2009). Ad-ditionally, health eare providersshould aecept patients with SCD withan open mind, be receptive to the indi-vidual's specific needs, and use com-passion to guide subsequent acfions(Howard et al., 2009; Solomon, 2010).

294 September-October 2012 Vol. 21/No. 5 MEDSURG

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

Literature SearchA literature search was performed

utilizing the following key words:subcutaneous PCA, sickle cell anemiaAND subcutaneous PCA, sickle cellpatient AND subcutaneous PCA, andsickle cell patient AND PCA. Databasessearched included Puh Med, emBase,CINALH Plus, and Scopus. There wasno parameter set on years for thesearch. Search findings yielded manyarticles written in the late 1990s andearly 2000s. A great deal of the infor-mation regarding fhe use of subcuta-neous PCA reflected treatmentin postoperative patients, burn pa-tients, end-of-life, and oncologypatients. The salient features of thisresearch are reported in brief detailto document scientific evidence forbest practice.

• PCA pain management is equalfo IV injection or continuousinfusion (Doyle, Morton, &McNichol, 1994; Moulin, Kreeft,Murray-Parsons, & Bouquillon,1991; White, 1990).

• Van Beers and co-authors (2007)conducted a randomized, con-trolled study (N=19) to compareefficacy of PCA administrationwifh continuous infusion of opi-oids and noted dose require-ments, as well as the duration ofuse, were less in fhe PCA groupthan the group with continuousinfusion. The mean daily painscores were comparable and sideeffects of nausea and constipa-tion were improved in the PCAgroup.

• Ellison and Shaw (2007) dis-cussed the assessment of VOEwith suggested pharmacologicalmanagement, including opioidadministration via oral, subcuta-neous, intravenous, transder-mal, and rectal routes. Theseauthors suggested opioids couldbe administered effectively viacontinuous infravenous drip,intermittent intravenous bolus,and PCA device.

• Schein, Hicks, Nelson, Sikirica,and Doyle (2009) conducted ameta-analysis of 55 randomizedcontrolled trials involving use ofPCA for pain managemenf andconcluded PCA provided better

pain control and enhancedpatient satisfaction.

• Urquhart, Klapp, and White(1988) compared the efficacy ofSQ-PCA fo convenfional IV-PCA. They concluded no differ-ences existed in analgesic scoresor overall side effects betweenthe two groups, but doserequirements were higher for SQthan IV.

• Doyle and co-authors (1994)compared IV PCA and SQ PCAadministration of morphine inpafients following appendecto-my, reporting SQ PCA was aseffective and safe as IV PCAadministration.

• SQ opioid administration is equi-gesic with IM opioid administra-tion (Semple, Upton, Macinfyre,Runciman, & Mafher, 1997;White, 1990). Comparing phar-macokinefics of morphine IMand SQ authors found bloodconcenfrations of the drug werecomparable.

• Solomon (2010) discussed onebarrier to the utilization of SCadministration for pain manage-ment as a lack of physician andpatient education. This form oftreatment was recommended tobe utilized in the future, even ifIV access was established.

Although some of the referencesdiscussed in the literature search aredated, they provide evidence for bestpractice. A current literature searchdid not reveal recent studies,although Johnson (2008) diddescribe three international casestudies outlining the effecfive use ofsubcutaneous PCA administrationfor pain management. The lack ofavailable information demonstratesthe need to disperse informationregarding this pain managementtechnique and potential futureresearch in this area.

Clinical PracticePatients with SCD have problems

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

DVT formation from clumped sicklecells in fhe vessel, placement ofperipherally inserfed central linesmay be difficulf and further limitvascular accessibility NIHCC (2011)suggests utilization of SQ PCA forpain management. Benefits includethe following: (a) decreased patientdependence on IV access beforereceiving pain medication; (b) IVaccess is available for administrationof blood products, iron chelatingagents, antibiotics, and other med-ications that may not be compatiblewith opioid therapy; (c) minimal fono risk of infiltration when com-pared wifh IV administration; and(d) increased patient satisfaction dueto timeliness of intervention.

PCA PrograiTiming andAdministration

PCAs are programmed in a varietyof ways: basal rate (the patientreceives a set dose as a continuousinfusion) and/or bolus dose (thepatient requests a prescribed amountof medication in a set time by press-ing a button) (Ellison & Shaw, 2007).For example, if a patient is fakinglong-acting opioids and the medicalteam wants to continue the dosewithout interruption, the bolus-onlyoption may be chosen to managebreakthrough pain and treat theacute crisis.

Pain management is multifaceted.To administer opioids effectively bySQ PCA, the nurse must performcontinual assessment of the patient'spain, satisfaction with therapy, vitalsigns, and infusion site. Assessingthe patient's pain and devising asuitable treatment plan to achievepatient satisfaction are essential. Apatient's ability to perform bolusdosing with SQ PCAS is critical andshould be evaluated by the nurse.Once the decision is made to begintreatment with SQ PCA, the drug pri-marily utilized at NIHCC (2011) ishydromorphone (Dilaudid®), butoccasionally fentanyl or morphine isused as well. Supplies include apump, batteries, documentationsheet, appropriate medication, tub-ing, SQ needle, chlorohexidine glu-conaate (Chloraprep®), and dressingsupplies. The PCA pump settings are

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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

checked independently by two nurs-es against the written medical order,validating the Five Rights and thecarrier solution (NIHCC, 2011).

For comfort and ease of mobilify,opioids administered via SQ PCA forSCD pain crisis are given via theabdomen, though other sites such asthe back, arms, and thighs can beutilized as well (see Figures 2 & 3).When assessing the patient for anacceptable site for SQ administra-tion, the nurse should avoid painfulor ecchymosed areas, enlist patientparticipation in site selection, andreassess the site per hospital policy(Justad, 2009). SQ sites may remain

Source: NIHCC, 2012

accessed, with assessment, dressingcare, and management based on thesame criteria as an IV site (see Figures4 & 5) (NIHCC, 2011).

At NIH, the policy for PCA man-agement via IV or SQ is comprehen-sive. Upon initiation of PCA, assess-ments are performed every 30 min-utes for 1 hour, gradually titrating toevery 4 hours (NIHCC, 2011). Morefrequent assessments should occurwhen there is a change in patientcondition, an increase in doseadministered, or other care require-ments that necessitate close observa-tion. Assessment of patients receiv-ing SQ PCA includes respiratory rate,oxygen saturation, sedation level,blood pressure, and heart rate. Thepatient's pain is assessed by ratinghis or her pain intensity on a 0-10Visual Analog Scale, reviewing thePCA program, documenting theamount of use and demand by the

patient, and determining if the goalsof pain management, safety, and sat-isfaction are being met. In addition,the SQ insertion site is assessed forirritation, ecchymosis, and dressingintactness. All these interventionsand assessments are documented onthe PCA flow sheet and within theelectronic medical record (see Figure6) (NIHCC, 2011). Successfijl painmanagement is achieved when thepatient identifies satisfactory resolu-tion or control of pain and the nurs-ing assessment indicates the patientis oriented with stable vital signs.

Complications may occur withSQ PCA that include oversedation,infection, pruritus (local and sys-temic), and equipment failure. Thenurse must be aware of these poten-tial complications, assess for anypossible problems, and be ready fointervene appropriately. Many com-plications can be avoided with accu-

FiGURE 4.PCA Supplies

FIGURE 5.Site Utilization for PCA

296 September-October 2012 • Vol. 21/No. S M E D S U R Gisr TJ n 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)G Epidural Location:n Epineural Location:

n Central (IV) D SC

S

I iÇg

| o

¡I

o oma

I I< E um E

YesNo

Loci< Level:

3. Container D4. Full Lock D

If

YesNo

YesNo

YesNo

YesNo

YesNo

YesNo

'Indicators

RA = RoutineAssessment

BC = Bag ChangePC = Program Changec e = Change of

Caregiver

Level of Sedation (LOS):

1 =2 = Drowsy/Dosing intermitteniy3 = Sleep/Easily Aroused4 = Sleeping/Difficult to Arouse5 = Confused/Hallucinating6 = Unable to Arouse

"'Motor and SensoryAssessment:

Details documented inelectronic medicalrecord (CRIS) or otherapproved medicalrecord form.

COMMENTS:

Source: NIHCC, 2011

rate patient assessment, vital signsmonitoring, competence with allequipment, and identification ofpotential problems. Insertion sitesthat are reddened should be rotatedand all sites monitored for furthersigns of infection. Locally occurringpruritus may be managed by changingthe site, while systemic pruritus mayrequire medical interventions such asdiphenhydramine (Benadryl®), lotions,or low-dose IV naloxone (Narcan®)drip. Many patients with SCD admit-ted to the NIH for the first time havenot had the experience of SQ PCA.However, with appropriate educationand trial of this altemative pain man-agement process, many patients ac-

cept this form of treatment and have apositive outcome.

ConclusionEffective pain management and

positive patient satisfaction can beachieved with the use of SQ PCA forSCD pain crisis. While there aresome disadvantages and potentialcomplications, there are many moreadvantages to using this form oftherapy. The need for continuedevaluation and research of this modeof pain management is acknowl-edged, specifically in patients with

REFERENCESBallas, S.K. (2007). Current issues in sickle

ceil pain and its management AmericanSociety of Hematology EducationProgram Book. Retrieved from http://asheducationbook.hematologylibrary.org/contenV2007/1/97.long

Ballas, S.K. (2010). Self-management of sick-le cell disease: A new frontier. Journai ofthe Medicai Association, 702(11), 1042-1043.

Doyle, E., Morton, N., & McNichol, L. (1994).Comparison ot patient-controlled analge-sia in children by IV and SC routes ofadministration. British Journai ofAnaesthesia, 72, 533-536.

Ellison, A.M., & Shaw, K. (2007). Managementof vasoocclusive pain events in sickle celldisease. Pédiatrie Emergency Care,23(11), 832-841.

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Haywood, C, Beach, M.C., Lanzkron, S.,Strouse, J.J., Wilson, R., Park, H., ...Segal, J.B. (2009). A systematic review ofbarriers and interventions to improveappropriate use of therapies for sicklecell disease. Journal of the NationalMedical Association, 10^^Q), 1022-1033.

Hernandez, S., & Patterson, GE. (2009). Whatyou need to know about acute chest syn-drome. Nursing2009, 6, 42-45.

Howard, J., Thomas, V.J., & Rawle, H.M.(2009). Pain management and quality oflife in sickle cell disease. Expert ReviewPharmacoeconomics OutcomesResearch, 9(4), 347-352.

Johnson, L. (2008). Management of pain dueto sickle cell disease. Journai of Pain andPaliiative Care Pharmacotherapy, 22('\),51-54.

Justad, M. (2009). Continuous subcutaneousintusion: An efficacious, cost-effectiveanalgesia alternative at the end of life.Home Healthcare Nurse, 27(3), 140-147.

Montalembert, M. (2009). Current strategiesfor the management of children with sick-le cell disease. Expert ReviewsHematotogy, 2(4), 455-463.

Moulin, D.E., Kreeft, J.H., Murray-Parsons, N.,& Bouquillon, A.I. (1991). Comparison ofcontinuous subcutaneous and intra-venous hydromorphone infusions formanagement 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? Retrievedfrom http://www.nhlbi.nih.gov/health/health-topics/topics/sca/

National Institutes of Health Clinical Center(NIHCC). (2012). Giving a subcutaneousinjection. 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 continuousand patient controlled analgesic infu-sions. 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). Patient-controlled analgesia-related medicationerrors 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 elderlypostoperative patients following adminis-tration via an indwelling subcutaneouscannula. Anaesthesia, 52, 318-323.

Solomon, L.R. (2010). Pain management inadults with sickle cell disease in a med-ical center emergency department.Journal of the National MedicalAssociation, ?02(11), 1025-1032.

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van Beers, E. J., van Tujin, C.F.J., Nieuwkerk,P.T., Friederich, PW., Vranken, J.H., &Biemond, B.J. (2007). Patient-controlledanalgesia versus continuous infusion ofmorphine during vaso-occlusive crisis insickle cell disease randomized controlledtrial. American Journai of Hematoiogy,82, 955-960.

White, P (1990). Subcutaneous-PCA: An alter-native to IV-PCA for postoperative painmanagement. The Clinicai Journal ofPain, 6, 297-300.

ADDITIONAL READINGSHopkins, D., Shipton E., Potgieter, D., Van

derMerwe, C, Boon, J., De Wet, C, &Murphy, J. (1998). Comparison of tra-madol and morphine via subcutaneousPCA following major orthopaedic sur-gery. Canadian Journai of Anaesthesia,45(5), 435-442.

National Heart, Lung & Blood Institute(NHLBI). (2004.). The management ofsickle cell disease (No .04-2117; 4th ed.).Bethesda, MD: NHLBI Health Infor-mation Center.

Sharpiro, B.S., Cohen, D.E., & Howe, C.J.(1993). Patient-controlled analgesia forsickle cell related pain. Journal of Painand Symptom Management, 8(^), 22-28.

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M E L S U R GIST Tjr DE s u asr o ,

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Several barriers can challenge the facilitation of human factors sci-ence (ergonomics) (HFE) in health care organizations. If the HFE inter-vention or innovation is too complex or employees do not understandthe benefits of HFE, adoption can be hindered. When employees canactually experience the positive impact of HFE on their day-to-day tasks(e.g., improved working conditions), it is more likely that these innova-tions will be accepted and used to their fullest over the long haul. Havingindividuals who champion the cause at the local user level, such as a headnurse, can influence the way other employees become early and lastingadopters.

For details, see Carayon, P. (2010). Human factors in patient safety asan innovation. AppUed Ergonomics, 41(5), 657-665.

New Resource Helps Hospitals Get Up to Speed onSafety Culture

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

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