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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.


September-October 2012 • Vol. 21/No. S


. and use compassion to guide subsequent acfions (Howard et al. orthopedics. and above all pain management (Hayword et al.000 worldwide (Hayword et al. ketorolae [Toradol®]. adjuvant medieations (antihistamines. To combat these negafive perceptions. 2010). In addition. unemployed. intramuscular (IM). and Barriers for Appropriate Treatment In Africa. Rees et al. Abnormal hemoglobin formstianás thatcauM sickle shatM Incidence. Prevalence. Additionally. such as antibiotics. ibuprofen [Motrin®]. Johnson. For example. and other nonmedieine treatment modalities. 2008). Individuals with SCD often are stereotyped with: behaviors of drug dependency.. Treatment of chronic pain is accomplished by fargefing a known cause or initiating generalized medical management (Johnson. 2010). leg uleers.000 babies are born with SCD annually. oxygen therapy. Treatment for pain associated with AVN may involve additional health care specialties (e. and symptom management (Ballas. 2010). blood products. Montalembert. chronic renal failure. Malformed RBCs Cannot Pass Easily through Blood Vessels and Capillaries Q Normal red blood e ^ rtoi c8li(RBC) RBCs Sow f f » ^ wtthinblooäves!«! ©Abnormal. antiemeties. heat therapy. and underinsured (Ballas. 2009. An acute pain crisis is an emergent medical condition requiring timely treatment strategies. sueh as physical therapy. life-threatening complications.. transeutaneous electrical stimulation.. be receptive to the individual's specific needs. 2010). 2009). physical rehabilitation) and medications such as non-steroidal anti-inflammatory drugs and/or gabapentin (Neurontin®) (Howard et al. nKt blood eelte (sickle cells) SicktoceSs y^ rces-sactron of siMe am naproxen [Naprosyn®]. venous clots. 2009. 2009). health eare providers should receive education regarding disease process. Ellison & Shaw. and stroke (Howard et al. 2009). biofeedbaek. iron chelation (for iron overload due to multiple transfusions). laxatives). non-opioid (acetaminophen [Tylenol®]). Lung & Blood Institute. 2009. In addirion to pain management. health eare providers should aecept patients with SCD with an open mind. undereducated. Low levels of nitric oxide may contribute to hypercoagulability and lead to additional complications such as avascular necrosis (AVN). 2007).Ciinical Practice FIGURE 1. In the United States.000 to 100. Source: National Heart. approximately 200. s k k M . acupuncture. All approaches are utilized at the National Institutes of Health Clinical Center (NIHCC) (2011). and IV or subeufaneous (SQ) patient-eontrolled analgesia (PCA) (Solomon. can be utilized (Bailas. 2009).000 individuals have SCD (Ballas. and relaxation exercises. 2011 bin is released into the plasma and may inhibit nitric oxide production. September-October 2012 294 Vol. Pharmacologie approaches to pain management include oral (PO). and 60. Solomon. 2008). eontinuous intravenous (IV) infusion.. as "frequenf flyers" to emergency departments. if a patient's chronic pain is related to leg ulcers. antidepressants. massage. 2007. 2010). and treatment with concurrent therapies. 5 MEDSURG . Chronie pain is treated with long-aeting opioids for extended management and short-acting opioids to address breakthrough pain (Howard et al. 2007. 72. patients should receive adequate hydration. treat- ment is centered on wound care. 21/No.g. surgery.

The lack of available information demonstrates the need to disperse information regarding this pain management technique and potential future research in this area. dehydration. Benefits include the following: (a) decreased patient dependence on IV access before receiving pain medication. 1991. Runciman. and Scopus. White. (c) minimal fo no risk of infiltration when compared wifh IV administration. & Mafher. and multiple accesses over time (Ellison & Shaw. 1997. S 295 . Kreeft. Although some of the references discussed in the literature search are dated.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. and (d) increased patient satisfaction due to timeliness of intervention. emBase. and other medications that may not be compatible with opioid therapy. appropriate medication. Morton. batteries. Supplies include a pump. 2007). As a result of • MEDSURG September-October 2012 • Vol. reporting SQ PCA was as effective and safe as IV PCA administration. • PCA pain management is equal fo IV injection or continuous infusion (Doyle. Schein. Comparing pharmacokinefics of morphine IM and SQ authors found blood concenfrations of the drug were comparable. • Doyle and co-authors (1994) compared IV PCA and SQ PCA administration of morphine in pafients following appendectomy. the drug primarily utilized at NIHCC (2011) is hydromorphone (Dilaudid®). Macinfyre. & McNichol. burn patients. Search findings yielded many articles written in the late 1990s and early 2000s. and infusion site. 1990). satisfaction with therapy. end-of-life. but dose requirements were higher for SQ than IV. decreased blood flow. transdermal. 1990). Klapp. 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. sickle cell anemia AND subcutaneous PCA. They concluded no differences existed in analgesic scores or overall side effects between the two groups. and sickle cell patient AND PCA. The PCA pump settings are Clinical Practice Patients with SCD have problems with vascular access (Johnson. To administer opioids effectively by SQ PCA. including opioid administration via oral. and oncology patients. and rectal routes. SQ needle. even if IV access was established. Moulin. 2008). controlled study (N=19) to compare efficacy of PCA administration wifh continuous infusion of opioids and noted dose requirements. subcutaneous. placement of peripherally inserfed central lines may be difficulf and further limit vascular accessibility NIHCC (2011) suggests utilization of SQ PCA for pain management. 21/No. This form of treatment was recommended to be utilized in the future. and White (1988) compared the efficacy of SQ-PCA fo convenfional IVPCA. Ellison and Shaw (2007) discussed the assessment of VOE with suggested pharmacological management. but occasionally fentanyl or morphine is used as well. The salient features of this research are reported in brief detail to document scientific evidence for best practice. Once the decision is made to begin treatment with SQ PCA. iron chelating agents. although Johnson (2008) did describe three international case studies outlining the effecfive use of subcutaneous PCA administration for pain management. DVT formation from clumped sickle cells in fhe vessel. (b) IV access is available for administration of blood products. tubing. the nurse must perform continual assessment of the patient's pain. & Bouquillon. 1994. Hicks. chlorohexidine gluconaate (Chloraprep®). and PCA device. 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. The mean daily pain scores were comparable and side effects of nausea and constipation were improved in the PCA group. White. as well as the duration of use. Upton. sickle cell patient AND subcutaneous PCA. if a patient is faking long-acting opioids and the medical team wants to continue the dose without interruption. Murray-Parsons. 2007). • SQ opioid administration is equigesic with IM opioid administration (Semple. intravenous. • Urquhart. Databases searched included Puh Med. For example. Nelson. A great deal of the information regarding fhe use of subcutaneous PCA reflected treatment in postoperative patients. CINALH Plus. These authors suggested opioids could be administered effectively via continuous infravenous drip. • Solomon (2010) discussed one barrier to the utilization of SC administration for pain management as a lack of physician and patient education. Pain management is multifaceted. they provide evidence for best practice. and dressing supplies. Sikirica. documentation sheet. A current literature search did not reveal recent studies. the bolus-only option may be chosen to manage breakthrough pain and treat the acute crisis. intermittent intravenous bolus. Van Beers and co-authors (2007) conducted a randomized. A patient's ability to perform bolus dosing with SQ PCAS is critical and should be evaluated by the nurse. These problems are due to veins that are hard to cannulate. were less in fhe PCA group than the group with continuous infusion. Assessing the patient's pain and devising a suitable treatment plan to achieve patient satisfaction are essential. antibiotics. There was no parameter set on years for the search. vital signs.

and be ready fo intervene appropriately. PCA Supplies accessed. the SQ insertion site is assessed for irritation. ecchymosis. 2009). or other care requirements that necessitate close observation. safety. A Subcutaneous Injection into the Fatty Layer of Tissue (pinched up to give the injection) Under the Skin FIGURE 3. and reassess the site per hospital policy (Justad. enlist patient participation in site selection. Upon initiation of PCA. . For comfort and ease of mobilify. 2012 checked independently by two nurses against the written medical order. Assessment of patients receiving SQ PCA includes respiratory rate. gradually titrating to every 4 hours (NIHCC. Site Utilization for PCA 296 isrR TJ n September-October 2012 • Vol. 2011). sedation level. All these interventions and assessments are documented on the PCA flow sheet and within the electronic medical record (see Figure 6) (NIHCC. and equipment failure. infection. 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. an increase in dose administered. 2011). the policy for PCA management via IV or SQ is comprehensive. documenting the amount of use and demand by the patient. When assessing the patient for an acceptable site for SQ administration. 2011). and determining if the goals of pain management. S M E D S U G s I isr Gt. and thighs can be utilized as well (see Figures 2 & 3). assess for any possible problems. 2012 Source: NIHCC. opioids administered via SQ PCA for SCD pain crisis are given via the abdomen. and heart rate. 21/No. the nurse should avoid painful or ecchymosed areas. and satisfaction are being met. dressing care. Complications may occur with SQ PCA that include oversedation. The patient's pain is assessed by rating his or her pain intensity on a 0-10 Visual Analog Scale. At NIH. assessments are performed every 30 minutes for 1 hour. blood pressure. 2011). reviewing the PCA program. injection Sites Source: NIHCC. oxygen saturation. More frequent assessments should occur when there is a change in patient condition. SQ sites may remain FiGURE 4. validating the Five Rights and the carrier solution (NIHCC. arms. In addition. Many complications can be avoided with accu- FIGURE 5. pruritus (local and systemic).Clinical Practice FIGURE 2. though other sites such as the back. The nurse must be aware of these potential complications. and management based on the same criteria as an IV site (see Figures 4 & 5) (NIHCC. and dressing intactness. with assessment.

. or low-dose IV naloxone (Narcan®) drip. (2007). Current issues in sickle ceil pain and its management American Society of Hematology Education Program Book. Many patients with SCD admitted to the NIH for the first time have not had the experience of SQ PCA. & McNichol. S.long Ballas. 2011 rate patient assessment. 832-841. Doyle. (2010). Pédiatrie Emergency Care. S 297 . lotions. However. & Shaw. Ellison. 10421043.K.. Retrieved from http:// asheducationbook. there are many more advantages to using this form of therapy. L. Management of vasoocclusive pain events in sickle cell disease. The need for continued evaluation and research of this mode of pain management is acknowledged. A. E. Self-management of sickle cell disease: A new frontier. Conclusion Effective pain management and positive patient satisfaction can be achieved with the use of SQ PCA for SCD pain crisis. Container D 4. competence with all equipment. Full Lock D |o ¡I S I iÇg 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. and identification of potential problems. Comparison ot patient-controlled analgesia in children by IV and SC routes of administration. specifically in patients with REFERENCES Ballas. with appropriate education and trial of this altemative pain management process. 21/No. 702(11).. 23(11). British Journai of Anaesthesia. 533-536.hematologylibrary. 72. K.A Novel Approach to Pain Management In Persons with Sickle Cell Disease FIGURE 6. (1994). while systemic pruritus may require medical interventions such as diphenhydramine (Benadryl®). vital signs monitoring. MEDSURG September-October 2012 • Vol. N. Insertion sites that are reddened should be rotated and all sites monitored for further signs of /contenV2007/1/97. (2007). COMMENTS: Source: NIHCC. PCA Flow Sheet Drug/Concentration Route: D Peripheral (IV) n Central (IV) G Epidural Location: n Epineural Location: D SC Loci< Level: 3. While there are some disadvantages and potential complications. Locally occurring pruritus may be managed by changing the site.M. S. Morton. Journai of the Medicai Association. many patients ac- cept this form of treatment and have a positive outcome.K.

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