1. Lentz, Megan B. BSN, RN
  2. Kautz, Donald D. PhD, RN

Article Content

SICKLE CELL DISEASE (SCD) is a group of chronic genetic disorders that affects an estimated 70,000 to 80,000 Americans.1 SCD is characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in red blood cells that distort the cells' normal rounded shape into a sickle or crescent shape.2 (See Sickle cells bend the rules.) Because sickle-shaped cells can't flow freely through blood vessels, they may cause vascular occlusion resulting in severe pain and other signs and symptoms. Patients with SCD are at high risk for acute and chronic complications that may result in disability or death.3


Acute vaso-occlusive crisis (VOC), the most common complication of SCD, results from tissue ischemia related to vascular occlusion.3 It's characterized by excruciating pain-usually of sudden onset, although gradual onset is also possible. Nearly all patients with SCD experience VOC at some point, but the frequency is variable and unpredictable.3


VOCs lead to 197,000 ED visits and 356 million dollars spent on pain management every year.4 Many patients with SCD visit the ED repeatedly for pain management because VOCs are typically recurrent. However, despite their severe pain, patients with VOC are too often undertreated for reasons discussed in this article. This article also proposes ways to improve pain management for patients experiencing this painful disorder.


Patients under scrutiny

Healthcare providers are acutely aware of substance abuse and drug-seeking behaviors among some patients. As a result, patients who frequent the ED for pain management may undergo special scrutiny to determine whether their pain results from a physical or a psychological disorder.


Some research has shown that healthcare providers have a negative attitude toward patients with SCD, and that this negative attitude is especially common among providers who care for more than one patient with SCD per week.5 Glassberg suggests that this negative attitude has caused slower treatment of pain, and in some cases, a deviation from national guidelines for appropriate care for patients with SCD.5 Patients have stated that they feel less than human when trying to obtain medical care needed during a VOC.6


Overcoming barriers

Because SCD is a hereditary disease, adults with SCD have been struggling with its signs and symptoms all their lives. They've learned to cope with daily pain and may appear perfectly normal as they perform activities of daily living.7 Consequently, when healthcare professionals see a patient with SCD eating, drinking, and talking normally, they may distrust the patient's self-report of pain.


Studies have shown that barriers between patients with SCD and their providers affect how they each react when confronted with a VOC.5,7 For healthcare providers, thoughts of drug-seeking and addictive behaviors affect their assessment of these patients, undermining the relationship.8 Patients anticipating this distrustful attitude may delay seeking medical care for VOCs. Failure to treat a VOC quickly and appropriately can lead to disability or death from complications.3,4,8


Research has also shown patients with a VOC and providers have starkly contrasting views and expectations.5,7,8 Patients may be distrustful of providers and feel they're being labeled as addicts or drug seekers. An additional barrier to appropriate care is a lack of information to help patients prevent further VOCs.


Because no objective biomarkers or imaging studies reflect pain intensity, treatment must be guided by the patient's self-report of pain. Rapid triage and initiation of analgesics (typically morphine sulfate or hydromorphone) within 30 minutes of triage or 60 minutes of registration is the standard of care based on current clinical practice guidelines. Some patients with mild-to-moderate pain respond well to nonsteroidal anti-inflammatory drugs.3,4,8


Fast action required

Rapid administration of medication and fluids for hydration is essential, but venous access may be difficult when the patient is experiencing a VOC. Clinicians may administer parenteral medications and fluids via the subcutaneous route when the I.V. route is inaccessible.8


When caring for a patient with a VOC in the ED, follow these evidence-based guidelines.3


* Perform a rapid assessment to determine pain characteristics, location, intensity, and associated symptoms.


* Document the patient's recent history of opioid and nonopioid analgesic use. Ask the patient what drugs and other interventions have been effective for controlling pain in the past.


* Administer pain medication as prescribed. Initiating patient-controlled analgesia in the ED is recommended.


* Provide supplemental oxygen if the patient's oxygen saturation is less than 95% on room air.


* Assess the patient's response to treatment every 15 to 30 minutes and provide additional analgesia as indicated and prescribed. Monitor the patient for adverse drug reactions, such as oversedation. Titrate the drug dosage down as pain resolves based on patient report.


* Consider use of adjunctive pain management therapies, such as local heat application.



If pain isn't well controlled after 6 to 8 hours of treatment, the healthcare provider may admit the patient for ongoing treatment to manage pain and prevent complications.




1. Genetics Home Reference. Sickle cell disease. 2016. [Context Link]


2. National Institutes of Health/National Heart, Lung, and Blood Institute. What is sickle cell disease? 2016. https:// [Context Link]


3. National Institutes of Health/National Heart, Lung, and Blood Institute. Evidence-based management of sickle cell disease. Expert panel report. 2014. https:// [Context Link]


4. Wilson BH, Nelson J. Sickle cell disease pain management in adolescents: a literature review. Pain Manag Nurs. 2015;16(2):146-151. [Context Link]


5. Glassberg JA, Tanabe P, Chow A, et al. Emergency provider analgesic practices and attitudes toward patients with sickle cell disease. Ann Emerg Med. 2013;62(4):293-302e10. [Context Link]


6. Jenerette CM, Brewer CA, Ataga KI. Care seeking for pain in young adults with sickle cell disease. Pain Manag Nurs. 2014;15(1):324-330. [Context Link]


7. Brown SE, Weisberg DF, Balf-Soran G, Sledge WH. Sickle cell disease patients with and without extremely high hospital use: pain, opioids, and coping. J Pain Symptom Manage. 2015;49(3):42-46. [Context Link]


8. Simon E, Long B, Koyfman A. Emergency medicine management of sickle cell disease complications: an evidence-based update. J Emerg Med. 2016;51(4):370-381. [Context Link]