Nalbuphine

hospital

Learn the complex problems this disease triggers and how to support your patient during a crisis.

Sterling Jackson, 21, who has a history of sickle-cell anemia, is admitted to the medical/surgical unit from the emergency department (ED). He developed severe pain in his chest, arms, legs, and back about 10 hours ago. After 4 hours receiving intravenous (LV) nalbuphine (Nubain), ketorolac (Toradol), and hydration in the ED, he still rates his pain as 6 on a visual analogue scale, and his blood work shows worsening anemia from his baseline level.

The most common genetic disorder in the United States, sickle-cell disease affects about 70,000 people. Although most common in African-Americans, it also affects people whose ancestors came from parts of the world where malaria is endemic. Having one sickle-cell gene (known as sickle-cell trait) provides some protection against malaria, but having two causes sicklecell disease.

Years ago, people with sickle-cell disease died in their teens. Today, with improved education and treatment, some live into their 60s, but they're susceptible to sickle-cell crises all their lives. In this article, we'll explain what's causing Mr. Jackson's problems and how you can help him through the crisis.

Faulty hemoglobin

Several variants of sickle-cell disease have been identified, but all are characterized by the tendency of hemoglobin to crystallize, forcing red blood cells (RBCs) into a rigid crescent or sickle shape. Deoxygenation, dehydration, acidosis, and temperature changes promote the crystallization. Instead of flowing smoothly through blood vessels, the misshapen cells break apart too soon and block flow in small blood vessels, causing great pain. Sickle-cell disease has the following effects: (See Beware of These Complications to learn more.)

* Anemia is a chronic problem. Sickled RBCs are destroyed in only 12 to 15 days, compared with a 120-day life span for normal RBCs. The RBCs can also become trapped in the spleen or liver rather than circulating in the blood. Finally, infection or another complication may prevent bone marrow from producing enough new RBCs, triggering an aplastic crisis.

* Bacterial infections such as meningitis, pneumonia, sepsis, and osteomyelitis pose a significant threat because the disease process reduces spleen function. The risk of infection with deadly pneumococcal bacteria, mycoplasma, and chlamydia is great, so a patient with fever, cough, and pain needs to start aggressive antibiotic therapy even before culture results are available.

* Organ and tissue damage occurs when anemia, increased blood viscosity, blood cells adhering to vessel walls, and activation of the clotting system reduce blood flow and oxygen delivery.

Intense pain episodes, which can affect the extremities, back, chest, and abdomen, can last hours to weeks and are the most common reason patients with sickle-cell disease require hospitalization. Some say the pain feels as though all their bones are breaking at once.

Sickle-cell pain probably originates with reduced blood flow and inflammation from bone marrow and muscle necrosis. Pain episodes, which vary in frequency among individuals, can be triggered by many factors, such as hypoxemia, infection, dehydration, and worsening anemia.

Assessing your patient

Mr. Jackson's admitting diagnosis is sickle-cell pain triggered by bronchitis with a reactive airway component and worsening anemia from aplastic crisis. When you enter his room, he's receiving I.V. fluids and oxygen via nasal cannula. As you take his history, he tells you he's been having coughing, fever, chills, and some shortness of breath for about 48 hours. He had asthma as a child but hasn't had an attack for years.

In the ED, he rated his pain as 9 out of 10 on a visual analogue scale, and now he rates it as 6. He tells you that his last pain episode was 1 year ago. Yesterday he started feeling run-down, and he awoke this morning with pain in his arms, legs, low back, and chest. He says its more severe than his typical crisis and the cough is unusual. This key information helps distinguish his usual pattern of sickle-cell symptoms from those associated with another disorder or complication.

Mr. Jackson's pulse is 110; respirations, 20; BP, 130/80 sitting and 120/65 standing; temperature, 102.2deg F (390deg C); and Spot level, 900. His tongue is furrowed, his eye scleras are yellow, and his conjunctivas are very pale. Auscultating his lungs, you hear diffuse wheezing. You don't detect swelling or heat in the painful areas, but he has diffuse tenderness in his back, arms, and legs.

Combating multiple problems

To care for Mr. Jackson, you need to address his pain, anemia, and the threat of complications.

Managing pain. Medications to treat sickle-cell pain should provide rapid, safe, and sustained analgesia. (See Medications to Manage the Pain for recommendations.) Administer them on a fixed schedule to maintain consistent blood levels. Assess the patients pain before you administer them and again 30 minutes later to measure their effectiveness. Reassess pain each time you take his vital signs or more frequently if it isn't controlled. For consistency, you and other nurses caring for Mr. Jackson should use the same pain scale for each assessment.

Bed rest and correcting underlying problemsdehydration, hypoxia, acidosis, and infection-will help reduce the pain. Also offer him nonpharmacologic techniques such as relaxation; distraction with music, videos, or games; biofeedback; heating pads; massage; and physical therapy.

Providing hydration. Hypotonic IN fluids, such as DSW or 5% dextrose in 0.45% sodium chloride solution, cause water to enter RBCs and markedly reduce the hemoglobin's tendency to crystallize. Because almost everyone with sickle-cell disease also has a renal defect that causes continuous water loss from the kidneys, give plenty of fluids to treat pain caused by dehydration. Monitor your patient's electrolyte levels, especially sodium, to detect hyponatremia and other problems.

Administering oxygen. Monitor Mr. Jackson's Spot level and administer oxygen as ordered to combat deoxygenation, the primary cause of sickling. Keep in mind, however, that severe anemia affects the reliability of Spot levels, so a symptomatic patient may need arterial blood gas measurements to accurately determine oxygenation. Giving oxygen isn't helpful for patients who aren't hypoxic because it could suppress bone marrow production of RBCs over time.

Administering transfusions. Packed RBCs are indicated in certain situations; for example, when symptomatic anemia, a hematocrit reading 7 points below baseline, orthostasis, and a reticulocyte count of zero signal aplastic crisis. When you administer a transfusion, monitor your patient for complications, such as signs and symptoms of volume overload, hyperviscosity, and pulmonary edema, and transfusion reactions, such as a falling hematocrit level, signs of hemolysis or organ failure, and increasing pain.

Managing complications. The pain of sickle-cell disease may be a precursor to a more serious complication and could persist until underlying medical problems resolve. Several life-threatening complications can present as pain or develop during a pain episode. Be on guard for new fever, shortness of breath, increasing pain, a falling hematocrit level, and signs of organ failure.

Carrying out the care plan

Caring for Mr. Jackson calls for aggressive pain management, adequate oxygenation, vigorous hydration, and protecting against infection. As ordered, administer the following:

* oxygen at 2 liters/minute via nasal cannula and albuterol via metered-dose inhaler, one to two puffs every 4 to 6 hours

* I.V. 5% dextrose in 0.45% sodium chloride solution at 2 ml/kg/hour (150 to 200 ml/hour in adults)

* nalbuphine every 3 hours and ketorolac every 6 hours, not to exceed 5 days of use

* incentive spirometry every hour while he's awake to prevent atelectasis and acute chest syndrome

* prophylactic ceftriaxone (Rocephin) IN every 12 hours with azithromycin (Zithromax) orally for 5 days

* transfusion of 5 ml/kg of packed RBCs (leukocyte depleted and matched for minor antigens) over 2 hours for each unit, not to exceed a hemoglobin level greater than 10 grams/dl

* serial complete blood cell and reticulocyte counts and electrolyte levels and a repeat chest X-ray after 24 hours' hydration. (Once hydration is restored, a lung infiltrate may appear, indicating acute chest syndrome.)

Getting ready for discharge

Three days after admission, Mr. Jackson has responded well to treatment and his symptoms have resolved. Teach him about his prescribed pain medications, including nonsteroidal anti-inflammatory drugs and long-acting opioids. Assess his level of knowledge about the following points, then review what he needs to know:

* Stress the importance of seeing his primary care provider for a 1-week follow-up and routine checkups every 6 months. If he doesn't have one, refer him to a generalist for routine health care and a specialist in sickle-cell care to treat complications. Several centers in the United States have multidisciplinary teams to address the complex problems associated with sickle-cell disease. (See Selected Web Sites at the end of this article.)

* Teach him to seek immediate medical attention if he develops a fever or signs and symptoms of infection.

* Advise him to get an influenza vaccine every autumn and pneumococcal vaccine every 5 or 6 years.

* Remind him to inform all health care providers of his condition and to take prophylactic antibiotics as prescribed for certain dental or invasive procedures.

* Tell him to drink 8 to 10 glasses of water a day-even more before or during periods of exposure to factors that could precipitate a crisis, such as a cold or the flu.

* Teach him to dress properly in hot and cold weather because an abrupt temperature change can precipitate a painful crisis. He should also avoid any type of excessive physical exertion that repeatedly leads to complications.

* Warn him not to take iron supplements unless his care provider prescribes them because aggressive iron supplementation could lead to iron overload in someone who receives multiple blood transfusions. However, he may need to take 1 mg of folic acid daily, as prescribed, to maintain erythropoiesis.

Crisis resolved

With careful monitoring and protective measures, you've seen Mr. Jackson through his sickle-cell crisis. And with comprehensive teaching, you've prepared him to ward off future problems related to his chronic disease.

SELECTED REFERENCES

Guideline for the Management of Acute and Chronic Pain in Sickle Cell Disease. Glenfiew, Ill., American Pain Society, 1999.

Serjeant, G.: Sickle Cell Disease, 3rd edition. Oxford, England, Oxford Medical Press, 2001.

Steinberg, M.: "Management of Sickle Cell Disease," The New England Journal of Medicine. 340(13):1021-1030, April 1, 1999.

Steinberg, M., et al. (eds): Disorders of Hemoglobin. Cambridge, England, Cambridge University Press, 2001.

Vichinsky, E.: "Current Issues with Blood Transfusions in Sickle Cell Disease," Seminars in Hematology. 38(1 Suppl.,1):14-22, January 2001. Vichinsky, E., et al.: "Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease. National Acute Chest Syndrome Study Group," The New England]ournal of Medicine. 342(25):1855-1865, June 22, 2000.

SELECTED WEB SITES

The Sickle Cell Information Center http://www.SCIn:fo.org

National Institutes of Health:The Management of Sickle Cell Disease, 4th edition, July 12, 2002.

http:llwww.nhlbi.nih.gov/health/prof/blood/sickle/index.htm Last accessed on November 5, 2002.

BY ALLAN PLATT, PA,C; JOANN BEASLEY, RN; GAYNELL MILLER, RN; AND JAMES R. ECKMAN, MD

Allan Platt is the program coordinator, JoAnn Beasley is the newborn screening coordinator and clinical manager, Gaynell Miller is an assistant vice president of patient care, and James R. Eckman is medical director of the Georgia

Comprehensive Sickle Cell Center at Grady Health System in Atlanta.

Copyright Springhouse Corporation Dec 2002
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