Painful crisis in sickle cell disease: recommendations for urgent treatment

Painful crisis in sickle cell disease: recommendations for urgent treatment

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In 2008, the World Health Organization (WHO) designed the World Sickle Awareness Day, which is held every June 19, as a way to draw attention to this important disease that affects about 60,000 people in our country. THE sickle cell disease it has a genetic basis and is hereditary, ie it is transmitted from parents to children. At the link above, it is possible to access the article I wrote here for the PEBMED portal that generally deals with aspects of this comorbidity.

In this article, we will focus on the treatment of the main vaso-occlusive complication in these patients, which often turns into a nightmare for the doctor on duty in the Emergency Room: a pain crisis.

painful crisis

understanding the problem

Sickle cell patients tend to have different levels of complications during their lifetime, depending on the severity of the disease, ie the levels of hemoglobin C present in the circulation. Therefore, most of these complications are characterized by “clogging” of red blood cells in the microcirculation and, consequently, symptoms of hyperviscosity and peripheral and visceral vasoocclusive crisis. Thus, the pain crisis can be understood as an ischemic symptom and venous congestion at the affected site, leading to tissue hypoxia and production of lactic acid and free O2 radicals. These attacks can occur several times a year and are usually associated with cold weather, infections, premenstrual periods, emotional problems, pregnancy, strenuous exercise or dehydration.

Patient in the emergency room

In general, these patients seek emergency care because of intense pain that may be localized or diffuse, and is more common in the lower limbs and chest. According to American College of Emergency Physicians (ACEP), opioid use in this group of patients was stable from 2008 to 2013, with an overdose-related mortality rate corresponding to only 0.77% of the total population. These data show us that in most cases, the demand for these drugs among sickle cell patients is more related to good experience in treating pain in past conditions than to drug addiction. It is therefore important to have confidence in the patient’s report, as there are no clinical or laboratory signs confirming the pain crisis. At the first assessment, the patient should undergo a thorough clinical examination for alarming signs such as hypotension, fever (axillary temperature> 38 ° C), tachycardia, tachypnea, hypoxemia, changes in consciousness, seizures or atypical headache. Such changes may indicate more serious conditions that are also part of the patient’s medical history, such as acute chest syndromes, pulmonary thromboembolism, splenic sequestration, cerebrovascular accidents, and septic conditions.

In case of hypoxemia, fever, dyspnea, tachypnea, cough or chest / back pain in search of acute radiological changes (criteria for acute chest syndrome) it is necessary to order an X-ray of the chest in two views (posteroanterior and lateral). In case of signs / symptoms related to the central nervous system (CNS), tomographic assessment of the skull may be imperative to rule out cerebrovascular accidents.

quiz: Allergy in a pediatric patient with sickle cell anemia

Resolving the pain crisis

It is important to remember that the approach to pain conditions must be multidisciplinary, including non-pharmacological measures (such as warming the limbs or taking the patient to a quiet place – ASH 2020 conditional recommendation with a low level of safety based on the evidence-based GRADE system). However, it is imperative to start analgesia in the first 60 ‘of reception, and antimicrobial drugs in the 1st hour if the patient has septic signs as mentioned in the above topic. We must also remember to ask for a basic laboratory assessment (blood count, kidney function, CRP, transaminases and bilirubin) at the time of admission to assess the worsening of anemia or other signs of specific organ dysfunction. Comparison of the degree of anemia should always be in relation to the initial levels of the patient, and not to the general population, avoiding even large increases in hemoglobin levels and potential worsening of the state of hyperviscosity. It is important to ask the patient about medications that have helped control pain in previous episodes, as this can serve as a guide to starting treatment, avoiding unnecessary escalations that can cost more time than bring relief.

The most commonly used analgesic regimens

Opioids and nonsteroidal anti-inflammatory drugs (NSAIDs) are the preferred drugs, and parenteral routes are one of the choices. Both drugs can be given separately or together depending on the severity of the pain. NSAIDs should be used with caution in patients with impaired renal function, but they are not an absolute contraindication for mild / moderate changes. In those for whom immediate venous access is not feasible, the subcutaneous route may be used for opioid administration. The use of dipyrone and paracetamol is recommended, if there are no contraindications, as adjunctive therapy. For children, we can also consider intranasal fentanyl to relieve pain. Subsequent assessments should be performed every 15 ‘or 30’, assuming the previous dose is increased by 25% until adequate analgesia is achieved. Below is a table customized with tools ‘point-of-care’ ACEP with suggested doses and routes of administration. Patients using long-acting opioids at home should continue to take doses after the initial approach.

Medications Dose Maximum dose after administration

Frequency

Intranasal fentanyl

2mcg / kg 100mcg or 1mL per nostril Can be repeated once every 10 ‘

Intravenous morphine

0.1 mg / kg 10mg

It can be repeated every 15 to 30 ′ until the control

Intravenous hydromorphone

0.015 mg / kg 1.2mg

It can be repeated every 15 to 30 ′ until the control

Intravenous ketorolac *

0.5 mg / kg 15-30mcg

One dose

Adapted from https://www.acep.org/sickle-cell/. Dose for children (<12 years or <50 kg)

* Attention to patients with underlying renal dysfunction.

Medications

Dose Maximum dose after administration

Frequency

Intravenous morphine

0.1 mg / kg 10mg

It can be repeated every 15 to 30 ′ until the control

Intravenous hydromorphone

0.015 mg / kg 1.2mg

It can be repeated every 15 to 30 ′ until the control

Intravenous ketorolac *

0.5 mg / kg 15-30mcg

One dose

Adapted from https://www.acep.org/sickle-cell/. Doses for adults or adolescents (≥ 12 years or ≥ 50 kg)

* Attention to patients with underlying renal dysfunction.

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Evaluation of other classical approaches in clinical practice

The latest guideline (2020) published by the American Society of Hematology (ASH) for the treatment of acute or chronic pain in these patients does not take a stand on strong hydration due to the lack of evidence to suggest that the approach works or not. On the other hand, ACEP is contraindicated in strong hydration due to the risk of congestion and worsening of the clinical picture, including the possibility of precipitation of the acute chest syndrome in the patient. In clinical practice, it is very important to correct dehydration that can be associated with the condition, warm the extremities and avoid triggers that cause pain.

We should not use corticosteroids as they can cause pain.

Ketamine use should be limited to hospitalized opioid-resistant patients. Conditional recommendation with a low level of security (according to the GRADE system). Recommended dose: 0.1 to 0.3 mg / kg / h; maximum dose: 1 mg / kg / h.

Regional anesthetics, ie. given through an epidural catheter or block, for opioid-resistant patients are conditionally recommended with a low level of evidence (GRADE) by ASH 2020.

Oxygen therapy should be used only in patients who show degrees of hypoxemia on peripheral saturation or arterial blood gases.

In the case of uncomplicated painful crises (not related to acute chest syndrome, cerebrovascular accidents, sepsis, splenic sequestration, among others), transfusion of compacted red blood cells is questionable. Transfusion of 01 concentrates of phenotyped and deleukocyte red blood cells is allowed if there is associated acute anemia, in order to improve symptoms, always taking care not to exceed the initial values ​​of the patient. We do not need to accept a certain limit point for transfusion, nor do we indicate exchange transfusions (bleeding transfusions) that target a certain level of hemoglobin C during hemoglobin electrophoresis.

The use of poetin alpha is contraindicated in the Clinical Protocol and Therapeutic Guidelines for Sickle Cell Disease published by the Ministry of Health in 2018 and also finds no support in the international literature.

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# Sickle cell disease management in ED. Available in: https://www.acep.org/sickle-cell/. Copyright 2021, American College of Emergency Physicians, Dallas, Texas. All rights reserved. Made in the United States.

# Amanda M. Brandow, C. Patrick Carroll, Susan Creary, Ronisha Edwards-Elliott, Jeffrey Glassberg, Robert W. Hurley, Abdullah Kutlar, Mohamed Seisa, Jennifer Stinson, John J. Strouse, Fouza Yusuf, William Zempsky, Eddy Lang; American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Treatment of Acute and Chronic Pain. Blood Adv 2020; 4 (12): 2656–2701. doi: https://doi.org/10.1182/bloodadvances.2020001851 # Figueiredo, FA; Firefighter, MAA. Clinical protocol and therapeutic guidelines for sickle cell disease. Common Regulation no. 05, dated 19 February 2018. Ministry of Health, Department of Health Care, Department of Science, Technology and Strategic Contributions. # Evidence-Based Sickle Cell Disease Management: Expert Panel Report, 2014. National Institutes of Health.

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