Painful crisis in sickle cell disease: recommendations for emergency treatment

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In 2008, the World Health Organization (WHO) established World Sickle Cell Disease Awareness Day, which is held every 06/19 as a way to draw attention to this important disease that affects approximately 60,000 people in our country. THAT sickle cell disease it has a genetic basis and is hereditary, that is, it is passed from parents to children. In the link above, it is possible to access an article I wrote here for the PEBMED portal, which in general terms deals with the aspects of this comorbidity.

In this article, we will focus on dealing with the most important vaso-occlusive complication that these patients present, and which often turns into a nightmare for the doctor on duty at the Emergency Room: the pain crisis.

pain crisis

understanding of the problem

Patients with sickle cell disease tend to have different levels of complications throughout their lives, depending on the severity of the disease, that is, the levels of hemoglobin S present in the circulation. Therefore, most of these complications are characterized by “sickling” of red blood cells in the microcirculation and consequently symptoms of hyperviscosity and peripheral and visceral vaso-occlusive crises. Thus, we can understand the pain crisis 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 seizures can occur several times a year and are usually associated with cold weather, infections, premenstrual period, emotional problems, pregnancy, hard training or dehydration.

The patient in the emergency room

In general, these patients seek emergency and emergency care due to intense pain that may be localized or diffuse, more commonly referred to in the lower extremities and chest. According to American College of Emergency Physicians (ACEP), the use of opioids in this group of patients was stable between the years 2008-2013, with a mortality associated with overdose corresponding to only 0.77% of the total population. These data show us that most of the time, the demand for these drugs among patients with sickle cell disease is more associated with good experiences with pain management under previous conditions than with substance abuse. Therefore, it is important to trust the patient’s report as there are no clinical or laboratory signs confirming the pain crisis. At the initial assessment, the patient should undergo a thorough clinical examination to look for warning signs such as hypotension, fever (Axillary temperature> 38 ° C), tachycardia, tachypnoea, hypoxemia, changes in level of consciousness, seizures or atypical headache. Such changes may indicate more serious conditions that are also part of the patient’s history of the disease, such as acute breast syndromes, pulmonary thromboembolism, spleen sequestration, cerebrovascular accidents, and septic conditions.

An X-ray of the thorax in two views (posteroanterior and lateral) should be ordered in case of hypoxemia, fever, dyspnoea, tachypnoea, cough or chest / back pain looking for acute radiological changes (criteria for acute breast syndrome). In case of signs / symptoms related to the central nervous system (CNS), tomographic evaluation of the skull may be urgently needed to rule out cerebrovascular accidents.

Quiz: Allergy in a pediatric patient with sickle cell anemia

Dealing with the pain crisis

It is important to remember that the approach to pain conditions must be multidisciplinary, including non-pharmacological measures (such as warming limbs or taking the patient to a quiet place – Conditional recommendation with low degree of safety at ASH 2020 taking into account the GRADE system of medication evidence-based). However, it is imperative to start analgesia in the first 60 ‘of hospitalization and antimicrobials in the 1st hour if the patient shows septic signs as indicated in the topic above. We must also remember to request a basic laboratory assessment (blood count, kidney function, CRP, transaminases and bilirubin) at admission to assess for worsening anemia or other signs of specific organ dysfunction. The comparison of the degree of anemia should always be in relation to the patient’s baseline levels and not the general population, avoiding even large increases in hemoglobin levels and potential worsening of the hyperviscosity condition. It is important to ask the patient about medications that helped control pain in previous episodes, as this can serve as a guide to beginning treatment to avoid unnecessary escalations that may cost more time than bringing relief.

Most commonly used analgesic regimens

Opioids and non-steroidal anti-inflammatory drugs (NSAIDs) are the preferred drugs and parenteral routes are the preferred ones. Both drugs can be administered separately or together depending on the severity of the pain. NSAIDs should be administered with caution to patients with renal impairment, but they are not an absolute contraindication for mild / moderate changes. In those where immediate venous access is not possible, the subcutaneous route may be used to administer opioids. The use of dipyrone and paracetamol is encouraged, if there are no contraindications, as adjunctive therapy. For children, we may also consider intranasal fentanyl for pain relief. Subsequent evaluations should be performed every 15 ‘or 30’, provided a 25% increase in the previous dose until adequate analgesia is achieved. Below is a table customized from a tool ‘point-of-care’ of ACEP with suggested doses and routes of administration. Patients using long-acting opioids at home should continue their doses after the initial approach.

Medicine Dosage Maximum dose per administration

Frequency

Intranasal fentanyl

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

Intravenous morphine

0.1 mg / kg 10 mg

Can be repeated every 15 to 30 ′ until check

Intravenous hydromorphone

0.015 mg / kg 1.2 mg

Can be repeated every 15 to 30 ′ until check

Intravenous Ketorolac *

0.5 mg / kg 15-30mcg

Single dose

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

* Attention to patients with underlying renal insufficiency.

Medicine

Dosage Maximum dose per administration

Frequency

Intravenous morphine

0.1 mg / kg 10 mg

Can be repeated every 15 to 30 ′ until check

Intravenous hydromorphone

0.015 mg / kg 1.2 mg

Can be repeated every 15 to 30 ′ until check

Intravenous Ketorolac *

0.5 mg / kg 15-30mcg

Single 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 insufficiency.

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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 address severe hydration due to the absence of evidence to suggest that one does or does not do the procedure. On the other hand, ACEP contraindicates severe hydration due to the risk of congestion and worsening of the clinical picture, including the possibility of inducing an acute breast syndrome in the patient. In clinical practice, it is very important to correct the dehydration that may be associated with the condition, warm the extremities and avoid triggers that trigger the pain.

We should not use corticosteroids as they can cause pain.

The use of ketamine should be restricted to hospitalized patients who are refractory to opioids. Conditional recommendation with a low degree of safety (according to the GRADE system). Suggested dosage: 0.1 to 0.3 mg / kg / hour; maximum dose: 1 mg / kg / h.

Regional anesthetics, ie. administered through an epidural catheter or block, to opioid-refractory patients is recommended conditionally with a low level of evidence (GRADE) of ASH 2020.

Oxygen therapy should only be given to patients who show degrees of hypoxemia with peripheral saturation or arterial blood gases.

In case of uncomplicated pain crises (not associated with acute breast syndrome, cerebrovascular accidents, sepsis, spleen sequestration, among others) the transfusion of packed red blood cells is questionable. Transfusion of 01 concentrate of phenotype and deleucocytosed red blood cells is permitted if acute anemia is associated, in order to improve the symptoms, always taking care not to exceed the patient’s baseline values. A specific cut-off point for transfusion should not be allowed, nor do we indicate exchange transfusions (blood transfusions) aimed at a specific level of hemoglobin S by hemoglobin electrophoresis.

The use of poetin alfa is contraindicated in the Clinical Protocol and Therapeutic Guidelines for Sickle Cell Disease published by the Ministry of Health in 2018 and also does not find support in the international literature.

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# Management of sickle cell disease 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 USA.

# 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: management of acute and chronic pain. Blood Adv 2020; 4 (12): 2656-2701. doi: https://doi.org/10.1182/bloodadvances.2020001851 # Figueiredo, FA; Brandmand, MAA. Clinical protocol and therapeutic guidelines for sickle cell disease. Joint Executive Order no. 05, of 19 February 2018. Ministry of Health, National Board of Health, Department of Science, Technology and Strategic Inputs. # Evidence-based treatment of sickle cell disease: Expert Panel Report, 2014. National Institute of Health.

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