Patients

What Is Acute Sickle Cell Pain?

People with sickle cell disease sometimes have painful episodes known as acute sickle cell pain.

Acute sickle cell pain can happen anywhere in the body, such as the arms, legs, joints, back, or chest. It can come on suddenly and range from mild to severe. The pain can last for a few hours, a few days, or sometimes longer.

What Causes Sickle Cell Pain?

Sickle cell disease changes the shape of red blood cells. Instead of being flexible and disc-shaped, they’re curved and stiff. These sickle-shaped blood cells don't flow through blood vessels easily, and can clog the vessels. If this happens, blood and oxygen can't get through, and parts of the body (like the heart, lungs, and kidneys) can't work the way they should. The blockage also can cause pain.

Pain is more likely when someone is sick, dehydrated, cold, or stressed.

Pain is a common problem for people with sickle cell disease (SCD). It happens when the sickle-shaped red blood cells that cause the condition get stuck in small blood vessels and block blood flow. That can cause a pain episode or crisis to start suddenly, usually in your lower back, arms, legs, chest, and belly. For others, health problems from sickle cell disease can cause pain that lasts longer and becomes chronic. Controlling pain associated with sickle cell can be difficult and could affect your mental health, causing stress and depression.

But no matter the type of sickle cell pain you have, there are many options that can help you find relief. Talk to your doctor about making a plan to manage your pain.

How to Manage a Pain Crisis

Most of the time, over-the-counter pain medicines like acetaminophen, aspirin, or ibuprofen can ease pain from an episode. (People under age 19…

Abstract

BACKGROUND

Hemoglobin SC (HbSC) is a common sickle hemoglobinopathy that causes acute complications, chronic organ damage, and early death with no established disease-modifying treatment. In this trial, we examined the safety and efficacy of hydroxyurea treatment in patients with HbSC.

METHODS

Prospective Identification of Variables as Outcomes for Treatment (PIVOT) was a double-blind, randomized, placebo-controlled, non-inferiority phase 2 trial in which we assigned children and adults with HbSC in Ghana to 12 months of hydroxyurea or placebo. The primary end point was hematologic dose-limiting toxicities (DLTs), including cytopenias or elevated hemoglobin levels during 12 months of blinded treatment. Clinical end points included vaso-occlusive pain events, acute chest syndrome, hospitalizations, transfusions, and malaria. Quality-of-life measures, organ function assessments, and rheological measurements were also collected.

RESULTS

Abstract

Sickle cell disease (SCD), which affects approximately 100,000 individuals in the USA and more than 3 million worldwide, is caused by mutations in the βb globin gene that result in sickle hemoglobin production. Sickle hemoglobin polymerization leads to red blood cell sickling, chronic hemolysis and vaso-occlusion. Acute and chronic pain as well as end-organ damage occur throughout the lifespan of individuals living with SCD resulting in significant disease morbidity and a median life expectancy of 43 years in the USA. In this review, we discuss advances in the diagnosis and management of four major complications: acute and chronic pain, cardiopulmonary disease, central nervous system disease and kidney disease. We also discuss advances in disease-modifying and curative therapeutic options for SCD. The recent availability of L-glutamine, crizanlizumab and voxelotor provides an alternative or supplement to hydroxyurea, which remains the mainstay for disease-modifying therapy. Five-year event-free and overall survival rates remain high…