Pain Crisis and Acute Chest Syndrome: The Two Most Common Sickle Cell Emergencies

The acute painful episode, or vaso-occlusive crisis, is the single biggest problem for patients with sickle cell disease and its most common clinical complication. Sickled cells become entrapped and occlude the capillaries, and this is accompanied by the release of inflammatory mediators, together driving the pain and the recurrent cycles of ischaemia and reperfusion that follow. Around one-third of patients have pain almost daily, and most learn to manage it at home, but severe or refractory crises may need hospital admission, sometimes lasting as long as two weeks. Crises are commonly triggered by very cold temperatures, dehydration, infection, fever or stress of any kind, including simple physiological stress such as a hypoxic environment at high altitude.

How Pain Crisis Is Managed

Non-steroidal anti-inflammatory drugs are the first-line treatment for acute pain crisis. Patients may subsequently need opioid medication, and if pain does not resolve, ketamine infusion or regional anaesthesia can be considered for refractory pain. Non-pharmacological approaches such as massage and yoga may also help. Supportive measures, oxygenation, hydration, identifying and treating any source of infection with antibiotics, and incentive spirometry, all contribute to recovery. Blood transfusion is not indicated for a simple, uncomplicated pain crisis.

Acute Chest Syndrome

Acute chest syndrome is the second most common cause of hospital admission in sickle cell disease and its leading cause of premature death, with a recurrence rate of around 80%. It is diagnosed on the basis of acute pulmonary infiltrates, though the X-ray picture is frequently delayed by as much as 12 hours behind the clinical picture. It typically presents like pneumonia, with fever, chest pain, shortness of breath, cough and reduced oxygen saturation, and its onset is often 24 to 72 hours after an acute pain episode. Causes include lung infection, embolism (including fat embolism), pulmonary infarction and hyperventilation; the standard work-up is a complete blood count, inflammatory markers, a blood gas, blood cultures and a chest X-ray.

Managing and Preventing Acute Chest Syndrome

Treatment combines IV fluids, oxygenation, control of the underlying pain crisis, adequate antibiotics, incentive spirometry and close monitoring. If the pneumonia progresses, saturations fail to settle, or oxygen demand keeps increasing, blood transfusion may be needed; if the situation still has not settled after 12 hours, a red cell exchange may be considered depending on the clinical picture. Primary prevention rests on optimally controlling pain crises, close monitoring, incentive spirometry, and pre-emptively treating infections, while secondary prevention centres on starting hydroxyurea and considering chronic transfusion.

This article is based on a Jivo Masterclass session conducted by Dr. Divya Doval, Senior Consultant, Bone Marrow Transplant & Haemato-Oncology, BLK-Max Super Speciality Hospital, New Delhi. The article has been summarised with the assistance of an AI tool from the original masterclass recording. Watch the full Masterclass recording