What is the count of sickle cell crises (Sickle Cell Disease, SCD)?

From the Guidelines

To count sickle cell crises, healthcare providers should track the number of painful episodes requiring medical intervention or treatment over a specific time period, usually annually, as recommended by the most recent guidelines 1. This involves documenting each acute pain episode, emergency department visit, hospitalization, or other medical encounter related to sickle cell disease complications. Patients can maintain a personal log recording the date, duration, pain severity (using a scale of 1-10), location of pain, treatments used, and whether medical care was sought. Some key points to consider when counting sickle cell crises include:

• The frequency and severity of painful episodes
• The number of emergency department visits and hospitalizations
• The presence of any complications, such as acute chest syndrome or stroke
• The effectiveness of current treatments and the need for adjustments to pain management protocols. Regular documentation of crises is essential for proper disease management as the frequency of crises can indicate disease progression or the need for more aggressive treatments like hydroxyurea or chronic transfusion therapy, as noted in a study published in 2021 1. Patients should work closely with their hematologist to establish a consistent method for tracking these episodes to ensure accurate monitoring of their condition, which is crucial for improving morbidity, mortality, and quality of life outcomes in patients with sickle cell disease, as highlighted in the 2019 American Society of Hematology guidelines 1.

From the Research

Sickle Cell Crisis Episodes

• The prevalence of acute pain crisis in sickle cell disease patients is high, with 92.6% of patients experiencing at least one episode in their lifetime and 72.4% in the last year 2.
• The mean severity of acute pain crisis is 6.8±3.1 on a scale of 0 to 10 2.
• Chronic pain and neuropathic pain are also common in sickle cell disease patients, with prevalences of 21.5% and 17.9%, respectively 2.
• Female sex, presence of current leg ulcers, and use of strong opioids are associated with higher odds of neuropathic pain, while older age, milder genotypes, and daily analgesic use are associated with higher odds of chronic pain 2.

Management of Sickle Cell Crisis Pain

• Oral morphine protocols can be effective in reducing the number of emergency department visits, total number of hours spent in the emergency department, and proportion of visits that end in hospital admission 3.
• Guidelines for managing acute pain crisis have been developed, but are often not followed, and acute care in emergency departments and on acute medical wards is suboptimal 4.
• Innovative means of delivering initial opioids, availability of specialist units, and individual care plans can potentially improve patient experience and outcomes 4.
• Alternative treatments, including pharmaceutical and non-pharmaceutical approaches, as well as procedural approaches, may be effective in reducing chronic pain in sickle cell disease patients 5.