If you suffer from Sickle Cell Anemia or have someone close to you who suffers from it, then you will know that a big part of Sickle Cell is the acute pain that such a person goes through.
Acute pain episodes are the most common reason for individuals with sickle cell disease (SCD) to seek medical attention. The frequency of pain episodes is likely to be underestimated because these episodes are managed at home, especially as individuals with SCD grow older and learn how to manage pain on their own.
Acute painful sickle cell episodes (also known as painful crises) are caused by blockage of the small blood vessels. The red blood cells in people with sickle cell disease behave differently under a variety of conditions, including dehydration, low oxygen levels and elevated temperature. Changes in any of these conditions may cause the cells to block small blood vessels and cause tissue infarction. Repeated episodes may result in organ damage.
Patients with the disease often experience acute or chronic pain. Opioids tablets sometimes may be used to manage this pain. However, some clinicians have biases against opioid use. As a result, the patient’s pain is sometimes not efficiently managed.
Acute painful episodes in sickle cell anemia patients usually develop as a result of a vaso-occlusive crisis (VOC) and should be treated as a medical emergency. VOC occurs when sickle-shaped red blood cells block small blood vessels so that the blood cannot flow normally. This leads to tissue damage and causes pain.
Acute painful sickle cell episodes occur unpredictably, often without clear precipitating factors. Their frequency may vary from less than one episode a year to severe pain at least once a week. Pain can fluctuate in both intensity and duration, and may be excruciating. The majority of painful episodes are managed at home, with patients usually seeking hospital care only if the pain is uncontrolled or they have no access to analgesia. Patients who require admission may remain in hospital for several days. The primary goal in the management of an acute painful sickle cell episode is to achieve effective pain control both promptly and safely.
The acute sickle cell painful crisis is the hallmark of SCD and the number one cause of hospitalization. It is unpredictable and may be precipitated by known or unknown risk factors and triggers. Much of the devastation caused by the disease is the result of the recurrent acute painful crises. Clinical features of a typical painful crisis were described accurately by Diggs, that “patients experience sudden onset of pain in the low back or in one or more joints or one of the extremities. The pain may be localized or migratory and is continuous and throbbing. The severe pain causes patients to grunt, groan, cry, twist and turn and to assume abnormal postures in the futile attempt to obtain relief.” Descriptors of pain also include throbbing, sharp, pounding, dull, stabbing, cutting, and gnawing or like a generalized toothache.
Treatment of an acute painful sickle cell episode should be as an acute medical emergency. Throughout an acute painful sickle cell episode, regard the patient (and/or their carer) as an expert in their condition, listen to their views and discuss with them: the planned treatment regimen for the episode treatment received during previous episodes any concerns they may have about the current episode any psychological and/or social support they may need.
Clinically assess all patients presenting at hospital with an acute painful sickle cell episode, including monitoring of: blood pressure oxygen saturation on air (if oxygen saturation is 95% or below, offer oxygen therapy) pulse rate respiratory rate temperature.
I cannot talk about what medication to take here because I am not a medical doctor. But I wanted you to know that a big part of looking after someone with SCD is managing the acute pain he or she is going through during a crisis.
Be aware of the possibility of acute chest syndrome in patients with an acute painful sickle cell episode if any of the following are present at any time from presentation to discharge, including: abnormal respiratory signs and/or symptoms chest pain fever signs and symptoms of hypoxia: oxygen saturation of 95% or below or an escalating oxygen requirement acute stroke aplastic crisis infections osteomyelitis splenic sequestration.
Non-pharmacologic approaches, such as distraction, local heat application, massage, and positioning might provide additional pain relief.
This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.