by Lauren Taylor from In The Cloud Copy
Sickle cell disease or SCD is a type of red blood cell disorder in which the normally round red blood cells are sticky, hard and look C-shaped rather than round. Red blood cells are responsible for carrying oxygen throughout the body via the small blood vessels. There are different types of SCD depending on the genes inherited and the severity of the disease. Commonly seen complications associated with SCD include stroke, organ damage, ulcers, blindness, pulmonary hypertension, acute chest syndrome, and more.
A stroke occurs when the blood supplying the brain is reduced or interrupted for a period of time. This results in the brain cells beginning to die and is an acute emergency that requires immediate medical attention. In SCD patients, a stroke can occur when the sickle-shaped cells begin to clump together and block the arteries and blood vessels that lead to the brain. Treating a stroke usually involves quickly dissolving the clot that is preventing the blood flow to the brain.
Managing a Stroke in SCD Patients Safely and Effectively
Patients with sickle cell disease hemoglobin S are most likely to be affected by strokes, but the overall rate of stroke is higher in any patient with SCD compared to the general population. The general population is typically treated with tissue type plasminogen activator or tPA which helps to quickly break down the clot that is preventing blood flow to the brain. Unfortunately, for SCD patients, there was an ongoing debate, spanning at least two decades, over whether or not giving tPA to stroke patients with SCD was contraindicated or not. Two years ago, the American Stroke Association and American Heart Association finally announced that SCD patients who presented with an acute ischemic stroke could be offered intravenous tPA safely.
A Successful Story of TPA Use in the SCD Population
A young woman, 35 years old, with known SCD came to an emergency room with an evolving left sided stroke. Her symptoms included aphasia that was worsening and right hemiparesis. She had previous complications of SCD and had required multiple admissions prior for vascular occlusive crises. She did not see her hematologist regularly for follow up care. A CT scan and a CT angiogram were both performed which revealed an occlusion of the left MCA and evolving infarction.
The patient was immediately given intravenous (IV) tPA to achieve thrombolysis. This was given within approximately 35 minutes of her arrival at the emergency department. She also was given an urgent hemoglobin exchange transfusion due to high hemoglobin S levels. She was set to be transferred to another hospital. Prior to her transfer, her symptoms were mildly improving, including both aphasia and her right sided weakness. She was discharged from the hospital shortly after with anti-platelet medication to prevent future episodes. Follow up in the clinic 3 months later showed drastic symptom improvement and no new symptoms.
Patients with SCD are much more likely to have a stroke compared to the general population, with a rate greater than 3.5%. Treatment with tPA was contraindicated until 2018 in patients of her profile. While the risk of intracranial hemorrhage exists when treating SCD patients with tPA and these risks must be explained to both patients and families, treatment with tPA should still be considered after looking for and eliminating any underlying causes. Treatment with tPA in this population is considered safe and effective.
Check out the original case study here.