Sickle Cell Crisis

Sickle cell crisis is best approached as an acute syndrome in a patient with sickle cell disease rather than as a single diagnosis. Vaso-occlusive pain is common, but acute care evaluation must account for acute chest syndrome, infection, acute neurologic events, and other complications that require disease-specific escalation or transfer. ASH-related implementation guidance emphasizes timely emergency evaluation for acute stroke, pain, and fever, with continuity of specialty care and prearranged transfer pathways when needed. ScienceDirectScienceDirectImplementing ASH’s guidelines for acute medical care for incarcerated children and adults with sickle cell disease

Acute chest syndrome is a high-priority diagnostic concern because it is frequent and serious in adults with sickle cell disease, accounts for a large share of ICU admissions, and may develop after admission for vaso-occlusive crisis. Reported contributors include lower respiratory tract infection, hypoventilation or atelectasis, bone-infarct-associated fat embolism, and in situ pulmonary thrombosis. NatureNatureInfectious aetiologies of severe acute chest syndrome in sickle-cell adult patients, combining conventional microbiological tests and respiratory multiplex PCR | Scientific Reports

ASH’s 2020 guideline addresses acute and chronic pain in children and adults with sickle cell disease and characterizes optimal care as interdisciplinary. The guideline also notes that limited sickle cell disease pain research and biologic differences between acute and chronic pain complicate targeted treatment decisions. ScienceDirectScienceDirectAmerican Society of Hematology 2020 guidelines for sickle ...

In emergency and inpatient settings, operational readiness matters. The ACEP Emergency Department Sickle Cell Care Coalition was formed to improve emergency care for this population, reflecting persistent care gaps and patient-reported poor ED experiences. acepacepEmergency Department Sickle Cell Care Coalition | ACEP Use a patient-specific acute-care plan when available, including the established analgesic regimen, prior effective agents, opioid tolerance, chronic analgesic exposure, disease complications, and specialist contact information.

Acute chest syndrome is clinically characterized by chest symptoms or signs such as chest pain, fever, or dyspnea together with new pulmonary abnormalities; the referenced adult study describes rapid clinical progression and identifies acute respiratory failure by respiratory rate above 30 breaths/min, increased work of breathing, or labored breathing. NatureNatureInfectious aetiologies of severe acute chest syndrome in sickle-cell adult patients, combining conventional microbiological tests and respiratory multiplex PCR | Scientific Reports Because the syndrome has multiple potential mechanisms, diagnostic evaluation should not focus solely on bacterial infection.

The available evidence describes a mixed pathophysiology that includes lower respiratory tract infection, hypoventilation and atelectasis, fat embolism related to bone infarction, and pulmonary thrombosis. NatureNatureInfectious aetiologies of severe acute chest syndrome in sickle-cell adult patients, combining conventional microbiological tests and respiratory multiplex PCR | Scientific Reports This breadth is clinically consequential: an unrevealing initial microbiologic evaluation does not eliminate acute chest syndrome when the clinical and radiographic syndrome is present.

Filgrastim products carry a specific warning for severe and sometimes fatal sickle cell crises in patients with sickle cell disorders. The RELEUKO prescribing information directs discontinuation if a sickle cell crisis occurs. Patients who report left upper abdominal or shoulder pain should also be evaluated for splenic enlargement or rupture, and patients with fever plus pulmonary infiltrates or respiratory distress should be evaluated for ARDS. dailymed nlm nihdailymed nlm nihThese highlights do not include all the information needed to use RELEUKO safely and effectively. See full prescribing information for RELEUKO. RELEUKO® (filgrastim-ayow) injection, for subcutaneous or intravenous use.Initial U.S. Approval: 2022 RELEUKO (filgrastim-ayow) is biosimilar* to NEUPOGEN® (filgrastim)

Sildenafil labeling warns about vaso-occlusive crisis in patients with pulmonary hypertension secondary to sickle cell disease, and the available label states that safety and effectiveness for pulmonary arterial hypertension secondary to sickle cell anemia have not been established. dailymed nlm nih+1dailymed nlm nihThese highlights do not include all the information needed to use SILDENAFIL TABLETS safely and effectively. See full prescribing information for SILDENAFIL TABLETS. SILDENAFIL tablets, for oral use Initial U.S. Approval: 1998dailymed nlm nihhighlights of prescribing information Do not interpret sildenafil’s approval for other pulmonary hypertension populations as evidence supporting use in sickle cell-associated pulmonary hypertension.

Sickle cell disease produces lifelong acute and chronic pain, end-organ injury, chronic anemia, and reduced survival. A U.S. primary care review emphasizes that optimal outcomes require a partnership among patients, primary care clinicians, hematologists, and other caregivers. ccjmccjmSickle cell disease: A primary care update Following a crisis, identify whether the patient has an established hematology team, a documented emergency plan, reliable follow-up, and barriers to medication access or specialist care.

For patients with severe complications or an appropriate risk-benefit profile, referral for curative-therapy assessment may be appropriate. ASH’s 2021 guideline states that hematopoietic stem cell transplantation is currently the only curative therapy for sickle cell disease, while also underscoring the need for evidence-based, individualized decisions. ScienceDirectScienceDirectAmerican Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation The supplied sources do not provide sufficient detail to specify transplant candidacy criteria, donor hierarchy, conditioning approach, or gene-therapy eligibility.