Sickle Cell Crisis

#Sickle Cell Crisis
Pert +/- Hx: Endorses/Denies: Vaso-occlusion and infarction symptoms: painful crises, acute chest syndrome, hand-foot syndrome, priapism. Denies/endorses: S/sx of infection (meningitis, bacteremia, Pneumonia) s/sx of cardiac disease (cardiomyopathy, heart failure, MI, dysrhythmia, sudden death), neurologic s/sx (TIA, stroke or CVA, seizures,PRES–posterior reversible encephalopathy syndrome), pulmonary s/sx (asthma, pneumonia, acute chest syndrome, sleep disordered breathing), skeletal s/sx (bone pain, dactylitis, etc); leg ulcers, VTE, retinopathy (no vision changes). Is the patient dehydrated or hypoxic? These may trigger sickling.
Pert +/- PE: Assess volume status
Pert +/- Labs: UA & CMP (to look for renal complications of SS); CBC (look for anemia, infection)
Pert +/- Imaging/Studies: CXR, EKG, CT head (if indicated)
Patient c/o of multiple joint pains. Patient has no hypoxia. Hemoglobin is not low enough. No need for transfusion for now.

Treatment:
Will admit to telemetry. cardiac monitor.
Treat pain crises with hydration, oxygen, and pain control (analgesia).
Hydration with NS (replacement PLUS maintenance fluids), oxygen via NC, and opioids for pain control. PCA would be optimal but is not available.
Will schedule continuous opioid (around the clock) pain control PLUS adequate breakthrough pain medication.
Will monitor for sedation; provide incentive spirometer (10 breaths hourly while awake.
Will provide stimulant (not osmotic) laxatives.
Will have naloxone and flumazenil available, prn.
Cont hydroxyurea (it causes incr. in HbF which leads to decr. in painful crises, acute chest episodes and may decrease mortality)
Cont folic acid daily.
Will treat insomnia as needed (b/c it may be exacerbated by anxiety and poor pain control).
Will consult social services as needed.
Will consider nonpharmacologic therapies such as heat packs or hydration for acute pain and deep tissue massage for chronic pain if necessary. However, we will avoid ice packs and cold compresses in the setting of acute vasoocclusive pain because cold may precipitate sickling.
Will consider simple transfusion or exchange transfusion if the patient develops TIA, stroke or severe acute chest syndrome.
VTE prophylaxis with Enoxaparin or heparin (Why? B/c SS patients appear to have a hypercoagulable state at baseline)
Verify that patient is UTD with pneumococcal, meningococcal, H. flu & HBV vaccinations (and other usual vaccinations).
If surgery is needed (and this is unlikely now), will keep preoperative hemoglobin goal of 10.
I recommend that the patient’s outpatient hematologist consider the possibility of allogeneic hematopoietic stem cell transplantation for this patient with severe disease.
Continue to monitor clinically.

Clinical Manifestations of Sickle Cell Disease

“This patient has acute chest syndrome (ACS), a serious vaso-occlusive complication of sickle cell disease (SCD). Its cause may be multifactorial, but infections are common and antimicrobials are indicated. However, the clinical course of ACS is significantly different from infectious pneumonia in patients without SCD, due to the damaged microvasculature that occurs in ACS. Studies have shown that atypical pathogens predominate in ACS and it is therefore important to treat all patients with ACS with antibiotics that cover Mycoplasma and Chlamydophila. Viral infections are also common, especially in children with ACS. Other possible pathogens include Staphylococcus aureus, Streptococcus pneumoniae, and Haemophilus influenzae. Therefore, the use of a third-generation cephalosporin along with azithromycin is the recommended antibiotic coverage.
In addition to antimicrobials, treatment includes supportive care with supplemental oxygen, intravenous fluids, pain control, and incentive spirometry. Depending on the degree of anemia seen, a simple blood transfusion or exchange transfusion is often indicated as well. Consultation with a hematologist is recommended in the care of patients with ACS. Even with appropriate care, mortality rates in ACS are as high as 3%.”

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