SCD patient management in ER (Emergency Department) (Grade C) [12]

ER assessment of SCD patients with fever: If SCD patient has consistent fever of 38.5 °C, transfer patient on ER bed and follow “Clinical Guidelines for Fever Management”.  Carry out a thorough assessment of the patient after administration of antibiotics (Appendix 1).

ER assessment of SCD patients without fever: Transfer SCD patient without fever to a BED at the earliest. Take brief history and carry out physical examination concurrently with measures as given in Table 1. If child is having fever, respiratory illness or/and fever, go for x-ray of the chest as well as monitor his/her oxygen (O₂) saturation. If patient is complaining of severe respiratory distress, request for arterial blood gas (ABC) level testing additionally [13, 14].

If you find patient febrile (feverish), dehydrated or complaining of moderate to severe pain, insert an intravenous (I.V.) catheter. Page and inform the Hematology consult service if available. Hematology consultant should examine all SCD patients who are have serious condition and should arrange their proper follow-up. Following are criteria to see if any patient has serious illness:

• Patients in shock (i.e. life-threatening medical condition) with symptoms are very low blood pressure etc.
• Patients with aplastic crisis, acute splenic sequestration crisis, sepsis, meningitis, stroke etc.
• Decompensating patients, for example, patients who progressing into chest crisis etc.
• Patients showing oxygen saturation less than 90% while breathing room air.

In case of fever, acute chest crisis, gallstones etc., please follow other guidelines of “Sickle Cell Management Protocols” specific to these complications [14-16].

Pain management through Medication in Emergency department (ER)

Please adopt protocols provided in table 2 to manage pain, depending upon pain severity [17, 18].

Adjunct pain relief management: For mild, moderate and severe pain, Acetaminophen 75 mg/kg/day PO (maximum dose 4g/day) and an non-steroidal anti-inflammatory drug (NSAID) can be given as adjunct pain relief medication along with morphine, unless there are any contraindications to their use. Before using NSAIDs, assess renal function and consult with pharmacist if you find results abnormal [17].

Recommended NSAIDs include:

Ibuprofen: For children 6 months-12 years old: 5-10 mg/kg/dose PO q6-8h (orally, every 6-8 hours); Not to exceed 40 mg/kg/day or 2400 mg/day.

Naproxen: 10-20 mg/kg/day PO divided BID (orally, twice daily); Not to exceed 1 g/day).

Ketorolac: 0.5 mg/kg/dose intravenous (I.V.) q6-8h (every 6-8 hours); Not to exceed 15 mg/dose), maximum for 48 hours and after that change switch to ibuprofen or naproxen.

Pain management for Indoor patients [18-20]

You need to admit patient if pain is inadequately controlled with oral analgesics or in case of other complications such as fever, dehydration etc.

Patients should be admitted to hospital ward only under General Pediatrics Service.

Pain management through Medication for outdoor patients [18, 19]

• Prescribe oral morphine dose that adequately relieved pain as stated in 2.1.7 (don’t exceed 15 mg/dose). In case patient needed higher doses during previous ER visits for painful VOC, consult patient chart and hematology service. Reevaluate the patient if in not relieved in 48 hours.
• For mild, moderate and severe pain, Acetaminophen 75 mg/kg/day PO (maximum dose 4g/day) AND a non-steroidal anti-inflammatory drug (NSAID) can be given as adjunct pain relief medication along with morphine, unless there are any contraindications to their use. Please consult table 3 for dosing.

Patients are needed to be observed closely for signs of deterioration and are required to be monitored continuously with cardiac and oxygen (O₂) saturation, for vital signs every four hours, for fluid input and output as well as daily weight.  It is required to carry out assessment of patient’s comfort level every 4 hours. This assessment is also required before as well as after every treatment provided for pain management and even after any non-pharmacological management of with pain.

If patient is hospitalized with chest and back pain, refer to him/her to Physiotherapy. It is recommended for physiotherapist to determine if incentive spirometry (10 breaths every 1-2 hours while awake) is appropriate for older children (8 years or older). For younger children, physiotherapist should motivate them use bubbles for deep breathing. The physiotherapist should also encourage walking without any support (ambulation) and any other activities keeping in consideration patient’s tolerance. Per recommendations of physiotherapists, other health professionals involved should also promote child’s mobility [13].

Referral of the patient to Physiotherapy is required if he/she is showing significant decrease in mobility or restricted joint movement.

To improve patient comfort level, non-pharmacological interventions such as massage, heating pads, warm baths, and other such methods should be utilized. It is advisable for “Child Life representative” to recommend structured daily activity for the patient. Use of Imagery and distraction prove to be helpful.

In case if pain is not relieved even after using analgesics or if any pain-related are encountered, consultation with Pain Management Team is highly recommended.

Give patient boluses of morphine intravenously for breakthrough pain (consult table 4 for exact doses).  After maintaining effective dose for 24 hours, gradually decrease dose by up to 20 micrograms/kg/hour in next 24 hours. If patient is stable, continuous intravenous morphine may be replaced by an equal dose of long-acting oral morphine (consult table 4 for exact doses).

Step-down therapy: For those patients who are comfortable, it is recommended to change to oral analgesics (morphine 0.3-0.5 mg/kg/dose orally every 4 hours as needed (PO, q4h, PRN prn). Don’t exceed 15 mg/dose (consult table 4 for exact doses).

For mild, moderate and severe pain, Acetaminophen 75 mg/kg/day PO (maximum dose 4g/day) AND a non-steroidal anti-inflammatory drug (NSAID) can be given as adjunct pain relief medication along with morphine, unless there are any contraindications to their use (consult table 4 for exact doses).

In case of pain persisting for more than 24-48 hours in patients older than 6 years of age, using Patient Controlled Analgesia (PCA) pump is recommended [21-23]. Similarly, utilize a PCA pump for any child already treated with a PCA pump or for children who did not achieve adequate analgesia. A PCA pump has capability to deliver a continuous infusion as well as intravenous boluses of morphine or any other medication per patient need and a required dose and interval of medication can be pre-programmed by the prescriber (physician) and therefore chances of overdosing are negligible if PCA pump is used. Please contact (through pager) the Pain Management Team to get PCA pump and they can set it at its basal rate, bolus rate, and lockout time. It is mandatory to observe patients carefully for opioid toxicity when patients are on morphine. The indications of opioid toxicity include hypotension, bradycardia, drowsiness, coma, pinpoint pupils, cold clammy skin, and hypoventilation. It is required to regularly monitor and record patient's heart rate, respiratory rate and oxygen saturation while observing patient for opioid toxicity.

Use of stool softeners: If the patient does not have diarrhea, it is required to administer him/her a stool softener such as Docusate Sodium (5 mg/kg/day for children). You administer it in three equal doses or as a single daily dose.  Adult daily dose is 100-200 mg.