Download Guidelines for Management in Children with Sickle Cell

This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Document Scope: Hospital-wide Patient Care
Document Type: Clinical Practice Guideline
Approved on 2016-08-05
Next Review Date: 2018-08-05
Priapism: Guidelines for Management in
Children with Sickle Cell Anemia
Version: 2
Pseudoephedrine dosing adjustments in the e-formulary approved by Drugs & Therapeutics (D&T) Committee: April 20, 2016
Version 02 Approved by Interprofessional Patient Care Committee: May 6, 2016
1.0
Background
Priapism, a painful, prolonged (>30 minutes) erection of the penis, has been found to occur at least once in 75% of males
with sickle cell anaemia before the age of 21 years. There are two bimodal peaks (ages 5–13 and 21–29y). In a majority
of cases, priapism occurs during early-morning sleep and awakens the patient. Sexual activity is a precipitating event in
approximately 20% of cases; however, in 40% of cases no provoking event is identified. Priapism occurs in two forms:
stuttering, which lasts 2h or less, and severe, which lasts more than 2h and may result in impotence.
The pathophysiology of priapism in sickle cell disease remains elusive. It is thought that the relative stasis of blood within
the corpora during normal erection decreases the oxygen tension and pH, both circumstances conducive to sickling.
Sickling of RBCs and sludging of blood within the corpora leads to further hypoxia and acidosis, which in turn promotes
further sickling.
Eventually an inflammatory response is elicited, resulting in fibrosis; it is this that ultimately is responsible for impotence.
Priapism is more common in patients with SS disease, but can also occur in those with SC disease or Sß thalassemia.
The best treatment for priapism in patients with sickle cell disease is not known. Analgesia and hydration are of benefit,
but the role of transfusion (if any) or surgery is not clear. Positive prognostic predictors in priapism include being
prepubertal, early presentation, and early treatment. In managing priapism, one should distinguish stuttering priapism
from the severe form. In stuttering priapism, no specific intervention is required for a single episode: Simple treatments
such as hydration, warm baths, and analgesics are usually sufficient to end it. Severe or prolonged events (>2h) are to be
considered emergencies requiring prompt medical intervention.
2.0
Clinical Practice Recommendations
Statement of Evidence: Recommendations were made by expert group consensus. (Grade C)
Table 1. Grades of Recommendation
A Recommendation supported by at least one randomized controlled trial, systematic review or meta-analysis.
B Recommendation supported by at least one cohort comparison, case study or other experimental study.
C Recommendation supported by expert opinion or experience of a consensus panel.
2.1
Emergency Department Management
2.1.1
Rapid triage - place immediately into exam room.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 1 of 4
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Document Scope: Hospital-wide Patient Care
Document Type: Clinical Practice Guideline
Approved on 2016-08-05
Next Review Date: 2018-08-05
Version: 2
Priapism: Guidelines for Management in
Children with Sickle Cell Anemia
2.1.2
Consult urology immediately on arrival in the Emergency Department. The Urology fellow shall advise on whether
he/she will do penile aspiration–drainage and irrigation with epinephrine (usually if priapism has been present for
> 4 hours).
2.1.3
Inform the Hematology fellow on consults (or the Hematology/ Oncology fellow on call if after hours and on
weekends).
2.1.4
Tests: CBC, reticulocyte count, blood typing and extended cross-matching, sodium, potassium, creatinine, urea,
glucose. Ensure that “Sickle Cell Disease” is written on the type and screen requisition. Urine - routine analysis
and culture. Blood cultures if febrile.
See Fever: Guideline for Management of Children with Sickle Cell Disease protocol ==>
2.1.5
.
If aspiration with sedation is being considered for management, keep NPO and administer IV fluids at
maintenance rate. If dehydrated, consider a 10 mL/kg bolus over 1 hour. If patient does not need to be NPO,
encourage oral fluids whenever possible. See Fluid and Electrolyte Administration in Children ==>
2.1.6
For patients in moderate to severe pain, refer to the Acute Painful Episodes Vaso-occlusive Crisis: Guidelines for
Management in Children with Sickle Cell Disease and accompanying order set.
2.1.7
If admission is required for pain control, admit under Paediatric Medicine with Urology consult.
2.1.8
Administer 1 dose of oral pseudoephedrine in the ED (refer to e-formulary link for dosing information).
2.1.9
Ice or cold packs should not to be used. Sitting in a warm bath can be helpful.
2.1.10 Administer oxygen to maintain O2 saturation ≥ 95%.
2.2
Inpatient Management
2.2.1
Consult Acute Pain Service to request an epidural to improve pain control if inadequate pain control with IV
morphine.
2.2.2
Continue oral pseudoephedrine (refer to e-formulary link for dosing information).
2.2.3
Ice or cold packs should not to be used. Sitting in a warm bath can be helpful.
2.2.4
Administer oxygen to maintain O2 saturation ≥ 95%.
2.2.5
After 12h, if there is no evidence of detumescence, do an exchange transfusion to reduce HbS <30%.
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 2 of 4
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Document Scope: Hospital-wide Patient Care
Document Type: Clinical Practice Guideline
Approved on 2016-08-05
Next Review Date: 2018-08-05
Priapism: Guidelines for Management in
Children with Sickle Cell Anemia
Version: 2
2.2.6
For recurrent priapism, discuss the use of GnRH analogues (Lupron, injected intramuscularly every month) with a
consultant from Urology. Sildenafil has been used with some success.
2.3
Discharge Criteria (applicable to discharges from the Emergency Department and Inpatient Units)
2.3.1
The priapism is resolving (complete detumescence may take 1–2 weeks).
2.3.2
The patient is afebrile.
2.3.3
The patient is taking fluids and medications orally.
2.3.4
Pain relief is adequate.
2.3.5
For recurrent priapism continue pseudoephedrine (refer to e-formulary link for dosing information).
2.3.6
Education and discharge pain plan provided. Urology follow-up arranged.
3.0
References
1. Burnett et al. Randomized controlled trial of sildenafil for preventing recurrent ischemic priapism in sickle cell disease.
Am J Med. 2014 Jul;127(7):664-8.
2. Hamre MR, Harmon EP, Kirkpatrick DV, et al. Priapism as a complication of sickle cell disease. J Urol. 1991;145:1-5.
3. Powars DR, Johnson CS. Priapism. Hematol Oncol Clin North Am. 1996;10:1363–72.
4. Sharpstein JR, Powars D, Johnson C, et al. Multisystem damage associated with tricorporal priapism in sickle cell
disease. Am J Med. 1993;94:289–95 [a prospective 25-year cohort study of 461 men with sickle cell, of whom 38
developed clinically significant priapism].
Revised (2016) – Listed alphabetically
1. Armando Lorenzo, MD, Staff Urologist, Urology
2. Averill Clarke, Senior Manager, Haematology/Oncology Clinic & Day Hospital
3. Carolyn Beck, MD, Staff Paediatrician, Division of Paediatric Medicine
4. Jeremy Friedman, MD, Staff Paediatrician, Division of Paediatric Medicine
5. Lynn Mack, RN, Quality Analyst 7BCD
6. Marcia Palmer, RN, Division of Haematology/Oncology
7. Marina Strzelecki, Clinical Pharmacist
8. Melanie Kirby, MD, Staff Physician, Division of Haematology/Oncology
9. Melina Cheong, RN, Nurse Practitioner, Division of Haematology/Oncology
10. Olivia Ostrow, MD, Staff Paediatrician, Division of Paediatric Emergency Medicine
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 3 of 4
This is a CONTROLLED document for internal use only. Any documents appearing in paper form are not controlled and should be checked against the
electronic SharePoint version prior to use.
Document Scope: Hospital-wide Patient Care
Document Type: Clinical Practice Guideline
Approved on 2016-08-05
Next Review Date: 2018-08-05
Priapism: Guidelines for Management in
Children with Sickle Cell Anemia
Version: 2
Attachments:
Revision History.docx
© 2012 The Hospital for Sick Children ('SickKids'). All Rights Reserved. This document may be reproduced or used strictly for non-commercial clinical
purposes. However, by permitting such use, SickKids does not grant any broader license or waive any of its exclusive rights under copyright or
otherwise at law; in particular, this document may not be used for publication without appropriate acknowledgement to SickKids. This Clinical Practice
Guideline has been developed to guide the practice of clinicians at the Hospital for Sick Children. Use of this guideline in any setting must be subject to
the clinical judgment of those responsible for providing care. SickKids does not accept responsibility for the application of this guideline outside
SickKids.
Page 4 of 4