Managing Emergencies Richard Ward, MSc, MRCP, FRCPath Director, Red Blood Cell Disorders Program, UHN Assistant Professor, Hematology, University of Toronto Chair, Canadian Hemoglobinopathy Association
When is Sickle Cell an Emergency? Parental instinct Fever Large abdomen Drowsiness or Extreme lethargy Severe headache or weakness Priapism
Bring with.. A friend or family member Something to drink (and eat) Pain medication Know your Hemoglobin level Let ED know you have SCD, immediately Ask the MD to contact your Sickle Cell team for advice Inform the clinic ASAP you have been to ED or admitted and book a clinic apt soon after discharge
https://www.sickkids.ca/myhealthpassport/
Why Do Patients Become Ill?
What Triggers Sickling? Hypoxia/Asthma Fever Extreme heat Obstructive Sleep Apnea Anxiety/Stress Menstruation Pregnancy Infection Extreme cold weather
Sickle Cell Bony Pain Crisis Ballas SK. Hemoglobin. 1995;19:323-333.
Treatment to Expect during a Crisis Tylenol + Advil + Opiate Regular assessment to achieve rapid, sustained, effective, and safe multimodal analgesia
Sickle Cell and Stroke 10% of children 1 in 4 adults
Neurological Events in SCD Moyamoya Overt Stroke Silent Cerebral Infarct Vasculopathy TIA
What may Signal a Stroke? Weakness, speech, loss of consciousness Can manifest differently from the general population Severe headache could be an hemorrhage Be aware of any subtle changes in personality or behaviour The only treatment is an exchange transfusion
Fever Lack of spleen predisposes to serious infection The body is often unable to fight the infection without prompt antibiotics Make sure you are up to date with vaccinations, including pneumoccus, meningococcus, Hemophilus, and influenza Take penicillin regularly in childhood
Always have a working, Celsius reading thermometer at home 38.0 o C x2 or 38.3 o C is significant Have oral antibiotics at home or when travelling, just in case Infection can be located: chest, urine, bone, brain A sickle cell crisis can cause a mild fever, but NEVER assume this to be the case until infection has been ruled out
In ED, you should receive IV antibiotics quickly and investigated for the source of infection This may involve x-rays, CT scan and a lumber puncture You will likely be admitted for several days and require at least a week of antibiotics Acute Chest Syndrome is most commonly triggered by infection You should stop Hydroxyurea if febrile
Acute Chest Syndrome fever (>38.5 o C) chest symptoms new findings on chest x-ray Precipitants are commonly: Infection Post-operative During a severe bony pain crisis Not breathing deeply enough VICIOUS CYCLE Sickling in lungs Lower Oxygen Low Oxygen More sickling
How Will I be Treated for ACS? Oxygen Fluids Analgesia Inhalers if wheezy or asthmatic Antibiotics Incentive Spirometry or respiratory therapist referral Blood transfusion Possible need for ICU
Splenic Sequestration
Break the Taboo of Priapism Painful, unwanted erection due to sickling in the penis Often not volunteered, stigma Triggered by a full bladder or intercourse Can cause permanent damage if left untreated Attend ED if persists >3 hours
Dealing with Priapism Treat it like any other crisis. but also. Pee before going to bed at night Don t drink too much in the evening Take a warm bath or shower Sedatives may be given for relaxation Urology may need to perform a small procedure
A Double Edged Sword Improve oxygen supply by improving anemia Reduce the sickling process Difficult to find compatible blood Iron build up if multiple transfusions
Exchange the S for A In certain situations, it s required to reduce HbS to <30%: Acute Chest Syndrome Multiorgan failure Stroke Prior to major surgery We remove patient s blood and replace it with donor s blood: This will lead to removal of sickle cell blood and replaced by normal blood