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Introduction Etiology - SCD

• Sickle cell disease (SCD) is a group of inherited red blood cell disorders.
• Sickle cell disease is an autosomal recessive disorder of a gene mutation.

• The hemoglobin is abnormal, causing the red blood cells to be rigid and shaped
• On chromosome 11, nucleotide mutation leads
like a "C" or sickle.

SICKLE CELL
to substitution of glutamic acid to valine at

• SCD results in anemia and "sickle cell crisis" position six on the beta-globin subunit. This

CRISIS
(SCC). leads to changes in the physical properties

• The main clinical feature of sickle cell disease of the globin chain.

is the ''acute painful crisis,’’ which often

requires hospitalization.
Zainab K. Manji, APN
(MScN, BScN)

Epidemiology Pathophysiology Diagnosis HPLC – high performance liquid

chromatography
• Globally – 300,000 births of Hb Electrophoresis

SCD per year.

• 75% of these births are in

Africa.

• Highest Childhood mortality

(50%-90%) in Africa.

• Tanzania - top 4 in the world • In 2004, Tanzania established the Muhimbili

with the highest estimated Sickle cell (MSC) program, integrating research
number of newborns with SCD and healthcare in a countrywide referral hospital
a year – 11,000 new births for all disease conditions, the Muhimbili
every year. National Hospital (MNH).
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Diagnosis Rapid Test

Therapy Sickle Cell Crisis
1) Hydroxyurea - increases red cell content of hemoglobin F levels (which reduces the
formation of hemoglobin S polymers), increases water content of red cells, increases • The term "sickle cell crisis" is used
deformability and successful microvascular navigation of sickled cells and altered adhesion to describe several acute
of red blood cells to endothelium. Treatment with hydroxyurea is associated with significant conditions such as:
Peripheral Smear
decreases in the yearly rate of painful crises, hospital admissions, incidence of chest
syndrome, priapism, hepatic sequestration, and blood transfusion requirements by as
much as 50%. Hydroxyurea treatment also reduces mortality by 40% - vaso-occlusive crisis
- aplastic & hemolytic crisis
2) Folic Acid - In patients with SCD, the RBC count is lower than normal because sickled cells - splenic sequestration crisis
usually die after 10 to 20 days, in contrast to 120 days for normal RBCs. Because of high
cell turnover, folate stores are often depleted. Folic acid replenishes the depleted folate - acute chest syndrome
stores necessary for erythropoiesis.
• Other acute complications -
3) Prophylactic antibiotics – preferably penicillin pneumonia, meningitis, sepsis
and osteomyelitis, stroke,
4) Immunizations avascular necrosis, priapism, and
venous thromboembolism.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2730092/

1. Vaso-occlusive Crisis (VOC) 2. Splenic Sequestration Crisis 3. Acute Chest Syndrome (ACS)
• The spleen is affected due to its narrow vessels, there is acute, painful enlargement
• It occurs when the microcirculation is obstructed by sickled of the spleen due to intrasplenic trapping of red cells. • This accounts for 25% of deaths and can follow VOC crisis.
RBC’s, causing ischemic injury to the organ supplied and resultant pain
• Trigger for ACS is frequently hypoxia due to hypoventilation of the chest caused by
• Patients present with moderate to severe pain • Patients with splenic sequestration crisis may have a sudden drop in hemoglobin
• Young children can have severe pain and swelling of both hands and feet levels (The hemoglobin may fall by half of its baseline value within a few hours)…be cautious of VOC crisis. It could also occur as a result of fat embolism originating from the
(dactylitis/hand foot syndrome). hypovolemic shock. distal bone in VOC.
• Fever can accompany vaso-occlusive crisis in some • If not treated promptly, this can be a life-threatening situation.
patients. • The hypoxia leads to adhesion of sickled RBC’s to pulmonary microvasculature,
• There is no objective measure or lab test to determine • Clinically, it presents as follows: setting up local hypoxia in the lungs and causing sickling of more RBCs; this sets
the quality and severity of pain, and therefore, patient - sudden onset of abdominal distension,
report is the only available guide. up a vicious cycle.
- left sided abdominal pain,
- pallor, tachycardia, tachypnea,
- splenomegaly,
- increased thirst, decreased activity,
decreased oral intake
- hypovolemic shock in severe cases.
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• The presenting signs & symptoms include fever, cough, tachypnea, chest pain, 4. Aplastic and Hemolytic Crisis
hypoxia, wheeze, respiratory distress, and even failure.

• Any pulmonary infiltrate on chest radiography accompanied by abnormal lung • Aplastic Crisis presents with sudden pallor and weakness confirmed by

findings should raise the suspicion of ACS. rapidly dropping hemoglobin levels (more than 3.0g/dL) that are accompanied

• Affected patients by reticulocytopenia

can rapidly • The usual trigger for aplastic crisis are viral infections (Especially Parvovirus

progress to b19) – these cause suppression of RBC production.

worsening • The infection is self-limited, typically lasting 7 to 10 days.

respiratory
failure and death • Hemolytic Crisis is characterized by an accelerated rate of red blood cell
if not aggressively destruction leading to anemia, jaundice, and reticulocytosis. It is common in
treated and patients with coexistent G6PD deficiency
monitored.

Precipitating Factors of Sickle Cell Crisis Pre Hospital Care and Transport Recognition of the Crisis
• Prehospital management of sickle cell crisis will focus on support of airway,
• Local tissue hypoxia
breathing, circulation, pain control and rapid transport to an appropriate facility.
• Dehydration secondary to a viral illness
• Administer supplemental oxygen as necessary, 2L by nasal cannula is adequate in
• Nausea and vomiting
most cases.
• Any event that can lead to acidosis, such as infection or extreme • Dehydration can worsen a vaso-occlusive event…establish IV access…administer
dehydration. fluid at a keep-open rate or as fluid bolus of 10 mL/Kg. (Be cautious of overload)

• Fatigue

• Exposure to cold

• Psychosocial stress • Cardiac arrest in sickle cell crisis is relatively uncommon and usually only
develops following respiratory arrest related to respiratory failure.
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Pain Assessment First 5 minutes Fluids in Sickle Cell Crisis

• Reserve fluid boluses for patients who are hypovolemic.

• Overhydration — especially with isotonic crystalloid — does not help resolve a pain
crisis and may have detrimental effects such as:
- Atelectasis, which may precipitate acute chest syndrome.
- Hyperchloremic metabolic acidosis, which may promote sickling.
- The concurrent use of opiates for pain control can increase vascular
leak and predisposes sickle cell disease patients to pulmonary edema.

• Boluses of IV fluid should not be given unless patients are overtly hypovolemic (sepsis,
diarrheal illness, vomiting). In these situations, resuscitate only to euvolemia.

• For maintenance fluids use a hypotonic solution such as ½ NS or D5-½ NS.

Helman, Anton. 2021. “Emergency Management of Sickle Cell Disease: EM Cases.” Emergency Medicine Cases. October 5. https://emergencymedicinecases.com/emergency-management-of-sickle-cell-disease/.

Fluids in Sickle Cell Crisis Analgesia in Sickle Cell Crisis Evaluation/Diagnosis

• There is a wide variation in the practice of IV Fluid administration in the treatment of
Consider multimodal analgesia as an adjunct to opioids:
patients in crisis. • CBC with differential
• Acetaminophen • Reticulocyte count
• Normal saline may be associated with worse pain control and volume overload.
• Complete metabolic panel including liver function tests
• Hypotonic fluids may be better choices of IV Fluid for crisis because they cause free
• NSAIDs (note that while NSAIDs have been shown to be effective in managing sickle cell • Type and screen blood for possible transfusion
water to enter red blood cells, which reduces hemoglobin concentration and red blood pain crises, they should be avoided for long term treatment of pain in Sickle Cell patients • Inflammatory markers include CRP, procalcitonin, and pancultures
because of the potential renal side effects, as these patients are at increased risk for
cell sickling may be considered for fever and identification of the source of
chronic renal failure – a short course of NSAIDs (<72h) may be appropriate
• However, there are no randomized controlled trials on their efficacy and safety in this infection.
patient population. Therefore, IV fluids use should be tailored to individual • Ketamine – case reports support the effectiveness of ketamine as an opiod-sparing drug • Chest X-rays to facilitate early identification of ACS.
• ECG
characteristics of each SCD patient.
• Note that while corticosteroids have been shown to reduce pain scores • An abdominal ultrasound may be considered for concerns of
• Keep in mind that Volume overload, new oxygen requirement, ACS and acute kidney and length of stay, there are associated with high rates of pain cholelithiasis/spleenomegaly.
injury are major concerns with inappropriate IVF use in SCD patients. recurrence, and so they are not recommended by our experts.
• ABG can be obtained for hypoxemia and respiratory failure
Ojo, Ademola S., Somtochukwu Ojukwu, Wassihun Asmare, Oluwamayowa Odipe, and Daniel Larbi. 2024. “Intravenous Fluid Administration • CT head and MRI brain would be indicated if there is suspicion of
Helman, Anton. 2021. “Emergency Management of Sickle Cell Disease: EM Cases.” Emergency Medicine Cases. October 5.
and the Risk of Adverse Outcomes in Sickle Cell Disease Patients Hospitalized for Vaso-Occlusive Crisis.” Journal of Hematology. Accessed
January 3. https://www.thejh.org/index.php/jh/article/view/1058/669.
https://emergencymedicinecases.com/emergency-management-of-sickle-cell-disease/. stroke.
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Continuing emergency management Complications

• Continuous Monitoring and Reassessment of pain is crucial

• The key is rapid hydration and pain control.

References
1. Sickle Cell Anemia Treatment & Management: Approach Considerations, Hydroxyurea Therapy,

Transfusion. (2021). Retrieved 11 February 2021, from

https://emedicine.medscape.com/article/205926-treatment#d11

2. Emergency Management of Sickle Cell Disease | EM Cases. (2015). Retrieved 11 February 2021,

from https://emergencymedicinecases.com/emergency-management-of-sickle-cell-disease/

3. Borhade, M., & Kondamudi, N. (2020). Sickle Cell Crisis. Statpearls Publishing. Retrieved from

https://www.ncbi.nlm.nih.gov/books/NBK526064/

4. (2021). Retrieved 11 February 2021, from

https://www.dshs.texas.gov/emstraumasystems/ND09PrehospitalManagementofSickleCellCrisis.pdf

5. Ndefo, U. A., Maxwell, A. E., Nguyen, H., & Chiobi, T. L. (2008). Pharmacological management of

sickle cell disease. P & T : a peer-reviewed journal for formulary management, 33(4), 238–243.