SICKLE CELL DISEASE

By: Vivian Kadus, PharmD

PGY-1 Pharmacy Resident
Lutheran Hospital

1 2
Summarize the Discuss the
pathophysiology of diagnosis of sickle
sickle cell disease cell disease
OBJECTIVES
3 4
Review treatment of Evaluate a patient
sickle cell disease case and determine
how to treat the
patient
EXPECTATIONS?
 WHAT IS SICKLE CELL DISEASE?
• Inherited red blood cell (RBC) disorder that is
resulted from a genetic mutation in the gene
that encodes hemoglobin (Hgb)
• Types of Hgb: A, F, C, E and S
• One gene is inherited by the mother and one is
inherited by the father
• Hgb mutation cause RBC to become sticky and
hard forming a C shaped RBC
• Can cause RBC to get stuck and block blood
flow leading to infection, pain, acute chest
syndrome, and stroke

https://medsurgeindia.com/cost/sickle-cell-anemia-treatment-cost-in-india /
 EPIDEMIOLOGY

SCD affects ~ 100,000 Americans

1 out of every 365 African Americans have been born

with SCD
About 1 in 13 African Americans is born with sickle
cell trait
 DIAGNOSIS OF SICKLE CELL
DISEASE

Newborn Hemoglobin
Screening electrophoresis
 Pain crisis/ Acute chest
Anemia
sickle crisis syndrome

Splenic
Stroke Jaundice
sequestration
SYMPTOMS/
COMPLICATION
Renal
Priapism Infections
impairment

Pulmonary
hypertension
TREATMENT OPTIONS
 Blood transfusions

Bone marrow transplantation

NON-DRUG
TREATMENTS Iron chelation therapy

Fluids

Eye exams
 IMMUNIZATIONS AND
ANTIBIOTICS
Vaccines
• Routine childhood series
• Haemophilus influenzae type B (Hib)
• Pneumococcal conjugate (PCV13, Prevnar)
• Additional
• Meningococcal conjugate series plus boosters
• Pneumococcal
• Meningococcal serogroup B (Bexsero, Trumenba)

Prophylactic penicillin
 HYDROXYUREA

Stimulates the production of hemoglobin F

Reduces the frequency of acute pain crisis's, episode of acute chest syndrome, and the need for
blood transfusions

Indicated for adults > 3 moderate to severe pain crises in one year

Contraception required

Folic acid supplementation recommended

 Analgesics
SUPPORTIVE L-glutamine
TREATMENT
OPTIONS
Voxelotor
Crizanlizumab
 Hydration

Thromboembolism prophylaxis
MANAGEMENT
DURING
HOSPITALIZATION Incitive spirometry

Splenic and hepatic sequestration

 PATIENT CASE
• MD is a 20 y/o F with a PMH sickle cell disease requiring blood
transfusions presents to the ED with sickle cell crisis. She is 33 weeks
pregnant, which has been complicated by the sickle cell disease. She was
recently hospitalized from 12/29-12/21 for similar symptoms. Prior to
admission she had a fever of 101 and developed worsening pain in her
right forearm, which is typical for her sickle cell pain. She endorses right
sided chest pain and severe SOB. When checked her O2 meter at home,
and in the ED her stat was 79. She was recently 12/28-12/31 for similar
symptoms.
 WBC: 15.2

Hgb: 7.9

Total bilirubin: 8.3

PERTINENT ED
Lactic Acid: 2.1
LABS ADD
CRCL Viral PCR: negative

CXR: cardiomegaly and mild vascular congestion

CTA: negative for PE

WHY IS IT PERTINENT TO CHECK T BILI
LEVELS IN A SICKLE CELL PATIENT?
 albuterol nebulizer solution

docusate- senna PRN

folic acid 1 mg tablet daily

MD HOME hydromorphone 2 mg table q6h

MEDICATIONS
prenatal multivitamin

ondansetron 4 mg ODT PRN

oxycodone-acetaminophen 7.5-325 mg tablet q6h PRN

 WHAT MEDICATIONS COULD BE ADDED TO
MD’S REGIMEN TO REDUCE HER SICKLE CELL
PAIN CRISIS?
• Hydroxyurea
• Voxelotor
 WHAT WOULD YOU NEED TO
START MD ON ADMISSION
• Fluids
• VTE prophylaxis
• Oxygen
 WHAT HAPPENED TO MD
Hgb Tbili (0.3- Fluids WBC
1)
Jan 3rd 7.9 8.3 100 mL/h 15.3
• She was hospitalized from 1/3/2023-
Jan 4th 7.3* 4.8 -- 13.9
1/10/2023
Jan 5th 7.9 4.1 75 mL/ h 15.9
• Received 2-3 L NC
Jan 6th 7.7 3.5 -- 7.6
• Started on Lovenox 40 mg q24h and was
Jan 7th 7.2* 2.8 50 mL/h 6.3
expected to continue at home
Jan 8th 8.7 2.4 -- 9.2
Jan 9th 7.8 3.4 -- 8.7
Jan 10th 8.3 -- -- --
Questions?
 REFERENCES
• Centers for Disease Control and Prevention. (2022, August 18). What is sickle cell
disease? Centers for Disease Control and Prevention. Retrieved January 17, 2023, from
https://www.cdc.gov/ncbddd/sicklecell/facts.html 
• Mayo Foundation for Medical Education and Research. (2022, March 9). Sickle cell anemia.
Mayo Clinic. Retrieved January 17, 2023, from https://www.mayoclinic.org/diseases-
conditions/sickle-cell-anemia/symptoms-causes/syc-20355876 
• Sickle cell disease. Sickle Cell Disease | Johns Hopkins Medicine. (2019, November 19).
Retrieved January 17, 2023, from https://www.hopkinsmedicine.org/health/conditions-and-
diseases/sickle-cell-disease