JP, a 25-year-old female, presented to the ED with symptoms of vaso-occlusive pain crisis. She endorsed pain in her abdomen, as well as her bones and joints. Workup is significant for low hemoglobin level and elevated reticulocytes.
PMH: Sickle Cell Disease
Weight 66 kg, Height 64 in
Vitals: BP 152/86 mmHg, HR 104 bpm
Labs on presentation:
Hgb 6.1 g/dL (Normal 12.0-15.5 g/dL)
Reticulocytes 20% (Normal 0.5-2.5%)
Current medications:
Hydrea 1000 mg PO QD
Jadenu 900 mg PO QD
Blood transfusion was initiated due to her low hemoglobin. Upon further examination, it was noted that JP was asplenic, which she reported occurred as a result of several episodes of splenic sequestration when she was younger. What vaccine(s) should JP be ensured that she is up-to-date long-term specifically due to her acquired asplenia? Select all that apply.
A. Haemophilus influenzae type b vaccine (Hib)
B. Pneumococcal vaccines
C. TB vaccine
D. Meningococcal vaccines
E. Hepatitis A Vaccine
Answer with Rationale
Sickle cell disease (SCD) is a hereditary condition characterized by the presence of sickle cell hemoglobin (HbS) in red blood cells (RBCs). The biochemical defect that leads to the development of HbS involves the substitution of valine for glutamic acid as the 6th amino acid in the β-polypeptide chain. SCD is a chronic illness with high financial and emotional burden on both the patients themselves, as well as their caregivers. Frequent hospitalizations can lead to repeated interruptions in normal daily routine, including school and employment. Acute complications, which include infections by encapsulated organisms, strokes, acute chest syndrome, vaso-occlusive pain, splenic sequestration, etc., are largely unpredictable, rapidly progressive, and life-threatening. SCD affects millions of people worldwide and is most prevalent in people with African ancestry. In the US, about 100,000 Americans have SCD with a prevalence of 1 in 2,500 newborns of all ethnicities, 1 in 365 Black births, and 1 in 36,000 Hispanic births. The cardinal features of SCD are hemolytic anemia and vaso-occlusion. Common signs and symptoms include painful swelling of hands and feet, weakness, anorexia, fatigue, chronic anemia and pallor, hematuria, enlargement of spleen, etc. A bone marrow transplant, also known as a stem cell transplant, is the only known cure for SCD.
Answer A is correct. In patients with SCD, impaired splenic function increases susceptibility to infection by encapsulated organisms, such as Haemophilus influenzae. The ACIP currently recommends that Hib vaccine be given in 3 or 4 doses, depending on brand. Infants will usually get their first dose of Hib vaccine at 2 months of age and will usually complete the series at 12–15 months of age. Children between 12 months and 5 years of age who have not previously been completely vaccinated against Hib may need 1 or more doses of Hib vaccine. Children over 5 years old and adults usually do not receive Hib vaccine, but it might be recommended for older children or adults whose spleen is damaged or has been removed, including people with SCD, before surgery to remove the spleen, or following a bone marrow transplant.
Answer B is correct. In patients with SCD, impaired splenic function increases susceptibility to infection by encapsulated organisms, such as Streptococcus pneumoniae. The ACIP currently recommends pneumococcal vaccinations for all children younger than 5 years old, children 5-18 years old with certain risk conditions (including SCD), all adults 65 years or older, and adults 19-64 years old with certain risk conditions (including SCD). There are 2 types of pneumococcal vaccines used in the US – pneumococcal conjugate vaccines (PCVs) and pneumococcal polysaccharide vaccine (PPSV).
Answer C is incorrect. People with SCD are not considered “high risk” specifically for tuberculosis due to their functional or acquired asplenia so it is not currently recommended by the CDC. In addition, the BCG vaccine (TB vaccine) is not routinely used in the US.
Answer D is correct. In patients with SCD, impaired splenic function increases susceptibility to infection by encapsulated organisms, such as Neisseria meningitidis. The ACIP currently recommends meningococcal vaccinations for all preteens and teens, children 2 months-10 years old at increased risk (including SCD), and adults 19 years and older at increased risk (including SCD). There are 3 types of meningococcal vaccines in the US – MenACWY vaccines, MenB vaccines, and MenABCWY vaccine.
Answer E is incorrect. People with SCD are not considered “high risk” for Hepatitis A due to their functional or acquired asplenia. Some higher risk patients for Hepatitis A include international travelers, people who inject drugs, or certain occupations with greater exposure as this is transmitted through the fecal-oral route.
Generic/Brand: Hydroxyurea (Hydrea), Deferasirox (Jadenu)
NAPLEX Core Competencies Covered:
- 1.1 – From instruments, screening tools, laboratory, genomic or genetic information, or diagnostic findings
- 1.2 – From patients: treatment adherence, or medication-taking behavior; chief complaint, medication history, medical history, family history, social history, lifestyle habits, socioeconomic background
- 1.5 – Signs or symptoms of medical conditions, healthy physiology, etiology of diseases, or pathophysiology
- 1.6 – Risk factors or maintenance of health and wellness
- 2.2 – Commercial availability; prescription or non-prescription status; brand, generic, or biosimilar names; physical descriptions; or how supplied
- 4.1 – Patient parameters or laboratory measures
· 6.3 – Disease prevention or screening programs; or stewardship
- 6.4 – Vulnerable populations, special populations, or risk prevention programs
Create a Free MyAccess Profile
AccessMedicine Network is the place to keep up on new releases for the Access products, get short form didactic content, read up on practice impacting highlights, and watch video featuring authors of your favorite books in medicine. Create a MyAccess profile and follow our contributors to stay informed via email updates.