Newborn Screening Result
Health Maintenance for children with SCD
Infection
Clinical Sequelae
Mystery!
100
FA
Normal
100
Retinopathy by an ophthalmologist
Annually beginning ta 10 years old
100
These 3 organisms are the most common cause of infection in pts with functional asplenia
- Strep Pneumo
- Haemophilius influenzae
- Neiseria Meninigitidis
100
What is the treatment of ACS?
The treatment of ACS includes supplemental oxygen, empiric antibiotics (including a macrolide for coverage of atypical pathogens), bronchodilators, and careful management of analgesia and intravascular volume
100
Before giving a transfusion, what 2 medications should you pre-medicate with?
Benadryl and Tylenol 30 minutes prior to pRBC transfusion
200
FAS
sickle cell trait
200
Trans cranial Doppler ultrasound
First screen at 2 years of age
If normal: repeat annually until age 16
If conditional repeat every 3-6 months
If abnormal x2 initiate transfusions
200
Your patient with Sickle Cell SS disease is presenting today with fever to 101 F. After collecting Blood and Urine cultures, which antibiotic should you start?
Administration of a third-generation cephalosporin (ceftriaxone, cefotaxime) plus clindamycin or vancomycin for severe infections is the current practice
200
Your 10 year old sickle cell SS disease pt has an elevated tBili. What are you concerned about?
Cholelithiasis
200
The sickle mutation leads to the replacement of hydrophilic _____ by a hydrophobic ____.
Glutamic acid, Valine
300
FS
SS
300
PFTs
Starting at 6 years of age, then every 5 years
300
What are the 2 most common bacteria associated with osteomyelitis in sickle cell patients?
Salmonella sp and Staph Aureus
300
What percent of boys with SCD will have a priapism episode by the time they are 15?
Priapism can occur as young as 3 years of age, and ∼30% of boys will have an episode by age 15.
300
Dactylitis
400
FSA
sickle beta thalassemia
400
Nephropathy Screening
Annually starting at 10 years of age
400
Your SCD pt presents with 2 days of fever, cough, and pallor. Labs are concerning for a decrease from baseline hemoglobin and a corresponding reticulocytopenia. BCx is no growth x1d. What are you most concerned about?
Parvovirus B19 / Aplastic crisis
400
What is caused when there is nitric acid depletion 2/2 to the release of free hemoglobin into the plasma from chronic intravascular hemolysis?
Pulmonary Artery Hypertension
400
ACS with diffuse bilateral infiltrates
500
FAS, Barts
sickle cell trait with alpha thalassemia trait
500
PPSV23 Administration
First dose at 2 years of age
500
Which (if any) imaging modality can you use to distinguish VOC from osteomyelitis?
No definitive imaging modalities (bone scan, magnetic resonance imaging) can differentiate with certainty between osteomyelitis and VOC.
500
A mother tells you that her child who has SCD recently went for his annual TCD. Velocity was read as 200 cm/s. How do you counsel this mother?
200 cm/s indicative of a 10% stroke risk over the ensuing year.
500
What is the typical baseline hemoglobin of a patient with Sickle Cell SS disease?
6-9