Sickle Cell Disease Flashcards
Key Vaccines in SCD
Routine Childhood series 2
- H Flu Type B (Hib)
- Pneumo conjugated (PCV13 Prevnar)
Additional Vaccines Due to Functional Asplenia
4 speciifc timing
- Meningococcal series + routine boosters
- Meno Serogroup B (Bexsero, Trumenda) >= 10
- Pneumo poly (Pneumovax)>= 2 booster 5 years later and at age 65
- Pneumo conjugated prevnar x 1 in any pt >= 6 years of age if not given in childhood
SCD most commonly effects what population?
African Americans
How long does it take for symptoms of SCD to develop and why?
2-3 months after birth and fetal Hgb ehich blocks sickling of RBCs
What are the two types of Acute Sickle cell crisis and what is the cause?
Caused by vascular occlusion
Most common types VOC: Vasooclussive crisis
or Acute pain crisis
If pain in chest it is acute chest syndrome
Common chronic complication of SCD?
Pulmonary HTN
Others but this is underlines
SCD causes what issue?
Fibrosis of the spleen causing functional asplenia
Non drug treatment for SCD? 2
Blood transfusion no higher than 10 g/dL
Bone marrow transplant
Drug tx for SCD? 6
- Immunizations
- Abx
- Analgesics for pain
- Hydroxyurea
- L-Glutamine
- Chelation therapy to reduce iron overload from tranfusion
Sepsis and mengingitis can occur in SCD due to what 3 main pathogens?
- S. Pneumo
- H. Influe
- N. Mengingitiis
What should be used in children when born?
How long should it be continued?
What is the dosing?
- PCNs Twice daily
- Until 5 yo
Pts with severe pain or VOC should be given what?
IV opioids or PCA
What is hydroxyurea and what does it do?
Disease modifying agent that stimulates the production of HgbF
When is Hydroxyurea indicated? In SCD
Adults who have >=3 acute pain crisis in 1 year
How is hydroxyurea taken?
Oral
Droxia, Hydrea, Siklos
Hydroxyurea
Boxed warning for hydroxyurea?
Myelosuppression
2 warnings for hydroxyurea?
- Fetal embryo toxicity
- Avoid live vaccines
How do you know if hydroxyurea is at toxic levels? 2 labs
ANC<2,000/mm3
Platelet <80,000/mm3
What should be monitored for hydroxyurea?
CBC q2-4 wks until stable then 2-3 months
What is required for hydroxyurea?
Contraception
What supplementation is used for hydroxyurea?
FOlic acid
What type of agent is hydroxyurea?
Hazardous
Wear disposable gloves to limit exposure dont open the capsule
How long is contraception needed for hydroxyurea?
during and after stopping 1 year males 6 months females
What drug interactions for hydroxyurea?
Drugs that cause myelosuppression
Limuss
Wear sun protection
Endari
L-Glutamine
Age indication for Endari
>=5 with SCD
What does L-glutamine do for SCD pts?
Reduces the number of acute complications
What type of formulation is L-glutamine?
Oral powder mixed with room temp beverage
Iron Chelation Tx
What isnt used and why?
Defuroxime was used but not any more cause of toxicities and its IV
What are the two oral chelators
Deferasirox
Deferiprone
Who is hydroxyurea also indicated for?
Pts with acute chest syndrome, chronic symptomatic anemia or disability
consider in all children > 9 months