Sickle Cell Disease Flashcards
Vaccinations
13-valent pneumococcal conjugate vaccine (Prevnar 13)
H. influenza vaccine
Vaccinations
>= 2 years
23-valent pneumococcal po|ysaccharide vaccine (Pneumovax) x 2
Administer at >= 2 years (at least 2 months apart from Prevnar 13) and at 5 years
Meningococcal c;onjugate vaccine x2, given between 2-6years and 5-9 years
Penicillin
Penicillin V Potassium 125 mg PO BID for age = 3- 5 years
Or
Benzathine penicillin 600,000 million units IM Q 4 weeks in nonadherent patients from 6 months to 6 years
Penicillin Allergy = Erythromycin 10 rng/kg PO BID
Pain Management
Mild to moderate pain can often be managed at home with rest, fluids, application of warm compresses applied to affected areas, and the use of NSAIDs or acetaminophen.
For severe pain, opioids are recommended. Hospitalization may be indicated.
Anemia Management
Folic acid is recommended in children with SCD with chronic hemolytic anemia at a dose of 0.1 mg/day up to age 1 year, 0.3 mg/day from 1-4 years, and 0.4-1 mg/day from 4 years through adolescence.
Stimulate HbF Production
Hydroxyurea (Droxia, Hydrea)
Initiate: 15 mg/kg PO daily (round to nearest capsule), max 35 mg/kg/day (divide higher doses)
BLACK BOX WARNING
Serious and life-threatening adverse events may occur.
CONTRAINDICATIONS ‘
Significant bone marrow suppression: WBC
Hydroxyurea Drug Interactions
Hydroxyurea can increase the effects of clozapine, didanosine, natalizumab, pimecrolimus, stavudine, tacrolimus (topical), tofacitinib, and live vaccines. Concomitant use should be avoided.
Concurrent treatment with antiretrovirals (including didanosine and stavudine), is higher risk for potentially fatal pancreatitis, hepatotoxicity, hepatic failure, and severe peripheral neuropathy.
Hydroxyurea Patient Counseling
Hydroxyurea can lower your body’s ability to fight infection and make you more prone to bleeding or becoming ill.
Call your doctor immediately if you experience fever, chills, sores in the mouth, easy bruising/bleeding, purple or red point spots under the skin, pale skin, rash, shortness of breath, rapid heart rate, painful or difficulty urinating, and/or confusion.
Wear disposable gloves when handling to reduce risk of exposure. Wash hands before and after handling. If you accidentally spill any of the contents, immediately wipe up with a damp cloth and throw cloth away in a sealed plastic bag.
Chelation to Reduce Iron Overload from Transfusions
deferoxamine. the antidote for iron toxicity, has been used but requires a pump for about 12 hours/day and has significant toxicities.
Deferasirox (Exjade) is taken once daily by mouth, mixed with liquid. It is easier to take with less toxicity. Deferasirox is indicated for the treatment of chronically elevated levels of iron in the blood caused by repeated blood transfusions (transfusional hemosiderosis) in patients 2 years of age and older. It is also used for iron overload from chronic transfusions in patients with beta-thallassemia, a blood disorder causing decreased hemoglobin production.
Deferoxamine (Desferal) Injection
Start at 20 mg/kg/day SC, max 2,000 mg/day
At 1 month add: ascorbic acid 50-200 mg/d
CONTRAINDICATIONS
Severe renal disease, heart failure
SIDE EFFECTS
Ototoxicity, visual impairment, arthralgia, headache, acute respiratory distress syndrome (dyspnea, cyanosis, and/ or interstitial infiltrates), agranulocytosis, growth failure,
hypersensitivity reactions (e.g., urticaria, angioedema),
injection-site reactions (erythema, pruritus), hypotension
MONITORING
Serum iron, ferritin, total iron-binding capacity, CBC with
differential, SCr, LFTs, chemistry panel, growth, and
body weight, monitor at baseline and every 3 months, audiometry and ophthalmologic exams
NOTES
Pregnancy Category C
Deferasirox (Exjade)
Start a 20 mg/kg PO daily, (round to nearest tablet), max 40 mg/kg/day.
Must be taken on an empty stomach at least 30 minutes before eating, mixed into orange juice, apple juice or water. Instruct patients using the Drop-Stir-Drink counseling method (refer to counseling section).
BLACK BOX WARNINGS (3) Gastrointestinal hemorrhage (including fatalities) may occur; monitor.
Hepatic injury and failure (including fatalities) may occur.
Acute renal failure (including fatalities and cases requiring
dialysis) may occur; monitor carefully.
CONTRAINDICATIONS
CrCl two-fold the age-appropriate upper limit of normal, platelet counts
Deferasirox Drug Interactions
Deferasirox is a substrate of UGT 1A1 as well as an inhibitor of CYP 1A2, CYP 2C8 and an inducer of CYP 3A4 and can be affected by many drugs; check prior to dispensing.
Avoid concomitant use of aluminum hydroxide, bile acid sequestrants (e.g., cholestyramine), and potent UGT inducers (e.g., rifampin, phenobarbital, phenytoin) as they may reduce the efficacy of chelation therapy.
Deferasirox Patient Counseling
Administer tablets by making an oral suspension in water, juice or in apple sauce. Do not chew or swallow tablet whole. Take on an empty stomach at least 30 minutes before eating.
Use this method:
- DROP the Exjade tablet(s) into a glass of orange juice, apple juice, or water. You can also use the Exjade mixer each day when taking your Exjade. Make sure that you use the amount of liquid directed by your doctor.
- STIR the liquid and Exjade tablet(s) until you have an even mixture. The consistency of the mixture may be thick.
- DRINK all of the Exjade mixture. Add more juice or water to mix anything that’s left over. And then drink that.
- Avoid aluminum containing or milk products as they can bind the chelation therapy and reduce its effectiveness. Seek immediate medical attention if you experience any of these signs of an allergic reaction, including difficulty breathing, hives, and/or swelling of your face, lips, tongue, or throat.
