1. Assess the patient's symptoms to determine whether the patient should be evaluated by a physician and/or receive immediate care. Determine the type of symptoms, onset of symptoms, frequency and exacerbating factors. Does the patient have evidence of SCD-related complications?
2. Review any available diagnostic data to determine the severity and status of the patient's SCD. When was the patient last hospitalized for SCD complications?
3. Obtain a thorough history of prescription, nonprescription and natural drug product use. For pain control, determine which treatments have been helpful to the patient in the past. Is the patient currently taking any medications on a chronic basis?
4. Educate the patient on lifestyle modifications that may lessen complications. These include maintaining adequate hydration status, avoiding extreme temperature changes, dressing appropriately for hot or cold weather, and avoiding physical exertion, smoking, and excessive alcohol intake.
5. Is the patient up to date on immunizations? Have they received their annual influenza vaccine? If not, why?
6. Is the patient taking appropriate doses of their pain medication to achieve effect? If not, why?
7. Develop a plan to assess the effectiveness of pain medications.
8. Determine if the patient is a candidate for hydroxyurea therapy.
9. Assess improvement in quality of life measures, such as physical, psychological, and social functioning and well-being.
11. Stress the importance of adherence with the therapeutic regimen, including lifestyle modifications. Recommend a therapeutic regimen that is easy for the patient/ parent to accomplish.
12. Provide patient education on disease state, lifestyle modifications, and drug therapy:
• Possible complications of SCD, both long- and short-term.
• When to take their medications.
• What potential adverse effects may occur?
• Which drugs may interact with their medication therapy
• Warning signs to report to the physician (increased or new pain, sudden headache, bleeding or bruising, fever, loss of energy, loss of appetite)
Starting with birth, SCD patients should have regularly scheduled health assessments and interventions when necessary. Obtain a urine analysis, complete blood count, liver function tests, ferritin or serum iron level and total iron binding capacity, blood urea nitrogen (BUN), and creatinine on at least a yearly basis and more often for children younger than 5 years of age to monitor for complications. All SCD patients should have regular screening of their hearing and vision.
All patients and parents of children with SCD should have a plan for what to do in the event of symptoms of infection or pain. Obtain a medication history when patients are admitted to the hospital. Assess compliance with prophylactic penicillin and childhood immunization schedules in all pediatric SCD patients.
Abbreviations Introduced in This Chapter
ACS Ac u te c hest synd rome
HbA Normal adult hemoglobin
HbAS One normal and one sickle hemoglobin gene
HbC Hemoglobin C
HbF Ft tol hemoglobin
HbS Sickle hemoglobin
H bS/^1-thalassemia One sickle hemoglobin and one ßP
thalassemia gene HbSß'-thalassemia One sickle hemoglobin and one ß'
thalassemia gene Hb SC One sickle hemoglobin and one hemoglobin C gene HbSS Homozygous sickle hemoglobin
HS CT Hematopoietic stem cell transplantation ISC Irreversibly sick led cell
M CHC Mea n t.o rpu scular hemoglob i n concentration MCV Mean corpuscular volume
NSAII) Nonsteroidal anti-inflammatory drug
PC A Patient-conti oiled analgesia
PCV ? 7-Valent pneumococcal conjugate
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