Pediatric Primary Care (123 page)

I.  Follow up.

1.  Follow recommendations in
Table 32-2
for rescreening. Treatment time is lengthy: There will be rebound levels as stored lead releases from bones and teeth.

2.  After chelation therapy: Obtain another blood lead level in 10-14 days.

3.  Blood lead level determines subsequent treatment.

J.  Complications.

1.  Lead encephalopathy and toxicity:

a.  Headache, persistent vomiting.

b.  Seizures, coma, death.

c.  Mental retardation, learning disabilities.

d.  Impaired growth.

e.  Hearing loss.

2.  Renal disorders: hypertension, kidney diseases (later in life), elevated blood urea nitrogen (BUN).

3.  Reproductive system:

a.  Diminished fertility, abnormal sperm and lower counts, sterility–male and female.

b.  Increased chance of miscarriage.

K.  Education.

1.  Prevention.

2.  Advice for families:

a.  If suspect exposure, test child.

b.  Use caution when purchasing older home.

c.  Maintenance to keep old lead-based paint intact.

d.  Watch for lead dust when doing home improvement projects: Protect furniture from lead dust, wet mop work area after projects using detergent.

e.  Wash work clothes separately from family's clothing.

f.  Encourage children to play in sand, grass rather than dirt.

g.  Wash hands, pacifiers before naps, bedtime.

h.  Avoid folk remedies or cosmetics containing lead.

i.  Test water supply for lead.

j.  Wash fruits, vegetables before eating.

k.  Eat healthy diet rich in iron: helps body to absorb less lead.

l.  Use only cold water from tap for drinking, cooking, making baby formula. Hot water more likely to contain higher levels of lead.

IV.  SICKLE CELL DISEASE

A.  Group of inherited heme disorders characterized by sickle hemoglobin (HbS); several variants within sickle cell disease. This section aimed at variant that results when one is homozygous for HbS, sometimes referred to as sickle cell anemia (HbSS).

B.  Etiology.

1.  Due to single defective hemoglobin module inherited as gene from both parents (autosomal-recessive disorder).

2.  Abnormal HbS is produced instead of HbA, normal hemoglobin.

3.  The abnormal hemoglobin S, when deoxygenated, deforms red cells into sickle shapes that occlude small vessels, slowing blood flow, creating vaso-occlusive crises in blood vessels and in organs such as spleen.

C.  Occurrence.

1.  Predominantly in African Americans with about 1 in 400 African Americans affected.

2.  Mediterranean or Arabic descendants also found to have sickle cell anemia, but in fewer numbers.

D.  Clinical manifestations.

1.  Chronic hemolytic anemia (aplastic crisis, hemolytic crisis, sequestration crisis).

2.  Vaso-occlusion resulting in ischemia to tissues.

a.  Painful crisis: from infarcts of muscle, bone, bone marrow, lung, intestines.

b.  Cerebrovascular accident.

c.  Acute chest syndrome.

d.  Chronic lung disease such as pulmonary fibrosis.

e.  Priapism.

f.  Ocular retinopathy.

g.  Gallbladder disease.

h.  Renal.

i.  Cardiomyopathy.

j.  Leg ulcerations.

3.  Susceptibility to infection.

4.  Growth failure, delayed puberty.

5.  Psychologic problems (narcotic addiction, chronic illness, unusual dependence).

E.  Physical findings.

1.  Depends on which clinical manifestation is presenting.

2.  Chronic hemolytic anemia: pallor/jaundiced skin, tachycardia, fatigue.

3.  Susceptibility to infection: fever, other symptoms related to causative organism or system infected: e.g., meningitis; urinary tract infection (UTI); cytomegalovirus (CMV); hepatitis A, B, C, D, E; pneumonia.

4.  Vaso-occlusive crisis: again depends on location of occlusion.

5.  Splenomegaly.

6.  Maxillary hyperplasia and dental malocclusion result from compensatory bone marrow expansion.

F.  Diagnostic tests.

1.  Newborn screening for hemoglobinopathies. Electrophoresis on cellulose acetate indicates type of hemoglobin: fetal (F), normal adult (A), sickle (S), hemoglobin C (C).

2.  Repeat testing with abnormal hemoglobin (FS) pattern on newborn screen.

3.  CBC with hemoglobin MCV.

G.  Differential diagnosis.

1.  Sickle cell trait (benign), beta-thalassemia anemia.

H.  Treatment.

1.  Preventive care.

a.  Education of the family.

•  Adequate fluid intake.

•  Immediate medical help for fevers.

•  Importance of prophylactic treatment.

b.  Immunizations per the schedule plus 23-valent pneumococcal vaccine at 2 and 5 years.

2.  Pharmaceutical therapies.

a.  Penicillin prophylaxis:

•  2 months to 3 years of age: Penicillin VK 125 mg PO bid.

•  3 years to 5+ years of age: Penicillin VK 250 mg PO bid.

b.  Alternative to penicillin: erythromycin (EES) 20 mg/kg/day divided bid.

c.  Folic acid: 1 mg/day.

d.  Multivitamin once daily.

e.  Hydroxyurea drug therapy.

•  Anti-sickling effect of hydroxyurea can decrease frequency of vaso-occlusive crises.