Please select the eligibility criteria that correspond to the clinical trial below.
Title: Pediatric Hydroxyurea Phase III Clinical Trial (BABY HUG)
Brief Summary: The purpose of this study is to determine if hydroxyurea therapy is effective in the prevention of chronic end organ damage in pediatric patients with sickle cell anemia.


Options:
A: Inclusion Criteria:

* Majority fetal and sickle (FS or SF) hemoglobin pattern confirmed centrally by electrophoresis (screening may begin at 7 months of age)

Exclusion Criteria:

* Chronic transfusion therapy
* Cancer
* Less than 5th percentile (10th percentile for the pilot study) height, weight, or head circumference for age
* Severe developmental delay (e.g., cerebral palsy or other mental retardation, Grade III/IV intraventricular hemorrhage)
* Stroke with neurological deficit
* Surgical splenectomy
* Participating in other clinical intervention trials
* Probable or known diagnosis of Hemoglobin S-Hereditary Persistence of Fetal Hemoglobin
* Known hemoglobin S-beta plus thalassemia (hemoglobin A present)
* Any condition or chronic illness, which in the opinion of the principal investigator, makes participation unadvised or unsafe
* Inability or unwillingness to complete baseline (pre-enrollment) studies, including blood or urine specimen collection, liver-spleen scan, abdominal sonogram, neurological examination, neuropsychological testing, or transcranial Doppler ultrasound (interpretable study not required, but confirmed velocity greater than 200 cm/sec results in ineligibility)
* Previous or current treatment with hydroxyurea (HU) or another anti-sickling drug
* The following exclusion criteria are transient; patients can be re-evaluated for eligibility:

  1. Hemoglobin less than 6.0 gm/dL
  2. Reticulocyte count less than 80,000/cu mm if hemoglobin is less than 9 gm/dL
  3. Neutrophil count less than 2,000/cu mm
  4. Platelet count less than 130,000/cu mm
  5. Blood transfusion in the 2 months prior to study entry unless HbA is less than 10%
  6. ALT greater than twice the upper limit of normal
  7. Ferritin less than 10 ng/ml
  8. Serum creatinine greater than twice the upper limit of normal for age
  9. Bayley standardized mental score below 70
B: Inclusion Criteria:

* Sickle cell disease, including sickle cell anemia (SS), sickle-hemoglobin C disease (SC), and sickle beta thalassemia disease (Sb).
* Ongoing opioid analgesic therapy for chronic sickle cell disease-associated pain.
* Subjects must be on a stable dose of analgesic medication (opioid or other) for at least 2 weeks before enrollment.
* All men and women in this study must agree to use adequate birth control during this study. Acceptable barrier birth control methods are a male condom, female condom, diaphragm, or intra-uterine (IUD).
* All women of reproductive potential (who have not reached menopause or undergone hysterectomy, oophorectomy, or tubal ligation) must have a negative urine b-HCG pregnancy test performed before initiating the protocol-specified medication.
* Prior history of use of cannabis. Subjects must have smoked cannabis on at least 6 occasions in their lifetime prior to enrollment.
* Subjects will self-report abstaining from smoking or ingesting cannabis for one week prior to their enrollment into the study.
* Able to understand and follow the instructions of the investigator, including completing the pain intensity rating scales.
* Karnofsky Performance Scale \>60.
* Able and willing to provide informed consent.
* Able and willing to spend two separate periods of 5 days and 4 nights in the Clinical Research Center at SFGH.

Exclusion Criteria:

* Severe coronary artery disease, uncontrolled hypertension, cardiac ventricular conduction abnormalities, orthostatic mean blood pressure drop greater than 24 mmHg, severe chronic obstructive pulmonary disease.
* Evidence of clinically significant hepatic or renal dysfunction based on judgment of physician.
* Positive serum THC level on Day 1 of study.
* Active substance abuse (e.g., alcohol or injection drugs) as determined by urine toxicity screening.
* Neurologic dysfunction or psychiatric disorder severe enough to interfere with assessment of pain or sensory systems.
* Current use of smoked tobacco products.
* Women who are pregnant or breast-feeding may not take part in this study.
* Unable to read or speak English.
C: Inclusion Criteria:

1. over 5 year old
2. less t5han 50
3. diagnosed with homozygous sickle cell anemia by Hb electrophoresis

Exclusion Criteria:

1. patients received blood transfusion within the last three months or admitted to the hospital within 2 weeks because of Sickle cell anemia -related events or crisis.
D: Inclusion Criteria:

* Diagnosis of Hemoglobin (Hb) SS or Hb Sβ0 thalassemia by electrophoresis
* Age ≥18 years
* Hematocrit (Hct) ≥ 20% and Hb \> 6.0 g/dL
* Capacity to understand and sign informed consent and can adhere to the daily regimen of BSH

Exclusion Criteria:

* RBC transfusion or change in hydroxyurea dose during the 3 months prior to study entry
* Ongoing pregnancy
* Diabetes
* Renal insufficiency (BUN \>21 mg/dL and/or Creatinine \>1.4 mg/dL)
* History of allergy to sulfonamides

Answer: A