Nutritional Outcomes After Vitamin A Supplementation in Subjects With SCD
Nutritional Outcomes After Vitamin A Supplementation in Subjects With SCD
This study establishes the safety and efficacy of vit A supplementation doses (3000 and 6000
 IU/d) over 8 weeks in children with SCD-SS, ages 9 and older and test the impact of vit A
 supplementation on key functional and clinical outcomes. Additionally, vitamin A status is
 assessed in healthy children ages 9 and older to compare to subjects with SCD-SS.
Suboptimal vitamin A (vit A) status is prevalent in children with type SS sickle cell disease
 (SCD-SS) and associated with hospitalizations and poor growth and hematological status.
 Preliminary data in children with SCD-SS show that vit A supplementation at the dose
 recommended for healthy children failed to improve vit A status, resulting in no change in
 hospitalizations, growth or dark adaptation. This indicates an increased vit A requirement
 most likely due to chronic inflammation, low vit A intake and possible stool or urine loss.
 The dose of vit A needed to optimize vit A status in subjects with SCD-SS is unknown.
Inclusion Criteria:

 -  Sickle cell disease, SS genotype (subjects with sickle cell disease only)

 -  Usual state of good health (no hospitalizations, emergency room visits, or unscheduled
 acute illness clinic visits for two weeks prior to screening)

 -  Commitment to a 119-day study (subjects with sickle cell disease only), or a 4-day
 study (healthy volunteers only)

 Exclusion Criteria:

 -  Hydroxyurea initiated within the previous 6 weeks (subjects with sickle cell disease
 only)

 -  History of stroke (subjects with sickle cell disease only)

 -  Other chronic conditions that may affect growth, dietary intake or nutritional status

 -  Retinoic acid (topical or oral), weight loss medication and/or lipid lowering
 medications

 -  Subjects with a BMI greater than 98th percentile for age and sex

 -  Pregnant or lactating females (subjects who become pregnant during the course of the
 study will not continue participation)

 -  Liver function tests >4 x upper limit of reference range

 -  Participation in another study with impact on vitamin A status (subjects with sickle
 cell disease only)

 -  Use of multi-vitamin or commercial nutritional supplements containing vitamin A (those
 who are willing to discontinue these supplements, with the approval of the medical
 care team, will be eligible for the study after a 1 month washout period. Subjects
 taking nutritional products without vitamin A will be eligible)

 -  Inability to swallow pills (subjects with sickle cell disease only)