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INTRODUCTION: Sickle cell disease is a hereditary
hemoglobinopathy characterized by many medical complications, including
unpredictable episodes of vaso-occlusive pain (VOP). A common trigger
for VOP in many females is the menstrual period. This pilot study
explores the association between menstrual periods and VOP, in preparation
for testing the hypothesis that Depo-Provera reduces the frequency
of vaso-occlusive pain crises. METHODS: A questionnaire on the association
between menses and vaso-occlusive pain crises was presented to 75
women. A retrospective chart review examined the association between
hormonal treatments and the frequency of medical encounters for
VOP. RESULTS: 75% of the women surveyed reported VOP association
with the menstrual period. Four of the five charts reviewed showed
an increase in the frequency of pain crises following hormonal treatment.
Only one teen showed a decrease in the frequency of pain following
treatment. DISCUSSION: These preliminary data present a complex
picture. The surveys show a high prevalence of menstrual association
with VOP, while retrospective chart review finds effectiveness of
treatments far lower than that reported by De Abood (1997) and de
Ceulaer (1982). Future plans include a randomized controlled study
to test the hypothesis, using a sample of both adolescents and adults,
for at least a year of treatment.
Sickle cell disease is a hereditary condition that
affects the hemoglobin in red blood cells. Hemoglobin is protein
that carries oxygen to different parts of the body. Sickle cell
disease is characterized by many medical complications. Complications
include anemia and susceptibility to infection. One of the most
common outcomes of sickle cell disease are periodic episodes of
vaso-occlusive pain(VOP). Although many studies have been done,
the cause of these pain crises are unknown. Until a cure, becomes
more readily available, treatments to reduce and prevent VOP are
being explored. Many female patients with SCD experience pain crises
with the onset of the menstrual cycle. This could be linked with
dysmenorrhea. Dysmenorrhea is pain associated the menstrual cycle.
It can be controlled with hormonal treatments, such as Depo-Provera.
Two publications have demonstrated that progestins may reduce the
frequency of painful crises in sickle cell patients. In a clinical
study of 43 sickle cell anemic females, de Abood (1997) reported
that 70% of the patients were pain free after being on Depo-Provera
for one year. Patients in the study ranged in age from 18-41. De
Ceulaer (1982) reported a 50% reduction in pain crises. Patients
in the study ranged in age from 20-41. In both studies t-tests were
done to analyze the data.
Chart Review Adolescent patients
were selected from the Georgia Comprehensive Sickle Cell Center
Each patient was assigned an acrostic, to ensure confidentiality.
Medical charts were examined to determine when Depo-Provera or oral
contraceptive treatment began. The number of hospitalizations and
emergency room visits were recorded for up to three years before
and after treatment began. Chart review was expected to provide
qualitative results, with sample size too small to do statistical
analysis.
Questionnaire Female patients ranging
in age from 18-41 were selected from the Georgia Comprehensive Sickle
Cell Center The purpose of the study was carefully explained to
each patient, so that informed consent could be obtained. A series
of survey questions was asked to the patients. Questionnaire project
is currently in data analysis using Epi-Info. All study procedures
were reviewed and approved by the Emory Institutional Review Board
and Grady Research Oversight Committee.
A)Chart Review A retrospective chart review was done on five medical
charts. Four of the five charts showed an increase in the number
of hospitalizations and emergency visits, following hormonal treatment.
Three of the patients were on Depo-Provera, while the other two
patients were on oral contraceptives. Only one of the five charts
showed a decrease in the amount of hospitalizations and emergency
visits. That patient was on Depo-Provera treatment. (see graphs
also) B)Questionnaire A questionnaire on the association between
menses and vaso-occlusive pain was presented to 75 women. Of the
women surveyed,75% reported having episodes of vaso-occlusive pain
along with their menstrual cycle. This project is currently in data
analysis using Epi-Info.
Past studies have shown that many females with sickle cell disease
often experience pain episodes with their menstrual cycle. This
pain could be linked to dysmenorrhea. Dysmenorrhea can be controlled
with oral contraceptives or progestogen, which decreases synthesis
of prostaglandins (Zahradnik & Beckwoldt, 1984). Prostaglandins
have been shown to play a role in promotion of endothelial adhesion
of sickle cells (Pintigny et al,1989). In addition endothelial adherence
levels correlate with clinical vaso-occlusive severity(Hebbel et
al, 1980). Although it is known that hormonal treatment may influence
vaso-occlusive pain, it is unknown whether estrogen or progestogen
play the major role in limiting adhesive properties on cell endothelial
adherence. Simoncini & Genazzani (2000) indicate that tibolone,
a synthetic steroid with mixed estrogenic and progestogenic/androgenic
activity, exert direct actions on the vascular wall, decreasing
the expression of endothelial-leukocyte adhesion molecules. These
preliminary data present a complex picture. The surveys show a high
prevalence of menstrual association with VOP, retrospective chart
review finds effectiveness of treatments far lower than that reported
by De Abood (1997) and de Ceulaer(1982). Although this study is
only in the beginning stages, the initial data provides valuable
information to be utilized in future studies. Future plans include
a randomized controlled study to test the hypothesis, using a sample
of both adolescents and adults, for at least a year of hormonal
treatment.
I would like to thank Dr.Lewis Hsu, Melanie Jacob, and all of the
staff of the Georgia Comprehensive Sickle Cell Center for making
this project possible.
Sickle cell disease is a hereditary blood disease
characterized by many medical complications. One of the most common
complications of sickle cell disease are periodic episodes of vaso-occlusive
pain crises (VOP). A common trigger for VOP in many females is the
menstrual period. Hormonal treatments, such as Depo-Provera have
been described to reduce the frequency of vaso-occlusive pain. This
study explores the association between menstrual periods and VOP,
in preparation for testing the hypothesis that Depo-Provera reduces
the frequency of vaso-occlusive pain crises. A chart review will
be done to determine the association between hormonal treatments
and the frequency of medical visits for VOP. A survey on the association
between menstrual periods and vaso-occlusive pain crises will also
be done. Four of the five charts reviewed showed an increase in
the frequency of pain crises following hormonal treatment. Only
one chart showed a decrease in the frequency of pain following treatment.
In addition 75% of the women surveyed reported VOP association with
the menstrual period. Preliminary data presents a complex picture.
The surveys show a high prevalence of menstrual association with
VOP, while retrospective chart review finds effectiveness of treatments
far lower than that reported by De Abood (1997) and de Ceulaer (1982).
Future plans include a formal study to test the hypothesis. Elements
of the study will consist of a larger sample size and a longer period
of treatment. Patients will include both adolescents and adults.
A randomized control group will also be used.
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