CPM Spring 2019 - 13

daup h i n c m s .o rg

A resolution on the recognition of sickle
cell anemia as a public health problem
was adopted by the United Nations (UN)
General Assembly in December 2008. The
resolution called for global efforts "to bring
the disease out of the shadows." Member
states and UN organizations were urged to
raise awareness of sickle cell disease (SCD)
annually on June 19th. Anecdotally, it is said
that June 19th was intentionally chosen as
World Sickle Cell Awareness Day.
Juneteenth is the oldest known celebration commemorating the ending of slavery
in the United States (US). Although President Lincoln's Emancipation Proclamation
became effective January 1, 1863, the news
that the war had ended and the enslaved
were free did not reach Texas until 2 ½
years later, in April 1865 (the reasons for
this are unclear). Celebrations of Juneteenth
have waxed and waned over the past 150
years, ranging from family gatherings and
prayer services to becoming an official
Texas state holiday, Emancipation Day.
The District of Columbia and other states
and US territories observe Emancipation
Day on various dates.
Nearly 90% of the world's SCD population lives in three countries: Nigeria, India,
and the Democratic Republic of Congo.1
Although inexpensive and simple methods
for newborn screening and adult testing
exist, there's limited availability in Africa
and systematic screening is uncommon.2
Newborn screening has resulted in most
patients with SCD surviving into adulthood
in high income countries such as the US. In
low income countries, children often die
undiagnosed, usually from malaria or other
infection. Survival is increasing, however, due
to urbanization, where there is improved access
to health care, rising demand for hospital
services, information through community-based services, prophylactic antimalarials
and antibiotics, and social support.3

to comprehensive healthcare, especially for
adults; and insufficient research resources.

Nearly 90%
of the world's SCD
population lives
in three countries:
Nigeria, India, and
the Democratic
Republic of Congo.

SCD affects approximately 100,000
people in the US and millions of people
worldwide. It is estimated in the US that
SCD occurs among 1 out of every 365 Black
or African-American births and among 1 out
of every 16,300 Hispanic-American births.
About 1 in 13 Black or African-American
The attention to and awareness of SCD babies are born with sickle cell trait. 4
in the US is also wanting-there is a lack of In 1949, SCD became the 1st inherited
overall public awareness; a shortage of clini- condition identified at the molecular level.
cians with expertise in SCD; limited access

SCD is a very complex, inherited recessive genetic disease with several genotypes
(hemoglobin SS, hemoglobin SC, sickle
beta thalassemia, others) & varying degrees
of severity. It derives its name from the
crescent or quarter moon shaped red blood
cells (RBCs). These deformed RBCs have
decreased flexibility, a shortened lifespan
(10-20 days vs 120 days for normal RBCs)
and cause a hemolytic anemia.
The vast number of complications seen
in patients with SCD as they age can be
explained in part by hemolysis, vaso-occlusion of vessels, and increased adhesion
of RBCs to the endothelial cells. The
hemolysis generates reactive oxygen species
and free plasma hemoglobin (Hb). The
free plasma Hb, a powerful scavenger of
nitric oxide, plays an important role in the
vascular pathology of SCD when deficient.
Vaso-occlusion of vessels which results from
the abnormality of the sickled RBCs is a
cause of bone, tissue and organ damage.
Increased adhesion of RBCs to endothelial
cells results in systemic vascular damage
and intimal proliferation.
Continued on page 14
Central PA Medicine Spring 2019 13

http://www.dauphincms.org

CPM Spring 2019

Table of Contents for the Digital Edition of CPM Spring 2019