Diseases and Conditions people with Sickle Cell are likely to
Acute chest syndrome
Dactylitis – swelling of the hands and feet
Painful crises: really painful episodes when blood
cells are blocked from going to certain parts of
the body – pain can occur anywhere, but it is
usually in the chest, arms, and legs
Enlarged spleen – sickle cells pool in the spleen,
and in some cases there is no spleen in the body.
An x-ray of a hand
swollen from dactylitis
Many people of African tribes were
the first to develop sickle cell disease
Most of the names they had for it
had multiple syllables – maybe to
account for the many painful
The tribes thought that there were
evil spirits and the babies that died
from sickle cell were sacrificing their
lives for the family
•1846: In the US, a scientist wrote a paper
called “Case of Absence of the Spleen,”
which talked about a runaway slave
whose body was autopsied and they
found he did not have a spleen.
•1904: A hospital intern discovered the
sickle shaped cells in a patient at a
•1910: The first formal report came out
that gave details about the strange
•1922: The disease was finally named
o It is estimated the up to 80,000 people
inAmerica have Sickle Cell Disease
o 1/500 AfricanAmericans have Sickle Cell
o 1/1000 -4000 Hispanics have Sickle Cell
o 1/12 African Americans are carriers for
o 1/50 Asians are carriers for Sickle Cell
o 1/100 Greeks are carriers for Sickle Cell
People can live a relatively normal
life with Sickle Cell if they have the
The average life expectancy for
males is 42.
The average life expectancy for
females is 48.
In 2003 the oldest patient to have
Sickle cell was an 85 year old
Sickle cell disease (SCD)
Sickle cell disease (SCD) is an inherited blood
disorder that causes the bone marrow to
produce red blood cells with defective
hemoglobin, hemoglobin S (sickled
Sickle Cell ulcers
Leg ulcers are the most common cutaneous
manifestation of SCD.
1These ulcers are characterized by an indolent,
intractable course, typically healing up to 16 times
slower than venous ulcers.
2 A patient who experiences his or her first sickle cell
ulcer is likely to ulcerate again: Approximately 97%
of healed sickle cell ulcers will recur in less than 1
3 Due to the recalcitrant nature of these ulcers,
patients may experience significant disfigurement,
social isolation, and loss of income.
The incidence of leg ulcers in patients with
SCD ranges from 25.7% to 75%.
Risk factors for the development of ulcers
include being older than 20 years,
having a lower level of fetal hemoglobin and a
hemoglobin level less than 6 g/dL,
having antithrombin III deficiency,
possessing certain human leukocyte antigens
living in certain geographic areas.
History of a sickle cell leg ulcer
Although the pathogenesis of leg ulcers in SCD has
not been completely elucidated, several theories
have been offered to explain this phenomenon. In
sickle cell disease, the amino acid valine is
substituted for glutamic acid in the beta-hemoglobin
chain of hemoglobin, leading to the polymerization
of hemoglobin when hemoglobin is deoxygenated.
This results in the sickling, or misshaping, of red
blood cells Sickled red blood cells are rigid and
inflexible, which allows them to become lodged
within smaller blood vessels and causes vascular
obstruction. Once obstruction occurs, ischemia
ensues and tissue necrosis results.
Sickle cell ulcers normally occur in areas with less
thin skin, and
decreased blood flow.
Common sites include the anterior tibial area,
dorsum of the foot, Achilles tendon area, and ankles,
with the medial malleolus being more affected than
the lateral malleolus .
Sickle cell ulcers classically appear as round,
punchcd-out ulcers with raised margins, deep bases,
and necrotic slough .Surrounding brown hyper-
pigmentation and scaling.
Multiple ulcers or scarring from healed ulcers
may be found in the vicinity.
Patients usually complain of extreme
tenderness or pain at the site of the ulcer.
This is because sickled cells lead to tissue
death, which is painful.
In essence, sickle cell ulcers represent a type
of ischemie ulcer.
A biopsy of the ulcer is nonspecific; however, sickled
red blood cells may be visualized within the dermal
Laboratory analysis for patients with suspected
sickle cell ulcers includes peripheral smear to
visualize the pathognomonic sickled red blood cells,
a complete blood count to determine the level of
anemia, and hemoglobin electrophoresis to measure
the levels of hemoglobinA, S, and F .Several types of
hemoglobin exist, with hemoglobinA being the
normal variant. Hemoglobin F is the fetal
hemoglobin, which is fairly resistant to sickling.
Therapy for sickle cell leg ulcers encompasses 3
treatment of existing ulcers,
and treatment of infection.
Because trauma is an inciting factor in these
ulcers, simple measures-such as wearing
properly fitting shoes, using insect repellent, and
promptly treating any minor trauma-are
essential components of prevention.
Given the risk of concurrent venous or
lymphatic disease, the use of support
stockings, leg elevation, and sodium
restriction may aid in decreasing edema and
preventing ulcer formation. Other important
prevention strategies include proper hygiene
and liberal use of emollients to reduce drying
and cracking of the skin.
Treatment options for sickle cell leg ulcers
can be divided into 4 categories:
and systemic medications
topical triple antibiotic ointments have been
shown to heal sickle cell ulcers and treat
A variety of dressings have been used to heal
sickle cell ulcers.The use of an Unna's boot, a
zinc-oxide impregnated dressing, controlled
edema in patients with sickle cell ulcers and
led to subsequently faster healing.5'12
Dressings may be used in conjunction with
elastic compression bandages for controlling
edema and have the advantage of requiring
weekly or twice weekly dressing changes,
minimizing cutaneous trauma
a hydrocolloid dressing that protects newly
forming epithelium while preventing
infection, has also been used alone and in
conjunction with an Unna's boot or
compression stockings to aid in the healing of
leg ulcers in SCD
Surgical interventions for sickle cell ulcers
include debridement and the use of
myocutaneous flaps and split-thickness skin
grafts. Debridement with dressings or
debriding ointments enables the removal of
Pentoxifylline is believed to work by
decreasing the sickling of red blood cells,
increasing erythrocyte deformability,
increasing leukocyte flexibility, inhibiting
platelet aggregation, reducing blood
viscosity, and decreasing plasma fibrinogen
Persons with sickle cell anemia have an increased
susceptibility to infection d Penicillin
Persons with sickle cell anemia have an increased
susceptibility to infection due to damage to one of
our major defenses against infection, the spleen,
due to restricted blood flow.
Penicillin is an antibiotic which would attack
these infections helping in quick healing and
Folic acid, which aids in the formation of healthy
blood cells could also be taken daily in large
Hydroxyurea was the first effective drug
treatment for adults with severe sickle cell
A study showed that daily doses of the
anticancer drug hydroxyurea reduced the
frequency of painful crises and acute chest
syndrome in sickle cell anemia. In addition,
patients taking the drug needed fewer blood
Blood transfusion is currently the single most
effective and proven treatment for severe
complications of sickle cell disease. It also
significantly reduces strokes.
Blood transfusions are mostly administered to
children who have severe cases of sickle cell
anemia. However, persons of all age can
receive this treatment.
Blood and Marrow Stem Cell
Bone marrow transplants are the closest things possible to
a cure for sickle cell anemia.
In bone marrow transplantation, the affected person’s
bone marrow is replaced with cells containing genes for
the non-sickle cell hemoglobin.
Production of healthy red blood cells starts and the sickle
shaped cells are prevented. If the transplant is performed
when the affected person is still young then success rates
can be as high as 90-95%.(Sickle Cell Society 2005)
Gene therapy is the relatively new idea of inserting genes
into the cells of an individual’s tissues and cells in order to
treat a hereditary disease, such as sickle cell anemia, in
which a defective mutant allele is replaced with a
Gene Therapy would be the best cure for sickle cell
anemia, but work in this method of treatment is still very