SYMPTOMS OF SICKLE CELL DISEASE
The symptoms of SCD are typically intermittent, meaning
they are experienced in episodes that come and go.
Symptoms also tend to vary in frequency and severity from
person to person and may change over time. They can occur
in conditions that cause hypoxia (low oxygen), such as
intense physical exertion or stress, infections including
malaria, cold and dehydration. Symptoms of SCD may
include:
* Painful episodes
Episodes of pain – also known as sickle cell crises (e.g
vaso-occlusive crisis) – are one of the most common
symptoms of SCD. The pain can vary in severity and may
come on suddenly. Most often it is felt in the bones and
joints, though it may also occur in other locations, such
as the hands, feet, arms, legs or abdomen. Any area with
both sensory nerves and blood supply can be affected
during a painful episode. Many people with SCD experience
chronic, low-level pain, often in bones and joints.
Painful episodes are caused by sickle-shaped cells
blocking blood vessels and cutting off the oxygen supply
to tissues. Unlike normal red cells, which are usually
smooth and malleable, the sickle red cells cannot squeeze
through small blood vessels. When the sickle cells block
small blood vessels, the organs are deprived of blood and
oxygen. This leads to periodic episodes of pain and damage
the vital organs. Sickle red cells die after only about 10
to 20 days instead of the usual 120 days, because they
cannot be replaced fast enough the blood is chronically
short of red cells, causing anemia.
Type of sickle cell crisis include:
-
Vaso-occlusive crisis (VOC)
-
Visceral sequestration crisis
-
Acute abdominal crisis
- Aplastic crisis
* Acute chest syndrome
Acute chest syndrome occurs when blood vessels in the
lungs become blocked, typically causing chest pain, a high
temperature, coughing and shortness of breath. It is
treated as a medical emergency requiring urgent
treatment.
* Infections
People with SCD are susceptible to severe infection from
certain types of bacteria. Common types of infection
include: blood infection, also known as septicemia lung
infection, also known as pneumonia, infection of the
surroundings of the brain and the spine, also known as
bacterial meningitis, bone infection, also known as
osteomyelitis and malaria infection.
* Severe anemia
People with SCD will normally also experience mild to
moderate anemia, a lack of hemoglobin. Periodically,
however, this anemia can become severe and require urgent
medical attention. This may occur suddenly or more
gradually. Signs and symptoms of the onset of severe
anemia include: Tiredness, Shortness of breath, Dizziness,
Nausea, Paleness, particularly in the eyelids, lips,
tongue and nail beds.
*Jaundice (yellowness of the eyes)
This occurs as a result of lysis of the sickled (crescent
shaped) red blood cells with a resultant yellow pigment
called bilirubin which is responsible for the yellow
coloration of the eyes, the mucous membrane of the mouth,
the Palm, the sole of the feet and the nail beds.
Abdominal protrusion
In individuals whose spleen has not gone through
auto-splenectomy (self-shrinkage) before the age of six
(6) years, the enlargement of the spleen makes the abdomen
big. If the liver is equally enlarged, these two organs
are responsible for the abdominal protrusion that is seen
in some of them.
Hypognatism and frontal bossing
Under development of the jaw results in this and it is
seen in most sickle cell disease individuals. Bossing of
the frontal part of the head is referred to as frontal
bossing, this is also common in SCD.
MANAGEMENT OF SICKLE CELL DISEASE
A number of treatment methods are available to manage the
symptoms of SCD and prevent complications. However, in
most cases, the disorder cannot be cured and will require
lifelong management. Individuals with sickle-cell disease
are placed on routine medications which includes
multivitamins like folic acid, vitamin Bco, multivite,
vitamin c, they are also expected to use malaria
preventive either Paludrine tablets which is taken on
daily basis or metaprim tablets which is taking once a
week. Iron supplements should be avoided as it is
injurious to their health, they have more than enough iron
from the broken down red cells. Liberal oral fluid intake
should be taken generously, at least about 5 liters of
water per day. They should practise good personal hygiene,
eat balanced healthy diet and avoid precipitating factors
that will cause sickle cell crisis such as cold, stress,
infection and extreme physical exertion.
People with SCD are managed by medical Doctors and will be
required to attend check-ups at regular intervals. They
will also be taught how to recognise the condition’s
symptoms, so that treatment can be given as soon as
possible after they appear. Staying healthy is an
important principle of managing SCD.
Goals of SCD management include maximizing tissue
oxygenation (perfusion), preventing and treating
complications associated with anemia and sickle-cell
crisis, preventing and aggressively treating infection
including malaria and other blood borne infections, and
managing pain.
If a painful episode is severe, professional medical
attention should be sought as soon as possible. If fever
appears, medical attention should be sought right away:
fever is a medical emergency in people with SCD.
Oral hydroxyurea (HU)
Long-term oral hydroxyurea (HU) administration reduces or
prevents many acute and chronic complications of SCD. HU
is rapidly absorbed, has near-complete bioavailability,
and is therapeutic with once-daily oral dosing. Decades of
research have established that the most effective
treatment for SCD is to increase circulating normal or
fetal hemoglobin (HBF) and reduce HBSS. Increasing the
concentration of HBF is the primary effect of HU. Other
benefits of HU include lowering the number of circulating
leukocytes (white blood cells) and reticulocytes (immature
red blood cells) and decreasing their adhesiveness, which
can reduce vascular occlusion leading to VOC. HU also
increases RBC size and improves cellular deformability,
which increases blood flow and reduces VOC. Nitric oxide,
a vasodilator, is released directly from HU metabolism and
may contribute to local vasodilation.
HU therapy substantially reduces the frequency of painful
episodes associated with VOC and the need for transfusions
and hospitalizations. Indications for HU therapy include
recurrent VOC (3 or more severe episodes requiring
admission in the last 12 months), recurrent acute chest
syndrome (2 or more episodes in a lifetime), severe
symptomatic anaemia, and recurrent priapism (unnessary
painful erection). Although HU isn’t as effective as
blood transfusion therapy in preventing stroke or VOC,
it’s the most widely used therapy and has proven
efficacy in children and adults with SCD. HU is the only
FDA-approved pharmacologic treatment for induction of HbF
in adults with SCD, and it’s also approved by the
European Medicines Agency for both children and adults
with SCD. The benefit is directly related to the amount of
HbF produced in response to the drug. However, several
clinical studies have shown that individual responses to
HU treatment vary.
… To be continued
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