SUHANNY1 (1).pdf

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A STUDY ON SICKLE CELL ANEMIA
A Report on
PRACTICE SCHOOL
Submitted for partial fulfillment of the requirement for the
Award of degree
OF
BACHELOR OF PHARMACY
BY
SUHAILA ABDULLA SEIF
REG. NO: 2115022018
B. PHARMACY 7TH
SEMESTER
(2023-2024)
UNDER THE SUPERVISION OF
MS. RAMANDEEP KAUR SANDHU
ASSISTANT PROFESSOR, UCOP, GKU
UNIVERSITY COLLEGE OF PHARMACY
GURU KASHI UNIVERSITY,
TALWANDI SABO, BATHINDA, PUNJAB
DECEMBER 2023

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CERTIFICATE
This is to certify that the work embodied in project report entitled “A STUDY ON
SICKLE CELL ANEMIA” has been carried out by MS. SUHAILA ABDULLA SEIF
under the supervision of Ms. RAMANDEEP KAUR SANDHU. University College of
Pharmacy, Guru Kashi University, Talwandi Sabo, Bathinda, Punjab, India, during
academic session 2023-2024 and has not been submitted for the award of any other
degree.
Signature
Ms. Ramandeep Kaur Sandhu
Assistant Professor
University College of Pharmacy
Guru Kashi University
Talwandi Sabo, Bathinda, Punjab, India-151302

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UNIVERSITY COLLEGE OF PHARMACY
CERTIFICATE BY DEAN / HOD
This is to certify that SUHAILA ABDULLA SEIF bearing university roll number
2115022018 has prepared her report on “PRACTICE SCHOOL” in partial fulfillment
of the requirement for the degree of “Bachelor of Pharmacy” in Guru Kashi University,
Talwandi Sabo Bathinda Punjab India. This work was carried out by her at the University
College of Pharmacy.
Signature
Mr. Devinder Kumar
University College of Pharmacy
Guru Kashi University
Talwandi Sabo, Bathinda, Punjab, India-151302

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DECLARATION
I hereby declare that the work reported in practice school report entitled “A STUDY ON
SICKLE CELL ANEMIA” has been carried out by me under the supervision of “MS.
RAMANDEEP KAUR SANDHU” at University College of Pharmacy, Guru Kashi University,
Talwandi Sabo, Punjab during the academic session 2022-2023 and has not been submitted for
the award of any other degree.
Place: Ms. SUHAILA ABDULLA SEIF
Registration No. 2115022018
Date:

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ACKNOWLEDGEMENT
Firstly, I would like to give my great and lovely thanks to ALLAH (S.W) for blessing me
with strong and comfortable health as well as courage for the accomplishment of my
project report.
I would like to extend my gratitude to our Dean and Head of Department for allowing
me to do my research and providing invaluable guidance throughout the research.
I wish to put on record my profound gratitude to my ever-available supervisor “MS.
RAMANDEEP KAUR SANDHU”, University College of Pharmacy and Paramedical
Sciences, Guru Kashi University, without whose guidance, this study would not have
been in the way it is presented. It has been an honor to be her student. I am thankful for
the excellent example, she has provided as an epitome of utter grace, poise, intelligence,
and above all humanity.
It is a genuine pleasure to express my deep sense of thanks and gratitude to the
Management of Guru Kashi University.
Also, I direct my sincerely gratitude to my mother and father and whole my family
member for their cooperation and their support that enable me to get strength during
whole life time of study.
SUHAILA ABDULLA SEIF
Date:
Place:

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ABSTRACT
Anemia is defined as a reduction in either the percentage of red blood cells (hematocrit),
or a reduction in the concentration of hemoglobin in a sample of venous blood when
compared with reference values.
Anemia is a common nutritional deficiency disorder and global public health problem
which affects both developing and developed countries with major consequences for
human health and their social and economic development (WHO 2005). According to
WHO (2004) reports, one third of the global populations (over 2 billion) are anemic due
to imbalance in their nutritious food intake. So in this review we investigate anemia;
types, causes, symptoms and their treatments.
Sickle cell disease is a single gene disorder causing a debilitating systemic syndrome
characterized by chronic anemia, acute painful episodes, organ infarction and chronic
organ damage and by a significant reduction in life expectancy.
Sickle cell disease is an inherited monogenic disease characterized by misshapen red
blood cells that causes vaso-occlusive disease, vasculopathy, and systemic inflammation.
Chronic complications contribute to end-organ damage and adversely affect quantity and
quality of life. Proximately 300,000 infants are born per year with Sickle Cell Disease
globally.
People may be born with certain types of anemia or develop anemia because they have
certain chronic diseases. But poor diet causes iron-deficiency anemia, which is the most
common form of anemia.

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TABLE OF CONTENTS
SR. NO CHAPTER CONTENT PAGE NUMBER
1 I INTRODUCTION 1-3
2 II LITRERTURE REVIEW 4-21
3 III DISSCUSSION 22-23
4 IV SUMMARY 24-25
5 V CONCLUSION 26-27
6 VI REFERENCES 28-29

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LIST OF FIGURES
Figure number Description of figure Page number
1 Anemia 5
2 Causes of anemia 6
2 Sickle cell anemia 10
3 Sign and symptoms of sickle cell anemia 12
4 sickle cell disease 17
5 Sickle cell transfer 18
6 Pathophysiology of sickle cell disease 19

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LIST OF TABLES
Table number Description of table Page number
1 Medical prescription for sickle cell
anemia
15

INTRODUCTION
Anemia is a condition in which the number of red blood cells or the hemoglobin
concentration within them is lower than normal. Hemoglobin is needed to carry oxygen
and if you have too few or abnormal red blood cells, or not enough hemoglobin, there
will be a decreased capacity of the blood to carry oxygen to the body’s tissues. This
results in symptoms such as fatigue, weakness, dizziness and shortness of breath, among
others. The optimal hemoglobin concentration needed to meet physiologic needs varies
by age, sex, elevation of residence, smoking habits and pregnancy status. The most
common causes of anemia include nutritional deficiencies, particularly iron deficiency,
though deficiencies in folate, vitamins B12 and A are also important causes;
hemoglobinopathies; and infectious diseases, such as malaria, tuberculosis, HIV and
parasitic infections.
Anemia is a serious global public health problem that particularly affects young children
and pregnant women. WHO estimates that 42% of children less than 5 years of age and
40% of pregnant women worldwide are anemic.
Anemia results from a lack of red blood cell or dysfunctional red blood cells in the body
which leads to reduced oxygen flow to the body’s organ. The cell travel with iron and
hemoglobin which is a protein that helps carry oxygen through blood stream to your
organs all through the body and when someone develops anemia the said to be “anemic
“this is due to your organs not receiving the oxygen they need to do their jobs.
Sickle cell Anemia is a genetic condition which is caused by production of abnormal
hemoglobin, which binds with other abnormal hemoglobin molecules within the red
blood cell to cause rigid deformation of the cell. This deformation impairs the ability of
the cell to pass through small vascular channel; sludging and congestion of vascular may
result, followed by tissue ischemia and infarction.
Sickle cell anemia is a hereditary type that is transmitted by genes from parents to their
children, that is, it is not contagious and cannot be picked up by a person (like: cold or
infection) to another. Both parents carrying a single gene mutation (sickle cell trait), there
is a 25% chance that the disease will develop in their off springs, a 25% chance that the

child will be unaffected and a 50% that they will possess the genetic mutation as an
asymptomatic carrier. {1}
Sickle cell disease is a serious, inherited condition (genetic). It affects the red blood cells
in the blood. With Sickle Cell Disease, the red blood cells have a tendency to go out of
shape and become sickle-shaped (like a crescent moon) instead of their normal disc
shape. This can cause various problems. The most common type of Sickle Cell Disease is
where you have two sickle cell genes (sickle cell anemia).
The medical shorthand for this is hemoglobin SS or HbSS. Other types of Sickle Cell
Disease involve one sickle cell gene plus another abnormal hemoglobin gene of a
different type.
Anemia can cause a range of symptoms including fatigue, weakness, dizziness and
drowsiness, shortness of breath, fatigue, skin pallor, light-headedness, fast heartbeat,
hemic murmur, episodes of pain, frequent infections, swelling of hands and feet, delayed
growth or puberty etc.
Treatment depends on the underlying diagnosis. Iron supplements can be used for iron
deficiency. Vitamin B supplements may be used for low vitamin levels. Blood
transfusions can be used for blood loss. Medication to induce blood formation may be
used if the body’s blood production is reduced. {1}
AIM AND OBJECTIVES
AIM
To explore and learn all about sickle cell anemia with its pathophysiology, sign and
symptoms, treatment, preventions etc, to help all patients suffering with sickle cell
disease in the world.
OBJECTIVES
1. To learn the typical characteristics of sickle cell anemia as well as its symptoms
that indicates complicated diseases.
2. To explain the underlying causes of sickle cell disease together with their
relationship to the patient’s sign and symptoms.
3. Educate patients about the disease state, appropriate therapy and drug therapy
required for effective treatment and prevention of complications.

4. To recommend appropriate pharmacotherapy and non- pharmacotherapy
interventions for the patients with sickle cell anemia. {2}

CHAPTER
II
LITERATURE REVIEW

LITERATURE REVIEW
Anemia is defined as a condition in which the blood doesn’t have enough healthy red
blood cells or it is a reduction in red cell mass below the normal range for the age and sex
in the individual.
Anemia results from a lack of red blood cell or dysfunctional red blood cells in the body
which leads to reduced oxygen flow to the body’s organ.
The cell travel with iron and hemoglobin which is a protein that helps carry oxygen
through blood stream to your organs all through the body and when someone develops
anemia the said to be “anemic “this is due to your organs not receiving the oxygen they
need to do their jobs.
Figure 1: anemia
Causes
Although many parts of the body help make red blood cells, most of the work is done in
the bone marrow. Bone marrow is the soft tissue in the center of bones that helps form all
blood cells.
Healthy red blood cells last between 90 and 120 days. Parts of your body then remove
old blood cells. A hormone called erythropoietin (epo) made in your kidneys signals your
bone marrow to make more red blood cells.
Hemoglobin is the oxygen-carrying protein inside red blood cells. It gives red blood cells
their color. People with anemia do not have enough hemoglobin.

Figure 2: causes of anemia
The body needs certain vitamins, minerals, and nutrients to make enough red blood cells.
Iron, vitamin B12, and folic acid are three of the most important ones. The body may not
have enough of these nutrients due to:
• Changes in the lining of the stomach or intestines that affect how well nutrients
are absorbed (for example, celiac disease)
• Poor diet
• Surgery that removes part of the stomach or intestines
Possible causes of anemia include:
• Iron deficiency
• Vitamin B12 deficiency
• Folate deficiency
• Certain medicines
• Destruction of red blood cells earlier than normal (which may be caused by
immune system problems)
• Long-term (chronic) diseases such as chronic kidney disease, cancer, ulcerative
colitis, or rheumatoid arthritis
• Some forms of anemia, such as thalassemia or sickle cell anemia, which can be
inherited

• Pregnancy
• Problems with bone marrow such as lymphoma, leukemia, myelodysplasia,
multiple myeloma, or aplastic anemia
• Slow blood loss (for example, from heavy menstrual periods or stomach ulcers)
• Sudden heavy blood loss
Anemia affects different people in different ways:
• Newborns: Some infants are born with low red blood cell counts. Most newborns
don’t need medical treatment for anemia, but some with severe anemia may need
blood transfusions.
• Infants: Infants may get less iron than they need when they start eating solid
food. That’s because the iron in solid food isn’t absorbed as easily as iron in
breast milk or formula. Infants with anemia may appear lethargic.
• Children: Children do a lot of growing between birth and age 2. Children going
through growth spurts need more iron. Children with anemia may develop related
problems such as delayed development of motor skills and issues with learning.
• Women who are pregnant: Women who are pregnant may develop iron
deficiency anemia, which may increase the chance of complications such as
premature birth or giving birth to babies with low birth weight.
• Women and people designated female at birth (DFAB): Women and people
DFAB who have heavy periods (menstrual bleeding) or conditions like uterine
fibroids may lose blood and develop anemia.
• People age 65 and older: People over 65 are more likely to have iron-poor diets
and certain chronic diseases that increase their risk of developing anemia. If they
develop anemia, they may have heart conditions or weakness that makes it hard
for them to get around. They may have confusion or depression.
• People with chronic conditions: Some chronic conditions like autoimmune
diseases or cancer may increase the risk of anemia. This is anemia of chronic
disease. {2}

Types of anemia
There are many anemia types, each causing red blood cell levels to drop.
1. Nutritional anemia
• Pernicious anemia: Pernicious anemia, one of the causes of vitamin B12
deficiency, is an autoimmune condition that prevents your body from absorbing
vitamin B12.
• Iron-deficiency anemia: As its name implies, iron-deficiency anemia happens
when your body doesn’t have enough iron to make hemoglobin. Hemoglobin is
the substance in your red blood cells that enables them to carry oxygen
throughout your body.
• Megaloblastic anemia: Megaloblastic anemia is a type of vitamin deficiency
anemia that happens when you don’t get enough vitamin B12 and/or vitamin B9
(Folate).
2. Inherited anemia
• Sickle cell anemia: Sickle cell anemia changes your red blood cells’ shape,
turning round flexible discs into stiff and sticky sickle cells that block blood flow.
• Fanconi anemia: Fanconi anemia is a rare blood disorder. Anemia is one sign of
Fanconi anemia.
• Diamond-Blackfan anemia: This inherited disorder keeps your bone marrow from
making enough red blood cells.
3. Anemia’s caused by abnormal red blood cells
• Hemolytic anemia: In this anemia, your red blood cells break down or die faster
than usual.
• Aplastic anemia: This anemia happens when stem cells in your bone marrow
don’t make enough red blood cells.
• Autoimmune hemolytic anemia: In autoimmune hemolytic anemia, your immune
system attacks your red blood cells.
• Sideroblastic anemia: In sideroblastic anemia, you don’t have enough red blood
cells and you have too much iron in your system.
• Macrocytic anemia: This anemia happens when your bone marrow makes
unusually large red blood cells.

• Microcytic anemia: This anemia happens when your red blood cells don’t have
enough hemoglobin so they’re smaller than usual.
• Normocytic anemia: In this type of anemia, you have fewer red blood cells than
usual, and those red blood cells don’t have the normal amount of hemoglobin.
PATHOPHYSIOLOGY OF ANEMIA
Anemia is defined as a reduction in red cells mass below the normal range in age and
sex. It is also reflected in hemoglobin concentration. In adult HEMATOCRIT below 40%
(or Hb below 12g/dl) in male and below 35% (or Hb below 12g/dl) in female, is a cutoff
level for diagnosis of anemia.
Estimation of hemoglobin level is usually adequate for the diagnosis of anemia except in
condition which expanded plasma volumes such as pregnancy or congestive heart failure.
Sign and symptoms
• Fatigue
• Weakness
• Tachycardia
• Dyspnea
• Bounding pulse
• Palpitations
• Hemic murmur
• Tinnitus
• Headache
SICKLE CELL ANEMIA
Sickle cell Anemia is a genetic condition or a disease which is caused by production of
abnormal hemoglobin, which binds with other abnormal hemoglobin molecules within
the red blood cell to cause rigid deformation of the cell. This deformation impairs the
ability of the cell to pass through small vascular channel; sludging and congestion of
vascular may result, followed by tissue ischemia and infarction.{2}
These irregular shaped cells can get stuck in small blood vessels which can slow or
block blood flow and oxygen to part of the body.
Sickle cell anemia is a hereditary type that is transmitted by genes from parents to their
children, that is, it is not contagious and cannot be picked up by a person (like: cold or

infection) to another. Both parents carrying a single gene mutation (sickle cell trait), there
is a 25% chance that the disease will develop in their off springs, a 25% chance that the
child will be unaffected and a 50% that they will possess the genetic mutation as an
asymptomatic carrier. {2}
Figure 3: sickle cell anemia
These irregular shaped cells can get stuck in small blood vessels which can slow or block
blood flow and oxygen to part of the body. The effect of this condition is due to
abnormality of the hemoglobin molecules found in erythrocytes.
The body produces normal red blood cells that are flexible and round and can easily
move through the blood vessels. In sickle cell anemia, red blood cells become rigid and
sticky and form like a sickle or crescent moon. These irregularly shaped cells can get
stuck in small blood vessels, which can slow or inhibit the flow of blood and oxygen to
parts of the body.
The red blood cells live for 120 days before being replaced; but sickle cells usually die
within 10 to 20 days, causing a red blood cell deficiency that leads to (anemia).
Causes:
Sickle cell anemia is caused by a change in the gene that tells the body to make the iron-
rich compound in red blood cells called hemoglobin. Hemoglobin enables red blood cells

to carry oxygen from the lungs throughout the body. The hemoglobin associated with
sickle cell anemia causes red blood cells to become rigid, sticky and misshapen. For a
child to be affected both mother and father must carry one copy of the sickle cell gene
(also known as sickle cell trait) and pass both copies of the altered form to the child.
If only one parent passes the sickle cell gene to the child, that child will have the sickle
cell trait. With one typical hemoglobin gene and one altered form of the gene, people
with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.
Their blood might contain some sickle cells, but they generally don't have symptoms.
They're carriers of the disease, however, which means they can pass the gene to their
children. {3}
Signs and symptoms:
Signs and symptoms of sickle cell anemia usually appear around 6 months of age. They
vary from person to person and may change over time. Signs and symptoms can include:
• Anemia. Sickle cells break apart easily and die. Red blood cells usually live for
about 120 days before they need to be replaced. But sickle cells typically die in 10
to 20 days, leaving a shortage of red blood cells (anemia). Without enough red
blood cells, the body can't get enough oxygen and this causes fatigue.
• Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a
major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood
cells block blood flow through tiny blood vessels to your chest, abdomen and
joints. The pain varies in intensity and can last for a few hours to a few days. Some
people have only a few pain crises a year. Others have a dozen or more a year. A
severe pain crisis requires a hospital stay. Some adolescents and adults with sickle
cell anemia also have chronic pain, which can result from bone and joint damage,
ulcers, and other causes.
• Swelling of hands and feet. The swelling is caused by sickle-shaped red blood
cells blocking blood circulation in the hands and feet.
• Frequent infections. Sickle cells can damage the spleen, increasing vulnerability
to infections. Infants and children with sickle cell anemia commonly receive

vaccinations and antibiotics to prevent potentially life-threatening infections, such
as pneumonia.
• Delayed growth or puberty. Red blood cells provide the body with the oxygen
and nutrients needed for growth. A shortage of healthy red blood cells can slow
growth in infants and children and delay puberty in teenagers.
• Vision problems. Tiny blood vessels that supply the eyes can become plugged
with sickle cells. This can damage the retina — the portion of the eye that
processes visual images — and leads to vision problems. {3}
Figure 4: sign and symptoms of sickle cell anemia
Diagnosis:
A blood test can check for the form of hemoglobin that underlies sickle cell anemia. In
adults, a blood sample is drawn from a vein in the arm. In young children and babies, the
blood sample is usually collected from a finger or heel. The sample is then sent to a
laboratory, where it's screened for the sickle cell form of hemoglobin. If you or your
child has sickle cell anemia, your doctor might suggest other tests to check for possible
complications of the disease and if you or your child carries the sickle cell gene, you’ll
likely be referred to a genetic counselor.

• Assessing stroke risk
A special ultrasound machine can reveal which children have a higher risk of stroke. This
painless test, which uses sound waves to measure blood flow in the brain, can be used in
children as young as 2 years. Regular blood transfusions can decrease stroke risk.
• Tests to detect sickle cell genes before birth
Sickle cell disease can be diagnosed in an unborn baby by sampling some of the fluid
surrounding the baby in the mother’s womb (amniotic fluid). If you or your partner has
sickle cell anemia or the sickle cell trait, ask your doctor about this screening.
Risk Factors:
• sickle cell anemia most commonly affects people of African, Mediterranean and
Middle Eastern descent
• Both mother and father are carriers of the disease.
• Both mother and father have the disease.
• One of the parents is a carrier and the other has the disease.
Complications:
Sickle cell anemia can lead to a host of complications, including:
• Stroke. Sickle cells can block blood flow to an area of the brain. Signs of stroke
include seizures, weakness or numbness of the arms and legs, sudden speech
difficulties, and loss of consciousness. If your child has any of these signs and
symptoms, seek medical treatment immediately. A stroke can be fatal.
• Acute chest syndrome. A lung infection or sickle cells blocking blood vessels in
the lungs can cause this life-threatening complication, resulting in chest pain,
fever and difficulty breathing. It might require emergency medical treatment.
• Pulmonary hypertension. People with sickle cell anemia can develop high blood
pressure in their lungs. This complication usually affects adults. Shortness of
breath and fatigue are common symptoms of this condition, which can be fatal.
• Organ damage. Sickle cells that block blood flow to organs deprive the affected
organs of blood and oxygen. In sickle cell anemia, blood is also chronically low
in oxygen. This lack of oxygen-rich blood can damage nerves and organs,
including kidneys, liver and spleen, and can be fatal.

• Spleen sequestration. A large number of sickle cells can get trapped in the
spleen, causing it to enlarge and possibly causing belly pain on the left side of the
body. This can be life-threatening. Parents of children with sickle cell anemia
should learn to regularly feel their child's spleen for enlargement.
• Blindness. Sickle cells can block tiny blood vessels that supply the eyes. Over
time, this can lead to blindness.
• Leg ulcers. Sickle cell anemia can cause painful open sores on the legs.
• Gallstones. The breakdown of red blood cells produces a substance called
bilirubin. A high level of bilirubin in the body can lead to gallstones.
• Priapism. In this condition, men with sickle cell anemia can have painful, long-
lasting erections. Sickle cells can block the blood vessels in the penis, which can
lead to impotence over time.
• Deep vein thrombosis. Sickling of red cells can cause blood clots, increasing the
risk of a clot lodging in a deep vein (deep vein thrombosis) or a lung (pulmonary
embolism). Either can cause serious illness or even death.
• Pregnancy complications. Sickle cell anemia can increase the risk of high blood
pressure and blood clots during pregnancy. It can also increase the risk of
miscarriage, premature birth and having low birth weight babies. {4}
Treatment:
There is no definitive cure for sickle cell anemia; however, the management of sickle cell
anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing
complications. [6] Treatments might include medications and blood transfusions. For
some children and teenagers, a stem cell transplant might cure the disease. [7] Some
medications like the following are used;
• Hydroxyurea (Droxia, Hydrea, Siklos).
• L-glutamine oral powder (Endari).
• Crizanlizumab (Adakveo).
• Voxelotor (Oxbryta). .
• Pain-relieving medications.

Table 1: medical prescription for sickle cell anemia
Prevention:
• Abidance by a comprehensive pre-marital medical examination helps to reduce
intergenerational transmission of sickle cell disease.
• If you are pregnant, you should see a genetic counselor before deciding to have
children.
• Children with sickle cell anemia might receive penicillin between the ages of
about 2 months old until at least age 5 years. Doing so helps prevent infections,
such as pneumonia, which can be life-threatening to children with sickle cell
anemia.
• Adults who have sickle cell anemia might need to take penicillin throughout their
lives if they've had pneumonia or surgery to remove the spleen.
• Childhood vaccinations are important for preventing disease in all children.
They're even more important for children with sickle cell anemia because their
infections can be severe.

• The doctor should ensure that the child receives all the recommended childhood
vaccinations, as well as vaccines against pneumonia, meningitis, hepatitis B and
an annual flu shot. Vaccines are also important for adults with sickle cell anemia.
• During the COVID 19 pandemic, people with sickle cell anemia should take extra
precautions, such as staying isolated at home as much as possible and for those
who are eligible, getting vaccinated. {4}
SICKLE CELL DISEASE
It is a serious, inherited condition (genetic). It affects the red blood cells in the blood.
Sickle cell anemia is the name of a specific form of Sickle Cell Disease in which there
are two sickle cell genes. With Sickle Cell Disease, the red blood cells have a tendency to
go out of shape and become sickle-shaped (like a crescent moon) - instead of their
normal disc shape. This can cause various problems. Sickle Cell Disease is therefore a
group of conditions that cause red cells to become sickle-shaped.
A sickle cell gene affects the production of important chemical called hemoglobin.
Hemoglobin is located in red blood cells, which are part of the blood. Hemoglobin
carries oxygen and gives blood its red color. [17]
The sickle cell genes make the body produce abnormal hemoglobin called HbS. (Normal
hemoglobin is called HbA.) HbS behaves differently from HbA. Under certain
conditions, HbS makes the red blood cells change shape - instead of the normal doughnut
shape, they become sickle-shaped, like a crescent moon. This is called sickling.
Conditions that trigger sickling are cold, infection, lack of fluid in the body
(dehydration), low oxygen, and acid (acid is produced in hard physical exercise) (figure
6)
The most common type of Sickle Cell Disease is where you have two sickle cell genes
(sickle cell anemia). The medical shorthand for this is hemoglobin SS or HbSS. Other
types of Sickle Cell Disease involve one sickle cell gene plus another abnormal
hemoglobin gene of a different type. These include: hemoglobin SC; hemoglobin S/beta
thalassaemia; hemoglobin S/Lepore; hemoglobin SO Arab.

Figure 5: sickle cell disease
A sickle cell gene affects the production of important chemical called hemoglobin.
Hemoglobin is located in red blood cells, which are part of the blood. Hemoglobin
carries oxygen and gives blood its red color.
The sickle cell genes make the body produce abnormal hemoglobin called HbS. (Normal
hemoglobin is called HbA.) HbS behaves differently from HbA. Under certain
conditions, HbS makes the red blood cells change shape - instead of the normal doughnut
shape, they become sickle-shaped, like a crescent moon. This is called sickling.
Conditions that trigger sickling are cold, infection, lack of fluid in the body
(dehydration), low oxygen, and acid (acid is produced in hard physical exercise).
Figure6: Sickle cell transfer
EPIDEMIOLOGY OF SICKLE DISEASE
Sickle cell disease is an inherited blood condition which is most common among people
of African, Arabian and Indian origin. In disease of African origin, research has led to

models of care which prevent serious complications, improve the quality of life, and
increase survival. In India, the disease is undocumented. Thus, there is an urgent need to
document the features of Indian disease so that locally appropriate models of care may be
involved.[3]
Sickle cell disease is prevalent in central India and most cases present before 5 year of
age. VOC is the commonest crisis seen, but death often occurs due to sequestration crisis
and usually below 5 years of age.
First described in the Nilgiri Hills of northern Tamil Nadu in 1952, the sickle cell gene is
now known to be widespread among people of the Deccan plateau of central India with a
smaller focus in the north of Kerala and Tamil Nadu. Extensive studies performed by the
Anthropological Survey of India have documented the distribution and frequency of the
sickle cell trait which reaches levels as high as 35 per cent in some communities.
PATHOPHYSIOLOGY OF SICKLE DISEASE
When there is insufficient oxygen in the vascular system, sickle cell hemoglobin
becomes considerably more insoluble, increasing the polymer formation in the blood and
its overall viscosity. This leads to the formation of tactoids, which are a gel-like form of
hemoglobin, b that exist in equilibrium with its ordinary soluble state.{14}
Symptoms of sickle cell disease
• Sickle Cell Disease children have high oxidative stress, and conversely, decreased
antioxidant activity.
• Decrease in antioxidant activity explains the reduction in lipid per oxidation &
protein carbonylation.
• Chronic inflammatory disease due to persistent alteration of hemoglobin content,
reticulocyte, total bilirubin, platelet, creatinine, leukocytes, and altered expression
of inflammatory mediators. {11}

Figure7: pathophysiology of sickle cell disease
CLASSIFICATIONS OF HEMOGLOBINS
Hemoglobin is the protein in red blood cells that carries oxygen. It normally has two
alpha chains and two beta chains. The four main types of sickle cell anemia are
caused by different mutations in these genes. There are various different types of
sickle cell anemia, which are classified according to the hemoglobin abnormality.
These types include:
1. Hemoglobin SS
2. Hemoglobin SC
3. Hemoglobin SB 0 (Beta-zero) thalassaemia
4. Hemoglobin SB+ (beta) thalassaemia
5. Hemoglobin SD, SE and hemoglobin SO
6. Sickle cell trait
1) HEMOGLOBIN SS:
Hemoglobin SS disease is the most common type of sickle cell disease. It occurs
when you inherit copies of the hemoglobin S gene from both parents. This forms

hemoglobin known as Hb SS. As the most severe form of Sickle Cell Disease,
individuals with this form also experience the worst symptoms at a higher rate.
2) HEMOGLOBIN SC:
Hemoglobin SC disease is the second most common type of sickle cell disease. It
occurs when you inherit the Hb C gene from one parent and the Hb S gene from the
other. Individuals with Hb SC have similar symptoms to individuals with Hb SS.
However, the anemia is less severe.
3) HEMOGLOBIN SB 0 (Beta-zero) THALASSEMIA
Sickle beta-zero thalassaemia is the fourth type of sickle cell disease. It also involves
the beta globin gene. It has similar symptoms to Hb SS anemia. However, sometimes
the symptoms of beta zero thalassaemia are more severe. It is associated with a
poorer prognosis.
4) HEMOGLOBIN SB+ THALASSEMIA:
Hemoglobin SB+ (beta) thalassaemia affects beta globin gene production. The size of
the red blood cell is reduced because less beta protein is made. If inherited with the
Hb S gene, you will have hemoglobin S beta thalassaemia. Symptoms are not as
severe: Hemoglobin SB+ (beta) thalassaemia occurs when you inherit the
hemoglobin beta S gene from one parent and a hemoglobin beta plus thalassaemia
gene from the other parent.
5) HEMOGLOBIN SD, SE, and hemoglobin SO;
These types of sickle cell disease are less common and usually have less severe
symptoms.
6) SICKLE CELL TRAIT:
People who only inherit a mutated gene (hemoglobin S) from one parent are said to
have sickle cell trait. They may have no symptoms or reduced symptoms. {12}

CHAPTER
III
DISCUSSION

DISCUSSION
Anemia is a serious global public health problem that particularly affects young children
and pregnant women. WHO estimates; that 42% of children less than 5 years of age and
40% of pregnant women worldwide are anemic.
Anemia is an indicator of both poor nutrition and poor health. It is problematic on its
own, but it can also impact other global nutritional concerns such as stunting and
wasting, low birth weight and childhood overweight and obesity due to lack of energy to
exercise. School performance in children and reduced work productivity in adults due to
anemia can have further social and economic impacts for the individual and family.
Anemia can cause a range of symptoms including fatigue, weakness, dizziness and
drowsiness, children and pregnant women are especially vulnerable, with an increased
risk of maternal and child mortality. The prevalence of anemia remains high globally,
particularly in low-income settings, where a significant proportion of young children and
women of childbearing age can be assumed to be anemic. Iron deficiency anemia has
also been shown to affect cognitive and physical development in children and reduce
productivity in adults.
Most anemia symptoms occur because of less oxygen getting to the body’s cells and
tissues (hypoxia). The hemoglobin in red blood cells carries oxygen. So having fewer red
blood cells leads to hypoxia. If you have mild anemia, you may not have many
symptoms.
Treatment will depend on your symptoms, age, and general health. It will also depend on
how severe the condition is.{13}

SUMMARY
Anemia is a common blood disorder. It happens when you have fewer red blood cells
than normal.
When you have anemia, your blood can’t carry enough oxygen to your body. Without
enough oxygen, your body can’t work as well as it should.
There are many forms of anemia, each with its own cause. Anemia can be temporary or
long term and can range from mild to severe. In most cases, anemia has more than one
cause. Anemia can be due to a condition present at birth (congenital) or to a condition
you develop (acquired).This can happen if: Your body doesn't make enough red blood
cells, or if bleeding causes you to lose red blood cells more quickly than they can be
replaced, or if your body destroys red blood cells.
Anyone can get anemia. But it's more common in women of childbearing age. It's also
more common during pregnancy, infancy, and in older adults.
Risk factors include: A diet low in iron-rich foods, Heavy menstrual periods, Lifelong
(chronic) diseases such as kidney disease, rheumatoid arthritis, HIV, Crohn's disease, and
heart, liver, or thyroid disease.
Treatments for anemia, which depend on the cause, range from taking supplements to
having medical procedures. You might be able to prevent some types of anemia by eating
a healthy, varied diet. However Acute, chronic, and acute Hematopoietic stem cell
transplantation is the only cure available today, but is not feasible for the vast majority of
people suffering from Sickle Cell Disease. Fortunately, new therapies are in late clinical
trials and more are in the pipeline, offering hope for this unfortunate disease, which has
increasing global burden.
WHO oversees several programmes across all WHO Regions, to help reduce the
prevalence of anemia, through treatment and prevention. These guidelines, policies and
interventions aim to increase dietary diversity, improve infant feeding practices and
improve the bioavailability and intake of micronutrients through fortification or
supplementation with iron, folic acid and other vitamins and mineral.

CONCLUSION
Anemia is a common nutritional deficiency disorder and global public health problem
which affects both developing and developed countries with major consequences for
human health and their social and economic development (WHO 2005). According to
WHO (2004) reports, one third of the global populations (over 2 billion) are anemic due
to imbalance in their nutritious food intake.
WHO estimates that even among the South Asian countries, India has the highest
prevalence of anemia, what is even more important is the fact that about half of the
global maternal deaths due to anemia occur in South Asian countries; India contributes to
about 80 per cent of the maternal deaths due to anemia in South Asia. There are several
types and classifications of anemia. The occurrence of anemia is due to the various red
cell defects such as production defect (aplastic anemia), maturation defect (megaloblastic
anemia), defects in hemoglobin synthesis(iron deficiency anemia), and genetic defects of
hemoglobin maturation (thalassaemia)or due to the synthesis of abnormal hemoglobin
(hemoglobinopathies, sickle cell anemia and thalassaemia) and physical loss of red cells
(hemolytic anemia).
Some people are born with forms of anemia, but most people who have anemia develop
the condition over time. That’s why it’s important to keep track of changes in your body.
Without healthy red blood cells that do their job, your body doesn’t get the energy it
needs to function. For example, we all have days when we feel worn out. But if you’re
feeling very tired for several days despite getting rest, consider talking to your healthcare
provider. Most of the time, anemia is a short term issue easily treated by changing your
diet or taking supplements. Untreated anemia may cause serious medical issues.
While iron deficiency anemia is the most common form and is relatively easy to treat
through dietary changes, other forms of anemia require health interventions that may be
less accessible. Accurate characterization of anemia is critical to understand the burden
and epidemiology of this problem, for planning public health interventions, and for
clinical care of people across the life course. An interventions to address the underlying
and basic causes of anemia looks at issues such as disease control, water, sanitation and

hygiene, reproductive health and root causes such as poverty, lack of education and
gender norms.

REFERENCES
1. Sundd, P., Gladwin, M. T., &, Novelli E. M. (2019). Pathophysiology of sickle
cell disease. Annual review of pathology: mechanisms of disease, 14, 263-292.
2. Lonergan, G. J., Cline, D. B., & Abbondanzo, S. L. (2001). Sickle cell anemia.
Radiographics, 21(4), 971-994.
3. Kamble, Á. & Chaturvedi, P. (2000). Epidemiology of sickle cell disease in a
rural hospital of central India. Indian pediatrics, 37(4), 391-396.
4. Biswal, S., Rizwan, H., Pal, S., Sabnam, S., Parida, P., & Pal, A. (2019).
Oxidative stress, antioxidant capacity, biomolecule damage, and inflammation
symptoms of sickle cell disease in children. Hematology, 24(1), 1-9.
5. Tshilolo, L., Tomlinson, G., Williams, T. N., Santos, B., Olupot-Olupot, P., Lane,
A., & Ware, R. E. (2019). Hydroxyurea for children with sickle cell anemia in
sub-Saharan Africa. New England Journal of Medicine, 380(2), 121-131.
6. Tisdale, J. F., Thein, S. L., & Eaton, W. A. (2020). Treating sickle cell anemia.
Science, 367(6483), 1198-1199.
7. Hayman, R. (2019). Use of Post-Transplant Cyclophosphamide with
Nonmyeloablative Conditioning in HLA-Haploidentical Stem Cell Transplants
for Sickle Cell Disease.
8. Journal of science and technology investigation -ISSN: 2456-8082
9. Soundarya, N et.al JOSTI-Vol-1-1-November-2016: 10- 17
10. 16 Brodsky, R.A. and Jones, R.J., 2005. Aplastic anemia. Lancet.
365(9471):1647-56.
11. Ezzati, M, Lopus, A.D., Dogers, A., Vander, H.S., Murray C., 2002. Selected
major risk factors
12. And global and regional burden of disease. Lancet 360 : 1347-60
13. Guidelines for the management of iron deficiency anemia, British Society of
Gastroenterology
14. Harper, J.L., Marcel, E.C. And Emmanuel, C. B., 2015. Iron Deficiency
Anemia: Practice

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