Sickle cell disease (SCD) is an inherited blood disorder caused by an abnormal hemoglobin. There are four main types of SCD - sickle cell anemia being the most common and severe form. Diagnosis involves tests like hemoglobin electrophoresis. Symptoms include painful episodes, anemia, infections, and organ damage over time. Treatment focuses on pain management, preventing complications, and increasing tissue oxygenation through medications, transfusions, and hydroxyurea. Nursing care emphasizes relieving pain, increasing fluid intake, preventing infections, and educating families. With good management, the life of a patient with SCD can be prolonged.

2. WHAT IS SCD?
 An inherited genetic abnormality of the hemoglobin
related to the presence of HbS which is very sensitive
to low oxygen
 It is a serious and life long condition
 Males and females are affected equally

4. TYPES/FORMS OF SCD
1. Sickle C Disease
2.Sickle Cell Thalassemia
3. Sickle Cell Trait
4.Sickle Cell Anemia

5. Types of SCD
1. Sickle C disease (less common and less severe)
a heterozygous HbS (45-55%) and HbC (45-55%)
2. Sickle Cell Thalassemia (less common and less
severe)HbA(0-35%, HbS(50-90, HbA2(3-8,and HbF (2-20%
3. Sickle Cell Trait (very mild to asymptomatic
a heterozygous state showing HbA and HbS
normal amount of HbA2 HbS 20%-50%
1 parent hbA and I parent HbS
4. Sickle Cell Anemia (most common and most severe a
homozygous state showing almost exclusively HbS. A small
amount of Hb F may also be present both parents has HbS

6. TRANSMISSION OF SCD

7. DIAGNOSTIC PROCEDURES

8. DIAGNOSTIC PROCEDURES

9. DIAGNOSTIC PROCEDURES

10. DIAGNOSTIC PROCEDURES

11. DIAGNOSTIC PROCEDURES
 AMNIOCENTESIS
Withdrawing a small sample of amniotic fluid from the uterus
with a long thin needle guided by ultrasound (14-16 wks)
OPD procedure takes 45-60 mins
An invasive procedure but generally safe
Rare risk and complications include miscarriage, amniotic fluid
leakage, infection and injury to the baby
 CHORIONIC VILLUS SAMPLING
Consists of withdrawing a small sample of placental tissue in
one of two ways 1. transcervical or 2. transabdominal (10-13wks)
Rare risk complication include miscarriage, dizziness, infection,
abdominal cramps, Rh incompatibility

12. DIAGNOSTIC PROCEDURES
 SICKLEDEX / SICKLING TEST
A simple blood test used to determine the presence of
sickle cell in the blood
 Hb ELECTROPHORESIS
A blood test that measures hemoglobin levels and looks
for abnormal types of hemoglobin
A confirmatory test for SCD

13. Clinical Features of Sickle Cell Anemia

14. Clinical Features of Sickle Cell
Anemia

15. Clinical Features of Sickle Cell Anemia

16. What happens during crisis
crisis is triggered by low oxygen tension in the blood
1. Vaso-0cclusive
Capillaries and small blood vessels are occluded by the sickle shaped cells,
causing ischemia and infarctions leading to acute or chronic tissue injury
2. Hyperhemolytic
Rapid death of RBC life span is 20 days when they breakdown they release
bilirubin causing jaundice to patients
3. Aplastic
RBC production coming to a halt bone marrow tries to produce
more RBC but cant keep up in producing every 20 days
4. Spleen sequestration
Spleen not working, blood stays in the spleen instead of flowing through it
which causes it to get bigger,swells and blood count falls.

17. FACTORS THAT CAUSES SICKLE
CELL CRISIS
S - significant blood loss
I - illness
C - climbing or flying to high
altitudes
K - keeping continued stress
L - low fluid intake
E - elevated Temperature

18.  S- significant blood loss like surgery and trauma which
increases the demand of oxygen
 I-illness spleen not functioning well because of the
deminished blood flow to it and the clogging of sickled cell
which causes the spleen to swell leading to illness or
infection (infection)
 C-climbing or flying to high altitudes increases the body
demands to oxygen
 K-keeping continued stress that can be mental or
physical stress that alters the increase need of the body for
oxygen
 L-low fluid intake that causes dehydration
 E-elevated temperature that arises from fever or strainous
exercise that increases O2 demand or COLD extreme
change in temperature to keep the body warm it needs
more oxygen

19. SYMPTOMS OF SICKLE CELL ANEMIA
EXTRAVASCULAR
HEMOLYTIC ANEMIA
 tired and pale
Jaundice
Urine discoloration
Splenomegaly
Hepatomegaly
Skeletal changes
VASO-OCCLUSIVE CRISIS
Dactylitis
Acute Chest syndrome
Osteomyelitis
Renal Papillary Necrosis
Chronic Abdominal Pain
Autosplenectomy
Recurrent Leg Ulcers
Aplastic Crisis
Recurrent leg Ulcers
Proliferative Retinopathy
Stroke

20. SYMPTOMS OF SICKLE CELL DSE
1. Episodes of PAIN
Periodic episodes of pain called crisis are a major
symptom of SCA./ Pain develops when a sickle shaped red
blood cells block blood flow to the through tiny blood
vessels to the chest, abdomen and joints. Opiods meds
around the clock are given
2. Painful swelling of hands and feet / Dactylitis
Caused by blocking blood flow to the hands and feet due
to sickled hemoglobin
3. Anemia
Sickle cell break apart easily and die 10-20 days. Normal
120 days signs includes loss of appetite paleness
weakness irritability and jaundice

21. SYMPTOMS OF SICKLE CELL DSE
3. Frequent Infections
Sickle cell can damage an organ that fights infection
’’spleen’’
4. Delayed Growth
RBC provides our body with oxygen and nutrients we need
for growth and a shortage of it can slow down growth for
infants and children and delay puberty in teenagers
5. Vision Problems
Tiny blood vessels that supply the eye may become
plugged with sickle cell that can damage the retina (the
portion of the eye that processes visual images.

22. COMPLICATIONS OF SCA

23. COMPLICATIONS OF SCA
 Brain (thrombosis or hemorrhage causing
paralysis,sensory deficits or death
Sickle cell block blood flow to an area of the brain,signs of
strokes include seizure, weakness or numbness of arms and
legs, sudden speech difficulties and loss of consciousness
 Eyes (hemorrahge, retinal detachment, blindness
retinopathy
Retinal vessel obstruction msy result in hemorrhage scarring,
retinal detachment and blindness
 Lungs (Acute Chest syndrome < acute pulmonary
complicationsincluding pneumonia tissue infarction and
fat embolism>, Pulmonary Hypertension, Pneumonia
A life threatening complication causes chest pain, fever and
difficulty of breathing. It is caused by lung infection or by sickle
cells blocking blood vessels in the lungs

24. COMPLICATIONS OF SCA
 Heart
become ischemic and enlarged leading to heart failure
 Kidneys
Can be injures from the increased viscosity and lack of oxygen
and can lead to renal failure
 Spleen
autosplenectomy can occur where this misshapen block
flow to the spleen causing scarring and eventually atrophy
 Bones and Joints
Dactylitis, osteoporosis and osteoclorosis occurs after
infarction

25. COMPLICATIONS OF SCA
 Liver and Gallbladder
Sickle cell that block blood flow through the blood vessels thus
liver can not get enough oxygen causing liver cell death.
Due to extramedullary hematopoeiesis in response to chronic
anemia
Due to transfusional haemosiderosis and chronic viral hepatitis
from repeated blood transfusion
The breakdown of RBC’s produces bilirubin. A high level of
bilirubin in the body can cause gallstones
 Leg Ulcers
 Gallstones
The breakdown of RBC produces bilirubin. A high level of
bilirubin in the body can lead to gallstones.

26. COMPLICATIONS OF SCA
 Penis
Priapism <persistent penile erection> occurs
when penile vein becomes occluded
 Skin
Stasis ulcers of hands, ankles and feet due to
vaso occlusion

27. AIM OF TREATMENT
1. Avoiding Crisis
2. Relieving Symptoms
3. Preventing
Complications

28. MEDICATIONS
Antibiotics
penicillin
Pain Relieving Medications
NSAID- diclofenac, ibuprofen paracetamol
<acetaminophen>
Opioids – tramadol,codeine, morphine, fentanyl and
pethedine
Hydroxyurea
increases production of HbF thus reducing hemolysis,
an increase in Hb concentration and a decreased in
sickled cell

29. TREATMENT OF SCA
 Vaccination to prevent infection
Pneumococal and flue vaccines
 Blood transfusions
 Nitric Oxide
Is a gas that helps keep blood vessels open and reduces the
stickiness of the RBC. Treatment with inhaled nitric oxide
might prevent sickle cell clumping together
 Bone Marrow Transplant
Also known as stem cell transplant. The only potential cure
for SCA.usually done 16 yrs below becoz of complications

30. Nursing and Collaborative Management
is directed toward giving quality of life to the pt. by alleviating the symptoms from the
complications of the disease and minimizing organ damage
RELIEVING PAIN / PAIN CONTROL
Identify and use effective measures to alleviate pain such as
Carefully position and support painful areas
Hold or rock the infant; handle gently
distract the child by singing reading stories, providing playing
activities
bath the child with warm water, applying heat or massage
maintain bedrest during crisis
remove tight clothes, watches or bp cuff or anything that can
occlude blood circulation
avoid cold compress as it causes vasoconstriction that
exacerbate the pain while warm compress causes vasodilation
that promotes circulation decreasing the pain and muscle
tension
give pain reliever medications as ordered some meds round the
clock and not PRN

31. Nursing and Collaborative Management
 INCREASING TISSUE PERFUSSION
Administer oxygen
Administer IV fluids to reduce blood viscosity and maintain
renal function
Administering blood transfusion for severe anemia, vaso
occlusion and aplastic crisis
 ASSESSING AND MANAGEMENT OF DEHYDRATION
Assessing for signs of dehydration such as poor skin turgor,
sunken fontannels and eyes dry tongue,. If the pt has vomiting
diarrhea or excessive sweating. Fever, increased heart rate,
decreased blood pressure and faster breathing are signs of
dehydration.Encourage increase fluid intake and administer Iv
fluids as needed

32. Nursing and Collaborative Management
 REDUCING INFECTION
Due to the infarcted and dysfunctional spleen which helps us to
fight against infection. Infection is the major cause of morbidity
and mortality
Administer antibiotic as prescribed
Give meticulous care to leg ulcers and other open wounds
Use good hand washing and meticulous technique in all
procedures
 PREVENTING HYPOXIA
Check the respiratory rate and depth frequently
Obtain pulse oximetry reading
Assess for change in consciousness or mentation
Minimizing stress and encourage deep breathing

33. Nursing and Collaborative Management
Monitor for and prevent respiratory depression caused by
narcotics (encourage deep breathing and coughing)
Help reduce the risks of anesthesia and blood loss during
surgery ( administer BT as prescribed to suppress the
formation of new sickle cells and to reduce the threat of
anoxia, maintain adequate hydration before and after
surgery and observe for sign of infection especially of the
respiratory tract)
 Improving Activity Tolerance
Maintain bedrest during crisis then increase activity
gradually to increase endurance
Encourage rest periods alternating with activity
Encouragegood eating habits, sleep and relaxation

34. Nursing and Collaborative Management
 FAMILY TEACHINGS
Avoiding Sickling Episodes
do not allow child to become chilled or to wear tight
clothing that might impede circulation
provide adequate fluids and notify health providers if
excessive fluids are lost
prompt treatment of cuts, sores, mosquito bites and
notify health care provider if exposed to communicable
disease.

35. .
 Teach Signs of Mild Crisis and Management
Fever, decreased appetite, irritability, pain or swelling in
abdomen extremities and back, Give fluids, administer
antipyretic as prescribed, encourage rest, keep the child
warm. Applying warm compress to painful area.
 Teach Signs of Severe Crisis
Pallor, lethargy and listlessness, difficulty in awakening,
irritability, severe pain, high grade fever

36. WE CAN DO IT!!!
GOOD MANAGEMENT OF SICKLE CELL DISEASE IN PREVENTING
SICKLE CELL CRISIS PROLONGS THE LIFE OF OUR PATIENT