Sickle Cell Disease in

the Educational Setting

School Nurse Perspective
 Objectives
•  Increase knowledge concerning sickle cell
disease including complications and
current treatments
•  Increase familiarity with issues related to
working with students with sickle cell, their
families and school staff
•  Address relationship of school with the
sickle cell disease treatment center.
What is Sickle Cell
Disease?
 Definition
•  Sickle cell disease (SCD) is a genetic
disease of the red blood cell
characterized by vaso-occlusion and
hemolysis
 Genetic Disease: Diagnosis

•  Aim of newborn
screening is to
identify infants with
SCD to start
penicillin
prophylaxis
Genetic Disease: Inheritance
• Both parents must have
an abnormal hemoglobin
trait to have a child with
SCD.

• If both parents have trait

there is a 1 in 4 (25%)
chance that each baby will
have SCD.
 Genetic Disease: Prognosis
•  Previous studies were done before the
use of hydroxyurea
•  Survival is now believed to be improved
•  Hb SS: mid-40s
•  Hb SC: mid-60s
Red Blood Cells
•  Single amino acid
substitution in the
gene for hemoglobin
•  Sickled cells are stiff
and sharp instead of
soft and round
•  Sickled cells can
cause problems all
over the body
 Vaso Occlusion
•  All cells have the genetic
defect but are not always
sickled
•  Sickled cells block flow
within blood vessels
•  Area of decreased flow is
deprived of oxygen →
tissue damage and pain
 Hemolysis
•  Normal red cell lives 120 days
•  Sickled cells may only last a few
days: the cells break apart
increasing billirubin levels
•  Bone marrow must work harder to
try and compensate
 Sickle Cell Diseases
•  Family of disorders: AA Normal (adult)

all are Hb S plus AS Sickle cell trait

another abnormal
SS Sickle cell disease: either
Hb (SS, SC, SD, Hb SS
Sthal, etc) SC Sickle cell disease: HbSC
•  This presentation S- Sickle cell disease: Hb S-
will use SCD to refer thal beta+ thalassemia; HbS-
beta0 thalassemia
to all forms
 Who has SCD?
•  1 in 8 persons of African or Caribbean
descent have sickle cell trait
•  Also found in Hispanics, and persons
from India, the Mediterranean and the
Middle East
 Case Study
•  Robert is a 15 y/o male
with sickle cell disease
in high school
•  Stephanie is his 7 y/o
sister in elementary
school
The Role of Vaso-Occlusion
 Pain

•  Occlusion of small vessels leads to

impaired oxygen delivery to tissues
•  Pain crises often occur in the same
areas of the body
•  Older children can often discern ‘sickle
pain’ from other pain
 Pain: Non-Pharmacologic
Treatment
•  Fluids, rest, warmth
•  Never ice, even for sports
injury
•  Keep parents informed, even
if child is staying at school
 Pharmacologic Treatment
Severe Pain
Strong
Moderate Opioid with
NSAID
• Important to
Pain
Weak Opioid
have pain plan
with NSAID for school
Mild Pain
APAP +/-
NSAID
• Children with frequent pain crises
can often stay at school, even if
taking strong pain medications
Pain: Prevention & School
Concerns
•  Avoid triggers: cold,
getting chilled,
dehydration
•  Cannot prevent all
crises
•  Goal is early
detection and
treatment
 Splenic Sequestration
•  Blood flows into the spleen,
but does not come back out
•  Causes life-threatening
anemia
•  Symptoms: left upper
quadrant pain, pallor,
fatigue, tachychardia
 Splenic Sequestration:
School Concerns (2nd slide)
• Often recurrent
• Young children with Hb SS
or Hb S-beta thalassemia
• Older children and teens
with Hb SC
• Hypersplenism is risk for
contact sports
 Infection

•  Sickling results in functional asplenia which

may begin in infancy
•  Increased susceptibility to infections with
certain bacteria
 Infection
•  Any fever above 101 degrees
requires evaluation and
treatment
•  Complications include:
bacteremia/sepsis,
meningitis, osteomyelitis, and
pneumonia/acute chest
syndrome
 Infection: Prevention &
School Concerns (2nd slide)
•  Treating fever is not an option,
even at family request
•  Handwashing is always a good
idea, especially during flu
season
Priapism
•  Persistent, painful,
unwanted erection
•  Blood flows into the
penis, but does not
come back out
•  May lead to
impotence
 Priapism: School Concerns
•  May refuse to discuss
•  Treat as a pain crisis
•  True priapism that lasts
beyond 2 hours requires
medical attention
•  Treatment center should
provide individualized plan
 Stroke
•  10% of children with
sickle cell will have a
stroke, mostly those
with Hb SS
•  Symptoms are similar
to an adult
•  Prompt medical
attention may reverse
damage
 Stroke: School Concerns
•  Stroke can cause subtle
deficits that are not easily
apparent
•  Change in academic
performance may be
important
•  Monthly transfusions can
prevent recurrence
 Vision Loss
•  Damage to retina causes
proliferative retinopathy
•  Can lead to blindness
•  Symptoms: seeing
floaters, impaired vision
 Vision Loss: Prevention &
School Concerns
•  Mild symptoms → significant
damage
•  All children with SCD over age
10 years need annual eye
exams
•  Eye injury (hyphema) is a
concern for those with SCD
and trait
 Dehydration/Kidneys
•  Kidneys of children with SCD are
damaged and lose more fluid
than normal
•  Symptoms: frequent urination,
increased thirst, dehydration,
bedwetting
•  Restricting fluids makes this
worse
 Dehydration and Kidneys:
Prevention & School
Concerns
•  Free access to the bathroom and water
available during class
•  Exercise and heat increase fluid needs
 Avascular Necrosis (AVN)
•  Damage to bone at the head
of the femur (hip AVN) or
humerus (shoulder AVN)
•  Can lead to collapse and
require replacement
•  Symptoms: pain, limp,
reduced range of motion
•  Mostly older children and
teens
 AVN: School Concerns
•  Structural accommodations: elevator
pass, 2nd set of books, transportation,
changing classes
•  NSAIDS before opiates
•  May be homebound
•  Encourage compliance
 Acute Chest Syndrome
•  Any change in chest x-ray with a fever or
respiratory symptom
•  Leading cause of illness and death
•  May be infection, occlusion or embolism
Acute Chest Syndrome:
Prevention & School
Concerns
•  SCD with asthma can be dangerous
•  These children have more episodes
of acute chest and should be taking
a controller/preventative medication
•  It is especially important that they
have Asthma Action Plans
 Case Study
•  Robert has frequent pain
crises in his back and also
has AVN of both hips, he is on
crutches
•  Stephanie had a stroke when
she was in kindergarten
The Role of Hemolysis
 Chronic Anemia
•  Because sickled red cells
do not live very long, the
bone marrow cannot keep
up
•  Symptoms: benign heart
murmur, fatigue
•  Infection can cause
severe anemia
 Chronic Anemia:
School Concerns
•  May tire easily
•  Delayed growth and development can
be a source of embarrassment
•  Unusual fatigue, especially with pallor
should be reported
•  Outbreaks of Parvovirus/B19
 Jaundice & Gallstones
•  Rapid red cell
destruction
increases
billirubin levels
•  Causes scleral
icterus and
gallstones
 Gallstones: School Concerns
•  Important to know baseline
icterus
•  Dehydration or illness will
increase jaundice
•  Children may be teased
because of yellow eyes
•  Gallstones are not from diet
 Case Study
•  Robert has gallstones and will
have his gallbladder removed
over summer vacation, he
always has yellow eyes and is
smaller than his classmates
•  Stephanie does not have
these problems because she
is chronically transfused
Treatments for Sickle
Cell Disease
• Preventative
• Symptomatic
• Curative
 Preventative Care: Infection
•  Penicillin prophylaxis until age
5 years
•  Immunizations: Prevnar™,
Pneumovax™ and Influenza
 Preventative Care: Blood
Transfusions
•  Blood transfusions to prevent stroke or
to protect lungs
•  Why not transfuse all patients?
Preventative Care: Hydroxyurea
•  Brand names are Hydrea™ or Droxia™
•  Increases levels of fetal hemoglobin
•  Red blood cells live longer and sickle
less
•  Can cause reversible neutropenia
•  Potential carcinogen or teratogen
•  Biggest problem is compliance
 Symptomatic Care

•  Pain medications
•  Fluids
•  Blood transfusions for
acute illness
 Cure for SCD
•  Bone marrow or stem cell
transplant
•  Can cure sickle cell, but risk of
serious and fatal complications
•  Reserved for the sickest children
with sibling matches
 Case Study
•  Robert is starting
hydroxyurea treatment and
must go to clinic every two
weeks
•  Stephanie has an implanted
port for monthly
transfusions, and uses
Desferal infusions 5 nights a
week
Special/Recurrent
Issues in
Sickle Cell Care
 Frequent Pain
at School
•  There is a small proportion of children
with SCD who require frequent use of
strong pain medications
•  Dependence versus addiction
 Strategies for Frequent Pain
Medication Use
•  Devise plan with family and
treatment center
•  Keep track of medication use
•  Watch for secondary gain from
trips out of the classroom
 Frequent Hospitalizations or
Home Illnesses
•  Some children will have periods
when they are unable to attend
school, but do not need to be in
the hospital
•  Missed school plan: tutoring,
assignment plan, 2nd set of books
•  Must allow student to complete all
required work
 Missed School for
Procedures/Transfusions
•  Elective procedures are often
scheduled in advance
•  Encourage family to plan for
missed school
•  Require documentation for
excessive absences
 Recess, Gym and Field Trips
•  Children with SCD should not be
allowed to get cold, wet or chilled
•  Swimming only allowed with permission
of family and treatment center
 Drugs and Alcohol
•  Persons who take pain medications
for medical reasons rarely become
addicted
•  Alcohol is dehydrating and can
precipitate a pain crisis
•  Alcohol and illicit drugs can cause
fatal complications when taken with
prescription pain medications
 Case Study
•  Robert has several severe pain
crises each year and will be
hospitalized for 1-2 weeks, he also
misses school on a regular basis
for smaller crises handled at home
•  Stephanie has planned
transfusions appointments and
scheduled visits with specialists
Collaboration
 Working with Students
•  Establish a relationship when well
•  Encourage the early reporting of symptoms
•  Teach about pain prevention
•  Plan for missed school
•  Expect achievement
 Working with Parents
•  Certain families have guilt and
secrecy about diagnosis
•  Establish 2-way
communication in the
beginning of the year
•  Require multiple contact
numbers
 Working with Parents
•  Keep appropriate medications at
school and require written plan
•  Make a plan for missed school
•  Reinforce academic
expectations
•  Advocate for reasonable
accommodations
 Supporting School Staff
•  Provide information and consultation
•  Clarify misconceptions
•  Ensure access to RN during school
•  Assist with limit setting
 Supporting School Staff
•  Advocate for missed school plan and
other reasonable accommodations
•  Engage parents in process
•  Evaluate need for 504 plan
•  766 referrals
 Working with the SCD
Treatment Center
•  Use staff of treatment center
as a resource
•  Keep center informed of
concerns
•  Require written treatment
plans, especially on
complicated children
 Community Resources for
Children and Families
•  DPH Special Health Care
Needs Program
•  Hole in the Wall Gang
Camp
•  Next Step Program
•  STRIVE
•  Community Support
Groups
 Case Study
•  Robert has a 504 plan and gets
door to door transportation, a
second set of books, an elevator
key and has a tutoring plan
•  Stephanie has an IEP for her
learning difficulties and her teacher
provides schoolwork before her
scheduled absences
 Conclusion
At any time, the patient with sickle cell disease
can be faced with a myriad of potentially life-
threatening and unpredictable complications.

Most patients and families with a good

understanding of the disease process have
good outcomes due to compliance with
medication regimens and an overall healthy
attitude that promotes well-being.

-Nedra Dodds MD, 2001

Questions?