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Manifestations:
Pallor; Pale mucus membranes
Enlarged spleen and heart
Poor muscle tone with decreased activity
Fatigue
Diagnosis
low hemoglobin (<11g/100mL)
Low hematocrit (<33%)
RBC are small and hypochromic
Serum iron levels are low ( 30g/mL)
Iron Deficiency Anemia
Treatment
Oral iron supplements
Ascorbic acid (vitamin C)
Diet high in iron
Iron-fortified formula or supplements
Limit cows milk to 24oz/day for children
>12 months
Increase age-appropriate iron-rich foods
Definition
Sickle-shaped
Disc-shaped
Soft (like bag of
Hard (like piece
of wood)
jelly)
Often get stuck
Easily flows
in small blood
through small
vessels
blood vessels
Lives for 20 days
Lives for 120 days
or less
Genetics
Hematuria
Minor Role:
Manufacturers RBCs toward end of fetal
life
and after birth, taken over by bone marrow.
Acts as a blood reservoir.
Spleen in the Infant
Soft, purplish-red
organ that lies under
the diaphragm on the
left side of the
abdominal cavity
Filters old blood and
clears bacteria
Teach Parents How to
Measure Spleen
A tongue
depressor can be
used to measure
and track spleen
size
Place tip on left
nipple and make
mark where the
spleen tip is felt
Treatment for Sequestration
Crisis
Intravenous fluids
Blood transfusion
Spleen removal
Effects of Chronic Crisis
States on the Childs Body
Spleen more susceptible to infection.
Gradual fibrosis and scarring with reduction
in spleen activity. Asplenia.
Liver enlarged, firm, tender
Brain single episode of sickling CVA,
seizures
Heart enlarges and murmurs develop
Lungs pulmonary edema and stasis
Kidneys hematuria, unable to concentrate
urine
Bone Infarcts hands and feet swell.
Effects of Chronic Crisis
States on the Childs Body
Eyes bleeds in retina.
Leg Ulcers
Are seen in 10-15% of older
children
May start as simple insect
bite or cut that does not heal
With poor circulation
develops into leg ulcer
Treated with dressings, leg
elevation and elastic
stockings.
Diagnostic Tests
Provide oxygenation
Adequate hydration
Pain Management
Circulatory overload
Iron Overload
Give Desferal, an iron-chelating agent,
to decrease the iron levels
Oxygenation
Hepatitis B series
Recipient of blood and blood products.
Pneumococcal
Pneumovax for now at age 24 months
and 5 years, with an improved vaccine
coming on the market soon for
administration to infants.
Meningococcal vaccine
H. influenzae vaccine
Child / Parent Teaching
Assess baseline knowledge and teach
accordingly:
Causes of disease and consequences, genetic
counseling
Situations that cause sickling
Signs of developing crisis, infection,
When to call the doctor
Keep in school, promote normal g&d as much as
possible
Allow for decreased endurance - let the child set his
or her own pace during strenuous exercise, and to take rest
breaks when fatigue
Explain some complications: CVA, anemia,
swollen spleen, liver problems
Support child and family
Critical Thinking
True or False
False
This is a myth. External wounds are usually not
serious.
Hematuria
As the hours pass, the joint becomes hot to the touch. Fully flexing or
extending the joint becomes painful. Weight bearing becomes difficult. By
this time, the joint is visibly swollen.
As the bleeding continues and the swelling increases, all movement in the
joint is lost. The joint becomes fixed in a slightly flexed position in an attempt
to relieve the interior pressure in the joint. Pain at this point can be
excruciating.
The bleeding slows after several days when the joint is so full of blood that
the pressure inside the joint cavity is equal to the pressure inside the broken
blood vessels. Slowly, the bleeding stops and the long process of absorbing
the blood in the joint cavity begins.
Assessment
CNS bleeding - Major cause of death
Signs and Symptoms of CNS bleeds
Persistent or increasing headache
Repeated vomiting
Sleepiness or a change in normal behavior
Sudden weakness or clumsiness of an arm or leg
Stiffness of the neck or complaints of pain with neck
movement
Complaints of seeing double
The development of crossed eyes
Poor balance when walking, a lack of coordination
Convulsions or seizures
Assessment of Child
Bleeding in the
mouth
Chemotherapy
Surgery
Fatigue
allow for naps an rest periods (coordinate care),
encourage parent to cuddle in bed with child, pillow,
blankets, favorite toys
Pain
Nursing Responsibilities
Related to Chemotherapy
Know OSHA guidelines for administering
antineoplastic agents
Confirm all measurements and calculations
Double-check ordered dosages
Obtain complete blood count within 48 hours of
chemotherapy administration
Note white blood cell and platelet levels before
chemotherapy begins
Know side effects of chemotherapeutic agents
and ways to alleviate these effects
Nursing Responsibilities
Related to Chemotherapy
(Contd)
Ensure patency of IV tubing by checking
for blood return
Ensure needle placement for
implantable infusion device
Give vesicants (agents that can cause
tissue necrosis) only through a fresh IV
site
Have emergency drugs available
Review of Common Side Effects
of
Chemotherapy and Radiation
Chemotherapy Radiation side effects
Bone marrow Skin reactions
suppression Fatigue
Alopecia Bone marrow
Malaise/fatigue suppression
Nausea Nausea
Vomiting Vomiting
Anorexia Anorexia
Stomatitis Mucositis
Surgery
Curative
Remove the tumor and
cancerous tissue
Palliative
Relieve complications due to
the cancer
Bone Marrow and Stem Cell
Transplantation
The goal of therapy is to administer a lethal dose
of chemotherapy and radiation therapy that will
kill the cancer and then re-supply the body with
bone marrow and stem cells to reconstitute
immunologic function.
Allogeneic
bone marrow comes from matched sibling (one in
four chances) or someone who is histocompatible.
Autologous
own bone marrow. May be harvested at time of
remission in preparation for relapse or when bone
marrow is free of malignant cells. Also being used
so toxic doses of chemotherapy and radiation can
be administered and the bone marrow rescued.
Procedure
First --All potential donors are typed for HLA (human
leukocyte antigen) compatibility.
Iliac crest
Side effects of
Transplantation
1. Graft-Versus-Host Disease (GVHD) potentially
lethal immunologic response of donor T cells against
the tissue of the recipient.
Signs and symptoms rash, malaise, high fever,
diarrhea, liver and spleen enlargement.
Because there is no cure, prevention is essential.
Careful tissue typing, irradiation of blood products
which helps to inactivate mature T lymphocytes.
Blood Work
Leukocytes are > 10,000
Platelet count, Hgb and Hct low
Blast cells appear (where they normally dont)
Intrathecal chemotherapy is
instilled in to the spinal canal for
cancers that have metastasized to
the brain
What is the rationale for
the use of cranial
radiation in addition to
chemotherapy?
Community Resource
Candlelighters Childhood Cancer Foundation (CCCF) is a
national non-profit membership organization whose mission
is to educate, support, serve, and advocate for families of
children with cancer, survivors of childhood cancer, and the
professionals who care for them.
http://www.candlelighters.org/
Tumor is vascular
Assessment
Palpable abdominal mass
Firm and smooth
**The abdomen should not be palpated once the
diagnosis is made. Avoid palpating the tumor mass
during assessment because of the risk of rupturing the
protective capsule. Excessive manipulation can cause
seeding of the tumor and spread of cancerous cells
Abdominal distention
Fever
Fatigue
Late signs
Anemia
Hematuria, dysuria
Hypertension
Diagnostic Tests
Abdominal ultrasound
CT, MRI
Biopsy
Interventions
Post-op
Monitor kidney function, Strict I & O
Monitor vital signsB/P and temperature
Monitor GI function assess bowel sounds and stool
production
NG tube to drainage. Measure abdominal girth.
Ewings Sarcoma
Clinical manifestations
Treatment sequence
How does this treatment differ from
osteosarcoma?
Clinical manifestations-
Treatment sequence-
Chemotherapeutic agents
(VAC)
Osteosarcoma
Clinical manifestations
Treatment sequence
Preschool to 5 years
More afraid of separation from parents than of
thought of dying. Greatest fear is separation.
Envision death as temporary, and have little of
adults fear of it
Think of it as a long sleep, not a final process.
Nightmares increase.
May feel pain / illness is a punishment for misdeeds
or thoughts
May ask questions about death
In long term illness may simulate adult response
with depression, withdrawal, fearfulness, anxiety
School Age 5-9
Begin to understand that death is permanent
May think it is something that only happens to
adults
Become aware of what is happening to them
when their disorder has a fatal prognosis.
Concerns center around fear of pain, fear of
being left alone and leaving parents and friends.
May associate death with sleep and may be
afraid to go to sleep without someone near
them.
May associate death with darknesswant light
left on in room
Understanding Death
Adolescent, older school age
By age 10 have an adults concept of death, realizing
that it is inevitable, universal, and irreversible. Have
more understanding than adults realize.
Understand that death is the cessation of life.
Emotional outbursts may reflect anger
View death as fearsome and fascinating (increase in
adolescent suicide).
May feel immune to death and deny symptoms for
longer than usual because they believe it is
impossible that anything serious could happen to
them.
Some adolescents consider themselves alienated
from their peers and unable to communicate with
their parents for emotional support feeling alone in
their struggle.
Nursing Care
Child
1. Elicit child's understanding of death before discussing
2. Encourage children to express feelings in own way
through play, drawings, or verbalization to promote
free expression.
3. Provide a safe, acceptable outlet for expressions of
feelings
4. Structure care of child to allow child choices and
participation in process within constraints of physical
condition
5. Help child maintain independence and control; normal
ADL as much as possible (set realistic goals)
6. Realize that they will go through the stages of dying:
denial, bargaining, anger, depression, acceptance
Nursing Care
Parents
1. Spend time with family to listen, answer questions, and
provide information. Discuss issues with parents before
discussing with child.
2. Provide opportunities for family to express their emotions
and deal with their feelings. Parental reactions: continuum
of grief process and usually depend on previous experiences
with loss, intellectualization.
3. Reactions may depend on relationship with child and
circumstances of illness or injury
4. Reactions depend on degree of guilt felt by parents-help
them sort out
5. Assist parents in expressing fears, concerns, grief to
enable them to appropriately support child
6. Assist parents to understand sibling' possible
reactions to terminally ill child
Guilt- believing they caused the problem or
illness
Jealousy- wanting equal attention from parents
Anger- feelings of being left behind
7. Support, enhance parent-child communication,
enhance parents' ability to support child
8. Refer to parent, family support groups- not alone,
help focus, open communication, provide
information
Nursing Care
Nurse
Nurse needs to care for self.