Cellular Regulation

Deborah Naccarini, DNP, RN, CNE

NUR 417 – Professional Nursing Care:
Children and Families
Anemia
Physiology
 Explain oxygenation of body tissues

Oxygen diffuses from alveoli into the capillaries

Oxygen binds with hemoglobin in the RBCs

When blood reaches tissues, oxygen is released

Oxygen diffuses from capillaries into cells of tissues

 What happens if there is a disruption of this

process?
 Hypoxia
Anemia - Causes
 Decreased RBC production
 Nutritional anemias are most common
 Deficiencies of iron, Vit B12 or
folic acid
 Thalassemia major
 Increased RBC destruction
 Sickle cell anemia
 Increased RBC loss
 Acute: Hemorrhage
 Chronic: GI bleeding, menorrhagia
Anemia – Priorities of Care

 Activity intolerance
 Decreased cardiac output
 Impaired oral mucous membranes
 Skin integrity
 Health promotion
 Knowledge deficit
 Anemia – Nursing Interventions
 Activity  Decreased cardiac  Impaired oral
Intolerance r/t output r/t ______ mucous
______________  Monitor VS membranes r/t
 Provide oxygen _____
 Monitor breath
 Monitor VS sounds  Assess condition
 Space activities of lips and tongue
 Monitor heart
 Rest periods sounds  Enc use of
mouthwash
 Encourage 8-10  Assess pallor,
hrs of sleep cyanosis,  Provide freq oral
dependent care
 D/C activity if
symptomatic edema  Petroleum-based
 Monitor for ointment to lips
 Smoking cessation
anaphylaxis to Fe  Soft, bland foods
 Implement safety
precautions d/t  4-6 small
dizziness, meals/day
confusion
Sickle Cell Anemia
(SCA)
SCA: Pathophysiology
 Inherited hemolytic disorder

 Causes synthesis of an abnormal form of Hgb in the RBC

 The “S” shape of the Hgb interferes with uptake of oxygen

 This causes the RBC to “sickle” when hypoxic

 Sickled cells are sticky and less flexible

 Cause clotting in the capillaries = Vaso-occlusive crisis (VOC)

 Tissue hypoxia  Ischemia

 Pain, Fever – hallmark symptoms of VOC

SCA: Cause and Incidence
 Autosomal recessive genetic defect
 Most common inherited blood disorder
 At risk populations – those living near the equator
 African descent – 1in 500 births
 Central and South Americans
 Cuba

 Saudi Arabia
 India

 Mediterranean countries (Italy, Greece, Turkey)

SCA/VOC: Complications
 Anemia is always present
 Bone marrow expands in childhood as a compensatory
mechanism which may lead to enlargement of the bones
of the face and skull
 Tachycardia, cardiac murmurs, cardiomegaly
 Thrombosis
 Any organ may be affected
 Primary sites: spleen, lungs, CNS, kidneys
 Infection
 The 12-day life span of the RBC causes engorgement and
malfunction of the spleen, leading to infections
 Bacterial infections – significant cause of mortality and
morbidity
SCA/VOC: Complications
 Acute chest syndrome
 Sickled cells become trapped in the lungs
 Occurs 2-3 days after VOC
 Fever, respiratory distress, infiltrates (atypical bacteria and
viruses)
 Tx: Blood transfusion, antibiotics, bronchodilators, poss
nitric oxide therapy, and mechanical ventilation. May lead to
acute respiratory distress syndrome and death. Early
intervention is key.
 Pulmonary HTN
 Elevated pulmonary artery pressures
 Often the cause of death
 Asymptomatic until damage is irreversible
 Need early and frequent screening
 Doppler echocardiography
 CT scan
Nisha
Nisha is a 14-year-old with sickle cell anemia. She lives with her
mother and grandmother in a rural neighborhood. Nisha has
experienced several sickle cell crises, however, they seem to have
become more frequent since she became an adolescent. Nisha is
enjoying her summer break from school. She is active in softball
and enjoys shopping with her girlfriends.

Nisha’s mother brings her to the hematology clinical at the

hospital with complaints of severe generalized pain following a
softball game in which she pitched seven innings. She is admitted
to the medical pediatric unit. Her vital signs are: temperature
37.6° C (99.7°F), pulse 110, respiratory rate 30, and blood
pressure 96/70. She weighs 110 lb. Her complete blood count
reveals Hgb 9 g/dL, Hct 24%, WBC 12,000 cells/mm3, platelet
count 140,000 cells/mm3. Her oxygen saturation is 89%.
Discuss your impressions of
Nisha’s clinical manifestations.
 Nisha  Others
 Severe generalized  Pallor
pain  Fatigue
 Low grade fever  Jaundice
 Tachycardia  Irritability
 Tachypnea/SOB
 Decreased O2 sat
What is a sickle cell (vaso-
occlusive) crisis?
 Obstruction of the microcirculation (capillaries)
 Obstruction causes vasospasm and stops blood
flow
 Ischemia, Infarction, Severe pain
 Lasts 4-6 days
 Infarction
 Extremities
 Priapism
 Abdomen
 Bones
 Integumentary
What are Nisha’s
precipitating factors?
 Common during summer months, when active outdoors,
d/t risk for dehydration
 Teen girl who is probably experiencing menses, another
physiologic stressor
 H/H = anemia
 Elevated temp may indicate infection, dehydration, or
vaso-occlusive crisis
 Children with SCA experience more frequent infections
than other children
What other assessment data
would be helpful for the nurse
to have to plan Nisha’s care?
 Pain assessment
 Date of last hospitalization
 Number of VOC Nisha has experienced
 Fluid intake
 Urinary output
 Neurological assessment
 Breath sounds
 Heart sounds
 Skin assessment
 CXR
Healthcare Provider Orders
The health care provider prescribes the following for Nisha:
 Vital signs q4h. Notify health care provider of temperature >38°C
(100.4° F)
 Regular diet
 Strict bedrest
 Complete blood count with differential in the morning
 Urine for urinalysis and culture and sensitivity
 Chest x-ray
 IV fluids of D5 ½ NS at 175 ml/hour
 PCA morphine sulfate 1.5 mg continuous and 1 mg every 8 minutes PCA
dose
 Acetaminophen 650 mg q4h PO for temperature >38°C (100.4° F)
 Oxygen 2 lpm/NC, titrating to maintain oxygen saturation >94%
Discuss the prescriptions and
if the nurse should question
any of them.
 VOC Standards of care are:
Rehydration – Reoxygenation – Pain management
 IVF D5 0.45% NS at 175 ml/hr
 O2 at 2 lpm/NC, titrate to keep sats >94%
 MSO4 PCA: basal 1.5 mg/hr; 1 mg q8min
 Strict bedrest
 Acetaminophen 650 mg q4h T>38C
What are the priority nursing
diagnoses?
 Acute pain r/t
 Tissue ischemia
 Ineffective tissue perfusion r/t
 Clumping of sickled cells in the vessels
 Risk for infection r/t
 Compromised state (poor splenic function)
 Risk for injury r/t
 CVA and organ damage r/t recurrent crises
 Deficient knowledge r/t
 Predisposing factors for vaso-occlusive crisis
What nursing interventions
would be appropriate in
meeting Nisha’s needs?
 Acute pain  Tissue perfusion
 Assess pain hourly with  Assess O2 sat
age-appropriate tool
 Maintain IV access
 Admin O2
 Teach re PCA  Assess CFT and VS
 Position for comfort  VS q4h and prn
 Move Nisha slowly
 Elevate HOB
 Reassure that MSO4
addiction is uncommon  Encourage use of
 Reassure pain resolves incentive
with rehydration and spirometer
reoxygenation
 Encourage turning,
 Moist heat (K-pad)
coughing, deep
 Assist with ambulation breathing
 Interventions

 Risk for infection  Risk for injury

 VS q4h and prn  Assess heart sounds q8h
 Avoid invasive  Assess neuro status q8h
procedures, including  Stress importance of
Ims
hydration
 Maintain aseptic
 Monitor diagnostic test
technique
results
 Universal precautions
 Follow policies and
 Assess breath sounds procedures for blood
q8h and prn administration
 Enc use of IS
 Administer
antibiotics and
antipyretics, as
ordered
Growth and Development

After 4 days of treatment, Nisha’s IV fluids and

medication are discontinued and her pain assessment
reveals a pain level of 1/10. When the nurse enters
Nisha’s room, Nisha is sitting quietly in a chair at the
bedside and seems sad.
Discuss your impressions of
Nisha’s condition based on her
level of growth and development.
 She is 14
 No friends for 4 days
 Pain is gone – she wants OUT!
 Anxious to get back to normal teen activities
Discuss the teaching priorities for
Nisha prior to her discharge from
the hospital after her crisis is
resolved.
 Assess level of knowledge of Mom and Nisha
 Risk factors for developing infection
 Preventative health
 Medications
 Signs and symptoms of VOC
 Contact phone # to report s/s
 Importance of regular handwashing
 Importance of f/u
Leukemia
Ashlee – A case Study
Ashlee is a 4-year-old preschooler who lives with her
parents and two older siblings in a suburban environment.
She attends preschool five mornings a week and enjoys
playing with her 5-year-old sister and 7-year-old brother.
She is very active and enjoys playing outside, riding her
tricycle, climbing on the family’s jungle gym, and playing
on the swing set. Her vocabulary consists of approximately
1,500 words and she speaks using four- or five-word
sentences. Her parents are very attentive to their children
and spend each weekend doing “family activities.” During
the week, her parents work and Ashlee and her siblings
stay with their grandmother after school. Their
grandmother lives in the same neighborhood. In the
evenings, the family eats together and maintains an
evening schedule that allows for family play time.
Ashlee – A Case Study
During the past 2 months Ashlee has been less active than
usual and has begun taking one or two naps in the
afternoon. Her grandmother and parents think she looks
pale, reasoning that it is because of her high activity level,
until her interest in going outside to play decreases
dramatically. When they take her temperature, it is
elevated so they administer acetaminophen without effect.
At this point they decide to take her to see her
pediatrician. Although the health care provider found
Ashlee’s manifestations consistent with an upper
respiratory infection, the pediatrician is concerned and
decides to admit Ashlee to the hospital to rule out
leukemia.
Ashlee – A Case Study
What symptoms does Ashlee exhibit that might alert her
pediatrician to something more serious than an upper
respiratory infection?

ASHLEE: OTHERS
 Fever  Enlarged lymph nodes
 Fatigue  Lethargy
 Pallor  Anorexia
 Bone or joint pain
 limp or refusal to walk
Ashlee – A Case Study
 Why are these diagnostic tests ordered for Ashlee?
 Labs: CBC, chem panel:
 Evaluate WBC, plt, liver and kidney function
 Bone marrow aspiration:
 Required to confirm the diagnosis and type of
leukemia
 Lumbar puncture:
 Determine CNS involvement (infection)
 Bone scan:
 Determine bone involvement
Ashlee – A Case Study
Discuss the significance of Ashlee’s vital
signs and CBC results.
 T 38°C  Hgb 11g/dL
 HR 120  Hct 31%
 RR 28  RBC 4.6 million/mm3
 BP 100/60  Plt 130,000/mm3
 WBC 4,000 cells/mm3
 Neutrophils 1,600 cells/mm3
 Lymphocytes 400 cells/mm3
 Monocytes 290 cells/mm3
 Eosinophils 120 cells/mm3
 Basophils 30 cells/mm3
Ashlee – A Case Study
Ashlee’s diagnostic tests confirm a diagnosis of acute
lymphocytic leukemia. Compare and contrast the two types
of childhood leukemia.

 Acute Lymphocytic Leukemia

Uncontrolled proliferation of immature lymphoblasts
Saturates the bone marrow, they do not mature and cannot
function as infection-fighting cells.
Longer life-span of immature lymphoblasts leaves the
patient at risk for infection
The saturation of the bone marrow with these immature
lymphoblasts results in decreased ability of the bone marrow
to manufacture RBCs and platelets – placing the patient at
risk for anemia and thrombocytopenia with resultant
bleeding.
Ashlee – A Case Study
Ashlee’s diagnostic tests confirm a diagnosis of acute
lymphocytic leukemia. Compare and contrast the two types
of childhood leukemia.

 Acute Myelocytic Leukemia

Uncontrolled proliferation of immature myeloblasts
The myeloblasts enter the systemic system and are able to
cross the blood-brain barrier
They compete with normal cells in the brain, skin, ovaries,
testes, and other organs, causing premature death of the
normal cells
In addition to problems of increased risk for infection,
anemia and bleeding, solid tumors also form
Ashlee – A Case Study
Ashlee’s mother is at Ashlee’s bedside crying. As you
approach her, she says, “How could God let me little girl
get leukemia? What can I do to make it go away?” How
would your respond to Ashlee’s mother?
 Employ therapeutic communication techniques!
 Remember that anger (blame) and bargaining are two
phases of the grief process
 Answers are not generally expected
 Silence may be the most appropriate response
 Offer to contact a chaplain
 Try to explain that the cause of leukemia in children is
not known, but that it is not the result of anything that
the Mom did or didn’t do.
Ashlee – A Case Study
What are the nursing priorities of care and associated priority
nursing interventions for Ashlee?
 Risk for infection r/t
 decrease in functional WBC secondary to ALL
 Risk for injury (bleeding) r/t
 decreased platelets and RBC secondary to ALL
 Ineffective tissue perfusion r/t
 decreased RBC secondary to ALL
 Risk for imbalanced nutrition: less than body requirements r/t
 nausea secondary to chemotherapy and fatigue secondary to
anemia (ineffective tissue perfusion)
 Fear/anxiety r/t
 Ashlee’s condition, prognosis, hospitalization, and Ashlee’s
level of growth and development and magical thinking
 Deficient knowledge r/t
 Ashlee’s condition, treatment and home care
Ashlee – A Case Study
Discuss the factors that affect Ashlee’s
prognosis.
 Positive Factors  Negative Factors
 Age between 2 and 10  Age <2 or >10 years
years (peak is 2-5 yrs)  Male gender
 Female gender  Elevated WBC
 WBC normal or slightly  AML
below normal
 ALL
Ashlee – A Case Study
Ashlee’s mother expresses concern because Ashlee “has been
potty-trained for 2 years, but she has wet the bed since she has
been in the hospital.” How would you respond to Ashlee’s
mother?
 Chemotherapy requires hyperhydration with IV fluids to ensure
that the chemo is dilute as it enters and is then excreted from
the urinary bladder.
 Children do not normally receive 75-100 ml of fluid/hour at home,
but they will in the hospital
 Intravenous fluids do not elicit the same bladder response as oral
fluids, so children lack the usual acknowledged stimulation to void
 Preschoolers are deep sleepers
 It is common for children to regress during hospitalization
 Suggest:
 Diapering Ashlee at night
 Allows for uninterrupted sleep
 Allows for decreased emotional stress
Ashlee – A Case Study
 Ashlee will undergo chemotherapy for treatment of
ALL. Describe the 3 phases of chemotherapy
treatment.
 Induction
 Consolidation
 Maintenance
Ashlee – A Case Study
Ashlee’s chemotherapy regimen is started and the
oncologist prescribes ondansetron 2.5 mg IV prior to
chemotherapy and the same dose every 4 hours for 24
hours. In addition, she prescribes dexamethasone 16 mg
IV prior to chemotherapy and lorazepam 1 mg IV every 4
hours prn for breakthrough nausea. Discuss these
prescriptions including drug classifications, when
medications should be administered, special
considerations when administering drugs, and safe doses
for Ashlee, who weighs 16.7 kg
Ashlee – A Case Study
Discuss how treatment for leukemia may affect Ashlee’s
growth and development.
 The major complication of chemotherapy is bone
marrow suppression, resulting in risk for infection.
 Interferes with socialization, affecting achievement of
initiative
 Interventions
 interactive play with dolls, stuffed animals, and puppets with
parents and siblings
 Parents and siblings can read to her, helping her vocabulary
and letter and word recognition
 When blood counts rise:
 Invite preschool friends over
 Return to preschool to regain some normalcy
Ashlee – A Case Study
How would you work with Ashlee’s parents to help
prevent complications associated with her growth and
development?
 Teach Ashlee’s parents about normal G&D
 Encourage appropriate activities based on current
laboratory results
 Protect from infection, bleeding, and ineffective tissue
perfusion
 Parents of these children are often overprotective –
consider support groups
Neural Regulation
Neuro Exam – Anatomical diff
 Neuro Exam
 Pediatric differences:
 Cushing’s triad is uncommon in children
 Bradycardia, irregular respirations, increased systolic BP
 Changes in pulse and BP are more important than the direction of
those changes
 Respirations
 Slow and deep RR seen in heavy sleep caused by sedatives, seizures, or
cerebral infections
 Slow and shallow RR seen with sedatives or narcotics
 Hyperventilation (deep and rapid RR) seen with metabolic acidosis,
salicylate poisoning, hepatic coma, Reye syndrome
 Periodic and irregular RR indicates brainstem dysfunction – often precedes
complete apnea
 Pediatric Coma Scale
 Glasgow coma scale
 Quantifies the LOC

 Pediatric criteria
 Takes into account developmental
age for each category of the test
 Eye opening
 Verbal response
 Motor response
Hydrocephalus
 Hydrocephalus
Hydrocephalus
Nursing
Dx Tests Communicating Assessments
hydrocephalus = impaired
absorption of CSF in the
subarachnoid space
Non-communicating
hydrocephalus = obstruction
Treatment of flow of CSF preventing it Nursing Dx
from getting to the
subarachnoid space for
absorption

Nursing
Complications Interventions
Clinical
Manifestations
Hydrocephalus
Frontal bossing and
Sundowning

Prominent veins
Transillumination
 Hydrocephalus

 Ventriculoperitoneal shunt
 Four parts:
 Ventricular catheter
 Pumping chamber or resevoir
 One way pressure valve
 Distal catheter