1
Q
RBCs at birth
A
- high level of erythropoietin
- elevation of # of RBCs
2
Q
erythropoietin
A
hormone secreted by the kidneys that increases the rate of RBCs production in response to drop in O2
3
Q
When breathing starts…
A
increased level of O2 which then starts to decrease erythropoietin
4
Q
2-3 mths, levels of RBCs…
A
continue to drop
-increased risk of developing anemia
5
Q
Adult levels of RBCs reached…
A
adolescence
6
Q
Adolescent boys…
A
have higher RBCs then girls
7
Q
Norm Adult male RBC
A
4.6-5.9 mil
8
Q
Norm Adult female RBC
A
4.2-5.4 mil
9
Q
Norm Children RBC
A
4.6-4.8 mil
10
Q
WBCs at birth
A
count is the highest
-values begin to decline after 12 hours of life and continue for the 1st week of life
11
Q
1 wk of age, WBC..
A
begin to stabilize and remain steady until 1 year of age
After that, WBC slowly decreases until the adult value are reached in adolescence
12
Q
Pediatric Differences in Platelets
A
- levels are lower in NB than older children and adults
- levels of many clotting factors are also lower in infants
- VIt K is required for synthesis of several clotting factors….prophylactic inj of vit K at birth
13
Q
Iron Deficiency Anemia
A
- most prevalent nutritional disorder and most common mineral disturbance in the US
- need iron to synthesize hemoglobin
- iron is lost in stool so need adequate intake
14
Q
Children 12-26 mths at risk for iron def anemia…
A
due to cows milk being main staple in this age group
15
Q
FT infants exhaust iron stores by…
A
6 months
16
Q
PT infants exhaust iron stores by…
A
3 months
17
Q
Adolescents and Iron def anemia?
A
rapid growth and poor eating habits
18
Q
Symptoms of Iron Def Anemia
A
- pallor
- fatigue
- irritability
- loss of appetite
- decreased physical activity
19
Q
Prevention of Iron Def Anemia
A
- family education
- PT infants: use iron supplement at 6-8 wks, continue to age 1
- Formula infants: use iron fortified formula and cereals
- BF infants: after 6 mths, use iron fortified cereals and iron rich foods. some may need iron replacement
- diet education for teenagers, esp females
20
Q
Iron administration
A
Iron 4-6 mg/kg for 3 months
- ferrous sulfate better absorbed
- give in 3 divided doses between meals when gastric aids are highest
- also give with juice (vit C, ascorbic acid)
21
Q
Iron Side Effects
A
- liquid iron can stain teeth, use straw or dropper towards back of mouth
- vomiting and diarrhea may occur, then give with meals and/or dose reduced then gradually increase
- stools will turn a tarry green color
22
Q
Sickle Cell Dz
A
- autosomal-recessive disorder affecting normal RBC development
- 25% chance with both parents carriers
- AA: 1 in 12 blks are carriers, hispanics, west africans
- Replacement of normal hemoglobin with Hgb S
23
Q
Sickled RBCs
A
- rigid, inflexible, can occlude small vessels
- increased destruction, life expectancy in 10-20 days
- vascular occlusion=tissue ischemia and organ damage
24
Q
Sickling causes…
A
- hemolytic anemia
- organ damage
25
Hemolytic anemia
- pallor
- fatigue
- jaundice
- irritability
26
Organ damage
- enlarged spleen
- enlarged liver
- renal insufficiency
- gallstones
- priapism
- abdominal pain
- skin ulcers
27
Sickle Cell and Spleen..
-splenic sequestration from trapping of blood in spleen
28
Spleenectomy
- severely compromised immune systems leading to increased rate of infections
- bacterial infections are #1 cause of death in young children
29
Causes of Sickle Cell Crisis
- infections
- dehydration
- acidosis
- physical exertion
- stress
- exposure to cold
- anything that increases body's need for oxygen or alters transport of oxygen
30
Manifestations of Sickle Cell
- depends on the severity of dz
- usually not manifested in first 5-6 mths of life
- anemia and fatigue
- infections and fever
- acute pain with decreased ROM
- jaundice
- hematuria
- splenomegaly
- hepatomegaly
- priapism
31
Complications of Sickle Cell
- CVA
- Acute Chest syndrome
- hand-foot syndrome (dactylitis)
- blindness
- multiple organ failure
- avascular necrosis
32
Management of Sickle Cell
-prevent complications, primary prevention
- NB screening
- folic acid
- hydration
- vaccinate
- prophylaxis with PCN or Amoxil until age 5
- annual transcranial doppler for 2-16 yr olds
- assessment for G/D delays
- parent teaching with focus on nutritional and fluid needs plus S/S of infection
- genetic counseling
33
If doppler results indicate stroke risk...
regular transfusions
34
Management of Crisis
- BR with BRP-rest
- hydration-oral and IV
- electrolyte replacement-metabolic acidosis
- pain control
- oxygen
- transfusions
- antipyretics
- antibiotics
35
Parental Education
- seek early intervention for problems
- adequate hydration, specify amount of intake
- avoid hot weather
- watch for signs of reduced intake, decrease UOP, increased thirst
- Prevent infection, immunizations esp flu, pneumo, mening, PCN
- seek early intervention for problems such as fever
- avoid high altitude or non-pressurized air flights
36
Hemophilia A
"Classic hemophilia"
- deficiency of factor VII
- X-linked recessive disorder
- Amounts for 80% of cases of hemophilia
- occurence 1 in 5000 males
37
Hemophilia A B
less severe form
- christmas disease
- caused by deficiency of factor IX
- accounts for 15% of cases of hemophilia
38
Pathophys of Hemophilia
- based on too little antihemophilic factor (AHF)
- AHF is produced in the liver and is needed to form thromboplastin
- Lower amounts of AHF directly relate to the severity of disease.
- bleeding can occur anywhere including skin, muscle, joints, GI, intracranial
39
Diagnosis of Hemophilia
- hx of bleeding episodes
- can be diagnosed through amniocentesis
- genetic testing of family members to identify carriers
- lab findings: low factor VIII levels and prolonged partial thromboplastin time (PTT)
- the platelet count, prothrombin time, and fibrinogen level are all normal
40
Clinical Manifestations of Hemophilia
- bleeding following circumcision
- prolonged bleeding at umbilicus
- slow, persistent and prolonged bleeding
- joint tenderness with swelling, warmth, and pain
- epistaxis
- bruising
- hematuria
- pallor
41
Safety/Precaution of Hemophilia
- teach family early recognition of a bleeding episode
- teach family how to manage bleeds - RICE
- appropriate activites to prevent accidents
- avoid IM injections and heel/finger sticks
- med alert
- provide support
42
Ultimate goal of medical management for hemophilia
prevent bleeding, particularly into joints
may lead to crippling deformities
43
Milder cases of hemophilia may respond to...
DDVAP, desmopressin acetate
44
Factor VIII concentrate replacement
to be started immediately after a bleeding episode for approx. 3 to 14 days depending upon injury
or short term prophylaxis for 4-8 wks
45
Prophylactic therapy for hemophilia
- prevents bleeding and joint destruction
- very expensive
- complications from long term catheter
- given on a schedule of 3x per week
- does not revere damage but can slow progression and improve quality of life
46
Medical Management for hemophilia
- NSAIDs help, but may interfere with platelet function
- Corticosteroids
- if bleeding, RICE and immobilize affected joint
- proper administration of Factor VIII or DDVAP
- ROM only after bleeding stops to prevent contractures
- analgesics
- avoid obesity to minimize joint stress
- PT to promote safe, age appropriate exercises to strengthen muscles and joints
47
Immune (idiopathic) thrombocytopenia purpura (ITP)
most frequently occurring thrombocytopenia of childhood
most freq occuring between 2-10 years old
acquired hemorrhagic disorder characterized by:
- thrombocytopenia
- purpura
- normal bone marrow
-chronic if over 6 mths duration (rare)...acute in 60-80 percent of cases
48
Probable autoimmune etiology
caused by antibodies that bind to platelet membranes, this can lead to splenic destruction of platelets
may follow viral infections such as measles, mumps, chickenpox, rubella, URI, parvovirus
49
Diagnosis of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)
- clinical findings
- CBC
- platelet count less than 20k
- bleeding time prolonged
- stool occult blood
50
Symptoms of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)
- rapid onset
- easy bruising esp over jt
- purpura
- petechiae
- epistaxis
- bleeding gums
- hematuria
- melena
- menorrhagia
- hepatosplenomegaly
51
thrombocytopenia
excessive platelet destruction
52
purpura
petechial discoloration of the skin
53
Management of Immune (Idiopathic) Thrombocytopenia Purpura (ITP)
-milder forms: supported with activity restrictions and monitoring
more severe:
1. methylprednisolone IV
2. IVIG
3. Anti-D immunoglobulin
- platelet transfusions
- splenectomy in cases lasting one year or longer
54
Pediatric Cancer Differences
- immune system less mature, does not respond well to invading substances
- cells grow quickly in children
- adoptosis less developed in children
- adults: cancer often due to dietary, lifestyle, prolonged exposure
children: continuing presence of fetal cells in small children related to some cancers
55
Most common childhood cancer
leukemia followed by brain and CNS tumors
56
Signs of Childhood Cancer
C: continual, unexpected weight loss
H: headaches often with vomiting at night or early morning
I: increased swelling, jt pain
L: lump or mass in abdomen, neck, chest, pelvis, or armpits
D: development of excessive bruising, bleeding, or rash
C: constant infections
A: A whitish color behind the pupil
N: nausea which persist or vomiting without nausea
C: contant tiredness or noticeable paleness
E: eye or vision changes
R: recurrent fevers unknown origin
57
3 categories of childhood cancer etiology
1. external stimuli causing genetic mutations
2. immune system and gene abnormalities
3. chromosomal abnormalities
58
external stimuli causing genetic mutations
radiation, immunosuppressant's for organ transplants
59
immune system and gene abnormalities
viruses can alter immune system, allow cancer to occur
60
Chromosomal abnormalities
reported link between Down syndrome and leukemia
61
Purpose of Diagnostic Tests for cancer
- identify source of cancer (primary site)
- determine if cancer has metastasized
- stage cancer
62
Clinical Therapy for Cancer
- sx
- chemo
- radiation
- biotherapy
- bone marrow/stem cell transplant
- complimentary therapy and traditional medical approach
63
Wilm's tumor
commonly surgically removed
64
Chemo
- damages dividing cells
- can be given IV, PO, or intrathecal
- circulate through bloodstream and damage any dividing cells
- drugs combined for optimum cell cycle destruction with minimum toxic effects
65
DNA in a normal cell...
can repair itself after chemo, a neoplastic cell cannot
66
Chemo Protocol
- plan of tx
| - goal: maximize lethal impact on cells in all stages of activity
67
Nursing implications for chemo administration
- must be chemo certified
- many chemotherapy agents are vesicants
- can cause severe cell damage if even min amts infiltrate surrounding tissues
68
Common SE of Chemo
- bone marrow suppression....infection, bleeding
- N/V...antiemetic before chemo
- anorexia and wt loss
- stomatitis--oral care
- neuro problems
- alopecia
- cushingoid changes...moon face
- mood changes
69
Radiation
- energy destroys DNA molecule and cell
- goal is to irradiate tumor but not health tissue
- may be used in combo with sx and drugs
- curative or palliative
70
Biotherapy
use parts of the boy already programmed to destroy cells and applies them to cancer cells
most are new or still under investigation
- antibodies specific to cancer
- vaccines that help body fight cancer
- interferon
- TNF --tumor necrosis factor
- gene therapy
71
Bone Marrow/Stem Cell Transplant
goal: kill cancer with chemo or radiation then resupply the body with stem cells
- tx of choice for some cancers that relapse
- sources of stem cells: childs bone marrow, compatible donor, cord blood
72
Cell Cycle Specific Agents
- antimetabolites
- alkaloids
- misc. phase activity
73
Cell Cycle Nonspecific Agents
- alkylating agents
- antibiotics
- nitrosureas
- hormones
74
Colony-stimulating factors
- epoetin alfa
- neupogen
- neumega
75
Nursing care for cancer
- ensure optimal nutritional intake
- admin meds
- manage SE
- ensure hydration
- prevent and tx infections
- manage pain
- psychosocial support
- discharge planning
76
Leukemia
most common form of childhood cancer under 14 years old
abnormal proliferation of WBCs
related to chromosomal defects and exposure to radiation
77
two main types of leukemia
1. acute lymphoblastic leukemia (more common)
| 2. acute myelogenous leukemia
78
Pathophys of Leukemia
- stem cells in bone marrow produce large number of WBCs
- WBCs are immature and do not fx normally
- proliferate rapidly by cloning instead of mitosis and replace normal WBCs
- risk for infection increases
- RBC and platelets are replaced by malignant WBCs
79
Results of Leukemia...
- anemia
- thrombocytopenia
- immunosuppression
- abnormal bleeding
80
Clinical Manifestations
- fever
- anemia
- Hepatospleenomegaly
- Lymphadenopathy
- CNS involvement, HA, V, papilledema
- immunosuppression...fever, infection, poor wound healing
- decreased platelets...bruising, petechiae, purpura, hematuria, epistaxis, tarry stool, frank bleeding
- wt loss and anorexia
81
Chemo Four Phases for Leukemia
1. induction: max cell death, remission after 3-4 wks
2. consolidation: CNS prophylaxis, prevent spread to brain, given with radiation
3. delayed intensification: additional drugs to target surviing leukemic cells
4. maintenance: may continue for 2-3 yrs
82
Prognosis for Leukemia
- generally better than in the past
- favorable factors...2 to 10 onset, initial HgB less than 10, rapid response to chemo, WBCs less than 50,000 ***most important indicator
- 10 percent relapse within 1 year after tx
83
Nursing Care for Leukemia
- prevent infection
- skin and mouth care
- attention to renal fx
- central line care
- RBC, platelet admin
- chemo admin
- monitor IV site for extravasation
- psychosocial
84
Hodgkin's Disease
- disorder of lymphoid system
- arises from single lymph nose or group of nodes
- leak occurrence in adolescent boys
- possible genetic link
- infectious agent or environmental hazard link
- main symptom is nontender, firm lymphadenopathy (**supraclavicular cervical or mediastinal)
85
Clinical Manifestations of Hodking's Dz
main symptom: non-tender, firm lymph nodes
- usual location: supraclavicular or cervical nodes
- others seen with aggressive: fever, night sweats, wt loss, mediastinal mass can cause resp. problems or superior vena cava syndrome
86
Diagnosis of Hodkin's
lymph node biopsy
-Reed-Sternberg cells
87
Therapy for Leukemia
- tests done to determine severity...blood, scans of nodes and lymp system, bone marrow biopsy if advanced suspected
- chemo four drug combo
- radiation
- bone marrow transplant
- 5 year survival rate 80-90 percent
88
Non-Hodgkin's Lymphoma
- malignant tumor of the lymphoreticular system - internal framework of the lymph system
- three subtypes
- third most common form of cancer in kids
- peak incidence is 7-11 boys over girls
89
Clinical Manifestations
- fever, wt loss
- enlarged, nodular lymph nodes
- mediastinum mass
90
Therapy for Non-Hodkin's
- chemo
- raditation not commonly used
- sx, tissue biopsy and tx complications caused by tumor
91
Osteosarcoma
most common bone tumor of childhood
malignant bone tumor
occurs primarily in adolescent boys
located in the metaphysis of the distal femur, proximal tibia or proximal humerus
may be associated with radiation exposure or gene abnormality
92
Osteosarcoma symptoms
pain
swelling
limp
93
Diagnosis of Osteosarcoma
CT
MRI
blood tests
tumor biopsy to confirm diagnosis
94
Tx for Osteosarcoma
- sx and chemo
- amputation sx
- limb-salvage surgery--insert internal prosthesis
- aggressive chemo: started before sx to shrink tumor also after sx to prevent spread
- metastasis is common
- physical rehab
95
Wilms' Tumor (nephroblastoma)
- intrarenal tumor (kidney)
- common abdominal tumor
- most common age: 41-47 mths of age
- associated with congenital anomalies
- tumor suppressor gene may be missing
- tumor grows rapidly--doubles its size in 11-13 days
- chemo is 90 percent survival
96
Clinical manifestations of Nephroblastoma
- usually asymptomatic, mother may find firm mass during bath
- located to one side of the midline of the abdomen
- bilateral 5-10 per. cases
- HTN may occur due to renin release
- hematuria
97
Dx and Tx of Nephroblastoma
- abdominal US, CT, CBC, renal fx tests...BUN, Creatinie
- Sx to remove affected kidney, look for metastasis
- chemo with or without raditation
- no palpation of tumor, may spread
98
Neuroblastoma
- smooth, hard, nontender mass
- originates in SNS
- usually diagnosed less than 5 years age
- best prognosis less than 1 year of age
- survival 90 per. stage 1 or 2, decrease 22 per for stage 4
99
common location for neuroblastoma
abdomen, thoracic, a`drenal, cervical
100
Etiology and Dx tests for neuroblastoma
- cause unknown
- environmental factors, prenatal drug exposure
- canada, increase in folate in flour may have led to decrease incidence in neuroblastomas
- dx: biopsy of tumor for initial dx, bone marrow biopsy to evaluate metastasis,
***dx is uaully made after metastasisis occurred
101
Clinical Manifestations of Neuroblastoma
- location of mass determines symptoms
- labs: increased catecholamines in urine and blood (VMA and HVA) used for dx
-CBC with diff--thrombocytopenia, anemia, WBCs may be elevated or decreased
102
Clinical Therapy and Tx for Neuroblastoma
- stage of tumor determines tx
- surgical removal of mass
- follow with chemo, multiple drugs used
- stem cell transplantation for advanced dz
- radiation often used
- best response to tx under age 1
103
Nursing Management of Neuroblastoma
Assess presenting site of tumor--No palpation!!!!
Assess vital signs,
bowel and bladder function
Monitor weight, height
Observe gait
Postoperative care
Administer medications
Child and family teaching
Psychosocial assessment
Peds Exam 2
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