week 5: caring for the child and family w hematological and immunological dysfunction Flashcards
Prodromal phase
it’s the beginning of the disease before you start showing symptoms
What is an indication that a pt needs to increase their pancreatic enzymes in the treatment of CF?
Pt stools noted to be greasy/fatty
The ability to resist invading organisms in children may be affected by cardiac abnormalities
true
what to look into for hematologic and immunologic dysfunction
- complete CBC
- history and physical exam
- comments by parent regarding child’s lack of energy
- food diary of poor iron sources
- frequent infections
- bleeding
Lethargic
Recurring Infection
Pale
CBC - extremely valuable to know in particular
- Would likely show: decreased RBC, hematocrit, mean RBC
Family history, vegetarian (poor iron), frequent bruising, more fatigue (with low iron)
If platelets are low they will bleed more, possible frequent nosebleeds, petechia (idk how to spell it)
what is anemia
- most common hematologic disorder of childhood
- decrease in # of RBCs and/or hemoglobin (Hgb) concentration below normal
- decreased oxygen-carrying capacity of blood
When do iron stores normally decrease
6 months, so at this time we introduce iron nutrient foods, cereals, formulas, etc.
anemia classified according too
- etiology and physiology: RBC and/or Hgb depletion
- morphology: characteristics changes in RBC size, shape, and/or colour
Is there a issue making RBCs?
Are the RBC size macro? Normal?
Looking at is it sickle cell shape? Normal?
Neonate anemia: prematurity, any hereditary disorders, infection
Older children: iron needs increase, diet
Is pt pale? Yellowing of the eyes?
Supplement with iron, possible IV fluids.
therapeutic management for anemia
- physical exam and diagnostics
- treat underlying cause: transfusion after hemorrhage if needed, nutritional intervention for deficiency anemias
- supportive care: IV fluids to replace intravascular volume, o2, bed rest
nursing care management anemia
- prepare child and family for lab tests
- decease o2 demands
- prevent complications
- support/educate family
iron deficiency anemia
- caused by inadequate supply of dietary iron
generally preventable: - iron-fortified cereals and formulas for infants
- iron supplementation for breastfed infants beginning at 4 months for term
- special needs of premature infants - supplementation at 2 weeks of age
- adolescents at risk bc of rapid growth and poor eating habits
- avoiding cow’s milk until age 12 mo and limit to no more than 600 mls daily
pathophysiology of iron deficiency anemia
caused by any # of factors
therapeutic management
3mg/kg of iron
increase amount of iron the child receives
Take iron w vitamin C
Stool colour changes (dark green/black)
Emphasize importance of iron in diet
Increase fluids because it can cause constipation
what is sickle cell anemia
group of inherited RBC disorders “hemoglobinopathies”
- normal adult Hgb is partly or completely replaced by abnormal Hgb
- most commonly affects black ppl - 9% incidence in Canada
Adaptation to prevent the body against malaria
pathophysiology on sickle cell anemia
- obstruction of sickled RBCs
- vascular inflammation
- increased RBC destruction
- abnormal adhesion + inflammatory process = vasoconstriction
- results in absence of blood flow to tissues causing local hypoxia, tissue ischemia, and infarction
clinical manifestations of sickle cell
- vary in severity and frequency
periods of exacerbation “crises” - vaso-occlusive crisis
- stroke
- chest syndrome
- acute splenic sequestration
- hyperhemolytic
- infection
management of sick cell crisis
- rest
- hydration
- o2
- electrolyte replacement
- pain management
- blood transfusions
- antibiotics
- education/support
What test determines lots of genetic abnormalities and is included in the newborn screening
Hydration: want to prevent blood from becoming clumpy
What should be included in teaching a mother of a 9 month old about administering liquid iron:
tarry green colour stool with adequate iron dosage
wait how long to give fortified foods or solids
6 months
What is primary cause of painful crisis experienced by individuals with sickle cell:
Vaso-occlusion
neoplastic disorders
leading cause of death from disease in kids past infancy
almost half of all childhood cancers involve blood or blood-forming organs
leukemia when is it more present
- most common form of childhood cancer
- 3-4 cases per 100,000 caucasian children <15 yrs old
- more frequent in males >1 yr old
- peak onset btwn 2 and 5 yrs of age
- survivability
Lots of long term complications that can come after leukemia treatment (95% survival rate with research but comes with other issues)
what is leukemia
- a broad group of malignant diseases of bone marrow and lymphatic system: acute lymphoid leukemia (ALL), acute nonlymphoid (myelogenous) leukemia (ANLL or AML), stem cell or blast cell leukemia
- complex disease w varying heterogeneity
- classifications are complex
Prognosis different depending on age and type.
pathophysiology of leukemia
- is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body
- liver and spleen are most severely affected organs
- although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count
- cellular destruction takes place by infiltration and subsequent competition for metabolic elements
symptoms of leukemia
- fever, fatigue
- aching bones or joints
- headaches
- skin rashes
- swollen glands (lymph nodes)
- frequent infections
- unexplained wt loss
- bleeding or swollen gums
- enlarged spleen or liver, or a feeling of abdominal fullness
- slow-healing cuts, nosebleeds, or frequent bruises
diagnostic evaluation of leukemia
- based on history, physical manifestations
- peripheral blood smear: immature leukocytes, frequently low blood counts
- bone marrow aspiration or biopsy
- lumbar puncture to evaluate CNS involvement/disease
prognosis of leukemia
identified thru:
- initial WBC count
- age
- type of cell involved
- cytogenetics
- immunophenotype
- response to induction therapy
if relapse after transplant: dismal prognosis
*females have a more positive prognosis
therapeutic management of leukemia
- chemo agents: use precautions in handling and administering
- cranial irradiation (rarely but in some cases)
- may require transfusions
- normally, 4 phases of treatment
4 phases of therapy for leukemia
induction therapy: 4-6 wks
- CNS prophylactic therapy: intrathecal chemo
- intensification (consolidation/post remission) therapy: to eradicate residual leukemic cells and prevent resistant leukemic clones
- maintenance therapy: to preserve remission
febrile neutropenia for kids w leukemia
- absolute neutrophil count <0.5
- fever
management
- travel to ER immediately
- admitted to hospital
- start IV antibiotics - Pip/Tazo
- min 48 hrs in hospital: if no fever within 48 hrs can be discharged, once 48 hr period is up must go 24 hrs without a fever to be discharged, if cultures positive 10-14 days of IV antibiotics
If have a fever - it IS life-threatening because they have no immune system and can become septic extremely quickly
CBC, see if they are neutropenic, differential, blood cultures
If no fever in 48hrs then they can go home, and cultures are negative
Why is neutropenic a complication
have a weak immune sys, kicks out both bad AND healthy cells
caring for the child receiving chemo
7 days is the patient cytotoxic
Double flush after child uses toilet
Trained to be chemo certified
Nitrile gloves
Bodily fluids of chemo are cytotoxic
what is the most common type of leukemia
ALL (acute lymphoblastic leukemia)
what does blood cancer affect
WBCs
what does low WBC indicate
cancer
leukemia prognosis overall
long treatment, but good prognosis
what is the induction phase of leukemia treatment
kills leukemic cells and puts disease into remission (lumbar punctures-did leukemia spread to cerebral spinal fluid and bone marrow aspiration)
if child manages treatment well after first week they can go home
POGO
2 main types of lymphoma
Hodgkin’s disease (15-19 ages) - usually diagnosed at earlier stage of disease
Non-Hodgkin’s lymphoma (younger) - more common, prevalent in <14 yrs
Hodgkin’s disease
Lymph tissue cancer - found in lymph nodes, spleen, liver, bone marrow, and other sites
Take a bit to figure out what type of cancer
They believe a past association with epstein-barr virus
sympt of hodgkin’s disease classification A
- painless swelling of lymph nodes in neck, armpits, groin (swollen glands)
sympt of non-hodgkin’s disease classification B
- painless swelling of lymph nodes in neck, armpits, or groin (swollen glands)
- fatigue
- fever and chills that come and go
- itching all over the body that cannot be explained
- soaking night sweat
- wt loss that cannot be explained
diagnostics for hodgkin’s and non-hodgkins
- clinical manifestations of Hodgkin’s disease
- lymph node biopsy for diagnosis and staging
- chest x-ray - verify presence of mediastinal mass
- bone marrow biopsy
- presence of Reed-Sternberg cells
therapeutic management for hodgkin’s disease
- radiation
- chemo
- prognosis
- nursing care management
non-hodgkins lymphoma
- cancer of lymphoid tissue
- s/s depend on what area is affected and how fast its growing
- presents as enlarged non-tender lymphadenopathy or s/s due to compression of surrounding tissues: new onset wheeze, facial swelling, resp distress, asymm tonsils, acute abdo pain
More symptoms=had it longer and likely a higher stage
non-hodgkin’s lymphoma treatment depends on…
- type of lymphoma
- stage of cancer at time of diagnosis
- age and overall health
- sympt, including wt loss, fever, night sweats
non-hodgkin’s lymphoma common treatments
- chemo is main type
- radiation not often used
- surgery used to biopsy lymph node or do BM aspirate
- another drug, called rituximab (Rituxan), is often used to treat B-cell NHL
POGO
(Pediatric Oncology Group of Ontario) supports finances and education (parents have to stop working for a year)
what do platelets do
Platelets control hemorrhage and help with clotting
what is a main thing w ALL to protect the child from
treatment-protect child from an upper respiratory tract infection
what is anemia defined as
the reduction of RBC’s or Hgb below levels normal for age
what is the main goal in prevention of nutritional anemia
parent education
