Nursing management:Hemophila & von-Wilebrand disease -
ANSWERS-•Stop the bleeding-direct pressure/ice; topical hemostatic
meds (thrombin)
•Administer the specific coagulation factor and monitor for allergy
reactions
•Bedrest in pts with hemarthrosis - ice; analgesics (e.g., Tylenol).
Mobilize/ROM when bleeding stops. Weight bearing should be
avoided till all swelling is resolved with muscle strength has returned.
•Monitor life-threatening complications from hemorrhage- until
bleeding stops, then they can ambulate and do ROM exercises
•Monitor hyponatremia from use of desmopressin
•Immunizations- use the smallest gauge needle (25) /apply pressure
and/or ice for 5 min post injection
•When to seek immediate medical attention
•Dental care- Routine dental care/teach proper brushing
•Exercise and athletic participation- avoid contact sports; prophylactic
factor infusions might be given around the activities
•Medications to avoid- ASA; NSAIDs; anticoagulants; use
acetaminophen for pain- cause pt to bleed more
•How to administer the factor replacement
•Wear gloves with household chores
•Plan for invasive procedure
•Medical alert bracelets
,•Psychosocial support- kids may feel social isolation, monitor if life
threatening and they should seek care
Neutropenia - ANSWERS-•A reduction in neutrophils, usually less
than 1-1.5 x 109/L (normal range 2.2 to 7.7 x 109/L)
•Severe neutropenia: ANC (absolute neutrophil count) less than 0.5 x
109/L
•Not a disease, occurs with other conditions or disease
•Neutrophils are granulocytes which plays a major role in immune
system
•Determine - the cause; gradual vs rapid decrease; the degree & the
duration of neutropenia
•Consequences? and what does the nurse needs to know? separated
from public- protect- less than 1 isolation , treat within 1 hour- give
antibiotics right away because they can go into sepsis easily, faster
and longer- greater chance of life threatening complications
Causes of neutropenia - ANSWERS-•Drug therapy (eg., chemo drugs-
affect all blood cells)
•Hematological disorders (eg., leukemia; aplastic anemia..)
•Autoimmune disorders (eg., RA; SLE; autoimmune neutropenia)
•Infections (e.g., hepatitis; HIV; parasitic..)
•Nutritional: deficiencies of vitamin and minerals (eg., vitB12; folate;
copper)
•Others (i.e., severe sepsis; hypersplenism; bone marrow infiltration;
nutritional deficiencies; congenital neutropenia; transfusion reaction;
hemodialysis..)
,CM of neutrophils - ANSWERS-•Decreased WBC or immature
WBCs = increased WBC, blood cancer)
•Neutrophils <1.0 x 109/L
•Classic signs of inflammation may not occur- lack of soldiers to fight
infection
•Presence of a low grade fever n neutropenic patients is of great
significance
•A fever >38°C and a neutrophil count <0.5 × 109/L is a medical
emergency, acute leukemia
Treatment of neutropenia - ANSWERS-•Identify and eliminate the
cause
•Identify infection site (physical exam; x-ray; culture for sputum and
urine; blood cultures; wound/throat swabs..) and causing organism -
start broad spectrum IV antibiotics immediately (within 1 hour) if
febrile- do not wait until results come back
•Hematopoietic growth factors [e.g., Granulocyte colony stimulating
factor (GCSF)]
-Filgrastim
-Pegfilgrastim- measure absolute neutrophil count, should be showing
improvement, requires 2 nurses to check
-They will measure ANC (absolute neutrophil count)
•GM-CSF (Granulocyte-macrophage colony-stimulating factor)
•Keratinocyte growth factor
•Prophylactic medications (antibiotics/antivirals)
, Nursing Management for Neutropenia - ANSWERS-•Diligent
washing/use aseptic technic
•Single protective (reverse) isolation room when neutrophil < 1 x
109/L
•Positive pressure or HEPA (high-efficiency particulate air) filtration
depending on risk
•Monitor S&S of infection and early septic shock
•No uncooked meats, seafood, or eggs
•Do not eat unwashed fresh fruits
•No flowers/plants in patient's room
•Discharge instruction (patient teaching)
-Notify health care providers when experiencing S&S infection - take
temp
-Avoid crowds and people with colds; flue; infection-wear a mask
-Bathe/shower daily and use a moisturizer
-Use soft toothbrush (4X); avoid alcohol based mouth wash- cause
ulcer in mouth
-Do not perform gardening or clean up after pets- signs that say
visitors know not to bring plants in
-Feeding pets is fine but wash hands after
-Wash hands; wash hands; wash hands, gown, glove, mask- pt wears
when they go out but should stay in room as much as possible