Peds Hematologic/Immunologic Dysfunction Flashcards
skin examination for children with hematologic dysfunction
pallor, petechiae, bruising
anemia
-Decrease in number of RBCs or hemoglobin (Hgb) which decreases the oxygen-carrying capacity of blood
Anemia with reduced Hgb concentration may be caused by
a dietary depletion of iron
s/s of anemia
lack of energy, easy fatigability, and pallor
Aplastic anemia
is a condition that occurs when your body stops producing enough new blood cells.
treatment for aplastic anemia
medications, blood transfusions or a stem cell transplant, also known as a bone marrow transplant.
red cell aplasia (PRCA) or erythroblastopenia
a type of anemia affecting the precursors to red blood cells but not to white blood cells. The bone marrow CEASES to produce red blood cells.
Immune thrombocytopenic purpura (ITP)
a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. People with the disease have too few platelets in the blood.
Disseminated intravascular coagulation (DIC)
a rare, life-threatening condition. DIC causes your blood to clot excessively. As a result, blood clots may reduce blood flow and block blood from reaching bodily organs.
treatment for anemia
-Transfusion after hemorrhage if needed
-Nutritional intervention for deficiency anemias
supportive care for anemia
-Intravenous (IV) fluids to replace intravascular volume
-Oxygen therapy
-Bed rest
iron deficiency anemia more common in
toddlers between 12 to 36 months and during the growth spurt in adolescence
Abnormal Hgb levels are common in what racial or ethnic groups
Southeast Asians, Africans , and Mediterranean
Hemoccult test
a lab test for hidden blood in the stools; tests for chronic intestinal bleeding
Decrease tissue oxygen needs:
Signs of exertions
tachycardia, palpitations, tachypnea, dyspnea, shortness of breath, hyperpnea (increased depth and rate of breathing), dizziness, lightheadedness, diaphoresis, and change in skin color. The child looks fatigued
Explaining Blood Components to Children: RBCs
Carry the oxygen you breathe from your lungs to all parts of your body.
Explaining Blood Components to Children: WBCs
Help keep germs from causing infection.
Explaining Blood Components to Children: platelets
Small parts of cells that help make bleeding stop by forming a clot (or scab) over the hurt area.
Explaining Blood Components to Children: Plasma
The liquid portion of blood, which has clotting factors that help make bleeding stop.
What is iron deficiency anemia caused by?
inadequate supply of dietary iron
Milk babies
overweight infant because of excessive milk ingestion
2 reasons become anemic: milk (poor source of iron)
increased fecal loss
liquid preparation of iron may
temporarily stain teeth; brush teeth after admin to lessen staining
iron-rich foods
iron-fortified cereals
lean meat, poultry, and fish
tofu
egg yolks
beans
raisins
what helps iron to get absorbed
Serve fruits and vegetables high in vitamin C or a glass of orange juice at mealtimes
Don’t give cow’s milk to babies under
1 year old
Sickle Cell Anemia
-Partial or complete replacement of normal Hgb with abnormal HbS
-Defect is inherited
sickle cell anemia onset
Newborns with SCA are generally asymptomatic because of the protective effect from the mother, Rapidly decreases during the first year, then child manifests symptoms
In areas of world where malaria is common, individuals with sickle cell trait tend to have
survival advantage over those without trait
Clinical features of sickle cell anemia
-Obstruction caused by sickled RBCs
-The vascular inflammation
-Increased RBC destruction
-Abnormal adhesion, entanglement, and meshing of rigid sickle-shaped cells
-Local hypoxia
-Cellular death
Dactylitis
inflammation of a digit (either finger or toe) . The affected fingers and toes swell up into a sausage shape and can become painful.
-found in patients with sickle cell anemia
Priapism
prolonged erection of the penis, persistent erection continues hours beyond or isn’t caused by sexual stimulation, usually painful
-found in patients with sickle cell anemia
management for sickle cell anemia
-Prevent sickling
-Rest and minimize energy expenditure
-HYDRATION and OXYGENATION
-Electrolyte replacement
-Analgesia (no aspirin)
-Blood replacement
-Antibiotics
-PAIN MANAGEMENT
what is the leading cause of death in young children with sickle cell
bacterial infection because these patients are immunocompromised
what is the usual life span of a patient with sickle cell
into the fifth decade
why is penicillin given prophylactically for patients with sickle cell
it significantly reduces the risk for pneumococcal infection in children with SCD; Started at 2 months of age
what are things that can cause a sickle cell crisis
-Anything that increases body’s need for oxygen or alters transport of oxygen
-Trauma
-Infection, fever
-Physical and emotional stress
-Increased blood viscosity caused by dehydration
-Hypoxia
Vaso-Occlusive Crisis (VOC)
“painful episode”; Characterized by ischemia causing mild to severe pain, Lasts from minutes to days or longer
Vaso-Occlusive Crisis (VOC) treatment
-hydration
-oxygenation
-pain management
Sequestration Crisis
-Pooling of a large amount of blood
-Usually in the spleen and infrequently in the liver
what does Sequestration Crisis cause
decreased blood volume and ultimately shock
Therapeutic Management of Sickle Cell Crisis
-Rest to minimize energy loss
-Hydration through oral or IV therapy
-Electrolyte replacement
-Analgesia for pain (children tend to be undermedicated)
-Blood replacement for anemia
-Antibiotics for infection
-Monitoring of reticulocyte count regularly to evaluate bone marrow function
-Blood transfusion: If given early in crisis, may reduce ischemia
analgesia for pain used for children in a sickle cell crisis
-mild to moderate pain: NSAID (ibuprofen) acetaminophen
-severe pain - opioids (morphine, oxycodone admin either IV or po)
-given around the clock; PCA
hemophilia
An X-linked recessive hereditary bleeding disorders that result from deficiencies of specific clotting factors
Diagnostic Evaluation of Hemophilia
-History of bleeding episodes (overt prolonged bleeding, hemarthrosis, ecchymosis)
-X-linked inheritance
lab findings for hemophilia
-Low levels of factor VIII or IX
-prolonged partial thromboplastin time (PTT)
-Normal: Platelet count, parathormone level, and fibrinogen level
Hemarthrosis
bleeding into the joint cavities
Ecchymosis
bruising
Therapeutic Management of Hemophilia
-Replace missing clotting factors (Factor VIII)
-Desmopressin (DDAVP)
Desmopressin (DDAVP) for hemophilia
a synthetic vasopressin that causes two to four times increase in factor VIII activity
Prognosis of Hemophilia
Mild to moderate: patients live near-normal lives, no cure, control the symptoms and limit joint damage
treatment options for hemophilia
uGene therapy
uA working copy of the factor VIII gene is introduced
care management for hemophilia: prevent bleeding
-provide safe environment
-dental hygiene (soften toothbrush in warm water, sponge tipped toothbrush)
appropriate sports for children with hemophilia
swimming, walking, jogging, tennis, golf, fishing, bowling
Recognize and control bleeding for hemophilia by
using RICE
Epistaxis
nosebleed
Recurrent or severe episodes of nose bleeds may indicate what underlying disease
Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia)
Care Management of Epistaxis
-Remain calm; keep child calm
-Bleeding usually stops within 10 minutes after nasal pressure
-Have child sit up and lean forward
-Apply pressure to the soft lower part of the nose
HIV/AIDS
Virus that destroys the immune system that should protect the body from diseases.
HIV/AIDS transmission
-sexual acts, blood transfusions, used hypodermic needles, or from mother to child during birth, breast milk
what is given to HIV infected mothers to prevent transmission of HIV to the infant
HAART - Highly Active Anti Retroviral Therapy
HIV takes over
CD4+T lymphocytes; CD4+T cell count drops, increasing risk of infections
manifestations of HIV/AIDS
-Malnutrition, short stature, cardiomyopathy
-Enlarged lymph nodes and spleen, oral candidiasis
diagnosis for infants with HIV
-Infants born to HIV + mothers will test positive
-Early testing using recombinase polymerase amplification (RPA)
Care management for HIV/AIDS
-Education (transmission, control, standard precautions)
-Prevention counseling for adolescents
-Pain management
-Look at psychosocial aspects
-Confidentiality
SCID (Severe Combined Immunodeficiency Disease)
Absence of both humoral and cell-mediated immunity.
most common manifestation of SCID (Severe Combined Immunodeficiency Disease)
susceptibility to infection early in life, most often in the first months
SCID (Severe Combined Immunodeficiency Disease) is characterized by
-chronic infections, failure to completely recover from infections, frequent reinfections, and infection with unusual agents.
-Failure to thrive is a consequence of the persistent illness.
treatment for SCID (Severe Combined Immunodeficiency Disease)
if diagnosed with the first 3 months of life - bone marrow transplant
IVIG provides passive immunity until transplant performed
Blood transfusion
-PRBCs, platelets
-Be alert to transfusion reactions
-Standard guidelines for all transfusion monitoring
-Use infusion pump
Apheresis
-Removal of blood, separation of components, reinfuse portion
-Used to remove and save platelets from healthy donors
