Care Of The Child With Hematologic Disorder Flashcards
Which type of anemia…
A specific type of Vitamin B12 deficiency anemia.
Caused by the body’s inability to absorb B12 due to lack of intrinsic factor (an autoimmune condition).
Pernicious Anemia
Folate Deficiency Anemia
Caused by inadequate intake or absorption of folic acid
Which vitamin is Folate…
(vitamin B9)
Nutritional deficiency anemia may come about from a lack of which vitamins/ minerals…
Iron (Most common)
Folic acid B9
B12 = Pernicious Anemia
Exposure to this substance may cause anemia…
Iron
Describe Aplastic anemia…
Acquired as a severe reaction to medication
Describe 2 types of hemolytic anemia….
Sickle cell anemia
Thalassemia
_____ is a genetic blood disorder in which the body makes an abnormal form or inadequate amount of hemoglobin—the protein in red blood cells that carries oxygen.
This leads to destruction of red blood cells and results in anemia.
Thalassemia
Iron deficiency anemia
The body lacks Iron to produce Hemoglobin
SS
Fatigue
Irritability
Tachypnea / Cardia
Pallor
Risk factors…
Blood loss
Inadequate intake
Malabsorption
Premature infants
Iron deficiency anemia
Describe treatment:
Blood Loss….(3)
Inadequate intake…(2)
Malabsorption…(2)
Blood loss
Upper endoscopy/ Colonscopy
Menstruation: Give birth control
Blood transfusion
Inadequate intake:
Increase dietary sources
PO iron supplement
Malabsorption
Increase vitamin C
IV iron infusion
List sources of Iron…
Red meats (easiest to absorb)
Tuna / Salmon
Eggs
Tofu
Enriched grains/ cereal
Dried beans, peas, fruit
Leafy green vegetables
Breast fed infants can begin Iron supplementing around 4-5 months
How much iron daily…
Can take a few months for iron levels to increase
Can cause constipation/GI upset
Avoid administration with…
Liquid Iron has this precaution…
4 - 6 mg daily
Avoid administration with milk / antacids
Liquid iron can stain teeth. Give behind teeth or dilute
Full term infants have maternal iron stores for how long…
6 months of life
If mother has anemia will the infant have proper levels of iron…
Doubtful
Pre-term infants don’t have proper maternal iron stores due to Iron buildup in fetus happens during last 3 months.
Types of risk for infant…
Anemia
Neurocognative delays
Hgb
Hct
Total iron concentration
Total iron binding capacity
Describe in an anemic…
Lower: Hgb,Hct,iron concentration
Higher: Binding capacity
Iron panel & CBC are used for…
Diagnostic of iron deficiency anemia
Body systems most affected by lead poisoning…
NS
Bone marrow
Kidney
Hypochromic & microcytic RBC describe appearance…
Dull color due to lack of hemoglobin
Small in size
Lead poisoning is most prevalent in these ages…
1 - 5 yrs
Lead poisoning may show SS of anemia
Risk factors….
Paint prior 1978
Soil where cars used leaded gasoline <1996
Ceramics, Stained Glass, Old toys / Furniture
Lead pipes
Adopted Children from 3rd world countries
Risk assessment how often for lead poisoning…
6,9,12,18 months
3,4,5,6 years
If risk assessment is positive then draw blood lead levels
MDHHS updated definition of elevated blood lead level from ___ to ____
5 ug/dL to 3.5 ug/dL
Why does lead poisoning cause anemia…
Lead replaces Iron in hemoglobin
SS Anemia (5)
Pallor
Fatigue
Weakness
SOB
Tachycardia
Anemia
Neurological
HA, Inattentive, irritation, hyperactive, speech, hearing, motor skills.
Describe high levels issues…..
GI
Failure to gain weight/weight loss, vomiting, constipation
All associated with lead poisoning.
High levels: encephalopathy, seizures, brain damage
Lead poisoning treatment
- Limit exposure
- Chelation therapy
Begin with lead levels >……
Levels >….. requires hospitalization with IV/IM Chelation
- Educate/ repeat blood levels
Chelation begins at >45
Hospital Chelation IV/IMA >70
Succimer
Therapeutic effect….
Given PO when….
Capsules contains small beads that can be mixed with food.
CANNOT BE GIVEN VIA SYRINGE
Chelation therapy/ Lowers lead level in body
Give PO q8h × 5 days , then q12h × 2 weeks
Chelation therapy
Edetate calcium disoriented
Route….
When used alone or without Dimercaprol can cause…
Use in combination with……
IM / IV 5 DAYS. Poorly absorbed through GI
Cerebral edema
Use in combination with (Dimercaprol & EDTA)
Chelation therapy
Dimercaprol
DRUG OF CHOICE IN SEVERE LEAD TOXICITY (BLL >70)
IM how often…
Contraindicated in this population…
Can cause toxic complex when mixed with ….
IM Q4H
Contraindicated with Peanut Allergy
Toxic complex when mixed with Iron
Autosomal Recessive where RBCs hemoglobin are less effective in carrying oxygen and tend to get stuck together causing blockage of blood flow…
Sickle cell anemia
Normal adult hemoglobin is referred to as HbAA
Describe
HbSS…
HbSC…
HbS….
HbSS = Most Severe Sickle cell anemia
Hbs = beta thalassemia
Describe life span of RBC with HbSS…
10 - 20 days vs 120 (Normal)
Sickle cell anemia
Describe SS
Cardiac / Pulmonary…
Why chronic pain…
Stroke
Hearing / vision loss
Swollen extremities
Increased Liver Enzymes, Failure/ Damage, Splenomegaly, Prone to infection
Chest pain / pneumonia
Chronic pain due to decreased blood flow to tissue
Describe the following lab values with Anemia.
Hemoglobin
Hemocrit
Reticulocyte count
BUN / Creatinine
AST / ALT
Hemoglobin Ioweree
Hemocrit lowered
Reticulocyte count Increased
BUN / Creatinine Increased
AST / ALT Increased
Reticulocyte = Immature RBC
Sickle cell anemia
Describe Fluid support…
Pharmaceutical support…
Non-pharmacologic support…
Oxygen….
Last Resort…
Fluid 1.5x maintenance
Pharmaceutical: Analgesics, Hydroxyurea, Folic Acid
Non-pharm Warm Compress / Child Life specialist
Oxygen Yes
Last Resort: Bone Marrow transplant
Inherited Autosomal Recessive disorder characterized by ineffective production and destruction of RBC…
B-Thalasemia
B- Thalassemia
Inherited Autosomal Recessive disorder characterized by ineffective production and destruction of RBC
3 subcategories based in severity
Minor (trait) mild microcytic anemia ….
Intermedia…..
Major (Cooley anemia)….
Minor (trait) hgb 2 - 3 g/dl less than normal
Intermedia Severe anemia & splenomegaly, frequently blood transfusion
Major (Cooley anemia): Blood transfusions required to sustain life, Iron Removal (Chelation Therapy)
RBC rigid and hemolyze easily
Severe anemia & chronic hypoxia
Describes….
B-thalasemia (Cooleys Anemia)
Cooley anemia B-Thalasemia
RBC destruction, body response is to increase bone marrow expansion (Thickened Bones)
Plateau in growth, pathological features , SS Anemia
Give 2 SS
Frequent nose bleeds
Skeletal deformities
Why can organ damage occur with thalassemia Major (Cooleys anemia)….
Extramedullary erythropoiesis
RBC production outside of bone marrow
Thalassemia Major Cooley Anemia
Hemosiderosis….
Hemochromatosis….
Treatment…
Diet…
Labs: Bilirubin, Iron, Hemoglobin/Hemocrit….
Hemosiderosis: excessive iron due to rapid RBC hemolysis Without Tissue Damage- Leads to Broze skin
Excessive iron supply With Cellular Damage- can compromise cardiac function
Treatment: Chelation therapy
Deferasirox / deferoxamine PO / SubQ
Diet: AVOID IRON Rich Foods & Increase Vitamin C
Bilirubin Increased
Iron Increased
Hemoglobin/crit Lowered
Thalassemia Major
Treatment ….
Cure…
Packed Red Blood Cells - Transfusion x 2 monthly
Splenectomy for noncompliance
Bone marrow transplant
NO CURE
X-linked recessive disorder that results in a deficiency in coagulation factor….
Hemophilia
Hemophilia A
Factor VIII (antihemophilic factor) deficiency aka….
Classic hemophilia
Most common 75%
Hemophilia B
Factor IX (plasma Thromboplastin component) deficiency aka ….
Christmas disease
Hemophilia
Categorize based on severity
Assess for bleeding after trauma
Bleeding common after injuries
Spontaneous bleeding without injuries
Mild
Assess for bleeding after trauma
Mod
Bleeding common after injuries
Severe
Spontaneous bleeding without injuries
Hemophilia
3 hospital interventions…
Stool softeners
No IM injections
No Rectal temp
WEAR Medic Alert Bracelet
Hemarthrosis bleeding into a joint cavity associated with hemophilia
PT needed for ROM exercises
Which type of Analgesics…
RICE…
NSAIDS but no aspirin
Rest
Ice
Compression
Elevated
With Anemia Desmopressin maybe given why…
Increased clotting factors VIII
Von willenbrand
Idiopathic thrombocytopenia Purpura….
Blood disorder caused by an autoimmune response after Viral Infection that causes decreased platelets
Blood disorder caused by an autoimmune response after Viral Infection that causes decreased platelets …
Idiopathic thrombocytopenia Purpura
Clinical Manifestions fir Idiopathic thrombocytopenia Purpura….
Blood disorder caused by an autoimmune response after Viral Infection that causes decreased platelets
Petechiae- Pinpoint Nonblanchable hemorrhages
Purpura: blood collection under skin causes large purple area
Bruising
Bleeding
Antiplatelet Antibody test
Normal range 150,000 - 450,000
CBC used to determine platelet level
Used to diagnose….
Treatment…
Idiopathic thrombocytopenia Purpura
Platelet <100,000
Excessive bleeding occurs at <10,000
Treatment: Most cases resolves spontaneously without complications
Avoid injury / NSAIDS
Critically low <10,000 Treatment
IVIG
Corticosteroids: block autoimmune destruction of platelet
Platelet transfusion/ Splenectomy
Before a blood transfusion
2 RNs are required verification
Obtain VS when…
VS also include this assessment…
Ped blood transfusion has this requirement..
Blood product needs to be completed within _____ from getting blood from bank
Before getting blood product. Checking for fever.
Assess lung sounds for fluid overload
Written in mL not just units
Blood infusion within 4 hrs of pickup
SS of blood transfusion reaction
Fever
Chills
Itching
SOB
Nausea
Hives
Changes in VS
If reaction occurs…
Why may blood bank want you to draw a blood sample…
May want blood returned to test for contaminación
Stop transfusion / Maintain IV with NS
Call provider
Depends on type of reaction- may administer meds (Acetaminophen/ Diphenhydramine)
Draw blood sample to determine existence of Antibodies presence
Hematopoietic Stem Cell Transplant / Bone marrow transplant
Goal: reestablish normal blood cell production
Before stem cells are infused intravenously this procedure happens first…
High doses Chemotherapy and or radiation to rid body of abnormal cells
Describe
Autologous….
Allogenic…
Autologous = Harvested from child’s own bone marrow
Allogenic = Human leukocyte antibody matched donor
HSCT Complications…
Graft vs host allogenic
Failure of transplant
Infection
Impaired growth & fertility
Lung / Heart disease
Necro of bone
2ndary cancers
Graft vs Host disease
T cells from donated stem cells attack recipients cells
Lower degree of HLA matching Higher risk of GVHD
Clinical Manifestions…
Liver failure: jaundice & elevated AST / ALT
Skin Rash: maculopapular rash
GI NVD painful
Graft vs Host disease
T cells from donated stem cells attack recipients cells
Lower degree of HLA matching Higher risk of GVHD
Treatment….Meds….
Tacrolimus Immunosuppression
Steroids
Methotrexate
______a medication commonly used for cancer, autoimmune diseases (like rheumatoid arthritis), and ectopic pregnancy.
It’s a powerful antimetabolite and immunosuppressant
Methotrexate
Labs to monitor before giving methotrexate…
Monitor Lab Values:
CBC: Watch for bone marrow suppression (↓ WBC, RBC, platelets).
Liver function tests (LFTs): Risk of hepatotoxicity (↑ AST, ALT).
Renal function: Monitor BUN/Creatinine—methotrexate is nephrotoxic.
Methotrexate levels (in high-dose therapy)
Methotrexate
PPE considerations…
Administer this medication along with methotrexate to reduce SE
High doses of methotrexate may require ____ to protect normal cells
Use gloves when handling—chemotherapy precautions.
Administer folic acid supplement to reduce side effects (often 1 mg/day).
High-dose methotrexate may require leucovorin rescue to protect normal cells.
Dactylitis….
Associated with Sickle Cell anemia
Infants / Toddlers
Painful swelling of hands / feet due to vaso-occlusion in small bones
Often 1st sign of Sicke Cell in children
Hemosiderosis…
Iron overload, usually from frequent blood transfusion
Priapism…
Painful, prolonged ejection.
Complications of Sickle Cell in older males
Poikilocytosis: Abnormal shapped RBC
Sickle Cell (Anemia)
Target cells (Bullseye)….
Schistocytes (fragments of cells)….
Target cells: Thalassemia, liver disease
Schistocytes: hemolytic anemia
Sickle cell is an Autosomal Recessive.
Describe how the child will get this disease…
Must have 2 copies of disease
1 from each parent
Best snack for child with Thalassemia
PB & rice cake
Small spinach salad
Apple slices & cheddar cheese
Small burger wheat bun
Apple slices & Cheddar
Thalassemia may require frequent blood transfusion & Low Iron Diet
Which is the first line medication for pain in a Sickle Cell crisis…
IV opiates until pain is controlled.
Pain should be prevented rather than treated reactively
Cause
________ Caused by mutations in genes that control the production of hemoglobin chains (alpha or beta chains). It leads to reduced or absent production of one or more globin chains.
_______ Caused by a mutation in the beta-globin gene, producing abnormal hemoglobin called hemoglobin S, which causes red blood cells to sickle (form a crescent shape).
Cause
Thalassemia: Caused by mutations in genes that control the production of hemoglobin chains (alpha or beta chains). It leads to reduced or absent production of one or more globin chains.
Sickle Cell Anemia: Caused by a mutation in the beta-globin gene, producing abnormal hemoglobin called hemoglobin S, which causes red blood cells to sickle (form a crescent shape).
Red Blood Cell Shape
_______ Red blood cells are small, pale, and irregularly shaped.
_________ Red blood cells are crescent or sickle-shaped, leading to blockage in small blood vessels.
Red Blood Cell Shape
Thalassemia: Red blood cells are small, pale, and irregularly shaped.
Sickle Cell Anemia: Red blood cells are crescent or sickle-shaped, leading to blockage in small blood vessels.
Symptoms
Common to both: Fatigue, anemia, delayed growth, and weakness
Sickle cell or Thalassemia:
Mild to severe anemia depending on the type
Bone deformities (especially in the face)
Enlarged spleen
Iron overload due to frequent transfusions
Sickle Cell Anemia or Thalassemia:
Pain crises (due to blocked blood flow)
Increased risk of stroke and infections
Vision problems
Organ damage
Common to both: Fatigue, anemia, delayed growth, and weakness
Thalassemia:
Mild to severe anemia depending on the type
Bone deformities (especially in the face)
Enlarged spleen
Iron overload due to frequent transfusions
Sickle Cell Anemia:
Pain crises (due to blocked blood flow)
Increased risk of stroke and infections
Vision problems
Organ damage
Treatment for Sickle Cell or Thalassemia….
Regular blood transfusions
Iron chelation therapy
Bone marrow transplant (possible cure)
Treatment for Sickle Cell or Thalassemia….
Pain management
Hydroxyurea (to reduce sickling)
Blood transfusions
Bone marrow transplant (possible cure)
Thalassemia:
Regular blood transfusions
Iron chelation therapy
Bone marrow transplant (possible cure)
Sickle Cell Anemia:
Pain management
Hydroxyurea (to reduce sickling)
Blood transfusions
Bone marrow transplant (possible cure)
Cows milk is a Big Factor in Iron Deficiency anemia
When can babies start to consume cows milk…
How much max…
1 year old they can start to consume cows milk
24oz daily max
Breast fed infants can begin Iron supplementing when….
4 - 5 months
Describe absorption rate of lead and malnourishment…
Increased rate of absorption of lead when malnourished
Type of cleaning to perform to prevent lead dust…
Wet mop
Go lightly/ IV fluids can be used to rid by of lead…
True
(High / Low) Reticulocyte (Immature RBC) number woth Aplastic anemia…
Low.
Aplastic anemia means a reduction in production of blood cells
Why desmopressin acetate for mild to moderate hemophilia…
Releases factor 8 from endothelial cells
For a blood transfusion stay with pt how long initially…
Start transfusion at which rate….
15 min
1/4 rate
Autologous (From own body) Hematopoietic stem cells transplant.
Site
Younger….
Older….
Young: Tibia
Older: Illiac crest
Allogenic Human leukocyte antibody matched donor mostly comes from….
Siblings
Tacrolimus is used for…
Immunosuppressant after bone marrow transplant to stop rejection
