Hematologic Peds

Most common anemai age

Sympt of iron def. anemia

Fatigue, lethargy, irritability

Muscle weakness, dizzyness, fainting with exertion

Sortness of breath, heart murmor, heart failure

Tachycardia, tacypnea, pallor

Impaired healing
thinning of hair

Iron supplement should not be given with

Encouring what feeding for iron def. anemia

Milk restrictions in toddlers

Increase what in diet to treat iron def. anemai

Iron rich foods are

Use straw to

How iron is given

Stool color in Iron def. anemia

length of tx ffo Iron def. anemia

monitor what labs with Iron def. anemia

prolong anemia can cause

Signs of iron overdose?

Vomiting, abdominal pain and bloody diarrhea

lethargy, dyspnea, shock

overdose may appear better before

used to bind iron

removes iron from body, _____________ agen

Inherited sickle cell anemia

SCA, genes come from

Vaso occlusion is seen in

What causes vaso-occlusion

Painful crises associated with

Prenantal screening for SCA

Newborn screening for SCA

DX tests for SCA

Check for intracranial vascular flow and detect risk for CVA in what disease?

Cold and SCA

Transcranial doppler for what, and who?

What vaccines in SCA

Prophylactic what in SCA?

02 for SCA

Fluids in SCA

Blood transfusions tx for

Splenectomy tx for

Goal in SCA

Manage pain in vaso occulive crisis

Contraindicated for pain in VO

Pain in V O crisis r/t

Ischemia in hands and feet seen in

Pain, cough, fever >102, retractions, decfreased 02 sats...think

Priapism seen in

Hematuria, hyposthenuria seen in

Hyposthenuria is

Osteomyelitis see in

Retinopathy, blindness seen in

Chronic skin ulcers seen in

Hepatomegaly, splenomegaly seen in

Dacylitis seen in

Increased electrolytes in VO crisis....why?

S/S of CVA

Blood transfusion how long following CVA?

CVA is a complication of

Nursing intervention for VO crisis

Avoid stress or trauma

Hydration

May have enuriesiss

Heat to affected area

Bed Rest

Prevent infection

ROM

Avoid exposure to cold

Monitor labs

Monitor liver function

Inherited blood disorders

Who gets thalasemia

Types of Thallasemia

Damages/hemolyzes RBC

3 types of Betal Thallasemia

Severe Anemia
Chronic hypoxia
Growth retaradtion
Fractures, skeletal deformities

Skeletal deformities

Chronic hypoxia

Severe anemai

Prominent and proruding forehead

Maxillary prominence

Wide set eyes

Flattened nose

Acccomodates hyperplastic marrow

Too much iron

Bronze sin, leg ulceres

Splenomegaly, endocrine abnormalities

Liver and gallbladder disease

Osteopororiss

DX for Cooley's anemia

What hemoglobing above what level in Cooley's anemia?

Deferoxamine

Splenectomy in some children

When spleet is removed be careful of....tx with....immunize for

Failure of bone marrow to prduce blood cells

Bone marrow aplasia & pancytopenia

Profound anemia
Leukopenia
Thrombocytopenia

Fanconi's inerited

Exposure to toxins, environement, viruses, myelosuppressive drugs, other drugs such as sulfanamides, anit inflamatory, gold salts, anticonvulsants, tridione, dilantin, tegretol, antimalarials, analgesics and radiotion can cause

Most cases are idiopathic

How to diagnose aplastic anemai

definistive dx of bone marrow aspiration

Guaiac positive stool

Hematuria

Bone marrow transplant

ALG

ATG

Cyclospirine

Hypertension
Excessive body hair
Urea stays in body
gingival hypertorphy
immunodefici
increased creat
irreversable nephrotoxicity
P. Carinni pneumonia

More successful before multiple transfusions adminsitered

Hematopoeitic stem cell transplant

85% survival rate in untransfused

70% survival rate in transused

Prevent complications
assess for hemorrhage
ecchymoses, petechiae, purpura
tachycarida
tachypnea
stool softener to prevent anal fissures

stool softener

80% X linked recessive

Mothers to sons

Factor VIII defienciey

Facor IX deficiency

Factor XI defenciency

Affects men and women

Def of factor VIII and Von willebrand

Bleeds into sq and IM tissue

Hemarthroisis, bleeds into joint space

Swelling, warmth, redness, pain, loss of movement, bruising

Hx of bleeding episodes, epistaxis, bleeding gums, hematuria, tarry stools

Headahe, slurred speech, decrease LOC

Chest or abdominal pain

Assess circulation

Factor levels, dx for

DNA test

PTT, dx for

hgb, hct dx for

Replace factor VIII

corticosteroids tx for

NSAIDS used in caution , tx for

Phyiscal activity and exersise, tx for

Infusion of VIII prophylaxis of...before onset of

Primary prophylaxis in hemophilia

Secondary prophylaxis for hemophilia

Active bleed tx

No cure

If older than 25, and blood transfusions

ITP

Excessive destruction of palteltes (thrombocytopenia)

Normal bone marrow with normial or increase number of immature platelets

Discoloration caused by petechiae beneath the skin

Acute, self limiting course...a form of

Chronic condition, greather than 6 months duration a form of

Acute ITP seen after

DX for ITP based on clinical manifestations

Platelet count reduced to 20,000

WBC's, HGB, HCT in ITP

Watch bleeding times in

No definitive test

May do tests to rule out other disorders

Restrict activty

IVIG or until paltelest up to 30000

Platelest transfusion in Idiopathic thrombocytopenic purpura only if

Splenectoy for ITP if it lasts longer than

NO contact sports

Avoid ASA and NSAIDS, use acetaminophne instead

Is a secondary condition

activation of coagulation

Thrombin generated, fibrin deposited in circualtion, platelets are consumed

Deficiency of coag and anticoag pathways occur

Results in hemorrage and organ/tissue damage

Septic shock is a trigger of

Endotoxins and viruses are triggers of

tissue necrosis or injury is a common trigger of

cancner treatment is a trigger of

Hemolytic transfusion reactions (anaphylaxis) can be a tigger for

Hypotension or hypoxia is a trigger of

Snake bites trigger

Pancreatitis, and liver disease can trigger

Suspect when there is a tendency to bleed

Bleeding from venipuncture and surgical sites, clinical manifestation of

Petechiae and purpura are manifestations of

Tx DIC

anticoagulants and clotting factors are treatmetns for

clotting factors, platelets and fresh frozen plasma are tx for

cryoprecipitates

Epistaxis tx

malignant disease of bone marrow an lymph system. all cell types are effected

normal elements in bone marrow are repalced with abnormal white blood cells

2 types of Leukemia

most common type of Leukemia

Leukemia in children 2-8

less than 1 year old with Leukemia

cancer seen in caucasions

Abnormal lymphocytes, no abilityt ofight infection, crown out normal cells

Cure rate 65-75%

Cancer peaks during adolescense

Poor prognosis for this cancer

Presenting signs of leukemia (TEST)

Hepatosplenomagaly more comman than lymphandenopathy in

Can have increase ICP from CNS involvement

Originates in lymphoid system

primary involves lumph

Metastisises to non lodal istes such as spleen, liver, bone marrow and lungs

Ann arbor staging for

A in ann arbor staging

B in Annn Arbor staging

Enlarged cervical or supraclavicular lymphandenopathy, and is the most common presentation

Multiple organ invoelvement

Lymph node biopsy for staging and histology diagnosis

Presense of reed sternburg cells

Giant cells with huge nuclei

painless enlarged lyph nodes, ervical most common

Nonproductive cough (mediastinal nodes)

Abdominal pain (retoperi toneal nodes)

Radiation and chemotherpay, tx for

Long term survival rates excellent

Mutation B & T cells

Agressive malignancy

Responseive to treatment

managed like hodgkins

Spreads easy to CNS

Progresses quickly