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Module 10.

3: Children Who Have Alterations in Tissue Perfusion

Stephanie Talbot

EO 1.1: Terms

X-linked, autosomal recessive bleeding disorder occurs as result of deficiencies in coagulation proteins

Classic Haemophilia most common

Also called haemophilia type A in which there is a deficiency in coagulation factor VIII (Antihemophilic factorAHF) produced in liver

EO 1.1: Terms
Christmas Disease

Also called haemophilia type B Characterized by deficiency in coagulation factor IX (Plasma thromboplastin componentPTC)


Bleeding into the joints Mainly knee, hip & elbow disabling

EO 1.1: Terms
Hydrops fetalis

Accumulation of fluid in two or more body areas in the fetus Serious condition

Breast Milk Jaundice

Occurs breast fed babies @ 5-7dys old Products (fatty acids, b- glucuronidase & pregnanediol) in breast milk inhibit bilirubin conjugation

EO 1.1: Terms
Icterus neonatorum (Physiologic jaundice)

Yellowing of the skin in the newborn due to increased bilirubin in blood Why?

Newborns immature liver cannot excrete bile @ same rate destruction RBCs occurs.

Kernicterus Neurologic condition in which high levels of bilirubin result in the deposition of bilirubin into the brain & spinal cord. Produces convulsions in infants.

EO-1.2:Describe w/ rationale, each of the following for the child w/ HAEMOPHILIA

Etiology & Pathophysiology

Deficiency in factor VIII inability to from thromboplastin during phase I of blood-clotting process = prolonged bleeding anywhere from or in the body. Bleeding into the mouth, neck or thorax serious cause can lead to obstruction

1. SQ & IM hemorrhages 2. Hemoarthrosis s/s: stiffness, tingling, achy joint 3. Bony changes as result repeated bleeding episodes 4. Prolonged bleeding 5. Spontaneous hematuria 6. Epistaxis-nose bleed 7. Ecchymoses & SQ hematomas

EO 1.3 Signs & Symptoms

EO-1.2:Describe w/ rationale, each of the following for the child w/ HAEMOPHILIA

Diagnostics Evaluation: 1.Hx bleeding episodes 2.X-linked inheritance 3.Laboratory findings

a)Factor VII & IX assay (deficiency) b)Clotting times c)PT=N, aPTT prolonged, INR=N

EO-1.2:Describe w/ rationale, each of the following for the child w/ HAEMOPHILIA 2. Corticosteroids tx hematuria, Hemoarthrosis, synovitis 3. NSAIDS for painibuprofen...NO ASPIRIN increases bleeding 4. E-aminocaproic acid (EAC, Amicar) to prevent clot destruction 5. Exercise & physical therapy active ROM so pt can detect own level of painNO CONCTACT SPORTS 6. Teach family: venipuncture if <8yr

Therapeutic Management: 1. Replacement of clotting factor

Factor VIII concentrates & Cryoprecipitate AHF (antihaemophilic factor) (not for VII deficiency though, )-for acute bleeding when factor concentrates not available ex. Aafact (Alphonate)from human plasma, Advaterecombinant antihaemophilic factor synthetic) DDAVP (1-deamino-8arginine vasopressin)synthetic vasopressin, results 3-4 fold increase factor VIII

EO-1.2:Describe w/ rationale, each of the following for the child w/ HAEMOPHILIA

Nursing Management:
1. Recognize s/s internal bleeding: headache, slurred speech, loss consciousness, black tarry stools Prevent bleeding-encourage appropriate exercise, safety equipment, no contact sports, soft tooth brush or spongetipped toothbrush & water pic, electric shaver Use SQ injections when possible & venipuncture for blood samples Wear medical bracelet No aspirin or aspirin containing products 6. Recognize & control bleeding: RICE (Rest, Ice, Compression, Elevate) 7. Prevent crippling effects bleeding- elevate & immobilize joint during bleeding episodes, AROM, physical therapy- admin analgesics before exercise 8. Adequate diet 9. Teach: child & family preventative measures, administration factor replacements 10. Genetic counselling as soon as poss. After diagnosis


3. 4. 5.

EO 1.4 & 1.5 expected outcomes: NSG DX Haemophila

NSG DX Expected outcomes

1. 2. 3. 4.

Bleeding, Risk for Impaired mobility Acute pain Deficient fluid vole r/t loss blood 5. Ineffective health maintenance 6. Fear r/t high risk HIV 7. Fatigue r/t loss blood volume

1.Goal = Prevent or treat bleeding Outcome: maintain stable VS w/ minimal blood loss 2. Goal= prevent contractures &joint deformities Outcome/intervention: maintain mobility/RICE, bed rest, ambulation, AROM, positioning 3. Goal = treat pain Outcome/interventions: be free of pain/admin analgesics

EO 1.6 Describe the classification, actions, & NSG implications of the following:
1. Antihemophilic factor (AHF)( Alphonate, - from pooled human plasma DDAVP synthetic vasopressin & cryoprecipitate AHF-) prevent & control bleeding those w/ Haemophilia A by increasing factor VIII levels in blood 2. Anti-inhibitor coagulant complex- (Amicar, EACA, Autoplex T)- prevents clot destruction (fibrinolysis), used if mouth trauma, surgery (dose factor concentrate must be given first)

3. Factor IX complex(Alphanine SD, Proplex, Proplex Tfrom pooled human plasma)-to prevent & control bleeding in those w/ haemophilia B by increasing factor IX levels in blood NSG implications: from plasma increased risk transmission of viruses & allergic rxs

EO 1.7: Describe each of the following for Rh & ABO incompatibility

Hemolytic Disease of the newborn is an abnormally rapid rate of RBC destruction st 24hrs cardinal sign hyperbilirubinemia in 1 Two main causes:
Isoimmunization (RhD)- a sensitivity rx to Rh antigens in which Rh-antibodies form ABO incompatibility

Blood contains agglutinogens which produce immune response. In ABO & Rh (recessive) blood group, antigens occur naturally.

EO 1.7: Describe etiology & pathophysiology for Rh incompatibility

Problem arises when mother is Rh- & fetus is Rh+ -sometimes fetal blood enters mothers circulation causing mothers defence system to produce anti-Rh antibodies enter fetal circulation where they destroy fetal erythrocytes fetus compensates by increases haematopoiesis--> immature RBCs (erythroblasts)Erythroblastosis fetalis. Most severe form erythroblastosis fetalis = hydrops fetalis hypoxia, heart failure, generalized Generalized edema(anasarca), hydrops, & effusions into pericardial, pleural, & peritoneal space Doesnt affect first pregnancy, next pregnancy

EO 1.7: Describe etiology & pathophysiology for ABO incompatibility

Main blood groups are A, B, AB, O & all contain anti-bodies except AB, so when mix one blood group w/ another = agglutination (clumping) Most common blood type = O, most common blood incompatibility is between a mother w/ O blood and an infant w/ A or B blood because the anti-A&B antibodies of O type cross placenta & attach to fetal RBC hemolysis. st pregnancy Can occur 1

EO 1.7/1.8: Describe Clinical Manifestations for Rh & ABO incompatibility

1. Jaundice w/in 24hrs birth
NB: most newborns not jaundiced @ birth due to nonfxning liver

2. Increased serum Unconjugated bilirubin 3. Anemia-from hemolysis lg amts RBCs 4. Hyperbilirubinemia-cause liver unable conjugate & excrete excess bilirubin 5. Hepatomegaly & varying degrees Hydrops 6. Anemia 7. Hypovolemic shock 8. Hypoglycemiadue to pancreatic cell hyperplasia

EO 1.7: Describe Diagnostic evaluations for Rh & ABO incompatibility

1. Indirect Combs test (maternal body titre drawn st prenatal visit) 1 2. Amniocentesis- if test positive for antibodies
Delta OD 450 test- tests optical density of amniotic fluid

3. Ultrasonography- adjunctivecheck for alterations placenta, umbilical cord, amniotic fluid volume & hydrops 4. Timing & appearance jaundice
Confirm w/ Direct Combs test or Direct antiglobulin test

5. Genetic testing- early ID of paternal zygosity

EO 1.7: Describe Therapeutic Management for Rh & ABO incompatibility

Goal therapeutic management is PREVENTION Involves phototherapy, exchange transfusion, pericardial & pleural fluid aspiration, mechanical ventilation & inotrope therapy

EO 1.7: Describe Therapeutic Management for Rh incompatibility

Prevention Rh Isoimmunization 1. Administration RhIg (RhoGAM) to all mothers @ 26 & 28wks gestation as well as after delivery, abortion & miscarriage- prevents ant-D antibodies & memory cells from forming 2. Admin tin mesoporphyrin IM tx Hyperbilirubinemia 3. Admin IV immunoglobulin (IVIG)- decrease severity RBC destruction & development of jaundice 4. Intrauterine transfusion of Rh O neg. RBCs via umbilical vein when mom already sensitized, raises fetal HCT levels 5. Exchange transfusions- tx choice severe Hyperbilirubinemia & Hydrops caused by Rhincompatibility

EO 1.7: Describe Therapeutic Management for Rh incompatibility

Exchange transfusions: sterile procedure Indication= Hyperbilirubinemia unresponsive to phototherapy Sm. Amts infants blood removed (5-10mL)& replaced w/ compatible Rh- blood in order to remove sensitized RBCs, lower serum bilirubin levels (prevents bilirubin encephalopathy), corrects anemia, prevents cardiac failure Must be @ least 35wks gestation

EO 1.7: Describe Therapeutic Management for Rh incompatibility

Signs blood exchange transfusion rx: Tachycardia or bradycardia Respiratory distress Dramatic change blood pressure Temperature instability rash

EO 1.7: Describe Therapeutic Management for ABO incompatibility

Goal= early detection & implementation phototherapy for reduction Hyperbilirubinemia IVIG transfusions are used in combination w/ phototherapy in some centers.

EO 1.7: Describe Nursing Management for Rh & ABO incompatibility

1. Recognize jaundice: anticipate from prenatal & Perinatal hx 2. If transfusion exchange needed:
NPO during procedure, IV dextrose & e-lytes Document blood volumes exchanged Monitor VS, cardiac & respiratory fxn , transfusion rxs Maintain adequate thermoregulation, blood levels & fluid balance

3. Support family, encourage express feelings

EO 1.9: Select the appropriate NSG Dx for infants w/ hemolytic dx

1.Risk for injury 2.Neonatal jaundice r/t Rh or ABO incompatibility 3.Deficient knowledge r/t tx regime 4.anxiety