Hematology Pediatric Round 2

Thalassemias:
Autosomal recessive disorders
Impaired rate of hemoglobin chain synthesis
Alpha or beta, major or minor
Major inherited genes from both parents,
Minor inherited from one parent - only mild anemia
Beta thalassemias most common and most difficult

Thalassemia face pic 2.jpg
Incidence/etiology:
Betas - Mediterranean decent - Italians, Greeks, Arabs, Jews
Alphas - Chinese, Vietnamese, Cambodian, Laotian
Present some in the AA community

Beta Thalassemia - Cooley's anemia
Normal hgb A has four globin components
Combine with four heme complexes to form one hgb molecule
In beta thalassemia beta globin synthesis impaired so carry less hgb.
Also free alpha chains are in these RBCs so they are destroyed in the marrow or spleen
Anemia + RBC destruction
Kidneys release erythropoietin which stimulates the marrow to produce more RBCs
Bone marrow becomes hyperplastic so bones enlarge



Thalassemia picture.jpg


Manifestations:
Noted in the second six months of life as hgb F replaced by hgb A
Signs and symptoms of severe anemia
Facies - protruding forehead, maxillary prominence, wide set eyes, flattened nose
Pathologic fractures - expansion of bones to accommodate hyperplastic marrow
Decreased bone mineralization - expansion of bones to accommodate hyperplastic marrow
Malocclusion - maxillary overgrowth
Splenic enlargement - Increased RBC destruction
Jaundice - RBC hemolysis
Bronzing of skin - iron accumulation in skin



Thalassemia Complications picture.jpg



Diagnosis:
Family history
Clinical presentation
Labs - extremely low hct.

Treatment :
Goal - no cure will die by 5 or 6 if not treated
Try and normalize hct and hgb relieve anemia
Can add 2 decades to life
Blood transfusions every 2-3 weeks *(can lead to iron overload)*
*Splenectomy eliminates the site of hemolysis so decreases need for transfusions*
*Iron from repeated transfusions builds up in organs and tissues = hemosiderosis*
*Chelating agents used to reverse or reduce effects of iron overload*
Desferal, SQ over 8-12 hours, 5-7 days a week or Exjade, new tablet can be dissolved in water or juice and drunk once/day
Stem cell transplant or cord blood transplant


Toxic iron levels pic.jpgThalassemia Exjade picture.jpg

Nursing Interventions:
Blood transfusions
Observe for reactions, follow protocol
Support services, emotional support
Refer to appropriate agencies

Case Study: Beta Thalassemia








Explain the cause and mechanism of Beta Thalassemia and the classifications.

What is Cooley’s disease?
What population is most affected?
What is the treatment?
What happens if there is not treatment?
Is there a cure?






Aplastic Anemia:

Primary (congenital)

Fanconi syndrome, autosomal recessive trait
Pancytopenia, hypoplasia of the bone marrow
Patchy brown skin discoloration
Multiple congenital anomalies associated with the musculoskeletal system and the GU

Secondary (acquired)

Idiopathic –drugs, infection, human parvovirus, hepatitis, irradiation, immune response
Can be moderate or severe
Treatments immune suppression or transplantation
Nursing care is similar to the child with leukemia


Hematology Coagulation disorders


Coagulation maintains hemostasis

Maintains blood volume, blood pressure, and flow

Three parts - platelets, clotting factors, vasculature



The inflammatory response





Normal coagulation reaction -

Injured vessel - endothelial sloughing exposes subendothelial - platelets attracted to this tissue and adhere to vessel wall - platelets release biochemical mediators serotonin and histamine direct platelet activity and vasoconstriction to minimize blood loss - WBCs sent to site - Platelets aggregate into plugs - blood clot formation.

Fibrin protein formed through a series of reactions called the coagulation cascade which involves numerous clotting factors. Fibrin created only when injury occurs but fibrinogen and prothrombin circulate all the time.







Clotting Disorders include:

Hemophilia

Von Willebrand's Disease

Idiopathic thrombocytopenic purpura

Disseminated Intravascular Coagulation

Epistaxis



Hemophilia:

Group of disorders one factor in the first phase of coagulation deficient

Incidence/etiology:

Hemophilia A - most common, 1/5,000 males, 80-85% cases

Deficiency factor VIII

Hemophilia B, Christmas Disease, deficiency factor IX (Christmas factor) 10-15%

Hemophilia C, deficiency of factor XI, = in males and females, less severe, mild bleeding disorders

All result in improper clotting can be fatal.





Pathology:

Missing or decreased levels of factor VIII, IX or XI impairs or prevents clotting



Clinical Manifestations:

Depend on the amounts of clotting factors in the blood

Mild has 5-35% of normal factor levels - excessive bleeding only occurs after severe trauma

Moderate - 1-5% of normal - excessive bleeding after trauma

Severe - less than 1% - spontaneously

First year of life bleeding occurences rare

Hematomas - pockets of blood

Hemarthrosis - bleeding into joints

Hematuria - blood in urine

Epistaxis - nose bleed



Diagnosis:

Thromboplastin generation test



Treatment Hemophilia A:

Recombinant Factor now instead of plasma

Amount depends on degree

May give prophylactically 3-4 xs a week

Caregivers taught to give immediately after injury

Desmopressin acetate (Stimate) effective prior to dental or surgical procedures



Desmopressin (DDAVP), synthetic vasopressin, used to treat mild hemophilia A. Available I.V. , SQ or nasal spray (Stimate), however, lower-dosage nasal sprays aren’t effective in treating bleeding. Desmopressin triggers release of clotting factors from blood-vessel walls into the bloodstream. It’s not effective in every patient (must undergo a test to determine if their body responds to the medication). Desmopressin causes fluid retention. Monitor patient fluid intake after administration to prevent hyponatremia, especially in children.



Treatment Hemophilia B

Plasma instead of recombinant factor

Desmopressin acetate doesn't work



Nursing Care:

Prevent bleeding safety in the environment

Education of care givers…

School, noncontact sports

Soft toothbrush

Medical ID bracelet

Pain tx acetaminophen

Venipuncture versus finger or heel stick

RICE for injuries with factor replacement therapy

Passive ROM for joints and hemarthrosis, rarely splint or aspiration

Healthy body weight

Team: pediatrician, hematologist, physical therapist, orthopedist, social worker, nurse



*If signs of neurological impairment are noted in a child with hemophilia such as irritability, altered level of consciousness, vomiting may = intracranial bleed > take to hospital. If head trauma occurs even without symptoms take to the hospital and treat as if intracranial bleed. *





Case Study: Hemophilia










Issac is 4 years old. His parents are divorced and are on good terms. Issac is supposed to spend the summer with his dad. Prior to this he has lived several states away so his involvement with Issac has been limited. The father has called the office to discuss Issac's needs to prepare for caring for him this summer. He is extremely motivated and excited to be able to spend more time with his son but he is also nervous.



Begin by explaining to the father the mechanism behind hemophilia.

What are the most important teaching topics which you will need to cover with Issac's dad?

What are some common places for bleeding in hemophiliacs?

What would constitute an emergency for Issac?

What is the treatment?







Von Willebrand's Disease







Von Willebrand's Disease

Hereditary bleeding disorder deficiency

Absence of Von Willebrand factor and factor VIII.

This anomaly affects males and females.

Nursing care is the same as for hemophilia



Idiopathic Thrombocytopenia Purpura (ITP)

Acquired, cause unknown

Thrombocytopenia, destruction of platelets

Most frequent thrombocytopenia of childhood

Purpura (petechiae beneath the skin)

Normal bone marrow

Normally seen between 2 and 10

Acute (self limiting) or chronic

Acute often follows URI, measles, rubella, chicken pox or parvovirus



Nursing Care:

Supportive

Course is self limited most often

Activity restricted

Prednisone

IV immune globulin

Anti-D antibody in lieu of above options

Splenectomy if persists over a year…risks

*Teaching, only acetaminophen for pain, no contact sports…





Disseminated Intravascular Coagulation (DIC)

Consumption coagulopathy…

Occurs as a complication of a number of pathogenic processes such as shock, hypoxia, acidosis, systemic disease, sepsis, rickettsial and some viral infections

When the first phase of the clotting process is abnormally stimulated

1st too much thrombin is generated > fibrinogen > fibrin

2nd fibrinolytic > destruction of clotting factors > uncontrollable bleeding/organs



Nursing Care:

Treat the underlying cause

Platelets and fresh frozen plasma

Exchange transfusion with fresh blood

IV administration of heparin to inhibit thrombin, limited to those unresponsive to other measures



Epistaxis:

Nose bleeding

Common in childhood

Nose especially septum, highly vascular



Causes:

Trauma, injury, nose picking

URI, allergic rhinitis

Persistent nose bleeds require evaluation



Nursing Care:

Stay clam

Educate

Bleeding usually stops in 10-15 minutes

Sit up, lean forward, do not lay down (aspiration)

Apply pressure for 10 minutes

Insert cotton or wadded tissue into nostril

Ice

May pack with epinephrine soaked gauze

Petroleum or water soluble jelly to prevent crusting

Environmentally add humidifier

Evaluate if prolonged > 30 minutes







The inflammatory response located at

http://youtu.be/_bNN95sA6-8



Coagulation Cascade video located at

http://youtu.be/co6ar6vVp70



Hemophilia video located at

http://youtu.be/FS64UXi74lY



Hemophilia and Kids video located at

http://youtu.be/4eK2AgP2-sE



UnderstandingVon Willebrands Disease located at

http://youtu.be/CNS6vXRusKI