Jumat, 06 Mei 2011
Thalassemia
UNDERSTANDING
Thalassemia is an inherited blood disorder characterized by deficient production of globin chains in hemoglobin.
Kind - kind of Thalassemia:
1.Thalasemia beta
Is among the most common anemia caused by an inherited defect in the synthesis of hemoglobin beta chains.
Beta thalassemia include:
a.Thalasemia major beta
Homozygous form is a hypochromic microcytic anemia with severe hemolysis in the bone marrow begins in the first year kehidupan.Kedua parent is a carrier "feature". Symptoms - symptoms are secondary to anemia and include pale, due to widening of facial characteristics in tabular tabular bone in the cranium, with varying degrees of jaundice, and hepatosplenomegaly.
Intermedia and minor b.Thalasemia
In heterozygous form, can be found a sign - a sign of mild anemia and splenomegaly. On examination of peripheral blood obtained Hb level varied somewhat low normal or increased (polycythemia). Increases in serum bilirubin, bilirubin levels increased slightly.
2.Thalasemia negligent
Is a deficiency in thalassemia with a chain
Etiology
Genetic factors
Pathophysiology
After the birth of a normal hemoglobin consists of two chains and beta polipeptide negligent. In beta thalassemia there is a decrease in part or whole in the synthesis of molecules of hemoglobin beta chains.Consequently an increase in process pensintesisan compensatori negligent chain and gamma chain production remained active, and causes the formation of hemoglobin imperfections. Polipeptid unbalanced is not very stable, easy to separate and destroy red blood cells which can cause severe anemia. To overcome the hemolytic process, red blood cells formed in significant amounts, or at least suppressed by bone marrow transfusion therapy. Fe excess of RBCs in a transfusion as well as the addition of the rapid destruction of cells defectif, stored in various organs (hemosiderosis).
Clinical manifestations
New baby born with beta thalassemia major is not anemis. Early symptoms are not obvious at first pale, usually become more severe in the first year of life and in severe cases occurred several weeks on after birth.If the disease is not handled properly, the life of a child's growth will be retarded. Children no appetite, diarrhea, loss of body fat and can be accompanied by recurrent fever due to infection. Severe anemia and long usually causes an enlarged heart.
There was hepatosplenomegaly. Mild jaundice may be present. There is a change in the bones that persist, namely the occurrence of Mongoloid face due to a hyperactive eritropoesis system. The existence of cortical thinning of long bones, hands and feet can cause pathological fractures. The divergence of growth due to anemia and malnutrition cause short stature.Sometimes found epistaxis, skin pigmentation, ulceration in the legs, and gallstones. Patients become susceptible to infection, especially if the spleen had been removed before the age of 5 years and susceptible to septicemia that can result in death. Pensitopenia can arise due to hypersplenism.
Hemosiderosis occur in endocrine glands (the delay and disruption of development of secondary sex characteristics), pancreas (diabetes), liver (cirrhosis), heart muscle (arrhythmia, electrical disturbances, heart failure), and pericardium (perikerditis).
In general, signs and symptoms that can be seen among others:
1.Letargi
2.Pale
3.Bloodles
4.Anoreksia
5.Crodek breath
6.Ticknes cranial bone
7.Enlargement spleen
8.Depletion bone cartilage
EXAMINATION SUPPORT
Hematological studies: there is a change - a change in red blood cells, namely mikrositosis, hypochromia, anosositosis, poikilositosis, target cells, immature erythrocytes, decreased hemoglobin and hematrokrit.
Hemoglobin electrophoresis: increase of hemoglobin
In beta thalassemia major bone marrow is found mainly hyperactive erythrocyte series. X-ray results include changes in the bone marrow hyperplasia due to excessive. Changes include the widening of the medulla, cortical thinning, and trabekulasi a more rough.
DNA analysis, DNA probing, gone blotting and PCR (Polymerase Chain Reaction) is a more advanced type of examination.
MANAGEMEN
1.Transfusi red blood cells (HR) to Hb levels around 11 g / dl. Giving the red blood cells should be 10-20 ml / kg body weight.
2.Gift chelating agents (Desferal) intravenously or subcutaneously.Desferiprone a peroral dosage form. But the benefit is lower than desferal and gives the danger of liver fibrosis.
3.Managemen splenectomy should be considered especially when there are signs - signs of hypersplenism or increased transfusion requirement or because a very large spleen.
4.Transplantasi bone marrow usually done in beta thalassemia major.
ASSESSMENT
Physical 1.Pengkajian
Perform a physical examination.
Review the medical history, especially those associated with anemia and a history of the disease in the family.
Observation of symptoms of anemia.
General 2.Pengkajian
Stunted growth
Chronic anemia.
Delayed sexual maturity.
Vaso-occlusive 3.Krisis
Pain is felt
Symptoms associated with ischemia and related areas.
Extremities: skin peeling hands and feet accompanied by pain radiating.
Abdomen: pain that is so it can be done surgery
Cerebrum: stroke, impaired vision.
Waist: symptoms such as severe pneumonia.
Liver: jaundise obstruction, coma hepatikum.
Renal: hematuria.
Effect of chronic crises are vaso-occclusive:
Heart: cardiomegali, systolic murmur
The lungs: lung function disorders, easily infected.
Kidney: inability to break down the compounds of urine, kidney failure.
Genital: aching, tense.
Liver: hepatomegaly, cirrhosis.
Eyes: abnormalities of the lens resulting in blurred vision, sometimes causing disruption of retinal layers and can cause blindness.
Extremity: change the bones especially can make stooped, easily infected with salmonella osteomyelitis.
Nursing Diagnosis
High 4.Risk injury associated with abnormal hemoglobin, decreased levels of oxygen, dehydration.
5.Paiful anoxia associated with membranes (vaso occlusive crisis)
6.Changes process within the family associated with the impact of child illness on family functioning; the risk of a long healing in children.
NURSING INTERVENTION
High 7.Resiko injury associated with an abnormal hemoglobin, the oxygen reduction, dehydration.
Objectives:
a.Watch for patients to get enough oxygen
Nursing Intervention:
Measure the pressure to minimize the complications associated with physical and emotional stress eksersi
Rational: to avoid the addition of oxygen required
Do not get an infection
Keep out of low oxygenated environments.
Expected results:
Do not expose your child from situations that can cause lack of oxygen in the brain.
b.watch so that children do not experience dehidasi
Nursing intervention.
1) Observation of intravenous fluids as recommended (150ml/kg) and the minimum fluid requirement of children; infusion.
Rational: ank for fluid needs can be met.
2) Increase the number of intravenous fluids above the minimum requirement when there is physical exercise or stress and submarine crisis.
Rational: to adequate intravenous fluid requirements.
3) Give written inforamasi in older people associated with specific fluid requirements.
Rational: to encourage complience.
4) Encourage the child to drink
Rational: to encourage complience.
5) Give information to families about the signs - signs of dehydration
Rational: to avoid delay in delivery of fluid therapy.
6) The importance of the emphasis will pentingnnya avoid heat
Rational: to avoid causing loss of fluid.
Expected results:
Son of a lot of drinking and amount of fluid so it does not happen dehidarsi fulfilled.
c.free of infection
Nursing intervention
1) Emphasize the importance of nutrition, routine immunization, including pneumococcal vaccine and meningococal; protection of the source - the source of infection is unknown; periodic health surveillance.
2) Report any signs of infection in a responsible immediately.
Rational: to avoid delay in handling.
3) Give antibiotic therapy
Rational: to prevent and treat infections.
Expected results:
Children free from infection.
d.reduced risks associated with the effects of surgery.
Nursing intervention
1) Explain the importance of blood transfusion
Rational: to increase the concentration of Hb A
2) Keep children to avoid dehydration
3) persuading children not to tense.
Rational: Anxiety can increase oxygen demand.
4) Give anlgesik
Rational: for children to feel comfortable and reduce anxiety response.
5) Preventing the unnecessary activities
Rational: to prevent the addition of oxygen demand.
6) Keep your postoperative airway clearance
Rational: to prevent infection
7) Perform passive ROM exercises
Rational: to stimulate circulation.
8) Collaboration for oxygen delivery
Rational: to increase the hemoglobin concentration.
9) observation sign - a sign of infection.
Rational: to be quickly addressed.
Expected results:
When children do not experience a crisis operation.
8.Painful anoksia associated with membranes (vaso-occlusive crisis)
Objectives:
To avoid the pain or at least the pain is not too painful for the child
Nursing Intervention:
1) Schedule a preventive medication to be kept - going even if not needed.
Rational: to prevent illness.
2) Identify the kinds of - kinds of analgesic medications including opioids and schedule may be required.
Rational: to find out how much pain is acceptable.
3) Reassure the child and family that analgesics, including opioids, medically necessary and may be required in high doses.
Rational: because of excessive pain can happen because of their suggestion.
4) Give heat stimulus to the area in question because the area of pain
5) Avoid compression with cold water
Rational: because it can enhance vasoconstriction
Expected results:
To avoid the pain or at least the pain is not too painful for the child.
9.Perubahan process within the family associated with the impact of child illness on family functioning; the risk of a long healing in children.
Objectives:
a.facebook get an understanding of the disease
Nursing Intervention:
1) Teach families and older children about the characteristics of measurement - measurement.
Rational: to minimize complications.
2) Emphasize the importance of informing the development of health, illness of the child.
Rational: to get the progress of proper care.
3) Explain the sign - a sign of the increasing crisis in particular fever, pale and breathing problems.
Rational: to avoid delay in treatment.
4) Provide an overview of hereditary diseases and provide health education on genetic keluargatentang their families.
Rational: for families know what to do.
5) Place the elderly as a supervisor for their children.
Rational: to get the best care.
Expected results:
Children and families can really - really know about the child's disease in the etiology and therapy - therapy.
b.facebook receive sufficient encouragement.
Nursing Intervention:
1) Refers to a trusted organization.
Rational: To support the maintenance process.
2) Register your child at the clinic anemia
Rational: to get the right treatment.
3) Always be aware of a family when 2 or more family members affected by this disease.
Expected results:
Families can take advantage of these services and abnak can receive proper care from the facility.
REFERENCES
Arif Mansjoer, et al. Capita Selekta Medicine. Edition 3. New York: Media Aesculapius, 2000
Sacharin, Ross M. Principles of Pediatric Nursing. Issue 1. Translation R.F. Maulany. Jakarta: EGC, 1996.
Suriadi, Rita Yuliani. Nursing the Child. Issue 1. Jakarta, 2001.
Wong, Donna L, Christina Algiere Kasparisin, Caryn Hess Stoer mer.Clinical Manual of Pediatric Nursing. Fourth edition. St. Louis: Mosby Year Book, 1996.
Wong, Donna L, Shannon E. Perry, Marilyn J. Hockenberry. Maternal Child Nursing Care. St. Louis: Mosby Company, 2002.
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