Concept about Hemophilia – Health of the children has been considered as the vital importance to all societies because children are the basic resource for the future of humankind. Nursing care of children is concerned for both the health of the children and for the illnesses that affect their growth and development. The increasing complexity of medical and nursing science has created a need for special area of child care, i.e. pediatric nursing.
Pediatric nursing is the specialized area of nursing practice concerning the care of children during wellness and illness. It includes preventive, promotive, curative and rehabilitative care of children. It emphasizes on all round development of body, mind and spirit of the growing individual. Thus, pediatric nursing involves in giving assistance, care and support to the growing and developing children to achieve their individual potential for functioning with fullest capacity.
Concept about Hemophilia
Definition of Hemophilia:
Hemophilia is an inherited bleeding disorder due to deficiency of plasma coagulation factors. It is primarily found in males but transmitted by female carriers
Or
Hemophilia is a rare disorder in which your blood doesn’t clot normally because it lacks sufficient blood-clotting proteins (clotting factors).
(Ref: Paediatric Nursing, Parul Datta/34/327)
Classification of Hemophilia:
Based on the deficient factors of coagulation.
1. Hemophilia-A (classical hemophilia)-It occurs due to deficiency of plasma factor VII, the antihemophilic factor (AHF). It accounts for 80 to 85 percent of all hemophilics. Hemophilia- A can be classified based upon the factor-VIII level in plasma. They are as follows:
- Severe hemophilia-A- In this condition, the factor level is found less than 1 percent of normal value. Patients have tendency of spontaneous bleeding and severe bleeding.
- Moderate hemophilia-A: Factor level remains between 1 to 5 percent of normal. Patients have no spontaneous bleeding and may not have severe bleeding until any trauma occurs.
- Mild hemophilia-A: Factor level is in between 6 to 30 percent of normal. Patients usually lead normal lives
2. Hemophilia-B (Christmas disease)- It results from deficiency of plasma factor IX, the plasma thromboplastin component (PTC). It accounts for about 15 to 20 percent of cases.
3. Hemophilia-C-It results from deficiency of factor XI, plasma thromboplastin antecedent (PTA). It accounts for few cases only
(Ref: Paediatric Nursing, Parul Datta/34/327)
Clinical Features of Hemophilia:
- Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work
- Many large or deep bruises
- Unusual bleeding after vaccinations
- Pain, swelling or tightness in your joints
- Blood in your urine or stool
- Nosebleeds without a known cause
- In infants, unexplained irritability
Nursing Management of Hemophilia:
Nursing assessment should include details history of illness with family history and history of prolonged bleeding after any injury or surgery. Thorough physical examination to be done to assess the nursing diagnosis and to plan nursing interventions.
Nursing interventions:
1. Assessing the child at frequent interval and recording of findings related to the disease.
2. Preventing hypovolemia by control of bleeding with the following measures:
- Applying pressure and cold on the area for 10 to 15 minutes even after injection and venipuncture.
- Placing absorbable gelatin foam or fibrin foam on wound and applying fibrinolytic agent for oral bleeding.
- Avoiding suturing and cauterization during surgery.
- Immobilizing and elevating the affected part.
- Providing rest, comfort and quiet environment
- Monitoring vifal signs and signs of shock.
3. Administering drugs as prescribed with necessary precautions. Missing factors or blood should be given slowly to minimize transfusion reaction (2-3 ml/min). Packet should be checked for negative test of hepatitis ‘B’. HIV etc.
4. Preserving joint mobility by treating hemarthrosis with prescribed drugs, immobilization in a slight flexion position, applying cold and following other instructions of supportive care Affected joint can be treated with plaster cast if necessary. Preventing weight bearing on the affected joint.
5. Providing protection against injury and bleeding
6. Teaching the parent and family members about home care, safety measures, regular follow- up, continuation of school education, avoidance of sports injury and overweight: need for hospitalization, complications, etc.
7. Providing emotional support to cope with the problem and involving parents in routine care of the child.
(Ref: Paediatric Nursing, Parul Datta/34/327-328)
Complications of Hemophilia:
Hemophilia may cause numbers of complications. They include:
- Airway obstruction caused by bleeding into the neck and pharynx. Intracranial bleeding leading to neurological impairment.
- Intestinal obstruction due to GI bleeding.
- Compartment syndrome due to compression of nerves by bleeding into deep tissues.
- Degenerative joint disease, osteoporosis and muscle atrophy due to repeated hemorrhage.
- Chronic hepatitis and cirrhosis due to contaminated cryoprecipitate.
- HIV/AIDS due to transfusion related infections.
- Physical, psychological and social handicaps.
(Ref: Paediatric Nursing, Parul Datta/3/328)
Read more: