| Hemophilia Symptoms and Treatment Posted: 02 Jan 2011 11:00 PM PST 
- Usually an inherited, congenitalbleeding disorder characterized by lack of blood clotting factors, especially factors VIII and IX
- An X-linked disorder primarily affecting males; females act as carriers
- Occurs in 1 in 5000 males
- No racial predilection; found in all ethnic groups
 Pathophysiology and Etiology
- Hereditary
- Spontaneous mutations may cause the condition when the family history is negative for the disease.
- The basic defect is in the intrinsic phase of the coagulation cascade. The blood clotting factors are necessary for the formation of prothrombin activator, which acts as a catalyst in theconversion of prothrombin to thrombin.
- The rate of formation of thrombin from prothrombin is almost directly proportional to the amount of prothrombin activator available.
- The rapidity of the clotting process is proportional to the amount of thrombin formed.
- The result is an unstable fibrin clot.
- Platelet function and number are normal. Therefore, small lacerations and minor hemorrhages are usually not a problem.
Signs and Symptoms - Abnormal bleeding
- Prolonged bleeding episodes after circumcision
- Easily bruise
- Spontaneous soft tissue hematomas
- Hemarthrosis or hemorrhages on the joints
- Spontaneous hematuria
- GI bleeding
Diagnostic Evaluation - prothrombin and bleeding time are normal
- partial thromboplastin time – prolonged
- prothrombin consumption – decreased
- thromboplastin – increased
- assays for specific clotting factors – abnormal
- gene analysis – detects carrier state for prenatal diagnosis
Management - Prompt, early, appropriate treatment is the key to preventing most complications.
- Must replace missing coagulation factor (VIII or IX) through the administration of type-specific coagulation concentrates during bleeding episodes.
- Factor VIII – made from cryoprecipitate that has been viral inactivated, monoclonal or detergent purified.
- Factor IX – made from fresh frozen plasma that has been viral inactivated by solvent detergent.
- No viral inactivated concentrate exists for Hemophilia C; fresh frozen plasma is given to supply factor XI.
- Mild and moderate VIII-deficient hemophiliacs may respond to desmopressin (DDAVP).
- Antifibrinolytics are given as adjunctive therapy for mucosal bleeding to prevent clot breakdown.
- Supportive therapies: NSAIDs, physical therapy, orthotics.
- Synovecty – orthopedic surgical intervention.
Complications - airway obstruction
- degenerative joint changes with osteoporosis and muscle atrophy
- intestinal obstruction
- compression of nerves by paralysis
- intracranial bleeding
Nursing Management - Provide emergency care for bleeding.
- Immobilize the affected part and elevate above the level of the heart.
- Administer recombinant factor VIII or factor IX coagulation concentrate.
- Apply fibrinolytic agents to wound for oral bleeding.
- Avoid rectal thermometers.
- Avoid injections if possible.
- Maintain a safe environment and teach patients and parent safety measures.
- Be aware that increased pain usually means that bleeding continues and further replacement therapy may be needed.
- Enhance family coping.
Photo credits: www.healthline.com Related posts: - Hemophilia
- Thrombin Time Test
- Nursing Care Plan – Hemophilia
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| Impetigo Posted: 02 Jan 2011 10:36 PM PST 
Definition Impetigo is a superficial infection of the skin. It is an infectious disease transmitted only by direct contact from the affected person. This bacterial skin infection is also known as school sores which is most common among the pre-school children. Information on Impetigo Causative agent: Beta-hemolytic streptococcus, group A (nonbullous), Staphylococcus Aureus (bullous) Incubation Period: 2-5 days Period of communicability: from the outbreak of the lesions until the lesions are healed Mode of transmission: direct contact with lesions. Scratching may spread the lesions. Immunity: none Classification - Bullous Impetigo – this type of impetigo primarily affects infants and children younger than 2 years. Painless and fluid-filled blisters are found on the trunk, arms and legs in children with bullous impetigo. The surrounding akin of the blisters may be red and itchy but not sore. When the blisters break, they scab over with a yellow-colored crust that lasts longer than sores from other type of impetigo.
- 2. Ecthyma – this is the more serious form of impetigo. The infection in ecthyma penetrates deeper into the second layer of the skin which is the dermis.
Clinical Manifestations This infectious disease begins as a single papulovesicular lesion that is surrounded by a localized redness or erythema. Later on, more vesicles will appear and they will become purulent. As the disease progresses, the vesicles ooze and form a honey-colored crusts. The lesions are found mostly on the extremities and the face. Local adenopathy may occur if an enormous number of lesions will be noted. Management - Penicillin or Erythromycin orally administered.
- Application of mupirocin (Bactroban) ointment for 7 to 10 days.
- Wash the crusts daily with soap and water for the lesions to heal quickly.
- Contact precautions should be implemented.
- Instruct the patient to stay indoors for a few days to stop any bacteria from getting into the blisters and making the infections worse.
- The infected person’s bed linens, towels, and clothing should be separated from those of other family members.
- The infected person should use separate towels for bathing and hand washing.
Prevention - Good hygiene.
- Injured skin areas should be kept clean and covered with clean gauze to prevent infection.
- Regular and effective hand washing practice.
- Keep fingernails cut short.
Possible Complications - Rheumatic fever
- Acute glumerolunephritis
image from faculty.ksu.edu.sa Related posts: - What is Scabies
- Chicken Pox (Varicella)
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| Intravenous Pyelography Posted: 02 Jan 2011 09:43 PM PST 
Intravenous Pyelography - Also known as IVP, pyelography, intravenous urogram or IVU
- Is a radiological procedure used to visualize abnormalities of the urinary system, including the kidneys, ureters, and bladder
- Gives a comprehensive view of the patient’s anatomy and some information on the functioning of the renal system
- Usually done to patient with severe renal colic and a positive hematuria test
- Used as a way to diagnose chronic pyelonephritis, kidney stones, renal cell carcinoma or RCC, Trnasitional cell carcinoma or TCC, Polycystic kidneys
Procedure - A cannula is inserted in the vein usually in the arm where in a contrast media is injected.
- Using the X-ray, the contrast media is seen and termed as “renal blush”.
- X ray shots are taken in intervals to capture the way it travels inside the urinary system.
- After three (3) minutes of X-ray shots, the calices and renal pelvis can now be seen.
- After 9 to 13 minutes it goes to the bladder.
- The contrast is excreted or removed from the bloodstream via the kidneys.
- A post micturition X-ray is then taken in order to compare the images for more evidence of pathology.
Contraindications: - Patients taking metformin should hold this medication 48 hours before and after the procedure to avoid such interaction.
- This is not indicated for pregnant women and those who have kidney disease or renal failure.
Adverse Reactions: - Nausea and vomiting
- Itching
- Difficulty in breathing or swallowing
- Swelling of the lips and tongue
- Low blood pressure
- Loss of consciousness
Nursing Considerations: Before the Procedure: - Assess the history of allergy, medications currently taken and risk of pregnancy for women.
- Check if consent is properly signed.
- Emphasize to the patient that nothing should be taken or ingested 12 hours before the procedure.
After the Procedure: - Monitor the intake and output strictly especially the next 24 hours.
- Assess for adverse reactions.
- Assess the puncture site for active bleeding
- Document the findings properly.
Photo credits: www.stonybrookphysicians.adam.com Related posts: - ADDING A BOLUS INTRAVENOUS MEDICATION TO AN EXISTING INTRAVENOUS LINE
- Blood Glucose Tests – IGTT
- Renal Problems – Renal Calculi
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| Pediatric Asthma Management Posted: 02 Jan 2011 07:19 PM PST 
Pediatric Asthma Management Asthma as defined by National Asthma Education and Prevention Program is a chronic inflammatory disorder of the airways in which many cells and cellular elements play a role, in particular, mast cells, eosinophils, T lymphocytes, neutrophils and epithelial cells. Pathophysiology: When mast cells activate, there is infiltration of inflammatory cells, edema, denudation and disruption of the bronchial epithelium, goblet cell hyperplasia and smooth muscle thickening resulting to asthmatic inflammation. Signs and symptoms: - Recurrent episodes of wheezing
- Breathlessness
- Chest tightness
- Cough particularly at night and in the early morning
Risk factors: - Children before age of 5
- "hygiene hypothesis" (suggests that living in a clean environment may predispose the immune system of the child towards allergic reactivity
- Heredity
Allergic triggers: - Allergens
- Irritants
- Weather changes
- Viral or sinus infections
- Exercise
- Reflux disease
- Medications (sensitivity to aspirin, NSAIDs sulphites)
- Food
- Emotional anxiety
- Rhinitis
- Sinusitis
Medical Management: - Use of short acting bronchodilator which is inhaled beta2-agonists as needed for symptoms with MDI spacer/holding chamber
- Bronchodilators should be given every 4 to 6 hours for 24 hours for patient with asthma and viral infection.
Nursing Management: - Regular monitoring and assessment of the symptoms in the past 2 weeks.
- Assess for exacerbation, proper compliance to medications.
- Assess for difficulty with feedings, changes in respiratory rate, altered sleep patterns, presence of retractions, decreased appetite and weight loss.
- Teach the parents to control or minimize the exposure to allergens.
- Emphasize the need for flu vaccine for patients with no restrictions.
- Limit the exposure to cigarette smoke
- Eliminate dust mites
- Encourage to use air conditioners at home and keep doors and windows closed to minimize the exposure to these triggers.
- Encourage a regular visit to the physician and evaluate the proper usage of equipment.
- Provide a written instruction for management plans when at home, at school or care givers.
- Remind the parents or care givers about the medications, its expiry date as well as having a reserve of it in times of exacerbations.
- Give an easy telephone access when the patient must be taken into the hospital.
Photo credits: http://health.nytimes.com Related posts: - Nursing Care Plan – Brochial Asthma
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