ECG is a test that measures the electrical activity of the heart and is used to detect possible heart rhythm problems, evidence of injury to the heart muscle, and coronary artery disease. Electrodes are attached to the arms, legs and front of the chest. The heart's electrical activity, communicated via the electrodes, is either displayed on a screen, or recorded as a tracing on paper. The ECG of a healthy heart has a characteristic shape. Any irregularity in the heart's rate, rhythm, or structure, as well as damage from prior heart attacks, can alter the heart's electrical activity, and will show up on the screen or print out. In a stress test or exercise ECG, the person exercises during the recording. The curves are labelled P, Q, R, S and T waves. The P wave represents the SA node and atrial depolarisation; the QRS complex represents ventricular depolarisation; the T represents ventricular repolarisation. The whole pattern is called ‘a complex’ and represents changes occurring during one heartbeat. Normally complexes occur at regular intervals. Variations from normal pattern occur in many heart diseases.

Atrial Fibrillation (AF): a disorganised heart rhythm leading to a rapid, irregular heart rhythm. In AF, the electrical activity of the atria becomes very disorganised with multiple waves of electrical activity cascading throughout the atria. Instead of the atria contracting in sequence, the atria quiver, leading to a 20-25% decrease in the heart’s function. Because the lower chambers receive multiple signals, the heart rhythm becomes rapid and irregular. Patients with AF are often aware of the heart beating irregularly and may notice a decrease in their ability to exert themselves.

Causes: Disease of the heart valves (particularly the mitral valve), previous heart attack, cardiomyopathy, coronary insufficiency, mitral stenosis, hypertension, post-op heart surgery (30% of patients), hyperthyroidism (thyrotoxicosis), excessive caffeine or alcohol, asthma medications, chronic nephritis, lung diseases and bacterial toxins. In many patients, atrial fibrillation occurs for no definable reason. The frequency of atrial fibrillation also increases with age.

Types: Primary (Lone) AF has no identifiable cause or associated abnormalities of the heart. Paroxysmal AF occurs intermittently and varies in frequency and duration from a few seconds to more protracted episodes lasting hours or even days. Lone and paroxysmal AF tend to be more common in people aged 30-50 years. Chronic AF, typically seen in the older population, is persistent becoming the primary heart rhythm and it is usually unresponsive to medical therapy or non-pharmacological interventions (electrical cardioversion). Familial AF, occurring in people under 20, may be influenced by genetic factors. Neurogenic AF (vagal AF - usually in people 30-50 years and adrenergic AF) indicates an imbalance in the nervous system regulation of the heart.

Manifestations: shortness of breath, loss of exercise ability, chest pain, rapid heart beating (as if one's heart is jumping out of one's chest), inability to lay flat, light-headedness and/or loss of consciousness. AF can be difficult to diagnose.

Diagnosis: ECG and event monitor (applied when symptoms of irregular heart beat start).

Treatment: the goal of treatment is to slow the heart rate to normal. Digitalis (digoxin) is the first drug of choice. Other drugs that may be used include beta-blockers and calcium channel blockers (eg. diltaizem, verapamil). AF may be intermittent and may convert spontaneously. If not, medications or electrical conversion of AF may be necessary. Medications to help restore and maintain normal sinus rhythm include amiodarone, propafenone, sotalol, flecainide, procainamide, quinidine, dilatiazem and beta-blockers. If AF does not convert to regular rhythm with medication, cardioversion (an electrical shock delivered to the chest reorganising the electrical activity of the heart) is performed. Warfarin is used to prevent thrombosis and embolism.

Complications: Thrombosis formation if AF has not converted back to normal rhythm within 48 hours of onset, and congestive cardiac failure.

Prognosis: AF may re-occur, AF results in strain on the ventricles and an increased tendency for thrombus formation.

Atrial Flutter: a condition in which the atrium beats 250 to 350 times per minute causing rapid, regular/irregular heart rhythms similar to AF. Atrial flutter differs from AF because the electrical activity of the atrium is rapid but not disorganised. Atrial flutter occurs when an abnormal electrical signal gets stuck rapidly circulating around the atrial muscle tissue. Most cases of atrial flutter occur in patients with underlying heart disease.

Causes: valvular disease, atherosclerosis, thyroid disease, heart failure, swelling and irritation of the endometrium, acute myocardial infarction, hypoxia, pulmonary embolus, electrolyte abnormalities, toxic effects due to medication (particularly digoxin or quinidine).

Manifestations: May be symptomless. Atrial rate over 250, ventricular rate depends on whether impulses arriving at AV node do so during or after its refractory period. Pulse shows missed beats in regular order, sometimes every third beat. Weakness, dizziness, fainting, pain or fluttering in the chest, breathing difficulty and nausea.

Diagnosis: ECG and examination reveals a rapid or irregular heart rhythm and pulse. The heart rate may be between 100 to 175.

Treatment: Heart rate can generally safely be slowed with calcium channel antagonists. Cardioversion (to re-establish normal sinus rhythm), and digitalis (to lower the ventricular rate). Assessment of airway and oxygenation, blood pressure support and rate control with medication. Treat the underlying disorder causing atrial flutter. Avoid procainamide.

Prognosis: The disorder is usually controllable with treatment.

Heart Block: an interference with the normal conduction of electrical impulses that control activity of the heart.

Causes: coronary artery disease, inflammation of cardiac muscle, rheumatic fever, or overdose of certain heart drugs.

Types: sino-atrial node, atria, AV node, bundle of His, fascicles, or a combination of these areas.

Manifestations: Pulse irregular and slowed to 25-40. May also show missed beats in regular order if block not total. ECG shows complete independence between atria and ventricles. Other symptoms include dizziness, fatigue, exercise intolerance, sudden loss of consciousness, occasional convulsions and episodes of acute heart failure. Occasionally heart block is symptomless.

Diagnosis: ECG

Treatment: Treatment depends on the degree of heart block experienced. Some cases need no treatment while others require medication (ephedrine, isoprenaline and beta-1 stimulants) or a pacemaker. Cardioversion or the use of a defibrillator to disrupt ventricular arrhythmia is used in emergency situations. Surgery involving removal of the cardiac tissue causing the arrhythmia is performed when all other treatments fail.

Prognosis: condition will be resolved with medication or a pacemaker.

Stokes-Adams Syndrome (Morgangni, Adams-Stokes disease): a transient condition caused by a heart rhythm disorder where the normal heartbeat passing from the atria of the heart to the ventricles is interrupted, This can result in inadequate blood flow to the brain and fainting.

Causes: inadequate cerebral blood flow associated with a high degree of heart block.

Manifestations: sudden loss of consciousness (with or without convulsions) sometimes with immediate recovery, but often followed by a period of faintness, dizziness, and nausea when the patient tries to sit up.

Treatment: An artificial pacemaker is the most effective form of treatment, although drug treatment may sometimes be used. Rapid recovery (if at all) with marked bradycardia.

Cardiac Arrest: cessation of the heart contractions, once considered irremediable. If circulation is maintained by cardiac massage and oxygenation maintained ventricles may start beating again. Research is being conducted into the effects of cooling the body on survival rate and time. Resuscitation should be started within 3 minutes of cardiac arrest otherwise irreversible changes take place in brain due to anoxia.

D – Danger: assess for danger (remove danger if possible or remove patient to safety).

R – Response: Speak to patient, if no response shake while speaking. If no response, call for help according to emergency procedure

A - Airway: Place victim flat on his/her back on a hard surface. Head-tilt/chin-lift - open victim’s airway by tilting their head back with one hand while lifting up their chin with the other hand.

B - Breathing: Look, listen, and feel for breathing (5-10 seconds). If not breathing, give 5 full breaths into victim’s mouth (use mask).

C - Circulation: check for carotid pulse (5-10 seconds). If there is a pulse but victim is not breathing, give EAR at rate of 1 breath every 5 seconds. If there is no pulse, begin CPR (15 compressions/2 breaths - Rate: 80-100/min). Check for return of pulse every minute.

CONTINUE UNTIL ADVANCED LIFE SUPPORT TAKES OVER.

Research is currently ongoing into the use of cooling to improve the length of time of survival and recovery of people suffering from cardiac arrest.

Digitalis: Derived from foxglove plant (Digitalis purpurea).

Action: Lengthens refractory period of AV node, slows ventricular rate, strengthens ventricular force, and increases stroke volume leading to improved cardiac contractions.

Indications: cardiac arrhythmias, cardiac failure

Precautions: record pulse before administration

Quinidine: Related to quinine.

Action: Lengthens refractory period, slows conduction of impulses.

Indications: atrial fibrillation, atrial flutter, various forms of paroxysmal tachycardia.

Precautions: Trial dose of 200 mg. Continue with full dosage if no nausea, sweating, headache, diarrhoea or rash. Sudden resumption of atrial systole may provoke serious embolism in patients with history of embolism.

Procaine Amide: a controlled release anti-arrhythmic drug

Action: reduces cardiac excitability

Indications: atrial and ventricular tachy-arrhythmia.

Precautions: may lead to complete A-V block, 2nd degree A-V block (unless pacemaker is insitu). Use with caution in myasthenia gravis, broncho-spasm, atrial fibrillation or flutter with abnormal myocardium, marked A-V conduction disturbances, hepatic or renal disease, CHF, prolonged use, pregnancy and lactation.

Diltaizem, Verapamil: calcium channel blockers.

Indications: angina pectoris, supra-ventricular arrhythmias, atrial fibrillation, atrial flutter, hypertension.

Pericarditis: A disorder caused by inflammation of the pericardium.

Causes: bacterial, fungal, or viral infections (HIV, polio, influenza, rubella, adenovirus, and tuberculosis), oesophagus, chest or heart trauma, systemic diseases (cancer, renal failure, leukemia, infections), auto-immune disorders, heart attack, myocarditis, radiation and immunosuppressants.

Types: Bacterial pericarditis and post-MI pericarditis.

Manifestations: sharp, stabbing chest pain (increased with deep breathing; relieved by sitting) which may radiate to the neck, shoulder, back, or abdomen, breathing difficulty, dry cough, abdominal swelling, peripheral oedema, anxiety, fatigue, fever, chills, and sweating. Tachycardia and increased venous pressure occur with fluid accumulation in the pericardium, which interferes with cardiac filling. If fluid develops slowly sac dilates to accommodate it; venous congestion does not occur but enlarged heart displaces lung tissue causing dyspnoea, orthopnoea, and cyanosis. General symptoms if purulent - rigors, intermittent temperature, malaise.

Diagnosis: crackles in the lungs, decreased breath sounds, pericardial rubbing and faint or distant heart sounds on auscultation. Fluid detected by chest x-ray, chest MRI scan, heart MRI or heart CT scan, coronary angiography, echocardiogram. An ECG may show ischaemic-like changes.

Cardiac enzymes (LDH and CPK tests) may be used to rule out acute myocardial infarction. Blood or pericardial fluid culture may show increased WBC counts and ammonium ions.

Treatment: The goal is improved heart function. Treat the underlying cause (eg. antibiotics for bacterial infection). Analgesics for pain, diuretics to remove excess fluid, anti-inflammatories (eg. aspirin, NSAID’s, corticosteroids) to relieve inflammation of the pericardium. Bed rest with the head of the bed raised to reduce the workload on the heart. Pericardiocentesis (removal of excess fluid from the pericardial sac) may be recommended if heart function is compromise by fluid. Surgical pericardiectomy (cutting or removal of part of the pericardium) may be advised if the disorder is chronic or recurrent.

Prognosis: fatal if untreated, outcome is good if treated promptly. Most people recover in 2 weeks to 3 months.

Constrictive Pericarditis: a chronic form of pericarditis caused by inflammation of the pericardium involving thickening, scarring, and contracture of the pericardium and adhesions forming between the layers restricting movement.

Causes: may result from no apparent cause, or may result from tuberculosis, radiation therapy to the chest, cardiac surgery, or infection.

Manifestations: heart sounds weak and distant, progressively worsening dyspnoea, excessive fatigue, weakness, oedema of the legs and ankles, swollen abdomen, ascites, prominent neck veins, radiating chest pain (less than that seen with acute pericarditis) which increases with breathing, splinting of ribs with deep breathing, fever, sweating, chills, dry cough, and anxiety. Venous obstruction - hepatic engorgement, portal obstruction, digestive disturbance, oedema, ascites, jugular pulsation, pounding headache.

Diagnosis: chest x-ray, MRI and CT scan, coronary angiography, and an echocardiogram. The spleen may be examined by touch. Constrictive pericarditis may resemble restrictive cardiomyopathy or cardiac tamponade. An ECG may show changes. Atrial fibrillation is present in one-third of cases.

Treatment: the goal is to improve heart function. Identify and treat the underlying cause (antibiotics, anti-tuberculosis medications, or other treatments). Diuretics to decrease excess fluid. Analgesics for pain. Rest and a low sodium diet may be recommended for some cases. Surgical pericardiectomy is the definitive treatment and may be recommended if scarring is severe.

Complications: cardiac tamponade, heart failure, pulmonary oedema, myocardial fibrosis and damage to the coronary arteries.

Prognosis: may be life threatening if untreated.

Acute Myocarditis: A disorder caused by inflammation of the middle layer of heart muscle.

Causes: complication during or after viral, bacterial, or parasitic infectious diseases (polio, influenza, rubella, rheumatic fever, coxsackievirus, adenovirus, echovirus), radiation, chemical or medication exposure, and connective tissue diseases.

Manifestations: fever, vague chest pain, joint pain, palpitations, rapid heartbeat, signs of CCF, decreased urine output, general oedema, decreased BP, rapid pulse (even when asleep) malaise weakness and occasionally, signs of encephalopathy and atrial fibrillation.

Diagnosis: The diagnosis is made on the basis of symptoms that appear within 6 months of recent infection. Physical examination shows tachycardia and signs of heart failure. Tests used in the diagnosis of myocarditis include: ECG (transient S-T wave changes and reduced QRS voltage), chest x-ray, a biopsy of heart muscle, confirming the diagnosis cultures.

Treatment: The goal of treatment is to reduce inflammation and the amount of heart damage. Treat the underlying cause (antibiotics or other treatments). Treat arrhythmia with digitalis or anti-arrhythmic medications (use digitalis cautiously). Analgesics and anti-inflammatory medications may be used to reduce symptoms. Bed rest and oxygen therapy to reduce the cardiac workload along with a low sodium diet. A heart transplant may be considered if the condition is severe and unresponsive to treatment.

Prognosis: Myocarditis can be very serious, and the outcome depends on the cause. The likelihood of complications varies.

Complications: heart failure, pericarditis, and cardiomyopathy

Prevention: immunisation, sanitation programs, and medications to prevent systemic infections will decrease the incidence if myocarditis.

Chronic Myocarditis (Myocardial Degeneration): An inflammatory condition of the myocardium that persists after a bacterial infection. Chronic myocarditis is characterised by degeneration of muscle tissue and fibrosis or infiltration of interstitial tissues.

Causes: Viral, bacterial, or parasitic infectious diseases (polio, influenza, rubella coxsackievirus, adenovirus, echovirus and rheumatic fever), radiation, exposure to chemicals or medication, or connective tissue diseases.

Manifestations: May be symptomless. Dyspnoea on exertion, rapid, irregular pulse and possibly extra systoles.

Diagnosis: ECG (transient S-T wave changes and reduced QRS voltage), chest x-ray.

Treatment: Treat underlying cause. Lead quiet life free from extra demands on heart.

Prognosis: Slow development, eventually CCF.

Endocarditis: An inflammation of the inner lining of the heart chambers and valves (endocardium).

Causes: Most people who develop endocarditis have underlying heart disease. Bacterial infection is the most common source of endocarditis. In some cases, no causative organism can be identified. Viruses, fungi, cardiac surgery, people with prosthetic heart valves, prolonged IV therapy and/or are IV drug abuser (younger sufferers). Older persons with valvular disease develop subacute endocarditis because of increased likelihood of developing bacteraemia.

Manifestations: fatigue, weakness, fever, chills, night sweats, weight loss, muscle aches and pains, heart murmur, abscess formation, shortness of breath with activity, swelling of feet, legs, abdomen, haematuria, sweating, excessive red skin spots on the palms and soles (Janeway lesions), paleness, nail abnormalities, joint pain, red painful nodes in the pads of the fingers and toes (Osler's nodes). Tachycardia, extreme toxaemia and delirium may be masked by original infection.

Diagnosis: Blood culture, ESR (erythrocyte sedimentation rate), CBC may show low grade, microcytic anaemia, echocardiogram, transesophageal echocardiogram, chest x-ray and CT scan, ASO. A history of congenital heart disease, intravenous drug-use, or chronic localised abscess or infection. Physical examination may show splenomegaly, new heart murmurs (or a change in a previous heart murmur). Examination of the nails may show splinter haemorrhages. Eye examination may show retinal haemorrhages with a central area of clearing (Roth's spots).

Treatment: Hospitalisation for administration of IV antibiotics. Long-term high dose antibiotic therapy is required to eradicate the bacteria from the heart chambers and vegetation’s on the valves. Mixed antibiotic therapy - penicillin, streptomycin, until sensitivity established, then massive doses of antibiotic to which organism sensitive. Therapy up to 6 weeks is not uncommon. Initially bed rest then gradually increased activity as the condition improves. No special diet is necessary, unless it is required because of an underlying heart disorder (such as a low-salt diet).

Complications: congestive heart failure if treatment is delayed (may require valve replacement surgery), blood clots or emboli that travel to brain (stroke, brain abscess), kidneys (glomerulonephritis), lungs, or abdomen causing severe damage, cardiac arrhythmia’s (atrial fibrillation), heart valve damage and jaundice.

Subacute bacterial endocarditis: A chronic bacterial infection of the heart valves.

Manifestations: Insidious onset with lassitude, anorexia, weight loss, anaemia. Embolism due to breaking off of soft vegetation’s from margins of affected valves. Small emboli may lodge in glomeruli (haematuria); skin vessels (petechiae); brain (hemiplegia); kidney (infarct and anuria); limbs (gangrene). Blood vessels may be weakened - aneurisms may occur. Low evening temperature; heart murmur; pallor; finger clubbing; splenomegaly; Osler's nodes; anaemia with leucocytosis.

Diagnosis: as for bacterial endocarditis

Treatment: Prophylaxis for people at risk of rheumatic fever or congenital heart lesions. Procaine penicillin course before and after tooth extraction or tonsillectomy. Curative - penicillin and streptomycin for 6 weeks. Observe carefully for 2 - 3 months to detect relapse early.

Complications: VaWar disease, CCF.

Rheumatic Heart Disease (Rheumatic fever): a condition in which the heart valves are damaged by a disease process that begins with a streptococcal infection.

Causes: Rheumatic fever from streptococcal infection.

Manifestations: See types. Symptoms vary from person to person but can include fever, arthralgia (pain around the joints), leucocytosis, elevated ESR and C-reactive protein (CRP), and prolonged P-R intervals on an ECG.

Diagnosis: Often the damage to heart valves isn’t immediately noticeable.

Treatment: antibiotics to treat streptococcal infection before acute rheumatic fever develops.

Prognosis: If untreated, the streptococcal infection can develop into acute rheumatic fever. May require life long antibiotic treatment to prevent future attacks of rheumatic fever and lower the risk of heart damage.

Complications: CCF.

Valvular Heart Diseases: any dysfunction of the valves of the heart, which causes a disruption of the blood flow through the heart and increases cardiac workload.

Causes: birth defects, rheumatic fever, infection, or the aging process.

Types: mitral insufficiency, mitral stenosis, aortic insufficiency.

General Manifestations: chest pain, shortness of breath, dizziness, fatigue.

Mitral Insufficiency: Occurs when blood regurgitates from the left ventricle to the left atrium during ventricular systole because of incomplete closure of the mitral valve.

Causes: rheumatic heart disease, mitral valve prolapse (a type of mitral insufficiency in which one or more of the mitral leaflets prolapse into the left atrium during systole, resulting in regurgitation of blood from the left ventricle to the left atrium and myocardial ischaemia/infarction that results in damage to the chordae tedineae, papillary muscles, or left ventricular muscle.

Treatment: A patient with effort intolerance or congestive failure and pulmonary oedema is usually treated with digitalis for treatment of paroxysmal atrial tachycardia or atrial fibrillation or flutter. Calcium channel blockers for arrhythmia’s and headaches, sodium restricted diet, diuretics, and limited activity. Valvular replacement is frequently recommended before there is clinical evidence of left ventricular failure. Because endocarditis is a major threat to these individuals, antibiotic prophylaxis is essential. Propanolol may assist in controlling anxiety and arrhythmia’s, and nitroglycerine can be helpful in the treatment of chest pains.

Complications: Atrial fibrillation is common and may cause palpations and symptoms of left ventricular failure; anti-coagulants are recommended to prevent a stroke. Once chronic mitral insufficiency has stabilised, the prognosis is good.

Mitral Stenosis: An obstructive lesion in the mitral valve caused by adhesions on the leaflets of the valve, usually the result of recurrent episodes of rheumatic endocarditis or age related calcification of the valve leaflets. Hypertrophy of the left atrium develops and may be followed by right-sided heart failure and pulmonary oedema. Reduced cardiac output characteristically produces fatigue, dyspnoea, orthopnoea, and cyanosis.

Causes: rheumatic heart disease, bacterial endocarditis, congenital mitral disease, a left atrial tumour, and calcified annulus.

Treatment: digitalis or quinidine to control atrial fibrillation, diuretics, a low sodium diet, and anticoagulants to prevent embolus. Surgery (mitral commissurotomy/mitral valvulotomy - breaking apart the connecting bands of the mitral valve) to increase the size of the orifice. Valve replacement may also be considered (tissue valve or bioprosthesis, caged ball valve prosthesis or tilting disc valve prosthesis).

Complications: paroxysmal nocturnal dyspnoea, orthopnoea, atrial fibrillation, and systemic emboli may occur. CCF and pulmonary oedema may be precipitated by the onset of atrial fibrillation.

Prognosis: depends upon the severity of the mitral stenosis.

Aortic Insufficiency: Aortic insufficiency or regurgitation permits blood from the aorta to return to the left ventricle during diastole.

Causes: congenital anomalies of the heart (eg. inherited disorders such as Marfan's syndrome), rheumatic heart disease (progressive scarring and retraction of the cusps), bacterial endocarditis, (destruction, perforation, or contracture of the cusps) and syphilis (causing aortic dilation).

Treatment: Patients with asymptomatic aortic insufficiency can lead a normal life but should obtain prophylactic antibiotics before dental or operative procedures. When cardiac enlargement occurs, digitalis therapy may be needed and strenuous activity avoided. When symptoms of left ventricular failure become evident, early surgical replacement of the aortic valve is indicated.

Complications: Left ventricular failure occurs late in the disease.

Prognosis: Patients with aortic insufficiency may survive 5 to 10 years. Death usually occurs within 2 years after cardiac failure develops if the aortic valve is not replaced.

Pulmonary Heart Disease: Any condition impeding free flow of blood through pulmonary circulation causing dilatation and hypertrophy of the right ventricle.

Causes: Breakdown of normal lung tissue, tuberculosis, pneumoconiosis, asbestosis, anthracosis and emphysema.

Manifestations: Dyspnoea (on exertion), cyanosis; respiratory infections, bronchitis, right ventricular hypertrophy and CCF.

Diagnosis: ECG, Angiogram with dye.

Treatment: Symptomatic. Open-heart surgery or balloon valvuloplasty may be indicated to correct the defect. Avoid exertion and treat infections promptly.

Atheroma (Ischaemic Heart Disease – IHD; Coronary Artery Disease CAD): atheromatous plaques (ATHEROSCLEROSIS) cause changes in the luminal diameter of the coronary arteries. The lesions may be discrete and localised or involve the greater length of the vessel. Acute changes in the plaques may account for unstable angina, myocardial infarction (MI) and sudden death. Under some circumstances atheromatous plaques may regress. This has been achieved by drugs, which lower LDL cholesterol and triglyceride and increase HDL cholesterol.

Manifestations: The cardinal symptom is angina pectoris (chest pain precipitated by exertion and relieved by rest), which is usually relieved rapidly with sublingual (or spray) glyceryl trinitrate. Dyspnoea frequently accompanies angina. MI pain may be similar but generally begins at rest, lasts longer than 20 minutes, does not respond so well to nitrates and is often associated with feelings of impending death, nausea, sweating and collapse.

There may be evidence of hypertension or hyperlipidaemia. Unfortunately, in many patients with IHD, the first manifestation of the disease may be sudden death.

Diagnosis: The aim of investigations is to confirm the clinical history, to determine the risk of infarction or progression and whether any remediable risk factors may be present. Tests include ECG, exercise test, radionuclide studies (MUGA and 201TI scans to detect ventricular wall motion abnormalities and regional perfusion), echocardiography, doppler ultrasound, ambulatory ECG (Holter) monitoring (to detect arrhythmia’s).

Cardiac catheterisation provides unambiguous information on the location of coronary artery stenoses. Coronary angiography may be performed if surgery or angioplasty is being considered.

Treatment: Low salt, low fat diet and exercise. If uncontrolled, HMG CoA Reductase inhibitors, Bile binding resins (cholestyramine, colestipol), nicotinic acid, oestrogen (post-menopause).

Thrombosis: The formation of a blood clot within a blood vessel. The most common form is Deep Vein Thrombosis (DVT) which occur in the thighs and calves.

Causes: increased platelet and clotting factors as a result of trauma or surgery, post-op bed rest and inactivity, vessel damage, absence of muscle pump (eg. due to paralysis of a stroke etc), long term flexion of limbs which interfere with venous return, use of oral contraceptives, venous infiltration by malignancies.

Manifestations: superficial: tender, hard segments along course of vein, localised reddening, swelling. Severe (DVT): may be symptomless, swelling of affected leg, variation in circumference of leg, tenderness especially when walking, red warm skin over area, discolouration of the limb, Homan’s sign (pain in calf with dorsi-flexion of foot). Shortness of breath, chest pain, sudden unexplained cough, rapid heartbeat pain may also occur.

Diagnosis: physical examination and medical history, doppler ultra-sonography, venography, I-fibrinogen leg-scanning test (fibrinogen coming in contact with thrombosis is traced with a radioscope), impedance plethysmography (measures changes in the electrical resistance of the limb’s blood volume).

Treatment: aspirin, anti-coagulants, NSAID’s, Streptokinase, heat therapy, leg elevation, restrict activity. Surgery is used only cautiously due to the risk of thrombosis formation re-occurring.

Prognosis: 3-6 weeks for recovery (usually related to primary problem being resolved) as long as pulmonary embolism does not occur. Risk of re-occurrence.

Prevention: avoidance of inactivity, early ambulation post-op, use of throbo-embolytic stockings, prophylaxis post-op with anti-coagulants (eg. heparin).

Complications: Severe inflammatory response which may constrict blood flow to leg, possibility of embolus formation leading to pulmonary embolus and/or inferior vena cava interruption, chronic venous insufficiency, risk of superior vena cava syndrome.

Heparin: A parenteral anticoagulant.

Action: accelerates formation of anti-thrombin 3 complex which deactivates thrombin preventing the conversion of fibrinogen to fibrin.

Indications: treatment and prophylaxis for DVT, MI and pulmonary embolism. Indicated for venous thrombosis, atrial fibrillation, open heart surgery, consumptive coagulopathy, patency of IV in-dwelling catheters and post-operative care.

Complications: can induce bleeding, should be used cautiously with other anti-coagulants.

Administration: heparin can only be administered by injection or IV and can be administered during pregnancy and post-natal periods. Observe for signs of bleeding, inflammation, haematoma formation, bleeding and bruising at injection site (rotate injection site), leave needle in situ for 10 seconds before withdrawing, do not massage injection site. Heparin is counteracted by protamine sulfate.

Warfarin: an oral anti-coagulant.

Action: antagonises vitamin K, blocking the production of prothrombin, and clotting factors VII, IX and X. overdose is treated with vitamin K supplements.

Indications: long-term prophylaxis of thrombosis, prevention of DVT and pulmonary embolism.

Complications: can induce bleeding, highly bound to plasma with many drug interactions, can cross the placenta and affect the foetus.

Administration: tablet.

Aspirin: an oral anti-platelet drug

Action: inhibition of cyclo-oxygenase needed to synthesis thromboxane A2, a chemical responsible for platelet aggregation.

Indications: prophylaxis of MI, prevention of re-infarction post-MI and prevention of CVA.

Complications: can induce bleeding, GI disturbance and bleeding.

Streptokinase: a thrombolytic drug from streptococci.

Action: converts plasminogen to plasmin, an enzyme that breaks up the fibrin meshwork of blood clots.

Indications: acute MI, DVT, pulmonary embolism.

Complications: may induce bleeding, antibody production which prevents re-use.

Administration: administered as soon as possible post MI/DVT/embolus to limit the amount of damage that occurs.

Tissue plasminogen activator (tPA; Ateplase): a tissue plasminogen activator created by recombinant DNA technology.

Action: similar to streptokinase.

Indications: acute MI, pulmonary embolism, CVA.

Complications: a safer drug as it does not cause antibody production.

Administration: administered as soon as possible post MI/DVT/embolus to limit the amount of damage that occurs.

Embolism: An embolus is any foreign substance (atheromatous plaques, air, clot, tumour tissue) moving within the circulation. Such an embolus blocks the lumen of smaller blood vessels occluding blood flow (especially dangerous in the heart).

Causes: heart disease, chronic obstructive pulmonary diseases, extended bed rest, surgery, cancer, paralysis, aging, and sickle cell disease.

Manifestations: sudden chest pain, chronic cough, blood-streaked sputum, severe dyspnoea, excessive perspiration, shock, cyanosis, anxiety, loss of consciousness.

Diagnosis: elimination of other lung diseases, V/Q scan (nuclear ventilation-perfusion study of the lungs), pulmonary angiography.

Treatment: anticoagulant therapy (to re-establish blood flow), oxygen therapy, sedatives and surgery to remove the embolism.

Angina Pectoris: chest pain due to coronary heart disease and symptomatic of insufficient cardiac oxygen supply (myocardial ischaemia). Angina is an indicator of increased risk of heart attack.

Causes: physical exercise, strong emotions, extreme temperatures, coronary artery spasm.

Types: typical angina - occurs with stress, variant angina (Prinzmetal's angina) - associated with acute MI, coronary atherosclerosis and coronary artery spasm, it occurs almost exclusively when a person is at rest, Attacks can be very painful and usually occur between midnight and 8 am.

Manifestations: chest pain, shortness of breath.

Diagnosis: ergonovine test (induction of coronary spasm), coronary arteriography/cardiac catheterisation (dye injection traced by high speed x-rays)

Treatment: drugs that affect the supply of blood to the heart or the heart’s demand for oxygen (coronary vasodilators such as Nitroglycreine), anti-hypertensives (reduce cardiac workload). Percutaneous transluminal coronary angioplasty (PTCA, angioplasty, balloon dilation or balloon angioplasty), laser angioplasty and atherectomy are surgical procedures that clear blockages of coronary arteries. Coronary artery bypass graft (CABG) may also be used to detour blood flow around blockages. Variant angina may also be treated with calcium antagonists, organic nitrates and PTCA.

Prognosis: Most people who survive an infarction or this initial three- to six-month period stabilise, and symptoms and cardiac events tend to diminish over time. Long-term survival is excellent, ranging from 89-97% at five years. Patients without significant obstructive coronary artery disease have an excellent long-term outlook.

Prevention: reduce stress, adequate but gentle exercise, weight loss, avoid excessive cold, small meals of easily digested food, rest after meals, and take vasodilator drugs before unavoidable exercise.

Myocardial Infarction: a disorder in which damage to an area of heart muscle occurs because of an inadequate supply of oxygen.

Causes: clot formation or spasm in a coronary artery, artery changes related to atherosclerosis.

Risk factors: smoking, hypertension, diabetes mellitus, high fat diet, high blood cholesterol levels, obesity, male gender, age over 65, overwhelming stress (rare) and heredity. A history of coronary artery disease, cerebro-vascular disease, peripheral vascular disease, angina or kidney failure indicate a higher risk for MI.

Manifestations: Chest pain (ranging from none to crushing) below the sternum which may radiate to the back, abdomen, arms, shoulder, neck, teeth and jaw, which is prolonged (greater than 20 minutes and is unrelieved by rest or medication). Feelings of chest being squeezed, rapid pulse, cough, light-headedness, weakness, shortness of breath, nausea, vomiting, sweating, dry mouth and extreme anxiety. Additional symptoms include seizures, fatigue, breathing (difficulty or temporary absence), lying down and unusual behaviour.

Diagnosis: ECG, coronary angiography, nuclear ventriculography (MUGA or RNV), echocardiography treadmill, blood tests revealing presence of LDH, LDH isoenzymes, CPK, CPK isoenzymes and alteration of other blood test values. Crackles in the lungs, heart murmur, or other abnormal sounds may be heard on auscultation

Treatment: Hospitalisation is usually required for 1 to 14 days. May require ICU or surgery. ECG monitoring immediately to detect life threatening dysrhythmia (the leading cause of death in the first few hours). Decrease cardiac workload – restrict activity and provide oxygen therapy. IV catheterisation for administration of emergency medications and fluids. A urinary catheter may be inserted to closely monitor fluid status. Low sodium, low fat and no caffeine diet. Morphine for pain, organic nitrates and calcium channel blockers to reduce oxygen requirements of the heart, beta-blockers (metoprolol and atenolol) reduce the cardiac workload, digitalis to improve cardiac contractility. Anti-arrhythmics and diuretics may also be prescribed.

Thrombolytic (clot-dissolving) therapy: usually initiated within 6 hours of the onset of chest pain. IV infusion of a thrombolytic medication (Streptokinase or tissue plasminogen activator) immediately followed by IV infusion of heparin (48 to 72 hours). Additionally, oral aspirin and warfarin may be prescribed to prevent further development of clots. Thrombolytic therapy is not appropriate for people who have had a major surgery, organ biopsy, or major trauma within the past 6 weeks or a history of GI bleed, intracranial tumour or stroke within the past 6 months. Also not indicated during pregnancy.

Surgery: angioplasty, CABG

Prognosis: The expected outcome varies with the amount and location of damaged tissue. Damage to the electrical conduction system worsens the outcome. 33% of cases are fatal (survival rate improves if patient is alive 2 hours after onset). After a MI, follow-up care is important to reduce the risk of developing a new MI.

Complications: ventricular tachycardia, ventricular fibrillation, heart block, CCF, cardiogenic shock, pericarditis and extension of the amount of affected heart tissue.

Prevention: Control blood pressure and cholesterol levels, cease smoking, modify diet, control diabetes, lose weight, exercise program to improve cardiovascular fitness.

Hypertension: high blood pressure, the most common cardiovascular disorder. The prevalence of hypertension increases with age. Hypertension is classified as blood pressure greater than 160 mmHg (systolic) or with diastolic pressure greater than 95 mmHg.

Types: primary (essential) and secondary hypertension.

Cause: Unknown, contributing factors include a family history of hypertension, race (more prevalent in African Americans than in White Americans) and age.

Manifestations: persistent headaches, nocturia, and flushed face. Normally hypertension is asymptomatic often discovered when routine tests are being carried out.

Diagnosis: ECG, urinalysis (to examine urinary sediment), serum creatinine, cholesterol, uric acid levels and a renal ultrasound.

Treatment: Initially a weight reduction diet, limit alcohol and salt intake, increase exercise, reduce stress levels and increase relaxation therapies.

Medication therapy includes diuretics, vascular smooth muscle relaxants, angiotensin converting enzyme (ACE) inhibitors and sympatholytic agents.

Complications: If detection is delayed, cerebral vascular accident (CVA), CCF, epistaxis and failing vision.

Secondary hypertension: hypertension as a result of another disease.

Renal hypertension: Reduced renal flow that occurs with reno-vascular disease causes an increase in the released of renin, increasing circulating levels of angiotensin 2. Angiotensin 2 increases blood pressure by producing arteriolar vasoconstriction to increase peripheral vascular resistance which increases aldosterone levels that increases sodium retention by the kidneys.

Pheochromocytoma: A tumour commonly located in the adrenal medulla but can arise at other sites where there is chromatin tissue. Hypertension develops as a result from a massive release of catecholamines - epinephrine and norepinephrine. This release may be paroxysmal rather than continuous causing periodic episodes of headaches, excessive sweating and palpitations, less commonly nervousness, tremor, pallor of the face, weakness, fatigue and weight loss.

Treatment: Surgical removal of operative tumours is curative.

Hypertension in pregnancy: occurs in approximately 10% of all pregnancies.

Diagnosis: based on an increase of 30 mmHg systolic and increase of 15 mmHg in diastolic pressure - measurements are compared to the average values before week 20 of gestation. Hypertension normally occurs after the 28th week giving rise to blood pressure, proteinuria and oedema. If the only symptoms are increased blood pressure, bed rest may be sufficient to alleviate problems. If the patient has proteinuria or oedema admittance to hospital is necessary.

Treatment: Bed rest, increase protein in the diet to counter albumin loss, restrict sodium intake to relieve oedema.

Complications: If detection is delayed eclampsia (which normally occurs in the last three weeks) may occur and the patient may experience convulsions or even become comatose, retinal haemorrhage and blindness may also occur.

Nursing management of hypertension: administer prescribed medications, educate patient to hypertension and the purpose of the therapy, weight reduction diet (with dietician) if the client is overweight,

Prognosis: depends on compliance with medication. Hypertension can be readily managed with medication/

Diuretics: increase fluid loss through the urine

Thiazide diuretics: decrease blood pressure by reducing of blood volume and reduction of arteriole resistance through blockade of sodium and water in the nephron.

High ceiling (loop) diuretic: Produce an increased in fluid loss compared to the thiazide diuretic, for this reason they are not used routinely. They are only used in patients who need greater diuresis than the thiazides produce (eg. in patients with low glomerular filtration rate). High ceiling diuretics lower blood pressure by lowering blood volume and promoting vasodilatation.

Potassium sparing diuretics: fluid loss caused by potassium sparing diuretics is small. They are not real effective when used alone. Because of their ability to conserve potassium they are essential in the anti-hypertensive regime - which is to balance potassium loss caused by thiazide and loop diuretics.

Sympatholytics: depress the influence of the sympathetic nervous system on the heart, blood vessels and other structures.

Beta - Adrenergic Blockers: mechanism of action unknown. They act to decrease heart rate and contractility thereby decreasing cardiac output, suppress reflex tachycardia, decrease production of renin and reduction of peripheral vascular resistance. Beta - adrenergic Blockers should not be used in patients suffering from CCF, 2nd or 3rd degrees AV block, sinus syndrome or asthma.

Centrally acting alpha2 agonists: act on the brain stem to suppress sympathetic outflow to the heart and blood vessels resulting in vasodilatation and decrease in cardiac output.

Adrenergic neuron blocker: decrease blood pressure through inhibiting release of norepinephrine - resulting in decreased sympathetic stimulation of the heart and blood vessels.

Alpha 1 adrenergic blockers: prevent alpha 1 receptors on arterioles and veins - resulting in vasoconstriction - reducing peripheral resistance and venous return to the heart.

Direct Acting Vasodilators (hydralazine and minoxidil): Decrease blood pressure by promoting dilation of arteriole.

Calcium Channel Blockers: work by dilating arterioles.

ACE Inhibitors: Work by preventing the conversion of angiotensin 1 to angiotensin 2 - thereby preventing angio 2 mediated vasoconstriction and aldosterone mediated volume expansion. ACE inhibitors should not be used in pregnancy as they may cause foetal harm.

Angiotensin 2 Receptor Antagonists: they act to prevent angiotensin 2 from occurring, therefore reducing vasoconstriction and volume expansion.

Patent ductus arteriousus: Ductus arteriousus is a normal structure that is open (patent) during foetal development in the womb allowing blood flow around, rather than through the foetal lungs. Patent ductus arteriousus is an unnatural connection which remains open after birth. The defect allows blood from the aorta to flow into the pulmonary artery and to recirculate through the lungs, where it is re-oxygenated and returned to the left atrium and left ventricle causing an increased workload on the left side of the heart and increases pulmonary vascular congestion and resistance.

Manifestations: Cardiomegaly (enlargement of the heart -especially the left side), bounding pulse, tachycardia and atypical murmur which is heard during all of systole and most of diastole.

Diagnosis: Auscultator and radiologic findings sufficient to confirm diagnosis.

Treatment: Surgical correction - however is delayed until the child is old enough to tolerate the procedure.

Complications: If untreated CCF, pulmonary vascular disease, calcification of the ductal site and infective carditis may occur.

Patent atrial foramen: The foramen ovale is a natural inter-atrial channel during foetal development. Most of the blood entering the right atrium is shunted directly to the left atrium, bypassing pulmonary circulation. As soon as the lungs inflate at birth, pressure in the right atrium falls lower than that in the left atrium causing the membrane to close. However in a substantial number of persons the foramen remains at least partially open, however it seems to cause no disability unless other congenital faults exist.

Manifestations: Slight dyspnoea, X-rays show enlarged atria, enlarged pulmonary arteries and over-inflated lungs.

Diagnosis: trans-thoracic echocardiograph with a contrasting medium and trans-oesophageal echocardiograph.

Treatment: None, surgical closure if symptoms become problematic.

Tetralogy of Fallot: a congenital cardiac anomaly that consists of four defects: pulmonic stenosis, ventricular septal defect, malposition of the aorta so that it arises from the septal defect or the right ventricle, and right ventricular hypertrophy.

Manifestations: in the infant - cyanosis and hypoxia, difficulty in feeding, failure to gain weight and poor development. In the older child a typical squatting position and clubbing of the fingers and toes are evident. A pansystolic murmur is usually heard, and the 2^nd heart sound is faint or absent.

Diagnosis: patient history and the physical symptoms which are present.

Treatment: palliative surgery involving the creation of a surgical shunt to increase pulmonary blood flow is often needed during early infancy, with corrective surgery being performed at a later age.

Eisenmenger’s syndrome: a progressive, cyanotic, heart condition where people are usually born with a large hole in the heart.

Manifestations: Cyanosis, clubbing of the fingers, increased red blood cell, breathlessness, fatigue, palpitations, chest pain, fainting and coughing up blood.

Treatment: control symptoms and prevent complications. Medication to treat the rhythm disturbances and optimise muscle control. CCF and oedema may occur and will need to be treated. Surgical repair of the defect after Eisenmengers’ syndrome has developed is not possible. Heart and lung transplantation or lung transplant with repair of the shunt defect are options.

Co-arctation of the aorta: a narrowing of the aorta, proximal or distal to the ductus. Occurs twice as often in males than in females.

Manifestations: disparity in pulsations and blood pressure with higher blood pressure in the upper body than the lower body. Pulse in the lower body is faint and weak and in the upper body the pulse is strong and rebounding.

Treatment: Surgical correction is needed. If untreated patients with a co-arctation have an increased risk of dying between the ages of 20-40.

Dissecting aneurism: an acute life-threatening situation. A dissecting aneurism is a false aneurism resulting from a tear in the intimal layer of the vessel that allows blood to enter the vessel wall, dissecting its layers to create a blood filled cavity.

Predisposing factors: Hypertension and degeneration of the medial layer of the vessel wall.

Manifestations: The major symptom is the abrupt presence of excruciating pain. Blood pressure is moderate to markedly elevated, syncope (brief loss of consciousness caused by transient cerebral hypoxia), hemiplegia or paralysis of the lower extremities may occur.

Diagnosis: history and physical examination of the patient. Aortic angiogram, trans-oesophageal echocardiograph, CT scan and MRI studies.

Treatment: Surgically a segment is removed and replaced with a prosthetic graph. Non-surgically it is important to keep the patient pain free and treat conditions, which may arise (CCF with anti-hypertensive medications and beta-blocking agents).

Prognosis: untreated 20% of patients die within 24 hours, 90% die within three months. With prompt aggressive treatment the mortality rate is 20%.

Thoracic aortic aneurism: an aneurism that develops secondary to one of the following disease process – Marfan’s syndrome, syphilis, hypertension and arteriosclerosis.

Causes: Hypertension and arteriosclerosis weaken the thoracic aortic wall. Marfan’s Syndrome produces fault with the elasticity of the tissues associated with the weakness of the aortic media. Syphilis attacks and weakens the smooth muscles of the aortic wall.

Manifestations: ptosis (drooping of the eyelids), anhidrosis (absent sweating), tracheal deviation, unequal blood pressure in the arms, oedema of the tissues in the head and neck, CCF, dizziness, confusion, dyspnoea, dysphagia, shoulder and neck pain depending on where the aneurism is located.

Diagnosis: Chest X-ray, aortograph, CT scan, MRI and ultrasound.

Treatment: graft to bypass the aneurism.

Congenital aneurism (Berry aneurism): a small, sacular dilation of the wall of a cerebral artery occurring most frequently at the junctures of the vessels in the Circle of Willis. A berry aneurism may be the result of a congenital development defect and may rupture with out warning causing intracranial haemorrhage.

Mycotic aneurism: a localised dilation in the wall of a blood vessel caused by a growth of a fungus usually as a complication of bacterial endocarditis.

Non-surgically: the goal is to improve blood supply to the extremities and stop the progression of the disease. Patient and family education is very important.

Surgically: Pre-operative education involving the patient and family (pain, deep breathing, coughing exercises). Encourage immobilisation of the affect area. Pressure area care (bed cradle, sheepskin, eggshell etc). Offer support and reassurance to maintain calm. Sedatives for anxiety levels and analgesics for control pain. Monitor vital signs for any significant changes. (Signs of shock, haemorrhage, adequate perfusion and signs of embolus, urine output, bowel sounds, assess the dressings and wound sites for signs of redness, swelling, drainage or infection). Pay special attention to the circulation in the extremities and assess for early signs of complications so that prompt treatment can commence.

Raynauds disease: a condition of intermittent attacks of ischaemia in the body’s extremities.

Causes: usually an inherited condition affecting 10% of the total population (more common in females than males) with occurrence more common in colder climates.

Manifestations: very cold, white extremities, intermittent numbness, tingling sensations, burning sensations and pain with attacks lasting from a few minutes to hours. The attacks begin with affected digits turning extremely pale, then to blue and very red before returning to normal colour.

Risk factors: emotional instability, hypertension, excessive sweating, migraine headaches and frequent operation of machinery that vibrates.

Diagnosis: symptoms must be present for two years. Arteriograms detect poor blood flow. Blood tests reveal elevated ESR (erythrocyte sedimentation rate) and increase in ANA (antinuclear antibody) and an increase in CPK (creatine phosphokinase) levels.

Treatment: varies according to the severity of the symptoms. Mild – maintain a constant body temperature, avoid cold and sudden temperature changes, reduce stressful situations and cease smoking. In secondary Raynauds, therapies involves treating the underlying disease, vasodilators are used to dilate the vessels for easier flow of blood.

Thrombo-angiitis obliterans (Buerger’s disease): an inflammatory arterial disorder that causes thrombus formation. The disorder normally affects the lower legs and feet. Normally Buerger’s disease affects men aged between 25 – 40 who are heavy smokers.

Manifestations: pain (related to the ischaemia from the thrombus blocking blood flow, sensitivity to cold weather, diminished or absent peripheral pulse and changes in extremity colour. If left untreated tissues eventually ulcerate and become gangrenous, amputation may be necessary.

For treatment to commence it is mandatory that a person cease tobacco use. Treatment is aimed at producing vasodilatation and preventing tissue injury. Therapies are aimed at treating the symptoms, for example keeping warm, elevating the lower leg to increase blood flow. Surgical treatment involves replacing the affected vessels.

Non-surgically: the goal is to improve blood supply to the extremities, maintain circulation and stop the progression of the disease. Patient education is important.

Surgically: as for aneurism.

ANAEMIAS: an abnormally low number of circulating red blood cells or haemoglobin level, or both, resulting in a diminished oxygen carrying capacity.

Acute haemorrhagic anaemia: the rapid or sudden loss of a large amount of blood resulting in anaemia.

Manifestations: pallor, restlessness, apprehension, hypotensive, diaphoretic, rapid, weak pulse, dyspnoea, shock, thirst, weakness, nausea, faint, dizzy, severe headache, confusion or unconsciousness.

Diagnosis: Blood results show a decrease in RBC count, haematocirt and haemoglobin levels

Treatment: patient history and assessment, control bleeding by applying pressure to wound sites, oxygen to ensure adequate perfusion, transfusion of whole blood packed cells or plasma expanders to restore circulating blood volume. Treat other symptoms and provide support.

Chronic haemorrhagic anaemia (CHA): continuous or frequent small haemorrhages, which depletes the circulating, blood faster than it can be replaced. CHA leads to iron deficiency anaemia when iron stores are depleted rather than decreased blood volume.

Causes: duodenal ulcers, haemorrhoids, intestinal cancer, hookworm infestation.

Manifestations: normally asymptomatic because of the body’s compensatory mechanisms. Symptoms that occur are weakness, lassitude, dyspnoea, pallor, tinnitus, headache, blue sclera, tachycardia and dysphagia.

Diagnosis: Blood results show a decreased haemoglobin and iron level, faeces and urine occult bleeding may occur.

Treatment: Diagnose and treat underlying cause, iron supplements and blood transfusion may be necessary.

Haemolytic anaemia: a disorder characterised by premature destruction of red blood cells with retention in the body of iron and other products of red blood cell destruction.

Causes: Intrinsic factors include defects of the red blood cell membrane, various haemoglobinopathies or inherited enzyme defects. Extrinsic factors include drugs, bacterial toxins, antibodies and physical trauma.

Treatment: some cases respond to splenectomy, others respond to corticosteroid hormones, others do not respond until the primary disorder is resolved.

Prognosis: depends on cause and individual response to therapy.

Erythroblastosis Foetalis: a type of haemolytic anaemia that results from maternal foetal blood group incompatibility, specifically involving the Rh negative factor and the ABO blood groups. First child usually unaffected, later children suffer severe consequences.

Causes: antibody reaction in the blood stream of the infant caused by placental transmission of maternally formed Rh-negative antibodies against the incompatible antigens of the foetal blood.

Manifestations: Child born anaemic and jaundiced, placenta enlarged, compensatory hyperplasia, enlarged spleen and liver (leading to hypoxia, cardiac failure, liver damage, respiratory distress, oedema, kernicterus, risk of permanent brain damage and stillbirth.

Diagnosis: pre-natal amniocentesis for presence of antibodies and bilirubin levels.

Treatment: Immunisation with Rh immune globulin injection prevents sensitisation in Rh-negative mothers but is useless if administered after sensitisation occurs. Intrauterine transfusion to the foetus. Exchange transfusion to the foetus.

Prognosis: fatal if undetected and untreated. Greater than 80% survival rate of foetus with quick appropriate treatment.

Pernicious anaemia: a progressive megaloblastic macrocytic anaemia that results from lack of cyanocobalamin (vitamin B12).

Causes: diminished intestinal absorption of vitamin B12.

Manifestations: extreme weakness, numbness, tingling in the extremities, fever, pallor, anorexia and weight loss.

Diagnosis: Full blood examination and examination of the red marrow may show signs of enlarged nucleated red blood cells and scanty normoblasts.

Treatment: immediate transfusion of packed cells if red blood cell count is low. Initial large dose of vitamin B12 will be needed and the follow up doses until erythrocyte count is normal, then maintenance doses will be required.

Iron Deficiency Anaemia (IDA): A microcytic, hypochromic anaemia caused by insufficient iron to synthesise haemoglobin (effects 2 in 1000 people with women having a higher risk).

Causes: insufficient iron in the diet, haemorrhage (acute and chronic), foetal demands on mother, deficient iron absorption, failure to utilise available iron, decreased acidity of gastric fluids and lead poisoning in children.

Manifestations: fatigue, weakness, shortness of breath, orthostatic hypotension, pallor of skin and mucous membranes, dry scaly skin, sore tongue, brittle nails, irritability, headache, decreased appetite (especially in children) and unusual food cravings. Asymptomatic if the anaemia is mild.

Diagnosis: morphological examination of red blood cells, low hematocrit and haemoglobin in CBC, low serum iron level, transferrin saturation, stool for occult blood, TIBC (Total Iron-Binding Capacity).

Treatment: Identification and treatment of the underlying cause of the IDA. Obtain history, assess for GI, respiratory and cardiovascular symptoms. Bed rest, warm environment, assess for skin breakdown. Increased dietary iron, iron supplements (Oral - ferrous sulfate; IM or IV - iron dextran and iron sorbitol) and blood transfusions. Iron supplements can be toxic to children even in small doses.

Prognosis: blood counts should return to normal in 2 months, but therapy should continued for 6-12 months. IDA may recur, so regular follow-up is encouraged. Children may have an increased susceptibility to infection.

Haemopoietic Anaemia (aplastic anaemia): acute or chronic anaemia (effecting 1in 500,000 people) caused by destruction, derangement or replacement of haemocytoblasts resulting in a failure to properly form all types of blood cells.

Causes: injury to the stem cells (possibly an auto-immune process), drugs, toxins, radiation exposure, polycythaemia, pregnancy, congenital disorders (leukaemia, neoplasm, systemic lupus erythrematosus).

Manifestations: Increased risk of infection and haemorrhage, fatigue, weakness, pallor, shortness of breath on exertion, rapid heart rate, irregular heartbeat, rash, easy bruising, nose bleeds, bleeding gums, prolonged bleeding time, lymph nodes may be enlarged (rare).

Diagnosis: examination reveals enlarged spleen, sternum tenderness and irregular heart rate; CBC shows low hematocrit, haemoglobin, white blood cell count, reticulocyte count and platelet count, elevated bilirubin; abnormal bone marrow biopsy, abdominal x-ray or CT, a sugar water (hemolysis test) shows fragile red blood cells.

Treatment: Mild cases are treated with supportive care. Blood and platelet transfusions to correct abnormal blood counts. Bone marrow transplant for people 30 and under. Adults over 40, or people without a bone marrow donor, are prescribed anti-thymocyte globulin - ATG (a horse serum that contains antibodies against human T cells). Immunosuppressants (eg. Cyclosporine) may be used. Other nursing care is similar to IDA.

Prognosis: Untreated haemopoeitic anaemia leads to rapid death. Bone marrow transplantation, gives a long-term survival rate of 80% in young people. Older people have a survival rate of 40 to 70%.

Complications: rejection of bone marrow graft, or severe reaction to ATG.

Polycythaemia: An abnormal increase in the number of circulating erythrocytes resulting from increased production by the bone marrow.

Types: (1) Polycythaemia vera (2) Secondary (3) Stress polycythaemia (pseudo-polycythaemia).

Polycythaemia vera: an acquired disorder, which occurs more frequently in men, and is rarely seen in those under 40 years old. The viscosity of the blood and the increased platelets result in a high potential for clot formation. Risk factors include smoking, heart or lung disease, stress and family history of polycythaemia. The incidence of polycythaemia vera is highest among people of Jewish ancestry.

Causes: unknown.

Manifestations: haemorrhage (due to altered platelet function), headache, dizziness, fatigue, fainting, itchiness, enlarged spleen and liver, red coloration of the face, vision abnormalities, shortness of breath, breathing difficulty when lying down, pain in bones and symptoms of phlebitis.

Diagnosis: CBC with differential, elevated hematocrit, elevated white blood count, elevated platelet count, bone marrow biopsy, vitamin B12 level, chemistry panel, increased blood volume.

Treatment: aims to reduce the high blood viscosity and to prevent haemorrhage or thrombosis. Phlebotomy (to remove excess blood volume) and chemotherapy (to suppress bone marrow). The use of anti-platelet therapy such as aspirin is controversial as it may cause gastric bleeding. Allopurinol is given for hyperuricemia (gout) caused by altered uric acid levels. Anti-itching medications and H2-receptor inhibitors or antacids for hyperacidity. Treat symptoms as they occur, maintain hydration levels assess for signs of hypovolemia, infection and DVT, encourage ambulation and maintain pressure area care.

Prognosis: The median survival is 11 to 15 years.

Complications: thrombosis (leading to CVA and MI), peptic ulcer disease, gastric bleeding, gout, kidney stones, leukemia, myelofibrosis.

Secondary polycythaemia: Normal response to low level of circulating oxygen or when extra demands for oxygen are made. Occurs in trained athletes, persons living at great heights above sea level, persons with impaired respiratory surface.

Cause: congenital heart disease; chronic lung disease; smoking; high altitude environments.

Manifestations, Diagnosis and Treatment: as for polycythaemia vera.

Prevention: Avoid smoking, dehydration and seek medical treatment for heart or lung disease.

Cause: use of diuretic drugs; smoking; dehydration.

Manifestations, Diagnosis, Treatment and Prevention: as for secondary polycythaemia.

Thalassemia (Cooley's anaemia): A condition of inherited haemolytic anaemia causing the production of defective haemoglobin. Thalassemia minor (usually symptomless, but person is a carrier) occurs with 1 defective gene, thalassemia major (occurs with 2 defective genes) has 2 types. Alpha thalassemia occurs in people from south-east Asia and China, Beta thalassemia mainly in people of Mediterranean origin. Affected children appear healthy at birth but develop anaemia during the first year of life.
Manifestations: Fatigue, shortness of breath, jaundice, enlarged spleen, bony deformities (mongoloid appearing faces).

Diagnosis: pre-natal screening, peripheral blood smear, CBC reflects anaemia and low MCV, haemoglobin electrophoresis.

Treatment: focuses on dealing with symptoms. History, treat anaemia, educate to risks, assess for infection signs, assess for cardiac irregularity maintain hygiene. Regular blood transfusions and folate supplements may be given, assess for signs of iron overload. Bone marrow transplant is being investigated as a treatment.

Prognosis: In severe thalassemia, death from heart failure usually occurs between the ages or 20 and 30. The prognosis of thalessemia appearing early in life (6 months to 1 year of age) is poor.

Complications: Heart, liver, and endocrine systems damage may occur from iron overload from blood transfusions.

Sickle cell anaemia: An inherited, chronic blood disease in which the red blood cells become crescent shaped and function abnormally when exposed to dehydration, infection, and low oxygen supply.

Causes: Sickle cell anaemia is caused by inheriting an abnormal type of haemoglobin called haemoglobin S, producing a chronic anaemia, which may become life threatening when haemolytic or aplastic crises occur. People who have inherited haemoglobin S from both parents suffer sickle cell anaemia, inheritance from one parent, leads to the person being a carrier without symptoms. The overall incidence is 8 out of 100,000 and occurs primarily in people of African descent.
Manifestations: fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections, ulcers on the lower legs, jaundice, bone, joint and abdominal pain, weakness, fever, vomiting, chest pain, haematuria, excessive urination, excessive thirst, penis pain, priapism and decreased fertility. Symptoms of sickle cell anaemia are most severe during periods called ‘sickle cell crisis’.

Diagnosis: pre-natal screening, CBC, haemoglobin S screening test, haemoglobin electrophoresis, sickle-cell test.

Treatment: There is no cure available for sickle cell syndrome. Therapy aims to control symptoms relating to crises. Bed rest and oxygen therapy (to avoid low oxygen levels, which cause the sickle cell crisis). Continuous folic acid supplementation, electrolyte replacement, analgesics, pressure area care, ROM exercises and adequate hydration for acute, painful episodes. Splenectomy may be indicated. Blood transfusions may be given for aplastic or haemolytic crises, but should not be given routinely. Research is currently ongoing with hydroxyurea, which can induce some patients to produce a more normal blood protein.

Prognosis: Repeated crises can lead to damage of the kidneys, lungs, bone, liver, and CNS. Death from organ failure frequently occurs between the ages of 20 and 40.

Prevention: Avoid strenuous physical activity, emotional stress, low oxygen environments and known sources of infection to prevent tissue deoxygenation, recognise signs of dehydration, avoid excess exposure to the sun, adequate fluids and wear a Medic Alert Bracelet.

Complications: recurrent aplastic and haemolytic crises, multi-system disease, necrosis of the kidney.

Agranulocytosis (Granulocytopenia; Neutropenia): a reduction in number of circulating granulocytes and neutrophils causing the patient to become susceptible to infection.

Causes: Destruction of parent cells in bone marrow by drugs and radiation. A rare form, infantile genetic agranulocytosis, is inherited.
Manifestations: Fever, aches, sore throat, pusless ulcers (especially in the mouth and throat) rapid onset of severe mouth, throat, respiratory, and GI tract infections.

Diagnosis: Self-observation of symptoms, medical history and physical exam. Laboratory studies of blood and bone marrow, and cultures of blood, nose, throat and urine.

Treatment: Immediate cessation of causative agent. Antibiotics for specific infections. Lithium to stimulate bone marrow and possibly transfusions of white blood cells. Hospitalisation for intensive treatment during the active phase, with reverse isolation techniques. Maintain personal and oral hygiene.

Prognosis: usually curable with intensive treatment.

Complications: Kidney damage, dangerous, sometimes fatal infections. May be an early sign of leukemia or aplastic anaemia.

Leukaemia: Malignant change in leucocytes or lymphocytes. Secondary anaemia due to crowding out of crythroblasts. The acute varieties are similar to each other and often difficult to distinguish.

Types: Lymphocytic leukaemia (acute and chronic); Myeloid leukaemia (acute and chronic).

Acute Lymphocytic Leukaemia: (ALL or acute childhood leukemia)

A progressive, malignant disease characterised by large numbers of immature white blood cells in blood, bone marrow, lymph nodes, spleen and other organs.

Causes: no apparent cause. Abnormalities in chromosomes, radiation, toxins (such as benzene) and some chemotherapy agents may contribute to the induction of ALL. Risk factors for ALL include Downs Syndrome, a sibling with leukemia, and exposure to radiation, chemicals, and drugs. The incidence of ALL is 6 out of 100,000 people (20% of adult leukemia; 80% of acute childhood leukemia, with the peak incidence occurring between ages 3 and 7).

Manifestations: enlarged spleen and liver, prolonged bleeding, bruising, bleeding gums/nose/into the skin, abnormal menstrual periods, skin rash/lesions, petechiae, pale skin, fatigue, increased risk of infection, bone pain or tenderness, joint pain, lymphadenopathy (enlarged lymph nodes), weight loss, fever, swollen gums, shortness of breath aggravated by exercise and heart palpitations

Diagnosis: Physical examination shows enlarged liver and spleen, bruising (ecchymosis) and evidence of bleeding. WBC count is abnormal, a CBC shows anaemia and low platelet count and bone marrow aspiration shows an increased number of cells (hypercellularity) and an increase in lymphoblasts.

Treatment: The goal is remission of the cancer. Isolation procedures in hospital may be necessary if the lymphocyte count is very low to prevent exposure to infectious agents. ALL is treated with a combination of 3-8 drugs, which may include: prednisone, vincristine, methotrexate, 6-mercaptopurine, and cyclophosphamide. Provide analgesia for pain and administration of blood products to correct anaemia and low platelet counts may be necessary. Prevent haemorrhage, injury and falls. Antibiotic therapy to treat secondary infections. After remission is achieved, chemotherapy and/or radiation therapy is administered in the spinal column to treat any leukaemia cells that may have invaded the spinal fluid. Subsequent therapy is directed at preventing relapse. A bone marrow transplant after high-dose chemotherapy is a treatment option for cases that relapse or do not respond to other treatments.

Prognosis: Without treatment, the life expectancy is about 3 months. 95% of children enter remission while the cure rate is 50-60. 80% of adults enter remission, while the cure rate is 30-50%.

Complications: DIC (disseminated intra-vascular coagulation), relapse of ALL, severe infection.

Chronic Lymphocytic Leukaemia (CLL): A malignancy of lymphocytes, characterised by a slow progressive increase of these cells in the blood and the bone marrow, causing immunosuppression, failure of the bone marrow, and infiltration of malignant cells into organs. CLL occurs in the elderly with 90% of the cases in people over 50 years old (especially in people with auto-immune diseases). The incidence increases with age to 15 out of 100,000 by 70 years old. Many cases exhibit no symptoms and are detected by routine blood tests.

Causes: The cause of CLL is unknown.

Manifestations: enlarged lymph nodes, liver, and spleen, fatigue, bone pain, abnormal bruising, excessive sweating, loss of appetite, weight loss, itching, flank pain and armpit lump.

Diagnosis: markedly elevated WBC count in CBC, bone marrow aspiration. Treatment: The early stages of CLL require no specific therapy. Chemotherapy or radiotherapy may be indicated for fatigue, anaemia, thrombocytopenia, or enlarged lymph nodes that are troublesome. Blood transfusions or platelet transfusions may be required.

Prognosis: Progression of the disease is slow. About 50% of people with CLL survive for 6 years, and 25% live more than 10 years.

Complications: increased risk of secondary malignancies, side effects of chemotherapy, auto-immune haemolytic anaemia, hypo-gamma-globulinemia and increased susceptibility to infection.

Acute Myeloid (Myelogenous) Leukemia (AML): a malignancy of blood-forming tissues characterised by the proliferation of immature granular leucocytes. AML occurs primarily in adults and children below 1 year old. In AML, the malignant cell loses its ability to differentiate and multiplies rapidly causing bone marrow failure as malignant cells replace normal bone marrow elements. The person becomes susceptible to bleeding and infection. The incidence of AML is 1 in 10,000 people.

Causes: no apparent cause. Risk factors include exposure to radiation and chemicals, immunosuppression following organ transplantation, and blood disorders (polycythaemia vera, essential thrombocytopenia, and refractory anaemia).

Manifestations: prolonged bleeding, bleeding gums/nose/into the skin, abnormal menstrual period, skin rash/lesion, petechiae, bruising, fatigue, infection (children under 1 year of age are extremely susceptible to streptococcal septicemia), fever, bone and joint pain/tenderness, lymphadenopathy (enlarged lymph nodes), weight loss, swollen gums, shortness of breath (aggravated by exercise), paleness, irregular palpitations.

Diagnosis: A physical examination shows enlarged liver and spleen. A WBC count is abnormal. A CBC shows anaemia, low platelet and reticulocyte (lymphadenopathy) counts. Bone marrow aspiration shows hyper-cellularity. Children have bone and joint manifestations with areas of bone destruction and periosteal elevation by x-ray.

Treatment: The objective of treatment is to eliminate the malignant cells with chemotherapy. Normal bone marrow cells take several weeks to recover and start producing normal cells. During this time supportive care consists of patient isolation to prevent infection, antibiotics to treat infection, transfusions of platelets to control bleeding, and red blood cell transfusions to combat anaemia. Intensive chemotherapy, or chemotherapy in conjunction with bone marrow transplant, are potential curative treatments after remission has been achieved. The US FDA has recently approved a new drug called Vesanoid (all-trans retinoic acid) to treat promyelocytic leukaemia, a rare but lethal form of AML leukemia. In preliminary clinical trials, this drug has resulted in a complete remission in half of the patients who failed standard treatment.

Prognosis: Without treatment, life expectancy is about 3 to 4 months. Complete remission occurs in 70% to 80% of patients. Long-term survival achieved by curative therapy occurs in 20% to 30% of people.

Complications: relapse of the disease and severe infection.

Prevention: minimise occupational exposure to radiation and agents associated with the development of acute leukaemia.

Chronic Myeloid Leukaemia (CML): (chronic granulocytic leukemia; chronic myelocytic leukemia): A malignant cancer of the bone marrow that causes rapid growth of the myeloid precursors in bone marrow, peripheral blood and body tissues. CML is characterised by a chronic phase, then a subsequent transformation to a more malignant blast phase which is very difficult to treat. The disease may remain stable in the chronic phase for years before becoming overtly malignant. Acceleration of the disease may be associated with fever without infection, bone pain, and an enlarged spleen. In most cases the disease progresses to a blast crisis within 5 years.

Causes: The disease tends to occur in middle-aged people and is associated with a chromosome abnormality called the Philadelphia chromosome. Known risk factors are exposure to ionising radiation and benzene.

Manifestations: Fatigue, weakness, excessive sweating, low-grade fever, pressure under the left ribs from an enlarged spleen, increased risk of infection, bleeding and bruising, petechiae, bone pain, penis pain and painful menstruation.

Diagnosis: A physical examination reveals an enlarged spleen. A CBC shows increased WBC count, bone marrow aspiration, presence of the Philadelphia chromosome and leucocyte alkaline phosphatase level.

Treatment: Treatment is aimed at suppressing the bone marrow and may induce a remission with good control of the signs and symptoms of the disease. The chronic phase can be controlled with chemotherapy, which can be given as an outpatient treatment. Interferon therapy has recently been used to achieve temporary remission. A bone marrow transplant preceded by high dose chemotherapy and radiation therapy has led to long-term disease-free survival in some people.
Prognosis: Without curative treatment, the disease is fatal. Median survival is 3 to 4 years. About 50% of those who receive a bone marrow transplant have long-term disease-free survival.

Complications: blast crisis chronic myelogenous leukemia.

Hodgkin's disease (Hodgkin's lymphoma): a cancer which causes the lymphatic tissue to become enlarged leading to pressure on nearby structures. Hodgkin’s disease can spread through the lymphatic vessels and the blood vessels. Hodgkin's disease is most common in early adulthood (ages 15-40), and late adulthood (after age 55).

Causes: drug abuse, infectious mononucleosis, reduced immune systems (AIDS and organ transplant patients). There does not appear to be any genetic or dietary factors in development of Hodgkin’s disease.

Manifestations: may be symptomless. Enlarged spleen and liver enlarged, painless lymph nodes (causing pressure symptoms - coughing, shortness of breath, fever, night sweats, weight loss, itching, tiredness, and decreased appetite) and progressive anaemia.

Diagnosis: There are no screening tests to provide early detection of Hodgkin's disease. Medical history, physical examination and lymph node biopsy

Treatment: hospitalisation during treatment with low level radiotherapy to local swellings. Chemotherapy includes MOPP (Mechlorethamine, Oncovin [vincristine], Procarbazine & Prednisone) or ABVD (Adriamycin [doxorubicin], Bleomycin, Vinblastine & Dacarbazine). Assess and treat side effects of chemotherapy.

Prognosis: Treatment for most cases of Hodgkin's disease is very effective. The 1-year relative survival rate after treatment is 93%; the 5-year and 10-year rates are 82% and 72% respectively. At 15 years, the overall survival rate is 63%.

Thrombocytopenia: any bleeding disorder resulting from of decreased or dysfunctional platelets (usually a symptom of another disease).

Causes: Deficient platelet production (marrow injury from radiation, immuno-suppressive drugs, thrombopoietin, vitamin B12, iron deficiency), accelerated platelet destruction (immunological processes – auto-antibodies, haemolytic anaemia, anaphylactic reactions, non-immunological processes – disseminated intra-vascular coagulation, infection, haemorrhage), abnormal pooling of platelets within the body – splenomegaly)

Types: Primary – Acute idiopathic thrombocytopenia purpura – most common in children and young adults usually as a result of viral infection. Chronic idiopathic thrombocytopenia purpura – most common in women between 20-50 years, usually results from auto-immune platelet destruction.

Secondary – resulting from auto-immune disorders, drug reactions, heparin therapy (white clot syndrome), bacterial and viral infection.

Manifestations: prolonged bleeding time, spontaneous bleeding, haematoma, epistaxis, oral bleeding, petechiae. Other symptoms may be present depending on the specific disorder.

Diagnosis: CBC and platelet counts, bone marrow aspiration/biopsy, PTT (coagulation studies), PT (coagulation studies), platelet associated antibodies, platelet aggregation test, capillary fragility determined by tourniquet test (BP cuff applied for 5 minutes – inflated to mid point between diastolic and systolic BP, number of petechiae formed counted).

Treatment: Some types will resolve without treatment. No treatment in mild cases. Obtain history, bed rest, document blood loss, minimise risk of injury, maintain hygiene, avoid injections, valsalva manoeuvre and anti-coagulants, administer analgesics and stool softeners and maintain pressure area care. Splenectomy in severe primary condition. Transfusion of platelets in severe haemorrhage with anaemia. Corticosteroids for chronic ITP. Treat underlying cause in secondary type.

Prognosis: The outcome depends on the causative disorder.
Complications: cerebral haemorrhage, GI bleeding (vomiting blood or blood in stools), haematuria, pulmonary complications (bleeding into diaphragm), nerve pain.

Haemophilia: a bleeding disorder in which one of the essential blood clotting factors is deficient, leading to a break in the cascade process of blood clotting which prolongs bleeding time. Haemophilia is hereditary effecting males almost exclusively, and is passed on through females who carry the defective genes. Haemophilia affects approximately 1 in 6,500 males.

Types: Haemophilia A (Classical Haemophilia), the most common form of haemophilia, is caused by a deficiency of Factor VIII. Haemophilia B (Christmas Disease) is caused by a deficiency of Factor IX.

Cause: Haemophilia A & B (Factor VIII and IX deficiencies) are X chromosome linked genetic disorders. Haemophilia occurs in males who inherit the defective gene from their mother. Female children who inherit the defective gene are carriers and do not suffer from haemophilia. If the faulty gene is passed on from the father, male children will be unaffected but female children will be carriers. The severity of haemophilia is related to the level of deficiency of the clotting factor.

Manifestations: Minor injuries result in prolonged haemorrhages and clotting time (1 - 12 hours). Bleeding is mostly internally into the joints and muscles and may occur or as a result of trauma. Bleeding is detected by warmth, swelling from the affected area and bruising. The person with the bleeding disorder will often be able to tell they are having a bleed before signs are visible.

Diagnosis: The first signs occur in early infancy when crawling and walking are first attempted. Bleeding will be difficult to control. Painful swelling or a reluctance to use an arm or leg is an indication that a bleed has occurred. Detection of haemophilia before birth is possible by amniocentesis and foetoscopy.

Treatment: There is no cure for haemophilia therefore vigilance is needed to prevent injuries. Haemophilia is managed by infusion (replacement therapy) with clotting factor concentrates when bleeding is suspected. Physiotherapy and immobilisation of affected area of the body can help to reduce damage caused by bleeding.

Prognosis: can be controlled but requires life long vigilance.

Prevention: A person with haemophilia should control weight as extra weight produces unnecessary stress and strain on weight bearing joints and increases the dose of concentrate required during replacement therapy. A non-contact aerobic sport such as swimming strengthens joints and muscles, helping to prevent bleeds while the water provides a natural cushion for the limbs.

Complications: Infant immunisation and other injections should be given subcutaneously. Surgery should always be undertaken at a major hospital to allow for immediate replacement therapy. Drugs containing aspirin should never be taken for analgesia. Over a period of time bleeding into joints and muscles causes permanent nerve, muscle and joint damage and chronic pain.

Complications of replacement therapy include infection by blood-borne viruses. Some people also develop antibodies to the clotting factors.

Vitamin K Deficiency: Inadequate or absent vitamin K, a fat-soluble vitamin necessary for proper blood clotting leads to a bleeding disorder. A newborn infant lacks vitamin K until its body begins to produce it.

Cause: Gall bladder disease, malabsorption disorders (coliac disease, pellagra), obstructive jaundice, hepatitis, Crohn's disease, ulcerative colitis, cystic fibrosis, excessive amounts of anti-coagulant drugs (warfarin or dicumarol), prolonged use of antibiotics.

Manifestations: Unusual bleeding from the gums, nose or GI tract and unexplained bruising.

Diagnosis: Medical history and physical examination. INR and Prothrombin time.

Treatment: history and assessment for signs of hidden bleeding. Provide vitamin K in the diet or oral/IV supplements.

Prognosis: Curable with vitamin K supplements oral or injection.

Prevention: vitamin k rich diet. Injections of vitamin K are given to newborn infants and to persons with gall bladder disease or malabsorption disorders to prevent deficiency.

Complications: Severe or fatal haemorrhage.

ABO grouping: Erythrocytes classified into 4 types according to presence of certain proteins, which, under suitable conditions, cause them to clump together, (ie. to agglutinate). These substances are called agglutinogens. ‘A’ type agglutinogen occurs in ‘A’ type cells, ‘B’ type agglutinogen occurs in ‘B’ type cells, both types of agglutinogens occur in ‘AB’ cells, type ‘O’ cells have no agglutinogens. When, erythrocytes come in contact with plasma antibodies called agglutinins - anti-A and anti-B, agglutination occurs.

Cross matching recipient and donor blood: Sample of recipient's serum mixed with sample of donor’s erythrocytes. If agglutination occurs, bloods are incompatible. Generally it is safe for a recipient to receive blood of his own group.

Universal donor: Group O cells contain no agglutinogens and are therefore unaffected by agglutinins that may be present in recipient’s blood. Hence, in emergencies, it is safe for any person to receive group O blood.

Universal recipient: Group AB blood contains no agglutinins hence it is safe for persons of group AB to receive any blood.

Other causes of agglutination: RH Factor. About 85% of people have this factor and are Rh-positive while the remaining 15% are Rh-negatives. If an Rh-negative receives Rh-positive blood, the Rh-negative person becomes sensitised and produces antibodies to the Rh factor. If on a future occasion more blood of Rh-positive type is received, or a Rh-positive foetus is conceived, the antibodies will cause agglutination of the Rh-positive cells. Several other proteins (Duffy, Lutheran, Kell, Lewis, Kidd, MNS, P, Diego) can have a similar effect.

Haemolytic reaction: Transfusions of a different blood type than that of the patient. The resulting destruction of erythrocytes releases free haemoglobin into the blood, which can block small blood vessels and nephrons leading to acute renal failure.

Non-haemolytic reaction: urticarial and febrile reaction caused by IgA antibody reaction to leucocytes. Less serious than the haemolytic reaction.

Other causes of reaction include transfusions from donors who carry infections, Multiple blood transfusions, Rh negative mother.

Haemolytic: Chills, fever, urticaria, tachycardia, pain/tightness in chest, shortness of breath, nausea, vomiting, lumbar pain, cloudy/reddish urine, crackles, hypotension, dyspnoea, wheezing, burning sensation along veins.

Non Haemolytic (allergic): urticaria (hives), pruritis, oedema of face/tongue/throat, asthmatic respirations, pulmonary oedema, anaphylaxis.

Pyrogenic (bacterial): severe hypotension, fever, chills, dry flushed skin, extremity and abdominal pain, vomiting, bloody diarrhoea.

Treatment: Haemolytic/Non-haemolytic: Stop transfusion at once, saline flush, notify doctor and lab. Administer fluids, oxygen and epinephrine (haemolytic reaction). Obtain urine sample, administer diuretics and anti-pyretics as prescribed, transfuse with properly matched blood if prescribed and monitor obs every 5 min (15 min for non-haemolytic) until stable.

Pyrogenic: stop transfusion, saline infusion, notify doctor and lab. Return unit of blood to lab for testing. Obs 15-30 minutes, observe for signs of shock and hypotension. Administer prescribed broad-spectrum antibiotics, cortico-steroids and IV fluids. Insert IDC, monitor fluid balance

Prevention: adhere to transfusion safety protocols, ensure blood for transfusion is correct to paperwork. Obtain history of past response to transfusion if known. If transfusion is part of planned surgery, have patient donate and store blood 1 month prior to surgery.

Complications: Acute kidney failure, anaphylaxis, CCF.

Prognosis: Most reactions clear gradually after the transfusion is halted. A few reactions are fatal.

Over-transfusion: circulatory system is overloaded with more blood than it normally contains.

Causes: excessive transfusion of blood products during emergency or treatment for anaemia and leukemias.

Manifestations: Skin becomes red, hypertension, pounding headache, awareness of heartbeat, oedema.

Treatment: Venesection.

Prognosis: Full recovery with prompt attention.

Complications: Haemorrhage especially from wounds, pulmonary oedema. CCF.