Progeria:  Causes of Death

 

BIOL. 2402 - ANATOMY  & PHYSIOLOGY
RICHLAND COLLEGE
SPRING 2001
Class Project
Web project by Christann Lall

    Children within the first year of life that experience growth failure resulting in

accelerated aging are diagnosed with the disease Progeria.  The rapid aging in the

Progeria victim leads to complications as atheroscleosis and myocardial

infarctions.

    Atherosclerosis is collected fatty deposits and cholesterol in the arterial walls.

Plaques and atheromas are the names for large accumulations in the arteries.  The

fatty tissues break down the elasticity of the arteries effecting the recoil

mechanism interfering with blood flow.  Restricted blood due to the deposits

leads to chest pain.  Arteriosclerosis which is related to Atherosclerosis is calcium

deposits in the arteries.  The deposits in the blood restrict blood flow and cause

pain in the patient in the upper thoracic region.  Atherosclerosis is not detectable

until complications occur; however since Progeria patients are expected to suffer

from Atherosclerosis special tests can be given.

    Blood in the blood vessels swoosh around and gurgle and could be detected by

a stethoscope.  The pulse is also decreased in the clogged region.  Tests that are

used to identify Atherosclerosis are:

      Ultrasound

      CT Scan

      Arteriography

    Cholestyramine, colestipol, nicotinic acid, gemfibrozil, probucol, lovastin are

medications prescribed to reduce the cholesterol and fatty deposits in the blood.

Anticoagulants such as Aspirin are used to inhibit clot formation helping blood

flow.  Balloon angioplasty is a balloon tipped catheter to flatten plaque and

increase blood flow.  Endarterectomy the surgical removal is also used.  The

patient must keep committed to healthy diet which is low in fat, cholesterol, and

salt.  Exercise is recommended to benefit circulation.

    The treatment buys the patient more time for survival however

Atherosclerosis is a progressive disease and leads to further damage of the

tissues.  The patient will suffer a deficiency of blood supply due to obstruction

resulting in damage to the blood vessels, muscles, and organs.  Damage inflicted is

diagnosed as ischemia/angina.  Transient ischemic attack or stroke is probable at

this stage as is acute myocardial infarction which usually end the patient's life.

Coronary artery disease is another probable outcome the patient faces due to

their body's weakness.

    The second reason for Progeria patient's death is myocardial infarction or heart

attack resulting form damaged heart tissue that is starved from oxygen.

Myocardial infarction is a result of Atherosclerosis in the patient.  The blocked

blood vessel cannot transport oxygen to the vital muscles of the heart and leads

to permanent damage to the region of the heart usually the coronary artery.  The

damage shuts down contraction of the region permanently causing positive

feedback until the heart shuts itself down.

    Myocardial Infarction has some symptoms of nausea, chest pain, vomiting, and

shortness of breath.  The pain feels like indigestion, squeezing or heavy pressure

near the sternum.  The patient may feel lightheaded or dizzy, sweaty, and have a

dry mouth.  Impending doom and anxiety are some emotional links to oncoming

heart attack.  Pain may radiate down one side of the body and the arms may feel

heavy.  If the patient is lucky enough to have a test done before having a heart

attack it would show that the pulse is elevated.  The only way to see the MI is by

ECG over two to three day which may be too late.  Most of the test given are

used after the MI has already occurred which is not beneficial for the prevention

but for the benefit of the patient's remaining options.  The following are used to

measure the damage to the heart:

      Coronary angiography

      Nuclear ventriculography

      Echocardiography

      Treadmill

    The physician will perform numerous tests for the Progeria patient to detect

how much damage to the heart has occurred and to determine at what rate of risk

the patient has of Myocardial Infarction.

    Once the patient has had a heart attack the outcome is one third not in their

favor due to the location and amount of damage left on the heart.  In the most

positive outcome is if the patient is alive in two hours of the attack the outlook is

good and recovery is more realistic.  In very rare isolated situations the patient

can resume to regular life activities.  The patient must stay in the hospital for up

to two weeks and sometimes in intensive care unit until stabilized.  ECG's

monitor the patient to avoid dysrhythmias which causes is leading cause of

death.  To decrease the demands on the heart the patient receives a intravenous

catheter for medications and fluids.  An administered for the tissues reducing the

strain on the heart.

    If the Progeria patient survives the MI which is very slim odds due to their

weak bodies they must still adhere to guidelines of a diet low in fat, cholesterol,

and salt and must not take any caffeine.  The patient must control blood pressure

as well.  Nitroglycerin, Beta-blockers, Digitalis, and Calcium channel blockers are

prescribed to lessen the workload on the heart due to oxygen demands.

Morphine is given for pain.  Anti-arrhythmics and diuretics are also prescribed.

Thrombolytic therapy in conjunction with heparin are administered to the patient

within six hours of onset of chest pain.  The therapy prevents the blood from

clotting however it can lead to hemorrhaging.  If all else fails the physician may

be forced to perform surgery to open blocked arteries.  Coronary artery bypass

surgery or angioplasty may be required.  If the physician's attempts to save the

victim are not successful further complications progress.  Arrhythmias such as

ventricular tachycardia, ventricular fibrillation, heart blocks which lead to

congestive heart failure.  Due to the amount of heart damage the patient to fall

into cardiogenic shock or pericarditis.

    The patient's outcome at this point is very grave and chances of survival are

slim.  Progeria patients do not live past their teen years due to Atherosclerosis

and Myocardial Infarctions.  The prevention is impossible and treatments are

unfortunately not successful due to the patient's weak bodies.

INTRODUCTION TO PROGERIA

CELLULAR MECHANISMS

HUTCHINSON-GILFORD SYNDROME

WERNER SYNDROME

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