ALS

What is ALS

For year 2014, social media was awash with people getting a ice bucket water dumped over them while they nominated other to do likewise, all to raise money and awareness for Amyotrophic Lateral Sclerosis (ALS). Also known as Lou Gehrig’s Disease in America (after its most famous sufferer, baseball star Lou Gehrig), this neurodegenerative disease is an insidious killer, where sufferer have a typical life-expectancy of three years from the date of contracting the illness. Even more chilling, there is no cure for als disease, although some symptoms of als may be controlled.

Discovered by French neurologist Jean-Martin Charcot in 1869, the most common form of ALS is sporadic, meaning it strikes randomly. The other type of ALS, “familial”, is hereditary, whereby there is a 50% chance that children of those affected by ALS will have the gene mutation, and thus, develop the als disease.

The inescapable feature seen in ailing victims is the progressive decline of muscle control, and thereby, voluntary movement. This occurs because nerve cells responsible for person’s motor capacity are malfunctioning.

When upper motor neuron (found in the brain) die, they stop signaling the spinal cord to initiate and control muscle movement. Meanwhile, impaired lower motor neuron (found in the lateral regions of the spinal cord) causing scarring or hardening (sclerosis) of the spinal cord, which also disrupt signaling to muscles. Owing to the continuous degeneration of both motor neurons, muscles atrophy, and the victim deteriorates physically, gradually losing abilities like moving, eating, speaking and eventually, breathing.

The general age bracket for susceptibility is between 40 and 70 years old. Interestingly, a study by US Department of Veteran Affairs showed that those deployed during the 1991 Gulf War are twice as vulnerable to the disorder compared to the general public.

What Happen During ALS

ALS Diagnosis

In the early stage of ALS, symptoms of ALS can be easily confused with those of other diseases and disorders such as human immunodeficiency virus (HIV), human T-cell leukemia virus (HTLV), polio, West Nile virus and Lyme disease. Physicians also consider the possibility of neurological disorders such as multiple sclerosis, post-polio syndrome, multifocal motor neuropathy and spinal muscular atrophy; all of which can mimic characteristics of the disease.

Hence, appropriate assessments are carried out to eliminate these possible conditions before drawing ALS as conclusion. Such test include an electromyography (EMG) to detect electrical activity in muscles, a nerve conduction study (NCS) to assess nerve signals, and a magnetic resonance imaging (MRI) scan which may substantiate the presence of other problems. Based on the findings, blood and urine samples are analyzed alongside other routine laboratory testing.

ALS begins very subtly and symptoms of ALS are difficult to perceive. General cramps, stiffness, muscle weakness in either arms or legs, slurred speech or difficulty in chewing or swallowing are among initial onset signs, but are easily interpreted as non-related.

When muscles in limb are affected, victims suddenly find themselves losing manual dexterity. Simple task like turning a key, buttoning a shirt and writing, for example, become difficult and walking or running feels awkward as they trip and stumble often. Then there are those who initially experience speech problem.

As the ALS disease advances, muscle atrophy spreads to other parts of body, and becomes more defined. Spasticity (unusual muscle tightness) and exaggerated reflexes indicate upper motor neuron damage, while lower motor neuron degeneration includes severe muscle weakness, cramps and fasciculation (flickering movements under the skin).

The rate of ALS disease progresses varies between individuals. But they all reach the unfortunate point of being in a vegetative state when limbs cease to function, and they are unable to eat without choking. Still cognitive and lucid, they behold their own steady destruction helplessly. In the last stages, the ability to breath diminishes as the muscles of the respiratory system weaken, thus they require a ventilator to breath.

ALS treatment

There is no cure for ALS, only the use of certain drugs to control symptoms of als. Riluzole provides modest benefits sufferers by delaying the onset of ventilator-dependence, and prolonging the survival of those with difficulty in swallowing, by several months. However, throwbacks include increased risk of liver damage and side effects, among which are allergic reactions (skin problems, chest tightness, swelling of lips, tongue and throat, fever, irregular heartbeat, jaundice, nausea, stomach problems and flu-like symptoms.

Physicians can also prescribe medication to help alleviate fatigue, muscle cramps, spasticity, and to lessen the excess saliva and phlegm produced. Drugs to relieve pain, depression, sleep disturbances and constipation may also be given but care is taken to prevent risky interactions.

Physical therapy is another ALS treatment aspect. Unaffected muscles are strengthened with low-impact aerobic exercises like walking, swimming and stationary bicycling, which also improve cardiovascular health and mood. On the social side of things, speech therapists provide adaptive strategies to speak louder and clearer, as well as techniques to respond non-verbally. Speech synthesizers and computer-based communication systems can be vital devices for those who have shutdown vocally.

Nutritionally, caregivers formulate small, easy-to-swallow food portions with the requisite amounts of calories, fibre and fluid. When patients are no longer able to eat, a feeding tube is inserted into the stomach instead. The use of invasive mechanical respirators indicates the beginning of the end Ventilation support eases breathing and extends life but curbs the patient’s mobility due to machine attachments.

Peripheral artery disease

How widespread it is may still be under appreciated, although hundreds of millions of people worldwide are victims of his disease. Peripheral artery disease is alarmingly prevalent, and longer life expectancy along with evolving lifestyle (not necessarily for the better) seem to be exacerbating its rise. Its cardiovascular risk implications (it frequently lead to heart attacks and strokes) mean that there is a dire need to assess preventative measures and treatment.

The free, uninterrupted flow of oxygen-rich blood in our bodies is essential, and this is facilitated by our blood vessels, of which there are two main groups – arteries and veins. The former carry nutrients and oxygen to the cells, while the latter bring back cells metabolic by-products, toxins and carbon dioxide back to the heart for recycling.

Peripheral arteries are those that do not supply the heart and brain. They are, however, still extremely important. Disease of the arterial tree can range from short segment narrowing to full blown occlusion / blockage and can have devastating effects on the area where the artery is supposed to supply. These are mostly related to diabetes mellitus, atherosclerosis (blockage), hyperlipidaemia (high blood cholesterol) or even trauma. Veins however have a different subset of condition ranging from the unsightly varicose veins on the lower limb to the painful, calf-bursting feeling of deep vein thrombosis (DVT) and its subsequent effects.

“Atherosclerosis” or blockage by plaque (fat, cholesterol, calcium and other substances) of these blood vessels, results in a debilitating condition known as peripheral artery disease (PAD). While the heart often takes centre stage when it comes to atherosclerosis, blood vessels in other parts of the body, are equally important, and should be given adequate focus and attention.

Blood vessels are lined by endothelium, a thin layer of cells which maintains a toned and smooth interior for good blood flow. When the endothelium is compromised by causes such as high blood pressure, smoking or elevated blood cholesterol, atherosclerosis takes place with the formation of plaque on the arterial walls, thus obstructing blood flow. Left unremedies, ensuing plaque builds up, and over years, as arterial clogging worsens, so does resulting incapacitation of limbs and the pelvis area.

In some, recurring cramps and pain in the leg while exercising, otherwise known as intermittent claudication, may signify the beginning of problems. Conditions deteriorate as peripheral arteries continue to constrict, and the discomfort then spreads to the thighs, hips and buttocks. Other manifestations thereafter include perpetually cold and numb feet and toes, the thickening of nails, a bluish tint and shine to the skin, erectile dysfunction in the men, and the poor healing of sores. As PAD progresses, pain occurs even at rest, intense enough to disturb sleep.

Serious complications also result from PAD. Sores and injuries hich do not heal, may turn full-blown into gangrenous tissue and require the amputation of the affected limb. And atherosclerosis is not confined to just the peripheral blood vessels but happens as well in the arteries supplying the heart and the brain as well, eventually leading to a stroke or heart attack.

SCREENING

Often dismissed as simply a part of aging, the aforementioned signs and symptoms are actually serious indication to get screened by a medical professional. Do not wait for pain to occur before taking action. Preemptive screening is especially advisable for the aged, diabetics, smokers, the obese and those with hypertension, because of their vulnerability to PAD.

Potential patients are asked about their symptomatic experiences, as well as their personal and family history. During a physical examination to determine PAD, pulses in certain areas of the feet, legs and groin are inspected for weakness or even absence. Whooshing sounds over arteries heard with a stethoscope, ulcers where blood flow has become restricted, and lowered blood pressure in affected limbs, are all indicative too.

More advanced testing includes a Doppler (ultrasound) test, whereby high-frequency sound waves evaluate blood flow and where arteries may be blocked or narrowed. But for a more precise picture, an angiogram is carried out – arteries are injected with dye to clearly watch blood flow, and how restricted it gets in certain areas.

To complete the investigation, blood tests are included to measure blood cholesterol and triglyceride levels as well as to check for diabetes.

PREVENTION IS BETTER THAN CURE

Lifestyle changes are a must for those at risk of PAD. First and foremost, it is never too late to quit smoking. Also, since “we are what we eat,” dietary discipline in the form of higher intake of fiber-rich foods like whole wheat, oatmeal, bran, brown rice, fruits and vegetables, is a major step in the right direction.

In addition, the reduction of salt, unhealthy fats and refined sugar in meals, will help treat or prevent related ailments like diabetes, high blood pressure, lipid problems and vascular disease. Physical activity three to four times a week keeps the blood flowing nicely. Brisk walking at least 20 minutes at a time, cycling and swimming are great options, but it is wise to first consult with a physician before starting any exercise programme.

MEDICAL INTERVENTION

Depending on the situation and severity, doctors will assess if it is necessary to resort to procedures like angioplasty, bypass surgery or thrombolytic therapy to treat PAD, and will advise their patients accordingly.

Angioplasty requires a tiny hollow tube (catheter) to be threaded through the artery to the affected location, when a small balloon on its tips inflates to reopen the constricted artery as well as flatten the blockage against the artery wall. Thus stretched, blood stream through unhindered. Further, a mesh framework (stent) may be added to ensure the artery stays open. Lately, thetr are balloons and stent coated with medication that prevent the endothelium from producing scar tissues that could lead to further blockage.

In bypass surgery, the doctor will graft a blood vessel from another part of the patient’s body (or a synthetic one) to allow blood to flow around, that is, to bypass the blocked or narrowed artery. There is also thrombolytic therapy which may be recommended for blood clots found blocking an artery, whereby there are injected with a drug to dissolve them in situ.