Tsar’s Disease, and what you need to know about it.

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Haemophilia or the Tsars disease is an inherited bleeding disorder.

This is called the Tsars disease because the Tsars of Russia were afflicted by it.  Haemophilia presents as A and B. Both are X-linked inherited recessive genetic patterns. It is more commonly seen in men while women tend to be carriers.

Haemophilia A is caused by the deficiency of clotting factor VIII while deficiency of Factor IX causes   haemophilia B or the Christmas disease as is called.

Symptoms are known to include excessive bleeding from any site in the body to long term damage to joints. There could be repeated episodes of spontaneous bleeding. In the case of an injury then bleeding is profuse and prolonged. The treatment involves coagulation factor replacement therapy.

Since the disorder is X-linked and recessive, it gets expressed only when a defective X-chromosome presents itself. In a boy when there is only one X-chromosome and it is defective the disorder gets manifested. For a girl to manifest haemophilia both the X-chromosomes need to be defective. With one normal gene and one defective gene the woman becomes a carrier there are 50% chances that her male child inherits the disease.

Very rarely Haemophilia C is accosted.

Haemophilia A is seen in about 1 in every 5000 live male births and is seen across racial groups.  While B is rarer than haemophilia B is seen in about 1 out 20-30.000 live make births.

Though haemophilia is popularly called the Car’s disease, it was also known as the Royal disease as Queen Victoria of England was a carrier and her daughters passed the mutated gene to the members of the Royal families of Germany, Spain, Russia, Alexandra.

Symptoms,

  1. Blood in the urine, stools.
  2. Deep bruises.
  3. Large unexplained bruises.
  4. Excessive bleeding.
  5. Bleeding from the gums.
  6. Frequent nose bleeds.
  7. Pain the joints.
  8. Tight joints.

It would be a good idea to see your doctor if the following symptoms are seen:

  1. Severe headache
  2. Frequent vomiting
  3. Neck pain
  4. Blurred or double vision.
  5. Extreme sleepiness
  6. Continuous bleeding from an injury.

Diagnosis is made depending on the clotting time after which the clotting factor percentages are evaluated.

  • 5-40% clotting factor is considered mild.
  • 1-5% is moderate
  • Less than 1% is severe.

Treatment is handled by the physician.

  • Haemophilia A is managed with prescription hormone injection, called desmopressin. This stimulates the clotting factors.
  • Haemophilia B is managed with blood plasma infused with clotting factors. Sometimes synthetic clotting factors are used.
  • Haemophilia C is managed with plasma infusion.
  • If there are joint damages then physical therapy is required.
  • Russell viper venom has been used as emergency first aid at times.

Alternate therapies are being researched:

A study was conducted in using hypnosis to handle haemophilia.  Thirty individuals experiencing severe haemophilia and who were on home therapy were studied. They were randomly assigned to treatments or to a control group’s waiting list. The treatment group was given comprehensive training for six weeks; it included self-hypnosis, education, deep relaxation and support. The follow up was done for 18 weeks.  The treatment group reduced the quantity of bleeding concentrates to controlled bleeding as compared to the control group. It was also cost effective as it could supplement the medical controlling haemophiliacs.world hemophilia day

On personal take hypnotherapy could be supportive to medications. And monitoring by the physician is essential.

Metaphysically speaking blood is about nourishment, so having this disorder,of the blood would mean having issues about self nourishment.

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World Voice Day

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World voice day

Laurie Haise Anderson says, it is easier not say anything, shut your trap, button your lip can it. All that crap you hear on TV about communication and expression feeling is a lie. Nobody really wants to hear what you have to say.

What she says is quite true. Forget about others not wanting to hear what we have to say, we do not respect the tool that helps us voice and express. Despite the fact that quite a few professions like teachers, preachers, actors do a living with their voices not to mentions singers.

World voice day or WVD is an annual worldwide event that occurs to celebrate voice. The event aims to demonstrate the importance of voice in the everyday life of people. It allows us speak and express.

The WVD also looks at helping to prevent voice related issues, rehabilitating voices that are deviant or sick, training the artistic voice and researching the function and application of voice.

Part of the agenda on the WVD is also to help people who do a living using their voice to keep it healthy.

The production of voice is studied by disciplines as varied as medicine, speech-language pathology, music, physics, psychology phonetics, art and biology.

History of day dedication:

1999 the Brazilian society of laryngologists launched a day dedicated to the voice.

2002 the Portuguese Laryngologist Mario Andrea who was also the president of the European laryngological society suggested that it should be made a global event.

2012 voice researchers Prof. Johan Sundberg from Sweden, Prof. Tecumseh Fitch from Austria and Prof. Filipa La from Portugal invited voice experts from number of countries to create an international group for celebrating the voice. Today there are 66 members who help to initiate and coordinate the events in their respective countries.

2016 saw 700 events taking place and all them are listed on the website of world-voice-day.org.

Few quick tips on voice hygiene.

I have listed few common problems with voice and how to handle it. But it is a good idea to get things cleared by a laryngologist.

  • Lack of hydration: this could be either internal or external. External is caused by mouth breathing, taking in dry air, smoking or medications. The internal one is triggered by caffeine, alcohol, lung drying medications, or lack of fluids. This should be dealt by:
    • Steam inhalations.
    • Constant sipping of warm water.
    • Replace coffee, soda with water.
  • Mucous spooling. Is bothersome, people tend to clear throat often; there is a feeling of something being stuck in the vocal cords.
    • Constant sipping of warm water helps.
    • Mucolytic medications also help, though it is advisable to take the physicians advice before taking them.
  • Chronic throat clearing this starts with the feeling of irritation, coughing and or irritating the vocal cords act like irritants and increase the secretion. At this point it becomes a vicious cycle. Dealing with this is a little tricky but:
    • Constant hydration with warm water helps.
    • Mucolytic medications can help. But it is best taken with the physician’s guidance.
  • Voice abuse, talking a lot in high pitches causes stress on the vocal cords. Speaking for long hours is also injurious. This should be handled by :
    • Resting the voice often.
    • Not raising the voice too often.
    • Constant hydrating.
  • Laryngopharyngeal reflux and gastro-oesophageal regurgitation reflex are common systemic issues that can disturb the voice. Laryngopharyngeal reflux is when there is a spill from the pharynx that is from the oesophagus to the larynx. While gastro-oesophageal regurgitation reflex is when the acids in the stomach creep up and spill. Dealing with these would definitely require consultation with the laryngologist.
    • Life style and dietary counselling would be essential.
    • Medications like proton pump inhibitors maybe administered.
    • Of course like all the other conditions constant hydration with warm water is essential.

If despite your care you have issues with laryngeal irritation that affects your voice it is time to consult the laryngologist.

 

Universal Health Coverage. an Overview

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World health day

“Health is a human right. No one should get sick and die just because they are poor, or because they cannot access the health services they need.”–@DrTedros

World health day is about health for everyone, the focus of the year is universal health coverage.

Some facts about health coverage.

·        About half the world’s population still does not have full essential health services.

·        Having to pay for health care pushes about 100 million people into extreme poverty.

·        About 12% of the world’s population that is about 800 million spend at least 10% household budgets to pay for health care.

·        Achieving of Universal Health coverage by 2030 as part of sustainable development goal is on the agenda of the UN members

What exactly is UHC

UHC is making health services accessible to individuals and communities without them having to suffer financial hardship.  Health Promotion, prevention of disease, diagnosis and treatment, rehabilitation and palliative care are all converged in this spectrum. UHC protects the people from the financial consequences of the treatment.

Often families tend to use up their savings, destroying the future of their children to address unexpected illness. Achieving UHC was of the targets the nations of the world set when adopting the sustainable goal development in 2015.

Good health contributes to the economic growth and stability of the community allowing long term economic development.

Getting clear on UHC

·        UHC does cover all possible health interventions, regardless of cost for that is not possible on a sustainable basis.

·        UHC is not only health financing, it is also health service delivery system, the health workforce, health facilities and communications network, health technologies, information systems quality assurance mechanisms and governance and legislations.

·         UHC is about ensuring minimum package of health services and ensuring a progressive expansion of coverage health services and financial protection as more resources become available.

·        In addition to individual treatment services UHC also includes population based services like public health campaigns, fluoridation of water, mosquito breeding management so on and so forth.

·        Along with taking care of health UHC also is supposed to take a step towards equality. Developmental priorities and social inclusion and cohesion.

Countries are already making progress towards UHC. Responding to ever-growing health needs is a challenge even in countries where health care has been accessible and affordable. The key requirement of moving towards UHC is a strong financing structure. Pooling of funds from compulsory funding sources like mandatory insurance contributions, spreads the financial risks of illness across a population.

To improve health services coverage and health outcome there has to be availability, accessibility and capacity of health workers to deliver quality people cantered integrated care. Sound systems of procurement and supply of medicines and health technologies and well-functioning health information systems are other critical elements.

UHC is not only about what services are covered but also about how they are funded, managed and delivered.  UHC is measured as the proportion of a population that can access essential quality health services and proportion and population that spends large amount of household income on health.

WHO and World Bank together have created a framework to track progress of UHC monitoring.  16 essential health services

Together with the World Bank, WHO has developed a framework to track the progress of UHC by monitoring both categories, taking into account both the overall level and the extent to which UHC is equitable, offering service coverage and financial protection to all people within a population, such as the poor or those living in remote rural areas.

  • Reproductive, maternal, newborn and child health.
    • Family planning
    • Antenatal and delivery care.
    • Immunization
  • Health seeking behaviour for pneumonia and other infectious diseases
    • HIV antiretroviral treatment.
    • Use of insecticide- bed nets for malaria prevention.
  • Non communicable diseases.
    • Prevention and treatment of hypertension.
    • Prevention and treatment of diabetes
    • Cervical cancer screening
    • Tobacco
  • Services capacity and access
    • Basic hospital access
    • Access to essential medicines.
    • Health security in compliance with international health regulation.

Every country is unique in its challenge so the focus area of each country varies and they might have to develop their own ways of measuring progress towards UHC.  However a global approach which uses internationally recognized standardized measures is useful in across countries comparison.

WHO 1948 constitution provides the base for UHC as it declares health a fundamental human right. WHO supports and helps countries to develop their health system to move towards sustain UHC and to monitor progress. WHO is partnered by different partners, in different situations for different purposes to advance UHC around the world?

India has made UHC a part of its 12th plan. Principals involved are

  1. universality
  2. equity
  3. non-discriminating and non-exclusive,
  4. Comprehensive and rational good care.
  5. Financially protection.
  6. Protection of patient rights and guarantees appropriateness of the treatment.
  7. Consolidation and strengthening of public health provision.
  8. Accountability and transparency
  9. Community participation.
  10. Putting health into people’s hand.

Today is also international geniocide awareness day and No Housework day.

Hypnotherapy for Management of Endometriosis

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Endo motorises is the growing of uterine tissue outside the pelvis.

This is painful and strips the quality of life. The release of chemicals such of cytokines and prostaglandins produced by the lesion renders the situation very painful, particularly when the estrogens levels increases.

Many healers have put down the metaphysical cause of the lesion as insecurity, disappointment and frustration of handling femininity and feminine issues.

Endometriosis presents as painful periods, and quite often the diagnosis happens when the patient presents with infertility as an issue.

Stress is also a major factor that adds to the condition.

The primary treatment is of course medical.

Hypnotherapy can help in a major way to manage the pain.

Hypnotherapy will not focus very much of pain control. When a person is in pain self hypnosis is an issue. Fixing the pain will not fix the problem. Hypnotherapy works on the fundamental acceptance that our belief systems, allow the manifestation of situations, which include ill-health.

So typically the approach would be identifying the belief system that has lead to the manifestation of the symptoms.

  • Stress would be addressed, the cause of the stress and the resolution.

A hypnotherapist would look at removal of the cyst and prevention of further migration, just the way a gynaecologist would look at the cyst.

  1. Step one is removal of the cyst. The body is scanned and the mental laser is used to eliminate the cyst.
  2. The cyst is then removed with psychic surgery and healing protection is placed using green/violent rays to prevent further migration.
  3. All related issues are addressed during the sessions.

This healing light has to be strengthened twice a week for the first month and then weekly for.

The patient recuperates and progresses well. Usually what happens is the patient then slides into a comfort zone and does not go through with the maintenance. This could cause sliding back, so as a healer I need to assure my patients that they need boosters and it is okay to come for it whenever needed.

Hypnotherapy does not negate the pain, it only aims to manage fear, or anxiety related to the pain.

Diet ,exercise, health all play an important role in managing the quality of life in a person with endometriosis.

 

Project Axshya

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Project Axshya

For TB care and control.

The role of community groups just a sum up

  • Anyone can get TB, this is not hereditary but infectious disease which spreads through air.
  • Any person with cough for two weeks or more should have their sputum tested at the nearest DMC
  • TB is curable
  • Under DOTS high quality drugs are provided free of cost.
  • Remember to complete the full course of TB treatment for sure cure.
  • Your role in spreading awareness of TB is very important.
  • You should advice anyone with cough for two weeks or more to go to the nearest DMC for sputum testing
  • You can help by becoming a DOT provider.
  • You should motivate TB patients to take medicines regularly and complete the course of treatment.

Please support the TB patients to gain social acceptance by reducing the stigma. Make TB awareness part of your agenda during meetings. You can support TB patients by linking them to social welfare awareness.

Function of Sleep…Sleep Awareness week.

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The function of sleep is still being researched. Though, evidence shows decreased metabolic activity,  repair, rejuvenation and growth during sleep.

Sleep comprises of two physiological states, these are very different from each other.  One having rapid eye movement is called REM and the other is non-REM

The NREM (non rapid eye movement) can be broken in

  1. Stage 1 the light drowsy phase where the transition from wakefulness to sleep occurs.
  2. The first unequivocal stage of sleep with sleep spindles and K compels appear on the electrocephalogram.
  3. Stages 3 and 4 are collectively called the slow wave sleep or the deep sleep because of the emergence of low frequency, synchronized waves.

REM is the stage where most dreaming occurs. It usually takes about 90mnts to transcend from NREM to first episode of REM, through the night cycles of episodes of REM and NREM occur. The NREM gets progressively shorter.  Which is why most of the REM occurs during the last third of the night?

With increased study of sleep patterns and sleep physiology, there is an emerging discipline of sleep disorder. Despite the wealth of information that is accumulated about biochemistry and physiology of sleep its precise nature and function are not known. Numerous theories have been forwarded including hypothesis that sleep is essential for memory consolidation, for binocular vision or as part of thermoregulatory evolution. Let’s just look at two of the accepted theories that provide both a synthesis of relevant research and background to understand the further developments that have occurred in the field.

CONSERVING ENERGY

“The metabolic rate is reduced at night and particularly during sleep by 5% to 25%.

Most people are more active during the day than the night. The concept of homeostasis is extended to explain that the energy that is expended during the day is balanced by recuperating during the night. The forms the foundation of the theory one of the functions of sleep is conservation of energy.

The expenditure of energy is measured by the metabolic rate which is raided during the day and reduced at night particularly at sleep. Oxygen consumption, heart rate and body temperature declines in the first few hours.  This is time particularly associated with the slow wave of the sleep waves.  There is a relationship across species between metabolic rate and sleep pattern, it has been seen that people who have high metabolic rates during the day exhibit lower wave sleep and sleep longer than people whose metabolic rates are lower.

Infants have the most slow wave sleep and it decreases with age. This suggests a parallel of expenditure of energy during the day, for example in a fit person after sustained exercise there is an increase both in the duration of sleep and slow wave sleep. Sleep deprivation is followed by increased amounts of slow wave sleep probably as a consequence of the delayed drop in metabolic rate that accompanies sleep. People who sleep for a long time high body temperatures during the day and so their metabolic rates could be raised as well

All these suggest that the primary function of sleep maybe to preserve energy.

THEORIES OF RESTORATION:

Accumulation of various evidence creates a hypothesis that sleep could be a process by which the whole body including the CNS gets restored. When the body is in the state of catabolism the consumption of oxygen increases. It gets lowered during sleep and is the lowest during the slow wave sleep. Paradoxically it is during this period of low oxygen consumption that anabolism is thought to take place. Low metabolic rates during sleep allow the concentration of protein to increase this allows both an increase in synthesis and a reduction in degradation. Though the process of catabolism and anabolism are continuous the relative rates vary depending on whether the subject is awake or asleep. It has been shown that anabolism is at its peak during sleep.

Growth hormone is released mainly at night, again in association with the slow wave sleep. Direct measures of bone growth in adolescents show that sleep is associated with anabolism. It has been observed that treatment of short stature with growth hormones is more effective if the growth hormone is given at night rather than the day.

When there is a need for growth the duration of slow wave sleep and over all amount of sleep increased for example during pregnancy, after exercise during adolescence etc. If the energy expended is less than the amount of slow wave sleep is also reduced. It is observed across species that cell mitosis is at its peak during sleep.

It has also been postulated that slow wave and REM sleep have different restorative functions. Slow wave sleep being important for macros molecular synthesis and REM for removal of synthetics products of slow wave sleep to maintain synaptic connection. It has been suggested that during REM sleep neuronal connections in the catecholamine system are formed and that this activity is necessary to maintain cognitive functions.

Just to summarize sleep has two cycles the rapid eye movement or the REM and the non rapid eye movement or the NREM. These cycles alternate with each other. Though numerous functions have been attributed to sleep all of them evidence gathered. The most accepted functions of sleep are growth, repair and rejuvenation of the body physiology.

 

The Impact of Sleep Disorder

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Merriam Webster defines sleep the natural periodic suspension of consciousness during which the powers of the body are restored. It is also a state of torpid inactivity. A state marked by diminution of feeling.

Sleep is a period where our conscious mind is resting and our subconscious mind is still working so all our involuntary muscles is working.

Sleep has been a major area of work in my space as a dentist and as a hypnotherapist, particularly since I am sleep challenged myself.

Sleep is the period of repair and rejuvenation, for the physical and the emotional body. It is a period of sorting for the mind.  I shall talk about this over the week, but as of now, physiologically speaking we are talking about REM sleep, NREM sleep, detoxing of the lungs, the body cycles coming to a balance etc. Etc.

Emotionally speaking we are talking of sorting of thoughts eliminating the unwanted, preserving what we perceive as relevant. We are also talking about predictive dreams, venting of excess.

Being sleep deprived is one of the most, physiologically and psychologically dangerous states. Sleep disorders have entered the Indian population, contesting with the American. But the other side is that it is estimated that one third of the population goes t sleep where they are standing when it is time to sleep.

As of now the impact of sleep disorders on morbidity and mortality is not very widely appreciated.

With sleep disorders there is an increasing in daytime sleepiness and resultant increase in traffic accidents.

There is a study that states that disturbed sleep cycle during child rearing has its effects at a later stage.

Sleep disruption makes it difficult to cope with many medical disorders. It also reduces the attenuation of school and work ability, giving rise to psycho-social consequences and a constriction of leisure and pleasure time. There are specific effects linked with each condition.

Sleep apnoea increases risk of myocardial infarction and stroke. Cognitive deterioration, presumably on basis of repeated nocturnal hypoxia and sleep disruption has been described in a number of studies, and personality change is part of continuous fatigue leading to marital friction, divorce difficult interpersonal relation, etc.

Insomnia though often trivialized by doctors, is a common symptom albeit often self-limiting. In up to 80% of the cases insomnia reported in general practises due to anxiety and depression.  Even in a specialized clinic one third of the cases are related to anxiety and depression.  Many insomniacs tend to self medication, which could be ineffective and dangerous. When associated with other disorder this could be a trigger for suicidal behaviour. If the patient resorts to hypnotic medication to deal with sleep it could interact with alcohol intake. Long term use of benzodiazepines can impair cognitive powers particularly in elderly.

Narcolepsy is a disorder with the longest duration from onset of symptoms before diagnosis. It impacts the quality of life, in a study of impact of various disorders to the quality of life, narcolepsy rates second only to quadriplegia.

Historically we have a belief system or rather a misconception that sleep is a passive period, but contrary to it, sleep is the active restorative processes of the body. Some authors look at the small hours of the morning a high risk window for mortality. F.Scott Frtozgerald wrote in 1945, that in the real dark night of the soul it is always 3 O’clock in the morning. Many studies show an increased mortality in late sleep and early waking hours.

Studies have shown that nocturnal asthma is pronounced in the latter part of the sleep and particularly during the REM phase. Though asthma is not fatal in itself, people dying of asthma tends to do predominantly in the night. This could be due to patho-physiology of the sleep.

There are large number of medical disorders that exhibit peak mortality at night, then are specific though rare disorders associated with sleep… this is seen in south asian men and is thought to be REM related arrhythmia.

American cancer society shows, that deviation from age appropriate sleep that is two hours longer or shorter than normal leads to increased mortality, which is more pronounced in women than men. This effect increased with age.

A random survey of more variable sleep patterns was among young adults that are between 18-34yrs of age and older adults that are over 65, as compared to middle aged adults.  People sleeping a normal duration of 7-8hrs reported lower rates of illness.  There are patho-physiological changes during sleep which lead to increased morbidity, like hypercapnic and hypoxic drive is diminished particularly in REM sleep. There are patients for whom epilepsy is exclusively sleep related. Gastro-oesophageal reflux is more common at night. Certain patterns of headache such as cluster headaches have an onset during sleep.

Care giving of elderly person is associated with disruptive sleep for the caregiver causing patho-physicologic changes that sometimes becomes intolerable.

Many sleep related disorders appear in the hospital, sometimes even requiring hospitalization, present as diminished productivity, could be either circumstantial or direct consequence of fatigue related accidents.

Fatigue peaks occur in the early hours of morning and a second fatigue peak occurs in the mid afternoon. These peaks show up as increased incidence of road traffic accident. Studies suggest that 20% of all the drivers have fallen asleep behind the wheel at least once. One third of heavy trucking accidents are the result of the driver slipping into sleep deficit fatigue. Aircraft pilots flying jets have exhibited similar dips during electroencaphalograhic recordings.

There is a seven fold increase in road traffic accidents in patients with sleep apnoea, and in a study 60% of these patients describe falling asleep at the wheel at least once a week.

Sleep walking infrequently leads to accidents but occasionally leads to death it is very rare that sleep related homicide is reported.

Some statistics of sleep disorders.

  • Narcolepsy about 0.15% manifests this.
  • Obstructive sleep apnoea about most common in middle aged overweight people. 4-8% men and 2-4% women manifest it.
  • Restless leg syndrome definitive data not available, 5-15% manifest it, of the subjects 11% were pregnant women 15-20% people with uraemia and about 30% with rheumatoid arthritis.
  • Shift-worker sleep disorder about 2-5%
  • Sleepwalking 1-15% though more common in children.
  • Sleep terrors is seen in 3% of children and <1% of adults.
  • Nocturnal leg cramps definitive data is not available, but about 16%of healthy people do present it.
  • Nightmares are seen in 10-15% of children between 3-5 years, it is enough to disturb their parents about 50% adult, have occasional nightmares and 1% have frequent that is more than once a week.
  • Isolated sleep paralysis is seen in 4-050% of patients, and 40% patients with Narcolepsy also present sleep paralysis.
  • Impaired sleep related penile erections are in 10% of the men as chronic.
  • Sleep enuresis is seen in 40% of 4 year olds, 10%6 year olds, 5% 10yr olds, 3% of 12year olds, 1-3%of 18yr olds. Primary enuresis comprises of 70 -90% of all cases while 10% of it is due to secondary enuresis.
  • Insomnia is seen in 30% annually and one third of them complain that the problem is severe.
  • Primary snoring is seen in 40-50% of the men and lover>65yrs.
  • Sudden infant death syndrome is estimated to occur in 1-2 / 1000 live births.

Sleep disorders like sleep apnoea, particularly when associated with other medical diseases, are associated with increased mortality.

Sleep related problems can cause financial loss. Three is a loss of productivity as a consequence of shift work and the increased medical care of shift workers. Admission to the hospital of elderly people with sleep duration, the cost of sleep debt fatigue related accidents are all factored in and in a country like America it is about 16,000 million. Increase in work time, which is almost 8% over the last century has the consequence of decreased leisure and sleep time. The consequence of this is fatigue and a drop in the quality of life. This again does not manifest tangibly but it reduces productivity and impairs performance.

In adolescents there is a decreased ability to retain learning, and an increased sleepiness, in the severely sleep deprived aggressive anti-social behaviour is also seen.

Unfortunately neither the medical fraternity nor the society takes this aspect of human requirement seriously.

reference :

sleep apnoea workshop hosted by Elder Pharmaceuticals.

Impact and Epidemiology of sleep disorders by Colin.M.Shapiro,William.C.Dement.