It is estimated that more than 5 million people are living with lupus worldwide.Lupus is a complex disease which can cause inflammation throughout the body damaging any part including skin,joints,organs-kidneys,heart,lungs- and bodily systems-blood vessels and nervous system.about 90% lupus sufferers are women of child bearing age.The illness is usually aggressive in children and teenagers than in adults.And it is a harsh reality that 20% of lupus diagnosis happens to be in children.
In lupus,immune system is hyper active and produces abnormal antibodies invading bodily tissues and organs.This condition is thought to be triggered by a combination of genetic and environmental factors.Lupus when referred generally means Systemic Lupus Erythematosus (SLE) which is the most common form of  lupus. SLE in its severe cases involve complications to major organ system.Before we analyse SLE in detail, let us have a brief look at three other types of lupus.

Cutaneous lupus erythematosus
This is the form of lupus limited to the skin but is worsened by exposure to sun. Discoid rash which is the most prevalent rash appears raised, scaly and red, but not itchy.In another form namely ‘Butterfly rash’ ,rash appears over the cheeks and across the bridge of the nose.It is to be noted that Butterfly rash happens to be the significant symptom of SLE. Also,10% of people who have cutaneous lupus will develop systemic lupus.

Drug-induced lupus erythematosus
Although ,the symptoms of drug-induced lupus resemble systemic lupus,it rarely affects major organs.It is a lupus-like disease and symptoms generally vanish in about six months once after the  drug intake is  stopped.Lupus inducing drugs include Hydralazine—Treatment for high blood pressure or hypertension,Procainamide—Treatment for irregular heart rhythms and Isoniazid—Treatment for tuberculosis.

Neonatal lupus
A condition which  affects infants of  whose mothers are lupus patients, but rarely.It is  due to the antibodies of mother acting upon the infant in the womb .Consequently,the baby at birth  may have skin rash, liver problems, or low blood cell counts. Fortunately,symptoms disappear completely  within months leaving no lasting effects. Some infants  can also have a serious heart defect.

Systemic Lupus Erythematosus (SLE)

Symptoms

Lupus range from mild to life-threatening forms.As mentioned earlier,butterfly rash or malar rash is one of the striking feature of Lupus in general.Lupus begin to manifest with a fever, vascular headaches, epilepsy, or psychoses.Several people will have to suffer from cardiovascular disease, strokes, disfiguring rashes, and painful joints.People also experience extreme fatigue, hair loss, cognitive issues and devastating physical impairments. For others, there may be no visible symptoms.
Symptoms also exhibit relapses(symptoms flare up and become worse) and remissions (symptoms settle down).For some,symptoms are seen to be constant.
In the mild variant,sufferers are usually young women.They experience photosensitivity and hypertension.
Symptoms are limited to arthritis-like joint pain,fatigue,sun light induced skin rashes,mild anemia and problems with blood platelet regulation.Moderate lupus inflame skin and other parts of the body,including lungs,heart and kidneys.
Severe SLE may  result in

• Kidney disease called lupus nephritis.
  It can be so damaging that dialysis or kidney transplant may be required.
• Major blood disorders; Inflammation in the brain’s blood vessels can cause high fevers, seizures, and behavioral changes.
• Neuropsychiatric symptoms such as mild cognitive dysfunction, organic brain syndrome,peripheral neuropathies, sensory neuropathy, transverse myelitis, and paralysis and stroke.
• Terrible psychological disorders like personality change, paranoia,mania,schizophrenia,epilepsy , psychosis
• Hardening of the arteries or coronary artery disease—the buildup of deposits on coronary artery walls—can lead to a heart attack.
• Problems with the lung, heart and pancreas.
• Serious form of lupus rash results in “bullous” lupus rash.
• Raynaud’s phenomenon-fingers and toes turning white or blue and feeling numb when a person is cold or stressed

Diagnosis

Diagnosis is challenging as there is no single test specifically for lupus.Tests are usually a combination of blood and urine tests,physical examination findings ,symptom analysis and medical history.

Complete blood count:For WBC,RBC and platelet counts.
Urine analysis: To check for higher levels of protein and RBCs or if  kidney is affected
Blood clotting test:To test for clotting problems.
Biopsy:Tests to check for signs of inflammation by removing a small piece of tissue from different parts of body.(For eg: skin biopsy,kidney biopsy)
Antinuclear antibody (ANA) test. A positive result for the presence of these antibodies  indicates a stimulated immune system. As most people with lupus have a positive ANA test ,if tested positive for ANA, more-specific antibody testing may be advised by rheumatologist.

As symptoms gradually present over time and also varies from time to time,diagnosis may require series of repeated tests.

Treatment

Currently,there is no complete cure for lupus.Treatments are generally used to ease the symptoms and prevent organ damage.Mild forms can turn into severe SLE due to flare ups leading to irreversible damage to the organs.Although still treatable,close monitoring is required to reduce the chances of organ damage.
Treatment must be tailored for individuals.Also,treatment for different forms are different.

Treatment for mild SLE

Nonsteroidal Anti-inflammatory Drugs (NSAIDs):For joint pain and muscle pain are classic early symptoms of mild SLE.
Anti-malarial Agents:The malaria drug hydroxychloroquine (HCQ) for both the muscle and bone pain of SLE as well as the rashes and other sunlight-induced skin problems caused by SLE
Corticosteroids:Low dose corticosteroids are often effective in treating the musculoskeletal inflammation, fatigue, occasional low-grade fever.
The bad news is that for those with mild SLE, the side effects of these conventional treatments outweigh their benefits
Emerging Therapies: Hormone Manipulation
Although it doesn’t mean that estrogen cause lupus,there is evidence that estrogen and prolactin somehow regulate the severity of lupus.This suggests that controlling these hormones through drugs can be groundbreaking treatment for SLE.

Treatment for severe SLE

Are you or someone you know diagnosed with Gullain-Barré Syndrome? You may want to read up here to find more about it.
Gullain-Barré Syndrome (GBS) is a serious neurological condition in which the immune system attack peripheral nervous system.As you might have guessed,the name originates from two scientists who first described it,George Guillan and Jean Alexandre Barré. Although this autoimmune disease cause lingering symptoms for a very long time,individuals can achieve spontaneous treatment induced recovery.Also,GBS is neither contagious nor hereditary.

Understanding GBS and variants

The peripheral nervous system -network of nerves outside brain and spinal cord- controls the ability to feel and move by transmitting electrochemical impulses.Neurons in these network have nerve extensions called axons,coated by myelin sheath.In GBS,the myelin sheath is damaged/removed(demyelinating neuropathy) or axon is degenerated (Axonal neuropathy) hindering the messages being delivered to the brain properly and thus muscles become unable to respond to brain commands.

It is not clearly discovered how immune system attacks healthy nerve cells.As GBS is usually preceded by a microbial infection,most established theory is the “molecular mimicry/innocent bystander theory”.According to this theory,some nerve cells resemble molecules of certain microorganisms making it vulnerable to attack when the immune system fights off the infection.
Until last three deacdes,GBS was thought to be only demyelinationg polyneuropathy.But several variants have been identified.

Acute Variants
Acute Inflammatory demyelinationg polyneuropathy (AIDP)
Acute motor axonal neuropathy (AMAN) and Acute motor-sensory axonal neuropathy (AMSAN)
Miller Fischer Syndrome

Chronic Variants
Paraproteinamic demyelinating neuropathy(PDN)
Multi focal motor neuropathy(MMN)
Lewis-Sumner syndrome(MADSAM)
chronic axonal neuropathy
Sub acute inflammatory demyelinating polyradiculoneuropathy(SIDP)

Symptoms

The onset of GBS is generally rapid from muscle weakness,tingling to paralysis.It is also accompanied by loss of bladder control,severe pain in the lower back,inability to breathe and difficulty moving eyes,face,talking or swallowing.
In AIDP,the paralysis is ascending indicating that it travels from limps towards upper part of the body. AMAN is characterized by symmetrical weakness and respiratory failure. ASMAN typically appears in adults as severe motor and sensory dysfunction.Miller-Fischer syndrome presents itself with ataxia-loss of muscle coordination, areflexia-stimuli non responsive muscles and ophthalmoplegia-progressive paralysis of movement muscles in eyes.
In most severe cases,people with GBS can be fully paralysed. Paralysis may eventually affect diaphragm or chest muscles preventing proper breathing becoming fatal.

Diagnosis

Lumbar puncture
Cerebrospinal fluid is tapped to test for levels of protein.
People with GBS have higher levels of specific proteins without any increase in WBCs.

Electromyogram (EMG)
In this effective nerve function test,electrical activity from muscles is recorded to check if the muscle weakness is caused by nerve damage.

Nerve conduction velocity (NCV)
NCV records the speed at which signals travel along the nerves. These signals are surely slowed in GBS.

Treatment

It is critical that patients are immediately moved for treatments.Prognosos is that 70% of affected individualy fully recover in 6 weeks provided the treatment was started in two weeks from the onset of symptoms.Treatment is usually followed by rehabilitative care to regain muscle strength and mobility.

Plasmapherisis(plasma exchange)
Plasma from the patient is extracted using a machine to remove the antibodies attacking the nerve cells and then the blood cells is returned back to the person.

Intravenous immunoglobulin
The immunoglobulins developed from a pool of donors which contains healthy antibodies are administered to the patient.This helps to commission off the harmful antibodies.

It is also very important to combine treatment with rehabilitative care.As the recovery can be non uniform,stronger muscles tend to take over the functions of weaker muscles.This process is called substitution.During the treatment,specific exercises may be needed to prevent substitution,blood stagnation and sludging. After the recovery,rehabilitation therapy facilitates the individual to resume physical strength to carry out daily activities normally.

Can you recall a time when you dozed off during work? Probably, it was on the day after you partied whole night long. You were really exhausted and managed only a few hours of sleep. But, can you imagine a situation in which you fall into sleep instantly every now and then? While driving or eating? Isn’t it stressful? This is what happens to people with Narcolepsy.
Narcolepsy is a chronic neurological disorder that affects the brain’s ability to control sleep-wake cycles. It is characterized by sleep attacks, sleep paralysis, hallucination and in some cases episodes of cataplexy (explained in a later section). People with narcolepsy are overwhelmingly drowsy throughout much of the day. They also experience intermittent, uncontrollable episodes of falling asleep during the daytime and uneven interrupted sleep during night. 

Narcolepsy explained

Narcolepsy can affect greatly affect daily activities ,ofcourse adversely. These sudden sleep attacks may occur during any type of activity at any time of the day. Sleep invades even in the middle of an activity like driving, eating, talking or dancing. Yes, you read that right! So, if left undiagnosed or untreated, this neurological condition can interfere with overall psychological, social and cognitive function and development which can in turn inhibit academic, work and social life.
In a normal sleep cycle, we initially enter early stages of sleep followed by deeper sleep stages and ultimately REM (rapid eye movement) sleep. It takes around 60-90 minutes. Dreams happen during REM sleep. Now during this stage, brain keeps muscles “limp” and as a result, people are incapacitated to act out of their dreams.
In contrast, narcoleptic people enter REM sleep rapidly in about 15 minutes of falling asleep without experiencing non-rapid eye movement sleep (NREM), both at night and during the day. The boundary between awake and sleep is blurred. Consequently, aspects of REM sleep intrude on wakefulness, while wakefulness intrudes on their sleep. 

Who is affected?

It can affect irrespective of the gender. Typical onset of narcolepsy is between the ages of 10-25; but can occur anytime in life, even in early childhood. Statistics show that about 1,35,000-2,00000 in US have narcolepsy. In India, it is fewer than a million. But in reality, the numbers may be higher since people with narcolepsy are often misdiagnosed with other conditions such as psychiatric disorders and emotional problems. It is estimated that up to 50% of patients with Narcolepsy may be undiagnosed.  

Causes of Narcolepsy

The cause of narcolepsy is not clearly established. Some experts suggest that narcolepsy may be due to the deficiency of certain chemicals in brain which signals sleep-wake cycles called hypocretin.        Current research points that narcolepsy involves multiple factors, which causes the neurological dysfunction and REM sleep disturbances, working together to cause a lack of hypocretin. 

Symptoms of Narcolepsy 

Narcolepsy is a lifelong condition. But symptoms can partially improve over time, but never disappear completely. All narcoleptics experience excessive daytime sleepiness, but all of the symptoms are seen only in 10-25 % of affected individuals. 

Excessive daytime sleepiness (EDS)
This most obvious symptom is the inability to stay awake and alert during the day regardless of how much sleep an individual gets at night. The real scare is that the sleepiness is not normally drowsiness, but ‘sleep attacks’ where the individual falls off to sleep suddenly.

Sleep disruption and insomnia
Narcoleptic sleep is disrupted by frequent awakening, also called fragmented sleep. Sleep may be also disrupted by insomnia, vivid dreams, sleep apnea, acting out while dreaming and rapid leg movements.

Cataplexy
Cataplexy is a sudden and uncontrollable, brief loss of voluntary muscle control (actually the ‘muscle paralysis of REM sleep) often triggered by experiencing strong and intense emotions such as laughter, stress, anger, fear or excitement. Cataplexy attacks may vary in severity from partial cataplexy, which involve the slurring of speech to full cataplexy where an individual collapse to the ground, awake but unable to move, speak or keep eyes open.
In about 10% of narcoleptics, this is the first symptom to appear and could be misdiagnosed as a seizure disorder. But unlike fainting or seizure disorder, people remain conscious even during the most severe attack.

Sleep Paralysis
While falling asleep or waking up, sleep paralysis causes temporary inability to move or speak. It usually lasts for only a few seconds or minutes. It resembles cataplexy except that it occurs on the edges of sleep.

Hallucinations
Very vivid and frightening images can accompany sleep paralysis and usually occur when people are falling asleep or waking up. Most often, the content is primarily visual, but any of the other senses can be involved too.

Automatic behavior
Sometimes, narcoleptics experience temporary sleep episodes that can be very brief, lasting no more than a few seconds. Person automatically continues any activity they were doing, for example, eating or talking for a few seconds or minutes without consciousness.
They cannot recall their actions and their performance is almost always impaired. If episodes occur during driving, individuals may get lost or have an accident. 

Types of Narcolepsy 

Type 1 narcolepsy (Narcolepsy with cataplexy).
It is diagnosed by low levels of hypocretin or reporting cataplexy/excessive sleepiness on a special nap test. 

Type 2 narcolepsy (Narcolepsy without cataplexy)
These people experience severe day time sleepiness but usually do not have muscle weakness triggered by emotions. 

Diagnosis

On average it takes 10 years from onset of symptoms to an official diagnosis of narcolepsy. Prior to diagnosis, it is not uncommon for people to be misdiagnosed with other sleep disorder, depression, psychiatric conditions, ADHD and even epilepsy.
If doctor suspects narcolepsy, he or she will most likely recommend the following sleep studies: 

Polysomnography (PSG)
During the PSG, a technician places multiple sensor, which are connected to a computer, on the patient’s head, neck, chest and legs to record brain waves, oxygen levels, eye movements, muscle tone and heat and breathing rates. Also, a video is recorded for monitoring the body movement. 

Multiple Sleep Latency Test (MSLT)
MSLT is a day time sleep study conducted after a PSG to assess the quality of night time sleep and the degree of sleepiness during the day. The MSLT involves several naps scheduled at 2-hour intervals beginning a couple of hours after you wake up in the morning. It documents how readily a person can fall asleep, REM patterns, eye movements, heart rate, chin movements and certain brain activities. 

Lumbar Puncture
The lumbar puncture is used to test the level of Hypocretin-I. It involves a spinal tap to collect the fluid. This test is specific to narcolepsy, as there are no other conditions known that can cause a decrease in hypo cretin levels. 

Treatment 

While is no cure for narcolepsy, individuals with narcolepsy can lead full and productive lives with a combination of pharmacological treatment and lifestyle adjustments.
Every individual with narcolepsy is different and medications that work best for one individual may not work for best for another. Therefore, it is necessary to talk to your doctor or health care professional about the option for specific specialized treatments.
Medications for narcolepsy can include, sodium oxybate (Xyrem), stimulants, antidepressants, and selective serotonin reuptake inhibitors (SSRIs) and serotonin/norepinephrine reuptake inhibitors (SNRIs). 

Life style changes
Improve sleep hygiene: Sleep hygiene is the practice of healthy sleep habits that optimize nighttime sleep quality.
Exercise: Daily exercise performed more than 3 hours before bedtime promotes better quality sleep.
Rise: When it’s time to wake up, let in as much natural light as possible. Natural light assists internal regulation of our sleep-wake cycle so getting outdoors during the day is important too.
Listen to your body: Scheduled daytime naps are effective ways recharging your energy level.
Avoid smoking: especially at night 

Due to narcolepsy being poorly understood and largely invisible, many people experience feelings of isolation, loss of self-confidence, depression and anxiety. Additionally, the diagnosis of narcolepsy may affect education and career choice, as not all activities and routines fit into the type of schedule an individual with narcolepsy may require. Children and adolescents with narcolepsy may struggle with emotional regulation and display aggressive behaviors that lead to social exclusion and feelings of self-loathing and shame.  

With adequate medications and life style changes, narcolepsy can be brought under control. It is believed, Thomas Alwa Edison was narcoleptic. Late night show host Jimmy Kmmel is narcoleptic. What more assurance is required to prove that narcoleptics can be successful in any direction they choose! But make sure to use proper medications. 

Symptoms and signs:

As the cornea becomes more irregular in shape, it causes progressive nearsightedness and irregular astigmatism to develop, creating additional problems with distorted and blurred vision. Glare and light sensitivity also may occur.

Causes:

New research suggests the weakening of the corneal tissue that leads to keratoconus may be due to an imbalance of enzymes within the cornea. This imbalance makes the cornea more susceptible to oxidative damage from compounds called free radicals, causing it to weaken and bulge forward.

Risk factors for oxidative damage and weakening of the cornea include a genetic predisposition, explaining why keratoconus often affects more than one member of the same family.

Keratoconus also is associated with overexposure to ultraviolet rays from the sun, excessive eye rubbing, a history of poorly fitted contact lenses and chronic eye irritation.

In the mildest form of keratoconus, eyeglasses or soft contact lenses may help. But as the disease progresses and the cornea thins and becomes increasingly more irregular in shape, glasses and regular soft contact lens designs no longer provide adequate vision correction.

The aim of corneal cross-linking is to strengthen the cornea by increasing the number of “anchors” that bond collagen fibers together. 

Treatments for progressive keratoconus include:

1. Corneal crosslinking

Treatment by providing contact lenses

1.  Custom soft contact lenses
2.  Gas permeable contact lenses
3.  Silicone hydrogel contact lenses
4.  Hybrid contact lenses
5.  Scleral and semi-scleral lenses
6.  Prosthetic lenses

Surgery Option:

1.  Intacs (Addition Technology)
2. Topography-guided conductive keratoplasty
3. Corneal transplant

Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin.

Nephrogenic systemic fibrosis can also affect internal organs, such as the heart, kidneys and lungs, and it can cause a disabling shortening of muscles and tendons in the joints (joint contracture).

For some people with advanced kidney disease, being exposed to certain gadolinium-containing contrast agents during magnetic resonance imaging (MRI) and other imaging studies has been identified as a trigger for development of this disease.

Symptoms

Nephrogenic systemic fibrosis can begin days to months after exposure to gadolinium-containing contrast, but progresses quickly. Some signs and symptoms of nephrogenic systemic fibrosis may include:

• Swelling and tightening of the skin
• Thickening and hardening of the skin, typically on the arms and legs and sometimes on the body, but almost never on the face or head
• Skin that may feel “woody” and develop an orange-peel appearance and darkening (excess pigmentation)
• Burning, itching or severe sharp pains in areas of involvement
• Skin thickening that inhibits movement, resulting in loss of joint flexibility
• Rarely, blisters or ulcers

In some people, involvement of muscles and body organs may cause:

• Muscle weakness
• Limitation of joint motion caused by muscle tightening (contractures) in arms, hands, legs and feet
• Bone pain
• Reduced internal organ function, including heart, lung, diaphragm, gastrointestinal tract, or liver, but direct evidence is often lacking
• Yellow plaques on the white surface (sclera) of the eyes
• Blood clots

The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death.

Causes

The exact cause of nephrogenic systemic fibrosis isn’t fully understood. Exposure to gadolinium-containing contrast agents during magnetic resonance imaging (MRI) has been identified as a trigger for development of this disease. The Food and Drug Administration (FDA) recommends avoiding gadolinium-containing contrast agents in people with acute kidney injury or chronic kidney disease.

Other conditions that may lead to or promote the disease when severe kidney disease and exposure to gadolinium-containing contrast are present include:

• Use of high-dose erythropoietin (EPO), a hormone that promotes the production of red blood cells, often used to treat anemia
• Recent vascular surgery
• Blood-clotting problems
• Severe infection

Treatment

There is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies.

• Hemodialysis.
• Physical therapy.
• Kidney transplant.
• Ultraviolet A phototherapy.
• Extracorporeal photopheresis.
• Plasmapheresis.

Osteoarthritis is the most common form of arthritis, affecting millions of people worldwide. It occurs when the protective cartilage on the ends of your bones wears down over time. Although osteoarthritis can damage any joint in your body, the disorder most commonly affects joints in your hands, knees, hips and spine.

Symptoms

Osteoarthritis symptoms often develop slowly and worsen over time. Signs and symptoms of osteoarthritis include:

• Pain. Your joint may hurt during or after movement.
• Tenderness. Your joint may feel tender when you apply light pressure to it.
• Stiffness. Joint stiffness may be most noticeable when you wake up in the morning or after a period of inactivity.
• Loss of flexibility. You may not be able to move your joint through its full range of motion.
• Grating sensation. You may hear or feel a grating sensation when you use the joint.
• Bone spurs. These extra bits of bone, which feel like hard lumps, may form around the affected joint.

Causes

Osteoarthritis occurs when the cartilage that cushions the ends of bones in your joints gradually deteriorates. Cartilage is a firm, slippery tissue that permits nearly frictionless joint motion.

In osteoarthritis, the slick surface of the cartilage becomes rough. Eventually, if the cartilage wears down completely, you may be left with bone rubbing on bone.

Treatment

Currently, the process underlying osteoarthritis cannot be reversed, but symptoms can usually be effectively managed with lifestyle changes, physical and other therapies, medications, and surgery. Exercising and achieving a healthy weight are generally the most important ways to treat osteoarthritis. Your doctor may also suggest:

Medications:

• Acetaminophen.
• Nonsteroidal anti-inflammatory drugs (NSAIDs).
• Duloxetine (Cymbalta).

Therapy

• Physical therapy.
• Occupational therapy.
• Tai chi and yoga.

Surgical and other procedures:

If conservative treatments don’t help, you may want to consider procedures such as:

• Cortisone injections. Injections of corticosteroid medications may relieve pain in your joint. During this procedure your doctor numbs the area around your joint, then places a needle into the space within your joint and injects medication. The number of cortisone injections you can receive each year is generally limited to three or four injections, because the medication can worsen joint damage over time.
• Lubrication injections. Injections of hyaluronic acid may offer pain relief by providing some cushioning in your knee, though some research suggests these injections offer no more relief than a placebo. Hyaluronic acid is similar to a component normally found in your joint fluid.
• Realigning bones. If osteoarthritis has damaged one side of your knee more than the other, an osteotomy might be helpful. In a knee osteotomy, a surgeon cuts across the bone either above or below the knee, and then removes or adds a wedge of bone. This shifts your body weight away from the worn-out part of your knee.
• Joint replacement. In joint replacement surgery (arthroplasty), your surgeon removes your damaged joint surfaces and replaces them with plastic and metal parts. Surgical risks include infections and blood clots. Artificial joints can wear out or come loose and may need to eventually be replaced.

Hemodynamic stability can be simply explained as the stable blood flow. If a person is hemodynamically stable, it means that he/she has a stable heart pump and good circulation of blood. Hemodynamic instability is defined as any instability in blood pressure which can lead to inadequate arterial blood flow to organs. It is also a state where there is a requirement for physiological and mechanical support to ensure there is adequate cardiac input and output or blood pressure.

Abnormal hemodynamic parameters include heart rate, blood pressure, cardiac output, central venous pressure, and pulmonary artery pressure.

Signs and symptoms

Following are some signs and symptoms of hemodynamic instability:

1. Hypotension

2. Abnormal heart rate

3. Shortness of breath

4. Pulmonary congestion

5. Cold extremities

6. Peripheral cyanosis

7. Decreased urine output

8. Alternative consciousness (restlessness, loss of consciousness, confusion)

9. Chest pain

Hypotension can produce insufficient perfusion in the brain and heart due to autoregulation of these organs. The individual is mostly affected, when the particular threshold of the blood pressure become drop down and may cause cerebral and coronary ischemia.

Who is at risk?

Hemodynamic instability is common during surgery conducted under general anesthesia. An anesthesiologist is continuously monitoring the patient for maintaining the normal hemodynamic. Usually anesthesiologist plan a clinical management before commencing the surgery, depending upon the pre-existing condition of the patient.

For made an effective plan for hemodynamic management, it is necessary to have thorough knowledge of medical history. The risk of hemodynamic instability is more with patient having cardiovascular diseases and major surgery need relatively more complex planning for  hemodynamic management than healthy patient.

Recognizing hemodynamic instability

For recognizing the critically ill patient and avoidance of hemodynamic instability, the following diagnostic tests should pursue:

Clinical assessment in the seriously ailing is mainly utilized for the initial three rationales: for example to decide whether the individual patient has hemodynamic instability, to decide whether the individual patient is countering to treatment and to stratify threat of instability.

When a healthcare staff checking the systolic BP (blood pressure) or vessels fills up time the measuring of the result should be exact, precise, and offer further direction to specify the presence of any cardiac syndrome. If the obtained result provides any information which can direct the clinician about the presence of hemodynamic instability or shock, then it is easier to make some decision about the management of the hemodynamic instability, before onset of the condition.

Blood pressure recording

Method of clinical assessment of Hemodynamic Instability

The assessment of  vital sign and surrogate bio markers for assessing the organ perfusion, including urine output and capillary refill time have frequently used clinical assessment method to estimate hemodynamic instability. The below mentioned techniques are used as diagnostic tests for hemodynamic instability.

Vital signs

The monitoring of the vital signs is the primary clinical assessment for evaluating hemodynamic instability of a patient. Vital signs measurement is very important in judging the severity of the patient’s condition and to decide the any emergency medical intervention need to support for life saving of the patient or not. The following are the included elements of the vital signs.

Pulse

Variation of pulse rate may provide initial symptom of the development of the hemodynamic instability. The factors which mainly affect the pulse rate are fever, physical exhaustion or exercise, disorder of the thyroid gland and certain medications.

The increase level of catecholamine hormone during stress, haemorrage or dehydrations are different causes which influence pulse rate.  The postural alteration can affect pulse rate and provide the indication of  hypovolemia. Increase or decrease rate of pulse is a marker for arranging of a medical emergency team.

Respiratory rate

Respiratory rate is an important element to assessing the hemodynamic instability, as it is a marker of severity of illness. Respiratory rate become drop down for acute respiratory failure and immediate emergency medical assistance is required  to manage the patient ill health. Respiratory rate also provides guidance of patient reaction after giving any therapy.

Mean Arterial Pressure / Blood Pressure

Proper maintenance of the blood pressure is required to maintain the autoregulation of the heart and brain by keeping sustain perfusion. Mean Arterial Pressure / Blood Pressure is a good marker of hemodynamic instability. Less than 90 mm Hg in systolic blood pressure or less than 65 mm Hg of  Mean Arterial Pressure indicates severe sepsis and septic shock.

Temperature

High body temperature is also used as an indicator of clinical condition of the patient, though it is not sensitive marker of hemodynamic instability.

Toe- Temperature Gradient

Most of the clinicians suggest Toe- Temperature or rectal temperature gradient is useful diagnostic measure for adequacy of circulation. Toe- Temperature Gradient is a better indicator than  Mean Arterial Pressure, cardiac index or lactate to discriminate between survivors or non survivors for patient admitted to a ICU.

Organ perfusion

Organ perfusion is very important marker for diagnosing hemodynamic instability. hypoperfusion in specific organ indicative of insufficient perfusion in kidney or cardiac output.

Urine Output

The small quantity of urine production (oliguria) is another way to diagnose hemodynamic instability, but may associate with other clinical conditions, so it is an important but not specific clinical marker for hemodynamic instability.  insufficient renal perfusion is one of the cause of oliguria. 0.5ml/kg/hour urine output is a sarogate marker for severe sepsis.

Capillary refill time (CRT)

This clinical element is used to diagnosis the severity of instability of a patient treating under emergency care unit or ICU. The CRT is usually measure by preseeing the finger nail bed to occlute the circulation and then release the pressure and time is noted for how long normal coloration is achieved. CRT more than 6 seconds indicates emergency condition. The abnormal level of CRT along with cardiac index and urine output may indicate acute lung injury.

Brain swelling, also known as cerebral edema in medical terminology is a serious neurological condition. Swelling can occur in any organ of the body. It is body’s response to any type of injury, infection or an overuse of that particular organ or part of the body. Brain edema can develop as a result of an injury to brain or infection in the brain. It may involve certain part of brain or the whole brain.

Brain swelling produces increase in intracranial pressure. Intracranial pressure prevents proper blood flow to the brain as a result the brain cells are deprived of vital food that is oxygen and glucose.

Causes:

• Brain Injury: An injury on the head can damage the brain. Usually a severe injury to the skull such as in vehicular accidents, falling from a height, etc is responsible for swelling in brain.

  The injury can cause swelling of the brain tissue. The broken pieces of skull can injure the blood vessels which can lead to swelling of brain.

• Stroke and high blood pressure: Brain stroke caused due to ischemia of the blood vessel in the brain or hemorrhage inside the brain caused due to trauma or high blood pressure can cause cerebral edema.
• Infections: Bacterial infection or viral infection such as meningitis, encephalitis can cause swelling of brain tissues.
• Brain tumor: Edema of brain can be caused due to brain tumor. The growing brain tumor can compress the adjacent area of brain and block the circulating spinal fluid. These may cause rise in intracranial pressure and brain swelling. The growing blood vessels in and around the tumor can also cause brain edema.
• High altitude: Brain swelling can be one of the symptoms of high altitude sickness although not very common. The swelling is more likely to develop at an altitude 5000 feet and above. The condition is often accompanied with acute mountain sickness.

 Symptoms:

• Headache
• Neck pain or stiffness
• Nausea or vomiting
• Dizziness
• Irregular breathing
• Vision loss or changes
• Memory loss
• Inability to walk
• Difficulty speaking
• Stupor
• Seizures
• Loss of consciousness

Treatment for Edema:

Minor cases of brain swelling due to causes such as moderate altitude sickness or a slight concussion often resolve within a few days. In most cases, however, more treatment is needed quickly.

The goal is to assure that the brain receives enough blood and oxygen to remain healthy while the swelling is relieved and any underlying causes are treated. This may require a combination of medical and surgical treatments. Prompt treatment usually results in quicker and more complete recovery. Without it, some damage may remain.

Treatment for brain edema may include any combination of the following:

• Oxygen therapy: Providing oxygen through a respirator or other means helps make sure that the blood has enough oxygen in it. The doctor can adjust the respirator to help reduce the amount of swelling.
• IV fluids: Giving fluids and medicine through an IV can keep blood pressure from dropping too low. This helps to make sure that the body — including the brain — is receiving enough blood. However, some fluids can make swelling worse. Doctors attempt to use the right amounts of the right fluids in someone with brain swelling.
• Lowering body temperature (hypothermia): Lowering the temperature of the body and brain helps relieve swelling and allows the brain to heal. Hypothermia as a treatment for brain swelling is not widely used because it is difficult to perform correctly.
• Medication: In some cases of brain edema, your doctor may start a drug to help relieve the swelling. Medication may also be given for other reasons, such as to slow your body’s response to the swelling or to dissolve any clots. The drugs your doctor gives you depend on the cause and symptoms of brain swelling.
• Ventriculostomy: In this procedure, a surgeon cuts a small hole in the skull and inserts a plastic drain tube. Cerebrospinal fluid is drained from inside the brain, helping to relieve the pressure.
• Surgery: Surgery may have one or more of these goals:
  • Removing part of the skull to relieve intracranial pressure; this procedure is called decompressive craniectomy.
  • Removing or repairing the source of the swelling, such as repairing a damaged artery or vein or removing a growth

Hydrocephalus is the buildup of fluid in the cavities (ventricles) deep within the brain. The excess fluid increases the size of the ventricles and puts pressure on the brain. Cerebrospinal fluid normally flows through the ventricles and bathes the brain and spinal column. But the pressure of too much cerebrospinal fluid associated with hydrocephalus can damage brain tissues and cause a range of impairments in brain function. Hydrocephalus can happen at any age, but it occurs more frequently among infants and adults 60 and over. Surgical treatment for hydrocephalus can restore and maintain normal cerebrospinal fluid levels in the brain. Many different therapies are often required to manage symptoms or functional impairments resulting from hydrocephalus.

Symptoms

The signs and symptoms of hydrocephalus vary somewhat by age of onset.

Infants

Common signs and symptoms of hydrocephalus in infants include:

Changes in the head

• An unusually large head
• A rapid increase in the size of the head
• A bulging or tense soft spot (fontanel) on the top of the head

Physical signs and symptoms

• Vomiting
• Sleepiness
• Irritability
• Poor feeding
• Seizures
• Eyes fixed downward (sunsetting of the eyes)
• Deficits in muscle tone and strength
• Poor responsiveness to touch
• Poor growth

Toddlers and older children

Among toddlers and older children, signs and symptoms may include:

Physical signs and symptoms

• Headache
• Blurred or double vision
• Eyes fixed downward (sunsetting of eyes)
• Abnormal enlargement of a toddler’s head
• Sleepiness or lethargy
• Nausea or vomiting
• Unstable balance
• Poor coordination
• Poor appetite
• Seizures
• Urinary incontinence

Behavioral and cognitive changes

• Irritability
• Change in personality
• Decline in school performance
• Delays or problems with previously acquired skills, such as walking or talking

Young and middle-aged adults

Common signs and symptoms in this age group include:

• Headache
• Lethargy
• Loss of coordination or balance
• Loss of bladder control or a frequent urge to urinate
• Impaired vision
• Decline in memory, concentration and other thinking skills that may affect job performance

Older adults

Among adults 60 years of age and older, the more common signs and symptoms of hydrocephalus are:

• Loss of bladder control or a frequent urge to urinate
• Memory loss
• Progressive loss of other thinking or reasoning skills
• Difficulty walking, often described as a shuffling gait or the feeling of the feet being stuck
• Poor coordination or balance

When to see a doctor

Seek emergency medical care for infants and toddlers experiencing these signs and symptoms:

• A high-pitched cry
• Problems with sucking or feeding
• Unexplained, recurrent vomiting
• An unwillingness to move the head or lay down
• Breathing difficulties
• Seizures

Diagnosis

A diagnosis of hydrocephalus is usually based on:

• Your answers to the doctor’s questions about signs and symptoms
• A general physical
• A neurological exam
• Brain imaging tests

Neurological exam

The type of neurological exam will depend on a person’s age. The neurologist may ask questions and conduct relatively simple tests in the office to judge muscle condition, movement, well-being and how well the senses are functioning.

Brain imaging

Brain imaging tests can show enlarged ventricles caused by excess cerebrospinal fluid. They may also be used to identify underlying causes of hydrocephalus or other conditions contributing to the symptoms. Imaging tests may include:

• Ultrasound. Ultrasound imaging, which uses high-frequency sound waves to produce images, is often used for an initial assessment for infants because it’s a relatively simple, low-risk procedure. The ultrasound device is placed over the soft spot (fontanel) on the top of a baby’s head. Ultrasound may also detect hydrocephalus prior to birth when the procedure is used during routine prenatal examinations.
• Magnetic resonance imaging (MRI) uses radio waves and a magnetic field to produce detailed 3-D or cross-sectional images of the brain. This test is painless, but it is noisy and requires lying still.Children may need mild sedation for some MRI scans. However, some hospitals use a very fast version of MRI that generally doesn’t require sedation.
• Computerized tomography (CT) scan is a specialized X-ray technology that can produce cross-sectional views of the brain. Scanning is painless and quick. But this test also requires lying still, so a child usually receives a mild sedative.Drawbacks to CT scanning include less detailed images than an MRI, and exposure to a small amount of radiation. CT scans for hydrocephalus are usually used only for emergency exams.

Treatment

One of two surgical treatments may be used to treat hydrocephalus.

1. Shunt

The most common treatment for hydrocephalus is the surgical insertion of a drainage system, called a shunt. It consists of a long, flexible tube with a valve that keeps fluid from the brain flowing in the right direction and at the proper rate.

One end of the tubing is usually placed in one of the brain’s ventricles. The tubing is then tunneled under the skin to another part of the body where the excess cerebrospinal fluid can be more easily absorbed — such as the abdomen or a chamber in the heart.

People who have hydrocephalus usually need a shunt system for the rest of their lives, and regular monitoring is required.

2. Endoscopic third ventriculostomy

Endoscopic third ventriculostomy is a surgical procedure that can be used for some people. In the procedure, your surgeon uses a small video camera to have direct vision inside the brain. Your surgeon makes a hole in the bottom of one of the ventricles or between the ventricles to enable cerebrospinal fluid to flow out of the brain.

Complications of surgery

Both surgical procedures can result in complications. Shunt systems can stop draining cerebrospinal fluid or poorly regulate drainage because of mechanical malfunctions, blockage or infections. Complications of ventriculostomy include bleeding and infections.

Any failure requires prompt attention, surgical revisions or other interventions. Signs and symptoms of problems may include:

• Fever
• Irritability
• Drowsiness
• Nausea or vomiting
• Headache
• Vision problems
• Redness, pain or tenderness of the skin along the path of the shunt tube
• Abdominal pain when the shunt valve is in the abdomen
• Recurrence of any of the initial hydrocephalus symptoms

Other treatments

Some people with hydrocephalus, particularly children, may need additional treatment, depending on the severity of long-term complications of hydrocephalus.

A care team for children may include a:

• Pediatrician or psychiatrist, who oversees the treatment plan and medical care
• Pediatric neurologist, who specializes in the diagnosis and treatment of neurological disorders in children
• Occupational therapist, who specializes in therapy to develop everyday skills
• Developmental therapist, who specializes in therapy to help your child develop age-appropriate behaviors, social skills and interpersonal skills
• Mental health provider, such as a psychologist or psychiatrist
• Social worker, who assists the family with accessing services and planning for transitions in care
• Special education teacher, who addresses learning disabilities, determines educational needs and identifies appropriate educational resources