EMPHYSEMA AND MANAGEMENT

DEFINATION-”Emphysema is a condition of the lung characterised by  Permanent dilation of the air spaces distal to thr terminal bronchioles with destruction of the walls of these airways”.It is always associated with chronic bronchitis and is difficult to distinguish the two  Conditions during life.
CAUSES AND TYPES OF EMPHYSEMA
causes and predisposing factors-
1-CONGENITAL OR PRIMARY EMPHYSEMA –May be caused by the alfa1-antitrypsin deficiency.This is a rare in-herited condition, which affect one person in 4000 and results in the complete absence of one of the key antiprotease systems in the lung.This consequence is the early development of COPD, especially if the patient is already smoker.Although alpha1-antitrypsin deficiency is responsible for less than 1 per cent of cases of COPD, Its hereditary nature means that is worth diagnosing.It should, therefore, be consideres in any young COPD patient.
EMPHYSEMA may be SECONDARY to other factors, such as:
1.Obsructive airways disease -e.g. asthma , cystic fibrosis and chronic bronchitis.
2.Occupational lung disease-e. g. pneumoconiosis.
3.Compensatory to contraction of one secton of the lung-e.g. fibrous collapse or removal , when the remaining lung expands to fill the space.
TYPES OF EMPHYSEMA
Emphysema is usually of the panacinar (panlobular) types.
1.CENTRIACINAR EMPHYSEMA-
In centrilobular emphysema the upper zones of the lung are usually affected.This causes gross disturbance of the ventilation /perfussion relationship since there is a relatively well-preserved blood supply to the alveoli, but the amount of oxygen reaching the capillary is decreased owing to the damage to airways proximal to the alveoli.
2.PANACINAR EMPHYSEMA-Predominantly affects the lower lobes and lower lobe involvement is more common in individuals with alfa1-antitrypsin deficiency. This has a less drastic effect on the ventilation/perfussion relationship, since the blood supply in the damaged areas is decreased in proportion to the decreased ventilation in those areas.
PATHOPHYSIOLOGY-

 



The alveoli and the small distal airways are primarily affected by the disease, followed by effects in the larger airways . Elastic recoil is usually responsible for splinting the bronchioles open. However, with emphysema, the bronchioles lose their stabilizing function and therefore causing a collapse in the airways resulting in gas to be trapped distally.
There is an erosion in the alveolar septa causing there to be an enlargement of the available air space in the alveoli.
There is sometimes a formation of bullae with their thin walls of diminished lung tissue.
Smoking contributes to the development of the condition initially by activating the inflammatory process . The inhaled irritants cause inflammatory cells to be released from polymorphonuclear leukocytes and alveolar macrophages to move into the lungs. Inflammatory cells are known as proteolytic enzymes, which the lungs are usually protected against due to the action of antiproteases such as the alpha1-antitrypsin . However, the irritants from smoking will have an effect on the alpha1-antitrypsin, reducing its activity. Therefore emphysema develops in this situation when the production and activity of antiprotease are not sufficient to counter the harmful effects of excess protease production . A result of this is the destruction of the alveolar walls and the breakdown of elastic tissue and collagen. The loss of alveolar tissue leads to a reduction in the surface area for gas exchange, which increases the rate of blood flow through the pulmonary capillary system .
CLINICAL FEATURE OF EMPHYSEMA-
1. PROGRESSIVE DYSPNEA-Shortness of breath occurs initially on exertion , but as the disease progresses it will gradually occure after less and less activity and finally at rest .
2. RESPIRATORY PATTERN –The patient has a ‘fishlike’ inspiratory gasp, which is followed by prolonged, forced expiration usually against ‘pursed lips’.A ‘flick’ or bounce of the abdominal muscles may be seen on expiration as the outward flow of air is suddenly checked by obstruction of the airways.
3.COUGH WITH SPUTUM-This will be present if the disease is associated with chronic bronchitis or if there is infection.
4.CHEST SHAPE-The chest becomes barrel-shaped and fixed in inspiration, with widening of the intercostal spaces.There may also be indrawing of the lower intercostal spaces and supraclavicular fossa on inspiration .This is also associated with the difficulty of ventilating stiff lungs through narrowed airways.The ribs are elevated by the accesory muscles of respiration and there is loss of thoracic mobility.
5. POOR POSTURES-There may be a thoracic kyphosis plus elevated and protracted shoulder girdle.
6. POLYCYTHEMIA-This may develop in response to prolonge decrease in pao2 owing to the ventilation/perfusion imbalance.
7. CORE PULMONALE-This occurs in advanced stages of thje disease.
8.LUNG FUNCTION-The FEV1/FVC ratio is usually below 70per cent. RV is increased and lung volume may exceed the predicted total lung capacity(TLC).
EXAMINATION-The percussion note will be normal or hyper-resonant due to air trapping. Auscultation will reveal decreased breath sounds and prolonged expiration.
The chest X-RAY shows low flat diaphrams and hyperinflation.

 

PROGNOSIS OF EMPHYSEMA-The patients become progressively more disabled, with death ultimately occuring from respiratory failure.Complications of the emphysema is pneumothorax due to the rupture of the emphysematous bulla, and congestive cardiac failure.
MEDICAL MANAGEMENT OF COPD-
PRINCIPLES OF TREATMENT-
1-Decrease the bronchial irritation to a minimum stop smoking , avoid dusty, smoky , damp or foggy atmosphere.
2- Control infections-The should have a supply of antibiotics at home and receive a vaccination against influenza each winter.
3-Control bronchospasm
4-Control/decrease the amount of sputum
5-Oxygen therapy
6-Long-term oxygen therapy (LTOT)
MEDICATIONS-
RELIEVERS-
1- Beta2 agonists-(salbutamol and turbutalin) ,and long acting beta agonist (salmeterol and formoterol) the 2-Anticholinergics-(Ipratropium bromoide and Oxitropium bromide)
3-Xanthen derivatives -(Theophyllin and Aminophyllin)
PREVENTERS-
1- Corticosteroids-(Beclometason, Budesonide)
PHYSIOTHERAPY MANAGEMENT IN COPD-
Goals of treatment for COPD include:

• Relieving symptoms
• Slowing progress of the disease
• Improving exercise tolerance (ability to stay active)
• Preventing and treating complications
• Improving overall health
• COPD is not a reversible condition and has no cure yet, but conservative treatment for COPD can slow its progression (smoking cessation being the most important).

PHYSIOTHERAPY TRATMENT FOR COPD-
Aims of COPD Therapy

• To remove excess bronchial secretion and reduce the airflow obstruction.
• To establish the coordinated pattern of breathing
• To promote relaxation and improve posture
• To improve the mobility of thorax, shoulder girdle and neck
• To increase the exercise tolerance
• To encourage a full and active life style.

Means of COPD Treatment
1.POSTURAL DRAINAGE (P.D) is necessary for all patients. In chronic bronchitis regular postural drainage should be given. In case of acute emphysema, postural drainage is not necessary but in an infection episode,  where sputum may be present PD may be needed. The optimum position must be established with individual and advice for postural drainage at home.
Clapping and Shaking are effective over the affected lung segments and help to loosen and move the secretions to central airways during expiration. Then ask the patient to take 2-3 coughs to remove the secretions out. If the patient is unable to clear the secretions which are accumulated in lungs, then increased ventilation and humidification by IPPB conjoint with P.D are provided. This is very effective for patient. But in presence of emphysematous bullae, it is contraindicated because of risk of causing pneumothorax.

2. ACTIVE CYCLE OF BREATHING TEQNIC

The Active Cycle of Breathing Techniques (ACBT) is an active breathing technique performed by the patient to help clear their sputum the lungs. The ACBT is a group of techniques which use breathing exercises to improve the effectiveness of a cough, loosen and clear secretions and improve ventilation.
ACBT consists of three main phases:
1.Breathing Control
2.Deep Breathing Exercises or thoracic expansion exercises
3.Huffing OR Forced Expiratory Technique (F.E.T)
Additionally, a manual technique (MT) or positive pressure can be added if and when indicated, to create a more complex cycle to help improve removal of secretions on the lungs.
1.Breathing Control
Breathing control is used to relax the airways and relieve the symptoms of wheezing and tightness which normally occur after coughing or breathlesness. Breathing should be performed gently through the nose using as little effort as possible. If this is not possible then breathing should be done by mouth. If it is necessary to breathe out through the mouth this should be done with ‘pursed lips breathing’. While performing this technique it is important to encourage the patient use it as an opportunity to reduce any tension they may have, Encouraging the patient to close their eyes while performing Breath Control can also be beneficial in helping to promote relaxation. It is very important to use Breathing Control in between the more active exercises of ACBT as it allows for relaxation of the airways. Breathing Control can also help you when you are short of breath or feeling fearful, anxious or in a panic. The length of time spent performing Breathing Control will vary depending on how breathless patient feels.
When using this technique with a patient as part of the ACBT the patient should be instructed to usually 6 breaths. Instructions to patient: Rest one hand on your stomach and keep your shoulders relaxed to drop down. Feel your stomach rise as you breathe in and fall when you breathe out.
2.Deep Breathing Exercises
Deep breathing is used to get air behind the sputum stuck in small airways:
Relax your upper chest.
Breathe in slowly and deeply.
Breathe out gently until your lungs are empty – don’t force the air out.
Repeat 3 – 4 times, if the patient feels light headed then it is important that they revert back to the Breathing Control portion of the cycle.
At the end of the breath in, hold the air in your lungs for 3 seconds (this is known as an inspiratory hold).
Deep breathing/thoracic expansion exercises recruit the collateral ventilatory system assisting, the movement of air distal to mucus plugs in the peripheral airways.
Deep breaths to utilise collateral channels and get air behind sputum to mobilise it towards larger airways and towards the mouth. Instructions to patient:

• Relax your shoulders.
• Place both hands on either side of ribs.
• Breathe in deeply feeling as your ribs expand.
• Breathe out gently as far as you can until your lungs feel empty.
• Deep breathing/thoracic expansion is usually repeated 4 times.

3.Huffing or FET (FORCE EXPIRATORY TECNIC)
The FET is an integral part of the ACBT described by Pryor and Webber .

• A huff is exhaling through an open mouth and throat instead of coughing.Huffing moves sputum from the small airways to the larger airways, from where they are removed by coughing. Coughing alone does not remove sputum from small airways.

1.Take a small-medium sized breath in

• Squeeze the breath out by contracting your tummy muscles and keep your mouth and throat open to perform a huff. This small-medium sized huff helps with the removal of sputum in the lower reaches of the lungs.
• To remove sputum in the higher portions of the lungs take a large breath in.
• Squeeze the air out as before to perform a huff.
• Cough and expectorate any sputum. If no sputum is produced with 1 or 2 coughs, try to stop coughing by encouraging the patient to use Breathing Control, the main technique used in between the more active stages of the as ACBT.
• Allow your breathing to settle with breathing control and then repeat the cycle until your chest feels clear.
  Small long huffs move sputum from low down into chest whereas big short huffs moves sputum from higher up into chest, so use this huff when it feels ready to come out, but not before; huffs work via dynamic compression.

2.Take a medium sized breath in.
Squeeze the breath out fairly hard and fast keeping mouth and throat open. Imagine trying to steam up a mirror or blow a tissue held out in front of you.
Attempt to clear sputum 2-3 times then return to breathing control (Phase one) to relax airways.
Repeat as above except for a larger breath in to remove secretions/sputum in other areas of the lungs.
In case of acute exacerbation of chronic bronchitis the physiotherapy must be given vigorously. IPPB has great value in this case. It is given with mask to improve ventilation. It is important to observe the chest wall movement and level of consciousness. The patient should be observed for any signs of drowsiness after taking the treatment. Assisted ventilation with vigorous chest shacking and postural drainage are more effective. A ventilatory mask providing controlled oxygen therapy is usually required.
3.BREATHING EXERCISE- should be given in a correct way in treatment for COPD. The main emphasis is given on diaphragmatic breathing with relaxed expiration. The diaphragmatic breathing with decreased upper chest movements and relaxed shoulder girdle is preferred. Expansion of basal lung segments are taught to ventilate these areas.
Pursed lip breathing with prolonged expiration is given as treatment for COPD especially in presence of emphysematous bullae.
3. POSTURE CORERECTION Patient should be taught to attain maximal relaxation of the upper chest as well as movements of lower chest. The main emphasis is on relaxed and controlled diaphragmatic breathing. For maintaining posture the patient should not be kept with forward head and rounded shoulder.
4. THORACIC MOBILITY EXERCISE are given along with shoulder girdle movements. Free active exercises for whole spine to prevent kyphosis and fixed inspiration.
Important COPD exercise in sitting is trunk turning with loose arm swinging in rotation for relaxation. It is also essential to emphesise postural awareness so that the patient practices shoulder girdle retraction and lateral rotation of arm.
5. These patients should be as mobile and active as possible. Their exercise tolerance may be increased by gradually increasing the distances walked both on the flat and upstairs or slopes while practising breathing control. A graduated exercise programme can also be given to these patients during the later part of their stay in hospital and should be continued at home.
6. In daily life style patient should avoid smoking and encouraged to keep fit and eat sensibly. For gaining relaxation, swimming helps very much. Jerky and quick movements should be strictly avoided.

CYSTIC FIBROSIS

DEFINATION – CYSTIC FIBROSIS(CF) is a hereditary disorder of the exocrine glands, with a high sodium chloride content in sweat and pancreatic insufficiency, resulting in the, malabsorption . There is hypertrophy and hyperplasia of mucus secreting glands, resulting in the excessive mucus production in the lining if the bronchi , which predispose the patient to chronic bronchopulmonary infection”.
Cystic fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with cystic fibrosis, a defective gene causes the secretions to become sticky and thick. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.
Although cystic fibrosis requires daily care, people with the condition are usually able to attend school and work, and often have a better quality of life than people with cystic fibrosis had in previous decades. Improvements in screening and treatments mean people with cystic fibrosis now may live into their mid- to late 30s, on average, and some are living into their 40s and 50s.
SYMPTOMS-

SYMPTOMS IN CF

Cystic fibrosis signs and symptoms vary, depending on the severity of the disease. Even in the same person, symptoms may worsen or improve as time passes. Some people may not experience symptoms until adolescence or adulthood.
People with cystic fibrosis have a higher than normal level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other signs and symptoms of cystic fibrosis affect the respiratory system and digestive system. However, adults diagnosed with cystic fibrosis are more likely to have atypical symptoms, such as recurring bouts of inflamed pancreas (pancreatitis), infertility and recurring pneumonia.
RESPIRATORY SIGNS AND SYMPTOMS-
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause signs and symptoms such as:

• A persistent cough that produces thick mucus (sputum)
• Wheezing
• Breathlessness
• Exercise intolerance
• Repeated lung infections
• Inflamed nasal passages or a stuffy nose

DIGESTIVE SIGNS AND SYMPTOMS-
The thick mucus can also block tubes that carry digestive enzymes from your pancreas to your small intestine. Without these digestive enzymes, your intestines aren’t able to completely absorb the nutrients in the food you eat. The result is often:

• Foul-smelling, greasy stools
• Poor weight gain and growth
• Intestinal blockage, particularly in newborns (meconium ileus)
• Severe constipation

Frequent straining while passing stool can cause part of the rectum — the end of the large intestine , to protrude outside the anus (rectal prolapse). When this occurs in children, it may be a sign of cystic fibrosis. Parents should consult a physician knowledgeable about cystic fibrosis. Rectal prolapse in children may sometimes require surgery. Rectal prolapse in children with cystic fibrosis is less common than it was in the past, which may be due to earlier testing, diagnosis and treatment of cystic fibrosis.
CAUSES AND RISK FACTORS-

    CYTIC FIBROSIS  AND HEREDITARY

Cystic Fibrosis is an autosomal recessive disease caused by defects in the CFTR gene . This CFTR gene normally encodes for a protein that functions as a chloride channel and is regulated by cyclic AMP, but in cystic fibrosis, the CFTR gene mutates and results in abnormalities of cyclic AMP, which regulates the transport of chloride across the epithelial cells on mucosal surfaces. This defective gene causes the body to produce an abnormally thick and sticky fluid in the respiratory and GI tracts, the pancreas and in the sweat glands.
In order to develop CF, two CF genes must be inherited, one from the mother and the other from the father. If only one CF gene is inherited, then the person is called a carrier and will have no symptoms. When both parents are carriers, with each pregnancy there is a: 1 in 4 chance of having a child with cystic fibrosis, 1 in 2 chance of having a child who is a carrier or 1 in 4 chance of having an unaffected child.
There are several different types of genetic mutation which are associated with different degrees of severity of the disease

• COMPLICATIONS-
  RESPIRATORY SYSTEM AND COMPLICATIONS-
  1.Damaged airways (bronchiectasis). Cystic fibrosis is one of the leading causes of bronchiectasis, a condition that damages the airways. This makes it harder to move air in and out of the lungs and clear mucus from the airways (bronchial tubes).
• 2.Chronic infections. Thick mucus in the lungs and sinuses provides an ideal breeding ground for bacteria and fungi. People with cystic fibrosis may often have sinus infections, bronchitis or pneumonia.
• 3.Growths in the nose (nasal polyps). Because the lining inside the nose is inflamed and swollen, it can develop soft, fleshy growths (polyps).
• 4.Coughing up blood (hemoptysis). Over time, cystic fibrosis can cause thinning of the airway walls. As a result, teenagers and adults with cystic fibrosis may cough up blood.
• 5.Pneumothorax. This condition, in which air collects in the space that separates the lungs from the chest wall, also is more common in older people with cystic fibrosis. Pneumothorax can cause chest pain and breathlessness.
• 6.Respiratory failure. Over time, cystic fibrosis can damage lung tissue so badly that it no longer works. Lung function usually worsens gradually, and it eventually can become life-threatening.
• 7.Acute exacerbations. People with cystic fibrosis may experience worsening of their respiratory symptoms, such as coughing and shortness of breath, for several days to weeks. This is called an acute exacerbation and requires treatment in the hospital.
• DIGESTIVE SYSTEM AND COMPLICATIONS-
• 1.Nutritional deficiencies. Thick mucus can block the tubes that carry digestive enzymes from your pancreas to your intestines. Without these enzymes, your body can’t absorb protein, fats or fat-soluble vitamins.
• 2.Diabetes. The pancreas produces insulin, which your body needs to use sugar. Cystic fibrosis increases the risk of diabetes. Around 30 percent of people with cystic fibrosis develop diabetes by age 30.
• 3.Blocked bile duct. The tube that carries bile from your liver and gallbladder to your small intestine may become blocked and inflamed, leading to liver problems and sometimes gallstones.
• 4.Intestinal obstruction. Intestinal obstruction can happen to people with cystic fibrosis at all ages. Children and adults with cystic fibrosis are more likely than are infants to develop intussusception, a condition in which a section of the intestines folds in on itself like an accordion.
• 5.Distal intestinal obstruction syndrome (DIOS). DIOS is partial or complete obstruction where the small intestine meets the large intestine.
• REPRODUCTIVE SYSTEM AND COMPLICATIONS-
  1.Almost all MEN with cystic fibrosis are infertile because the tube that connects the testes and prostate gland (vas deferens) is either blocked with mucus or missing entirely. Certain fertility treatments and surgical procedures sometimes make it possible for men with cystic fibrosis to become biological fathers.
• 2.Although WOMEN with cystic fibrosis may be less fertile than other women, it’s possible for them to conceive and to have successful pregnancies. Still, pregnancy can worsen the signs and symptoms of cystic fibrosis, so be sure to discuss the possible risks with your doctor.

OTHER COMPLICATIONS-
1.Thinning of the bones (osteoporosis). People with cystic fibrosis are at higher risk of developing a dangerous thinning of bones.
2.Electrolyte imbalances and dehydration. Because people with cystic fibrosis have saltier sweat, the balance of minerals in their blood may be upset. Signs and symptoms include increased heart rate, fatigue, weakness and low blood pressure.
DIAGNOSIS-
With a complete medical history and physical examination, diagnostic procedure may include the following:
SWEAT (CHLORIDE)TEST: A test, which measures the amount of chloride in the sweat. If the amount of chloride is higher than the normal amounts it may suggest cystic fobrosis. The sweat test is not painful.
1.BLOOD TEST: Blood or cheek scraping cells can also be used to detect mutations of the CFTR gene.
2.CHEST X-RAY: A diagnostic test, which produce images of internal tissues, bones and organs.

         CHEST X-RAY IN CYSTIC FIBROSIS

cystic-fibrosiss3.PULMONARY FUNCTION TEST: Measures the lungs’ ability to exhange oxygen and carbondioxide appropriately.
4.SPUTUM CULTURE: Is often performed to determine if an infection is present.
5.STOOL EVACUATION-: To measure stool fat absorption.Pancreatic function
6.COMPUTED TOMOGRAPHY SCAN: Detect early pulmonary disease before synptoms occur.
7.MAGNETIC RESONANCE IMAGINE(MRI): Is not only used to screen the lung, but also the heart, sinuses and GI tract.
PROGNOSIS-
Prognosis has improved over the past fifty years because of advancement in the management, especially before the irreversible pulmonary changes. The median age for survival is 35 years, long term survival is better in patients without pancreatic insufficiency. The following have been associated with worse prognosis; females, early colonization with mucoid Pseudomonas, presentation with respiratory symptoms, tobacco smoke exposure and airway hyperactivity.
MEDICAL MANAGEMENT-
Although airway clearance continues to be an integral part of care, physical exercise, postural care and the need to address the unique complications which emerge as a result of improved longevity have significantly changed the nature of physiotherapy in CF.
There is no cure for CF and most people with the disease die between the ages of 20 and 30 as a result of lung failure. Early diagnosis and a complete treatment plan can improve both survival and quality of life for the person suffering from CF.
MEDICAL MANAGEMENT-
Treatment of CF includes:
Antibiotics for respiratory infections
Vitamin supplements
Inhaled medicines to help open the airways
DNAse enzyme replacement therapy to thin the mucus and makes it easier to expectorate
Pain relievers
Enzyme therapy is done with each meal or snack, where most people with CF will need to take replacement enzymes such as pancreatin (eg Pancrex). These supply the missing pancreatic enzymes and allow proper digestion. People with CF normally need vitamin and mineral supplements too.
The usual childhood vaccinations, such as measles, mumps and rubella (MMR) and diphtheria, tetanus and whooping cough (DTP) are important for the children with CF. If you have CF you should also be vaccinated against flu and pneumococcus to help prevent chest infections.
CF is one of the most extensively researched genetic diseases as a target for gene therapy development and this can also serve as an important model for gene therapy of other diseases. The aim of gene therapy is to correct the basic defect in CF. This can be achieved by the development of a vector. This can be done by doing preclinical and clinical research. Clinical studies have identified the host immune response and low vector efficiency as key impediments to effective CF gene therapy.
PHYSIOTHERAPY MANAGEMENT-
The main aim of physiotherapy is to remove excessive mucoid secretions in order to improve ventilation. A person suffering from CF will require intensive chest physiotherapy. This will include the following:

• VIGOROUS MASSAGE to help loosen the sticky mucus
• PERCUSSION-This technique is also known as chest clapping, and is used to help loosen secretions. To perform this technique a cupped hand is used to clap the chest firmly and rhythmically (over a layer of clothing or a towel) .
• SHAKING AND VIBRATION- This technique consists of several short rhythmical squeezes to the chest while exhaling to mobilze secretions .
  Regular assessment and monitoring is necessary during physiotherapy treatment as the patient may require supplemental oxygen, especially in advanced cystic fibrosis.

Other techniques include:

• POSITIONING- lying on either side, front or back and sitting
  Active cycle of breathing (ACBT): This technique consist of 1.Breathing Control (BC), 2.Thoracic Expansion Exercises (TEE), and 3.Forced Expiration Technique (FET).
• ACBT
  1.Breathing Control (BC): Relaxed diaphragmatic breathing which allows for rest and helps avoid any tightening of airways, which can make it difficult to clear secretions
  2.Thoracic expansion exercises (TEE): Deep breathing exercises that help the lungs to expand more effectively and allow air to get behind secretions so that they can be “pushed” up the airways towards the mouth. The breaths should be slow and deep with a pause at the end of inspiration then followed by relaxed quiet expiration .
  3.Forced Expiration Technique (FET): This consists of huffing or a sigh, to help move secretions from the smaller to the larger airways from where they can be cleared more easily.
• Positive expiratory pressure (PEP): A technique that helps to open up airways and get air behind secretions to help move them higher up the airway. The PEP device (mask or mouthpiece) gives a small degree of resistance to the breath out and this resistance splints open the airways. Bubble PEP is used in young children and involves the child blowing through a straw placed in a bottle of water and soap to produce bubbles
• Oscillating PEP- These devices combine vibrations of airways with PEP and there are several different types. The Flutter is a small pipe shaped device that interrupts the flow of air and gives an intermittent back pressure to the airways as well as causing them to vibrate. The Cornet and the Acapella are two other oscillating PEP devices that function similarily to the Flutter device
• POSTURAL DRAINAGE-
  Postural drainage sounds complicated, but it’s really just a way to use gravity to drain mucus out of your lungs by changing positions. It’s used to treat a variety of conditions, including chronic diseases such as cystic fibrosis and bronchiectasis, as well as temporary infections, such as pneumonia.

If you have a bad cold or flu, you can also use postural drainage to help keep mucus out of your lungs. The goal is to move mucus into the central airway, where it can be coughed up. It’s safe for people of all ages and can be done either at home or in a hospital or nursing facility.
Postural drainage is often done at the same time as percussion, sometimes called clapping, which involves someone clapping on your back, chest, or sides with a cupped hand in order to shake mucus loose from the lungs. These techniques, along with vibration, deep breathing, and huffing and coughing, are referred to as chest physiotherapy, chest physical therapy, or airway clearance therapy.
TEQNIC OF POSTURAL DRAINAGE-
You can do postural drainage with many positions, either on your own or with a physical therapist or nurse.

• General guidelines
  Each position should be held for a minimum of five minutes.
  Positions can be done on a bed or on the floor.
  In each position, your chest should be lower than your hips to allow mucus to drain.
  Use pillows, foam wedges, and other devices to make yourself as comfortable as possible.
  While in the positions, try to breathe in through your nose and out through your mouth for longer than you breathe in for maximum effectiveness.
  Do these positions in the morning to clear mucus that’s built up overnight or right before bed to prevent coughing during the night.
  A respiratory therapist, nurse, or doctor can recommend the best ways to perform postural drainage based on where the mucus is.
• ON YOUR BACK-
  Your chest should be lower than your hips, which you can achieve by lying on a slanted surface or propping your hips up about 18 to 20 inches with pillows or another item.
  This position is best for draining the bottom front parts of your lungs.
• ON YOUR SIDES-
  With pillows under your hips, lie on one side so that your chest is lower than your hips.
  To clear congestion from the bottom part of the right lung, lie on your left side.
  To clear congestion from the bottom part of your left lung, lie on your right side.
• ON YOUR STOMACH-
  Drape your body over a stack of pillows or other object, such as a beanbag, and rest your arms by your head, with your chest lower than your hips.
  This position is best for clearing mucus in the lower back area of the lungs.

More research is needed to know how effective postural drainage really is. In the meantime, your doctor may be able to suggest postural drainage positions or other chest physiotherapy techniques that may work for you. They can also refer you to a respiratory therapist or physical therapist who specializes in chest physiotherapy.
RISK ASSOCIATED POSTURAL DRAINAGE-
You may vomit if you do postural drainage right after eating. Try to do the positions before eating or 1 1/2 to 2 hours after a meal.
If left untreated, mucus in the lungs can turn into a serious condition, so make sure to follow up with your doctor if you decide to try postural drainage. You may need additional treatment. Mucus in the lungs can also be a sign of an underlying condition that needs medical treatment, such as chronic pulmonary obstructive disease (COPD).
Get emergency treatment if you have any of the following symptoms during or after postural drainage:

• Shortness of breath
• Trouble breathing
• Confusion
• Skin that turns blue
• Coughing up blood
• Severe pain

BRONCHIECTASIS AND MANAGEMENT

BRONCHIECTASIS

MAY 17, 2018ADMIN0 COMMENTS

BRONCHIECTASIS

FILED UNDERANATOMY DISEASE EXERCISE DETAIL MUSCLE DETAIL PHYSIOTHERAPY TECHNIQUE

DEFINATION- Bronchiectasis is an obstructive lung disease that results from the presence of chronic inflammatory secretions and microbes leading to the permanent dilation and distortion of airway walls, as well as recurrent infection .

MECHANISM OF INJURY/ PATHOLOGICAL PROCESS-
Bronchiectasis is chronic irreversible dilation of the bronchi on the lungs. It follows a severe lung infection or aspiration. It is more common in conditions such as Cystic Fibrosis, Rheumatoid Arthritis, Immunodeficiency, Young’s syndrome and Allergic Bronchopulmonary Aspergillosis, and after chilhoos diseases such as whooping cough, TB and measles.

CLINICALLY RELEVANT ANATOMY-

ANATOMY OF LUNG

Bronchiectasis involves inflammation of the airway walls, specifically the bronchial walls . The airway walls are the ‘tubes’ that run from the mouth and nose and travel to the lung. The main area that is affected in bronchiectasis is the bronchi . The trachea bifurcates into the right and left main bronchi, which then further divide into secondary bronchi; one for each lobe of the lung . They then divide into tertiary bronchi; one for each bronchopulmonary segment .

The cilia are also damaged in bronchiectasis. Cilia line the airway and are attached to the epithelium. They are hair-like with tiny hooks on the tip to grab the mucous and help move the mucous up to the throat .

EPIDEMIOLOGY-
It is underestimated in prevalence, incidence, and morbidity because symptoms are often ascribed to smoking. A study by Hill found that one in 1000 Britans have bronchiectasis, with associated deaths increasing at a rate of approximately 3% per year in England and Wales . Though the overall mortality rate is increasing in these countries, rates in older groups are rising whereas rates in younger groups are falling . A disproportionate amount of cases found in developing countries and in Aboriginal populations in affluent communities . In some populations, an improvement in socio-economic status, housing, and education can improve overall health and reduce the incidence of respiratory infection and development of bronchiectasis .

AETIOLOGY-
The cause is unknown for 50% of cases, but it has been linked to inflammatory bowel disease, rheumatoid arthritis, and in 29-50% of patients, COPD .There are several associated conditions with bronchiectasis including; cystic fibrosis, primary ciliary dyskinesia, lung tuberculosis, allergic bronchopulmonary aspergilosis, rheumatoid diseases, symptomatic lupus erythematosus, immune deficiency, severe childhood respiratory infection and exposure to foreign body or corrosive substance . It has also been linked to an x1-antitrypsin deficiency . Bronchiectasis tends to predispose patients to acquired and congenital lung infections.

PATHOPHYSIOLOGY-
Currently, a high-resolution CT scan is the gold standard for diagnosis of bronchiectasis. Parallel tramlines and ring shadows may be present on the scan, indicating thickened airway walls and dilated airways, respectively. There may also be ‘glove finger shadows’, which are finger –like projections that represent the dilated bronchi filled with solidified secretion.
Spirometry will simply identify if there is an airway obstruction, but this can be indicative of many other diseases.
Microbiology can also be used to identify the bacteria present during an exacerbation . A sputum sample is sent off and the results are returned with the incidence of bacterial colonization measured .

CLINICAL MENIFESTATION-
As delicate cilia are often damaged due to the presence of thick mucus, the voluminous quantities of sputum formed cannot be efficiently cleared. These secretions will lead to the production of wheezes, coarse crackles and squeaks on auscultation. Often times, patients will also present with muscular dysfunction due to inflammation, gas exchange abnormalities, inactivity, malnutrition, hypoxia and medications. Dyspnoea on exertion seems to be a major clinical manifestation as it is experienced by three out of four patients. Other physical manifestations include chest pain, snoring, finger clubbing, fatigue, persistent cough, recurrent infection, and loss of appetite. Stress incontinence can also be a result of excess coughing. Bronchiectasis tends to also have psychological manifestations, such as anxiety, depression, reduced confidence, altered relationships and time off work.

Exacerbations can occur several times a year and are identified by four or more of the following signs and/or symptoms: change in sputum, increased dyspnoea, increased cough, fever of greater than 38°, increased wheeze, decreased exercise tolerance, fatigue, lethargy, and radiographic signs of a new infection.

DIAGNOSIS-

   ABNORMAL AIRWAY DILATION OF LUNG

Currently, a high-resolution CT scan is the gold standard for diagnosis of bronchiectasis . Parallel tramlines and ring shadows may be present on the scan, indicating thickened airway walls and dilated airways, respectively. There may also be ‘glove finger shadows’, which are finger –like projections that represent the dilated bronchi filled with solidified secretions.
Spirometry will simply identify if there is an airway obstruction, but this can be indicative of many other diseases.
Microbiology can also be used to identify the bacteria present during an exacerbation . A sputum sample is sent off and the results are returned with the incidence of bacterial colonization measured .

PHYSIOTHERAPY AND OTHER MANAGEMENT-

Treatment for bronchiectasis aims to:

• Decrease symptoms (such as cough and fatigue)
• Decrease frequency and severity of exacerbations
• Decrease hospital admissions
• Improve quality of life
• Improve exercise tolerance
• Maintain or improve lung function
• Prolong survival
• Defining patient specific treatment goals.

An important part of a management plan is to define patient specific treatment goals. This may include:
Identifying:

• Symptoms type and duration
• Sputum volume and characteristics (colour thickness, smell, ease of clearance, presence of blood)
• Frequency of exacerbations (previous hospitalisations and antibiotics)
• Responses (and adverse effects) to previous treatments (antibiotics)
• Haemoptysis volume and frequenc

• Identifying co-morbidities that can contribute to bronchiectasis symptoms
• Sinusitis
• Gastro Oesophageal Reflux (GORD)
• Asthma / ABPA
• COPD and chronic bronchitis
• Identify and promoting self-care activities
• Exercise
• Airway clearance
• Symptom monitoring

The overall treatment plan aims to minimise symptoms and exacerbations with the long-term objective of maintaining lung function.

Physiotherapy is an essential part of the treatment for bronchiectasis. Because of the infection in the lung, secretions build up. Being able to cough the secretions up is important as this drains the chest of infected mucus.

All patients with bronchiectasis will have time with a physiotherapist, who will teach the techniques for getting the mucus up and draining the secretions from the lungs. The three methods illustrated in the video are the commonest forms of physiotherapy.

The first two, Acapalla and Active cycle of breathing can be taught by a physiotherapist. This will enable the you to perform these chest clearance methods on your own helping to keep you as healthy and infection free as possible.

The Active Cycle of Breathing Techniques (ACBT) is an active breathing technique performed by the patient to help clear their sputum the lungs. The ACBT is a group of techniques which use breathing exercises to improve the effectiveness of a cough, loosen and clear secretions and improve ventilation.

ACBT consists of three main phases:

                   ACBT TEQNIC

1.Breathing Control
2.Deep Breathing Exercises or thoracic expansion exercises
3.Huffing OR Forced Expiratory Technique (F.E.T)
Additionally, a manual technique (MT) or positive pressure can be added if and when indicated, to create a more complex cycle to help improve removal of secretions on the lungs.

1.BREATHING CONTROL
Breathing control is used to relax the airways and relieve the symptoms of wheezing and tightness which normally occur after coughing or breathlesnes. Breathing should be performed gently through the nose using as little effort as possible. If this is not possible then breathing should be done by mouth. If it is necessary to breathe out through the mouth this should be done with ‘pursed lips breathing’. While performing this technique it is important to encourage the patient use it as an opportunity to reduce any tension they may have, Encouraging the patient to close their eyes while performing Breath Control can also be beneficial in helping to promote relaxation. It is very important to use Breathing Control in between the more active exercises of ACBT as it allows for relaxation of the airways. Breathing Control can also help you when you are short of breath or feeling fearful, anxious or in a panic. The length of time spent performing Breathing Control will vary depending on how breathless patient feels.

When using this technique with a patient as part of the ACBT the patient should be instructed to usually 6 breaths. Instructions to patient: Rest one hand on your stomach and keep your shoulders relaxed to drop down. Feel your stomach rise as you breathe in and fall when you breathe out.

2.DEEP BREATHING EXERCISE
Deep breathing is used to get air behind the sputum stuck in small airways:
Relax your upper chest.
Breathe in slowly and deeply.
Breathe out gently until your lungs are empty – don’t force the air out.
Repeat 3 – 4 times, if the patient feels light headed then it is important that they revert back to the Breathing Control portion of the cycle.
At the end of the breath in, hold the air in your lungs for 3 seconds (this is known as an inspiratory hold).
Deep breathing/thoracic expansion exercises recruit the collateral ventilatory system assisting, the movement of air distal to mucus plugs in the peripheral airways.
Deep breaths to utilise collateral channels and get air behind sputum to mobilise it towards larger airways and towards the mouth. Instructions to patient:

Relax your shoulders.
Place both hands on either side of ribs.
Breathe in deeply feeling as your ribs expand.
Breathe out gently as far as you can until your lungs feel empty.
Deep breathing/thoracic expansion is usually repeated 4 times.

3.HUFFING or FET
The FET is an integral part of the ACBT described by Pryor and Webber .

A huff is exhaling through an open mouth and throat instead of coughing.Huffing moves sputum from the small airways to the larger airways, from where they are removed by coughing. Coughing alone does not remove sputum from small airways.

Take a small-medium sized breath in
Squeeze the breath out by contracting your tummy muscles and keep your mouth and throat open to perform a huff. This small-medium sized huff helps with the removal of sputum in the lower reaches of the lungs.

To remove sputum in the higher portions of the lungs take a large breath in.
Squeeze the air out as before to perform a huff.

Cough and expectorate any sputum. If no sputum is produced with 1 or 2 coughs, try to stop coughing by encouraging the patient to use Breathing Control, the main technique used in between the more active stages of the as ACBT.
Allow your breathing to settle with breathing control and then repeat the cycle until your chest feels clear.
Small long huffs move sputum from low down into chest whereas big short huffs moves sputum from higher up into chest, so use this huff when it feels ready to come out, but not before; huffs work via dynamic compression.

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PLEURAL EFFUSION

Pleural effusion :

A pleural effusion is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. This excess fluid can impair breathing by limiting the expansion of the lungs. Various kinds of pleural effusion, depending on the nature of the fluid and what caused its entry into the pleural space, are hydrothorax (serous fluid), hemothorax (blood), urinothorax (urine), chylothorax (chyle), or pyothorax (pus).  A pleural effusion is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. This excess fluid can impair breathing by limiting the expansion of the lungs. Various kinds of pleural effusion, depending on the nature of the fluid and what caused its entry into the pleural space, are hydrothorax (serous fluid), hemothorax (blood), urinothorax (urine), chylothorax (chyle), or pyothorax (pus). A pneumothorax is the accumulation of air in the pleural space, and is commonly called a "collapsed lung".is the accumulation of air in the pleural space, and is commonly called a "collapsed lung".

 PLEURAL EFFUSIN DEVELOP-
The pleura creates too much fluid when it’s irritated, inflamed, or infected. This fluid accumulates in the chest cavity outside the lung, causing what’s known as a pleural effusion.

Certain types of cancer can cause pleural effusions, lung cancer in men and breast cancer in women being the most common.

OTHER CAUSES OF PLEURAL EFFUSION INCLUDE-

Congestive heart failure (the most common cause overall)
Cirrhosis or poor liver function
Pulmonary embolism, which is caused by a blood clot and is a blockage in the lung arteries
Complications from open-heart surgery
Pneumonia
Severe kidney disease
Autoimmune diseases, such as lupus and rheumatoid arthritis

TYPES OF PLEURAL EFFUSION-
There are several types of pleural effusions, each with different causes and treatment options. The first classification of pleural effusions is either transudative or exudative.

TRANSUDATIVE PLEURAL EFFUSION-
This type is caused by fluid leaking into the pleural space as a result of either a low blood protein count or increased pressure in the blood vessels. Its most common cause is congestive heart failure.Transudates describes fluids that have migrated from other parts of the body usually due to unusual hydrostatic or oncotic pressures. They may even result from misguided catheters or nasogastric feeding tubes. Some medical conditions that can lead to transudates include;

Atelectasis
Cirrhosis
Congestive Heart Failure
Peritoneal dialysis

EXUDATIVE PLEULRAL EFFUSION-
Exudates describe fluids that have been produced by a process of inflammation. Not only will excess fluids be drawn to the area, tissue permeability will be altered and lymphatic drainage is impaired. Some conditions that can lead to exudates building up in the pleural space include;

Blocked lymph or blood vessels
Inflammation
Tumors
Lung injury
Common conditions that could result in this type of pleural infusion include pulmonary embolisms, pneumonia, and fungal infections.

COMPLICATED AND UNCOMPLICATED PLEURAL EFFUSION-
There are also complicated and uncomplicated pleural effusions. Uncomplicated pleural effusions contain fluid without signs of infection or inflammation. They’re much less likely to cause permanent lung problems.

Complicated pleural effusions, however, contain fluid with significant infection or inflammation. They require prompt treatment that frequently includes chest drainage.


SYMPTOMS AND SIGNS OF PLEURAL EFFUSION-

Sharp chest pain that is worse with deep breaths due to inflamed pleural surfaces rubbing together.
Laboured breathing due to the impairment of lung volume and mechanical lung movement.
Dry coughing from lung irritation.
Diminished chest expansion on the side of the effusion with deep breaths.
With a stethoscope breath sounds may sound louder due to resonance with the fluid, you may also hear the rubbing of the pleural layers.
The variety and severity of symptoms experienced will vary with the size of each pleural effusion. Many smaller effusions will have no obvious outward signs and may only be detected whilst investigating other conditions. These may quickly resolve when the underlying condition is addressed.
Some people show no symptoms of pleural effusion. These people usually find out they have the condition through chest X-rays or physical examinations done for another reason.

COMMON SYMPTOMS OF PLEURAL EFFUSION-

Chest pain
Dry cough
Fever
Difficulty breathing when lying down
Shortness of breath
Difficulty taking deep breaths
Persistent hiccups
Difficulty with physical activity

DIAGNOSIS-
Your doctor will perform a physical examination and listen to your lungs with a stethoscope. They may also order a chest X-ray to help diagnose pleural effusion. Other possible tests include:
 
CT scan
Chest ultrasound
Pleural fluid analysis
Pronchoscopy
Pleural biopsy

In a pleural fluid analysis,  doctor will remove fluid from the pleural membrane area by inserting a needle into the chest cavity and suctioning the fluid into a syringe. The procedure is called a thoracentesis. This also works as a common procedure to drain the excess fluid from the chest cavity. The fluid will then be tested to determine the cause.

Doctor may also choose to perform a pleural biopsy, which involves taking a sample of tissue from the pleura. This can be done by inserting a small needle from outside the chest wall into the chest cavity.

If they discover  a pleural effusion, but they’re unable to diagnose which type, your doctor may schedule a thoracoscopy. This is a surgical procedure that lets the doctor see inside the chest cavity using a fiber-optic camera.

For this procedure, doctor will make a few small incisions in the chest area while you’re under general anesthesia. Then they’ll insert the camera through one incision and the surgical tool through the other to extract a small amount of fluid or tissue for analysis.


TREATMENT OF PLEURAL EFFUSION-
The underlying cause of the condition and the severity of the effusion will determine treatment.

DRAINING FLUID-
Generally, treatment involves draining the fluid from the chest cavity, either with a needle or a small tube inserted into the chest.


You’ll receive a local anesthetic before this procedure, which will make the treatment more comfortable. You may feel some pain or discomfort at the incision site after the anesthetic wears off.

You may need this treatment more than once if fluid builds up again.

Other treatments may be necessary to manage fluid buildup if cancer is the cause of the pleural effusion.

PLEURODESIS-
Pleurodesis is a treatment that creates mild inflammation between the lung and chest cavity pleura. After drawing the excess fluid out of the chest cavity, a doctor injects a drug into the area. The drug is often a talc mixture. This medication causes the two layers of the pleura to stick together, which prevents the future buildup of fluid between them.

SURGERY-
In more serious cases, a doctor surgically inserts a shunt, or small tube, into the chest cavity. This helps redirect the fluid from the chest into the abdomen, where it can be more easily removed by the body. This may be an option for those who don’t respond to other treatments. PLEURECTOMY, in which part of the pleural lining is surgically removed, may also be an option in certain cases.


RISK OF PLEURAL EFFUSIONS-
Treatment for some cases of pleural effusion may be managed with medication and other supportive care. Most people recover within a few days or weeks. Minor complications from more invasive treatments can include slight pain and discomfort, which often go away with time. Some cases of pleural effusion can have more serious complications, depending on the severity of the condition, cause, and treatment used.

SERIOUS COMPLICATIONS CAN INCLUDES-

Pulmonary edema or fluid in the lungs, which can result from draining fluid too quickly during thoracentesis
Partial collapsed lung
Infection or bleeding

PLEURAL EFFUSION AND CANCER-
Pleural effusions can be the result of cancer cells spreading to the pleura. They can also be the result of cancer cells blocking the flow of normal fluid within the pleura. Fluid may also build up as a result of certain cancer treatments, such as radiation therapy or chemotherapy.

Certain cancers are more likely to cause pleural effusions than others, including:

Lung cancer
Breast cancer
Ovarian cancer
Leukemia
Melanoma
Cervical cancer
Uterine cancer

The principle treatment approach with pleural effusion is to treat the underlying cause, which is usually more serious and will prevent further effusion. If the cause is not yet known and the fluid retention severe then a doctor can arrange to have it drained.

PHYSIOTHERAPY  MANAGEMENT-:

Treatment of your underlying condition and fluid drainage through a chest tube will be your primary sources of treatment for pleural effusion. Physiotherapy has an important role in stabilising and controlling your breathing, aiding in chest fluid drainage and clearing chest secretions. People whom receive respiratory physiotherapy as part of their treatment will tend to achieve a quicker recovery with fewer complications. Some of treatment techniques physiotherapy provides include;

Secretion clearance:
Effective / productive coughing techniques.
Postural drainage in sitting and lying.
Manual assistance, including percussion, vibrations and shaking.

Breathing technique retraining
:
Controlling respiratory rate
Diaphragmatic breathing
Controlling / reducing breath volume
Relaxation breathing exercises

Education and Advice:


1- Treatment of the primary cause.
2- Build up the body resistance by proper diet.
3- Aspiration of the excess pleural fluid to reduce dyspnea.
4- Physical therapy treatment:
• Positioning: on the normal side to improve ventilation/ perfusion ratio, also it
helps the movement on the affected side and subsequently helps the
drainage.
• Breathing exercises: diaphragmatic and localized breathing exercises.
• Postural exercises: to maintain good posture and avoid chest wall unilateral
contracture.
• Aerobic exercises: as walking and up and down stairs to maintain physical
endurance and fitness.

List of Congenital Deformities :

Congenital Deformities :

Congenital Deformities Are From Born Deformities But Some Some Unusual Deformities Seen in Some Child, Here in this Video Rare Congenital Deformities.