Cystic Fibrosis
Cystic fibrosis (CF) ...
... is an ‘autosomal recessive’ genetic disorder, meaning that both parents must carry and pass on the recessive defective CF gene. The defective gene normally codes for the production of the CFTR protein – cystic fibrosis transmembrane conductance regulator. This protein is normally found in the secreting epithelial cells of the lungs, pancreas, liver and sweat glands. The protein acts as a chloride ion channel, expelling chloride ions from the cell into the secretions. This lowers the water potential of the secretions and causes water to enter by osmosis. Without this protein the secretions become thick and sticky.
The lungs:
Mucus plays a huge role in the lungs. The mucus normally traps any bacteria in the lungs and the cilia move the mucus out of the lungs, removing the bacteria. In CF, the mucus is too sticky and thick to be removed by the cilia, so the bacteria remain in the lungs and can cause an infection. This is why sufferers of CF are prone to lung infections. CF sufferers are also prone to chronic coughing and coughing up green sputum which is viscous and pus filled. The pus is present as it is an immune response to any bacteria in the mucus.
The pancreas...
...is very important in digestion, producing digestive enzymes and insulin. The thick sticky mucus in CF blocks the ducts in the pancreas; this means that the digestive enzymes don’t reach the intestines resulting in the loss of ability to digest nutrients properly. This can lead to malnutrition, weight loss and poor growth. Because the mucus also blocks the ducts that carry insulin, this can cause diabetes.
Types;
There are over 1,400 identified mutations of the CFTR gene; any mutation, no matter how slight or severe causes CF of varying degrees. Obviously, the more severe the mutation the more severe CF will be.
Treatments ;
There is no cure for CF, but the condition is treated to improve quality and length of life. Treatments include:
• Antibiotics – to combat the frequent lung infections,
• Bronchodilators and Steroids – to reduce lung inflammation and open up airways,
• Insulin and pancreatic enzymes – to aid digestion and treat diabetes
• Diet – a high energy, high protein diet along with vitamin supplements to prevent malnutrition
Wednesday, 30 March 2011
Disability living allowance DLA
Help available for those unable to work - Disability living allowance DLA
Who is eligible?
“You may be eligible for Disability Living Allowance if you are under 65 and have walking difficulties or need help with your personal care. You must have had these needs for three months and expect to need this help or have these difficulties for at least another six months.” Direct Gov
You are able to apply for DLA if you have care needs, mobility needs or if you are terminally ill.
Some people will need to undergo a medical examination to assess their condition
How much is given?
Depending on your individual circumstance you could be given anything between £18.95 and £71.40 per week.
Help available for families with sick children
Depending on how serious the illness is you can claim DLA for the child. Different charities for different illnesses are able to provide information on what help is available and also how to manage the condition.
Missing school due to illness
Pupils are not expected to attend to school when they are not well enough to do so whether as a result of short term, long term or recurring illnesses. But they should not be kept away from school when they are well enough to go to lessons or when they have minor ailments which do not prevent them from taking part in their education. A useful rule of thumb is whether the ailment would keep parents and teachers away from work.
Schools should have an established system in place to enable parents/carers to report their child’s absence because of illness. It is reasonable for the school to ask the nature of the illness, ask the parent the expected length of absence and make arrangements for the parent to call the school again if the situation changes.
Absences should be authorised and on return, a note should be sent in notifying whoever is in charge why an absence occurred.
Help for those trying to quit smoking
Smoking cessation: People are put on a program to help them quit smoking and are helped along by a smoking cessation officer who is on hand to provide support
No smoking day: an initiative to help people quit smoking. Leaflets and free things such as nicotine patches are handed out.
People can buy things like nicotine patches or nicotine gum to help the stop smoking.
Who is eligible?
“You may be eligible for Disability Living Allowance if you are under 65 and have walking difficulties or need help with your personal care. You must have had these needs for three months and expect to need this help or have these difficulties for at least another six months.” Direct Gov
You are able to apply for DLA if you have care needs, mobility needs or if you are terminally ill.
Some people will need to undergo a medical examination to assess their condition
How much is given?
Depending on your individual circumstance you could be given anything between £18.95 and £71.40 per week.
Help available for families with sick children
Depending on how serious the illness is you can claim DLA for the child. Different charities for different illnesses are able to provide information on what help is available and also how to manage the condition.
Missing school due to illness
Pupils are not expected to attend to school when they are not well enough to do so whether as a result of short term, long term or recurring illnesses. But they should not be kept away from school when they are well enough to go to lessons or when they have minor ailments which do not prevent them from taking part in their education. A useful rule of thumb is whether the ailment would keep parents and teachers away from work.
Schools should have an established system in place to enable parents/carers to report their child’s absence because of illness. It is reasonable for the school to ask the nature of the illness, ask the parent the expected length of absence and make arrangements for the parent to call the school again if the situation changes.
Absences should be authorised and on return, a note should be sent in notifying whoever is in charge why an absence occurred.
Help for those trying to quit smoking
Smoking cessation: People are put on a program to help them quit smoking and are helped along by a smoking cessation officer who is on hand to provide support
No smoking day: an initiative to help people quit smoking. Leaflets and free things such as nicotine patches are handed out.
People can buy things like nicotine patches or nicotine gum to help the stop smoking.
respiratory system
Nose – Filters, moistens and warms inspired air and in doing so assists
the normal process of respiration. [1]
Pharynx – A muscular tube lined with mucous
membrane, that extends from the beginning of
the oesophagus to up to the base of the scull.
Acts as an air passage. [2]
Epiglottis – Thin flap of cartilage covered in mucous membrane, situated immediately behind the root of the tongue. It covers the entrance to the larynx during swallowing. [3]
Larynx - The organ responsible for vocal sounds. Also acts as a passageway for air from the Pharynx to the lungs. [4]
Bronchus – Any of the air passages beyond the trachea that has cartilage and mucous glands in it’s wall. [6]
Alveoli – There are approximately 300 million alveoli in each lung.
There are two types:
• Type I pneumocytes which have thin layer of cytoplasm.
• Type II pneumocytes are slightly more numerous but cover less of the epithelial lining. [7]
Diaphragm – A thin dome-shaped muscle that separates the thoracic and abdominal cavities. Attached to the lower ribs at each side along with the breast bone and the backbone. [8]
Pleura – A layer of connective tissue covered with squamous epithelium that secretes small amounts of fluid to provide lubrication so that the visceral and parietal surfaces can slide painlessly over each other during breathing. [9]
the normal process of respiration. [1]
Pharynx – A muscular tube lined with mucous
membrane, that extends from the beginning of
the oesophagus to up to the base of the scull.
Acts as an air passage. [2]
Epiglottis – Thin flap of cartilage covered in mucous membrane, situated immediately behind the root of the tongue. It covers the entrance to the larynx during swallowing. [3]
Larynx - The organ responsible for vocal sounds. Also acts as a passageway for air from the Pharynx to the lungs. [4]
Bronchus – Any of the air passages beyond the trachea that has cartilage and mucous glands in it’s wall. [6]
Alveoli – There are approximately 300 million alveoli in each lung.
There are two types:
• Type I pneumocytes which have thin layer of cytoplasm.
• Type II pneumocytes are slightly more numerous but cover less of the epithelial lining. [7]
Diaphragm – A thin dome-shaped muscle that separates the thoracic and abdominal cavities. Attached to the lower ribs at each side along with the breast bone and the backbone. [8]
Pleura – A layer of connective tissue covered with squamous epithelium that secretes small amounts of fluid to provide lubrication so that the visceral and parietal surfaces can slide painlessly over each other during breathing. [9]
Digestive system
The digestive system
Mouth
1. Mastication (chewing of food) occurs where food is broken up into pieces that are more easily digestible.
2. Salivary glands secrete saliva into the mouth which provides lubrication and acts as a buffer.
Oesophagus
1. Receives food from the mouth and passes it into the stomach by a series of contractions called peristalsis.
Stomach
1. The stomach has many functions:
a. It is used a storage tank due to the folds in the stomach, it can expand and store up to 2-4 litres of food. This is important as we eat food faster then we can digest it into nutrients.
b. The stomach can churn and mix water and the gastric juice within it to produce a think mixture called chime.
c. The stomach can break food down food physically and chemically. The hydrochloric acid which is secreted by the parietal cells unfolds proteins that are present in the stomach and also kill any bacteria that we may have digested along with our food. Chief cells secrete pepsin in an inactive form called pepsinogen. HCl converts pepsinogen into pepsin and protein digestion in the stomach begins.
Small intestine
1. Mechanical digestion occurs by segmentation which mixes the enzymes (from the pancreas and small intestine) and chyme together.
2. Chemical digestion occurs by enzymes which are present and theybreak down all four food groups (polysaccharides, fats, proteins and nucleic acids) into their component molecules. The bile from the liver emulsifies any fats present into smaller fat globules
3. The small intestine is the primary location for absorption
4. Peristalsis moves the chyme along the small intestine.
Pancreas
1. Secretes pancreatic juices which contains enzymes which are secreted into the duodenum and break down all four food groups.As the fluid secreted is alkaline, it neutralizes any acidity that may be present in the chyme so that the enzymes can function.
Liver and Gall bladder
1. Bile is produced in the liver which consists of bile salt, bile pigments (mostly bilirubin), cholesterol, phospholipids and electrolytes. The excess bile is stored in the gall bladder is released in response to chyme being present.
Large intestine
1. Contractions of the muscle walls form segmentations which mix and move the contents further along.
2. Absorption of water happens
3. Mucus is produced by goblet cells for lubrication.
4. Bacteria present digest the remiaing nutrients and produce vitamin K and B which are then absorbed.
5. The remaining undigestible materials are removed from the body as feces.
Mouth
1. Mastication (chewing of food) occurs where food is broken up into pieces that are more easily digestible.
2. Salivary glands secrete saliva into the mouth which provides lubrication and acts as a buffer.
Oesophagus
1. Receives food from the mouth and passes it into the stomach by a series of contractions called peristalsis.
Stomach
1. The stomach has many functions:
a. It is used a storage tank due to the folds in the stomach, it can expand and store up to 2-4 litres of food. This is important as we eat food faster then we can digest it into nutrients.
b. The stomach can churn and mix water and the gastric juice within it to produce a think mixture called chime.
c. The stomach can break food down food physically and chemically. The hydrochloric acid which is secreted by the parietal cells unfolds proteins that are present in the stomach and also kill any bacteria that we may have digested along with our food. Chief cells secrete pepsin in an inactive form called pepsinogen. HCl converts pepsinogen into pepsin and protein digestion in the stomach begins.
Small intestine
1. Mechanical digestion occurs by segmentation which mixes the enzymes (from the pancreas and small intestine) and chyme together.
2. Chemical digestion occurs by enzymes which are present and theybreak down all four food groups (polysaccharides, fats, proteins and nucleic acids) into their component molecules. The bile from the liver emulsifies any fats present into smaller fat globules
3. The small intestine is the primary location for absorption
4. Peristalsis moves the chyme along the small intestine.
Pancreas
1. Secretes pancreatic juices which contains enzymes which are secreted into the duodenum and break down all four food groups.As the fluid secreted is alkaline, it neutralizes any acidity that may be present in the chyme so that the enzymes can function.
Liver and Gall bladder
1. Bile is produced in the liver which consists of bile salt, bile pigments (mostly bilirubin), cholesterol, phospholipids and electrolytes. The excess bile is stored in the gall bladder is released in response to chyme being present.
Large intestine
1. Contractions of the muscle walls form segmentations which mix and move the contents further along.
2. Absorption of water happens
3. Mucus is produced by goblet cells for lubrication.
4. Bacteria present digest the remiaing nutrients and produce vitamin K and B which are then absorbed.
5. The remaining undigestible materials are removed from the body as feces.
Vaccines - hpv
Overview of vaccines:-
• A vaccine contains either a dead or a weakened form of a disease causing microbe (antigen) or its toxins. The body’s immune system then recognises the agent as foreign and it’s destroyed. The immune system is able to remember the agent and so if the body encounters it again the immune system is able to react and destroy it more quickly in the future.
• The most common way of administering a vaccine is via an injection, however some can be taken orally and others via a nasal spray.
Artificial Active Immunisation
A non pathogenic form of a pathogen or part of a pathogen is injected into the recipient. The body thinks that it’s a real pathogen and its B cells produce antibodies to destroy the pathogen. These antibodies are stored and so when the body comes into contact with the “real” pathogen in the future the immune response is quicker.
Artificial Passive Immunisation
This is the type of immunisation where antibodies are injected directly into the recipient. Immunity only lasts for a short while and there is a risk of hypersensitivity reactions especially if the antibodies are from a non-human source. This type of immunisation provides immediate protection, however not memory and so the patient is still at risk if infected by same pathogen in the future.
HPV specific
• Human Papilloma Virus also referred to as “wart virus” because can sometimes lead to genital warts.
• Virus that affects the skin and moist membranes lining the body e. g. Cervix, anus, mouth and throat.
• The most common high risk types of Hpv are Hpv-16 and Hpv-18 which cause around 70% of cervical cancers.
• It’s the most common sexually transmitted infection.
• There are two different types of the HPV vaccine available. These are called Gardasil and Cervarix. The vaccine used in the UK national vaccination programme is Cervarix.
• Cervarix protects against Hpv-16 and Hpv-18, but not other types of Hpv viruses. Gardasil also protects against types 6 and 11 which can cause genital warts, however this is only available privately.
• Cervarix contains an inactive form of Hpv-16 and Hpv-18 and therefore falls under the artificial active immunity category.
• The vaccine is administered via 3 injections into the arm. The second injection is given 1-2 months after the first and the third injection given approximately 6 months after the first one.
• The Hpv virus is given to girls aged 12-13 as it’s thought to be more effective before girls become sexually active.
• Side effects of the vaccine can include headache, fever, sickness dizziness, diarrhoea, muscle pain and in some very rare cases an allergic reaction leading to breathing problems and sometimes collapsing.
• The HPV virus has a long incubation period that typically ranges from three weeks to eight months.
• A vaccine contains either a dead or a weakened form of a disease causing microbe (antigen) or its toxins. The body’s immune system then recognises the agent as foreign and it’s destroyed. The immune system is able to remember the agent and so if the body encounters it again the immune system is able to react and destroy it more quickly in the future.
• The most common way of administering a vaccine is via an injection, however some can be taken orally and others via a nasal spray.
Artificial Active Immunisation
A non pathogenic form of a pathogen or part of a pathogen is injected into the recipient. The body thinks that it’s a real pathogen and its B cells produce antibodies to destroy the pathogen. These antibodies are stored and so when the body comes into contact with the “real” pathogen in the future the immune response is quicker.
Artificial Passive Immunisation
This is the type of immunisation where antibodies are injected directly into the recipient. Immunity only lasts for a short while and there is a risk of hypersensitivity reactions especially if the antibodies are from a non-human source. This type of immunisation provides immediate protection, however not memory and so the patient is still at risk if infected by same pathogen in the future.
HPV specific
• Human Papilloma Virus also referred to as “wart virus” because can sometimes lead to genital warts.
• Virus that affects the skin and moist membranes lining the body e. g. Cervix, anus, mouth and throat.
• The most common high risk types of Hpv are Hpv-16 and Hpv-18 which cause around 70% of cervical cancers.
• It’s the most common sexually transmitted infection.
• There are two different types of the HPV vaccine available. These are called Gardasil and Cervarix. The vaccine used in the UK national vaccination programme is Cervarix.
• Cervarix protects against Hpv-16 and Hpv-18, but not other types of Hpv viruses. Gardasil also protects against types 6 and 11 which can cause genital warts, however this is only available privately.
• Cervarix contains an inactive form of Hpv-16 and Hpv-18 and therefore falls under the artificial active immunity category.
• The vaccine is administered via 3 injections into the arm. The second injection is given 1-2 months after the first and the third injection given approximately 6 months after the first one.
• The Hpv virus is given to girls aged 12-13 as it’s thought to be more effective before girls become sexually active.
• Side effects of the vaccine can include headache, fever, sickness dizziness, diarrhoea, muscle pain and in some very rare cases an allergic reaction leading to breathing problems and sometimes collapsing.
• The HPV virus has a long incubation period that typically ranges from three weeks to eight months.
Asthma
Asthma Revision
Asthma is a chronic disease that affects the airways of your lungs, this causes them to constrict in response to certain allergens or triggers, severe contractions are known as an asthma attacks and can restrict oxygen to vital organs, this requires medical intervention such as the use of various inhalers.
Symptoms of asthma may be coughing wheezing, heavy and interrupted breathing, and in severe cases cyanosis can occur (skin/extremities turn blue due to lack of oxygen)
-in our case involving the child infected with asthma and cystic fibrosis, the severity tightening of the airways is going to increase due to the build-up of mucus characteristic of CF
---
Those with asthma are predisposed towards chest infections due to the build-up of mucus in the lungs which traps bacteria isn’t being shifted as efficiently as it should be
There are two primary types of chest infection, acute/chronic bronchitis and pneumonia. Acute bronchitis is self-limiting and usually clears up within a few weeks, though if the condition is quite severe it can cause permanent damage to the lungs. Bronchitis is a condition were by the bronchi produces too much mucus and they become swollen, the coughing is the bodies attempt to remove the mucus. Complications can arise when the patient is already infected with asthma, which can cause the airways to become swollen more regularly. The chronic variant occurs when bronchitis occurs for 3-4 months
Pneumonia is a condition whereby the alveoli in the body become inflamed and as such cannot efficiently diffuse oxygen into the blood stream; it is caused by a virus bacteria or fungus. Again this condition is worsened by bronchiole inflammation which further cuts down the bodies supply of oxygen1
---
Seasons of asthma and chest infection
-Cold air thickens the mucus in the lungs and nose making it harder to remove allergens or triggers
- Histamine is also produced by the lungs in response to cold air which causes wheezing
-poor air quality (histamine causes nearby vessels to become permeable causes blood fluids to enter the site and cause swelling)5
-prevalence of flu, and cold viruses
-Even the psycho-social aspect of depression can lead to poor asthma management and more attacks 4
Interestingly vitamin D deficiency has also been proposed to causes asthma by recent studies, this makes sense as asthma cases increase in seasons that lack sunlight (a major source of vitamin C)2+3
Inhalers6
Relievers – use to relieve an occurring asthma attack, contains drugs such as salbutamol or terbutaline. These drugs cause the bronchioles to dilate and allow air into the lung compartments. They are blue in colour.
Preventers – taken every day, or at a prescribed timetable after consultation with your GP, these inhalers contain steroids such as Beclometasone, reducing the inflammation of the airways. Their purpose is to prevent asthma attacks throughout the day. Though it may take many weeks for them to become fully effective.
Long acting – Often taken in combination with the preventer if fails to control the symptoms. They work similarly to relievers, but over a 12 hour period and contain the drug salmeterol, these are often green in colour.
---
Spacers
Pressurized reservoir ensures than none of the drug is lost, ensures the patient receives full medication without needing good coordination.
Nebulisers
Used In hospital in emergency cases allows a high quantity of drug to be administered at once.
---
Triggers
Triggers are essentially anything that can cause an asthma attack; this can be pollen, smoke, dust mites, Household pet and even negative emotions stress causing higher blood pressure and increased heart rate which can leads to heavy breathing. This can trigger the airways to contract. In addition to this allergens of all kinds can trigger an asthmatic attack.
(IgE which is bound to mast cells binds to a complementary antigen, if the antigen binds to the IgE then histamine is released which can cause an asthmatic attack)5
In our case the dog household pet could be a potential trigger- it can’t be factored out
----
Green sputum and the 3 day delay…
Green mucus is clearly indicative of infection (white blood cells mixed with bacteria) which gives it the colour, this is also characteristic of respiratory infections7. Though this may not be a direct response form the HPV vaccine there may be other indirect causes… HPV symptoms at their most severe can be vomiting fever muscle pain and vomiting, nothing particularly linked to the respiratory system. However like all vaccines there is the chance for an allergic reaction or a compromised immune system, this could explain the same day coughing (Though further tests may need to be performed to establish if there really is a causal linkage)8
Asthma is a chronic disease that affects the airways of your lungs, this causes them to constrict in response to certain allergens or triggers, severe contractions are known as an asthma attacks and can restrict oxygen to vital organs, this requires medical intervention such as the use of various inhalers.
Symptoms of asthma may be coughing wheezing, heavy and interrupted breathing, and in severe cases cyanosis can occur (skin/extremities turn blue due to lack of oxygen)
-in our case involving the child infected with asthma and cystic fibrosis, the severity tightening of the airways is going to increase due to the build-up of mucus characteristic of CF
---
Those with asthma are predisposed towards chest infections due to the build-up of mucus in the lungs which traps bacteria isn’t being shifted as efficiently as it should be
There are two primary types of chest infection, acute/chronic bronchitis and pneumonia. Acute bronchitis is self-limiting and usually clears up within a few weeks, though if the condition is quite severe it can cause permanent damage to the lungs. Bronchitis is a condition were by the bronchi produces too much mucus and they become swollen, the coughing is the bodies attempt to remove the mucus. Complications can arise when the patient is already infected with asthma, which can cause the airways to become swollen more regularly. The chronic variant occurs when bronchitis occurs for 3-4 months
Pneumonia is a condition whereby the alveoli in the body become inflamed and as such cannot efficiently diffuse oxygen into the blood stream; it is caused by a virus bacteria or fungus. Again this condition is worsened by bronchiole inflammation which further cuts down the bodies supply of oxygen1
---
Seasons of asthma and chest infection
-Cold air thickens the mucus in the lungs and nose making it harder to remove allergens or triggers
- Histamine is also produced by the lungs in response to cold air which causes wheezing
-poor air quality (histamine causes nearby vessels to become permeable causes blood fluids to enter the site and cause swelling)5
-prevalence of flu, and cold viruses
-Even the psycho-social aspect of depression can lead to poor asthma management and more attacks 4
Interestingly vitamin D deficiency has also been proposed to causes asthma by recent studies, this makes sense as asthma cases increase in seasons that lack sunlight (a major source of vitamin C)2+3
Inhalers6
Relievers – use to relieve an occurring asthma attack, contains drugs such as salbutamol or terbutaline. These drugs cause the bronchioles to dilate and allow air into the lung compartments. They are blue in colour.
Preventers – taken every day, or at a prescribed timetable after consultation with your GP, these inhalers contain steroids such as Beclometasone, reducing the inflammation of the airways. Their purpose is to prevent asthma attacks throughout the day. Though it may take many weeks for them to become fully effective.
Long acting – Often taken in combination with the preventer if fails to control the symptoms. They work similarly to relievers, but over a 12 hour period and contain the drug salmeterol, these are often green in colour.
---
Spacers
Pressurized reservoir ensures than none of the drug is lost, ensures the patient receives full medication without needing good coordination.
Nebulisers
Used In hospital in emergency cases allows a high quantity of drug to be administered at once.
---
Triggers
Triggers are essentially anything that can cause an asthma attack; this can be pollen, smoke, dust mites, Household pet and even negative emotions stress causing higher blood pressure and increased heart rate which can leads to heavy breathing. This can trigger the airways to contract. In addition to this allergens of all kinds can trigger an asthmatic attack.
(IgE which is bound to mast cells binds to a complementary antigen, if the antigen binds to the IgE then histamine is released which can cause an asthmatic attack)5
In our case the dog household pet could be a potential trigger- it can’t be factored out
----
Green sputum and the 3 day delay…
Green mucus is clearly indicative of infection (white blood cells mixed with bacteria) which gives it the colour, this is also characteristic of respiratory infections7. Though this may not be a direct response form the HPV vaccine there may be other indirect causes… HPV symptoms at their most severe can be vomiting fever muscle pain and vomiting, nothing particularly linked to the respiratory system. However like all vaccines there is the chance for an allergic reaction or a compromised immune system, this could explain the same day coughing (Though further tests may need to be performed to establish if there really is a causal linkage)8
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