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What is Cystic Fibrosis
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Meet James a boy with CF. This is the medication he takes each day to stay well.

 

What is Cystic Fibrosis?

 

Cystic fibrosis (CF), also called Mucoviscidosis, is a hereditary disease that affects the entire body, causing progressive disability and early death. Formerly known as cystic fibrosis of the pancreas, this entity has increasingly been termed simply 'cystic fibrosis.’

 

Difficulty breathing and insufficient enzyme production in the pancreas are the most common symptoms. Thick mucous production, as well as a low immune system, results in frequent lung infections, which are treated, though not always cured, by oral and intravenous antibiotics, inhalation of mucolytics and hypertonic saline solution, chest physiotherapy and daily exercise. It is vital for CF patients who are pancreatic deficient to use sufficient pancreatic enzymes with every meal and to consume high caloric nutritional foods. A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and potential infertility (mostly in males) result from the effects of CF on other parts of the body.

 

1 in 2500 children is born with cystic fibrosis, and it is one of the most common fatal inherited diseases. It is most prevalent among Europeans and Ashkenazi Jews; one in twenty-two people of European descent carry one gene for CF, making it the most common recessive genetic disease among them. Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing or in early childhood by a sweat test. There is no cure for CF, and most individuals with cystic fibrosis die young — many in their 20s and 30s from lung failure although with many new treatments being introduced the life expectancy for people with CF are increasing. Ultimately, lung transplantation is often necessary as CF worsens.

 

While the earliest clear medical descriptions date from the 1930’s, CF obviously existed prior to this date but was un-recognized. Its clinical characteristics individually resemble those of other diseases such as pneumonia, bronchiectasis, failure to thrive, and celiac disease. Indeed, where these conditions are prevalent CF may still lie un-recognized. Moreover, if clinicians believe that CF is absent from their population they will not consider it in a differential diagnosis. A better awareness of and the increasing availability of diagnostic tests- the sweat test and/or DNA tests – frequently leads to the identification of a higher number of affected individuals.


In the last two decades, CF has been increasingly diagnosed in Latin America, the Middle East, and populations derived from the Indian subcontinent that have emigrated to Western Europe, thus implying the presence of CF in significant numbers among the citizens of India and Pakistan who have remained in their homeland.

 

CF is a multi-organ disease and children with CF need multidisciplinary team care in CF centres regularly.  This multidisciplinary team should consist of a CF Nurse, Paediatric Pulmonologists, Pulmonologists, Gastroenterologist/Nutritionist, Nurse, Physiotherapist, Microbiologist and Psychologist/Social worker who have been trained in the specific needs of patient care. Daily home care that requires both parent and patient participation is also needed to ensure the patients experience a better quality of life and life expectancy.

 
 
 

   
A long road
many years ago it seems now i left this place when it was once starting to get off the ground. many a misadventure later i am returned, and i cant unfortunately say the better for it.
 
 
   
 

Notes on HIV and AIDS:Live happy

Notes on HIV and AIDS

What are H IV and Al DS?

Acquired immunodeficiency syndrome - is caused by a virus, the human immunodeficiency virus (HIV).  Viruses are very small living organisms that cause many different diseases in humans, animals and even plants. Viruses are so small that even if thousands of them are put together they cannot be seen with the naked eye.  In fact, viruses are among the smallest and simplest living things.  They cannot reproduce on their own but depend on the animal or human they infect to act as a host.  HIV reproduces in certain cells in human blood, called white blood. cells (WBCs).  These WBCs are a very important part of what is called the immune system.  The immune system, with its WBCS, defends the body from infections - it recognizes things that are foreign or dangerous to the body and begins to attack them.  It also starts to make specific substances called antibodies which are very active against the particular disease-causing organism that has infected the body.

When a person becomes infected with HIV, the virus begins to live and reproduce in the WBCS, multiplying until there are millions of viruses present.  The WBCs begin to make antibodies to HIV which are found in the blood about six weeks after infection.  Unfortunately, these antibodies cannot eliminate the virus completely from the body.  The virus gradually damages the WBCs so that they can no longer do their job of protecting the body from infections, which healthy people can normally fight off without any problem.  It is when. these infections occur that a person is said to have AIDS.  The bacteria, viruses and parasites present in the environment that cause these infections take the opportunity given by the weakened immune system to grow unhindered.  This is why many of the illnesses that people with AIDS get are called opportunistic infections.

For most of the time, people infected with HIV are healthy and usually unaware that there is anything wrong with them.  It is not unusual for the period of time between infection with HIV and becoming ill with AIDS to be eight or nine years, and sometimes as long as 15 years.  The length of time between infection and the appearance of AIDS can vary widely in different people.

HIV, the virus that causes AIDS, is passed (transmitted) between people by the ways shown in the box below.

Ways in which HIV is transmitted

·         through unprotected sexual intercourse with an infected person; that is, intercourse without a condom

·         from an infected mother to her unborn or newly-born child

·         through contact with infected blood, for example by:

Þ     the transfusion of infected blood

Þ     the sharing of skin-piercing instruments, such as injection needles, that are not sterile

HIV infection is most often passed by sexual intercourse.  It can be passed from either a man or a woman.  A person who is not infected with HIV can take steps to avoid infection just as a person who is infected with HIV can take Steps to prevent passing the HIV infection to someone else.

 

How can you avoid AIDS?

Abstinence (not having sexual relations at all) is the most sure way of preventing sexual transmission of HIV infection.

For many people, however, this may not be acceptable or realistic.  The use of condoms and other safer sexual practices are the only ways of decreasing the risk of becoming infected with HIV or transmitting HIV infection to a sexual partner.  Safer sexual practices are described in the box below.

What is "Safer sex" ?

Safer sex is any sexual practice that reduces the risk of passing (transmitting) HIV from one person to another.

The best protection is obtained by choosing sexual activities that do not allow semen, fluid from the vagina, or blood to enter the mouth, anus or vagina of the partner, or to touch the skin of the partner where there is an open cut or sore.

Safer sex practices include:

Þ     staying in a mutually faithful relationship where both partners are uninfected

Þ     masturbation, massage, rubbing, dry wising, and hugging using a condom for all types of sexual intercourse (anal, vaginal and oral) avoiding certain practices that increase the possibility of HIV transmission, for

Þ     example "dry" sex which may lead to breaks in the skin

Þ     avoiding sex when either partner has open sores or any sexually transmitted disease (STD)

Þ     oral sex - this may be an acceptable alternative for some people, but oral

Þ     sex should be avoided if there are sores present in the mouth or on the genitals

Couples should talk about sex and learn to please each other. This can allow for the negotiation of safer sex and make the intercourse more pleasurable for both and less likely to cause discomfort or minor

Condoms are best known as a means of preventing pregnancy.  However, they are also the most effective means of protection against the organisms that cause sexually transmitted diseases, including HW.

How do you use condoms to prevent pregnancy and HIV transmission?

Condoms are effective if they are used properly every time you have sexual intercourse.  Instructions on how to use condoms are given in the box below.

How to use a Condom

Þ     Be sure you have a condom before you need it

Þ     Each time you have sex put a new and unused condom on the penis before it enters the vagina or rectum.

Þ     Put the condom on only when the penis is erect

Þ     If you are not circumcised, pull the foreskin of the penis back before putting on the condom.

Þ     Do not pull the condom tightly against the tip of the penis but pinch the end when unrolling it this leaves a small empty space, to hold the semen.

Þ     Unroll the condom all the way to the base of the penis.

Þ     If the condom tears during sex, withdraw the penis immediately and put on a new condom.

Þ     After ejaculation, hold on to the bottom of the condom as you pull the penis out, so that the condom does not slip off, then take off the condom carefully without spilling semen.

Þ     Wrap the condom in paper (such as newspaper) until you can dispose of it in a toilet, a pit latrine or a closed garbage bag, or by burying or burning it

The following tips will help to prevent condoms breaking or leaking.

  • If lubricant is needed use a water-based one (like KY jelly, or glycerine).  Do not use a lubricant made with oil, like Vaseline, which can cause condoms to break more easily.
  • Store condoms in a cool, dark, dry place, if possible.  Heat, light, and humidity can damage condoms.
  • If you have a choice, choose pre-lubricated condoms that come in square wrappers and are packaged so that light does not reach them.
  • Open the wrapper carefully so that the condom does not tear.
  • Do not use condoms that are sticky, brittle, discoloured or damaged in any way.

 

What is an HIV test?

Shortly after infection with HIV, the body starts to respond by making antibodies against the virus.  This usually takes 8-12 weeks.  An HIV test can find out if these specific antibodies are present in the blood - it does not detect the virus itself.

What do the results mean?

o              A positive test result in a person over 15 months old means that:

   The person has antibodies against HIV, and is thus HIV-infected and can transmit the virus to others.

o              A positive test result in a child under 15 months old can mean either that:

   the child is infected with HIV, or

   the child is not infected with HIV, but has received antibodies against HIV from its mother, in the same way as many other antibodies are transferred during pregnancy.

Because of these two possibilities, it is impossible to tell if a baby is HIV-infected until it is at least 15 months old, when the antibodies from the mother are usually no longer present.

 

 

o        A negative test result can mean either that:

-     the person is not infected with HIV, or

-     the person is infected with HIV, but has not yet made antibodies against the virus.    (This is sometimes called the "window" period.)

 

o        The HIV test.

-     does not provide any information about a person's present state of health

-     does not determine if a person has HIV-related disease

-     cannot tell when or how a person became infected with HIV

-  does not provide any information about whether a person with HIV infection has    transmitted the virus to anyone else.

Because the results of an HIV test can have dramatic effects on families, relationships, employment and the individual's own psychological well-being, it is important that people be tested only with their consent, that they be counselled before and after testing, and that the results be kept confidential, that is, shared only with the individual, or others designated by the individual.

Ways in which HIV is not transmitted

Ordinary social contact:

o           physically close

-           in the same home

-           breathing the same air; coughs and sneezes

-           at work

-           on the bus

-           at the market

-           at school

-           playing together

o           touching

-           shaking hands

-           hugging

-           kissing on the cheeks, hands or forehead

Sharing:

-                      toilet seats

-                      towels

-                      washing water, bath water

-                      swimming pools

-                      eating and drinking utensils

-                      communion cups

-                      work tools

 

 

 

Being bitten by:

-                      mosquitoes

-                      bed bugs

-                      other insects

-                      any other animal

 

Otherwise, as people begin to see AIDS as a serious problem, they may panic and reject infected people.  They may isolate them and their families.  These reactions are deeply upsetting for people who are already facing the trauma of AIDS, and they are unhelpful in stopping the transmission of HIV.  If people are not encouraged to share the problems they have, then they cannot receive help from their fan-dlies and enjoy the time they have together.


Living Positively with AIDS

 

Responses to AIDS

Each of these feelings or reactions is part of a normal response to a situation of great stress.  A person n-dght move from one response to the next in a progression leading finally to acceptance of their situation, or more commonly their. feelings will keep changing. One day they might feel rejected and lonely - the next day hopeful and energetic. One day depressed, another day angry.  Let us look at each of these reactions separately and think about how these make someone feel, and how a person feeling like this might be helped.

SHOCK

No matter how much someone prepares, it is a shock to learn that one has HIV infection or AIDS.  A person might feel confused and not know what to do.  It is good for people to be with someone they trust at this time.

DENIAL

At first they might not be able to believe that they really have HIV or AIDS.  They might think, "The doctor must be wrong" or "It can't be true - I feel so strong".

Not wanting to believe is a strong force that people may use subconsciously to protect themselves from the threat posed by AIDS.  If you are trying to help such people, don't be angry or impatient with them if it seems that they are not facing facts.  Try to remember that as a health care worker you can help them to understand what having HIV or AIDS means and that this is the best way to help overcome denial.

ANGER

People might become very angry when they learn that they have HIV or AIDS. is a common feeling and can come when they blame themselves or the person they think gave them HIV.  Some may even blame God.

Anger is normal but it may not be helpful since it can focus on blaming others (being angry with them) or themselves (feeling guilty), rather than taking positive actions.  Talking to someone can help a person overcome feelings of anger and help them accept their situation.

If you are trying to help someone with HIV or AIDS, anger is a difficult reaction to cope with, especially when it is directed at you.  It is important for you to try to understand and not take the anger personally.  It is difficult, of course, to receive anger without responding.

BARGAINING

A person with AIDS might try to bargain, thinking, "God will cure me if I stop having sex" or "The ancestors will make me better if I slaughter a goat" or "I will be good and it (AIDS) will go away".  People with HIV or AIDS need to be helped to get through the feeling of bargaining.

 

 

 

 

FEAR

People with HIV or AIDS fear many things, for example:

·               Pain                               

·               Losing their job

·               Other people knowing that they are infected

·               Rejection

·               Leaving their children

·               Death.

These fears become less when they talk to someone who understands.  Someone with AIDS might also find that they are worried about things that they do not need to fear.  For example, they may find that when other people learn they have HIV, they show great love and kindness rather than the feared rejection.

LONELINESS

A person with AIDS n-dght often feel lonely.  This feeling may come and go for. a long time and depends on the support given by family and friends.  Anyone who has AIDS must be helped to remember that they are not alone; that they are surrounded by family, friends and a community that cares about them.  Many other people have HIV or AIDS.

Help families and communities to understand that people with HIV and AIDS need companionship.  Infected people can often find others with HIV and AIDS and provide companionship and support for one another.

SELF-CONSCIOUSNESS

When a person has HIV or AIDS they might think everyone is looking at them or talking about them.  This may make them want to hide.  Sometimes a person with AIDS may feel unworthy of friendship.

 

You can help them not to hide or feel discouraged by encouraging them to stay active in the community.  This can increase the acceptance of people with HIV AIDS by showing the world that people with HIV and AIDS are valuable members of society, just like everyone else.

Help them to think well of themselves and to be proud.  They are still important.

DEPRESSION

If a person finds out that they have HIV or AIDS they may feel there is no good reason for living.  They may feel useless, and want to stay at home, not eat, and not talk to anyone.

Depression can make someone weak both in mind and body.  It is important to try and help them overcome tl-ds depression and not give up.  Encourage them to put on nice clothes, visit friends, keep busy with things that matter, do something that helps others, and to think about their children and friends who still need them.

ACCEPTANCE

After some time, a person with HIV or AIDS will usually begin to accept their situation.  This will help the person to feel better.  Such a person will feel more peaceful in their mind, and will begin to think about the best ways to live.

 

They might think:

  • "What can I do to make the best of the rest of my life?"
  • 'What foods should I eat to help me stay healthy?"
  • "What plans should I make so that my children are provided for in the future?"
  • "Let me be grateful for every single day.  Let me appreciate my family and friends and show them how much I care for them."

HOPE

You can help someone with HIV or AIDS have hope about many things.  For example:

  • hope that they will live a long time
  • hope that their baby will be healthy
  • hope that each sickness will be treated as it comes
  • hope because they are loved and accepted for who they are
  • hope that scientists will find a cure
  • hope because of belief in a life after death.

It is important to have hope.  Hope lifts spirits and gives strength to face each situation. Hope can help each person to fight HIV and AIDS - to live positively and to live longer.

Remember, even if a person has hope today, it is possible to feel angry or depressed tomorrow. This is normal. Even people without HIV or AIDS go up and down emotionally every day.  The important thing is to try to instill the feelings of hope again and again.

Preventing HIV transmission in the home

As you learned from the first part of the story, HIV can be transmitted from one person to another. However, unlike some other infectious organisms, IRV is not easily transmitted except by unprotected sexual intercourse or close blood to-blood contact.  The virus dies quickly outside the human body.

Because AlDS is known to be fatal, people are concerned about whether it is safe to care for an infected person.  Those providing home care should be taught to follow these rules:

·         Wash your hands with soap and water after changing soiled bed sheets and  clothing, and after having contact with body fluids.

·         Keep wounds covered.  Both caregivers and people with AIDS should cover any open wounds they may have on their hand or other places likely to have contact with other people, their bedding or clothing.  Cover open wounds with a bandage or cloth. Use a piece of plastic or paper, gloves or a big leaf to handle soiled items.

·         Keep bedding and clothing dean. This will help keep sick people comfortable and prevent skin problems. If you follow the first two rules the risk of transmission through contact with soiled clothing or linens is extremely low. To clean clothing or sheets stained with blood, diarrhoea or other body fluids:

1.     Keep separate from other household laundry

2.     Holding an unstained part, rinse off any blood or diarrhoea with water

-  be particularly careful if there are large amounts of blood, such as after childbirth.

3.     Wash in soapy water, hang to dry and fold or iron as you would normally.

Any action you want to take beyond this, such as the use of bleach or boiling water, will also be effective but is not necessary.

Don't share sharp skin-piercing instruments. Don't share toothbrushes, razors, needles, or anything else that can cut or come into contact with blood.  If it is necessary to share these types of objects, boil them in water prior to use.

It is important to emphasize that HIV is not spread during normal social contact.  People should not worry about getting other sexually transmitted diseases (STDs), like syphilis or gonorrhea, from this type of contact with people, and they should have no greater worry regarding HIV/AIDS.  However, it is important to avoid other common infections that are spread by normal social contact, such as diarrhea and respiratory infections.

Good Hygiene

Always wash your hands before:

  • Cooking
  • Eating
  • Feeding another person
  • Giving medicine

Always wash your hands after:

  • Using a toilet or changing nappies.

Other practices that ensure good hygiene:

·      Use clean water whenever possible and boil drinking water, especiallyforyoung children.

·      Wash bed linen, towels and clothes with soap and water.

·      Store food properly to prevent it from spoiling and causing infection.

·      When someone in the family is sick, wash drinking cups before you share them.

·      Cover your mouth when sneezing or coughing.

·      Avoid spitting or always spit into a container, not on the ground.

·      Kiss babies on the top of their heads rather than on the lips.

·      Wash eating utensils, including items for babies, with soap and water.

·      Carefully wash all raw fruits and vegetables with clean water.

·      Wash objects that a child or infant frequently puts in its mouth with soap and clean water.

·      Dispose of waste properly.

·      Put dirty things like nappies, used tissues, handkerchiefs, and other soiled objects out of the reach of children until they can be removed from the home.  Put them in a container that is hard to open until you can clean or dispose of them properly.

·      Proper waste disposal may include using a pit latrine, or burning or burying objects.

Avoiding malaria

In many parts of the world malaria is a very common illness caused by an infection passed by the bite of a mosquito. There are many things you can do to prevent this infection and therefore prevent the further weakening of the body, including the following:

·         Use bed nets (mosquito nets preferably treated with insecticide) or a sheet to protect the family when they sleep - cover the baby's cradle or bed with a mosquito net or thin cloth.

·         Use insecticide sprays and repellents to protect your home or body from any mosquitos in the area.

·         Drain any standing water which may be a mosquito breeding ground in your community.

 

General rules on caring for a child with HIV infection or AlDS

1. Feed the child well

 

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For a child less than four to six months old the best food is breast milk.  Breast milk is important because it gives an infant protection against many types of infections.  Also, since breast milk is dean, the infant is not at risk of getting diarrhoea as with milk of other types.

However, the fact that HIV can be passed through breast milk makes it more difficult to decide what is best for the child. in many areas of the world the risk of transmission of 14IV by breast-feeding is low compared with the risk of the infant dying of other infectious diseases if not breast-fed.

As the health care worker, you will have to help the HIV-infected mother weigh the possible risks to the infant of breast-feeding versus not breastfeeding, taking into account such things as:

  • Whether many of the children in the area are at risk of, or die from, infections and poor nutrition
  • Whether there is a good alternative to breast-feeding available that is clean, safe, nutritious and affordable.

It is recommended that if the HIV-infected mother lives in a place where many children die at a young age from infectious diseases (like respiratory infections or diarrhea), or if the child is likely to be manlnourished, she should breastfeed her infant, even if she is infected with HW or has AIDS.

            However, if she has the possibility of giving a dean, safe and nutritious substitute for breast milk that is affordable for the entire period it is required, it would be a good choice.  If she decides to feed her baby using breast-milk substitutes rather than breast milk, she must use dean water, which has been boiled and then cooled, and clean equipment (teats and bottles, cups and spoons).  Remind her to follow the directions carefully and not to add more water in an effort to save money - this can lead to malnutrition in her child.  If she cannot follow all of the above requirements all of the time, advise her to breast-feed her baby.

An infant who is breast-fed should receive only breast milk until it is four to six months old.  Once a child is four to six months old it should be given some solid foods along with the breast milk.  Healthy foods that are warm and either soft or mashed can be given with a spoon or your fingers. (Don't forget hands must be washed first!) Milk alone is not enough for a child after six months, but it is very important that breast-feeding or a nutritious substitute be continued along with other foods.

2. Have the child immunized

You maybe asked whether an infant with FHV/AIDS should be given vaccines against the common childhood illnesses.  All infants, including those with HIV infection and AIDS, should be given the standard vaccines against diphtheria, pertussis (whooping cough) and tetanus (DPT vaccine), and against poliomyelitis (polio) and measles.  This should be done as early as possible in accordance with the immunization schedules of your country.  In many countries, the BCG vaccine is given to all infants at birth to prevent tuberculosis.  The only exception is that if an infant has clinical symptoms of AIDS, such as failure to thrive and frequent infections, it should not receive BCG, but should receive all other vaccines mentioned above.

3. Make sure the child gets early treatment for infections

The advice given earlier in this chapter on avoiding common infections is important.  As children become older they will need to learn these things for themselves, for example about washing their hands after going to the toilet or latrine, and about the importance of washing their hands before eating.  Protecting children with AIDS from other infections is more difficult than protecting adults because children tend to put things in their mouths and they are exposed to more illnesses that are new to them.

It is much better for a family to go to the same health care setting where the child's health history is known rather than keep changing or "shopping around".

4. Treat the child as normal

Many of the infants who are infected with HIV will have months or years of life without symptoms.  Every effort should be made to help them lead as normal a life as possible.  This includes letting them spend time playing with other children.

A child with HIV infection should go to school as usual, except when there is an outbreak of an infection in the school which could make the child ill.