Cystic Fibrosis has advanced with medical treatments and advocacy by patient groups such as the Cystic Fibrosis Foundation (CFF).

Short review of what is cystic fibrosis?

Cystic fibrosis (CF) is a genetic disease. This means that CF is inherited.

Mutations in a gene called the CFTR (cystic fibrosis conductance transmembrane regulator) gene cause CF. The CFTR mutations causes changes in the body’s cell’s electrolyte transport system. Electrolytes are substances in blood that are critical to cell function. The main result of these transport system changes are seen in the body secretions, such as mucus and sweat.

The CFTR gene is quite large and complex. There are many different mutations in this gene that have been linked to CF.

A person will be born with CF only if 2 CF genes are inherited–one from the mother and one from the father.  An individual must inherit two non-functioning CF genes – one from each parent – to have CF. If both parents are carriers there is a 1 in 4 (25 percent) chance that both will pass on the non-functioning gene, which would result in a pregnancy affected with cystic fibrosis.

A person who has only one CF gene is called a CF carrier. They are healthy and don’t have the disease. But they are a carrier of the disease.  See above box in specifics how much a infant is at risk getting the disease.

CF affects various organ systems in children and young adults, including the following:

• Respiratory system
• Digestive system
• Reproductive system

How does CF affect the respiratory system?

The abnormal electrolyte transport system in CF causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move. These thick secretions increase the risk for frequent respiratory infections.

Recurrent respiratory infections lead to progressive damage in the lungs, and eventually death of the cells in the lungs.

Because of the high rate of infection in the lower respiratory tract, people with CF may develop a chronic cough, blood in the sputum, and often even have a collapsed lung. The cough is usually worse in the morning or after activity.

People with CF also have upper respiratory tract symptoms. Some have nasal polyps that need surgical removal. Nasal polyps are small protrusions of tissue from the lining of the nose that can block and irritate the nasal cavity. People with CF also have higher rates of sinus infections.

How does CF affect the gastrointestinal (GI) system?

CF mainly affects the pancreas. The pancreas secretes substances that aid digestion and help control blood sugar levels.

The secretions from the pancreas also become thick and can clog the ducts of the pancreas. This may cause a decrease in the secretion of enzymes from the pancreas that normally help digest food. A person with CF has trouble absorbing proteins, fats, and vitamins A, D, E, and K.

The problems with the pancreas can become so severe that some of the cells in the pancreas die. Over time, this may lead to glucose intolerance and Cystic Fibrosis-Related Diabetes (CFRD), a unique type of insulin-dependent diabetes.

The symptoms of CF that may be due to involvement with the GI tract include:

• Bulky, greasy stools
• Rectal prolapse (a condition in which the lower end of the bowel comes out of the anus)
• Delayed puberty
• Fat in the stools
• Stomach pain
• Bloody diarrhea

The liver may also be affected. A small number of people may develop liver disease. Symptoms of liver disease include:

• Enlarged liver
• Swollen belly
• Yellow color to the skin (jaundice)
• Vomiting of blood

How does CF affect the reproductive system?

Most males with CF have blockage of the sperm canal. This is called congenital bilateral absence of the vas deferens (CBAVD). This results from the thick secretions clogging the vas deferens and keeping them from developing properly. It causes infertility because sperm can’t travel out of the body. There are some newer techniques that allow men with cystic fibrosis to have children. These should be discussed with your healthcare provider. Women also have an increase in thick cervical mucus that may lead to a decrease in fertility, although many women with CF are able to have children.

Who is at risk for cystic fibrosis?

Cystic fibrosis (CF) is inherited, and a person with CF had both parents pass the altered gene to them. The birth of a child with CF is often a total surprise to a family, since most of the time there is no family history of CF.

Symptoms can include with above symptoms the following:

All U.S. states require that newborns be tested for cystic fibrosis (CF). This means that parents can know if their baby has the disease and can take precautions and watch for early signs of problems.

The following are the most common symptoms of CF. However, people may experience symptoms differently, and the severity of symptoms can vary, too. Symptoms may include:

• Thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems, and chronic lung disease.

Many other medical problems can point to cystic fibrosis, as well. These include:

Sinusitis, Nasal polyps, Clubbing of fingers and toes. This means thickened fingertips and toes because of less oxygen in the blood, Collapse of the lung often due to intense coughing, Coughing up blood, Enlargement of the right side of the heart due to increased pressure in the lungs (Cor pulmonale), Abdominal pain, Excess gas in the intestines, Rectal prolapse. In this condition, the lower end of the bowel comes out of the anus, Liver disease, Diabetes, Pancreatitis, or inflammation of the pancreas that causes severe pain in the belly, Gallstones, Congenital bilateral absence of the vas deferens (CBAVD) in males. This causes blockages of the sperm canal.

The symptoms of CF differ for each person.

Infants born with CF usually show symptoms by age 2. Some children, though, may not show symptoms until later in life. The following signs are suspicious of CF, and infants having these signs may be further tested for CF:

Diarrhea that does not go away, Foul-smelling stools, Greasy stools, Frequent wheezing, Frequent pneumonia or other lung infections, Persistent cough, Skin that tastes like salt, Poor growth despite having a good appetite.

The symptoms of CF may resemble other conditions or medical problems. See a healthcare provider for a diagnosis.

“The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.

Cystic fibrosis is genetic disease that affects the lungs, pancreas, and other organs. It is progressive, meaning that it gets worse over time.

There are close to 40,000 children and adults living with cystic fibrosis in the United States and an estimated 105,000 people have been diagnosed with CF across 94 countries. CF can affect people of every racial and ethnic group.

There are many things that are misconceptions about CF.

In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to not function properly. When the protein is not working correctly, it’s unable to help move chloride — a part of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.

CF affects multiple organs in the body.”

The Cystic Fibrosis Foundation (About Cystic Fibrosis | Cystic Fibrosis Foundation)

Cystic fibrosis (CF) is a genetic disorder that causes problems with the lungs=breathing and digestion sytem.  It can obstruct the pancreas. CF affects about 35,000 people in the United States. Cystic fibrosis (CF) can be life-threatening, and people with the condition tend to have a shorter-than-normal life span.   This diagnosis can have mucus that is too thick and sticky, which

• blocks airways and leads to lung damage;
• traps germs and makes infections more likely; and
• prevents proteins needed for digestion from reaching the intestines, which decreases the body’s ability to absorb nutrients from food.

Cystic fibrosis is a hereditary disease that affects the lungs and digestive system. The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas. 

What is this disease?

“Drinking water does more than just quench your thirst. It’s essential to keeping your body functioning properly and feeling healthy.

Nearly all of your body’s major systems depend on water to function and survive. With water making up about 60% of your body weight, it’s no surprise what staying hydrated can do for you.

Some ways the water works in our bodies is the following:

• Regulates body temperature
• Moistens tissues in the eyes, nose, mouth and other areas
• Protects body organs and tissues
• Carries nutrients and oxygen to cells.”

MAYO CLINIC (Water: Essential for your body – Mayo Clinic Health System)

“No matter what stung you, you’re likely to feel some pain, throbbing and stinging, along with redness in the affected area. Unless you have an allergic reaction, these symptoms shouldn’t stick around for too long.

“Redness and swelling at the site of the sting is a normal reaction and will subside over time, usually in one to two days,” says Dr. Fertel. In terms of treatment, bee, wasp and hornet stings can be treated the same — unless the person has a specific allergy.

You may have heard that you should swipe the stinger out with a credit card or your fingernail rather than pinching and pulling it out. But, says Dr. Fertel, there’s no evidence that the technique makes a difference. What does matter is how quickly you remove the stinger. So, don’t stress about how it’s done — just get it over with fast. This is because the quicker you’re able to remove the stinger, the less venom will be pumped into the wound. This will help decrease the side effects of the sting and reduce the risk for anaphylactic shock if you’re allergic.”

Cleveland Clinic (Bee Sting Treatment and Prevention)

What you should do if you or your child or family gets stung is:

One get into a safe area and away from a hive or outside where more stinging insects can come and attack you.

Two look at the area and if you see the stinger DO NOT SQUEEZE IT OUT since you will squeeze out more venom from the stinger but what you can do is get a tweezer and pull it our or if not available you can attempt to scratch it out with a nail (like if you are out camping and have no tweezers for example).

Three than wash the area out with soap and apply ice if the area is in pain to give the numbing affect to the area and decrease the pain with decreasing the venom from spreading.

If the area is itching apply oatmeal or a antihistamine cream to the area to decrease the itching or maybe even a cool bath and if you are not allergic (the majority of us are not) but you DO get stung by a bee outside hiking look for some plantain – chew it up a bit at the front of your mouth – and then spit the chewed up leaf and saliva on the sting.

Most stings will cause a small red bump to the area that got stung. For most part they can be treated at home depending on the area that was stung (Foot vs EYE for example). It would also include the reaction the individual has (LOCAL vs SYSTEMIC or even ANAPHYLACTIC=An allergic reaction that needs to be treated immediately or fatal, usually with epinephrine injection.).

Stung in the eye it will get swollen and shut and immediate evaluation from a MD is needed to make sure there is no other injury to the eye or that they didn’t even actually get stung in the eye itself.

If you show hives with DIFFICULTY BREATHING or DIFFICULTY SWALLOWING you NEED TO CALL 911 IMMEDIATELY since this is indicating a ANAPHYLACTIC REACTION most likely that needs treatment ASAP!! Since this can lead to shock or unconsciousness.

If you have reason to think you may be seriously allergic to bee venom, you should carry an Epipen (further discussed below).

How to determine if your even allergic to stings:

The diagnosis is made by a specialist, an allergist, by interviewing the patient and doing special allergy tests. If someone has had what is described as a systemic reaction, they should have venom skin tests done by an allergist to identify which venoms they are allergic to. The allergist can then recommend, based on the kind of reaction that the patient had, what kind of prevention would be the best idea for that person. For some people, it might be enough to be careful and carry an EpiPen, but for most people with insect skin allergy the best recommendation is to be immunized with venom treatment, because the allergy shots are highly effective to prevent dangerous reactions. This would all be done after any serious reactions were first taken care of in the ER if you had to call 911.

If you have reason to think you may be seriously allergic to bee venom, you should carry an Epipen (further discussed below). What it this exactly? An EpiPen is one kind of injector to deliver epinephrine, also known as adrenaline. It is a spring-loaded injector that makes it easy for somebody to give themselves an emergency injection that can be life-saving when there’s a severe allergic reaction. An EpiPen is useful for someone to carry if they have had a severe allergic reaction in the past. This is true for insect sting allergy and for some food allergies or other causes of anaphylaxis.

Let me point outthat there is no other medicine that can counteract a severe allergic reaction, but sometimes even the EpiPen isn’t enough; so when someone needs to use an EpiPen they should call 911, because they may need intravenous fluids or oxygen or other medicines.

BE SAFE RATHER THAN SORRY!

So let us remember it is coming onto summer and their BACK AGAIN!

 References

1-Read more: http://www.ehow.com/how 2-NEWS4JAX.com Published On: May 30 2014 09:38:22 AM EDT

3-http//beestrawbridge.blogspot.com/2013/03/which- bees-sting and which-don’t.html with Phil Chandler of Biobees.

4-Wikipedia-2013 published Bees

5-MedicineNet.com Bee and Wasp Sting 12/11/2013

6-See more at: http://www.about-bees.com/carpenter-bees.html#sthash.NywAhKk2.dpuf

“You can do a few things to minimize your sun exposure and reduce your risk of skin damage.

• Try to stay in the shade as much as possible. If you are in the sun, wear protective clothing such as a long-sleeved shirt and pants.
• You should also apply sunscreen with a high SPF rating to exposed skin.
• Avoid being in the sun during peak hours, typically between 10 am and 4 pm.

By taking these precautions, you can help to protect your skin from the harmful effects of UV radiation.

UV rays are just as harmful on cloudy days as on sunny days.

While it’s true that people with darker skin have a higher natural SPF than those with lighter skin, this does not mean that they are immune to the effects of UV radiation.

While sunscreen is a vital part of any sun safety plan, it’s not the only thing you need to do to protect yourself from UV radiation. You should also take steps to avoid excessive sun exposure and wear protective clothing when you are in the sun.”

Skin and Cancer Institute (July is UV Safety Month | Skin And Cancer Institute)