2019 Cystic Fibrosis Awareness Month!

Each day of May we are going to answer a question about CF! We will be linking to as many article as possible through out this post so you can discover more information on each topic. Most of them will be from the Cystic Fibrosis Foundation website as it is the trusted source for all things related to CF.

Come back to this post or follow us on social media to discover the answer! If you have questions you’d like answered please let us know! Comment or message us!

1. How do you get Cystic Fibrosis?

To test positive for Cystic Fibrosis you have to have two mutated CFTR genes. Each parent has to pass down their mutated gene. This means there is a 1/4 chance that a baby will have Cystic Fibrosis if both parents are carriers. A 2/4 chance that the child will only be a carrier of a single mutated gene, and a 1/4 chance that the child will not have either mutated gene. (The genes do not have to match to create Cystic Fibrosis, any two mutated gene will result in the disease.)

All 50 states have been required to test for CF in the newborn screening since 2010, only 10 states were doing so in 2005. Even though they test for it, not all mutations are common and included in the test. Many mutations are still unknown so other test will be run to confirm CF.

Many hospitals will run a full blood panel to check for all known mutated gene. This test can be run on the parents or the baby while it is still in the womb to determine the mutated genes.

The sweat test is another common method to test for Cystic Fibrosis. This test will measure the amount of chloride in the sweat. Most babies will have the sweat test done even though they already had a blood test or newborn screening showing a positive Cystic Fibrosis diagnosis.

You cannot “catch” Cystic Fibrosis, it is only passed through the genes you inherit from your parents.

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2. How does the mutated gene work?

Located on human chromosome 7, the CFTR gene is made up of 250,000 DNA nucleotides. CFTR stands for Cystic Fibrosis Transmembrane Conductance Regulator. The CFTR protein functions as a channel for the movement of chloride ions in and out of cells, which is important for the salt and water balance on epithelial surfaces, such as in the lungs or pancreas. In Cystic Fibrosis, mutations affect the composition of the mucus layer lining the epithelial surfaces in the lungs and pancreas.

More than 1,700 different mutations have been discovered, and many more are still unknown. They are separated into 5 different classes, each class affecting the body differently. You can learn more about these mutations and how they work here.

Kitty’s mutation is DDF508, (or 2 copies of the F508del mutation, the most common combination), this is a Class 2 mutation meaning “the CFTR protein is created, but misfolds, keeping it from moving to the cell surface.” (Awesome PDF Gene Chart Source)

The most common CF mutation, F508del, is primarily considered to be a processing mutation. The F508del mutation removes a single amino acid from the CFTR protein. Without this building block, the CFTR protein cannot stay in the correct 3-D shape. The cell recognizes that the protein isn’t the right shape and disposes of it.
The drug combination lumacaftor/ivacaftor (Orkambi®) works by enabling CFTR protein with an F508del mutation to fold in a more correct shape, and then activates the protein to allow more chloride to pass through. Although this drug combination is not a perfect fix, it helps the mutant CFTR protein to move some chloride. This movement of chloride reduces the symptoms of CF.

Source

While that was a lot of technical jargon, we’ll get into what that means for someone with Cystic Fibrosis as the month goes on! If you’d like to share this post via Facebook click here!

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3. What is the life expectancy?

When looking at ‘how long someone with cystic fibrosis will live’ there are many things to consider and understand. In truth, cystic fibrosis varies so much from person to person, it’s hard to nail down a blanket life expectancy. This is a great break down from the CFF – Understanding Changes in Life Expectancy

Cystic Fibrosis is a terminal disease, meaning it’s likely that anyone living with it will die from complications of the disease long before the average human life expectancy. (yes, anyone can die at any time from anything, but that is not what we are here to discuss)

Most living with it currently will not see their 47th birthday. While there are numerous advancements being made yearly, we’ve got a long way to go before we can comfortable say, ‘someone with CF will live long into adulthood’. While it’s great to see these numbers increase, it isn’t a cure, and it doesn’t mean everyone with CF will hit this landmark number.

Currently, the CF Foundation states:

Based on 2017 Registry data, the life expectancy of people with CF who are born between 2013 and 2017 is predicted to be 44 years. Data also show that of the babies who are born in 2017, half are predicted to live to be 46 years or older.

Awesome 2017 Registry highlights PDF

Any time the life expectancy raises many will misunderstand the meaning of that number. The number released is a median, not an average. It means that half of the people born between the years listed will not live to that age.

We must keep in mind that this current median doesn’t apply to at all living with CF, and too many will not live to their 18th birthday. Life Expectancy isn’t once size fits all, and it’s certainly not where we should focus our attention as it’s not reliable. No matter the number listed, we fight for a cure, for extra years, for our kids to our live us.

Take it from someone with CF when pondering what Life Expectancy means each time there is an increase in the median.

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4. How does CF affect the body?

Many people like to call Cystic Fibrosis a lung disease, while this is a quick introduction to what CF is, it is an over simplification and slightly incorrect. CF is a genetic disease that affects the whole body differently. Not all with CF have the same lung complications, or body complications. To treat CF you have to know how it affects every part of the body not just the lungs.

The foundation has a great video on how it affects the whole body here. I’ll list a quick list of all that can be affected by CF.

  • Mental HealthDepression, Anxiety, etc from living with a terminal disease, spending half your life in the hospital or attached to treatments will wear on your mental health. It’s important for people with CF to be aware of this and have a support team to help them cope.
  • Lung Health – The build up of thick sticky mucus holds onto bacteria and causes scarring inside the lungs, leading to weaker lungs and frequent chest infections. Over time this will lead to shortness of breath and constant wheezing and coughing. As the lung function decreases the need to a transplant will grow. A transplant doesn’t cure these problems, but brings on new issues so it is a well thought out decision.
  • Liver Health – “In the liver, the thick mucus can block the bile duct, causing liver disease.” (Source)
  • Skin Health – Due to the mutated gene, sweat contains more salt than normal, which can affect the health of the skin.
  • Hands – Many with CF will develop Clubbing of the fingers.
  • Digestive System – From in womb, the digestive track begins it’s life of not being able to process the mucus thrown into it. The pancreas clogs and looses it’s ability to absorb nutrients properly. As soon as infants are diagnosed they are usually put on a pancreas enzyme supplement to help with the absorption. This failure can lead to poor weight gain, thus having to use a g-tube to get as much nutrition into the body as possible. The lack of absorption will lead to greasy, loose or bulky stools, it can also affect constipation. Many with CF are on a regiment to help them stay regular. The mucus build up in the pancreas causes scarring which will lead to the pancreas not being able to release the proper amount of insulin. CF has a unique category of diabetes that is a combination of Type 1 & Type 2. (CFRD) You can learn more about it here. With the life expectancy increasing new complications from high cholesterol & other gut health related issues are showing up. When people with CF weren’t living past childhood weight gain no matter the source was of dire importance, but now people with CF are focusing on healthy high calorie diets to prolong overall body health with weight gain.
  • Reproductive System – 95% of males & 20% of females are infertile due to complications from CF. Learn more about Family Planning and CF here.
  • Bones – With people living with CF living well into adulthood new complications are being discovered, such as weak and brittle bones, plus arthritis. You can read more about this here.

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5. How common is Cystic Fibrosis?

According to the Cystic Fibrosis Foundation Patient Registry, in the United States:
– More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
– Approximately 1,000 new cases of CF are diagnosed each year.
– More than 75 percent of people with CF are diagnosed by age 2.
– More than half of the CF population is age 18 or older. 

About Cystic Fibrosis – CFF

One of the most sobering facts that I’ve realized in the past 7.5 years that I’ve been apart of the CF community is that these number don’t change. About a 1,000 people are diagnosed yearly, but the average amount currently living with CF doesn’t rise enough to warrant a new “people living with CF” number.

In looking into this, I found that in 2016 29,326 were living with CF, and by 2017 the number changed to 29,887. While 880 were diagnosed, 380 lost their battle.

The silver lining in all of this? With the advancements in treatments, these numbers are slowly changing. If you compare 2002 to 2017, it becomes obvious that we are on the right path.

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6. How do you treat Cystic Fibrosis?

No two cases of Cystic Fibrosis are alike, even if they have the same two mutations. This creates a wide variety of treatment strategies and doctor opinions. It can be frustrating to navigate the treatment options due this wide variance.

With the advancements in treatment options, many will start required, and preventative treatments as soon as they are diagnosed. People with CF will take oral pills & liquids plus nebulized medications. In the US it is also common for kids to start using the vest by age 2.

Oral Pills

  • Pancreas Enzymes – The majority of people living with CF are also pancreas insufficient. They are required to take a supplement enzyme to help do the work of the pancreas. A common brand in the US is Creon. Infants will have the capsules opened up, and sprinkled onto something acidic like apple sauce to delay activation. Maintaining a high body weight has been shown to benefit healthy lung development. When a person is having trouble gaining, the care team will look into adding a G-Tube to their treatment plan. (explanation to come!)
  • Mulivitamin – Due to not being able to properly absorb nutrition, many people with CF are on specially formulated vitamins that provide that key nutrients they lack. These vitamins are usually made from whole foods, and are formulated slightly different than the over the counter variety to make sure they get the most out of them.

Nebulized / Inhaled Medication & Airway Clearance

Think of this regiment as a can of cream of mushroom soup, with the mucus being the condensed soup.

  • Albuterol – Expands the airways. (open the can)
  •  
  • Hypertonic Saline – The salt brings water into the airways, to aid in thinning the mucus (adding a can of water to the soup)
  • Pulmozyme – Breaks up the mucus. (breaking up the condensed soup with your spoon)
  • Vest / CPT – Shakes the mucus out of the lungs (hitting the back of the can to get the soup into the pot)

Antibiotics

It is common for people with CF to be on antibiotics for various reasons such as culturing a bacteria like Pseudomonas, due to being on antibiotics often, they can become resistance.

CFTR Modulators

The newest addition to the CF treatment plan are modulators that correct the mutated protein. Thanks to the research of companies like Vertex, each year new modulators are being tested and released to the public. These modulators have been life changing for many with Cystic Fibrosis.

To be able to take a modulator you must have the designated mutation.

Lung Transplants

Many assume that because the new lungs will not have the mutated CFTR genes, it will cure the person with cystic fibrosis, but this is a misconception. While lung transplants will improve & prolong life, they come with a new set of problems. Because of this, people with CF work with their care team to make sure it’s the right step for them to take.

Hospital Stays / Ports or PICCs

It is common for people with CF to require PICCs or ports due to the frequent hospital stays and IV medicines that can be required. If you are the friend or family member of someone with CF, make sure to check in on them often, and let them know how much you care, hospital stays can be isolating without help from a support team. Don’t know what to do? Check out this post.

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7. A History of Past Treatment Ideas!

Even within the past 10 years, the treatments plans for Cystic Fibrosis have changed drastically. But looking at how far we’ve come is an awesome way to appreciate the advancement of medicine.

The Cystic Fibrosis News Today site did a great post on the complete history of treatments that you can read here. Here is a few treatments methods of the past:

Mist Tents

“Patients sleep in “mist tents” filled with misted 10 percent solution of propylene glycol and 3 percent saline. Mist tents are discontinued by most clinics in the 1970s.”

Poor Diet for Calories

In the past, when children weren’t surviving to their teens, the goal was to pack on as many calories & fat as possible. Doctors would suggest feeding the child many (unhealthy by the non-cf standards) items like all the Mc Donalds they want, or a diet full of cake and ice cream. While these items are high in calories, they are empty calories. Now that people with CF are living well into adulthood, the science is in, a diet of healthy fats, and high whole food calories will help build healthy lungs and a better body. The CFF has a great post on healthy high calorie eating here.

We like to tell our daughter, that when she eats an empty calorie she will have to end up eating it twice, to make up for it later. Every calorie counts, why not make them the best calories possible.

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8. Why do people with CF have salty skin?

If you were to kiss the skin of a CF patient, you would find they taste salty. After a day of fun in the sun many people with CF find salt crystals built up around their hair line, or on their nose. This is due to the high concentration of salt in their sweat from the faulty protein channels on the cell surface.

Doctors use a Sweat Chloride Test as a method to test for CF, commonly known as a Sweat Test. This test measures the amount of salt that is in a persons sweat. People with CF have 2 – 3 times more sodium and chloride in their sweat than people without CF.

Due to this excessive loss in salt, people with CF also dehydrate faster than expected. It’s important for them to intake extra salt & stay hydrated.

Cystic fibrosis can lower the normal salt levels in the body, which can lead to a variety of short- and long-term problems.
Sweat glands cool the body by releasing perspiration (sweat) from the lower layers of the skin onto the surface. Sodium and chloride (salt) help carry water to the skin’s surface and are then reabsorbed into the body. As the water evaporates, heat is carried away, and the body cools.
In people who have cystic fibrosis, the salt travels to the skin’s surface with the water and is not reabsorbed. Because of this, the skin of a child who has cystic fibrosis is abnormally salty. Parents may notice salty-tasting skin when they kiss the child.
People who have cystic fibrosis can become quickly depleted of salts, especially when the weather is hot, when they exercise strenuously, or when they have a fever. Low salt levels in the body lead to fatigue, weakness, fever, muscle cramps, stomach pain, vomiting, dehydration, and heatstroke. To avoid these conditions, people who have cystic fibrosis need to keep well hydrated and keep healthy salt levels in the body. Sports drinks that contain electrolytes (such as sodium and potassium) are especially good to help replace lost salts. Do not use salt tablets without talking to your doctor first.

Source

Currently at 7 years old, little miss is taking an 1/8 teaspoon of salt a day. During the summer months we up that amount. She also drinks pickle juice and electrolyte drinks to help re-hydrate her. You can learn about our other hydration tricks here.

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9. What is a typical day with CF like?

Our day is still on the lower side as far as meds go, while this is our typical schedule it is below average for someone with CF so after I post our routine, I am going to link to some more people with CF that have talked about their typical days. “On average, a person with Cystic Fibrosis spends the equivalent of four months of full-time work doing life sustaining treatments every year.” – Source

  • 7:15 – Wake up
  • 7:30 – 8:00: Albuterol, Hypertonic Saline, Vest
  • 8:30 – Breakfast – 4 Creon
  • 10:00 – Second Breakfast – (usually fruit)
  • 11:00 – elevensies – 4 Creon
  • 2:00 – Lunch – 4 Creon
  • 4:00 – Afternoon tea – (usually fruit)
  • 6:00 – 6:40: Albuterol, Hypertonic Saline, Vest, Pulmozyme
  • 7:00 – Dinner – 4 Creon, Vitamin
  • 8:00 – Bedtime

A Day in the Life of Cystic Fibrosis:
(if you have a day in the life of post, let me know and I’ll add it here!)

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10. Where did 65 roses come from?

So many use 65 Roses to represent Cystic Fibrosis, tattoos, fundraising, and inspiration to keep fighting. But where did it come from?

Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had CF. Her duty was to call every civic club, social, and service organization, seeking financial support for CF research. Mary’s 4-year-old son, Richard (Ricky), listened closely to his mother as she made each call.


After several calls, Richard came into the room and told his mom, “I know what you are working for.” Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had cystic fibrosis.


With some trepidation, Mary asked, “What am I working for, Ricky?” He answered, “You are working for 65 Roses.” Mary was speechless.
He could not see the tears running down Mary’s cheeks as she stammered, “Yes Ricky, I’m working for 65 Roses.”


Since 1965, the term “65 Roses” has been used by children of all ages to describe their disease. But, making it easier to say does not make CF any easier to live with.


The ugly fact is that cystic fibrosis is a life-threatening genetic disease that affects 30,000 children and adults in the United States.


Sadly, Richard lost his fight against CF in 2014. Richard is survived by his devoted wife, Lisa, his adored dog, Keppie, and his brother, Anthony.


The “65 Roses” story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation.

Source

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11. What is aquagenic wrinkling?

Aquagenic Wrinkling of the palms often occurs in CF patients and at times carriers. It is rapid pruning of the hands and feet when exposed to water. (Washing hands, holding a drink that is sweating)

It may be caused by a salt imbalance in the skin cells, which results in increased water retention within these cells and increased transepidermal water loss.

Here are two studies done on this:

RESULTS:
Mean time to wrinkling was 11 min in controls, 7 min in carriers and 2 min in patients with CF. AWP was not seen in controls, but occurred in 80% of patients with CF and 25% of carriers. There was a significant difference between groups

Full Study

Results The mean AWP score of the CF group was significantly higher than the mean score of the control group (1.5 vs 0.6; P < .001). Patients with CF who were homozygous for the ΔF508 mutation (n = 27) had significantly higher scores than patients with CF who were not homozygous for the ΔF508 mutation (n = 17) (1.7 vs 1.1; P = .02). The 17 patients with CF who were not homozygous for the ΔF508 mutation still had higher scores than the control group (1.1 vs 0.6; P = .03). There was no correlation between sweat chloride concentrations measured at the time of diagnosis and AWP score.

Full Study

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12. What is clubbing of the nails?

Clubbing of the nails can occur in people with Cystic Fibrosis and other lung/heart related diseases where oxygen in the blood is affected.

Only a doctor can diagnose clubbing of the nails, but the clubbing is a clinical finding characterized by bulbous fusiform enlargement of the distal portion of a digit.

For further reading on clubbing check out this article.

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13. A History of the CF Foundation!

The Cystic Fibrosis Foundation has been a long standing beacon of hope for those with CF. Through their diligent work they have created a support network for those with CF and their families.

They have created a network of trusted doctors, and programs to help every aspect of Cystic Fibrosis. They continue to fight for a cure, and our are great asset. If you haven’t reached out to your local chapter I urge you to do so. Ours has been a dream to have apart of our tribe.

If you’d like to read about the CF Foundation you can do so here.

A Brief History:

  • 1955 – Foundation Created
  • 1961 – National Network of Centers created to streamline therapies and research.
  • 1980 – Research development Program
  • 1989 – Program discovers CF Gene
  • 1197 – Foundation establishes the Therapeutics Development Program
  • 2006 – CF Congressional Caucus is launched

Today:

  • 11 Research Centers
  • 70 Local CFF Chapters
  • 130+ CF Care Centers

Read more about the history of the foundation here.

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14. What is CF related diabetes?

CFRD or Cystic Fibrosis Related Diabetes is not as straight cut as Diabetes Type 1 or Type 2. As with all aspects of CF, diabetes related to CF will affect each person different, and have a wide range of treatment paths.

The mucus build up in the pancreas causes scarring which will lead to the pancreas not being able to release the proper amount of insulin. CF has a unique category of diabetes that is a combination of Type 1 & Type 2.

It is hard to diagnose as many of the symptoms over lap with general CF symptoms. So people with CF monitor their symptoms, and can begin testing for CFRD around 10.

You can learn more about it here.

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15. Folklore of the CFTR Gene!

CFTR gene mutation is in 1 in 25 of Caucasian decent (White European) people. It is thought that this is so high due to the advantage carriers had over non carriers during the outbreaks of Cholera and Typhus. These diseases create an electrolyte imbalance and acute diarrhea which leads to dehydration. Carriers of CFTR only secreted half the fluid which was enough to flush the toxins.

While Cholera & Typhus might have helped push the CFTR gene into being more prevalent, Tuberculosis has been traced back to being one of the main reasons for the large population that are carriers. You can read more about this phenomenon here and here.

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16. Why do people with CF eat a high calorie diet?

We often joke that our daughter needs to eat a hobbit style diet. With her high calorie intake needs I feel like we are constantly feeding her some days.

People with CF have mucus clogging many of their organs, one being the pancreas. This causes them to not absorb nutrients properly. By eating more calories, they have a better chance of absorbing what they need to stay healthy. It has also been shown to help lung development, when the person is in the higher range for BMI. As such, our clinic closely monitors her height and weight growth path.

Individuals with CF use a lot of energy to breathe and fight infections. But protein and fat digestion problems mean there are fewer nutrients for the body to use. A lack of protein and fat are the main causes of muscle wasting, which also can be caused by chronic low-grade inflammation, use of oral corticosteroids and hormonal changes.

Source

As I mentioned on Day 7, when children weren’t living to adulthood, the goal was any calorie, but now it is more well known, that each calorie needs to be the best possible calorie to promote better over all body health. There is still a lot of research to be done on Gut Health and CF.

There are many great resources for healthy diets and CF on the CF Foundation website, check out this and this for further reading on this topic!

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17. What is nasal polyposis?

Nasal polyps are common in people with cystic fibrosis, affecting between 10 to 50 percent. Polyps are small, sac-like growths of inflamed nasal mucosa (masses in the nose) caused by chronic inflammation in the nasal lining. Examined under a microscope, nasal polyps are essentially bags of tissue and fluid.

Source

Symptoms include nasal airway obstruction, anosmia, rhinorrhea, and exacerbation of the rhinosinusitis that affects these patients. The polyps are probably a consequence of chronic inflammation; however, their specific etiology is unclear. Treatment includes antibiotics, topical nasal steroids, decongestants, and mucolytics. Surgery is necessary when conservative measures fail.

Source

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18. What is an invisible illness?

I can’t count the number of times someone will say “But she looks so healthy! She must be doing great right?” when they find out Kitty has CF.

We’ve been deceived by her healthy appearance many times over the years. As xrays show CF progression, but we see no outward signs of it. This is why CF is called an invisible illness.

An invisible illness is an illness that you cannot see immediately. There are many illnesses that fall under this blanket term, and many fight the stigma of people not understanding that even though they appear healthy, their body or mind is fighting a hidden battle.

Even in the doctors office, many with an invisible illness (such as lupus) can be miss diagnosed for years before they finally receive a correct diagnosis and proper treatment. It’s so important to not make assumptions about what someone may be fighting, and know that appearances are not always what they seem.

With Cystic Fibrosis, once the signs of the disease are visible from the outside, it is very liking the person is in end stages. Don’t wait until you see the visible signs to acknowledge their disease and offer the support they need.

You can read more about invisible illnesses here.

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19. Why can’t people with CF be near each other?

With the hype of 5 Feet Apart, a lot more people are aware of the importance of people with Cystic Fibrosis staying 6 feet apart at all times. The movie title was taking a stance on the fact that cystic fibrosis has taken so much from so many, so they were going to take back one more foot.

While people with CF cannot be together in the same room or area, this rule excludes siblings and family as keeping them apart would be more detrimental to their mental health than the potential harm that may happen to their physical health. It is important to note that siblings are living in the same environment anyway.

The 6 foot rule came about due to the bacteria that sticks around in the mucus inside the lungs. Germs can spread as far as 6 feet when released into the air through coughing or sneezing.

This is a key factor in why it is important to stay home if you are sick, you never know who is around you.

There will be times that being 6 feet apart is unavoidable, like in school, or a workplace. The CFF has a great article on how to manage this here.

And this is a great video on why cross contamination awareness is so important.

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20. What is Pseudomonas Aeruginosa?

Kitty first cultured Pseudomonas back in 2017, I wrote up a full post on what it is, and what that culture meant for her here. She has cultured it again this past April so we are back on Tobi for the foreseeable future. 
 

Pseudomonas Aeruginosa is a common bacteria that is considered opportunistic. It only affects compromised people, such as those with CF or weak immune systems.  It is found in damp places, drains, standing water, and contact with someone infected, or something they’ve touched such as doorknobs. This bacteria is one of the few reasons the 6 foot rule is so important. 

There are very few things that the entire CF community agrees on for “things to avoid” but due to this bacteria, things like hot tubs, humidifiers, and standing water are avoided by nearly all. Bacteria will begin to grow in standing water after about 24 hours.

It is extremely difficult to get rid of, as it is resistant to most antibiotics. Thanks to research, medicines such as tobramycin are helping combat the bacteria. Currently over 50% of the CF population culture Pseudomonas. (70% of adults with CF) 

The CFF has a great video and article covering Pseudomonas that you can find here for further reading. 

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21. What is Burkholderia Cepacia?

Burkholderia Cepacia Complex (B. Cepacia) consists of about 20 different species of bacteria that are found in the natural environment, 6 of which are found most often in people with CF that contract B. Cepacia. Most are resistant to many antibiotics so it’s hard to get rid of after contracting. It can cause a rapid decline of lung function if left untreated. This bacteria can spread person to person or object. It can be found in soil and water, and has been linked to rotting onions.

The CFF has a great video and article covering Burkholderia Cepacia that you can find here for further reading. 

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22. What is MRSA?

MRSA (methicillin-resistant staphylococcus aureus) is a bacteria that can cause an infection on the skin & in the lungs. It is resistant to most antibiotics. It is spread through contact with an infected person or object (doorknob). To prevent contracting this bacteria it is important to wash hands with soap & water, keep wounds covered until healed, always cough & sneeze in a tissue, don’t share personal items, and wipe down shared equipment before using with alcohol wipes.

MRSA may worsen lung disease. A 2010 study, published in the Journal of the American Medical Association (JAMA), looked at MRSA lung infections in people with cystic fibrosis. The study researchers found that having MRSA in your lungs for longer than two years might affect survival. However, there is much more to learn about MRSA. This includes how it affects people with CF and the best treatments for MRSA lung infections.
Source

The CFF has a great video and article covering MRSA that you can find here for further reading.

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23. How do common illnesses affect CF?

Influenza or “the flu” is a highly contagious respiratory illness that is caused by a virus. The virus is well known for its severity in all people but for those with Cystic Fibrosis it can be hospitalizing, & it can lead to a severe lung infections such as pneumonia. 

Many common illnesses such as colds are easily battled by the average person. 

24. What is a G – Tube?

In previous generations when a child failed to thrive the only thing that could be done was to feed them as much food as possible and hope for the best. But thanks to scientific advancements things like the G-Tube (Gastronomy) have revolutionized weight gain in patients. Cystic Fibrosis patients aren’t the only ones that use G-Tubes but it has helped the community in life changing ways, as I’ve said above, weight gain in people with CF can be a major challenge. Even when on the correct dose of enzymes, people with CF can still have trouble gaining and maintaining weight. 

The G Tube can help people with CF get the nutrition they need to thrive when faced with weight gain, breathing or infection issues that harbor calorie absorption and intake. There are 5 different tube options, depending on the persons need. In 2014 about 11% of people with CF were using a G Tube to help supplement nutrition. 

Doctors look for these signs: Weight for height less than 85%, weight loss for 2 consecutive months, no weight gain for 2 – 3 months in children under age 5, and no weight gain for 6 months in children 5 years plus. But above all of these signs, the doctors work closely with the parents/patient to make sure that a G Tube is the right solution to the problem. 

The CFF has tons of resources on G Tubes, you can check out the general page here, and the considering a tube page (with information on the types and why) here

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25. What happens at a clinic appointment?

When a newborn is diagnosed with Cystic Fibrosis their Clinic appointments will usually start out monthly, as everything moves along, they will spread out to bimonthly, then settle into an every 3 month pattern. (this can vary from clinic to clinic, but for the most part, appts are never further apart than 3 months.)

Each appointment can vary from 2 – 6 hours long, and will happen at an accredited CF hospital. Because there are less than 150 of these locations in the US many families will find themselves driving any where from 30 minutes to over 6 hours to get to these appointments. Discover how far you’d have to travel by clicking here!

Once there, the patient will be seen by the following, some of these people will only pop in once a year, or as needed and others will be seen at each visit. 

  • Pulmonologist
  • Respiratory Therapist
  • Dietitian
  • Child Life Specialist
  • Social Worker
  • Team Registered Nurse
  • GI Specialist (as needed)
  • Physical Therapist
  • Psychologist
  • Pharmacist

You can click here to learn more about each of these people and their role on the care team.

At every clinic visit a throat swab (once older a spit cup) is completed to test for bacteria growth. These culture results come back within 2 weeks after the visit and the clinic will adjust treatment plans as needed, depending on the bacteria grown. 

The patient will also complete a Lung Function test starting around 6 years of age. These tests have the person blow into a monitor, and uses their BMI to help determine lung function.

Once a year, the clinic will draw blood to check vitamin levels, and take an x-ray to compare it to previous years and monitor disease progression. These tests can be run more often as health demands them. 

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26. What advancements have been discovered? 


All of these advancements are thanks to donations from people like you. The fundraising efforts of every person connected to CF aren’t for nothing. They move the research forward by leaps and bounds. 

It is a common misconception that CF doesn’t receive government funding, when CF does in fact receive money for research. Check out how $83 million dollars was used in 2018! You can also check out this link to read about Cystic Fibrosis Research & Translation Centers.

You can read more about Research Advancements on the CFF website by clicking here

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27. What is meconium ileus?

A Meconium Ileus is a bowel obstruction that occurs when the meconium (the first bowel movement for an newborn) in your child’s intestine is even thicker and sticker than normal, creating a blockage in a part of the small intestine called the ileum.

MI can lead to bowel perforation, a twisting of the bowel, or inflammation and infection of the abdominal cavity. About 18% of CF patients are affected by this. Many discover their child has CF because they were born with MI, about 98% born with MI have Cystic Fibrosis.

Here is an article that discusses how they diagnose and treat this obstruction. 

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28. How does smoke affect CF?

There are many different types of smoke, such as cigarette smoke (and vape smoke), bonfire smoke, and humidifier smoke. Many are harmful in different ways to those that have lung complications. 

A lot of these things can often be present in every day life, it’s important to be aware of their dangers, and weigh out the risks to the health of those with diseases like CF. 

Bonfires/BBQ Pits/Fires – We all know and love most types of outdoor fires, but within these fun past times lurk dangers to those with compromised lungs. The smoke from these fires contains tiny particles of what is being burned. These particles can get into the lungs and damage them further. But it’s not just the smoke that can cause lungs harm, if you are feeling the heat from the fire on your face, you lungs are also feeling that heat. Breathing in heat can damage the lining on the lungs.

While to the naked eye we might not see or feel these dangers, it’s one of the reasons CF is an invisible illness. There are many great articles on how to enjoy these past times but also protect the lungs. Here, Here and Here. This article has tips on detoxing after a wildfire. 

Poor Air Quality – Air quality in general can cause major complications to someone with Cystic Fibrosis. Again, these complications may not show themselves the minute a person with CF enters a poor air quality environment, but the long term health complications can be detrimental. Here is a great article on Outdoor air pollution, and Indoor air pollution. Reading over these lists can make a person want to clean and hide, but it’s important to weigh out quality of life, with crazy bubble life and find a balance to keep healthy while living life to the fullest. 

Cigarettes & Vaping – It is well known that first, second and third hand smoke is extremely dangerous to anyone, and even more so to those with lung complications. These dangers aren’t readily seen unless someone is visibly coughing but the damage done by the fumes coating the lungs will have long term effects. 

Over the years there have been many studies done on cigarettes and health but with vaping still relatively new less is known about the dangers. Many thing that vaping is safe, but the fumes from vaping are just as dangerous to those with lung diseases. If you are new to vaping in general this article is a great place to start learning about it. 

Other articles worth reading:


Humidifiers – 
While it seems strange to warn against products like humidifiers, it’s important to note that they can be a breeding ground for bacteria like Pseudomonas. 

29. Do people with CF avoid anything?

 

30. Take the CF Straw Challenge!

Hold your nose with a straw in your mouth. Breathe through the straw for 1 minute. 

Not that hard right?

Keep going, your lungs will begin to strain, your head hurts, and your palms will sweat. Thankfully you can stop when it gets too hard to breathe. This is breathing with Cystic Fibrosis. 

How long did you last? 

I challenge you to donate that amount to the CF Foundation! 20 Minutes = $20 raised for a cure!

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31. How can we spread awareness?

  • Learn about Cystic Fibrosis and commit to spreading awareness!
  • Join a Great Strides Team, or check out all the other awesome fundraising events!
  • Interact with any CF posts you see on social media! It helps the reach of the post as all these platforms use interaction as a way to determine who the post is shown to, which in turn spreads more awareness!
  • Whether it’s virtual or in person, your support, understanding ear, and reassuring friendship makes every day easier to get through. Never second guess reaching out to the CF families in your life. Their struggle isn’t always visible, and your support can change their life! 

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Post Author: Mommy Khaos