CYSTIC FIBROSIS AWARENESS
This summer, 7-17, Carl and I were blessed with our first grandchild, Linkin Ronald Vietzke. He was born
with Cystic Fibrosis. His brother Liam was born 11-01, a year later, and was also diagnosed with CF. We
were not aware that the CF gene ran in our families and we were ALL shocked to find out that mom
AND dad had it. When both boys were born, they were immediately tested and found
positive for the gene. Thanks to this early testing though, mom and dad were able to begin treatments
immediately and start down the path of care.
Linkin and Liam look and act like any "normal" child. The difference is that they are not and for their entire
lives, they will face challenges that will alter how those around them will perceive them. Instead of thinking
about future weddings, grandchildren and the long range future, mom, dad and grandparents are hoping to get
to tomorrow. We are hoping to see kindergarten, a school play, elementary, high school, sports games,
prom, college. We cross our fingers for a future and do everything in our power to make it happen.
Unless someone is told that the boys have CF, it will be assumed that they can go swimming in a pond, eat an ice cream cone on a whim, sit around the camp fire and roast marshmallows, and so much more. But, that will not be the case. Smoke and bacteria can wreak havoc on their lungs. It could send them to the hospital for weeks. If they don't take their enzymes before eating, they could have major issues with digestive functions. There's no spontaneity without planning. There's no taking chances without first thinking about every consequence. But we choose to look at CF, not so much as a problem but more like we have to do life a bit differently. There are many things Linkin and Liam will NOT be able to do as they grow older, but there are still thousands of things that they can. And as time moves forward and the Cystic Fibrosis researchers learn more, maybe someday life won't include the word different at all. Maybe some day, life won't have to include the term Cystic Fibrosis at all...except in our history books.
(The following information was duplicated with permission from the Cystic Fibrosis Foundation website. Links are provided. Additional information and how to donate is found on their website at Cystic Fibrosis Foundation or their Facebook page.)
Cystic fibrosis is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time.
In people with CF, a defective gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.
People with CF can have a variety of symptoms, including:
Very salty-tasting skin
Persistent coughing, at times with phlegm
Frequent lung infections including pneumonia or bronchitis
Wheezing or shortness of breath
Poor growth or weight gain in spite of a good appetite
Frequent greasy, bulky stools or difficulty with bowel movements
Cystic fibrosis is a complex disease and the types and severity of symptoms can differ widely from person to person. Many different factors, such as age of diagnosis, can affect an individual's health and the course of the disease.
People with cystic fibrosis are at greater risk of getting lung infections because thick, sticky mucus builds up in their lungs, allowing germs to thrive and multiply. Lung infections, caused mostly by bacteria, are a serious and chronic problem for many people living with the disease. Minimizing contact with germs is a top concern for people with CF.
The buildup of mucus in the pancreas can also stop the absorption of food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.
Breakthrough treatments have added years to the lives of people with cystic fibrosis. Today the median predicted survival age is close to 40. This is a dramatic improvement from the 1950s, when a child with CF rarely lived long enough to attend elementary school.
Because of tremendous advancements in research and care, many people with CF are living long enough to realize their dreams of attending college, pursuing careers, getting married, and having kids.
While there has been significant progress in treating this disease, there is still no cure and too many lives are cut far too short.
The type and severity of CF symptoms can differ widely from person to person. Therefore, although treatment plans can contain many of the same elements, they are tailored to each individual's unique circumstances.
People with CF need to work closely with their medical professionals and families to create individualized treatment plans.
The CF Foundation accredits more than 120 care centers that are staffed by dedicated healthcare professionals who provide expert CF care and specialized disease management.
Each day, people with CF complete a combination of the following therapies:
Airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs. Some airway clearance techniques require help from family members, friends, or respiratory therapists. Many people with CF use an inflatable vest that vibrates the chest at a high frequency to help loosen and thin mucus.
Inhaled medicines to open the airways or thin the mucus. These are liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer. These medicines include antibiotics to fight lung infections and therapies to help keep the airways clear.
Pancreatic enzyme supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF also usually take multivitamins.
The CF Foundation supports research to discover and develop new CF treatments and maintains a pipeline of potential therapies that target the disease from every angle.
In 2015, the United States Food and Drug Administration approved the second drug to treat the root cause of cystic fibrosis, a defective protein known as CFTR. The first drug targeting the basic genetic defect in CF was approved in 2012. The arrival of this group of drugs, called CFTR modulators, signals a historic breakthrough in how CF is treated. It's expected that CFTR modulators could add decades of life for some people with CF.
Today, the Foundation is focused on developing lifesaving new therapies for larger numbers of people with CF and pursuing daring, new opportunities to one day develop a lifelong cure.
CLINICAL TRIAL RESOURCES and INFORMATION
If you are a person with CF or a family member, and you are looking for clinical trials or other information
on CF or one of the many other life changing issues, you can find information to help you at
WITH POWER. Power is the only place on the internet where patients can access clinical trials directly.
We help more patients find treatments and speed up the process of medical science as we go.
A DAY IN LIAM and LINKIN'S LIFE
The boys have a routine they must do EVERY DAY. When they wake up, the
first things we do are breathing treatments. When they were young, we did what I call
the "poundings". For the first year, we "pound" on their back and chest to help loosen
the mucus in the lungs to keep them clear. They also use an inhaler for medication (like
an asthma bottle), a nebulizer with medication and, of course the "cup" for loosening
the mucus. As they get older, around 1 year, they use a vest. The machine they use to
loosen the mucus costs $20,000.00, but it is a much needed piece of equipment that makes
life a gazillion times easier for the parents and child. And we know that it does a
way better job than we can do.
Then, we start the day with breakfast. They boys' bodies cannot break down food
because the pancreas enzymes don't get to the stomach to break it down in order for the
body to use them. They take a drug called Creon before EVERY meal in order to be able
to ingest and use the nutrients in the food. Ok, that sucks you say, but at least they can
take drugs. Well, that bottle costs $200 each. And you cannot get a regular monthly
prescription because how can you determine how many times a person will eat?
They only last for so long. What if they take them and the food isn't there or
something comes up to delay his meal? Then he has to take more. What
happens when he goes to a relatives? What happens if he takes a trip on a plane
and his medicine is packed in a bag that is held over for 3 days? What happens
if he can't get a refill where he's at? What if insurance doesn't cover it? These
and many other questions and concerns are tossed around in the back
of the minds of a CF person and family continually. And thank Pete for the ACA.
Without that we would have limits on insurance payouts as well as pre-existing conditions
and skyrocketing insurance premiums. Many people say so what and why should I pay
for things I'll never need Why should I care if you pay more as you use more? No one asked
for genetic mutations. No one wanted their children to have life threatening problems, but
it happens. We need some damn compassion and education in this country. But that's another
rant for later.
Before bedtime, and sometimes twice additional for a total of four times (or more) a day,
the boys have to repeat the first step of the day. Why? Bacteria and viruses.
The lungs have a thick mucus in them. Ours is thin so when the bad stuff
gets in, we cough it out. The boys' lungs cannot do that. Because the mucus is so thick,
EVERYTHING gets caught in it. It is not uncommon for CF people to be hospitalized several weeks
out of the year. Without proper care, without financial help, the family can be devastated and yes,
the CF person can die. That's the horrible reality and one we have to deal with daily. It's sad that,
because of this reality, many families with CF children lose friends AND family members because
those people are afraid of what they don't know or they think CF families are being mean
because they cannot do what everyone else can or things have to be done differently. Instead of trying to understand and be supportive,
people pass judgement and accusations. In the end, it hurts, but, we don't have time to worry about their misunderstandings
in regards to CF. (Obviously I have experienced this personally.) All we can do is help to educate others and, hopefully, their
attitudes will change. If not, we're better off without them. Life is already a challenge.
THIS SITE IS NOT AFFILIATED WITH THE CYSTIC FIBROSIS FOUNDATION.
This is my own personal site, considered a third party site, and I am a proud sponsor of the foundation. Some of the information on this page has been used with permission from the Cystic Fibrosis Foundation and all appropriate links are provided in bold, blue, underlined text. The donation button will take you directly to the Cystic Fibrosis Foundation's website where you can donate directly to them.