Real Life with CF - a story of a father

http://www.thedailybeast.com/articles/2016/07/17/everlast-on-raising-a-kid-with-cystic-fibrosis.html

PARENTING 07.17.16 6:01 AM ET
Everlast on Having to Face Your Newborn Baby’s Mortality
The former House of Pain frontman on a complicated fatherhood.

Some days you really don't know how you're going to make it through.

I once was a carefree, self-centered, full-grown man-child. I started making records when I was 17 with Ice T. By 21, I had my first real taste of success with a band called House of Pain. At 28, I released my biggest record to date right after I suffered a torn aortic valve and had to have a lifesaving valve replacement surgery. (I now have a titanium valve so I’m sort of bionic, I guess.)
I never banked on being around very long after my surgery. I was pretty sure I might get five to ten years more out of life and I lived accordingly. Until my first daughter was born.
I know everyone says becoming a parent changes everything, and it does. But the change that came for my wife and I was not the kind we were expecting at all.

My oldest daughter was born with Cystic Fibrosis. I found this out when the obstetrician called saying how sorry he was and how terribly he felt—without his realizing that the hospital had not yet called to tell me. So being on the spot and having me ready to jump through the phone, he broke the news. I remember thinking, “I’ve heard of that,” but having no clue at all what cystic fibrosis was, so I immediately Googled it.
I remember the numb feeling that washed over me as I read about all the complications (diabetes and lung transplants) and the life expectancy (30 years, if you were lucky). I remember thinking, “Am I seriously contemplating my newborn’s mortality right now?”

How was I going to tell my wife? What words do I use to tell her that the 3 week-old baby she’s completely in love with has this life-threatening genetic disease? What I didn’t understand was she already knew something was very wrong. She felt it in her gut. When Laila was born she couldn't eat. She had some sort of blockage (meconium) that was actually common to Cystic Fibrosis, which I’ll refer to as CF from here.
They took Laila from the room and Lisa did not get to hold or feed her that night. She was devastated. They kept her for a few days in the hospital. When we got to bring her home, we hoped we were out of the woods but, as I said, my wife knew something wasn't right. A genetics test was done at the hospital in which they scraped Laila’s heel and used the DNA to test for all kinds of disorders. This was the test that told us Laila had CF. There was an additional “sweat test” to confirm the excess salts purged through perspiration.  When you kiss her skin you can taste the salt.

“Cystic fibrosis is an inherited disorder that causes severe damage to the lungs and digestive system. Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with cystic fibrosis, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.”
That's the Mayo Clinic’s short definition of CF. But what it really is is a total mind-fuck.

Hey, here's this beautiful baby girl and your only job is to protect her and keep her healthy even though no matter what you do, the disease is going to progress. Also, here's a long list of all the little ways you can fuck up and the test will be graded by your daughter’s health. All that you can do is hope to contain and slow down that process and pray that the scientists and doctors get this thing cured or treated faster than it can degrade her bodily functions.    
Because the fluids in Laila’s body are thicker, mucus gathers in the lungs and becomes a breeding ground for infection.

The most common one is pseudomonas, a bacteria that is basically anywhere moisture gathers. So it's everywhere and it’s harmless to those of us without CF. However, if a CF patient is exposed to it, it can colonize in the lungs and start the deterioration of those organs’ tissue.
Instantly, Lisa and I became germ freaks. We stopped having people over without a third-degree inquisition on how they felt and where they’ve been and what they've been exposed to, which didn't create the most welcoming environment for company.

We formed a bubble of protection around ourselves to minimize any chances of Laila being exposed to sickness or infection. We had to literally beat on our newborn baby’s back and chest several times a day to keep any mucus from building up. It wasn’t painful from what I could tell, but it was very jarring for her and, quite honestly, I did it through tears many nights terrified at the prospect of screwing up and doing anything to put her at risk. We didn’t go out to restaurants or movies. We stayed away from the park because God knows how many kids with colds were playing out there. We scrubbed everything furiously, washed our hands and sanitized like mad people.
If you did happen to clear the germ police security check and came around for a visit, then you also were slathered with sanitizer and had your shoes removed at the door. It was like TSA, only we were much more vigilant.
My wife's hands are cracked from washing and sanitizing all the equipment that came along with the treatments. We have four different nebulizers and a compression vest that shakes Laila’s lungs two or three times a day, each time taking around 45 minutes—and I haven't even begun to talk enzymes. Laila must take 30 enzymes a day. Every time she eats anything with any fat in it, she needs digestive enzymes to absorb the fats properly. We also load her food with extra fats—butter and cheese. And we can’t forget the salt.

Now, because CFers don't process fats properly, gaining and keeping weight on is a struggle. When Laila was turning four, she hadn't gained weight for many months and we were presented with the option of using a feeding tube to supplement her daily diet by a slow drip of protein shakes directly into the belly while she sleeps.
For a while we struggled trying to avoid the tube, feeling like it was a failure on our part that we couldn't get the job done. When we finally decided it was for the best, we realized how much pressure was put on her meal time and we understood it was time to not only worry about the quantity of her life; quality had to be as important if she was going to enjoy life at all.
I remember when we first got Laila home from the hospital after her feeding tube surgery. My wife and I were sitting in her room staring at the equipment and the button in her belly like, “Who's going to do this?” We were thrown to the wolves. No one had given us a lesson on how to connect or change the tube or told us how much to feed her during the night. A nurse had been scheduled to come to the house but not for several days. We sat and cried out of frustration and we did not tube feed her that night.  
The next day, we called the nurse. She was merciful and kind and came right over and showed us the ropes. We can now do this in our sleep and often do as it finishes in the middle of the night. What was terrifying yesterday is today’s routine. I’ll save the story of when we had to actually take the feeding tube out of her belly at home for another day.
We struggled hard with sending Laila to school. We knew she would get sick a lot more often and be at risk of pseudomonas and God knows what else. In the end, we decided she should be the kid she is. CF has already stolen too many things from her—from here on, childhood won't be one of them.

This year Laila turns seven. Her weight is still a struggle and a few of CF’s awesome symptoms are beginning to show their faces. We are still pretty isolated. We go to restaurants occasionally, though we wipe the entire place down when we get there. I’m sure people think we’re nuts.
Laila has struggled emotionally with all the rigors of CF and exhibits anger about it sometimes. There is nothing that has felt worse to me than to be helpless while your little girl just wants to know why she got stuck with the treatments and enzymes and hospital visits and four surgeries by 5 years-old.
Sometimes when I share some of these things online I get a lot of comments about how they don’t know how we do it. The truth is, you would more than likely do what we’ve done.  First you freak out then you start trying to figure out how to raise this child regardless of the circumstances. You’re not a hero for doing what you’re supposed to do.
Some days you really don’t know how you’re going to make it through but you get to work and before long the day is over and you've earned your rest. And you know it.

Nothing I have ever done or will ever do, will be more important to me than being a Dad.

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"65 Roses" is what some children with cystic fibrosis (CF) call their condition

because the words are much easier for them to pronounce.





Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in America in 1965 after learning that her three little boys had CF. Her duty was to call every civic club, social and service organisation seeking financial support for CF research. Mary's 4-year-old son, Richard, 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, Richard?"

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 Richard, I'm working for 65 Roses."
Since 1965, the term "65 Roses" has been used by children (and adults of all ages to describe this condition and has been adopted by many CF organisations all over the world as their brand. 

CHARITY - TEAM HUGO supports Cystic Fibrosis

What is Cystic Fibrosis?
Cystic fibrosis is a life-threatening, genetic disease that causes persistent lung infections and progressively limits the ability to breathe.
In people with CF, a defective gene causes a thick, 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.


The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for cystic fibrosis.

We are dedicated to attacking CF from every angle. Our focus is to support the development of new drugs to fight the disease, improve the quality of life for those with CF and -- ultimately -- to find a cure. 

TTON ABOVE!!



​TEAM HUGO


JOIN TEAM HUGO

GUARDIAN ANGEL RUN IN ZELL-AM-HAMERSBACH/ GERMANY - May 2017
supporting the University Klinik of Freiburg / Cystic Fibrosis department.
Team Hugo will participate in the first "Guardian Angel Run" in Zell-am-Hamersbach in Germany on May 21st 2016.
Join TEAM HUGO on our virtual run during the GUARDIAN ANGEL RUN in Zell-am-Hamersbach.
Thank you so much for your support!

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Some science info about CF:

Millions of people in the United States, Canada, and Europe carry a ticking time bomb in their cells — a mutated copy of a gene known as CFTR. If both mother and father possess the mutation, each of their children has a one in four chance of dying before age 30. A single copy of the mutated CFTR gene is present in one out of every 20 people of European origin. The 25 percent of those children who inherit two mutant copies get cystic fibrosis, a lethal disease that attacks the lungs. Until the 1950s, almost all such newborns died in early childhood.
Cystic fibrosis sufferers produce unusually salty sweat, a trait used to detect the disease. In the past, if a baby tasted salty when kissed, people knew the infant would die before its second birthday. Even today, when lung infections can be controlled with antibiotics, most victims of cystic fibrosis, 30,000 people in the United States, die before age 30.
Men with cystic fibrosis are usually sterile, and only recently have women with the disease been able to become pregnant.
This lethality and sterility present medical scientists with a mystery. Why does the mutation persist when, until quite recently, those who got the disease perished before passing it on? To survive the ruthless culling of evolution, the mutation must provide some advantage.

But what is it?
Researchers at Harvard University and their colleagues at the universities of Bristol and Cambridge in England have found a likely answer. “People with only one copy of the mutated gene apparently gain protection from infection by the bacterium that causes typhoid,” says Gerald Pier, professor of medicine at Harvard Medical School.
Typhoid comes from eating food or drinking water contaminated with Salmonella typhi, a bacterium common in places with poor sanitation. Carried into the gut with corrupted water or food, the bug gets into the intestinal wall, then moves into the bloodstream. People with one copy of the mutated CFTR gene gain protection against such infection.
In lungs, a protein produced by the CFTR gene binds to another bacterium, usually Pseudomonas aeruginosa, and causes the germ to be expelled by coughing, sneezing, or expectoration. But cystic fibrosis patients lack this protein and thus suffer infections that clog airways and destroy lung tissue.

The Good and Bad
The same type of benefit occurs in people of African descent who carry mutations in the gene responsible for making hemoglobin, a vital blood protein that carries oxygen. If genes from both parents are mutated, each offspring has a 25 percent chance of getting sickle-cell anemia, a painful, disabling disease that affects approximately 4,000 African-American babies born every year in the United States.
Those with only one mutated gene, however, gain resistance to the parasite that carries malaria. Such resistance gives blacks a big advantage in Africa, where malaria kills about one million children a year. However, this resistance is not so advantageous in this country, where the mosquito-borne disease is well controlled.