Heat Related Issues in Cystic Fibrosis

High temperatures present an unique challenge to people with cystic fibrosis (CF).When the body is subjected to higher temperatures, it responds by producing an artificial fever. The skin appears flushed or reddish because blood surges to the skin surface. A portion of the body's water content appears on the skin surface as sweat. The body's natural way to cool itself is to sweat. Salt is a natural component of sweat, but in people with CF, the amount of salt that gets secreted is abnormally high, because individuals with CF lose 10 times more salt through sweat than the normal individual. 

Because people with CF tend to secrete high concentrations of salt in the sweat, they are prone to electrolyte imbalance, dehydration, heat exhaustion and heat stroke if precautions aren't taken. Humid conditions pose an additional risk, because the moisture content in the air can interfere with the body's ability to produce sweat droplets.

The most common heat-related illnesses are heat exhaustion, heat cramps and heat-stroke. When the body cannot cool itself, any of these conditions can occur. Patients with CF are likely to experience heat exhaustion and heat cramps when the body loses fluid, salt (also called electrolytes), and other essential nutrients. Heat related illness is also more likely to occur in people who are deficient in vitamins and minerals such as potassium and magnesium.

Preventing these complications is simple. Be sure to drink plenty of water and consume salty snack. Many people with cystic fibrosis enjoy snacking on pretzels. They are a good source of salt, complex carbohydrates, and are easily digested because they contain no fat. Sports beverages are preferable since they contain electrolytes to replace what the body loses when sweating. Bananas are also a calorie-rich snack that contains the important electrolyte, Potassium. People with cystic fibrosis are encouraged to add extra salt to their meals during hotter months. Small amounts of table salt can be added to drinking water as well to help guard against heat-related illness. People with cystic fibrosis should be careful not to overexert themselves in warmer weather. They should also avoid foods and beverages that contain caffeine,natural diuretics, and alcohol. 

Heat exhaustion is dangerous to those with CF and can happen faster than you think. 

• Heat exhaustion is one part of the spectrum of heat-related illnesses that begin with heat cramps, progresses to heat exhaustion, and finally to heat stroke.
• The body cools itself by sweating allowing that sweat to evaporate. This requires enough fluid in the body to make sweat, air circulating across the skin, and low enough air humidity to allow that sweat to evaporate.
• Causes of heat exhaustion is activity in a hot environment can overwhelm the body's ability to cool itself, causing heat-related symptoms.
• Symptoms of heat exhaustion include 
  • profuse sweating
  • weakness, 
  • nausea, 
  • vomiting, 
  • headache, 
  • lightheadedness, dizziness, confusion, irritability 
  • muscle cramps.
• Heat exhaustion can progress to heat stroke when the body's temperature regulation fails. The the person develops a change in mental status, becomes confused, lethargic and may have a seizure, the skin stops sweating, and the body temperature may exceed 106 F (41 C ). This is a life-threatening condition and emergency medical attention is needed immediately.
• Treatment for heat exhaustion includes recognizing the symptoms, stopping the activity, and moving to a cooler environment. Rehydration with water or a sports drink is the cornerstone of treatment for heat exhaustion. 
• Complications of heat exhaustion include nausea, vomiting, dehydration and muscle weakness. If the activity is not stopped and the person left in a hot environment, there can be progression of symptoms to heat stroke, a life threatening emergency.
•  Heat exhaustion can be prevented by being aware of the your environment, especially on hot, humid days.

Severe heat-stroke may lead to death. As the blood from the brain flows to other parts of the body in an attempt to cool it down, the brain is unable to carry out normal function. No longer able to cool itself through sweating, the body temperature climbs as blood pressure drops. Victims of heat-stroke may collapse and pass out, never to regain consciousness. The body's central nervous system, spinal cord, brain and vital organs can be irreparably damaged. Every year emergency rooms are flooded with people who fall victim to heat stroke.

Lynsey's Make A Wish to The Grammy Awards

My baby girl before she leaves for The Grammys!

November 2014, when her wish was approved! 

Ever since she was diagnosed at five months old with Cystic Fibrosis, we have had a roller coaster of a life, with ups and downs, knock downs and moments where we felt like we were unable to breathe. After multiple times in the hospital, lots of surgeries, Ports and Picc lines, feeding tubes, and millions of breathing treatments and pills taken. After 14 years of fighting for her life against this disease and trying our best to let it take anything from her, the amazing Make a Wish organization has blessed her and us, beyond words, making her want to continue in the fight for her life! After long thought, Lynsey decided she want to attend the Grammy Awards. With only four in the country able to attend, we didn't think it was possible for her to be able to go, but In December 2015, I received a call that would change her life, she gets to go in 2016.... 🌟💛🌟💛🌟

Here we come.... 58th Grammy Awards 

Day one

Today we left for LA for a week to go on Lynsey's Make A Wish trip, which is to attend the famous 58th Grammy Awards! 

We arrived in LA, with beautiful sunny skies and 85 degree temperatures (in the teens back home) and stayed at Loews Hollywood Hotel on the famous Hollywood boulevard, with the infamous HOLLYWOOD sign within just miles and the walk of fame just a few steps from our hotel. We ate dinner at Hard Rock Cafe, which is next door to the Dolby Theater..... Which in itself is amazing, hosting The Oscars, which takes place in just a week, and the finale season of American Idol, which had a show while we were here!! Also across the street is the studio of late night talk show, Jimmy Kimmel!

We walked the walk of fame, where thousands of celebrities have earned a star.... And took lots of pictures with our favorites, and some legends that never die!

On the airplane headed for LA!

Hollywood sign

Kaylee and Lynsey walking down the walk of fame 

Lynsey and Jonathan with one of their favorites, Michael Jackson! 

Day Two

We started our day by driving up Hollywood Hills to see LA and the Hollywood sign from up close. I couldn't believe the gorgeous homes upon that hill! And unfortunately going up to the sign was against the law, so we were only able to enjoy it from a distance, in our pictures it seems far, but it's so close that we could walk to it, but oh well! 

Then, We met with our other three Make A Wish families which was Anna from Nebraska, Natajasha from New Jersey, and Isabella from Pittsburgh Pennsylvania, and also met with our Make A wish America granters Jen and Lauren, who have been doing this amazing opportunity for years, for hundreds of special kids. We ate lunch in downtown Los Angeles at The Yard House, which was really great food, I recommend it to anyone staying in LA, Lynsey and Jonathan LOVED the queso!

Then afterwards we all went around the corner and toured The Grammy Museum and saw years and years of music history!! Saw all the different Grammy award trophies and how they have changed over the last 58 years. Music sure has came a long way as well!!! 

Lynsey with the current Grammy award! 

Then we walked over to the Staples Center where the Grammys are taking place and took some pictures and saw all the hard work and long hours to get ready for such an event that only lasts a few hours!

After that we drove down to the West Coast to Santa Monica Beach and Pier, we saw upcoming musicians performing trying to catch their big break, rode rides on the famous pier walked on the beach and saw the sunset! The pier is also part of history, it is the end trail to famous road Route 66, which travels from Chicago to California!

Day Three

We again went to downtown LA where lots was taking place in preparation for the upcoming event and some Pre Grammy parties as well. Tons of celebrities at every turn. We started by eating lunch at WolfGang Puck, and all I can say is WOW!!!  While me and Lynsey were eating lunch, Chris, Kaylee and Jon hung out in the lobby of the Marriott Hotel where a Pre Gifting Grammy Party was taking place, Kaylee saw Joe Jonas and his new band, sat right beside of JoJo and had a 5 minute conversation with her about Joe and she didn't realize who she was talking to, until after the conversation, JoJo got up, hugged Joe and went into the party herself (Kaylee is still upset over that!!) She also uploaded a picture of Joe Jonas for his PR rep, without knowing until later!!! 

While we were eating, she's sending us pictures and texts saying what's all going on and we couldn't believe what surprise came afterwards.... We to got to go in to that same Pre Party!!!!! The girls were treated as celebrities, and received a huge bag full of free gifts!! There, we met Lindsey Shaw who played Paige from our favorite TV show Pretty Little Liars!!!! 

Anna, Natajasha, Lynsey and Isabella at gifting party! 

Then we went into the Staples Center and attended Grammy rehearsals!!!!

At rehearsals, we got to witness behind the scenes action, of what it takes to put together the show, and also saw the amazing celebrity performances rehearsing, such as Alabama Shakes, Little Big Town and Justin Bieber!!!!  We were all star struck, the girls went crazy over Justin!! Then we were asked to get up, and we got to go backstage, we had no idea what was about to happen, but WE GOT TO MEET JUSTIN BIEBER!!! Needless to say, the girls all went crazy, Lynsey received two hugs and two kisses from him, which I got pics and video of!!!  I have to admit, I myself was excited and Proud of how amazing he was to our girls, he just finished rehearsing his set  five times and then treated them with the upmost respect and honor!!! He spoke with them and took individual pictures and signed autographs!! He even met with all their parents! 

That was the BEST day by far, and the Grammys were still days away!!!! 

Day Four

Today we had a free day to do whatever we wished, so Lynsey decided she wanted to go to Universal Studios Hollywood, which was also included in her trip! But we just didn't go, we got the best tickets they had, front of the line to every attraction in the park!!! When Make A Wish does something, They go to the extreme to make all your wishes come true!!!

We toured the park in it's entirety!!! Rode rides, Harry Potter world and The Mummy were our favorites, also saw shows like, Water World, which featured an all star celebrity cast, it was very enjoyable to watch. We also took a tour and saw how all the movies and TV shows are made!! All of them are fake, it's totally crazy how they make everything seem so real with props, images and cameras! We saw sets of Jaws, Desperate House Wives and Psyco just to name a few. 

We even saw a woman get her arm cut into, a man catch on fire, but neither got injured!!! Totally crazy!! That's the magic of Hollywood!!! 

Day Five

Today was also a free day to do whatever  Lynsey wished. But do to Lynsey having extreme exhaustion and not feeling well we just hung out around Hollywood! We took a tour and saw celebrities homes, and went to Rodeo Drive in Beverly Hills.

 I mean who can come to LA and not go to Beverly Hills!!? Then afterwards we went to the pool to relax and Lynsey went and took a long needed nap! Later that night we went back to Rodeo Drive to walk around, I'm pretty sure we saw Katy Perry doing some late night shopping as she drove past us in disguise! We also went into the Beverly Wilshire Hotel, set of some famous movies, like Pretty Woman! 

Sitting on rodeo drive on Valentine's Day!

GRAMMY DAY

Today we had an early morning appointment for hair and makeup for our big night. We were remade by Paul Mitchell Salon, like I said when Make a Wish does something, they do it right!!!

Hair and makeup took two hours, so afterwards we went to eat a big breakfast at the hotel before we left for the awards since we wouldn't be eating until after the  show!! 

Then we dressed into our gowns and went down to our two stretched limousines awaiting to take us to the Red Carpet! As we arrived, they had security like I've never seen, I mean secret service, cameras under our limos and thousands of police to protect every person going to the show. We all felt like celebrities ourselves as we stepped out onto the red carpet, Grammy sign over head, huge Grammy trophy replicas, crystal chandeliers hanging and tons and I do mean tons like thousands of paparazzi and cameras. We saw celebrities getting interviewed, Red Carpet coverage being filmed!!!! The we arrived at the end of the red carpet and had reserved seating in the front three rows!!  There, the girls took at least, a hundred pictures, met celebrities, got autographs and even made blogs, celebrity's snapchats and TV appearances! It was beyond spectacular, better than anything that I have ever been apart of in my life, I was so proud and excited for my baby girl because all her dreams and wishes, for a fourteen year old was coming true and I got to witness it!! The only downfall, was she was an arms length away from her all time favorite, Taylor Swift, but still didn't get to meet her. She was opening for the Grammys so she had to be rushed off to get ready. Lynsey had an unforgettable night regardless, meeting absolutely everyone else that performed and presented an award; 40 autographs in total!!

Then the show opened with Taylor, we had spectacular seats, 6 rows from the floor and was right next to the entrance to go backstage! The show was magical with some incredible talent, like Carrie Underwood and Sam Hunt, Little Big Town, Lady Gaga, Chris Stapleton, Demi Lavato, Adele just to name a few!

After the show we went over to the LA Convention Center for an after Grammy Party! 

We entered a world of color, Candy Land themed, music playing, entertainers dancing and flying and bands playing and tons and tons of food and drinks, unlimited supply! And of course Jonathan wanted McDonald's, which was also available!!! 

The night had come to a close, 16 hours later we rode back in our private limo to our hotel reliving all that the day had brought!!!! Lynsey was extremely pleased and happy that she wished to come to the Grammys, it was an unbelievable experience that will last all of us a lifetime and give us memories that will never end! Make A Wish made it possible and to them I am forever grateful and will do anything to give back to this organization to help other kids who unfortunately have to suffer with sickness get their wish of a lifetime to come true as they did for Lynsey and Jonathan! It is also an unforgettable senior year that Kaylee will never forget herself!!

Cystic Fibrosis has given us all years of tears and heartbreak......but because of Make A Wish and their wonderful supporters, this week has brought extreme tears of joy and happiness that we can reflect back on for life! 

🌟 Make a Wish made all our wishes come true!!! 🌟

Family before leaving to go to the Grammys! 

Johnny Depp! 

Girls with Ariana Grande! 

Girls walking into the red carpet 

Carrie Underwood 

Demi Lavato 

With LA celebrity at after Party! 

Candy land theme after Grammys party!

Lauren and Jen.... Our wish granters! 

THANKS to everyone who made all of this possible and please help, Make a Wish grant more wishes to others by donating!!! 

My sweet Children

To Kaylee, Lynsey and Jonathan

As I sit here once again waiting for them to perform surgery on my child, I think about my role as a mom. My heart overflows with love, joy and heartbreak to the point I feel like it may explode! Words could never tell you how much love I have for you. 

 Sweet Child of Mine

I know you are so big now. You’re growing up faster than I could have ever imagined. In my mind, you went from 6 months to 16 years in about two weeks. It’s gone by so quickly for me, and even as you wish away your youth in exchange for budding independence, I can still see you as a baby in my arms. Oh it was not so long ago I would rock you to sleep. And now, the nights where we will be under the same roof are quickly slipping away. Dear God, how did that happen so fast?

But so many times, what I want to say to you in the heat of our “discussions”  is not what comes out. In my fear for your safety, for protecting you from every hurt I endured, every pain I see you walking straight toward, I come across as angry.

It seems to you that I just don’t understand.

Sweetheart, nothing could be further from the truth. I do understand. I understand completely.And what you need to understand is that my first job as a parent is not to win a popularity contest—it is to protect you. I am here to first stand guard. To keep you safe—from even yourself if necessary. If that makes me unpopular with you or your friends, oh well. Such is the price of being right. And I am willing to pay it.

Second, my job is to lead you. In this phase of your life, you may think you understand everything and everyone—but let me assure you—you do not. I see things you are blind to. I sense things you are clueless about. Dear sweet child, you must trust me. How many times have I been wrong before? I have nothing but your best interest at heart. So if I am leading you away from something or someone, you can trust that it is not because I am trying to ruin your life, but to save it.   And raise you the way I feel God intends me too..... With ultimate, unconditional love and sacrifice. I parent you the way he parents me, hoping one day you will love him just as much as he and I love you and he did die for you!!!!

Last, please remember this. There is no one else, not a single living soul on this earth, that loves you as much as I do. You shared my heartbeat. I’d do anything for you. I would give my life in exchange for yours any day of the week. If a doctor said you needed my heart to survive, I’d gladly give it. It’s with every cell of my being that I ask you to remember this when we argue. When you think I’m wrong. Or mean. Or stupid. Or that I don’t care or love you. I want you to know I’d give my last breath for you.

So don’t think for a minute that I’ll give up fighting the good fight. I win, because I love you. I fight because I’m fighting for what’s best for you. And I love you so fiercely that it’s crazy.

I love you my sweet child.

I LOVE LOVE LOVE LOVE you, 

Mom

Daily struggles of fighting CF

Why are CFers so tough.... Could you handle this daily schedule, plus school, homework, extra curricular activities!? 

Not to mention hours of surgeries, and months in the hospital a year! And I have two with this disease...... WE NEED A CURE, not more medicine!!

Daily medications:
Inhaled:
Dueoneb 4xday ( 9/1/5/9)
Pulmozyme 2xday with PEP (1pm/9pm)
Hypertonic saline 3x day (9am/1pm/5pm)
Tobramyacin 28mgx4 2xday( 8 pills day)
Advair 115/21 2xday
Cayston 75mg 3xday
Vancomycin 500mg 2xday
Dulera 2x day

Vest 3-4 xday ( 1pm/5pm/9pm) 40 min each 
Acapella 2x day (9am/9pm)

Po meds:
Bactrim 800/160mg 2xday
Prednisone 60 mg 2xday
Keflex 250mg 4x day
Viokase 20,000 units 12pills a day
Singulair
Bisacodyl once day
Loratdine
Ibuprofen 600mg
Sertraline 25mg
Vitamin D 1000u twice day
Adek vitamins 4 pills day
Benadryl 25mg 4 xday before 
Zenpep enzymes 6 tabs with all meals and snacks and tube feeds(30/day)
Flonase 2xday
Saline rinses 3-4xday
Bactroban with Pulmozyme and acetic acid solution
Miralax 2xday
Colace 1xday
Ondansteton 4 mg
Cyproheptadine 4mg 2/day
Tylenol with codeine every 4 hours pain

Feedings:
Two cal HN 5 can night
Pediasure w Fiber 5 cans night
2500-3000 calories day... More when sick

Glucose checks:
Morning, after meals, midnight thru Feedings.

Iv medications:
Vancomycin 2/day
Cefepime 3/day
Tobramycin
Linazolide every 6 hours

106 meds day, 9 hours IV meds, 8-9 hours Feedings at night, 2 hours of airway clearance, plus 2 hours of treatments. 

This picture shows only two weeks worth of meds!!! 

Change in the World

I’ve been thinking a lot about how I want to make a difference in the world. What’s a good way for me, Stacia to make a real difference. I dream of having a grand event planned for raising money for the Cystic Fibrosis Foundation. Where we have bands playing, kids activities, a gala where we all get dressed up, where tons of people come and participate and together we raise thousands of dollars to help in the aid of finding a cure for cystic fibrosis….. Or  I dream of having a grand CLEAN home, where my children and their friends and future grandchildren come to leave the cares of the world behind. I want to be smart, fun, forgiving, kind, helpful and loving. I want to create a place of happiness and love. I want to inspire people to be their best selves, the person the Lord created them to be. Today I learned that one of the best ways to help people was to help yourself become closer to the Lord. And through Him you can help others.

Trials suck. They are really hard…. and complicated. They make me doubt and fear and really stress out! My life is a hot mess. I can’t even imagine myself making a “difference” in the world when I’ve got  dried food on my shirt, dirty hair and yesterday’s smeared make-up on my face. I’ve got way too much on my plate, my TO-DO list seems to be growing my the nano second, a nursing job, raising three kids to be smart and honorable, and raising two kids with Cystic Fibrosis to make them want to fight for their lives, and being a forgiving wife, the way God wants me to be and trying to just make it thru the day without crying and all I can find the time to really do is ‘survive’ the day. If you can even call it that. How could little ol’ me possibly make a difference.

Endure Today.

Hug your babies, smile at a stranger, be kind when all you can think to do is scream, say the words ‘i love you’ out loud to the ones who need to know it, be happy, say a prayer.

These are simple things and yet they can change the world. Love can change the world. You don’t need to raise a million dollars for charity, or have a perfectly clean house, or be a successful….. whatever.

You are enough.

I’m grateful I got dressed today. I’m grateful I got to kiss my babies before They went to school. I’m grateful I smiled at the girl sitting in the doctors office. I'm glad I have a nursing job to make a difference in my residents last days. I’m grateful I get to talk my mom today and everyday other day and share my issues with her. Even though my accomplishments today were small and I have soooo much more I didn’t do or forgot to do, I did enough. I am enough. I can change the world. I have BIG dreams. Most of which will never come true, but that’s ok. You did ok. Keep going. Keep dreaming.

Endure today and love God.

He will always love you, and love can change the world.

First time using Metaneb

The MetaNeb® System

Three Therapies in One

The MetaNeb® System is indicated for the mobilization of secretions, lung expansion therapy, the treatment and prevention of pulmonary atelectasis, and has the ability to provide supplemental oxygen when used with compressed air.

The MetaTherapy® Treatment maximizes efficiency for patients and clinicians by combining lung expansion, secretion clearance and aerosol delivery into a single integrated therapy by the MetaNeb® System.

Conditions for MetaTherapy® Treatment. 

• Chronic obstructive pulmonary disease 3 (COPD)
• Post-operative airway management
• Bronchiectasis
• Neuromuscular disorders
• Cystic fibrosis
• Asthma
• Emphysema
• Reversal of atelectasis
• Chest wall trauma

Heartbroken mother

As I sit here once again to beeping machines, call bells, treatments and vests. My heart is breaking for a family that I never met, her name was Chloe and she's 13!! The same age as Lynsey and at the age of Jonathan she received a lung transplant because of CF. Today she gained her wings against the 13 year battle against this horrible disease. She passed away in her moms arms..... Looking on her moms FB page at the things she posted to her mom and how much love she has for her, will be there forever. 

I sit here broken but relieved and scared all at the same time. I wonder everyday.... When will be the day, that one of my children will inhale a bacteria or virus that may destroy their lungs. I sit here in the hospital beside my son who's fighting against a virus in his lungs and watching how strong he is against infection in his lungs trying to take over, I watch him battle against fevers of 104 and  I am relieved that he isn't worse. I hate this disease, it has no control or it doesn't play favorites. Every person that has it is strong, so why do some pass away so young and others don't!  No one understands the love and fear of seeing their child sick. But CF moms are faced with this disease every morning we wake up, no days off, no holidays... We in the back of our mind, with every cough, we think, is today that day?  So everyday I choose to love unconditionally with every breathe in me. I know God knows and it's all in his control. I just can't understand the pain I feel everyday seeing this disease take another life. A disease that my kids carry because I gave it to them. Life isn't fair. I just pray and try to live everyday to the fullest because none of us really know when our time has ended. So live life to its fullest. Hugs and love those around you!!!