Let’s Talk about CF

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Hello friends! As you all know our annual fundraiser is approaching and I thought this was a great time to blog about some things you may not know about CF and our daily lives.

Cystic fibrosis is an illness caused by a teeny tiny genetic mutation that causes a whole lot of problems. The mutation is a recessive gene so when both parents are carriers there is a 25% chance of their child having CF. There are no symptoms to being a carrier and you may be surprised that 1 in 20 people in the US are carriers and don’t know. The mutation in her genetic code causes her mucus to be thick and sticky. Mucus is present more places that one might think and helps your body in a lot of ways, unless it is thick, sticky mucus. Mucus lines the digestive track, is present in the lungs, and is present in the sinuses. Some of the problems that this causes are frequent lung infections, frequent sinus infections and sinus problems, and she is unable to digest food without enzymes taken orally with every meal. Anna also has some vitamin deficiencies because of malabsorbtion and she releases much more salt in her sweat that is not absorbed like most people. To remedy these issues Anna takes vitamins daily and has to stay extra hydrated. She still gives super salty kisses in the summer time but I love them :) Sometimes you can even see the salt crystallized on her skin and in her hair, it’s kind of crazy! We just make sure she’s always got a drink around and are very aware when she’s in a more risky environment. She has to take breaks from the sun and running around getting too sweaty but we don’t let it stop her too much, and at the beach we pack lots of gatorade!

Some other issues that come along with her not being able to fully digest are a lot of trips to the bathroom and stomach pain. We always have to be aware and make sure she isn’t experiencing any blockages in her intestine and she drinks miralax every other day and takes medicine to help with indigestion and gas.

Overall, Anna takes about 25 pills a day, 4 droppers of medicine, an antacid, and a vitamin. Those are the easy parts! She spends an hour a day doing her vest therapy that shakes her lungs loosening the mucus and the same amount of time doing breathing treatments. She does albuterol and hypertonic saline in her nebulizer. These treatments go in good spells and rough patches, both seem to last a few months.  In the beginning it was so easy! She was so little and the shaking would put her to sleep. She went through a major rough patch when the napping stopped and she just wanted to be free and mobile but now it is like clockwork unless she’s having a bad day… and then it’s meltdown inducing. But the meltdown can usually be cured with amazing gifts to moms like Paw Patrol, Wallykazam, and if its a major major meltdown Frozen will always do the trick. Limiting TV seems to be a motivating factor because our saying is “all done vest, all done shows” and at this point she’d probably do some extra vest time for extra TV time so she hasn’t put up much of a fight.

There are about 30,000 people in the US living with CF with about 1,000 new CFers born each year. Unfortunately, Anna can’t meet any of her Cystas and Fi-”bros” out there safely until there is a cure. Bad bacteria gets trapped in the lungs of CFers and at some point they are colonized and can’t be cleared from their lungs. These bacteria can easily pass from one CFer to the next, especially since they are prone to coughing. So far, Anna hasn’t cultured anything harmful and has only ever cultured normal staph which is very present in the environment and they only treat when it causes symptoms. One day I dream of Anna meeting her best friend and cysta Charlotte, while her mom and I look on crying tears of joy and hug each other as they hug.

We fund-raise because the truth is that CF is a horrible, ugly, selfish disease. Anna looks perfect but her insides are far from it. I don’t want Anna to ever suffer, I never want her to struggle to take a deep breath, I don’t want her sitting in a hospital bed, I don’t want her to have so many lung infections that eventually her lungs are scarred and losing function. But all of those things inevitably lie in our future without the researchers working so hard to find new therapies and a CURE for this disease and without the generosity, love, and support from people willing to fund that research.

Our way to cope is to find the positive. We are so blessed with a loving support system from family, extended family, friends, anyone who has read our story and been touched, anyone who has seen that beautiful blond with the curly hair and fallen in love. We get through every day by appreciating the time together, the healthy time, the silent nights where she doesn’t wake up coughing at all, watching her run at the park, appreciating this beautiful time and doing our part to ensure her healthy future.

When donating to the Cystic Fibrosis Foundation, 90 cents of every dollar goes directly toward research. This is a huge margin compared to other organizations and shows their dedication to finding a cure.

Bill Gates is a pretty cool guy. Not to mention a smart and generous one. He plans to do with his money as much as he can to make a real difference. The Cystic Fibrosis Foundation is one of the places he donates to because he knows that this is another disease that he can have a real hand in eradicating. A cure is truly on the horizon and you can be a real active part of curing a disease that has claimed the lives of far too many.

Here is a link to the drug pipeline for drugs currently being tested as cures and treatments for CF:

http://www.cff.org/treatments/Pipeline/

Kalydeco is already making a difference in the lives of the people with that specific mutation and many people are seeing a huge improvement in their lives. Unfortunately, Anna does not have this mutation but Ataluren, VX 809 +ivacaftor, and VX 661+ivacaftor, are all working their way through the pipeline now and target Anna’s mutation. Anna’s mutation is the most common, affecting around 53% of the CF population. Needless to say, researchers are working hard to find a cure for this mutation specifically and it is their top priority. You can also see the many new therapies they are working on that can improve the quality of life for CF patients and increase life expectancy.

Cystic fibrosis is an invisible disease, which makes fundraising a challenge. Look at the beautiful princess up there, she looks like the picture of health. But Anna and all of our family work really hard to keep her that way. We have been blessed with her health so far and we have nothing but hope for her future, but we have to fight for it just like we fight to keep her healthy every day. We have to do our part to raise funds that will one day give her the chance at a completely full, happy, and healthy life. Not to mention it will help me to stop embarrassing myself by crying my eyes out at every father-daughter dance at a wedding, heartbroken and feeling guilty for even letting myself think, “Will I see this?.” While she still wants to marry Daddy at this point, the day I see her dance with him in her wedding dress, breathing deeply, feeling wonderful, and looking so beautiful and happy, it will be the best day of my life.

Finally, I want to introduce you to someone very special. Her journey has inspired me beyond words. Her positivity gives me hope and lights my way in the darkest times. I am rooting with my whole heart for this girl who calls herself a “chronic badass.”  Her name is Caleigh Haber, she is 23 years old and from San Francisco. I’ve been following her journey on Facebook and you can too at Fight2Breathe https://www.facebook.com/fight2breathe.

Her website is fight2breathe.org. She is in the end stages of this disease and as of April 1st she was officially placed on the transplant list for a double lung transplant. She is fundraising for her transplant and I am hoping to donate a portion of the money raised by Anna’s Amazing Race to her fund. Please read her story and you will see why this tiny girl has given me big hope for Anna to live a positive and happy life and also some of the challenges that come with CF over time. Once Caleigh gets her new lungs, a cure will be more important than ever for her too. So get your teams together, spread the word to friends and family, and let’s have fun on race day and do our part to make a difference for Anna, Caleigh, Charlotte and all of their the cystas and fibros.

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March Check Up


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Even though we had a rocky winter with antibiotics and steroids on and off and many trips to the pediatrician, we STILL managed to make it the full 12 weeks in between Hopkins clinic appointments. I love the 12 weeks between because by the time the appointment is here we feel like we’ve had a small break and I’m usually ready to check in on her weight and get a culture so I can know what’s going on in that little body.

We had our clinic appointment last Monday and overall it was good. She didn’t gain as much weight as they would have liked to see her gain, but she is still at a healthy weight and they’re happy with that. She is increasing her enzymes and taking 4 pills with everything she eats and we will see if that helps her put on more weight and if she is still having issues in the next few weeks we might switch her from the enzyme she is using, Zenpep, to a different kind. We were surprised to hear that it was time for her annual blood work. I think there are some kids that you can try to distract and hope for the best when it comes to tests and that kind of thing but Anna definitely isn’t one of them. She is so fully aware and listening to everything that there is no getting anything past her! I decided the best course of action was to explain to all to her before it happened and hoped she understood. I had already done the same about getting her throat culture and she wasn’t happy about it but she didn’t cry which is the first time she hasn’t while getting it. Surprise seems to have a very negative affect on her. I explained that we were going to go into a room and they were going to give her a shot in her arm and take some blood out to test it and then she would be all done. I made sure to tell her how proud we are of her and how brave she is, and there may have been a mention of Mimi taking her to Target if she was big and brave ;)  She surprised all of us with how amazing she did! We went into the room, I held her on my lap and she was totally calm the whole time. We didn’t have to hold her down in any way, she just sat there. I asked her if she wanted to look away and look at me but she said no and just watched the entire process. She didn’t shed a single tear! Needless to say, we were amazed and SO proud!  She picked out Cinderella high heels at Target later that night :)

Her culture came back normal (hooray for no bad bugs!) and her blood work was a little low in Vitamin D. It’s common with CFers to have vitamin deficiencies which is why they test them annually to make sure their levels are good, but after this winter I’m pretty sure nearly everyone on the East coast is vitamin D deficient. Her vitamin D was around 24 and they want it to be around 33 so she is taking a gummy vitamin with her meds now everyday. She thinks it’s a fruit snack so it’s our easiest med by far. Our next appointment will be the week of Anna’s 3rd birthday in June. If we make it until then it will mark a full year of 12 weeks between appointments. So exciting! Anna is a princess and a ballerina, but most of all one tough little lady who is fighting CF (and her parents ;) ) with everything she’s got.

Now we’re looking forward to SPRING and Anna’s Amazing Race! Don’t forget to sign your teams up!!! We can’t wait see everyone on May 17th.

Here’s the link to sign up and donate:

http://www.mycommunityevents.com/common/eventDetail.aspx?id=25061

Here Comes the Sun…. IT HAS TO RIGHT???!!! SPRING.. PLEASE!!?


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Wow. This winter has been pretty brutal for nearly everyone I know. There’s been a lot of cold, a lot of snow, and a lot of illness. The past month and a half has been full of illness for this family.

Jack got a cold that he couldn’t get rid of, he’s having his adenoids assessed to see if we need to remove them. Anna fought her little butt off trying to get rid of her cold for weeks but she’s officially off of antibiotics after a month and so far she’s still at her baseline of zero cough. So hooray!!! And she is a beautiful little ballerina in her ballet bunnies class, she couldn’t love it more! However, as much of a princess as she has become she still loves to wrestle too :)

Surprising, I’ve been the sickest of us all, while also being the one never allowed to get sick!! I’m currently fighting bronchitis that turned into pneumonia so it’s my turn for heavy antibiotics, breathing treatments, and steroids. As hard as it is to take care of everyone I’d take it over Anna ANY day. Feeling my lungs compromised in this way makes it’s makes me so much more dedicated than ever knowing how painful coughing is to make sure that what I’m feeling now is NEVER EVER Anna’s day to day life.

Oh and did I mention we moved this weekend?!  We are loving our new house but we are very ready for some warmth and calm in our lives.

Like I said, it’s been a crazy winter! To say we are looking forward to Spring is an extreme understatement…. AND the most exciting part of spring is ANNA’S AMAZING RACE!!!!! Our talented coordinator Robert has been working hard getting his creative clues made up and we are on our way to a great event. All we need now is YOU! We promise to make this year better than ever, with better routing, really fun and challenging tasks, and ultimately an awesome and unique experience for a fantastic cause. Every year we are learning from the last to make things better and more fun. I am SO excited about this years tasks that I wish I could race myself!!!  Sadly, I know too much.  This is a competition after all!! But I’ll be in charge of a really fun task, if you’ve been there the past few years you know it’ll be a good one!

This year our focus is going to be on fun AND on reaching out. Here are some awesome ways to raise money for your team:

- Tell your friends! It’s even more fun competing against people you know. Ask your co-workers or friends to make their own team and you can even get some bragging rights once you win :)

-Send emails!!!! You’d be shocked how many people you can reach who haven’t even heard of CF, and you’ll also be shocked by some people you may not know who also have been personally affected by CF and are willing to join into our fight. And donation or not you’ve informed one more person and we appreciate that so deeply.

- Check into your company and any match program they may run. Over the past few years more and more companies have been joining in a company match program where they match an employees donation to a charitable cause.

- Have a party! Doing something fun like having a party with games, beer olympics, flip cup tournament, or games of some kind that require a pay in that you can dedicate to your teams donation. Can’t fundraise without FUN right?!

- If you work in a school or grocery store where you can sell flyers please let me know and I can get them to you! Great way to earn money for your team.

- Ask your family! Being charitable is something to be proud of any ANY amount of donation is priceless to us :) P.S – Easter is on it’s way, great time to see your family and you can always look for some money filled Easter eggs :)

- If you work somewhere that they allow you to put a collection jar out for donations, contact me! I will set this up for you and help in any way I can.

- Check into restaurants that are willing to donation portions of a night of sales to your team. A lot of restaurants will be willing to give you a night where a portion of proceeds go to our event, I know Friendly’s and Isaac’s are two that definitely participate.  AND come to our event at Mudhook Brewery in York on Wednesday May 7th! Our very own Robert will be guest bar tending  and 20% of your check will go to our event!

Raising money is our passion because we are funding research for a CURE. And it is on the horizon, it’s so close but yesterday wasn’t soon enough when you’re a mom watching your daughter struggle and be different because of mutation in her genetic code. Raising awareness is our second passion, putting a face to this disease that so many people have never heard of. So please, get out there! Get a team together!

Please share our story on social media, it’s such an amazing tool that can truly change our lives and giving Anna a chance at a TRULY healthy life, free of this debilitating and currently fatal disease. One day we can all say WE had a part in curing the most prominent genetic illness.

So let’s do this my amazing friends and family (this means you especially Musso’s ;) We want to see some old man Musso teams out there! ). If we can get teams signed up all throughout this month then you’ll have all of April to raise funds and reach out! My heart is full with the love everyone has shown our family if over the past two years and I want you to know this means the absolute world to us. Thank you all for being part of this journey with us.

Now let’s all forget this miserable winter, look forward to Spring, and  have some fun!!!

You can up your teams here:

http://mycommunityevents.com/common/eventDetail.aspx?id=25061

You can donate to our event here:

http://www.mycommunitytickets.net/common/eventDetail.aspx?id=25062

We will also be creating a paypal account to make it easy for paypal users to donate that we will have available in the next few days OR you can also send donations to our NEW address:

233 S Pitt Street

Carlisle, PA 17013

All donations are tax deductible using the tax ID code 13-1930701

If anyone has ANY questions about the registration or donation process please feel free to contact me anytime at kmmusso@gmail. com.

We are looking forward to an amazing event with great people! See you all very soon!

 

 

Potty Training – CF Edition

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A lot of the blog posts I write are to update friends and family on Anna and our family. When I started the blog it was to not only keep them informed but to help new CF parents looking for advice or wondering what day to day life is like since that is what I was searching for when I first got Anna’s diagnosis.  This is one of those posts that is probably not interesting to our friends and family, warning: some gross content to follow. BUT it’s a big part of our lives and something that I think can really help some CF parents looking for information. SO here is our potty training experience….

Cystic Fibrosis affects not only the lungs but also the digestive system. Her body is unable to break down and digest food naturally, which is why she takes enzymes with everything she eats. As she grows her enzyme dosage gets adjusted to do the best possible job for her but there are still always issues.  She has to eat as many calories as possible in order to gain weight because even with her enzymes she only takes in a certain amount of everything she eats. She also needs to stay hydrated since her body sweats out a higher concentration of salt. These things equal A LOT of trips to the bathroom. I was wondering how we were EVER going to potty train her…

We were very pleasantly surprised at how easy it ended up being! I think that because she’s always had belly issues she is already very aware of when she needed to go number 2. She started potty training right around her 2nd birthday and she was actually excited about it. Surprisingly, she was MUCH easier to potty train than Jack.  She very quickly only went number 2 on the potty.  For a while we had to have her little potty nearby at all times because of how frequently she has to go. It took her a little longer to get a hang of always going on the potty (again because she drinks so much and has to pee so often) but she is fully in big girl underwear now. She still wears a pull up most nights though because she doesn’t always wake up. The only issues we’ve run into so far are much more issues for us, and not for her. First of all, we’re trying to get her to only go in the big potty instead of her little one because clean up is not fun! CF parents will know that bowel movements are extra super gross. Secondly, we just have to adjust our routines to include a lot of potty time. We always have to make her try to go before we get in the car, and we have to make a lot more stops while driving. And most importantly I have to know where the bathroom is whenever we go anywhere outside of the house. We are now very acquainted with the bathrooms at every restaurant and store we’ve been to in the past few months.

The good thing is that she is really happy about it. I know she is proud of herself and happy to not be wearing diapers anymore.  Sometimes it’s exhausting and I almost miss the days of diapers when I didn’t have to rush anywhere or visit the bathroom 4 times during a dinner out at a restaurant.  But it makes me happy that we can make her a little more comfortable about going to the bathroom since it’s such a big part of her life. I always feel so helpless when her belly hurts but she is so calm now and proud going on the potty that I think making it a priority and getting her there is the least we can do. So to parents approaching potty training their CFer in the future: it’s possible! And to parents with a young potty trained CFer: I feel your pain! But one day this will be something she has to deal with on her own when she’s older and in school. Thinking about her having to deal with it on her own makes my heart ache, so I’m just happy to be able to be a part of it with her now. I want her to know I’m here for her, she’s not alone, and she’s not abnormal, and she’s not a burden, even on that 5th bathroom trip while shopping. In the future we’ll fight her digestion battles as they come but for now I think we’re very lucky with how she is gaining weight and how healthy she has been. We continue to be thankful for being so blessed every day!

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A Summer of Sun, Fun, and HEALTH!

It has been a tradition for many years now that we spend my birthday (July 2nd) and the week of the 4th at the beach.  This is the first year since Anna’s birth that we fulfilled the tradition. Two years ago we spent my birthday at Johns Hopkins getting Anna’s first dose of enzymes. Last year we spent the 4th of July in bed long before the fireworks. But this year we made it to the beach!

We haven’t traveled much since Anna was born but this time it was markedly less stressful than in the past. Just visiting my parents house an hour away has gotten my CF packing down to an efficient routine. It’s only gotten efficient because of a few mistakes though (hint: always check your fridge when packing meds because refrigerated antibiotics can cause extra hours of driving when left behind). Luckily we had no antibiotics to pack this time! I actually felt a lot of peace about packing because I knew anything I forget I can always get there as long as I have Anna’s medicines, vest, and nebulizer.

I made sure to consult with her doctors before our trip and they said that a trip to the beach is always a good thing because the salt water/air is therapeutic. One of the nebs so does twice a day is hypertonic saline which is pretty much just really really concentrated salt water so she was just getting a little extra dose :) We let Anna use a baby pool while on the beach just for the week and then got rid of it. CFer’s are told to stay away from pools like that because water can remain in it and bacteria like pseudomonas can grow. We asked the doctors and they made it clear that using a brand new pool just for the week was safe as long as it dried out outside every night. One of the important things we needed to keep in mind was keeping Anna cool on the beach, so the pool was helpful for that. We also used a big canopy to set up a shady camp, although she much preferred running around in the sun! We made sure to keep applying sunscreen so that she didn’t get too much sun, the doctors made sure to tell us to not allow her to get a sunburn. CF kids can easily get dehydrated since they sweat out a higher concentration of salt than other people. Because of this we had to make sure we were replacing the salt her body was losing so we kept her snacking on salty snacks like chips and pretzels and she drank nice cold Gatorade on the beach. Overall she didn’t have ANY health problems at all. She did great!

While we were on the beach I was definitely feeling the CF pressure a little bit and just making VERY sure she didn’t get dehydrated but in general CF was really not a major part of our vacation at all. We had such a fun time with family and friends, and my kids are total beach bums! They loved everything about it!

We loved it so much that we went 3 more times :)

We soaked up every minute of summer and were blessed with incredible health for Anna. She has been consistently gaining weight, baseline cough is still non-existent, and she is potty trained! So we had a major couple of months. She did a course of antibiotics a few weeks ago after she caught a little cold from her big brother being back to school. The antibiotics did their job and she cleared up over time. At her last clinic appointment she cultured a small amount of normal staph (normal meaning not MRSA or anything concerning) which is common and doesn’t cause any problems. We are still crossing our fingers and praying for these kind of culture results because we are still SO blessed that she hasn’t cultured anything bad, ever.

While we’ve been taking some time to just enjoy life, we haven’t taken any time off working toward a cure.  We are already planning next year’s Anna’s Amazing Race that we promise will be more amazing than ever! And we are lucky to have so many of our loved ones behind us who are consistently supporting our cause. Two of our friends ran the NYC marathon last week for Team Boomer raising $3,500 for the Cystic Fibrosis Foundation. And WebpageFX, Alex’s the most incredible and generous employer, donates year round using the Amazon Smile program and making a cure for CF one of their passions. So the fight continues! And in the meantime we are just purely thankful. We are thankful for everyone reading this, for everyone supporting our family and the search for a cure, and for this beautiful time in our lives with our babies.

Here are some pics of our summer and our SUPER Halloween!