CF Awareness: Answering Your Questions

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Thanks to everyone who submitted questions! You really got me thinking!

If you could give up one of your treatments/therapies/CF-related routines for a week, which one would it be?

I can only choose one? ūüėČ The treatment that I struggle with most is the breathing treatment and respiratory therapy that I do before bed, so I’d give that up for a week if I could do it without consequences. My morning treatment is actually harder because I’m far more congested first thing in the morning, but that one is so necessary for me to function that I never hesitate to do it. The instant gratification of being able to breathe and walk around without massive coughing fits is pretty motivating.

The evening treatment, on the other hand, is such a drag. I work four evenings during the week and my last students leave at either 8:30 or 9:00 pm. After that I’m pretty beat and I just want to relax, watch TV, spend time with Jaime, or go to bed. I hate having to initiate coughing and get my lungs all hot and bothered. Sometimes if I get coughing too hard, it’s difficult to stop and get to sleep.

If I miss the evening treatment, I still sleep just as well. But if I skip it, I have a harder time the next morning with more congestion and extra coughing. So there is a reward for doing it, but it’s not until the next morning. ¬†Honestly, sometimes I’m just too tired to care that I’m going to pay for it the next day.

Are there are new CF medications or treatments that might help your lung function?

I think a little background information is helpful in answering this question. As far as medications and treatments for CF, there are five main categories. The great news is that thanks to the Cystic Fibrosis Foundation, all five categories are under research and new drugs and therapies are being tested and developed!

The first category is nutritional. I currently take digestive enzymes and CF-tailored vitamins to meet my nutritional needs. Because I am stable in this area, I don’t think any of the medications under development would be of great help to me.¬†The next area is anti-infectives which include antibiotics. Most of the research is focusing on inhaled antibiotics which go straight to the lungs. Because of my significant asthma component, I cannot tolerate any of the inhaled antibiotics that are currently available, so I don’t have much hope that new ones will help me either.

The third category is anti-inflammatory drugs. I am particularly excited about this area of research because the asthma component of my CF causes a great deal of lung inflammation. There are currently five medications in the drug development pipeline. I truly hope one of them is a winner and will help me!¬†The fourth category is mucus clearance which includes medications to thin the mucus and devices to help us loosen the congestion and cough it out. I currently inhale saline solution and a mucus thinner and blow in a device that vibrates my lungs. My mucus is still quite thick, however, so I’m excited that there are five drugs in the development pipeline and hope that one will turn out to be more effective than what I currently take.

The fifth and most exciting category is drugs that restore CFTR function. The symptoms of CF are caused by a faulty protein which is known at the CF Transmembrane Conductance Regulator (CFTR). ¬†These drugs are designed to correct the defective protein. The defective protein inhibits sodium and chloride from moving in out and of the cells properly. There are many CF mutations and different types of mutations cause the protein to malfunction in different ways. There are currently two medications available to patients that help fix CFTR function. One medication has been particularly effective and patients who can take it have seen some significant gains in lung function and overall health. The second one has had more modest results but has still been very helpful to many patients. Due to my specific mutations, neither drug is effective for me (or available to me). I have one copy of the most common CF mutation and one rare mutation. Because I do have one common mutation there is still a good chance that there may soon be a drug in this category that will help me. I’m hoping!

If this topic sparked your interest, check out the Cystic Fibrosis Foundation’s Drug Development Pipeline¬†for more information.

Would you ever consider a lung transplant?

The short answer is yes, I would consider it. But at this time I don’t feel certain that I would decide to go for it. My CF care team has never broached the subject with me because thankfully, at this time, I am functioning well even with diseased lungs. However, I have read some about the process and “know” people through online support groups who have gone through transplantation.

My understanding is that survival rates for patients who receive lung transplants are rising, but lungs are still a difficult organ to transplant. According to the Cystic Fibrosis Foundation, 80% of patients who receive a lung transplant are still alive after one year, and about 50% are still alive after five years. ¬†The wonderful thing about transplant is that the new lungs will never have cystic fibrosis! However the rest of the body still does, so lung transplants are not cures. Also, transplantation can cause other problems. I’ve heard people say that when you have a transplant you trade one set of issues for another. First off, transplant surgery is a major operation and there are inherent risks there. As someone who has had two major surgeries and three minor ones, the thought of the surgery itself is more than a little frightening. Patients require 24-hour-a-day care for at least a month after the procedure and most centers require at least three family members or friends to sign up to be caretakers. It is also very expensive. It would be a lot to put my family through.

In addition, patients are always at risk for rejection, which is where the body attacks the new lungs as a foreign invader. Severe rejection can lead to lung failure. Because of this, transplant patients must take many strong medications to fight against rejection. Those medications can cause other complications such as cancer, recurrent infections, kidney infection, or diabetes.

I feel there are emotional implications as well. I haven’t studied this or talked to other transplant patients about it, but the thought of receiving the lungs of someone who has died a tragic death is a little hard for me. I wouldn’t feel right about praying for new lungs or hoping to get the transplant call just knowing that what I’m ultimately waiting for is someone’s untimely demise. ¬†That said, I can imagine that the gift of organ donation might be a positive thing for the grieving family, and that blessing someone else with life from that tragedy might be a healing experience. I haven’t worked it all out in my mind, but these are my preliminary thoughts.

I’ve just listed a lot of negatives about lung transplantation, but the whole reason I would consider it is that when it is successful, it can add wonderful, healthy years to people’s lives! What a gift that would be. ¬†Some lung transplant recipients go on to become athletes or climb mountains or do all sorts of things they could never even attempt before. That sounds pretty great to me. Also, there comes a point in most CF patient’s lives where transplant is the only remaining option for treatment. If that time comes for me, I feel confident that God will show me the path to take.

What’s one really hard thing about having CF that most people don’t know about?

Great question and a hard one!! I can’t decide between two things so I’m going to cheat and write about both. The first may better answer the “most people don’t know about” part of the question, and the second better answers the “really hard thing” part.

I hesitated to write about this at first because it’s kind of dark and depressing. But I’m trying to raise CF awareness, so here’s the honest truth.¬†The first thing that came to mind is moments of panic. I have some pretty specific fearful thoughts that come into my mind. I’m not talking about the more obvious fears of an uncertain future or fears of getting sick. These are different. Sometimes I imagine that I will need to flee from something or someone and¬†I can’t get away because I can’t breathe well enough to run fast. ¬†I wonder what would happen if¬†I was kidnapped or ended up imprisoned for some reason. How long would it take me to die a miserable death without access to my medications?

I have fears of being overpowered. I’m afraid of suffocating or being stuck in tight places. I’m scared of being buried alive in sand or snow. I can hardly stand to hang out in a blanket fort with Lucas or hide under the covers with him because after a few minutes, I feel smothered. I even hate to wear protective face masks because any sort of breathing obstruction makes me nervous and uncomfortable. I’m afraid I’ll need to rescue Lucas from something horrible and I won’t get to him in time. Now that my lung function is reduced, I don’t like having my face and head under water. I’m afraid of drowning.

When these thoughts or fears come to me during the day, it’s not hard for me to push them away and think about other things. But they also come in vivid dreams, and those can be downright terrifying. I have a recurring dream where I am unable to move, chained by some invisible force. There have been a few times in my life where I have been sick enough that I thought I was going to die for lack of breath during or after a bad coughing fit. It’s an awful feeling and I started having these fears and dreams after those experiences.

Even though I hate that part of the disease, it’s not as hard as other aspects because it’s not a constant problem. Which leads me to my second answer. I’m not sure people understand how often those of us with cystic fibrosis deal with change and loss, and how lonely that can make us feel. Because CF is a progressive disease, symptoms get worse over time. It may be obvious that we have to deal with more sick days, more coughing, more infections, and reduced lung function, but the resulting losses are more subtle. The sicker we become, the less we can do, and over time it feels like we are losing bits and pieces of ourselves along the way.

There are a whole host of things I did in my younger, healthier years that I can’t even attempt now. Some of them I have a wistful longing for–like being able to run the 8-mile race around the perimeter of Mackinac Island, or run down the side of a sand dune. ¬†Some of them are simple and seemingly trivial…like helping someone move furniture. But both were a part of my identity that I lost. Betsy the runner is gone. Betsy, that girl who’s surprisingly strong for her size and who will¬†pitch in and help with whatever–the one who enjoys a physical challenge–she’s gone too. That hurts. It’s really hard. Sometimes these changes happen slowly over time, and sometimes they come fast and furiously. At times I haven’t even had time to accept and adjust to my new limitations before I’m faced with a whole set to get used to.

These losses happen to everyone as they age, but when you have a disease like CF, it begins¬†far too early and only to you. Your peers aren’t going through it. When you’re facing these things together, there’s a camaraderie, maybe even humor to be found in the situation. But it’s just painful when you’re experiencing losses alone. Because of the dangers of sharing germs, cystic fibrosis patients are not permitted to mingle. There are no face to face meetings or support groups. ¬†Any support we seek has to be online. The online groups are wonderful, but it’s not the same as having a friend to share life with. ¬†It’s different to chat online than to talk face to face over coffee, or laugh (and therefore cough) during a playdate at the park with our kids. ¬†Sometimes I think it would feel really good just to get a hug from someone who really¬†understands. I am blessed with so many wonderful, true friends and a family that loves me and helps me to bear my burdens, but at times I still feel alone in my world of disease.

How is your Fitbit streak going?

It’s going great! Today will be my 300th day in a row of 10,000 steps or more (assuming I finish this post and get off the couch). I’m pretty proud of that! It has certainly taken resolve on my part, but I’ve found that it’s not that hard as long as I plan activity into every day. If I don’t have a dedicated portion of the day to go for a walk or exercise, I make sure to spend 10 minutes here and there walking around the dining room table or the playroom. If I haven’t gotten my steps before I start tutoring, I pace back and forth in my office between students or if they’re working on something independently. Those steps really add up and it’s heart healthy and lung healthy just to move!
This long streak was only possible because I had a very good winter health-wise. I did get sick twice, but both illnesses were (relatively) mild and I was able to shuffle around the table even on the worst days. I find that if I’m having a harder day health-wise, a reasonable amount of movement goes a long way in making me feel better and creating energy. This exercise goal has improved my stamina overall and I’m thankful for that! I know I’ll break the streak eventually, but hopefully not anytime soon. I’d love to reach a year!
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Thanks for sticking with me through this long post! If you didn’t get a chance to ask your question, or if my answers raised more questions, feel free to submit one now in the comment section or by email (bvbrighthope@gmail.com). I’d be happy to write another post or answer you directly. Thanks for reading!

CF awareness

Great Strides (Again)

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Over the years Betsy and I have participated in the Cystic Fibrosis Foundation’s Great Strides fundraising walk. It is held annually across the country during the month of May, coinciding with Cystic Fibrosis Awareness month. Last year, for the first time, some friends helped us organize a team to walk on behalf of Betsy. It was a chilly morning but such an encouragement to see so many friends and¬†family come together to support Betsy.

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Team Breath4Betsy 2016

We are walking as a team again this year. “Breathe4Betsy” was coined by one of my sisters a couple of years ago to use as a social media hashtag as a way to encourage Betsy before a particularly nerve wracking breathing test. Through a lack of other great ideas, we decided to recycle the name for our team. ¬†I’m glad the name has stuck. The name¬†is a reminder of how a simple thing such as taking a breath of fresh air is difficult for Betsy.

Of course the actual walk is only one component. Equally important is the fundraising that we are trying to do as a team. We set a team goal of $3500. Earlier this week we hit our fundraising target. We are thankful for everyone who donated to this important cause. There is so much promising research going on. The Cystic Fibrosis Foundation has a webpage dedicated to all of the medications in various stages of the development pipeline. None of these new treatments would be possible without research dollars. Every dollar that goes towards CF research provides Betsy and many others with the hope of a different life. When Betsy was born, the life expectancy of the average person with CF was around 18 years old. Now, a cure for the disease is within the realm of possibility.

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If you would be interested in partnering with us, we would love your support! You can donate to our team’s ongoing fundraising efforts¬†by clicking¬†here. It’s a pretty painless process. And every dollar really does make a difference!

Great Strides 2017 – Breathe4Betsy

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It’s that time of year again, Great Strides time! Great Strides is the Cystic Fibrosis Foundation’s largest national fundraising event. ¬†Here is an explanation from their website:

Each year, more than 125,000 people participate in hundreds of walks across the country to support the Foundation’s mission to cure cystic fibrosis and raise awareness for this rare, genetic, life-shortening disease that makes it difficult to breathe.

The CF Foundation is leading the way in innovative research and drug development, promoting high-quality, individualized care and helping people with CF live better todays. Nearly every CF drug was made possible by the Foundation and because of funds raised from Great Strides, people with CF are living longer, healthier lives and pursuing their dreams. But still, the lives of people with this disease are still cut far too short.

Great Strides provides a fantastic opportunity for family, friends, students and colleagues to come together to help make a difference in the lives of people with CF.

Together, let’s LACE UP. WALK. CURE CYSTIC FIBROSIS.

We had a great time walking with our teammates last year and we raised over $4000. Please join us in our efforts this year! ¬†If you’re local and can come to the event, it is being held on May 13th at 11:00am. ¬†This year’s location is at Millpond Park in Saline due to construction at the usual walk site. ¬†To join our team, please sign up here. If you’re not local, you can still¬†sign up for the team and raise money as a virtual walker.¬†If you would like to support my fundraising efforts, please visit¬†my personal page.

 

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Come take steps and make strides with us!

We have so much hope that new life-altering treatments and maybe even a cure will be discovered in my lifetime. There is a lot of positive momentum now and strides are truly being made.

We’re not there yet, but we’re closer than we were yesterday. And so we keep walking.

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Thank you all for your love and support!

The Chilliest Great Strides Walk of All Time

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I don’t know that for certain but I do know it was 36¬†degrees this morning when I woke up and not a whole lot warmer by the time we all met up for the Great Strides fundraising walk today. Come on people, it’s May for crying out loud!

Overall though, it was a great success! We had about 30 team members who braved the chilly weather to walk with Betsy and our team managed to raise over $4000 for cystic fibrosis research.

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(Most of) Our Great Strides Team!

We are very thankful for all of our friends and family who participated in one way or another. We are thankful for tangible expressions of solidarity and support. And we are thankful for the ongoing research that is working to develop new treatments for people with cystic fibrosis and hopefully, one day, a cure.

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Betsy, Lucas, and I rocking our pink #Breathe4Betsy headbands!

The Great Strides campaign is now done for 2016 but you can support cystic fibrosis research any time at the Cystic Fibrosis Foundation website!

Thanks again to everyone for their love and support!

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A few weeks ago, some dear friends of ours approached us about forming a team to walk in this year’s local Great Strides walk. They’ve named the team Breathe4Betsy and we are working to gain members and raise money for the walk!

Here is an explanation of Great Strides from the Cystic Fibrosis Foundation’s website:

Great Strides is the Cystic Fibrosis Foundation’s largest national fundraising event. Each year, more than 125,000 people participate in hundreds of walks across the country to support the Foundation’s mission to cure cystic fibrosis. The funds raised from Great Strides helps provide people with CF the opportunity to lead full, productive lives by funding research and drug development, promoting individualized treatment and ensuring access to high-quality, specialized care.

Great Strides continues to gain momentum, as do our research efforts and the progress we’ve made in the search for a cure. The CF Foundation has raised and invested hundreds of millions of dollars to support the development of new CF drugs and therapies.

Jaime and I have participated in this walk on and off over the years as individuals and are excited and honored by the formation of this team!¬† If you are local and you can join us, check out¬†Breathe4Betsy’s Team Page. We’d love to have you on the team. This year’s walk is Saturday, May 14th at 11:00am at Gallup Park in Ann Arbor. You can also join as a virtual walker if you want to raise funds but¬†can’t make the event. If you would like to support my fundraising efforts, please check out My Personal Page.

Because I love including¬†photos in my blog posts, I’ve added these pictures of Jaime, Lucas and I walking at Nichols Arboretum this past fall (see the connection?!) Gallup park should be equally beautiful in May! The Great Strides walk is a 5K though, so with that in mind, I’ll definitely be wearing different shoes.

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Lucas always takes us¬†off the beaten path…in more ways than one.

Thank you for your support! We hope and pray that CF will soon stand for “Cure Found.”

 

Who I Am

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It’s a sunny day here, but the brightness surrounding me cannot seem to penetrate my heart. My gloominess comes from the several nights of poor sleep that have accompanied my start of oral antibiotics and the abdominal and back pain that have been near-constant companions these past few days. Pain wears me out and getting less sleep than I need tends to shade my optimism.

Days of illness, even illnesses that are mild in the grand scheme of things, make my world close in. I haven’t left the house much in that last week. My normal routine has been disrupted by the presence of this infection. It’s hard not to focus on what’s not getting done and what’s not working correctly in my body.

Recently, I read a blog posted by the Cystic Fibrosis Foundation, written by a woman named Katharine Scrivener. ¬†One thing Katharine¬†said resonated with me. ¬†She said, “The older I get, the harder it is not to become my disease…[i]t’s a daily struggle to live my life outside of my disease–to not let it seep into each moment of my day.”

I couldn’t agree more. At one time, CF seemed to be just¬†a footnote in my life. ¬†Now, more often than not, it seems to be the headline, especially on days like today. I know I am much more than my disease, but the struggles of CF have seeped into so many moments of my day. There are times when I just want to forget all of it–the medications, the breathing treatments, the physical therapy, the health-preserving exercise, the worries, the uncertainties, the limitations, the pain. ¬†I want to cast it all aside and just live free. ¬†I want to be “me” untethered.

There was a time in my life where I had to make a conscious effort to accept CF as part of who I was since my main struggles started after I became an adult. I have done that, but now I feel it has become too much of me. ¬†That it is beginning to color too many aspects of¬†my life. To quote Katharine, that it is “chipping away at who I am.” It occupies too much space in my mind.

This post is partly a challenge to myself–a challenge to keep cystic fibrosis in its place. It’s something I’m still learning how to do–how to keep up with the demands of living with this disease, how to weather the illnesses, how to accept the limitations and push past the disappointments without becoming the disease. ¬†How to live outside of CF even while dealing with its implications.

Today I read Isaiah 43.  The first three verses are as follows:

But now, this is what the Lord says‚ÄĒ
    he who created you, Jacob,
    he who formed you, Israel:
“Do not fear, for I have redeemed you;
    I have summoned you by name; you are mine.
When you pass through the waters,
    I will be with you;
and when you pass through the rivers,
    they will not sweep over you.
When you walk through the fire,
    you will not be burned;
    the flames will not set you ablaze.
For I am the Lord your God,
¬†¬†¬†¬†the Holy One of Israel, your Savior.”

These verses¬†spoke to me today about my identity. Who am I? I am God’s child. He has summoned me by name and I belong to Him. It reminds me that when I pass through the waters, the rivers, the fires of this life and this disease, God will be with me, and I, the real “me,” will not be swept away. When I shift my gaze away from my circumstances and onto God, CF goes back to its rightful place. This disease is only one of the particulars of my life. It has in many ways shaped me, but it is not my identity.

Who I am? I’m Betsy–God’s child, a wife, a mother, a daughter, a friend, and a teacher. I am a joyful, loving, hopeful woman. And my CF? It’s there, but it’s just a footnote.

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A Crowded Corner

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I have happy things to report today! First an update on Operation Lace Up. ¬†Last night I reached my goal of running two miles without stopping! ¬†I consolidated the first five weeks of the Couch to 5K because I wanted¬†to complete the two-mile run before my follow-up pulmonary appointment. A good friend of mine came over and ran the two miles with me. ¬†It was the hottest day of the year so far (a high of 90 degrees) but we ran around 8:00pm and it felt surprisingly pleasant. I was able to complete the two miles in about 27.5 minutes (roughly a 13 minute 45 second per mile pace) which I was very pleased with! My friend just ran a marathon a few weeks ago at a much faster pace, so I’m pretty sure it was more of a stretch for me than her, but she was kind enough to break a sweat at least ūüėČ ¬†It was so great to have her support!

Today I had my pulmonary appointment. ¬†I’ve had a few moments of anxiety about this appointment the past few weeks. ¬†Even though I’m feeling great with low levels of congestion and (relatively) high levels of energy and activity, I was worried about that cantankerous FEV1 score. ¬†I was hoping to see a gain but my scores don’t always correlate with how I’m functioning. This morning I woke up feeling peaceful with a verse from the Psalms running on repeat through my head:

“She will have no fear of bad news, her heart is steadfast, trusting in the Lord. ¬†Her heart is secure, she will have no fear; in the end she will look in triumph on her foes.” Psalm 112:6-8.

What blessed assurance!

Then my phone started blowing up. ¬†At first I wasn’t sure what was happening. ¬†I got one text with a photo, than another, than another. ¬†You see, unbeknownst to me, Jaime had purchased some “breathe” bracelets from the Cystic Fibrosis Foundation and offered them to family and friends to wear as a show of support and solidarity. People started sending me pictures of themselves wearing the bands, saying they were thinking of me and praying for me this morning since it was my appointment day. I didn’t have any time to worry because I spent all morning overwhelmed and moved by the amazing show of love and support.

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My wrist is in the center surrounded by pictures sent to me today from family and friends wearing the breathe band.

I carried that joy with me into my appointment and scored a 42 on my FEV1! ¬†That is an eight point gain from five weeks ago and boy, I was thrilled. During my exam my lungs sounded clear, my oxygen saturation was normal, and my doctor was extremely pleased with the improvement in my symptoms and my increased exercise tolerance. ¬†I’ll go back in 6-8 weeks for another check. ¬†They will be monitoring me more closely as long as I’m taking the anti-fungal drug. I hope to see more healing take place in the next few months and hope to see my FEV1 get back to my baseline of 49 (or surpass it!).

I’ve mentioned before that a lot of good has come from the presence of cystic fibrosis in my life. ¬†Today was a day overflowing with blessings that come only from this struggle. Fighting a tough battle like I did this winter with my health and experiencing victory is a spectacular feeling. Setting and reaching reasonable exercise goals in spite of a compromised body feels fantastic too. Physically, running is hard and not enjoyable. But persevering through the challenge is exhilarating. I thank God for the strength He gives me to press on.

Being on the receiving end of extravagant love and support from family and friends is also an amazing, moving experience. Jaime¬†commented that I have a lot of people who love me and care for me and that are in my corner. And it’s true. I’ve been blessed with a wonderful family and many loving friends. In case you didn’t know, the phrase “in my corner” comes from boxing. The cornerman is a teammate or a coach who assists the fighter during the match. They stand back from the battle, in the corner of the ring, and help the boxer with the fight through coaching, advice, instruction and encouragement. They can’t throw punches or get right in the fray, but because of their proximity, they¬†provide invaluable help and support to the fighter.

It’s the same with my cystic fibrosis. I’m the only one who can actually fight my battle. No one else can do the dirty work for me. But my loved ones stand in close proximity to me and help me through my battles by¬†reaching out with love, with prayer, with help, and with the encouragement I need to keep up the good fight. I know for certain I could not do this alone. ¬†So thank you friends and family for being in my corner, in the ring with me, experiencing the bitter moments of frustration and defeat as well as the sweet moments of triumph and victory. ¬†Thank you being God’s dispensers of grace, and love, and strength. Thank you for crowding my corner.

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