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

Hair, Hair Everywhere

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Sometimes chronic conditions such as cystic fibrosis come with secondary issues. They can be directly related to the disease itself such as Cystic Fibrosis Related Diabetes. There can be damage to organs, bones, or veins due to ramifications of the disease, extended medication use, and the poking and prodding that becomes necessary to treat the condition.  Or there can be temporary secondary issues caused by drug side effects.

If you’ve been following along, you know that I was put on an anti-fungal drug to treat aspergillus (a mold growing in my lungs) as an effort to raise my lung function after a series of serious illnesses last winter and spring. I was warned that the drug came with a lengthy list of nasty side effects. ¬†A few of them were instantaneous–vision changes, photosensitivity, hallucinogenic dreams. They were not fun, but they stopped as soon as I was off the drug.¬†Recently my hair starting falling out, not en masse, but at a much, much higher rate than normal. This problem has persisted even though I’ve been off the medication for over a month. I talked to my doctor, and sure enough, hair loss is on that lengthy list. No one can tell me with confidence when this will stop. I have valid concerns about how much hair will actually be left on my head if it keeps exiting at this rate!

All this hair loss has made for an interesting several weeks at our house. When it first started falling out, I was constantly slapping the back of my arms, sure there were spiders or mosquitos eating me alive. This garnered a few interesting looks from Lucas who was wondering what on earth I was doing to myself.

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Sometimes when the light hits the carpet just right I’ll see a hairball…or two…or ten. ¬†I find hairballs stuck to our clothes. I’ve been pulling hair out of Lucas’s play doh. I’ve been picking it out of our¬†food. The other day at lunch, Lucas and I were playing the raise-your-hand-if-you-like-to-eat game. After a few normal items such as apples, tomatoes, cucumbers, he said, “Raise your hand if you like to eat hair!” Poor kid, he’s been eating a lot of hair lately. Neither of us raised our hand.

The hairballs on the carpet and in the laundry are bad, but they don’t hold a candle to my bathroom. ¬†I’m constantly picking hair out of the shower, out of the¬†sink, off the floor, and out of my brush. I looked in my trash¬†can the other day and did a double take. It looks like a family of furry little rodents have taken up residence in there!

I’ve been told the hair loss is not that noticeable. It looks and feels very different to me, and based on the current circumference of my ponytail, I’d estimate I’ve lost at least a third and maybe as much as half¬†of my hair. But I’m glad it’s not obvious to the rest of the world. I thought about getting my hair cut shorter in an attempt to make it look fuller, and give the new baby hairs (which I hope are already sprouting), a chance to catch up. But I have a little boy who loves my long hair. He comments on whether it’s down, or in a ponytail, or in a braid. He positions my pony tails and braids so they wrap around my neck. He might be heartbroken if I cut it. My big boy might be too.

I’m not enjoying this and¬†I certainly have had my moments of eye rolling and exasperated sighs. I don’t know anyone, male or female, who feels good about losing their hair. It’s one of the hard things about a chronic disease–all these additional annoyances. But I’ve learned that in instances like this, it’s important to keep perspective. After all, this is not a weighty issue such¬†as lung function and has no bearing on my survival. I can live just as well without the hair, and the hair loss should be temporary. People lose their hair for many reasons, and I’m thankful that this is¬†not a result something much worse, like cancer.

It isn’t always easy to keep a positive attitude. These types of issues, minor though they are in the grand scheme of things, feel like insult added to injury. The sum of many of these little insults¬†can seem unfair and can accumulate into a real burden if I focus on them. It’s easy for negative thoughts and attitudes to creep in…but it is precisely that which steals joy from my life. I cannot waste my energy worrying about the little things because I need that energy for dealing with the serious problems that come my way. Part of living victoriously in a battle with chronic illness is to push through the negative and focus on all the good, all the blessing, all that’s going right in my life–to have a joyful heart in spite of the aggravations that come along with this disease.

Even in this circumstance, I have many things to be thankful for. I’m grateful for strong medications that treat my disease and extend my life, even when they have negative side effects. I’m thankful for my¬†four-year-old who thinks it’s hilarious when I dance around trying to get imaginary spiders off my arms. He makes it a lot easier to choose a joyful attitude. I’m thankful for husband who will never stop loving me no matter how much hair is on my head.

What I’m most thankful for, is that I’m loved and cared for by my Heavenly Father. Matthew 10:30 says that God knows me so intimately, He even knows the exact number of hairs on my head. Knowing I am loved by God, that He understands every hurt and frustration, that He will never leave me and is using my light and momentary troubles to achieve eternal glory, that is where the true joy and peace comes from.

Hair, hair, everywhere…I don’t really need you, so I’m trying not to care ūüėČ

CF Suitcase

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It’s been a while since I’ve been by!  First an update. Things are going well! I’ve been feeling great and have kept up with my running. I’m still running two miles three times per week and am slowly trying to reduce my time.  My current best is 26 minutes 33 seconds which I got on the treadmill this week.  I was able to run outside several times due to cooler temps which was great!  Here were my last three outdoor runs:

  
I was intruiged that my time got better by 11 seconds each time. I’m hoping to get my time down to 25 minutes for the two miles. It’s going to take a few weeks but I think I can do it. 

I wear my breathe bracelet every time I run. It reminds me of all those loving people in my corner who give me support, encouragement, and help. It reminds me of Jaime and Lucas and the rest of my family who need me to be as healthy as I can be. It gives me a feeling of solidarity with all those CFers who work hard and struggle to stay healthy. It is motivation to push through the tough moments.

Summer has been great! Lucas is enjoying preschool and I like working during the day rather than in the evenings. Jaime has a break from soccer coaching for the next few months as well so we have more family time than usual.

We are heading out for vacation soon and today I got busy packing. I don’t know anyone that loves packing. Unpacking is even worse! For a CFer, it’s practically impossible to pack light. Since cystic fibrosis doesn’t take a vacation, there are lots of things I need. Take a look at the “extras” that I have to bring along to manage my disease:

  
I have my air compressor and nebulizer kits for breathing treatments, two inhaled medications, my anti-fungal drug, child-strength super sunscreen to combat the photosensitivity caused by the anti-fungal drug, a medication to reduce inflammation, two CF-specific vitamin supplements, digestive enzymes, a cooler for the medications that need refrigeration, two inhalers, an airway clearance device, and supplies for sinus rinses.

The orange and green bands are for my posture exercises. The body’s natural inclination is to roll the shoulders forward to combat shortness of breath, and these exercises are an attempt to keep proper posture in spite of my breathing issues. The jar is full of water kefir (a probiotic drink) which helps me manage the stomach issues associated with CF. (My friend Joyce taught me how to make it and gave me a starter. Click here to see her recipe for water kefir if you’re interested!) I drink a little with each meal and it helps my digestion.

You may notice that I included workout gear and shoes with my “extras.” That’s because as much as I might want a break, I can’t afford to take a week off from exercising if I want to keep my lungs clear. Thankfully the resort where we’re staying has a very nice workout facility and therefore I have no excuses!

CF requires its own suitcase. But I’m grateful that my gear is portable so I can enjoy some time away with my family. I’m all packed…now to survive the nine hour drive with a three and a half year old.  Thank goodness for iPads and orange tiger headphones!