My most recent discovery on nutrition related to CF came from a couple brown bananas that I had in my kitchen. I was debating what to do with them when I remembered a recipe I’d come across for banana cookies. So I looked it up in my bookmarks, and as I was reading over the ingredients, I noticed it called for coconut oil. I haven’t used coconut oil before so I read through the comments to see if anyone else asked about it. Sure enough, there were lots of comments on it - including a link to this article about the health benefits of coconut oil. Reading through it I was surprised to see that cystic fibrosis was listed as one of the diseases that can benefit from the oil.

It turns out that coconut oil has long been recognized for antifungal, antibiotic properties. Additionally, the fats in coconut oil are structured in such a way that they are fairly easy to break down, making it a great source of fat and calories for CF patients who have trouble with fat digestion. The article above goes into more detail on the numerous health advantages of coconut oil (and coconut milk, which contains coconut oil). The site also has another article on potential ways to incorporate coconut oil and milk into your diet, including mixing it into teas and smoothies, using it for baking, and Thai and Indian curries. Sounds pretty tasty to me!

I baked these cookies last week and was quite pleased with the results. They were pretty simple to make, and they have so many nutrition advantages: low carb/sugar, good protein from the almond meal, and of course the coconut oil. The hardest part for me was finding all the ingredients, because some are a bit unusual. I found almond meal at Trader Joe’s, and bought the coconut oil at Whole Foods. If you can’t find almond meal you can always grind up almonds in a food processor, and the recipe states that you can substitute olive oil for the coconut oil. If you do, you might want to add a splash of coconut milk (which is easier to find, usually in the ethnic section of grocery stores) to add in some of the nutritional benefits. I did not add the shredded coconut, and when the cookies first came out I thought they could use some more sweetness. Next time I might either add the shredded coconut or a bit of brown sugar (I have a sweet tooth), but the fact that they don’t have sugar added makes them great for diabetics. I also was happy to discover that the flavor improved after a day or two in the fridge. My husband was pretty happy with them too - they didn’t last very long!

Here’s the recipe (from 101 Cookbooks, a great site with healthy recipes):

3 large, ripe bananas, well mashed (about 1 1/2 cups)
1 teaspoon vanilla extract
1/4 cup coconut oil, warmed up just a bit so it isn’t solid (or alternately, olive oil)
2 cups rolled oats
2/3 cup almond meal
1/3 cup coconut, finely shredded & unsweetened
1/2 teaspoon cinnamon
1/2 teaspoon fine grain sea salt (I used regular salt)
1 teaspoon baking powder
6 - 7 ounces chocolate chips or dark chocolate bar chopped

Preheat oven to 350 degrees, racks in the top third.

In a large bowl combine the bananas, vanilla extract, and coconut oil. Set aside. In another bowl whisk together the oats, almond meal, shredded coconut, cinnamon, salt, and baking powder. Add the dry ingredients to the wet ingredients and stir until combined. Fold in the chocolate chunks/chips.The dough is a bit looser than a standard cookie dough, don’t worry about it. Drop dollops of the dough, each about 2 teaspoons in size, an inch apart, onto a parchment (or Silpat) lined baking sheet (I just greased regular baking sheets with a tiny bit of the coconut oil). Bake for 12 - 14 minutes.

Makes about 3 dozen bite-sized cookies.

Brad maintains that keeping himself in top physical condition has helped him stay healthy enough that he can be active on baseball teams and with his family. His doctor agrees:

Marshall says physical conditioning “is one of the keys to combating CF and other chronic lung diseases. It’s part of what we preach.”

This agrees with a trend I’ve noticed where exercise is cited as the best therapy for CF patients. Push yourself to the limits and who knows how far you will go!

This month’s edition of Nature Reviews in Drug Discovery features a review of new treatments for cystic fibrosis that are in the process of undergoing clinical trials. The review is short, but it is exciting to see these drugs highlighted in a prestigious scientific publication like Nature. Some interesting content includes the future perspective on the therapies that will be most prevalent for treating CF over the next ten years, and a chart of the biggest drug development programs, shown below:

Some of these have been highlighted in previous posts (the Vertex drugs, Denufosol). Another drug featured in the review that seems quite promising is Bronchitol. This drug is an inhaled dry powder that encourages the flow of water across the lung surface in order to reduce stickiness of the mucus. The dry powder formulation offers several advantages: it can penetrate the lower airways more effectively than nebulized products (like Pulmozyme), and it is easy and convenient for patients to use as no nebulizer is necessary. The drug is currently in Phase III of testing, and the article predicts that it will have a large impact on future CF treatment if approved. The convenience of the dry powder means it could potentially reduce the burden of nebulized medications and inhaled antibiotics, and therefore has significant potential to positively affect quality of life for CF patients.

Some other drugs mentioned in the article include Moli1901, a drug designed for regulating salt transport (like Denufosol), and two antibiotics - Aztreonam and a dry powder formulation of tobramycin. Many of these drugs are anticipated to be available in 2009. More products available for treating CF means potential for better lung function and longer lives!

Reference: Storey S and Wald G. Novel agents in cystic fibrosis. Nature Reviews Drug Discovery: 2008 Jul;7(7):555-6.

Recently, my husband and I celebrated our first wedding anniversary. We were definitely ready to celebrate making it through our first year of marriage. It was our hardest year so far. It was also our best year so far. During that year we had some periods where we went through some pretty rough patches. There were moments when we lost hope.

Experiencing those times without hope made me more certain that hope is essential. I spend a lot of time reading up on new treatments for CF. When reading those studies, I frequently come across reminders of just how serious the disease is. But I have to hold onto hope, focusing on the progress that comes through, instead of letting fear hold me back.

I clearly remember the day that fear first caught hold of me. It was a sunny summer day and I was visiting the guy I’d been dating at college before we both graduated and moved to opposite ends of the country. We tried breaking it off because of the distance, but it didn’t last. Instead we ended up talking all the time and eventually visiting each other. We were sitting by a lake on a Sunday afternoon after one of those visits. I was basking in the sunshine and the warm glow of this excited feeling that there was something real there between us, that maybe we didn’t have to break things off, and that maybe I had finally found my special someone. He was sitting quietly next to me, and then suddenly said he had to tell me something. I was taken aback by how serious he sounded. “Sounds serious,” I said hesitantly. He told me that it was serious. A thought flickered through my head - what if he was sick? Like seriously sick? But I dismissed it with the naive thought that those things don’t happen to people I know. Then he told me he had a disease called cystic fibrosis.

That moment changed everything in my life. I had breezed through it pretty easily without any major problems: I had a happy childhood, got through my teenage years without too much angst, had a great time in college while maintaining good grades. My friends and family were healthy and happy as well. I hadn’t found love in the romantic sense yet, but I was hoping I would soon, and this guy seemed like he had potential. But suddenly he also had CF. He told me what it meant: lung problems, digestive problems, reproductive problems, shortened life span. It was a lot to process. I tried not to cry but a few tears escaped. He told me not to worry, that he was fine, for now anyway.

That night, before I left, I asked him about our relationship, and if he wanted to try making it an official long distance thing. I had never wanted to do a long distance relationship. And I had never anticipated a complication like the one I had just learned. I was pretty terrified by the whole situation. But somehow I was more scared of losing him. So I chose hope. I decided to risk the pain of long distance dating, and risk dealing with the health issues that were ahead of us, because deep down I knew I had found someone special.

I have never regretted that decision I made on that night. Looking back over the time we had dating each other, and especially the past year of marriage, I’m just more certain I made the right choice by choosing hope. The choice has let me experience so much joy. The past year as I said was our hardest year. But it was also our best year so far. We find happiness in each other every day.

I don’t know what’s in our future. I don’t know where we’ll be in 5, 10, 15, 20 years. Maybe we’ll have a brood of kids and our lives will be jam-packed every minute of every day filled with rambunctious laughter and yelling and energy. Maybe it will be just the two of us, alternating between travels to exotic places and quiet evenings in reading a book and enjoying each other’s presence. I do know what I want out of life: laughter and love and friendship and fond memories and knowing I made a difference, somehow, to someone. I already know that by having my husband in my life, I can achieve those things, because I’ve already experienced them during this first year with him. I can’t wait to experience more of the same in the years ahead of us.

Please never forget all the possibilities that we have in life. That is why I hope, so that I never forget those possibilities. We should remember those possibilities every day. I hope, so that I can most enjoy the laughter and love and friendship we experience every day.

I cannot live
I can’t breathe
unless you do this with me
and here we go, life’s waiting to begin.
~”the adventure”, angels & airwaves

This positive announcement of another treatment for CF gives even more evidence that all the funding going towards CF research is paying off. Hopefully further exciting results will come from the additional studies on this drug, and the others in the pipeline!

You can read the full press release from Inspire Pharmaceuticals here.

Another way to promote CF Awareness month is to pass information along to friends and family who may not know anything about CF. You could try forwarding a website or handing out pamphlets from your local CFF chapter. If you’re looking for some ways to help people realize why it’s important to know about CF, here are some reasons why people should care, even if they don’t know anyone with CF.

The fact that CFers are living longer and having kids means the disease will become increasingly visible in our society in the future. You might not know someone now, but you could someday. Current data estimate that one out of every 29 Caucasians, one out of 46 Hispanics, one out of 65 African Americans and one out of 90 Asians are carriers of a defective CF gene. So even if you don’t know anyone with CF, there’s a good chance that you know several people who are carriers, although they don’t know it. These carrier rates will likely increase as more CF patients have children. If two parents both are carriers of a defective CF gene, they have a 25% chance that their child will have CF. For all of these reasons, it is becoming more and more likely that one day, someone you know will be affected by CF.

Despite the fact that this disease has historically affected a very small percentage of the population, the approach taken by advocates for CF has resulted in many great advancements in treatment that have enhanced both the lifespan and quality of life of those affected by the disease. These advancements in medical treatment over past two decades, highlighted in this article, are a good example of science and medicine at work, fighting a disease and making real progress. The amount of progress has been inspirational to me as a scientist. Who doesn’t love seeing results?

Hopefully even those who do not know anyone personally who has CF will appreciate these facts. Pass them along and help promote CF Awareness!

Some more ideas:

• If you are already signed up to participate, recruit one more team member to your team. Each additional member can potentially increase the amount raised
• Throw a fundraising party for your team. Even if your walk was last weekend, you can usually continue fundraising for several weeks, so have a celebratory party to increase your team’s contribution. Check with the Great Strides website or your local CFF chapter for details on how to organize a party.
• If you can’t participate this year, make a donation - every little bit helps! Then, pass this information on to friends and family, who may be able to make donations too.

The national goal for this year’s Great Strides campaign is to raise $40 million for the CFF. Remember why we fundraise - every amount that can go to research can bring us closer to new, better treatments for CF.

What’s an activity that can be lots of fun and involves breathing exercises? Singing! If you like music, you might want to consider taking a voice class or participating in a local chorus group or church choir. Breathing control is a big part of singing, and some studies have shown that trained singers have higher lung volumes and better lung function than normal. This may not come as a surprise, but an article in the NY Times last month discusses some benefits of singing that made me think of potential benefits to CF patients, not just because of lung function, but because of the effects on fat levels in the body.

The article, found here, discusses several studies that have found that benefits to singing, including:

• stronger chest-wall muscles, which helps with breathing
• more efficient heart pumping, which helps the body distribute oxygen
• maintenance of lung capacity that normally declines with age
• respiratory improvements even after a short period of singing training

One surprise, though, was the effect of singing on leptin, a hormone that regulates fat storage in the body. It seems that singers have an increased tendency to be resistant to leptin, which can increase their tendency to gain weight (think about the stereotype of opera singers: “it’s not over to the fat lady sings!”). The article mentioned this as a downside, but when I was thinking about CF, this could be a good thing! Since many CF patients struggle with maintaining weight, if singing really does increase fat storage in the body, this might be helpful for those looking to put on weight.

Now, this is just my own hypothesis, but regardless of the theories on fat storage, it seems clear that singing can be very helpful to lung function. That alone means it would be a good activity for CF patients to consider. Of course, it would not be a replacement for airway clearance, cardiovascular exercise, and other treatments, but it never hurts to include fun activities that can improve lung health!

Previously I mentioned the importance of increasing awareness for CF with regards to politics. Since it is a fairly rare disease, it can be easy for patients with CF to be overlooked in the health care system and in political arenas that control funding for medical research. Well recently, the Senate officially recognized May 2008 as National Cystic Fibrosis Awareness Month.

It is very exciting to have CF be acknowledged this way. May has traditionally been a month for CF awareness because of Great Strides, the biggest fundraiser by the CFF, occurs in many cities through out the country during May. Being granted official recognition by Congress means that CF Awareness is in fact increasing, and that the government recognizes the importance of increasing education on the disease.

Adding in this new success of recognizing CF to all the recent advancements in medication and therapies, and the success of fundraising organizations like the CFF, I am excited and hopeful that great progress for cystic fibrosis will be in our future!

Pulmozyme is the first drug that was specifically marketed for treating cystic fibrosis. It was approved by the FDA in 1994 and has been a common treatment for many CF patients. Pulmozyme is a mucolytic medication, which means that it reduces the ’stickiness’ of the mucus in lungs.

How it works

The thick, sticky mucus that forms in the lungs of CF patients is the main contributors to infection. Bacteria thrive in this environment, and because the mucus is sticky, it is hard for CF patients to remove the bacteria by the body’s normal mechanism of coughing. As more bacteria grow, the body’s immune system sends cells to attack the infections. This results in fragments of cell parts that accumulate in the lungs, further increasing the stickiness of the mucus.

A main component of the cell parts is strands of DNA that are inside the nucleus of every cell. Studies have shown that bacteria like P. aeruginosa like to attach to these DNA strands. Pulmozyme works by breaking apart these DNA strands. This can improve lung function by reducing the stickiness of the mucus in the lungs and by removing the attachment sites that many bacteria adhere to. Clinical studies over a 6 month period have shown that mild to moderate CF patients who took Pulmozyme daily had reduced risk of lung infection and an average of 5% increase in lung function (FEV1).

Medication Administration

Pulmozyme is taken with a nebulizer. Patients inhale a mist of the medication. Time of treatment and dosages vary with individual patients, but usually this treatment should be performed daily.

Who can take it?

Pulmozyme is recommended for patients with an FEV1 of 40% or higher predicted. It is not yet recommended for children under 5 years of age, although additional studies are looking at potential for use in infants and young children. It’s also important to remember that not everyone will respond to the drug: while the results of the clinical trials showed an average increase in lung function, some patients will show no difference.

Further information:
Pulmozyme website