Sound Off Saturday: Treatments and Vacation

Being a holiday weekend, we couldn't help but think of a great Sound-off Saturday topic: Vacation and Treatments.

When we're not in the routine of work (which is really just on vacation or during holiday weekends), treatments are the one part of our routine that must stay the same - which isn't always easy while trying to enjoy a break from our normal day-to-day routine. Obviously, when we travel, go on vacation, or have a "staycation" during holidays, treatments are still a must. For some reason, vacations are the only time that I really notice treatments. When we're in our normal routine, I never really notice Ronnie's first 2-3 treatments during the day. He does them back in his office, while we both work in separate rooms, so it doesn't affect my day, routine, plans, etc. The night treatment (which Ronnie always does right before bed) I notice more because I normally wait up for him to finish so that we can fall asleep together - maybe silly, but I just really love going to be together. I hate the thought of Ronnie crawling into bed and not having his wife say, "Goodnight" and "I love you" right before falling asleep. But all in all, on a typical day, treatments go unnoticed by me and they don't affect my daily life and routine.

But the reality of 3-4 treatments a day sets in when we're on vacation. And I will be honest, I am not the biggest fan of all the treatment time when we're on vacation. Often times we'll have to push off breakfast, come back mid-day regardless of what we're doing, or rearrange plans to make sure that we get all of the treatments in, while still getting out and enjoying vacation. I start to feel even more aggravated (maybe a harsh word, I can't find the right one. Annoyed? Perturbed?) when we're with other people (friends or family), and I feel like we're holding up the whole group because we have to get treatments in. And I'll be honest, I always feel a little guilty on vacation because I get annoyed with the treatment schedule, but then I realize that it is only my reality for a week, while it's Ronnie's reality every day. And that really puts into perspective that me feeling inconvenienced is silly and selfish.

ANNND now that I've let you into my horrible, inner-most thoughts, let me now give you the flip-side and what I constantly remind myself when these nasty little thoughts creep in. The rational side of me knows that the treatments are necessary...and maybe even more necessary because we're generally thrown off our workout routine as well - so we're not working his lungs with as much exercise either. And whenever the thoughts start to arise of, "Maybe he could just do 2 treatments today?" or "How about he just does 3 instead of 4?" I always step back and remind myself of one thing: I'd rather get home from vacation and have him healthy, than get an extra two hours a day of "fun," only to get home and have him feeling like crud; having to climb and uphill battle to get feeling good again.

I feel a little bad verbalizing my not-so-positive thoughts about treatments on vacation, but I know that I'm not alone in my thinking. Sticking to treatments during vacation (or anytime your routine is thrown off) is simply just not easy. But I always find that when I really step back and think about it, doing the treatments is far more awesome and beneficial than anything we could do with the two extra hours we'd have if Ronnie skipped his treatments. So this holiday weekend, Ronnie will be getting in all of his treatments, but you better believe we'll be packing the other 22 hours of the day with TONS of fun!!

Ahhhhh yes, treatments and vacation, my ultimate double edged sword...

So which side of the sword should I describe first? Let's go with the side that cuts some of my fun time out of vacations. Now, let's be clear, I'm the first to admit that I'm not Mr. Vacation and rarely am I the one chomping at the bit to go on one. We'll get to the reason I'm that way when we get back to the other side of the sword, but for now let's stick to the side that cuts me away from what I enjoy about vacations. Generally when I'm away and enjoying some other place I'm around family. That has to be the number one thing that I like about vacations. It's nice to be able to be around family 24/7 and the fact that you have the option to go out and "explore" with them is a bonus. One bummer about treatments and vacation is that it takes some of the spontaneity out of just "getting up and going". Things need to be more mapped out to make sure I have the time to get in my 3-4 treatments for the day. Whether it's 3 or 4 usually depends on the altitude, weather and how I handled the flight to where I was going. That's the thing with this CF life - there's really no "getting away" which is the biggest reason why most people want to go on a vacation in the first place.

Alright, enough of Debbie Downer. Since only about 7% of me feels the way I was talking about above, let me get to what the rest of the 93% of me thinks; the other side of the sword if you will. I love treatments on vacation because they feel like they restore some order or a sense of routine while away from home. I know that I've mentioned this before, but I am a routine guy. If my routine is thrown off, my whole day seems thrown off. It's especially that way if I miss my morning treatment - which probably happens 1 out of every 365 days. On the rare occasion that I do miss that first treatment, or heaven forbid, my coffee, you can pretty much count me "off" for the rest of the day. I usually like to go all out on vacation and pack in as much stuff as we can, so it's nice to have 3-4 times throughout the day that I can recharge my battery.

Another thing I think about while doing treatments on vacation is the more I consistently take care of myself, the better chance I will have to enjoy more vacations down the road with my family. Plus, being at the top of my game while on vacation gives me the energy to "keep up" with the rest of my family as they are doing the "vacation thing". I've always found that if I get out of whack with my treatments on vacation, not only do I not feel as good during the activities, but that it's hard to get back into rhythm when I get back home. You guys know how it works - one missed treatment leads to two, leads to 4, leads to a week, leads to a month. I don't know about you, but I can't afford to even miss 2 treatments in a row. I figure the longer I can keep up my treatment routine, the longer I'll have to feel good while on vacation. Does that all make sense?

Anyway, we thought it'd be fun to bring Sound Off Saturday back. Keep in mind that we just threw out a general topic to each other and then agreed to "sound off" on it. I have no clue what Mandi wrote and she has no clue what I wrote. The first time we read it will be the same time that you do.

Now it's your turn...sound off!!!

Dear Parents...

I saw this blog over on CysticLife and wanted to share it with all of you...

I can not imagine what went through your minds and hearts when your child was diagnosed with CF. I have always felt i should help parents by giving them some tips. I am not a parent with a child who has CF but i am a wife of someones child who has CF.

From the second I met my husband i knew he was special. We were in highschool and every once in a while he would miss school because he was sick. I never knew the severety of CF because it didn't define him. He was very smart, determined, had a rock band, was drum line captain and worked. It wasn't until we got serious that his disease ever came up. His parents have always had high expectations of him and his abilities and never allowed him to be a victim.

I know as a parent you may feel helpless at times and that there is nothing you can do. The best thing you can do for your child is treat them the same way you would if they didn't have a disease. I can not thank his parents enough for raising him this way and helping me to pick up and follow their lead. The reality is it sucks at times and you can get sad or pissed at the world, but then get over it. It won't change anything. There are some things you can't control, but your attitude towards them is 100% in your control.

I want you all to know you have the opportunity to raise a child who can inspire many and experience a full life. My husband has gotten to go to college, get married, have a successful career and now we are attempting to have children. I understand not all kids are as fortunate as my husband has been, but give them the tools and strength to have a chance. I hope to one day have my own children and I will teach them how to live their life and never feel like a victim.

Have hope in your childs future. My husband is 28 and we will celebrate 5 years of no hospitilization in February. There is no cure but there are great doctors and treatments. Giving your children strength and determination to deal with the life they were given is the best thing you can do for them.

Thank you Cheryl for writing such a powerful blog. I thoroughly enjoyed reading it.

Good ol' Family Fun...

It's not Monday - but it's Mandi here..

Ronnie and I saw Despicable Me, starring Steve Carell, with some friends last night. We were going to see Knight and Day, but Chrissy, my sister in law, had her little sister (not real little sister, but little sister through big brother, big sister), so we decided to go to a kid-friendly movie so they could join us. BEST.DECISION.EVER!! It is an adorable movie, perfect for kids, hilarious for adults. We laughed quite a bit through it and I absolutely fell in love with a little girl in it. Steve Carell did a GREAT job. You could barely tell that it was him. I wanted to share a clip, to hopefully convince you to spend your hard-earned money on some good ol' family fun!

**Please note, the little brunette is my FAVORITE character. I wish she was real, because I would adopt her!

Enjoy!

Teens and Cystic Fibrosis

I found this post online and thought I would share. What do you guys think, does he make some good points?

Infants born and diagnosed early with cystic fibrosis are now growing up, going through puberty, and eventually, most are becoming mature adults with families of their own. The teen years are a challenge both to the parents and the teens. Teens are beginning to assert their independence, trying to be grown up, and rebellion is a stage most teens will go through.

Teenagers feel awkward and uncertain and often lose confidence in their abilities and skills. They may feel frustrated at the drugs they need to take or the time spent having physiotherapy. They may want to pretend they are normal when they are out with their friends. They may not eat healthy, take their digestive enzymes, and teenagers often start smoking at this age. Rebellion may be a perfectly normal part of going through puberty but teenagers with rebellious habits could lead to their death.

Start when your children are small to help them understand their disease. Talk about it openly and encourage them to talk about it. Help them to be educated about cystic fibrosis. You can encourage them to be normal and still impress on them the urgency of keeping up with their treatments. Teens are easily embarrassed but if they have a good self-esteem it will be easier for them to talk about their disease with their friends. If you find your teenager is not taking care of their health, have started smoking, or are not taking their medications get them in to their doctor or a good psychologist.

Teenagers can make it through this difficult time in their life. They need a good support system that should include parents, teachers, spiritual leaders, and a peer support group. If they are comfortable enough to talk about their disease and explain it to their circle of friends, they should be better about having treatments and taking medications.

You can help your teenager by offering healthy meals, being able to listen to them and understand their frustration and fear. They should be treated normally and a parent should not become overprotective. Parents can be a tremendous help to their teen by allowing them the freedom to make decisions, fall down, get hurt, and learn from their mistakes. You should step in if they are neglecting their health and not taking their medications, as they should.

Encourage your teen to be frank with their friends. To other people it may seem strange to take a handful of pills before each meal or snack; for cystic fibrosis teens it is a necessity.

Complications for teens are nearly the same as for a younger child. The danger is they will take unnecessary chances and forget to take care of their health. Repeated infections that linger may cause serious damage to their lungs. There is a danger of a lung collapsing, coughing up blood, and even stroke or heart damage. Encourage your teen to take care of their health especially if they have the potential life-threatening disease called cystic fibrosis.

Posted by David Hobson

Original article can be found at http://www.pressdistribution.net/8159/helping-teens-with-cystic-fibrosis

The Kitchen- Kinda Before, Kinda After

Acapella vs Flutter

vs.

I often get questions on which form of therapy I feel works best for CFers. My answer is pretty much always the same, "Different strokes for different folks". Now, I myself prefer hand pounds, but that doesn't work for everybody. I ran across this article comparing the performance of the acapella vs the flutter and wanted to share it with you guys. It basically says that performance wise there is no difference, but the acapella is easier to use. My question for you guys though would be different. Did you feel that either of these worked for you in terms of moving mucus up and out? And if you have done both, which do you prefer??

Performance comparison of two oscillating positive expiratory pressure devices: Acapella versus Flutter.

Volsko TA, DiFiore J, Chatburn RL.

University Hospitals of Cleveland, Case Western Reserve University, Ohio, USA. tvolsko@advancedhealthsystems.com.

BACKGROUND: Oscillatory positive expiratory pressure (PEP) with the Flutter device facilitates secretion removal. In the Flutter a steel ball vibrates inside a cone, causing air flow vibration. A new device, the Acapella, uses a counterweighted plug and magnet to create air flow oscillation....We hypothesized that the Acapella and Flutter would produce similar mean PEP, oscillatory pressure amplitude, and frequency over a clinically relevant range of flows. METHODS: We measured oscillatory amplitude, PEP, and frequency. Values for frequency, peak, trough, and mean pressure were recorded automatically every 3 seconds at flows of 5, 10, 15, 20, 25, and 30 L/min. The pressure waveform for 1 second was also graphically displayed and recorded. The devices were adjusted to give low, medium, and high mean expiratory pressure (Flutter angle at 0, 20, and 40 degrees; Acapella by dial setting)...RESULTS: There were statistically significant differences between the devices for mean pressure, pressure amplitude, and frequency, for all experimental conditions. However, the differences were relatively small and may not be clinically important. Both devices produced similar pressure waveforms at the medium flows. At 5 L/min the Acapella produced a more stable waveform, with a lower frequency, higher amplitude, and a slightly wider range of PEP than the Flutter. CONCLUSIONS: Acapella and Flutter have similar performance characteristics. Acapella's performance is not gravity-dependent (ie, dependent on device orientation) and may be easier to use for some patients, particularly at low expiratory flows.

In the interest of full disclosure, I have never done the acapella and the last time I did the flutter, I threw it against the wall and it broken into a handful of pieces. Oh, and I guess I better mention that I neither represent nor do I have any monetary interest in either company that provide these devices. Stupid blog laws.

Runner with CF Finishes Marathon

Irvine runner with Cystic Fibrosis finishes L.A. Marathon

Aliso Viejo resident Tyler Polasek from Crean Lutheran South in Irvine took another big step by finishing theLos Angeles Marathon over the weekend.

"Tyler has Cystic Fibrosis, nevertheless, he ran an incredible time and accomplished something that many never thought would be possible," said Crean Lutheran cross country coach Tim Brink.

"Even with this condition he was still able to push through the pain and training and finish in a very respectable time of 3:11.20. Tyler plans on continuing to improve his marathon time and eventually phase into triathlons."

Original article can be found at http://www.ocregister.com/news/ammann-241001-coach-game.html

Eastern European CF Statistics

Call for European Cystic Fibrosis healthcare gap to be closed

A healthcare gap amounting to a ‘death sentence’ forCystic Fibrosis (CF) children born in Eastern Europe must be closed say researchers from the EuroCareCF Coordination Action for Cystic Fibrosis. A new study led by the University of Dundee and published today in The Lancet, has found that CF patients in Eastern European countries die far younger than in other, wealthier, EU countries.

EuroCareCF, led by Dr David Sheppard of the University of Bristol, provided a forum for groups from all over Europe to collaborate to improve the quality of life and life expectancy of individuals with CF. As part of this collaboration, Dr Anil Mehta of the University of Dundee led a team from 35 countries that examined outcomes for almost 30,000 CF sufferers born in long-standing European Union member states compared to those born in countries who joined after expansion in 2003. The resulting paper shows that despite similar population sizes and underlying gene frequencies for CF, the numbers of CF-affected children were lower in post-expansion EU member states. Dr Mehta says this disparity can most likely be explained by the tragic fact that a lack of healthcare facilities in new member states means that the majority of children born with CF in these countries will die in very early childhood, a situation not encountered in the wealthier EU countries for many decades. “We know that this disease occurs randomly in about 1 in 4,000 children born to healthy parents across the EU,” he said. “Despite this, the team encountered many fewer people with CF in poorer countries. CF patients there die far younger than in long standing EU countries.”

Nick Fahy, head of the health information unit in the Health and Consumers Directorate General at the European Commission, said, “Knowledge is key to improving health in Europe. For these rare diseases, there are so few centres of expertise that only by working together across the EU can we enable all citizens to have access to the best possible care. “This European cooperation also shows member states how they compare to best practice in Europe for different conditions, so that every health system can prioritise their resources to meet the needs of their patients."

Cystic fibrosis is an inherited chronic disease that affects many organs, particularly the lungs and digestive system. CF patients carry a defective gene that disrupts the transport of salt across cell borders. As a result, the body produces thick mucus that blocks ducts and tubes. Blockage of air passageways causes chronic cough and lung infection; blockage of the pancreasprevents enzyme delivery to the intestine to break down food; and blockage in the intestine prevents food absorption. Countries in Western Europe have committed significant resources to making the necessary treatment available to CF sufferers, helping them to live longer and fuller lives.

Read the rest of this article by clicking here or going to http://www.medicalnewsbase.com/medical-conditions/call-for-european-cystic-fibrosis-healthcare-gap-to-be-closed/

Exciting Article About Drug Delivery

Beating the cystic fibrosis barrier

Biodegradable nanoparticles capable of penetrating the mucus barrier in the lungs and gut of cystic fibrosis (CF) sufferers have been developed by researchers at John Hopkins University, led by Justin Hanes, professor of chemical and biomolecular engineering.

“Cystic fibrosis mucus is notoriously thick and sticky, and represents a huge barrier to aerosolised drug delivery,” said Pamela Zeitlin, CF expert and professor of paediatrics, who collaborated on the study. “Nanoparticles were engineered to travel through cystic fibrosis mucus at a much greater velocity than ever before, thereby improving drug delivery,” she said.

The nanoparticles comprise of two parts made of molecules regularly used in existing medications. An inner core of polysebacic acid (PSA) traps therapeutic agents inside while a dense outer coating of polyethylene glycol (PEG) molecules allows the particle to move through the mucus by preventing it from reacting with proteins.

Hanes previously demonstrated that latex coated with PEG could slip past mucus coatings, but because the body could not break it down into harmless components it was impractical. “The major advance here is that we were able to make biodegradable nanoparticles that can rapidly penetrate thick and sticky mucus secretions and that these particles can transport a wide range of therapeutic molecules,” said Hanes.

The nanoparticles have potential applications treating lung and cervical cancers, and inflammation of the sinuses, eyes, lungs and gastrointestinal tract where mucus is produced to protect sensitive areas, said Benjamin C Tang, lead author and postdoctoral fellow in the department of chemical and biomolecular engineering. Zeitlin concludes: “This work is critically important to moving forward with the next generation of small molecule and gene-based therapies.”

Read the full article here: http://www.labnews.co.uk/laboratory_article.php/5285/2/beating-the-cystic-fibrosis-barrier

Smart Pseudomonas

Adapting To Clogged Airways Makes Common Pathogen Resist Powerful Antibiotics

People with cystic fibrosis frequently have lung infections that defy treatment. Cystic fibrosis is an inherited disease that clogs airways with thick mucous. While the life expectancy for children with cystic fibrosis has increased over the past few decades, many lives are still shortened in young adulthood by the ravages of lung infections.

These chronic infections are often caused by common, environmental microbes that mutate in ways that let them live and thrive in viscous lung secretions. The same adaptations also make the pathogens less likely to be killed off by powerful antibiotics, according to a recent study led by Dr. Lucas “Luke” Hoffman, University of Washington assistant professor of pediatrics.

Surprisingly, he added, the pathogens don’t need any previous exposure to the antibiotics to resist their effects. The results were published in the latest edition of PLoS Pathogen.

The researchers looked at Pseudomonas aeruginosa, a microbe that can infect a cystic fibrosis patient early in life and then undergo various changes as it establishes a chronic lung infection. Pseudomonas aeruginosa with specific alterations tend to give patients a poor outcome. Some of those alterations diminish the chances of eradicating the infection with antibiotics.

It’s believed that these adaptive alterations in Pseudomonas, all of which are caused by genetic changes, could be selected for by the environment inside a patient’s airways, the researchers noted. Characteristics that facilitate microbial survival begin to emerge.

The specific airway conditions that select for these genetic changes, Hoffman said, remain unclear. “But,” he added, “we have some clues from what is known about airway mucus.”

From the point of view of Pseudomonas, the physical properties of cystic fibrosis mucus, Hoffman said, “make it a great place for the stuff people routinely breathe in to set up shop.” Cystic fibrosis secretions contain a lot of nitrates and amino acids, which Pseudomonas can use to grow.

Inside mucus plugs oxygen levels are low. Some Pseudomonas strains can live in this oxygen-poor, nutrient-rich environment. Hoffman and his team found that a mutation that occurs commonly in Pseudomonas from cystic fibrosis patients allows the pathogen to grow better in the nutrient environment in cystic fibrosis secretions. This particular mutation inactivates a gene named lasR. Pseudomonas with this mutation apparently undergo a metabolic shift: consuming less oxygen while utilizing nitrate more efficiently. lasR mutant bacteria also can handle oxidative stress resulting from an imbalance of damaging substances called free radicals forming faster than they can be detoxified.

One source of oxidative stress encountered by Pseudomonas is the antibiotic treatment that is frequently given to people who have cystic fibrosis. Antibiotics like ciprofloxacin and tobramycin kill bacteria partly by inducing the overproduction of free radicals and causing oxidative stress. Hoffman and his team found that, because these mutant microbes are resistant to oxidative stress, they were relatively resistant to these antibiotics when grown in conditions that were like cystic fibrosis mucus.

“We learned that simply by adapting to the conditions inside the airways of cystic fibrosis patients, mutated Pseudomonas can withstand the effects of ciprofloxacin and tobramycin,” Hoffman said. They did not need any previous exposure to these antibiotics to reduce their susceptibility.

Hoffman and his team suspect that Pseudomonas is not the only microbe that can do this. Some of the characteristics conferred by the mutation in Pseudomonas are also exhibited in other microbes found in chronic lung infections, such as tuberculosis or the fungal pathogen, Cryptococcus neoformans, Hoffman noted. Metabolic shifts may be a way many microbes get the upper hand over their hosts — and over antibiotics.

This report, Hoffman said, may point to new ideas for treating chronic lung infections. Luckily, colonies of Pseudomonas with the lasR mutation are relatively easy to identify in hospital laboratories by their distinctive iridescent sheen. Because lasR mutant Pseudomonas has been associated with worse outcomes in cystic fibrosis patients, indentifying Pseudomonas with the lasR mutation may be of prognostic value and may indicate the need for treatment with specific antibiotics like monobactams, tetracyclines, or polymyxin, whose mode of action differs from ciprofloxacin and tobramycin. Other treatment methods may be targeted at preventing adaptive changes, such as the lasR mutation, in Pseudomonas, the researchers said.

View the full article here: http://www.bmedreport.com/archives/10015

Rondi and J-Dizzle Sing Wild Horses

For this sound off Saturday - you get the hear the WONDERFUL sounds of our voices.

Sorry Jezzy doesn't chime in more form the backseat. Enjoy!

Do you procreate if you have bad genes??

I found this article and the subsequent comments very interesting. To view the comments and full article, click here.

Do you procreate if you have a bad gene? Discuss

January 22, 2010 at 11:27 am by Tracy Ormsbee

I’m reading a gripping and scary novel called “Still Alice,” by Lisa Genova, about a Harvard psychology professor who develops early onset Alzheimer’s. The novel is written from Alice’s perspective so you are in her head as she slowly loses her grip on reality and what’s going on around her.

Bear with me, this does relate to parenting: After she learns she has the disease, she is told that it is genetic and a test exists to screen her three grown children. One of her grown daughters is undergoing fertility treatment to try to have a baby. Alice thinks about what she would have done had she known she carried the gene before she got pregnant.

Would she have conceived these children or taken precautions to prevent them? Would she have been willing to risk the random roll of meiosis? Her amber eyes, John’s aquiline nose, and her presenilin-1. Of course, now, she couldn’t imagine her life without them. But before she had children, before the experience of that primal and previously inconceivable kind of love that came with them, would she have decided it would be better for everyone not to?

This is interesting because most of us carry a gene for disease in our family history. My mother has diabetes and I knew the gene could be passed on. I decided to have children. Part of my decision was based on the fact that my mother took good care of herself and lived a normal life without complications (she continues to do great). I also developed diabetes at 28 after having gestational diabetes with my first pregnancy. My oldest daughter does not have diabetes; my youngest was diagnosed when she was 4. Don’t know how I could possibly have regrets. She lives a normal life on the insulin pump and is perfectly healthy. I would make the decision again today.

In the book “Still Alice,” her daughter decides to have children. Since she is doing in vitro fertilization, they will screen the embryos and only implant those without the gene for Alzheimer’s.

To view the full article and comments, please go to http://blog.timesunion.com/parenting/6222/do-you-procreate-if-you-have-a-bad-gene-discuss/

Can CF Teens and Parents Find Common Ground??

A good friend of the CF community and puppet master Josh Mogren, brought up a great "debate" question over on CysticLife.org that I wanted to get the RSR reader's take on:

I have spoken with a few teenagers who claim that their parents are too overwhelming of a presence in their lives. While this is common place among most teenagers across the globe, what happens when you add Cystic Fibrosis to the equation? Teens feel trapped by their parents because they don't feel like they are allowed to make their own choices about their lives and learn from their mistakes. Most parents I have talked to say the reason that they are so concerned for their children is because they don't see their children doing the best they can with their healthcare and daily routines that would improve their lives. Some have seen other people's children pass away from CF and fear the possibility that this might happen to their child. So they refocus their energy toward making sure their child thrives. The parents feel (rightfully so in some respects) that they have given their blood, sweat, and tears to their children and that CF lives are too precious to learn from mistakes because the results are far too costly. CFer's and Parents....let's honestly discuss...where can we meet in the middle? How can teens understand their parents point of view and earn their trust? How can parents learn to start letting go and trusting their children? Positivity and respectful responses are a must! This is thin ice territory, but it must be traveled on...

Parents, what do you think? What is your responsibility to your child and at want point does that responsibility shift? Teens, when can you begin to shoulder some of the burden for your "cystic life"? What's the best way for your parents to handle this transition?

If you'd like to view the answers over on CysticLife please click here.

Breastfeeding and Cystic Fibrosis

I can't claim to have a lot of experience on the topic of breastfeeding in relation to Cystic Fibrosis, but recently, a super mom out there, Kacie, gave a great interview that at least partly spoke on the subject. The complete interview is certainly worth the read and I highly encourage you guys to check out the full transcript by clicking here.

Wendy: Since you were exclusively breastfeeding Lucy, did the diagnosis affect this? What did your doctors recommend and what did you do?

Kacie: I had always known that I was going to exclusively breastfeed my children so the decision was made before Lucy was even born. When Lucy arrived, and because she had the blockages, I was not able to nurse her for 2 whole weeks. Imagine my frustration and heart ache to not be able to nurse my child when she cried for me! She never ingested a single thing until 2 weeks old, waiting for her insides to heal up. She was given fluids and nutrients through an IV while I pumped, pumped and pumped, and saved every last drop of milk I could get. I made the NICU nurses write in huge, red lettering, "MOTHER'S BREAST MILK ONLY!" in her file so she would never be given formula. The very first thing that went into Lucy's repaired intestines was exactly 5ml of my milk. She was not to be given more than 5-10ml at a time to insure that the surgery went well. After 2 days of "testing" her new tummy out, we were finally, at last, able to nurse. I will never forget the first time either! She latched on like she had done it before. We were off and running. The doctors were very pleased that I was so adamant about breastfeeding. The recommendations about switching to formula came later. Lucy was not gaining as much as she should have been gaining according to the AAP weight charts. When she dipped below 50th percentile, it was mentioned that we may have to supplement with formula for added calories. I told them that giving her formula would never happen in a million years! I went home that day and started nursing her around the clock like normal, but I added in feedings as well, even when she really didn't even have hunger cues. I became a human pacifier because I refused to put her on formula. Extra nursing worked. She gained enough weight to get above the 50th percentile so Lucy's nutritionist never mentioned formula to me again.


Wendy: How has breastfeeding proved to be a great help in relation to Lucy's diagnosis and her illness in general?

Kacie: I make jokes that my boob has been my best friend and still is! Lucy has been in and out of doctors offices her whole life. Her life is very chaotic and stressful because of CF. More stress than a child should have to go through: blood tests, needles, throat cultures, wearing a vest that shakes her chest and taking up to 9 different medications a day. By nursing Lucy on demand for the last 2 years, we have created a safe place for her to go where she knows everything will be okay. Nursing is her neutral place, a safe place, the quiet place among the storm. I am more in tune with her and her physical and emotional needs because of our nursing relationship. Nursing her for this long has helped her immune system. She is sick less often and when she does get sick it is very short-lived. If I ever get a cold or illness, she has never gotten it from me. She is receiving the antibodies from me that are needed in fighting the infection. I really cannot think of one negative thing to say about breastfeeding Lucy.

Read the full interview at Aruban Breastfeeding Mamas

So what do you guys think? Does/did breastfeeding create a "safe place" for your CF child? Would you recommend breastfeeding to the mothers that are out there reading this?

The Cells of CF

I thought this article did a good job explaining what is going on in the cells of a CFer, cause remember, it's not a lung disease. Cystic Fibrosis is a CELL disease.

Inhibition of Proteostasis Restores Ion Channels in CF Cells

Tuesday January 5, 2010

Our cells are full of enzymes that mediate activities like growth, metabolism, replication,transcription and cell signalling. They are all unique in their structure, and have evolved over time to perform the specific functions for which they are made. Some enzymes are hydrophillic(water-loving, or water soluble) and remain in the cytoplasm of the cell, while others arehydrophobic, thus more lipophillic. The lipophillic enzymes are generally found embedded in cell membranes and tend to have roles such as mediating the transport of small molecules and ions across the membrane. Cystic fibrosis transmembrane conductance regulator (CFTR) is one such membrane protein.

In order to study its structure and function, it is necessary to first purify a protein. Like all hydrophobic transmembrane proteins, study of CFTR is hindered because it is difficult to purify and study in a stable form. However, it has been determined that mutations in the gene encoding CFTR result in an improper protein structure, that folds incorrectly, is recognized as defective by cellular machinery, and is destroyed.

Without CFTR, chloride ion transport in the lungs is hindered, resulting in a mucus buildup characteristic of the disease. Efforts to treat CF by altering the gene or protein structures in patients have not been terribly effective, but last month a study was published that showedrestoration of ion transport could be achieved by controlling the actions of a class of enzymes responsible for proteostasis (destruction of proteins) called histone deacetylases (HDACs). A compound called suberoylanilide hydroxamic acid (SAHA), already approved by the FDA for treatment of lymphoma, was able to restore up to 28% of channel activity in isolated cell cultures from CF patients. The authors, Hutt et al., postulated that by preventing destruction of imperfect proteins, those polymorphisms that are not completely disfunctional can restore some ion exchange capacity in cell membranes.

Full article at http://biotech.about.com/b/2010/01/05/inhibition-of-proteostasis-restores-ion-channels-in-cf-cells.htm

Make sense??

Question from Researcher

Happy Saturday!!! I was recently contacted by a researcher who is gathering info for her PhD. She asked some questions that I've decided to turn over to the community. I appreciate you guys taking the time to think about the following question and give it your most thoughtful response. I think it is always wise for us to help out anybody doing any type of research on Cystic Fibrosis-whether the physical, mental or spiritual affects of the disease. Just in case your curious, I'm not getting paid to participate and either are you :)

Question 1. In your blog you often mention your body, or parts of your body. You talk about running to keep your body healthy, you talk about feeling pain, fatigue, feeling out of breath and you talk about pushing through those feelings to go running or walking to stay healthy. Your most recent posts have been about getting your body healthy, about how the different drugs are making your body feel, concerns about how they might affect your body and so on.

I am interested in finding out how people with CF experience their bodies. Not symptoms of CF but the thoughts, feelings, and ideas about your body that having CF and also dealing with CF in everyday life brings up for you.

For example, I have some problems in my neck and shoulders and it has really changed the way I see my body, and not only that, but my outlook on life. I thought it was strong and would last forever and all i had to do was feed it and sleep. Now I know that you can't rely on anything to stay the same or be what you expected. I know your body is something you have to care for. Sometimes I feel angry, sad or frightened when I am in pain and can't sleep or do my work. I feel frustrated and disappointed when it interfere with my plans. Sometimes I feel excited because I think my body is teaching me important lessons about life - how you can't put too much store in plans for the future for example. So these are some of the thoughts, feelings and ideas that my body brings up for me.

Bio: I am an Australian health researcher and have been doing research with people with CF since 2006. I do social research, not the treatments or laboratory research, which aims to understand about people's lives, their experiences and perspectives. My current project is my PhD in which I am trying to understand the experience of being an adult with CF. The idea is to gather, in a 'scientific' kind of way the sort of information that can give doctors, nurses, policy-makers and so on an insight into the more personal aspects of managing and living with CF in adulthood as opposed to the medical bits. I hope this sort of research will eventually lead to more appropriate treatments, better relations with medical professionals, better health policy and community support. I have contacted Ronnie to help me thrash out some of the ideas that are coming up in my research

So, what do you guys think? Do you have any answers to "how you experience your body" as a result of CF?

PICC Line in the Neck!!!

Sometimes the right foot NEVER seems to know what the left foot is doing here in the hospital. After a series of ball-dropping, x-ray delaying and boondoggling, I'm now stuck with a PICC line that can't be used. So now I have a new IV and must wait until Monday to get this PICC line fixed. Oh what a beautiful day!!!!

Any "nice" hospital stories for me???

Rondi sings "I'll Be There"

If you're new to this blog, I must tell you, Mandi and I love to do car karaoke. And fortunately for you (or unfortunately in all cases), we also record our "singing". So please sit back and enjoy this tasty little treat.

How Can I Simply Be Abby?

I often get emails asking for my opinion or advice on different topics surrounding the life of a CFer. Most of them I will answer directly and you guys never see them. Once in a while however, I get a question that I believe needs to be answered and addressed by the community. This is one of those times. I received this email yesterday by a high school girl named Abby (I changed her name for privacy sake). I know that many of us have been in this exact same position before and would have killed to hear the advice of other "older and wiser" CFers. I figured that the more insight she had on this particular subject the better. I also have a feeling that there are about 1000 "right" answers out there. Let's come together as a community and help this cyster out! Please read this short email and respond however you see fit. Thank you.

I have one question today.

You've inspired me. I wanna make the change in my life, to not let CF rule who i am, I let it define me and who i am way to often. I just don't know how to become simply "Abby" now that I've always been "Abby with that lung disease" .

Suggestions on how to change it ?

So, what do you guys think? How do you have CF without being CF? Is there a way to be different without being treated differently? I really appreciate you guys taking the time to answer this question!!!

Parents, Would You Roll the Dice on CF?

**I posted this a couple of months ago and got a great response. I thought I'd give it another go around this week. Make sure you take the time to read through the comments too!**

I would really really really like all of your opinions on the following article...

Should Bad Genes Mean No More Kids?

THURSDAY, SEPTEMBER 24, 2009

filed under: health

My husband and I chose to have more kids, knowing we have a 25% chance of having another child with cystic fibrosis. Are we selfish?

Maybe, but I think my daughters are pretty grateful to be here.

Homeschool Mom: Is it selfish to have more kids when you know you and your husband have a bad gene and you have already passed it on to two kids? Frankly, I'm not sure. I know I love my kids and I am taking care of their health needs and they are growing and thriving, but I also know that they have a big burden in their lives. I have the burden as well, of course, but it is different when you are the person who is suffering with the physical symptoms. They definitely feel the burden, they deal with it, and they are not happy dealing with it. In fact, I asked each of my children with cystic fibrosis if they could change one thing about their lives, what it would be. Each one said they would want to be free of CF. My husband and I have the same wish. Even the youngest, who does not have it, said her second wish would be that her siblings no longer had CF (her first being that she wants a pony).

Let's face it, having a chronic illness that demands many inhaled breathing treatments, oral drugs, sometimes intravenous drugs, and hospital stays, and which causes you to have pancreatic insufficiency so you have to take pills every time you eat, plus a host of other symptoms and problems that would take up my entire page, is not fun. But does that mean that never having been born would have been better? Well, how can anyone truthfully ever know?

I do know my children are happy and as healthy as their friends. Their condition does not prevent them from doing anything. They do have to work hard to maintain their health, and they have to see many doctors on a regular basis, but for now they are as energetic and exhausting as any other kids. The tragedy would be if their health deteriorates while they are young and they succumb to CF. Obviously that is not something I contemplate too often, because it would be heartbreaking, but when I really consider it, I know that a tragic end does not mean a life was meaningless, it means a tragedy has occurred.

There are no guarantees when you have a child. I remember when I heard about the Christian singer Steven Curtis Chapman, who lost his 5-year-old daughter when his son hit her while backing out of the driveway. Who could foresee such a tragedy? Children get cancer and other illnesses, and sometimes they die horribly and painfully. We do the best we can with our children's difficulties. I think my husband and I are good and loving parents who try to do the very best for our children. I would take my children's illness upon myself if I could. I would give my life for them, but I would never give them up, nor second-guess a decision to have given them life in the first place. The world is a better place because my children are in it, and you should all just count your blessings they are here!

Follow up questions:

What would your decision be?

Would you have more children?

CFers, how would you feel if you knew your parents made that choice?

Any parents out there struggling with this very issue?

I would love your feedback on this issue guys, so please, comment away!!!