Douglas Conrad, MD

Marianne asks a very good question.   She states she is having “troubles” post transplant adjusting the dose her pancreatic enzymes.  You don’t state which pancreatic enzyme preparation you are using but let us assume that it is a preparation recently approved by the FDA.  Also, what specifically are the “troubles”?  I will assume this means you are having difficulty with malabsorption symptoms such as loose oily stools that are frequent foul smelling and are associated with abdominal bloating and some discomfort.  I have reviewed drug information for some of the preparation and they don’t specifically mention any particular issues with these preparation in patients post transplant.

Pancreatic enzymes replace naturally occurring enzymes produced by your pancrease that help you digest food and allow it to be absorbed through the digestive tract.  It is unlikely that there is any absorption of the enzymes through the intestines and so systemic symptoms are unlikely unless they are due to an absorbable excipient of the preparation or if you are allergic to a part of the drug.  A change in the preparation may be helpful in this case.

I suspect the problem is more likely due changes in your diet which may be precipitated by some of the anti-rejection drugs.  Some of these can promote fluid retention and cause swelling in your feet and weight gain in which case a diuretic and lowering of sodium intake would be helpful.  Corticosteroids such as prednisone can stimulate appetite and this could increase your weight.  The increase in appetite (and calories) could also increase your need for pancreatic enzymes in which case increasing the dose would be helpful.

If your major problem is weight gain and fluid retention is not a problem, I would not advise decreasing your enzymes to control weight.  A discussion with your transplant physician would be helpful but better control of diabetes and using exercise to control your weight is a healthier alternative.

I hope this  response is helpful and I wish you well.

Isabel Stenzel Brynes, MSW, MPH

Dear Marianne-

Thank you for being open with your GI challenges; you are giving permission for others to do the same. According to my peers, it is not uncommon for people with CF to need to change their dose of enzymes after transplant.  Keep in mind everyone is different, but I have known people to dramatically reduce their enzymes or in some cases stop taking them all together– not to lose weight but because their stools were normal without them. I have a personal hypothesis: I had less mucus in my body overall with my old CF lungs removed- hence I wasn’t swallowing sputum constantly and my overall GI tract was clearer, so my absorption was better. Plus perhaps having good oxygenation helps the GI tract.

In my personal experience, I need very few enzymes now, 7 years post-transplant, unless I deliberately eat a very fatty meal. Some reasons are as follows: my cholesterol is high so I have changed all of dairy products to non-fat. This reduces the need for as many enzymes. I also no longer eat red meat and dramatically reduced eating pork.  I try to eat vegetarian twice a day. To respect the gift I’ve received, I do not eat high fat processed or junky food either.

When I started gaining considerable weight (20 lbs over baseline, because I was hungry all the time!; and I had no ‘off’ button) I would consume large amounts of vegetables, fruit, and low sodium veggie soups (to fill me up) when I was hungry.

My primary solution to weight gain is exercise. I try to work out several times a week so I can stay fit, fat can be turned into muscle, and I feel better overall. I also don’t have time to think about food when I am exercising! Also, I drink a very very large amount of water, and that also helps to keep my stomach full so I’m not always feeling the sensation of hunger.

Now for the tough answer- when was your transplant? How old are you? I am 39 now, and I’m afraid some of my weight gain is … well, because I’m 39! The gift of aging!

Also, are you diabetic? I’ve noticed if I eat a ton of carbs and use the equivalent large amount of insulin, I will gain lots of weight easily. So, I try to eat a low to moderate amount of carbs and switch to low glycemic foods like whole wheat, brown rice, etc.

As for your enzymes working and not working, that is tough to address. Could it be related to anything you are eating along with the enzymes– ie acidic foods, extra fat, etc? It would be interesting to see if there’s a patter at all. As you know sometimes our calibration of meds is a moving target. I wonder if you are also on a PPI- acid medication like protonix.

Just an aside, I take an enzyme with every Prograf, and with my vitamins. My main challenge is constipation, which is independent of the number of enzymes I take. Thank God for Miralax.

We are all so unique in this illness, so you will have to watch and wait and see how your issues turn out. I wonder if you have a good nutritionist you can turn to for more advice.
Good luck in your health and enjoy breathing free. It’s quite a gift, isn’t it!
Breathe easy,
Isabel Stenzel Byrnes

Roberta Cox, RRT – Respiratory Therapist at Denver Children’s Hospital

Studies show that Hand CPT is only as effective as the strength, force and speed provided.  So as you grow older and larger it becomes less effective.  With our older patient’s we tend to change things up by adding Autogenic drainage, PEP or exercise to improve the effect of their airway clearance.  These tend to work better with our adult population.

We have not been doing the head down position with any of our patient’s for quite a while as it is believed to increase blood pressure as well as pressure in the brain.

Ronnie Sharpe – Community Servant and Founder of CysticLife

Just like many other areas of this CF life, I think selecting the right chest physiotherapy is all about trial and error. I know for me I will always prefer hand CPT over the vest, but it may be because I was pounded on by hand at least twice a day for the first 20 years of my life. There are obviously pros and cons to just about every treatment option out there, but at some point it will come down to preference and our ability to access various forms of chest physiotherapy. Now, it is important to note that trying something once or twice probably won’t give us the best indication of whether or not it will work for our desired outcome. When I was younger I couldn’t stand doing the flutter valve because it made me cough. Now that I’m older I understand that that was the whole point!

Meg Benefield, MSW, LICSW

I am impressed by your question because it shows that you are a thoughtful parent, doing your best to take of yourself and your children.  I believe parenting is the most difficult job in the world, and balancing that with CF is a tremendous challenge.  Health declines leave most adults with far less energy and more anxiety about their family’s future.  In turn, children pick up on their parents’ stress and have worries of their own.  Children may act out, and parents have less energy to set limits.  Feelings of guilt also make discipline more challenging because we feel responsible for our children’s behavior, want to make them happy and try to avoid negative interactions.  However, setting and keeping limits, even during the most difficult times, teaches kids important lessons about self-discipline and resilience.

Your openness about your emotions and willingness to share information with your children are tremendous strengths and will help you make changes.  From there, I recommend you evaluate your situation and address several key areas.  In parenting, you are always working with at least three things: 1.your needs, as person who is parenting, 2. your children’s needs, as individuals and 3. how you and your children interact with each other.

Your Needs

As a person who is parenting, you need to address your own needs.  In CF, you have the challenges of fatigue and keeping up with numerous health-related tasks.  You are coping with unknowns, including some things you can’t control.  I encourage you to take stock of your own stressors and areas where you can use more support.  For example, are there tasks you can delegate to someone else, i.e., chores, meals, shopping?  This may mean reaching out to your support system for help, but it will give you more time and energy.  Emotionally, you also need a safe place to share your concerns and fears.  Guilt is a common and difficult emotion in chronic illness.  It commonly rears its head when people are grieving losses related to their health.  I have had many parents tell me they feel guilty that they can’t be as physically active with their children or miss out on occasions when they are ill.  Parents have also shared fears of dying and how this would impact their children.  These are normal but difficult emotions.  It is vital to have trusted friends/family, medical providers, a counselor, or a clergy person with whom you can openly share your concerns.  The more clearly you recognize and receive support for your emotions, the more empowered you will feel.  The emotions will ebb and flow, but you will recognize when your feelings are affecting your parenting and be better equipped to change your responses.

Your Children’s Needs

Children have different needs, depending on their ages and personalities.  It is not uncommon for parents to tell me a particular child is struggling more openly, or that each of their children has a very unique way of coping with CF.  Some kids like to be involved with hands-on medical tasks, others do not.  Some kids want a lot of information and ask questions, others seem satisfied with basic answers and rarely seek information.  In general, I think some of the most important things for kids to hear when coping with CF are: “you will always be cared for, we will always be connected, and nothing that is happening is anyone’s fault.”  It is important that kids have support outside the family.  While spending time with other trusted adults, kids may talk about concerns more easily because they don’t have to worry about upsetting their parents.  School-based supports, when available, are an excellent starting point.  Some schools have social workers or counselors available to check in with children.  You can also consider counseling in the community.  Getting kids extra support teaches a great lifelong lesson that everyone faces challenges, and seeking help ultimately makes us stronger.  Professionals can also assess whether a child’s behavior issues are a normal response to stress versus a separate condition.  (See the article below for additional thoughts on helping kids cope with a parent’s chronic illness).

Parenting Tools

Finally, it is helpful to have as many parenting “tools” as possible.  Using a framework for discipline helps with consistency and gives you specific ideas for addressing challenging behaviors.  One book I recommend is Parenting with Love and Logic by Foster W. Cline, M.D. and Jim Fay (you may have noticed Dr. Cline is on the Blooming Rose Foundation’s expert panel; I have always loved his work and was thrilled to see him here!!). Raising Your Spirited Child by Mary Sheeda Kurcinka is one of my favorites for kids who tend to be more emotionally intense.  I like these books because they build on families’ strengths and focus on discipline rather than punishment.  These are just two suggestions; your children’s schools/your community may offer other books, parenting classes, etc.  As you start to use specific parenting techniques, you will build a “toolbox” of ways to address issues and have “mantras” to fall back on when you’re feeling overwhelmed by kids’ behaviors.  You can build messages like, “This is a hard time for all of us.  In hard times, it is extra important that we stick to the rules that keep our family strong.”  Parenting techniques will then give you concrete ways to follow through and be as consistent as possible.

In my work, I have met incredible families, and some of the wisest things I have ever heard about coping with CF have come from the children of my clients.  Once, a 12-year-old girl said to me, “I’m both sad and happy.  I’m sad that Mom is sick right now, but I’m happy that she’s here.”  To me, that captures perfectly where things can land in the balance; coping with CF is difficult for families, but simultaneously full of joy and hope.  Kids are resilient, and you are helping your children immensely by continuing to work on parenting during a difficult time.

Resources:

“Living with a Parent’s Chronic Illness” by Claire Berman

http://claireberman.com/mag_chronic.html

An article about support kids as they cope with a parent’s illness; while this is not CF-specific, it has many applicable ideas.

Love and Logic Institute: www.loveandlogic.com/

This website contains numerous Love and Logic^® resources, including parenting books.

Raising Your Spirited Child

A helpful book for parenting more “intense” children.  The website also contains links to other parenting resources

http://www.parentchildhelp.com/SpiritedChild/tabid/59/Default.aspx

Sharlie Ross Kaltenbach

I can definitely relate to your question—especially to the part about feeling guilty and wanting to forgo discipline or punishment.

First of all, bravo to you! Three kids, and you are managing it all. You are an inspiration! You have more experience in this department than I do as I only have one four year old. However, I have already faced the issues you brought up and here are some of my thoughts and some of the things we have implemented in our home.

I believe communication is the most important thing. Growing up, my mom was always very honest with my sister and I about our disease and was constantly checking in on how we were feeling and what we were thinking. It was a very safe, open environment. I want to create that same environment in my home, so, just like you, we have used appropriate language to talk to our son about momma’s lungs, hospital stays, treatments, medications, I.V.’s etc. etc. When my son gets older I will also encourage him to talk to me about his fears and frustrations about what our family is dealing with.

I believe that kids act out when they want attention. My son used to struggle when I was doing my treatments in the morning and I know he was frustrated that I couldn’t play with him. So, we decided to make this a special momma/son time. Now, I try to finish most of my treatments before he wakes up in the morning. If I don’t quite finish them then he gets to come in bed with me and play a game on the iPad or pick out several books to look at while I am finishing. He also loves to help and I have him squeeze the medicine into the nebulizer and turn it on. He also loves helping me connect my VEST hoses and feeling the vibrations. I have even put the VEST on him so he could see how it feels. When I do home I.V.s, he loves to put on sterile gloves and help flush my I.V., throw things away for me or play with the tape. I always try to involve him so he is comfortable with everything I do and so he knows that it is all for my benefit. Maybe putting them to work organizing your medical supplies and/or helping with your treatments would help them feel needed and less anxious if it became kind of a fun activity.

I try to be very consistent in disciplining my son. I always explain to him what I expect from him and make sure he knows what the consequence will be if he disobeys. When I am not feeling well, I really make sure he understands that I need him to be good. I think it’s super important that our kids understand that just because we are sick and can be physically weak at times, we are still the parents and will enforce the family rules. I think the key is in your word, “redirect.” Distract your daughter by giving her little jobs she can do that will help you. Also I think it is important to get one on one time with her. Maybe you could implement a weekly or monthly date with her to let her feel some normalcy with you. Even if it is just being at home but telling her that you guys can do whatever she wants to do for a half hour or hour. I think giving her some special attention while still being firm and consistent with her when she acts out will send her some powerful messages.

I hope and pray your health improves and that you can continue spending glorious time with your family. Blessings to you!! Keep up the amazing work!

Response from Angie Bates, DPT

YES!  Yoga is an excellent form of exercise for people of all ages and there are many different types.  All forms of Yoga use breathing techniques and teach you to draw air down deep and control your exhale.  The diaphragm is a muscle that can be trained for maximum benefit.  Breath of Fire uses accessory muscles in addition to the diaphragm to help heat your core (great for cold days on the ski lift!)  Bikram (hot yoga) in a studio may not be the best idea for a person with CF due to the risk of infection.   Bacteria, yeast and some viruses like warm/moist environments.  Group Ashtanga and Hatha classes in large, well ventilated studios are better if you use your own mat, wash hands right after and perhaps bring disinfecting wipes for shared equipment (blocks, bands, rolls, etc…)   Netflix instant view has many yoga classes for the privacy and sanitation of your own home if immunity is an issue.  A private instructor would give you 1:1 instruction and work towards your specific goals whether they be relaxation, strength, respiratory or flexibility.

Response from Emily Schaller, Rock CF

I cannot say that one form of exercise is the best or better than the other. Staying active and breathing hard is good and very important to people with CF. For me a balance of running and cycling is complemented very well with yoga. Running and cycling puts stress on my bones, joints and my muscles and yoga does a good job helping to care for those areas and my whole body and mind. Yoga is all about finding and using your breath. After many months of intense yoga classes I have really found my breath and am able to fit all of my practices around it. Since starting yoga I have never felt better, both physically and mentally. Some classes like vinyassa can be physical while gentle or slow flow classes are less physical and more mental. They all focus on core strength and breath though which really are important when living with CF. So I think a good balance between different yoga practices and other sports is great. Just find something you love and have fun with it, and of course talk to your doc before starting!

Response from Terri Laguna, MD, MSCS, FCCP, FAAP

The parent asks a very good question.  At this point, the Cystic Fibrosis Foundation does not recommend a baseline chest CT at an early age as a “baseline” examination.  There are a number of reasons for that:

1.       The radiation exposure.  A chest CT scan provides a radiation dose of approximately 3-7 mSv (compared to a chest xray which is approximately 0.06 mSv).  It is substantially more radiation.  The issue is that most patients with CF will likely require multiple chest CT scans at some point to evaluate their lungs.  If you start early, then the radiation exposure over time can certainly add up and increase the risk to the patient.
2. It is not clear how early chest CT scans affect medical treatment.  As physicians, we usually order a test or a study with the hope of using the result to help improve treatment and therapy.  If we are getting a “baseline” chest CT, it will likely not affect or change our management.  Therefore, it is unclear how a baseline study would impact therapy.

At this point, there are other ways to obtain information about how infant and toddler lungs are doing.  Many CF Centers can perform infant pulmonary function testing until about age 2.  However, the parent raises a good point in that from about age 2 – 5 (or until a child can perform regular PFTs), there really aren’t very many good ways of evaluating the lungs of a child with CF.  There are multiple studies that are looking at CT in infants and toddlers to see if they provide enough information to outweigh the risk.  We may be performing routine chest CT scans in the future on all of our patients, but as of right now, that is not the case.

Roberta Cox, RRT

Our recommendation is heat sterilization as it has been determined the best at killing pseudomonas.  Micro steam bags, baby bottle sterilizer or dishwasher (top rack without soap/must be 160 degrees) or boiling for 5 minutes at a rapid boil are the best methods.

We recommend the Pari LC plus nebulizer for delivery of all the medications.  It provides the best particle size and % of delivery and lowest deposition in the lungs.

Ronnie Sharpe

Like the RT said above, there are many available options when it comes to sterilizing our neb cups. I have done many different techniques throughout the years including the dish washer, a hot pot, boiling water on the stove as well as a bleach mix (I would not recommend this one). However, I have recently been using a new method which I have found works the best with my schedule and is also the least time consuming to perform. I have been using a baby bottle sterilizer for the past couple of years and I absolutely love the results. It’s quick, it’s easy and it get’s the job done! I would recommend this method to anyone and everyone in the CF community.

It’s important to remember that the sterilizer must be cleaned every so often as well. My particular sterilizer, the Philips Avent iQ24, recommends the cleaning every four weeks or so. It’s important to clean the sterilizer so that it works to its full potential and avoids any sort of limescale buildup. To clean it, I just simply follow the instructions that were included in the manufacturer’s manual. I mix 100ml of vinegar with 20ml of cold water and let it sit in my sterilizer for a while. I then pour out the solution, rinse out the basin and then hand dry the unit. I also run the sterilization cycle with no neb cups just to get any remnants of vinegar out of the sterilizer.

Lisa Greene’s Answer: Early education about CF is critical. Children need to learn the nuts and bolts about CF including care requirements starting at about age three. This will probably come naturally because around the ages of three and four, children start to ask a lot of questions about everything. “Why is the sky blue?” “Why is a ball round?” Why, why, why… Of course when a child has CF, some of the questions will be about CF as well. “Why do I have CF?” And, some may ask, “Will I die from CF?”

That’s what my two children with CF asked me, at around ages 4 and 6, as we were driving home from school one day. Here’s how I answered:

“Well, everybody dies of something. Some people do die from CF. Some die from cancer and others die in car accidents. We just don’t know the future. And, if you keep taking good care of yourself like you do now, you will be more likely to live a long time.  The doctors are working hard on finding new medicines to help. So, there’s a lot of hope for people with CF.”  Then, my son (the six-year-old who had asked) said, “Oh. Okay. Can we stop at McDonald’s for a snack?”

These “pivotal parenting moments” can take us by surprise so be prepared ahead of time. Our answers should be honest, calm, matter-of-fact, and hopeful. We can emphasize our child’s role in good self-care. We shouldn’t use words like “fatal” or “life-shortening” nor should we make empty promises. Use words like healthier rather than healthy, more likely to live a long time rather than will live a long time.

We need to try our best not let our own fears and worries show- both in our words and body language. Children pick up on (and tend to mirror) their parents’ emotional cues especially when they are young. If you are having trouble controlling your own emotions about these tough issues, grief counseling might be helpful.

For younger children, use pictures to help them learn about CF. Books I like are:  ”Mallory’s 65 Roses”, “Cadberry’s Letters” and “Taking CF to School.”  CFVoice.com has great resources by age for children. There are also good videos available for young kids and I have them on my website at www.TipsForCFParents.com under “Teaching Children About CF.” With older kids and teens, CFF.org has good videos to learn about CF. Watch them with your teen; you can learn together and be there to answer questions.

At some point we do need to address the issue of life-expectancy if it doesn’t come up naturally. Hopefully this will be clarified by around the age of eight (around 3rd grade) depending on the maturity of  your child. This might sound young to some of you but we want our children to hear this information from us, not on the playground or online. And sadly, this does happen. I’ve heard so many stories of kids who become terribly upset after hearing this from a thoughtless peer at school or on Facebook. It’s best for parents to be in control of how this information is delivered.

One way to address this issue is to ask your child questions to open up dialogue. Some examples are:

• “How much do you know about CF?”
• “How are you handling it?”
• “Is there anything about CF that worries you?”

When your child asks a tough question and you are at a complete loss for words, it’s perfectly okay to say something like, “Wow, that’s a really good question and I want to do a good job of answering it. Let me think about it and then get back to you in a little while.” Just be sure you do- preferably over a big bowl of ice cream or other special treat that you both enjoy.

With a little awareness and preparation, you can make talking about these difficult issues a positive experience. Relationships can grow closer when people go through tough times together.

*************

Lisa C. Greene is the mother of two children with cystic fibrosis, a parent educator, certified parent coach, and public speaker. She is also the co-author with Foster Cline MD of the award-winning Love and Logic book “Parenting Children with Health Issues.”   For more information, see www.ParentingChildrenWithHealthIssues.com.

For more Tips for CF Parents, visit Lisa’s website at www.TipsForCFParents.com.

© 2011 by Lisa C. Greene and Foster W. Cline, MD. All rights reserved. Permission granted to Blooming Rose Foundation to reprint.

Josh Mogren’s Answer: In the CF world, those deep conversations seem to come earlier than others. Most of the time you barely have to scratch the surface of the “CF conversation” to satisfy their curiosity, but some children have greater understanding and want more info particularly when they notice the differences they have with their peers.  Every child’s intellectual and emotional IQ is unique, so the success of those conversations depend on you and your relationship with them. You’ll know the best way to reach them. You may not think you will…but trust me…the right words will come when you need them to.

That being said, using words like “fatal” or “life threatening” will more than likely cause your child stress, while empowering them by reinforcing good health care habits—taking meds every day, and doing therapy as directed—and reminding them that they do have some control over their situation and instill the confidence that all children need at an early age. I was always told that I was a regular kid with a little extra to deal with every day.  I wasn’t worried about all the meds and the treatments because my parents didn’t make a big deal out of it. They just let me be a kid. I played sports and got into trouble. I had friends come over and play games while I did therapies like it was something everyone did.

It wasn’t until I was 14 and my sister was really sick that ever talked about the realities of this disease. We talked about how everyone has a different CF journey and—CF or not—we all will eventually pass away. Even though Angie passed away at 16,  I needed to know that my CF was different from hers and I had a long life ahead of me and my parents were strong enough to share that with me. It was a conversation that I’ve always appreciated, but has always made me wonder why my sister and I were so different even though we came from the same parents.

Their is no perfect answer, but the best advice I give to parents is to Know your children and how they respond best and be honest and always optimistic, but never forget to be realistic. And, no matter what happens, always promise to walk beside them in their CF journey.

Thoughts whirling through my mind…..CFer…..cyster…..fibro…..why the labels? Is it a need for camaraderie? A sense of belonging, understanding? Or is it just a label that does more damage than good? I do not know, I am truly asking the question. I know only two things for sure. First, that I had the philosophy drilled into my head in the school of Social Work that NO ONE should ever be defined by what they have but rather by who they are. It is the idea that a person who lives with depression is not a depressed person, but rather someone that deals with depression, depression is not what they are but what they have. So, why do we as a community, and a very empowered and gifted community at that, find solace in defining ourselves by our (or our child’s) disease?
Second, since the CFRI conference, I decided that I was going to be more dedicated to blogging, not in a way to just spew crap…but in a Josh, Mandi, Ronnie kind of way. A way that makes a difference, if possible (for me!). Within two weeks, I have received three emails. One from a woman who is amazing, not because she is past her “life expectancy,” not because she is a post transplant survivor, but because she is Marianne Phillips Bertucci and she is a beautiful and incredible person. One from Josh…need I say more? He is my hero in more ways than one and not just because he has a perspective on CF that I hope Maylie embraces, but because he is and always will be one of the people that I feel closest to in this lifetime, for many reasons outside of CF. And finally Brandon, who I truly no very little about except that he is from the beautiful “Big Sky” and he sent me an email that made me feel proud, excited and proud again to be in the family of CF. I do not feel like anyone I knew twelve years ago when I was his age was even close to being as intuitive and insightful as he is. Below are quotes from these three incredible individuals. If you have CF and like to label yourself with these words….please tell me why. If you are a parent, does this evoke change in how you refer to your child? It did in me. I am just wondering…..

“I have been thinking of the issue of people using the term cystic, cysters, cfer’s and so on when describing someone who has CF. My main concern is I don’t want children growing up thinking that is their identity and that they can’t achieve their goals and dreams. I never thought I would live to be 45 or to see my son graduate high school. I have a strong will and that is what I want children to know is that they can achieve what they put their mind to.” – Marianne Phillips Bertucci

“CF doesn’t control who we are, we decide who we want to be. Yes I know what could happen later on in life, but I never let that stop me, I love my life and would never change anything about it. I have the greatest doctors, the bestest friends, and most of all the most amazing family, that I would never give up if I could change my life. The world is amazing and everyday I love it even more, and people can say CF can ruin ones dreams, but it doesn’t, it gives us more motivation to prove to people that we can do more than what we’re supposed to, and there are no boundaries for us or anyone, we can do whatever we want in our lives, we just have to believe in ourselves.” – Brandon Bagley

“This weekend convinced me we are no different than anyone else. The Jones’, Sarah M., the Easts, and Josh and Carly are just people. Not CF people. Just people and that is a very wonderful thing.” – Josh Mogren

The Tylenol recall is FRIGHTENING to say the least. I am so grateful to have an “aunt-in-law” who works at OHSU in Portland and has connections with the CF docs there, the following is an email from one of the leading Pulmonologists. Hope this helps to ease some of the anxiety….

” I wanted to do a little reading on the recall and give you a more informed answer. Based on what I’m seeing from the FDA and the press releases, I would assess the risk to this child as extremely low.

Evidently, the B. cepacia was found on a drum used to transport raw materials – from a subcontractor/vendor that supplies the manufacturer. The actual manufacturing plant did not have any positive cultures for the bacteria. No reports of contamination down the line have surfaced – evidently the bacteria is not appearing in Tylenol bottles or packaging.

To be sure, B. cepacia is dangerous to CF patients; it’s a particularly resistant bacteria, causes significant lung damage over time, and is associated with increased morbidity and mortality. It’s usually obtained from normal environmental exposure and patient-to-patient contact, spread through droplets. Thus, as an educated guess, if it were present in packaging for Tylenol — and by all accounts, it’s not – the risk would come from handling the product or breathing in its “air”, so to speak. One could argue that having a contaminated Tylenol tablet on the tongue might be a risk for colonizing the throat, and subsequently the lungs, but that’s purely academic speculation on my part, and though theoretically possible, it’s highly unlikely.

The long story short, I don’t think there’s cause for alarm: There’s no contamination of the product, and if there were, the chance of getting pulmonary colonization from it would be very remote.”

I want to take a moment to do a couple of things. Both of which I have been meaning to do for a long time. I want to explain my vision for the Blooming Rose Foundation and what our goals are for the upcoming future. Also, I want to give some MUCH deserved credit to a number of amazing foundations and individuals who I believe have the power to revolutionize the face of CF. And not with a cure, but with a new perspective of what this disease means for new parents and how to teach our kids how to view their futures and unlimited potential.

Okay, so initially the Blooming Rose Foundation was created to send out packets to families with a new diagnosis in Montana. It quickly became the surrounding states, then the west coast, midwest and east coast. We are now across the nation and Canada and we are even offering support and packets as far out as Sweden, Portugal, UK, Ireland, Australia, Germany, South Africa and the Ukraine. So, my vision has had to adapt and adapt quite quickly. I want to keep my vision of making sure that anyone that has a new diagnosis is not alone. This applies to absolutely ANYONE. We want to be able to support parents in all countries. The information is often very similar or it takes a couple hours of searching and contacting folks to find programs and support systems in the area, but it is not difficult. It also gives me great thanks for our medical care and services her through the Pharma companies. Albeit, I still think Sweden is at the top….I give the US a close second! So, that is our primary service. In addition we want to help by sending baskets to CFers in the hospital. While this is more of a long term goal, I hope to be able to do it from time to time while we wait for sponsorship, ect. We also hope to work on a couple of projects that help to get the word out about CF to those not familiar with it as well as educate those that thrive with CF about the latest social and support networks, assistance programs and lastly we hope to bring a swedish team over  here for a CE conference regarding their practices, how they differ and why their approach is so different from ours as well as their outcomes.

So, that brings me to funding. While we do not have any secured funding at this point, we do have a couple of great fundraisers coming up and hope to find a wonderful, wealthy, good-hearted individual to take on our operational and salary costs. Not that I mind working for free…..

Once we have our operational cost covered our fundraising efforts will go directly to packets, baskets and CE conferences. We also hope to hold a conference at some point for parents and families to learn more about the social, emotional and mental aspects of living and loving in a CF world. All based on the idea of learning to frame your thinking and vision around what you want for your child and what you see for their bright future. This would not be a conference like CFRI or CFF, it would not discuss research at all, the primary purpose would be to have parents in an environment where they can meet, talk and become empowered by positivity.

All other proceeds above our projects will be donated to the CFRI, Inc. CFRI is a wonderful non profit that focuses not only on research, but supporting the families of those thriving with CF. They have given so many wonderful advances in CF research to all our loved ones, but also have an amazing support system for new and veteran families and CFers. We hope to donate a lot to them!!

Moving on……

I want to say that I feel blessed to be a part of this community. CF brought us all together, but I feel like we are now so much more than that and I am so grateful to all of you that I correspond with or who comment on the BRF page. On Monday, there was a virtual meeting between a couple of us non profits that have some similar visions for CF. First of all, I want to say that I still feel in awe that I get to be a part of anything with these folks as they all seem “famous” to me! But, I am so grateful to have their expertise, their support and their friendships. With that in mind, I want to also say that the list of non profits and organizations involved in the CF world that I would hope to someday collaborate with is so incredibly long, but the five of us seem to stay in touch and involved in each other’s projects or hopes for future missions.

(In alphabetical order)

Sarah from Breathe for Tomorrow, who was there in spirit….but we had a good pow-wow the week before!  What an amazing spirit and an incredible mentor for parents and CFers of all ages. I am amazed by all that she has accomplished for families since the launch of her nonprofit.  She has a way of being realistic, positive, hilarious and unbelievably good hearted all in one moment. So, huge thanks for all she does for the CF world.
Ronnie and Amanda (some know her as Mandi) Cystic Life and RunSickBoyRun. Rhondi….what can I say that you all don’t already know. They have an amazing following for a number of reasons, most obviously their unending dedication to the CF community. I am constantly amazed that they actually have time to sleep, eat, or anything else. They pour their heart and soul into these two incredible projects. I am so grateful for Ronnie’s dedication to lifting people up and keeping them up and all the research and honest answers on the life of a 30 year old CFer. Mandi, what a gift she is. The amazing spirit and confidence that I strive for in this life. In addition she is the poster child for the phrase “heart of gold.” I have never known anyone else in the NP world that wants to help with other NPs projects as much as her. She is such a gift to this community and to me. Thank you for pumping me up before soliciting for money!

Emily from Rock CF oh girl, what is there to say. She has an energy and love for this life that is so contagious. Em is one that always supports anyones ideas (no matter how crazy) and then actually ends up convincing us that they are going to be incredible. What an amazing person, the support from her is unending. I am so grateful for her laugh and smile, it has a way of letting people know that life with CF is good, without saying a word. Em, you ROCK!

And last but certainly not least, Josh our fearless leader from Welcome to Joshland. Joshman, is are the most honest (brutally at times) person I think I have ever met. He knows himself and what  he hopes to see in the evolution of this CF community of ours and thinks constantly how he can help make the new generation of CFers a positive one. He gives so much to the new parents and adults, which is amazing but it is the heart that he has that allows him to speak to the littlest ones that are learning what lies ahead in this journey. You give them hope, laughter and smiles when they are doing their treatments or  their visits in the “hole.” So, thank you for your humility and your vision. Your sister is so very proud of what you have done with this life, for that I am certain.

Wishing you peace from Montana~