Recently, two families with newly diagnosed infants with G551D have contacted me with a few questions about CF and Kalydeco. It feels like such an enormous privilege to be able to help those people through such a scary time. Of course, I get to try to explain to them how lucky they are to have been given that G551D, because right now that is like the golden ticket in the CF world. But also, I get to simply help them understand this new CF Club they have unwillingly become a part of. This blog is dedicated to "the newbies" and anyone else who just wants to know some of the tricks of the trade I have learned to make CF life healthier and easier :). These are my top 10 "secrets of the pros" that I wish someone had told me about when Brady was diagnosed:
1) Invacare Mobilaire 50 psi compressor. Insurance might not cover this compressor, but I swear it is worth every penny out-of-pocket. We bought this bad boy when Brady was about 9 months old and it is still going strong today (Brady is almost 5). I love the adjustable pressure feauture, because you can crank it full blast for meds that are not "dose dependent," and adjust the pressure appropriately for other meds that you want to nebulize more slowly to get the maximum dosage out of. It is more powerful (higher psi) than smaller less expensive models and actually blasts medicines into smaller particles, allowing drugs to reach the smallest of airways (*Side note, this is the compressor recommended by Genentech for use with Pulmozyme). http://www.pulmozyme.com/taking/treatment.html. The best thing about this compressor is that it will allow you to nebulize medicines FAST and will cut treatment time for most meds. When you have a baby or toddler getting used to treatments...reducing the time it takes can be HUGE! I give this compressor two big "thumbs up" and if it ever breaks down, we will likely buy another one to replace it! We ordered ours online and I've seen them range in price from about $250-$315 or so. http://www.allegromedical.com/respiratory-therapy-c534/mobilaire-50-psi-compressor-p551975.html
2) Omron Micro Air. Treatments are a part of life with CF. Why not make them as easy as possible? We just recently acquired this handy little device for treatments on-the-go and so far I'm impressed! It takes 2 AA batteries to operate and uses a new "vibrating mesh" technology to break medicines into a fine mist. It is seriously almost SILENT when it is nebulizing a medicine! We used it on our recent camping trip and it was really awesome to watch Brady sit shirtless and do his treatments in the woods! I can't comment on the durability yet, because it is still new. I like having this device around and wish we had gotten it sooner!
There are lots of different ways to keep your nebulizer equipment clean. This is how we do it. First, wash the neb parts in warm soapy water (I use a clean paper towel to wipe down parts), rinse well, and then place them in the electric steam sterilizer http://www.amazon.com/Philips-AVENT-iQ24-Steam-Sterilizer/dp/B001C3MHF0. It takes 6 minutes to sterilize. Then I move the neb parts to the Germ Guardian, http://www.onestepahead.com/catalog/product.jsp?productId=487759 which uses dry heat to kill germs and as a side-effect, quickly dries the nebulizer parts with hot air. **Always make sure to handle nebulizer parts with freshly washed hands!! After the parts are dry, I toss them in their special tupperware in the cupboard. Voila! Clean and ready to use again!
4) Power Inverter for Car
We recently got a power inverter installed in our vehicle and I wish we had done it a long time ago! Power goes out--no problem! You can still do treatments in the car! Travelling and don't want to stop for an hour and find a place to do treatments? No problem! Do your treatments en route. We bought our inverter and cables online and had a local stereo shop install it for us. We got this inverter: http://www.theinverterstore.com/1250-watt-power-inverter.html You have to order the cables separately. It is also pretty handy to have a power source in your car when you need to plug-in the air pump to inflate your camping mattress or water toys at the lake :).
|We had our inverter installed under the driver's side seat, so the plug-ins are easily accessible for Brady to do his treatments in the backseat while we are travelling.|
5) Hand Hygiene
This is the #1 way to prevent the spread of germs and I can't underestimate the value of maintaining really good hand hygiene for your CFer. For us, this means that I pack hand sanitizer in my purse and use it frequently when I'm out and about. I use my own pen rather than the one at the counter. I keep hand sanitizer in the door of my car and use a bit everytime I get back in the car from being in the grocery store etc... In our house, we wash everytime we come in from being outside (playing, running errands, whatever). I installed paper towel dispensers in the kitchen and bathrooms in my house. Hand towels remain damp for long periods of time and get used by everyone. They are bacterial traps and we don't use them. Using a clean paper towel to dry your hands and then throwing it away is a much more effective way to keep germs from getting around. I found a company that makes a less industrial looking paper towel holder and I buy paper towel refills at Costco. http://brickhousedispensers.com/
6) Home Hygiene
I realize that many of the bugs that can affect CFers are ambient in our environment and cannot ever be completely eliminated. With that said, there are many things you can do around the home to reduce the concentration of the most harmful bacteria and reduce the spread of viruses. Brock and I team up for our cleaning routine every single weekend. For us, cleaning is less about tidying up and more about killing! Our weekly routine includes:
*Wipe down all doorknobs, kitchen cupboard handles, lightswitches, cell phones, remote controls, and any other frequently touched items with a disinfecting wipe.
* Vacuum carpeted areas with our UVC vacuum (UVC is a wavelength of light that kills bacteria, viruses, mold spores, and dust mites upon exposure).
* Steam clean our hardwood and ceramic tile floors with the trusty Steam Shark (steam mop). I love cleaning with UVC and steam because no chemicals are involved and there is no irritating smell left behind.
*Clean showers and sinks frequently (any place that remains wet for longer periods of time is going to be a bacterial "hot spot"). It might be worth paying some special attention to your showerhead with a bleach/water soak every few weeks. http://www.boston.com/news/nation/articles/2009/09/15/bacteria_in_showerheads_may_be_harmful_study_finds/ It is extremely important to follow package instructions on cleaning products for appropriate disinfection. Products that are not left on long enough or applied in the appropriate concentration can actually contribute to bacterial resistance.
*We wash Brady's clothes, bedding, and towels in HOT water. Laundry can be another unexpected source of bacteria. It is important to move wet clean laundry to the dryer right away after washing, and dry completely. http://abcnews.go.com/Health/Wellness/washing-machines-loaded-bacteria-dirty-clothes/story?id=10751420
*No shoes in the house. We remove our shoes at the door to avoid bringing germs and bacteria into the home via the bottom of our shoes. We ask our guests to do the same. I keep a small basket of slippers and cozy socks by the front door in the winter so guests can keep their feet nice and warm.
If anyone wants more detail on our cleaning schedule or UVC technology, I wrote a whole blog about it and even made a little video! http://luckycfmom.blogspot.com/2011_04_01_archive.html
7) Smart Exposure
As much as I'd like to, I can't control the outside world. I try to limit Brady's exposure to germs in public places with smart scheduling. For example, I always make Brady's Dr. or CF clinic appts. first thing in the morning, when the waiting room isn't full of sick kids and the exam rooms haven't been recently occupied by patients. In the middle of winter (cold and flu season), I don't take Brady with me to the grocery store during the busiest times of day, when the store is packed with people. We schedule our trips early in the morning or on Friday or Saturday nights (when the rest of the world seems to have other/fun things to do!). Our friends are fully aware of our "sick policy," which states that if you are currently ill...we don't want to hang out. Let me stress that we DO NOT LIVE IN A BUBBLE. I just try to make smart decisions about Brady's exposure and I think it has gone a long way.
8) The Bravery Box
I keep a special box of toys and books to use when Brady has to undergo an unpleasant medical procedure like a blood draw. I tell Brady what I'm offering (a book or little stuffed animal he has been wanting) in exchange for his bravery and holding still during the procedure. Most of the time, he can't wait to get to the lab or wherever so he can get his reward! The rewards don't have to be expensive, just whatever you know your kid will go the extra mile for! The Bravery Box has made our lives so much easier and Brady typically feels really heroic and awesome after he successfully earns a bravery reward!
9) Praise Praise Praise Compliance to Breathing Treatments and Meds.
From the time Brady began breathing treatments at 6 mo. of age, we have tried hard to make treatment time special. We pay loads of attention to Brady during this time--because every kid wants attention from their parents! Brady gets to watch whatever he wants on TV during treatments and I usually sit with him and massage his legs. Figure out what YOUR kid likes best and praise the crap out of them when they do things RIGHT! It is so easy to wait until they start misbehaving or throwing a fit for treatments before you pay attention...but it is more effective to simply praise them when they are being responsible by taking all their meds and doing great! We still give Brady a high-five and tell him how proud we are of him after every single treatment session! Not only has it kept him healthy, it is a great confidence builder because staying compliant to treatments is hard work and he knows it!
10) Don't Forget about Nutrition
Most CF patients visit with a dietician during clinic. We know that our CFers need more fat and calories...but what about actual nutrition?? Doctors in Europe pay closer attention to the nutrition aspect of CF and encourage a "Mediterranean style" diet rich in healthy fats. Here in the U.S., Doctors tend to focus more on quantity vs.quality of calories and food consumed. The problem with this is that the CFers need more than just extra calories and fat--they also need more NUTRITION! CFers struggle to metabolize fat soluble nutrients in particular--vitamins A, D, E, and K (hence the specialty CF vitamins). A diet rich in antioxidants is vital to maintain good health for ANYONE and is particularly important for individuals with CF. Brady isn't always cooperative with his diet, so I give him some antioxidant supplements to help fill in the gaps. I try to focus on healthy sources of fat like those found in avocados, coconut, nuts, and seeds rather than relying on junk food filled with sugar and unhealthy trans fats. We like JuicePlus Chewables (whole food gummies made with fruits and veggies) https://www.juiceplus.com/nsa/content/OtherProducts.soa. I'm also a fan of the supplement NAC (N-acetyl cysteine) . It is a powerful antioxidant that is most effective in the effervescent form sold by Bioadvantex (NAC is an easily oxidized compound. Bioadvantex produces its NAC in an oxygen-free environment to preserve potency). http://www.bioadvantex.com/us/pharmanac.html My husband and I are also big proponents of juicing fresh fruits and veggies. We juice every day! Brady still turns his nose up at our green juice blends...but I think adding concentrated nutrition to the diet through juicing would be beneficial to ANYONE--especially CFers!
I hope people find some useful tips in this entry! These are the things that have made our CF lives easier! Remember that this isn't necessarily the "right" way to do things...it is just what works for us!