Cystic Fibrosis My Way: Managing Daily CF Care

Children and families with cystic fibrosis (CF) must carefully manage daily care at home to stay as healthy as possible. In this series of six videos, patients from The Children's Hospital of Philadelphia's Cystic Fibrosis Center demonstrate how to properly perform some common CF therapies at home – while also sharing some of their hopes and dreams!

Topics in the series include: using a therapy vest, huff coughing, using a puffer, doing acapella treatment, taking enzymes, swallowing pills, checking blood sugar levels, and doing chest PT (physical therapy).

Be sure to always consult with your cystic fibrosis care team for individualized instructions on managing your child’s CF.

Transcript

Cystic Fibrosis My Way: Enzymes and Chest PT with Johnny

Ashley Bricker: My name is Ashley Bricker and this is my son Johnny and this is Cystic Fibrosis our way.

Johnny is 5 months old and has been treated here at CHOP for CF since he was five days old. Every day Johnny receives enzymes to help him digest and absorb his food. He also gets chest PT twice a day and we try to make it as normal in our daily routine as possible.

And this is how we do his enzymes and chest PT:

To give Johnny his enzymes we put them on a spoon with a little applesauce.  He receives them before every bottle or anytime he eats. Enzymes have no taste at all, just make sure you don't crush them or chew them. I always wash my hands and remove any rings or bracelets on the hand that I use the percussor with before I start his chest PT.

Johnny's chest PT is done in two-minute increments in twelve positions on his body. We try to make Johnny's chest PT fun. We sing fun songs, we do fun rhyming games and we know it takes an entire Disney show to do all of his chest PT. 

I never do percussion on his bare skin and as he gets older, we'll use larger percussion cups or we can use cupped hands. And if he needs it throughout or after his chest PT we can suction his nose to make sure we get everything out. 

(written note on video) Make sure to also include the lower lobe position during Chest PT. Place the child on his or her stomach, head lower than feet, and percuss above the bottom edge of the ribs.

Everything I do on the one side, I repeat on the other. So that is how enzymes and chest PT are done. 

Johnny is obviously too young for us to know what he wants to be when he grows up but we already think he's a super hero. He's the toughest little kid we know.

Cystic Fibrosis My Way: Therapy Vest and Huff Cough with John

John Palmer: My name is John Palmer and this is Cystic Fibrosis my way. 

I'm 15 years old and have been treated for CF at Children's Hospital for as long as I can remember. But it doesn't get in my way. I just make it a part of my day. 

When I grow up I wanna play basketball for the Cleveland Cavaliers. It's an exciting team.  Maybe play with Kyrie Irving, he's number 2 on the team. If I wanna play basketball professionally, I need to make sure I do my therapy, my nebulizer and my huff coughs every day. Two times a day when I'm healthy and up to four times a day when I'm sick.

Make sure you shake the inhaler before using it the first time.

First I take two puffs of the inhaler — this opens up my airway. Then I hook the machine up. I put the vest on the clamp and then I buckle it. Now I make sure the holes are inside the vest.  After that you have to put your tubes in. Then I put the medicine in the nebulizer. Next I turn the nebulizer and vest on for twenty minutes.  My pressure is 7 and my frequency is 11. That's how fast it goes but every kid has a different setting. Just make sure you know what your doctor told you to do. Sometimes the treatment takes longer if there's more medicine to do. After ten minutes, I do my huff coughs.

When I'm wearing the vests, I get to play with my computer, play with my phone or I do anything I want for the time being; till my vest is done. After ten more minutes, I have to do another huff cough. After I'm done, I take my Flovent and my nasal spray. If any mucus comes up, then I spit it out and then I'm done.

Then I get to have fun and play ball.

Cystic Fibrosis My Way: Acapella Treatment with Emily

Emily:  My name is Emily Apakian and this is Cystic Fibrosis my way.

I'm 17 years old and I'm a senior in high school. I live with CF and I'm treated at CHOP.  When I grow up, I think I wanna become a doctor and cure CF but my dream job is to get my scuba certification and become an underwater photographer.

I have such a busy schedule that I don't always have time to do my vest therapy so I do my acapella treatments instead. It helps get mucus out of my lungs and it's portable too so this is how I do my acapella treatment:

I place my mouth around the mouth piece, making sure that it has a tight seal.  Next I breathe in slowly and hold my breath for two to three seconds.  It's important that you blow all the way out while keeping your cheeks tight so that you can feel the vibrations in your chest. I do this about ten times and at the end I do a huff cough and then I'm done. 

Now I feel better and can get in shape to learn how to deep sea dive. I have so many big dreams that nothing, not even CF is going to get in my way.

Cystic Fibrosis My Way: Learning How to Swallow Pills with Braeden

Braeden: My name is Braeden Cummings and this is Cystic Fibrosis my way.

I'm 5 years old and I'm treated for CF at CHOP. When I grow up, I wanna be a race car driver. Yea, I'm a winner!  I may even be good enough to race in the Indy 500 but I also need to stay healthy. 

I don't like taking pills but I learned to do it so it can be fun. 

Desiree, Braeden's Mom: This is how we taught Braeden to swallow pills:

First we gave him a sip of water so we can practice swallowing. Then we give him tiny candy sprinkles. They're small and they melt in his mouth. Then we move on to practicing with mini M&Ms and Nerds. Once he could do that, he graduated to empty pill capsules.

(written note on video) Children can begin swallowing pills between the ages of 3 and 8 years old. If you're not sure your child is ready consult your doctor.

We taught him to swallow by tilting his head back. You can tilt your head forward or to the side — whichever way works best for you. Doing all these steps first made it easy for him to learn to swallow pills.

Braeden: I just wanna be able to play with my dinosaurs and go to school with my friends. The quicker I can swallow my pills, the quicker I can go have fun.

I hope these dinosaurs don't think I'm a little pill and try to eat me. Oh no! Hey! Get out of here!

Cystic Fibrosis My Way: Using an Inhaler with Jacob

Jacob Harvey: My name is Jacob Harvey and this is Cystic Fibrosis my way.

I'm 13 years old. I have to use an inhaler every day. I like to call it a puffer to keep my lungs healthy so I can breathe easier.

When I grow up, I want to be an astronaut. How cool would it be to go up in a rocket into outer space and maybe one day land on Mars?  But if I'm going to make my dreams come true, I need to take care of myself and stay healthy. So these are the steps that I take to get there:

First, I take the cap off of the puffer; then I place it into the back of the spacer. Next, I shake the spacer and the puffer together for about five seconds. Then I put the spacer into my mouth making sure that my lips make a tight seal; that way I get all the medication.

Next I push down on the inhaler and breathe in deeply. Then I hold my breath for about ten seconds. Finally, after I wait one minute and repeat it again, I'm all done.

Now I get to go play. When I grow up and if I do get to work for NASA, how awesome would it be to be able to find a new planet where humans could live?

Cystic Fibrosis My Way: Checking Blood Sugar with Leah

Leah Cummings: My name is Leah Cummings and this is Cystic Fibrosis my way.

I'm 6 years old and I'm treated for CF at CHOP. I also have CF related diabetes which means you have to check my blood sugar levels every day. 

When I grow up, I wanna be an adventurer. Maybe go on a safari and see all the animals and their natural habitat. My favorite animal's the hippo.

So if I'm gonna stay healthy and get to do all these fun things, this is how I need to take care of myself:

First I take everything out and lay it on the table. Then I clean my fingers, and I prick one finger — then I squeeze my finger to get blood out. Then I put my blood on the test strip. It reads my sugar levels in three to five seconds; 65 — if it's low then I take a sip of juice and I know my body is safe and I can go play.

I think I wanna change my mind and be a rock star when I grow up. Can I do that?

Topics Covered: Cystic Fibrosis

Related Centers and Programs: Cystic Fibrosis Center