I had an excellent appointment today. My PFTs were good. They were about the same as last time. I am very thankful to be stable. I am incredibly grateful to be given the gift of Trikafta. And I would like to keep an exacerbation as far away from me as possible, knock on wood.

There are many countries that need to improve their technology and support systems, and many CFers are still doing their best without Trikafta, without the vest.

Studies and practice have shown that for folks with extremely low lung capacity, Trikafta doesn’t seem to help immensely, and some people feel worse taking it. Many CFers in the U.S. are not eligible to take it because of their genetic mutation, of course.

Physical Therapy!

I also met with the Children’s physical therapist today. I needed to relearn numbers, exercises, and most importantly my focus.  

Breathing

Heart

Strength

So – the most significant breakthrough is I started exercising with a weighted vest. I love it. I went with my gut and thought, this may be the thing to help improve my capacity overall.

Since starting Trikafta and gaining a little weight, my strength and endurance have significantly increased, as has my clothes size. Nothing has moved the meter. I am healthy, and that’s wonderful, and that’s the most important, but I feel better at a certain weight. Since wearing the weighted vest, I have surprisingly nudged the scale just two, maybe three pounds since Christmas. I have to watch that (or eat more), but it proves that the weighted vest makes my body work harder doing essentially the same exercise.

“What you are doing is weight training and cardiovascular at the same time – allowing the muscles to breathe,” said the physical therapist.

I am on the treadmill. I have my neb. I wear my weighted vest, and then I do interval training. I do the same speed but set incline intervals. I have bad knees, so I can only go so fast. I work. My lungs are working harder but in a good way, and that has become a tiny bit easier. I still believe I can nudge my lung capacity.

I am just doing three pounds for now. I have half-pound weights that are evenly distributed in tiny pockets around my torso.

The physical therapist added, “I encourage you to wear the vest for all your exercises.”

She shared some exercises I have attached below. Remember your legs. They are important—also, stretching, of course.

Target heart rate!

Subtract your age from 220, then calculate 60-85% of your target rate.

If you decide to do interval training, try to and step up every five minutes, or do what you can!

We tested my apple watch, it is accurate within a couple beats. She tested my resting heart rate, she counted 72, the watch 75 – on target.

She said, “This here, is the gold.”

Some other things we talked about –

The CF population is doing so well. Since Trikafta, Children’s has closed the CF inpatient floor. It’s incredible!

CFers who come in now, for the most part, are the folks who don’t take care of themselves.  

Since the pandemic, In the general children population, Children’s has seen a sharp increase in urinary incontinence and constipation, as well as a myriad of psychological, physical, and behavioral challenges. Children are still struggling in their daily lives.

And then The Good Work:

Children’s is planning on deploying a CF team to a country that does not have access to the vest or Trikafta. To train and educate patients and caregivers. Some patients still do percussion therapy as their primary therapy. Education and partnerships between patients, caregivers, and corporations can do good with further community outreach.

This is one hospital, one team, one specialty – and there are many hospitals and many teams, nonprofits, and communities that go out on missions for people in need consistently throughout the year worldwide.

Good work and good people moving and creating more good energy, suppressing and eliminating the lack. We just don’t hear enough of it.

Many blessings to each of you – keep moving forward. Always do what you can, and a tiny bit more.

Exercises and stretches:

post script.

I created a better diabetic safety net. I placed this little bucket below in my nightstand. No matter what I do, my sugars still crash at night. My metabolism keeps going. I eat all day and a snack before bed, but sometimes I crash in that 3-4 a.m. typically. I do not like it. I am not going to lie. It doesn’t happen all the time, probably once a month, still too frequent. Instead of double checking that I have a snack in my drawer each night, I now have a better safety net. Water plus pills, of course.