CF Happens

I had my regular 3 month CF check up today. My lung functions are steady (my baseline = my normal). My FEV1 is 0.87L/32%, which is identical to the last time I did them at the end of October after 3 weeks of IVs.

My weight is down a little more, which isn’t a bad thing right now. When I started taking Trikafta in November of 2020 (if I remember correctly, haha) I started to gain weight, a lot of weight. It’s not about the actual numbers but I’ll share them just to show how much I gained and how much I’ve lost to this point. After about 6 months or so of taking Trikafta I got up to the heaviest I have EVER been at 155-158 pounds! That was a lot of weight on my 5 foot 2 inch body! I was very uncomfortable at that weight. Before I started taking Trikafta, I was around 120-125 pounds. The CF clinic dietitian had me start tracking my calorie intake with an app to see if I was taking extra calories that I wasn’t noticing. Let me just caveat that with, as a kid with CF (in the 80’s) I was told to eat a lot of calories (close to 3,500 calories) and fat to put weight on and keep it on. My CF body burns A LOT of calories just breathing and coughing in general. When I get CF sick, obviously my breathing gets worse (more labored) as does my coughing (increase in frequency and intensity). I need the extra weight to help my body fight infections when I’m sick.

Now back to counting calories…the app I was using had you to put in a goal weight along with your current weight. I wasn’t reaching the goal calories for weight loss according to the goal weight I put in the app. I would get close to the calorie goal but rarely did I go over the calorie goal. This showed us that Trikafta was basically the sole reason for my weight gain. When you take Trikafta you need to take it with fat containing food/drink to help your body absorb the medicine. My dietitian said it was ok to start just taking my morning dose with a glass of whole milk instead of the recommended 20G of fat (or as close as you can get). I believe this change helped contribute slightly to at least not gaining anymore weight.

Fast forward to today…my weight was 133 pounds!! No, I don’t have any idea how I lost the weight because I have NOT been exercising or cutting calorie and fat intake. Honestly, I think my body is finally used to the Trikafta. My body has realized that Trikafta is here to stay therefore it has leveled out in my system somehow. I don’t know how the weight loss happened but I’m not questioning it, I’m accepting it. So to you other CFer’s, I have no idea how the weight loss happened and I don’t have any tips and tricks to give you.

I will also add, skinny people get made fun of just like overweight people do, neither of which is right. I didn’t share my weight to brag or for any other reason but to show you that 30 pounds is a lot no matter what type of body you have. I was made fun of as a kid for being so skinny and it hurt just like when overweight people are made fun of. Not everyone has a choice in the weight they are and it’s not as easy as “just eat me” or “just eat less.”

Now onto all the other aspects of my appointment…I’m holding steady at my baseline (my normal) in all other aspects. Yes, I get short of breath often and need to take breaks but that’s normal for me. My cough is my normal, not increased. My guts are my normal CF guts. As you know CF affects A LOT of parts of the body. The main areas it affects me are my lungs and my digestion (aka pancreas and intestines). There are other areas it affects me but those the most prominent.

It’s a new year which means all of the regular yearly CF testing is due to be done. I like to call it CF maintenance, just like your car needing to go in for maintenance. Haha. On my to do list is a DEXA scan (bone density scan) because with CF I don’t absorb all the vitamins and nutrients that I should because I’m pancreatic insufficient which means my pancreas doesn’t produce all the enzymes it needs to digest my food which is why I take pills (enzymes) every time I eat. Since I don’t absorb everything I need to osteoporosis is possible (which I do already have). Next on the to do list is an abdominal ultrasound to look at the liver and pancreas, both of which are affected by CF. The third and final thing on my to do list is the oh so lovely GTT (glucose tolerance test) to determine if I have diabetes or if I’m close to it.

Diabetes goes hand in hand with CF. In people who have CF it is called CFRD (CF related diabetes) so it’s not the run of the mill diabetes the “regular” people have/get. I know that CFRD is coming at some point for myself. I already am hypoglycemic where my blood sugar will drop somewhat quickly out of nowhere. At that point I need to eat/drink something ASAP to get it back up. Also, CFRD is almost always a guarantee post lung transplant with all the prednisone that needs to be taken as part of the anti-rejection meds. Prednisone messes with your blood sugar a lot. That’s not to say that everyone that is post lung transplant with develop diabetes but it’s no surprise when it does happen.

That about sums up my appointment. All is good. We are all set for me to start IVs (Cefepime 2 grams every 8 hours) at home on the 8th for my upcoming surgery on the 13th. I will do 5 days of IVs before my surgery. I will continue for another 10 days after my surgery for infection control. I will update my blog after my surgery when I’m feeling decent enough to do so.

Until next time…