I had CF clinic today. It was the first time I’ve been seen since I was in the hospital in July with really bad asthma issues. My lung functions were up today. My FEV1 was 34%/.93L. They were 29% in July. My baseline is around 32%, so I’ll take 34%!! My weight was down a few more pounds which is not a bad thing considering I gained close to 40 pounds after starting Trikafta. The weight gain was strictly from Trikafta because I counted calories to try and see if I could adjust my intake to help lose the extra weight. I was barely taking in the number of calories that was the allotted amount for my weightless goal. I wasn’t willing to stop Trikafta to lose the weight. Thankfully my body figured it out that Trikafta was staying for the long haul and it needed to get its sh*t together and adjust my weight. haha. Sometimes you have to decide if the side effects of a medicine that’s helping you are worth it. Risk vs Reward. No, I didn’t gain any lung function with Trikafta like others have but if it means I’m able to put off lung transplant for longer then it’s worth to me.
My lungs sounded good, no wheezes or rattles. That’s thanks to my asthma behaving right now. My doctor got all the notes and labs from Barnes from August when I was last there getting all my transplant testing up to date. Of course he wants a few more labs (vitamin levels being one) that they didn’t draw in the 12 tubes they took. haha. Everyone said I looked great!! They all know I just got back from a trip to Hawaii and that it’s my happy place. I took the phonebook I made of our trip and everyone loved it. They all said that Hawaii obviously did you good. I said of course! There’s no humidity which my lungs are not a fan of, I get to breathe in the salty air and I get to be in the sunshine which are all great things if you ask me. I go back in 3 months for a regular check up. The hope is always that I make it the 3 months without needing to call them to seen sooner because I’m sick. Fingers crossed as always! I also have appointments at Barnes in March for transplant clinic check up.
Ok, I’m going to go off on a tangent for a second (hear me out, it’s going to teach you something)…
I’m going to cover the things that are the regulars of CF world. Not all clinics are the same, so some don’t do everything the same as my clinic do. Although, there are things that CF clinics need to do to make sure they stay an accredited CF center and to get funding. This is NOT the gold standard of CF care but it may look similar to this. At every appointment they do your height, weight, blood pressure, oxygen saturation. Your height and weight are monitored closely because they need to make sure you are maintaining a good BMI. If you drop weight it usually indicates that you may have an active infection going on that your body is using your weight reserve to fight an infection which takes calories to do as does breathing in general when you have crappy lungs. You should see your CF clinic at least 4 times (quarterly) a year. In that you should do at least 2 pulmonary function tests to measure lung function. I do this at every appointment and more frequently when I’m actively sick. You should also get a chest x-ray/chest CT every 1-2 years, sometimes clinics like to do both to have all the bases covered. You get them much more often than that if have an active exacerbations (CF sick). A sputum culture should be done quarterly as well. I do this every appointment and when in the hospital to make sure we are using the correct antibiotics. A bone density scan is recommended every 2 years because with the crappy pancreas you don’t get all the vitamin D and calcium that you need to keep your bones healthy and strong. Also, yearly labs that include vitamin levels (once again thanks to a crappy pancreas) and liver enzymes because a lot of meds are filtered through the liver so it has to work overtime. Plus CF related liver disease is very common in CF. You should do a yearly GTT (glucose tolerance test) to check for CFRD, CF related diabetes. This is one of my least favorite tests because I have bad veins and you have to be stuck at least 3 times and sometimes the glucose drink gives me a headache. Once again thanks to having a crappy pancreas it needs to be checked often. If you have been diagnosed with CFRD you don’t have to do the GTT anymore because you already know you have CFRD. You should have colonoscopies every 5 years after 40 years old unless you have family history of polyps, colon cancer or other colon issues. If you have any of the things I just mentioned, you should do colonoscopies sooner and more frequently. Next up, yearly abdominal ultrasound to check the liver, gallbladder (if you have one, haha), spleen and kidneys…basically anything and everything you have in your gut because CF doesn’t play nice and can affect many different areas in the body. The abdominal ultrasound was just added as a recommended maintenance test a few years ago at the national CFF conference.
I saved an important one for the last one…yearly depression and anxiety screening with the PHQ-9 survey and the GAD-7 (social worker gibberish) survey. Living with CF isn’t for the faint of heart and isn’t not easy. It takes a toll on your mental health. As you know, I’m an open book about my depression and anxiety struggles. I preach to anyone who will listen to me to GET THE HELP you need! Whether that be antidepressants, anti anxiety meds or talk therapy with a therapist/counselor/social worker…or a combo of all three. Having a great support system helps immensely! With them you know that even though you are the one living the day to day physical side of CF, your support system has your back and they are there to help anyway they can…just ask! Something as little as doing carpool for you if you are a parent, cleaning your house for you (even one room), doing the dishes, making dinner, joining you at appointments so you have company because clinic days are long, hanging out on your couch while you talk with the TV on for background noise or even picking up your prescriptions for you (among many other ways to support the CFer) means much more than you know or more than the CFer will let you know.
I cherish my family and friends that are a part of my support system. They help me more than they realize. I am a stubborn patient/person at times (my husband would probably say ALL the time) and don’t like to ask for help. Over the years I’ve learned that asking for help is NOT a sign of weakness. If anything it helps me to be stronger. I still struggle asking at times though. I’m working on it every day. Ok, I think I have “talked” your ear off enough. I hope you stuck it out and read this post ALL the way to the end. As always, I’ll update if/when there’s something to update about.
Until next time…
CF Happens 