The new triple drug for CF has been approved! You can read the FDA article here. It is supposed to help about 90% of the CF population. It is for my CF mutations (double delta f508). The name of it is Trikafta (elexacaftor/ivacaftor/tezacaftor). I can’t help but to think trifecta when I see the name…haha. Continue reading
I have not been feeling good for about 3 or so week, maybe more. I did the 2 weeks of IVs 2 weeks ago and wasn’t much better but that is probably due to the fact that my pseudomonas is multi drug resistant. We are limited on what will “work” on the stubborn drug resistant bug (pseudomonas) in my lungs. I made the dreaded call after the 4 days of 40mg of prednisone and no improvement. I need something more therefore I had to call with all the I have coming up. I have a busy 5 weeks ahead. Next weekend we are going to the Zac Brown Band Concert, a bucket list item for myself. Then I have a girls trip to the Gulf Shores the next week. Then in 5 weeks we have our good friends wedding weekend. Continue reading
Cystic fibrosis (CF) is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:
- clogs the lungs and leads to life-threatening lung infections; and
- Obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.
It’s order time folks!
Please share this on your facebook, twitter, instagram and other social media so more people can see it and get Shannonigans gear! Shirts will not be printed and sent until the deadline date of May 17th, so be patient. I set the date out a little bit (Sunday May 17th…38 days to order) to give everyone a few paydays in there. Now buy and share it with your friends and family!
This is what the shirts will look like. PLEASE share this with your friends and family as well. The more shirts that are sold means a bigger donation to the CF Foundation! I really need this to be a good fundraiser as well as getting Shannonigans and CF awareness out there. Thank you for your support!
Until next time…
CFRLD/CFLD or CF related liver disease is yet another common thing in CF. Mild liver involvement with abnormal liver blood tests or fat in the liver is common. In some patients, the liver problems caused by CF are much more important than the lung disease. Advanced liver disease in individuals with CF is called CF liver disease (CFLD). Severe scarring of the liver, called cirrhosis, can occur in CFLD. Many CF patients with liver involvement do not have any symptoms. In patients with advanced CFLD, patients may have a large liver and spleen. Problems with weight gain can occur. As the scarring in the liver becomes more severe, the pressure in the vein leading to the liver (the portal vein) rises and complications of portal hypertension can occur. These can include a large spleen, fluid accumulation in the abdomen (ascites) and bleeding from dilated veins (varices) in the esophagus or stomach. Rarely jaundice and bleeding problems can occur. Basically because of the thick sticky mucus it makes it hard for all things to flow and work correctly in our bodies. They can do a abdominal CT scan to see if there is any narrowing of the ducts and other areas affected. Continue reading
The last thing I will talk about is infertility and all that goes along with that. Unfortunately, men with CF get the raw end of the deal. Upwards of 97% of men with CF are infertile. That’s not to say that through the magic of medicine that they can’t reproduce. It just take a lot more time, effort and money to do so. Once again the dang thick sticky mucus puts a stop to things in our bodies. There is mucus throughout your body which is to include the cervix of women. With that said, the thick mucus doesn’t allow the sperm to get where it needs to go to fertilize the egg. The egg itself might have trouble traveling down the fallopian tube also. So needless to say it could take a real miracle of God for some CF women to get pregnant. Continue reading
CFRD or CF related diabetes is also common in people with CF. Due to the pancreas not functioning properly, it throws many things off whack, to include blood sugars. People with CF take a lot of steroids like prednisone to help with inflammation in the lungs. Steroids throw blood sugars all out of sorts, usually resulting in really high blood glucose numbers. With the diet needs in people with CF the amount of monitoring of blood sugars is high. Our bodies pull so much from us to fight infection and just to breathe that we can bottom out and have really low numbers and also go really high in the matter of hours. Continue reading
Nasal polyps are very common in CF. I, myself, have had a number of sinus surgeries to remove polyps. I have lost count how many surgeries. During the surgery they go up through your nose into the sinuses and remove all the polyps and make bigger openings if needed. It is not a fun surgery by any means.
There have been cases where an ENT (Ear, Nose and Throat) doctor has noticed polyps and suggested that the person be tested for CF because polyps are so profound in people with CF. Then they tested positive for CF. It is interesting how different signs/symptoms are prominent in some and nearly undetectable in others. The reason for that is that CF is very unpredictable. Continue reading
People with CF are very prone to having osteoporosis, or any degree of osteopenia. Osteopenia is a condition where bone mineral density is lower than normal. Osteoporosis is where there is a loss of bone mass and density. This means that you are more prone to fractures. CFers are prone to these bone conditions because of the absorption issues with digestion. We don’t get all of the nutrients from the foods we eat because they can’t be digested correctly. Most all CFers should take a multi-vitamin, whether it is the vitamins specifically for CF or an over the counter one. Extra vitamin D and calcium should also be taken to help keep bone health strong. Continue reading
CF is not only a disease that affects the physical body but also affects your mental/emotional state. Many people with CF have depression and/or anxiety or other mental health issues. It is a hard fight mentally as well. I have not been shy with my depression diagnosis. It hasn’t always been peaches and cream. It is a fight. I have clinical depression. There others that have situational depression meaning that depression takes over when they get sick with a CF exacerbation or other CF related health situation. There is no shame in having Depression but there is a lot of stigma that comes with it.