The gut (belly/bowel/intestines) are greatly affected by CF. There are a lot of issues that can come up. Like I have said before, not all CFers have the same symptoms and problems, nor do they have them at the same rate/severity. I am going to add some pictures to help describe all of these gut related this. The pictures will help describe some of the different aspects of these areas that are affected. I have included information about feeding tubes because maintaining weight is something many pancreatic insufficient CFers deal with. We have to keep the weight on so that we can fight infections. Continue reading
The lungs are a delicate thing. They are sacks of air that keep you alive. It is an understatement to say that breathing with crappy lungs is hard work. There are many things besides just the standard crappy CF lungs that can happen in someone with CF. A short list is the following; muscle/skeletal pain from all the coughing, collapsed lung (pneumothorax), pneumonia, pleurisy and lung bleeds (hemoptysis).
One thing people with CF are good at is hoarding bugs in our lungs. If you have seen the Mucinex monster commercials, our bugs are kind of like that but on steroids! They like to hang out in there and not budge. The problem is, that once the bugs are in there, it is hard to get them out. The bugs thrive in a wet environment which is defiantly our lungs. All of the thick sticky mucus in there holds onto the bugs and doesn’t let them go. Therefore we have to take antibiotics to help kill the bugs, whether it is pills, IV or inhaled. It can be a combo of those 3 methods also. Reoccurring infections lead to scaring in the lungs which leads to lung damage. Once again CF is progressive so it will get worse over time. I like to describe my lung infections like this, “it’s like mono (the kissing disease), once you have it, it’s always there, it’s just whether it is active or not.” So yes my lungs will most likely always have these bugs in them but it is whether or not it is making me show signs and symptoms of an active exacerbation (infection). I have not had a clear sputum culture in too many years to count. It was when I was a kid. Don’t worry you “normal people” don’t get bugs like us. We are special. Continue reading
There is no cure for CF. There has been leaps and bounds in research for medicines to help treat CF. They have made headway in research for a cure but nothing solid. The saying, “the cure is right around the corner!” has been around for many many years. My parents heard it when I was diagnosed 34 years ago and “they” (CFF, docs, etc.) are still saying it today. With that said, we are closer to a cure than we were 34 years ago. I don’t think I will see a cure in my lifetime but who knows, someone may surprise me with that. If I can live my life as an example and do my part for research then that is all that matters for the generations behind me. They are doing research with stem cells in hopes of helping correct the defective CF gene, which is promising. Continue reading
Airway clearance is a big part of life with CF. There are many different ways to do airway clearance. Pictured are a few handheld devices. All of these you either inhaled into to expand your lungs or you breathe into to help vibrate the lungs, which loosens up mucus and makes you cough. There is also the vest, which, I use. There are others not pictured or mentioned. The goal of airway clearance is to loosen up all the mucus in the lungs so that you can cough it out. It only has one way out and we have to work for it. I joke that I wish I had a spit release valve, like on a trumpet, so that I could open it and blow it all out. It is a lot of work coughing out all the junk. We don’t have control over when we cough. Well I should say that doesn’t mean that we sure don’t try to control it. My husband likes to park in the “back 40” in parking lots. When he pulls in the spot he says, “AIRWAY CLEARANCE!” (in a sweet singing voice) Exercise is also another great form of airway clearance.
Some CFers dislike the VEST and prefer the old standard of clapping (PD&C=postural drainage and clapping). That is where you lay down or sit up (depends on what you prefer) and someone claps with cupped hands on your back and sides to help loosen the stuff up so you can cough it out. When I was growing up we called it “beating,” heck we still do. Many times I would hear my mom says, “Shannon, come here I need to beat you.” I have been known to ask my husband to beat me. The kids like to joke that “dad beats Shannon and she takes drugs.” There is also a percussor (pictured below) that does the same thing as clapping through vibration. It vibrates the lungs to loosen the junk so make it easier to cough out.
A support system can make or break someone, that is no different and probably more true in people with CF. Everyone needs support in their life. CFers need a lot of love and support as well. Having CF is physically and mentally draining at times therefore we need people around us whenever we need it. I personally hate needing and asking for help. I want to do everything on my own. I’m stubborn like that. It is a real hit to the self esteem when you can‘t do things you used to do or that you want to do and need help. There are some people that can’t handle loving someone that has CF, for whatever reason and that is another knock to the ol self esteem. Continue reading
Very salty-tasting skin (my mom used to kiss my forehead and lick her lips to see if I needed to eat a salty snack to replenish salt)
Persistent coughing (coughing is what we do and we do it well)
Coughing that produces thick sticky mucus (oh the things that come out of our lungs)
Frequent lung infections (frequently diagnosed as pneumonia or bronchitis before CF diagnosis and classified as CF Exacerbation and/or pneumonia after diagnosis)
Wheezing or shortness of breath (a lot of people with CF also have asthma like I do…double whammy)
Poor growth and slow weight gain, in spite of a good appetite, failure to thrive (what lead to my diagnosis)
Frequent greasy, bulky stools or difficulty in bowel movements (oh boy, you don’t even want to know)
Small, fleshy growths in the nose, called nasal polyps (there have been people that have been diagnosed because their polyps led to questioning CF because they are so prominent in CF patients) (I have had multiple sinus surgeries which I will talk about on another day)
Liver issues, enlarged or scaring or narrowing of the ducts
CF affects multiple parts of the body but is mostly known as a lung disease and/or problems with pancreatic/stomach.
With CF comes a lot of medicine and by a lot of medicine I mean drawers, cabinets and storage bins full. Here are a few pictures of my medicine collection.
I thought I would share a little history about Cystic Fibrosis so you can see where it all began. Well, I mean that metaphorically.
In 1936: A Swiss pediatrician first named the disease. He referred to it as cystic fibrosis with Bronchiectasis. A scientist from Columbia University first developed a complete profile and description of cystic fibrosis symptoms.
In 1938: Dr. Dorothy Andersen described the characteristic cystic fibrosis of the pancreas, correlating it with the lung and intestinal disease prominent in CF, but this was not yet much help with treatment.
May is Cystic Fibrosis Awareness month. I will be sharing things about CF that you may or may not know throughout the month. I will start with this little tid bit to make you come back for more. I started IVs at home yesterday, what a way to celebrate CF awareness month huh. I had to access my port so that I could get started. Here is a pic of what the process entails. Continue reading