loveandcf

Having test run is another thing that goes hand in hand with CF. In the post I will introduce and explain the most common test done.                                                                                                                                                                        

Test to Diagnose                                                                                                                                                                         Sweat Test- Considered the best way to diagnose CF when done correctly. This test measures the amount of salt chemicals in sweat. This test may be done as early as 48 hours after birth  but may need to be repeated due to newborns not producing enough sweat.

Genetic Carrier Testing- This test looks at a persons DNA an is able to determine if this person is a carrier of the altered genes causing CF. This test is useful for any and everyone considering or trying to conceive. This test is done by scraping cells from the inside of the mouth or by drawing blood.

Newborn Screening- A test to detect the possibility of having CF early on. The earlier CF is diagnosed the less damage may be caused. For this test blood is drawn a couple of days after birth and sent to the lab to be checked for high levels of  IRT (enzyme created by the pancreas).                         

Pulmonary Function Test                                                                                                                                                              A group of tests that measure how well the patients lungs are working. In the doctors office you will hear alot of random letters used during the test or when reading the test that may not make sense to you if your new but I will add my own little glossary in a post =).

Spirometry- This is the most common test for measuring breath. During this test the patient will take the deepest breath they can, and then exhale into the sensor as hard as possible, for as long as possible. After repeating the test a few times to insure consistency the test will show the amount and speed of air that can be inhaled and exhaled. This test is highly dependent on patient cooperation and effort and may be difficult for small children.  

Lung Volumes- This is a set of test where 1 of the 3 may be done or all 3 will be done in attempt to measure the amount of air.                                                                                                                                                                                                       1. Nitrogen Washout – done by normal breathing of pure oxygen while exhaled gas is collected and analyzed.                   

2. Body Box – Test done is while sitting in an enclosed chamber and asked to perform a series of very small panting breaths. This is the most accurate way to measure lung volumes.                                                                                             

3. Helium dilution – Normal breathing of gas mixture of helium and oxygen.

Blood Test                                                                                                                                                                                      Arterial Blood Gases- This test shows how well the lungs are getting oxygen into the blood and carbon dioxide out of the blood. For this test a sample of blood is drawn from your artery, from your wrist or elbow area.

Diffusing Capacity-This test measures how well gases such as oxygen move from the lungs into the blood. The most common way to do this test is the most common way is the ten second single breath-hold technique. This test will tell you about the amount of damage where the air and the blood meet.

Theses are just some of the many test A CF patient will do. The more I write about CF the more you should see how strong a CF patient must be. Between the treatments, feeling bad, hospital stays and test it leaves little time for personal or play time. Its time right now to find a cure! Thousands of precious people are depending on it and every second counts! Please visit www.cff.org today and find out how you can help.

So now that we have established the core parts of CF lets talk the “other” parts of CF

The things you can and will find if you search “complications of Cystic Fibrosis” will be along the lines of Respiratory complications,  Nutritional complications and Reproductive complications. Much like my last post this blog is not about what you can already find but about my personal experience and opinion. CF is a very complex and misunderstood disease, it takes time, a whole lot of reading, asking questions and it is not something I can explain in 1,2 or even 5 post so know now that this blog will be broken down in many many post and it will take many detours from the original path but that’s what is necessary when attempting to explain or give a better understanding of something as complex an CF.

When you hear lung infection you automatically know that’s not good but again its a bit more complex than that. When a CF patient hears lung infection here’s some of the things they get to think about.  Aspergillus- A nasty fungus that’s present in everyday life, harmless to most people, impossible to avoid and detrimental to 15 % of CF patients, Pseudomonas aeruginosa- Most common recurring bacteria in CF patients, near impossible to get rid of and responsible for 60% of deaths in CF patients, Burkholderia cepacia- I call this the “devil germ”  its a group of bacteria that is found naturally in wet dirt, soil and rotten plants. This bacteria is possibly one of the greater of the many evils and leads to damaging lung infections and deterioration of lung function. MRSA- common bacteria that normally lives on the skin or in nasal passages and can enter the body easily through a cut or sore causing major staph infection for most CF patients. All of these germs and bacterias just add insult to injury so to speak. Now that you see what we thought was just a already scary lung infection is an even scarier, nastier lung infection let me tell you that when a CF patient is growing most of the above germs then not only feel worse than normal but they cant be around other CF patients in fear of spreading the germ. Sounds kind of crappy huh? But what I didn’t express is that some if not all of their closest friends are in fact of CF patients. Selfish..I told ya. CF is now not only taking their breath but its resisting treatment and secluding them from their friends.

CF don’t stop here though.                                                                                                                                                               Due to thick mucus secretions that clog or “stop up” the ducts in your pancreas fats and proteins don’t reach the intestines meaning gaining weight is often a up hill or loosing battle. Sounds so good don’t it “Eat whatever and stay thin” well how does feeding tube sound? In my household the worst issue with eating is when hunger is just not present. It don’t matter if your hungry or not. You must eat and you must eat excessive amounts to attempt to maintain a healty weight. The pancreas is also a huge affected area in most CF patients. Because the pancreas controls the level of sugar in your blood, up to one in five people with CF may develop cystic fibrosis-related diabetes. So now they eat, eat, eat and have to take insulin, insulin, insulin. In addition, the bile duct, the duct that carries bile from your liver and gallbladder to your small intestine, may become blocked, causing  liver problems. Now we are up to scary lung infection, secluded from friends, eating until you could pop, giving shots of insulin and possibly on the fast track to a no good liver. Shall we keep gonging?

CF goes into a personal zone and affects the reproductive system. The same thick mucus secretions I mentioned above also block the tube connecting the testes and prostate gland making many CF men infertile. However certain fertility methods and surgical procedures may sometimes make it possible for men with CF to reproduce. Women with CF may be less fertile than other women, it’s possible for them to conceive and to have successful pregnancies but not without the chance of affecting up the symptoms and causing great risk. Here we are again with a simple solution right?  Take birth control. Oh except I forgot to add most oral contraceptives aggravate certain symptoms of CF. So is the right path to take the path that says you can not naturally conceive a child because of CF. The same CF that has caused the scary lung infection, temporarily kept some of your friends away, made you eat when your not even hungry, made you give yourself shots and is messing with your liver?

Where will it end?

Check back tomorrow for part 3 =)

XoX

  When you hear Cystic Fibrosis what do you think?

If like most you possibly think nothing past “oh their sick” until now.   Allow me to explain exactly what Cystic Fibrosis is. Cystic Fibrosis also known as CF is  an inherited chronic disease that attacks the lungs and digestive system mainly.  About 30,000 adults and children in the US are affected by CF and struggle everyday to breath, eat enough food and maintain general health. Symptoms range from salty skin to intense lung infections to difficulty in bowel movements and are far from the same in each patient.

Clear understanding right? WRONG. What you just read is no diffrent from any other article you can pull up in your search engine of choice so now let me ask again.. What do you think when you hear Cystic Fibrosis? Up to speed a bit now and you may think something along the lines of “A lung disease” or “A disease that affects the digestive system” or possibly both and you are very right but still so very much in the dark.

The following paragraph is from a 1st person point of view, it is in no shape form or fashion from a Doctor a nurse or even a CF patient. It is however from a person that for the past 6 years has seen many many up’s, down’s, near death experiences, un answered questions and a massive amout of questions, studing, reading and personal times.  I now will explain to you what CF is in real life, no text book stuff here only me, my terms and the ocassional input from the patient himself.

Cystic Fibrosis is a selfish, careless, nasty and ugly disease. It creates unbelievably sticky mucus all over the body mainly in the lungs.. Its so sticky patients have to do medicine in aerosol form numerous times a day to loosen the mucus, after completing the aerosols they need some sort of CPT ( chest physiotherapy or chest percussion ) which, can be done in several different forms but bottom line is something or someone has to beat on their back and sides to knock mucus from the lungs making it possible to cough up. Aside from the aerosols and CPT they generally have more than a handful of pills to stat the day and another hanful for the night. Alot of pills right? Well that was before I got to tell you about enzymes.. CF causes some major problems for the digestive system including but not limited to: Loose greasy bowel movents, frequent bowel movements ( like 12 a day ), pain, and the inability to retain calories.. To help with the said digestive problems they have enzymes.. Some patients take 1 per meal some take 12 per meal..Are you following?? Everytime a CF patient sits down for breakfast, lunch or dinner they take another handful of pills. Now were looking at alot of pills but we must keep going because oral meds only get a CF patient so far. Due to the severity of the infection in the body IV antibiotics are often needed..Simple right? WRONG again. More than half of the bacteria and germs that grow in a CF patient are resistant to most antibiotics making it quite difficut to treat and get rid of.

Moving right along..CF also affects the sinuses causing Chronic Sinusitis. We all know how a sinus infection feels right? Now triple that pain and stuffy nose add in a severe headache and make sure you realize nothing OTC is going to help you, chances are perscription meds are not going to help you out much either. Surgery however will make you feel some better for a little bit. But of course after your 5th or 6th or 11th surgery it should all be a piece of cake right?  How about we throw in some Diabetes and some Arthritis or maby I should mention the countless other surgeries like getting a port ( is a small medical appliance that is installed beneath the skin ) in attempt to make treatment easier or a bronchoscopy to help clean out the lungs or a nice pulmonary embolism done through the groin.. Let me not fail to mention the insane amount of time blood work that must be done or the feeding tube that is often needed.

Now what do you think when you hear Cystic Fibrosis?

Stay tuned for part 2 of “Understanding Cystic Fibrosis”

XoX