什么是囊性纤维化

There are over 30,000 在中美和个人 70,000 世界范围内,从囊性纤维化患 (CF), 医生诊断有关 1,000 每年新发病例.

CF影响的细胞在体内,使汗液, 消化液和粘液. 一般, 这些都是保持身体平稳工作非常滑,然后系统. 然而, 如果您有CF, 他们变得厚和胶水. Causing these funds to black tubes and ducts within the body.
People with CF have chronic and persistent lung infections that make it difficult to breathe over time.

Over time, mucus accumulates inside your airways; This makes breathing a struggle. The mucous traps germs and leads to infection. IT can also cause severe lung damage like a cyst (fluid-filled sacs) and fibrosis (scar tissue). That is how CF got its name.

Symptoms of CF

People with CF have several symptoms, Including;

  • Very Salty -tasting skin
  • Non-stop coughing, at times generating phlegm
  • Frequent lung infections including bronchitis and pneumonia
  • Poor growth or weight gain in spite of a healthy appetite
  • Wheezing or shortness of breath
  • Frequent greasy, or bulky stools, difficulty with bowel movement
  • Male infertility

What Causes Cystic Fibrosis?
It is not contagious; It is produced by a deviation (change) within a single gene described as the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR,) This controls the flow of salt and fluids in and out of your cells. 如果CFTR基因不作为工作它应该, 糯米黏液整个身体向上安装.
要获得CF, 你必须从你的父母收到基因的突变副本.
如果你只继承一个副本, you will not exhibit any symptoms, But you will be a carrier for the disease. That indicates there’s a possibility you could pass it on to your child one day.
关于 10 million Americans are CF carriers. Every time two CF carriers have a baby, there is a 25% (1 和 4) chance that their baby will be born with Cystic Fibrosis. There is a 50% chance the child will be a carrier but not have CF. There is a 25% chance that the child will not carrier CF gene or have CF.

Which Portions of the body does CF affect?
肺是不是身体的唯一部分由CF影响. CF也影响肝脏, 胰腺, 小肠, 大肠,
和肾脏, 膀胱和生殖器官.
囊性纤维化的人,患肺部感染的风险更大,因为粘液的建立, 创建其中细菌能茁壮成长和发展的环境. 肺部感染主要是由细菌引起的,往往是一个很严重的问题.

  • : 如果管,交通运输胆汁被堵塞, 你的肝脏发炎得到, 和严重的疤痕或肝硬化发生.
  • 胰腺: 该粘稠的粘液在你的胰腺造成CF块管. 这会停止消化酶 (,打破了你的食物中的蛋白质) 到达你的肠道; 结果是, 你的身体有困难的时候吸收它需要的营养物质. 随着时间的推移,这也可导致糖尿病.
    Small intestine- Since breaking down high-acid foods that come from the stomach is difficult, the lining of the small intestine can start to erode.
  • Large intestine: Thincscreations (liquids) in the stomach can make feces very thick. This can trigger blockages, IN some cases the intestine may also start to fold in on itself like an accordion in disorder called intussusception.
  • 膀胱: Chronic coughing weakens the bladder muscles, Almost 65 of omen with CF suffer from stress incontinenceThis means that you leak urine when you sneeze, cough, laugh or try to lift something, Although this is more common in women, it can occur in men.
  • 肾脏: Some people with CF get kidneys stones. These small, hard mineral deposits can generate nausea, vomiting, and pain. If ignored, they can lead to a kidney infection.
  • Reproductive organs: surplus mucus affects fertility in both men and women. Most men with CF have problems with the tubes that transport their sperm, or what;s called the vasa differentia. Women with CF have very thick cervical mucus, which can make it harder for sperm to fertilize an egg.
  • Other parts of the body: Cystic Fibrosis can also lead to thinning of the bones or osteoporosis ) 和肌肉无力. Since CF upsets the balance of minerals in the blood, it can also produce lower blood pressure, fatigue, a fast heart rate and overall feeling of weakness.

Treatments for Cystic Fibrosis:
The type of severity of CF symptoms can differ based on the person. So it’s important to have a treatment plan that contains elements that are unique to each’s situation.
People with CG should work closely with their medical providers and families to create personalized treatment plans.
Look to the CF Foundation, which accredits more than 120 care centers that are staffed by dedicated experts in CF to help care for patients through specialized disease management.

Each day, people with CF typically have a combination of the following therapies:

  • Airway clearance to help loosen and clear away the thick mucus that can build up in the lungs, Some airway clearance techniques may require some help from family, friends or respiratory therapist. Many people with CF utilize an inflatable vest that is worn on the chest and vibrates at a high pulse to help loosen and thin mucus.
  • Inhaled medicines to open the airway or thin the mucus. These are liquid drugs that are made into a mist or aerosol and the inhaled through a nebulizing. Tese medicines include antibiotics to fight lung infection and therapies to help the airways clear.
  • Pancreatic enzyme supplement capsule to help improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF will usually take multivitamins.

Although CF is a serious condition that requires daily care, many treatments for it have advanced. People who have CF live much longer than they use to and the quality of life have improved too.