Symptoms of Cystic Fibrosis

Table of ContentsView AllTable of ContentsFrequent SymptomsLate-Stage Symptoms/ComplicationsWhen to See a Healthcare ProviderFrequently Asked QuestionsNext in Cystic Fibrosis GuideCauses of Cystic Fibrosis

Table of ContentsView All

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Table of Contents

Frequent Symptoms

Late-Stage Symptoms/Complications

When to See a Healthcare Provider

Frequently Asked Questions

Next in Cystic Fibrosis Guide

Cystic fibrosis (CF) symptoms can develop soon after birth and may include salty-tasting skin, greasy and bulky stools, chronic breathing problems, and poor growth. Because the genetic disease interferes with the flow of water and salt in out and out of cells, it causes thickening of mucus that not only clogs the lungs but prevents the pancreas, intestines, liver, and heart from functioning normally.

Over time, the ongoing obstruction of air passages and buildup of mucus can lead to recurrent infections, irreversible lung damage, and other serious complications such as malnutrition, diabetes, and more.

As a progressive disease, cystic fibrosis needs to be treated early to preserve lung function and reduce the inflammatory burden on the body. By doing so, you can avoid many of the severe complications of the disease and maintain a high quality of life for now and years to come.

Many children today are diagnosed withcystic fibrosisbefore symptoms appear. In the United States, mandatory newborn screening programs can identify CF with one or two simple tests.

Despite the advent of newborn screening, only around 65 percent of children are positively diagnosed with cystic fibrosis at birth.

Among the more common early symptoms of CF:

Children who experience these symptoms will often be smaller and weigh less than other children of the same age. Your healthcare provider may describe this as failure to thrive (FTT).

FTT is more than just a general assessment of your child’s development. It is the decelerated or arrested growth in which a child’s weight and height falls below certain accepted parameters (known as percentiles).

Within the context of cystic fibrosis, FTT is associated with poorer outcomes. In fact, according to a 2014 study published in theAnnals of the American Thoracic Society, FTT is the single leading factor for severe CF lung disease.

The later-stage symptoms of cystic fibrosis are less associated with the disease and more with the damage it has inflicted on organs of the body. While the lungs are primarily affected, the pancreas, intestines, liver, and endocrine (hormonal) system are also commonly involved.

Later-stage symptoms due to complications of CF are often multifactorial with one symptom influencing another, usually for the worst.

Respiratory

When the lungs are clogged with mucus, they can incur damage due to infection, obstruction, and inflammation.

In term of infection, microorganisms that normally inhabit mucus without issue can grow out of control and lead to pneumonia and other infections (includingStaphylococcus aureus, Aspergillus fumigatus, Haemophilus influenzae,andPseudomonas aeruginosa).

People with CF often get recurrent infections which can cause permanent scarring in their lungs.

Meanwhile, the accumulation of mucus in the air passages can increase the blood pressure in the lungs, referred to aspulmonary hypertension. This, in turn, can affect the heart, leading tocor pulmonale(right-sided heart failure).Edema, the abnormal accumulation of fluid in the legs and ankles, is a common symptom.

The accumulative damage can lead to a condition known asbronchiectasis, in which damaged lung tissue makes it all the harder to clear mucus. Symptoms include:

Persistent sinus inflammation can cause tissues to thicken and formnasal polypsin up to 86% of people with CF.Symptoms include mouth breathing, nasal drip, and, in some cases, the complete obstruction of nasal passages.

MortalityRespiratory failureaccounts for roughly 80 percent of deaths in people with CF.Cardiorespiratory complications (heart and lungs) are the second leading cause.

Mortality

Respiratory failureaccounts for roughly 80 percent of deaths in people with CF.Cardiorespiratory complications (heart and lungs) are the second leading cause.

Respiratory failureaccounts for roughly 80 percent of deaths in people with CF.

Cardiorespiratory complications (heart and lungs) are the second leading cause.

Gastrointestinal

The accumulation of mucus can also affect thepancreas, the organ responsible for producing digestive enzymes. The impairment of this function, known as pancreatic insufficiency, is at the heart of malnutrition problems seen in people with CF.

Beyond its effect on digestion, the obstruction of enzyme secretions can trigger the painful inflammation of the pancreas, known aspancreatitis. Symptoms include:

Over time, the pancreatic ducts can become completely blocked, resulting in extensive scarring of the organ tissue.

Acute pancreatitis can trigger fatal complications including heart, lung, or kidney failure.

In addition to the pancreatic complications, a bowel obstruction can sometimes occur, either as the result of thicker and stickier stools (referred to as distal intestinal obstruction syndrome in adults) orintussusception(in which part of the intestine folds into the section next to it).

Thickened mucus secretions can also block bile ducts of the liver, leading to the development of gallstones andcirrhosis. In fact, liver disease is the third most common cause of death in people with CF.

Endocrine

When mucus accumulates on the pancreas, it can block theislets of Langerhans, which are responsible for producinginsulin. When this happens, it can lead to the development of cystic fibrosis-related diabetes (CFRD), a form of the disease with characteristics of both type 1 and type 2 diabetes.

Among the other hormone-related abnormalities:

Infertility

As many as 97% of men with CF will have infertility.

Infertility can also affect women with CF, either due to thick cervical mucus that interferes with conception or chronic malnutrition, which can cause anovulation (the failure to ovulate) andamenorrhea(the failure to menstruate).

Urinary Incontinence

Women with cystic fibrosis who are over age 20 are likely to experience urine leakage to some degree; men do not usually experience this as a result of CF. While the reason why this occurs isn’t exactly clear, it’s believed that prolonged, frequent coughing from the diseases weakens the pelvic floor muscles.

Coagulation Disorder

Symptoms of coagulation disorder include easy bruising, excessive bleeding, bleeding gums, nosebleeds, and bloody stools or urine.

Fortunately, with the implementation of newborn screening practices, children with CF are more likely than ever to be diagnosed at birth and placed on immediatetreatment. With that being said, many kids still fall through the cracks.

In fact, according to theCystic Fibrosis Foundation, no less than 6.8 percent of people with CF are diagnosedafterthe age of 16. There may be several reasons for this:

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Yes. Atypical CF is a milder form of thegenetic disease.The symptoms vary widely. In general, though, there may only be one organ affected. Diagnostic tests, especially the sweat test, may come back negative, so the condition can go unnoticed for years.

You won’t have any symptoms if you are only a carrier, which means that one of your two CFTR genes has a CF mutation. You may pass CF on to a child if your partner is also a carrier. The only evidence that you’re a carrier would be seen ingenetic screening, which can identify up to 90% ofCF carriers.

Causes of Cystic Fibrosis

16 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Cystic Fibrosis Foundation.About cystic fibrosis.Cystic Fibrosis Foundation.Newborn screening for CF.Sanders DB, Li Z, Laxova A, et al.Risk factors for the progression of cystic fibrosis lung disease throughout childhood.Ann Am Thorac Soc. 2014;11(1):63-72. doi:10.1513/AnnalsATS.201309-303OCFilkins LM, O’toole GA.Cystic fibrosis lung infections: Polymicrobial, complex, and hard to treat.PLoS Pathog. 2015;11(12):e1005258. doi:10.1371/journal.ppat.1005258National Heart, Lung, and Blood Institute.What is bronchiectasis?American Academy of Allergy, Asthma & Immunology.Nasal polyps.Martin C, Hamard C, Kanaan R, et al.Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!.J Cyst Fibros. 2016;15(2):204-12. doi:10.1016/j.jcf.2015.09.002Singh VK, Schwarzenberg SJ.Pancreatic insufficiency in cystic fibrosis.J Cyst Fibros. 2017;16 Suppl 2:S70-S78. doi:10.1016/j.jcf.2017.06.011Cystic Fibrosis Foundation.Cystic fibrosis-related diabetes.MedlinePlus.CFTR gene.Cystic Fibrosis Foundation.Fertility in men with CF.Neemuchwala F, Ahmed F, Nasr SZ.Prevalence of pelvic incontinence in patients with cystic fibrosis.Glob Pediatr Health. 2017;4:2333794X17743424. doi:10.1177/2333794X17743424Mcphail GL.Coagulation disorder as a presentation of cystic fibrosis.J Emerg Med. 2010;38(3):320-2. doi:10.1016/j.jemermed.2007.10.038Sathe M, Houwen R.Meconium ileus in cystic fibrosis.Journal of Cystic Fibrosis. 2017;16:S32-S39. doi:10.1016/j.jcf.2017.06.007Schram CA.Atypical cystic fibrosis: Identification in the primary care setting.Can Fam Physician. 2012;58(12):1341-1345, e699-704.Zvereff VV, Faruki H, Edwards M, Friedman KJ.Cystic fibrosis carrier screening in a North American population.Genet Med. 2014;16(7):539-546. doi:10.1038/gim.2013.188Additional ReadingBush A, Sly P.Evolution of cystic fibrosis lung function in the early years.Curr Opin Pulm Med.2015;21(6):602-8. doi:10.1097/MCP.0000000000000209

16 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Cystic Fibrosis Foundation.About cystic fibrosis.Cystic Fibrosis Foundation.Newborn screening for CF.Sanders DB, Li Z, Laxova A, et al.Risk factors for the progression of cystic fibrosis lung disease throughout childhood.Ann Am Thorac Soc. 2014;11(1):63-72. doi:10.1513/AnnalsATS.201309-303OCFilkins LM, O’toole GA.Cystic fibrosis lung infections: Polymicrobial, complex, and hard to treat.PLoS Pathog. 2015;11(12):e1005258. doi:10.1371/journal.ppat.1005258National Heart, Lung, and Blood Institute.What is bronchiectasis?American Academy of Allergy, Asthma & Immunology.Nasal polyps.Martin C, Hamard C, Kanaan R, et al.Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!.J Cyst Fibros. 2016;15(2):204-12. doi:10.1016/j.jcf.2015.09.002Singh VK, Schwarzenberg SJ.Pancreatic insufficiency in cystic fibrosis.J Cyst Fibros. 2017;16 Suppl 2:S70-S78. doi:10.1016/j.jcf.2017.06.011Cystic Fibrosis Foundation.Cystic fibrosis-related diabetes.MedlinePlus.CFTR gene.Cystic Fibrosis Foundation.Fertility in men with CF.Neemuchwala F, Ahmed F, Nasr SZ.Prevalence of pelvic incontinence in patients with cystic fibrosis.Glob Pediatr Health. 2017;4:2333794X17743424. doi:10.1177/2333794X17743424Mcphail GL.Coagulation disorder as a presentation of cystic fibrosis.J Emerg Med. 2010;38(3):320-2. doi:10.1016/j.jemermed.2007.10.038Sathe M, Houwen R.Meconium ileus in cystic fibrosis.Journal of Cystic Fibrosis. 2017;16:S32-S39. doi:10.1016/j.jcf.2017.06.007Schram CA.Atypical cystic fibrosis: Identification in the primary care setting.Can Fam Physician. 2012;58(12):1341-1345, e699-704.Zvereff VV, Faruki H, Edwards M, Friedman KJ.Cystic fibrosis carrier screening in a North American population.Genet Med. 2014;16(7):539-546. doi:10.1038/gim.2013.188Additional ReadingBush A, Sly P.Evolution of cystic fibrosis lung function in the early years.Curr Opin Pulm Med.2015;21(6):602-8. doi:10.1097/MCP.0000000000000209

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Cystic Fibrosis Foundation.About cystic fibrosis.Cystic Fibrosis Foundation.Newborn screening for CF.Sanders DB, Li Z, Laxova A, et al.Risk factors for the progression of cystic fibrosis lung disease throughout childhood.Ann Am Thorac Soc. 2014;11(1):63-72. doi:10.1513/AnnalsATS.201309-303OCFilkins LM, O’toole GA.Cystic fibrosis lung infections: Polymicrobial, complex, and hard to treat.PLoS Pathog. 2015;11(12):e1005258. doi:10.1371/journal.ppat.1005258National Heart, Lung, and Blood Institute.What is bronchiectasis?American Academy of Allergy, Asthma & Immunology.Nasal polyps.Martin C, Hamard C, Kanaan R, et al.Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!.J Cyst Fibros. 2016;15(2):204-12. doi:10.1016/j.jcf.2015.09.002Singh VK, Schwarzenberg SJ.Pancreatic insufficiency in cystic fibrosis.J Cyst Fibros. 2017;16 Suppl 2:S70-S78. doi:10.1016/j.jcf.2017.06.011Cystic Fibrosis Foundation.Cystic fibrosis-related diabetes.MedlinePlus.CFTR gene.Cystic Fibrosis Foundation.Fertility in men with CF.Neemuchwala F, Ahmed F, Nasr SZ.Prevalence of pelvic incontinence in patients with cystic fibrosis.Glob Pediatr Health. 2017;4:2333794X17743424. doi:10.1177/2333794X17743424Mcphail GL.Coagulation disorder as a presentation of cystic fibrosis.J Emerg Med. 2010;38(3):320-2. doi:10.1016/j.jemermed.2007.10.038Sathe M, Houwen R.Meconium ileus in cystic fibrosis.Journal of Cystic Fibrosis. 2017;16:S32-S39. doi:10.1016/j.jcf.2017.06.007Schram CA.Atypical cystic fibrosis: Identification in the primary care setting.Can Fam Physician. 2012;58(12):1341-1345, e699-704.Zvereff VV, Faruki H, Edwards M, Friedman KJ.Cystic fibrosis carrier screening in a North American population.Genet Med. 2014;16(7):539-546. doi:10.1038/gim.2013.188

Cystic Fibrosis Foundation.About cystic fibrosis.

Cystic Fibrosis Foundation.Newborn screening for CF.

Sanders DB, Li Z, Laxova A, et al.Risk factors for the progression of cystic fibrosis lung disease throughout childhood.Ann Am Thorac Soc. 2014;11(1):63-72. doi:10.1513/AnnalsATS.201309-303OC

Filkins LM, O’toole GA.Cystic fibrosis lung infections: Polymicrobial, complex, and hard to treat.PLoS Pathog. 2015;11(12):e1005258. doi:10.1371/journal.ppat.1005258

National Heart, Lung, and Blood Institute.What is bronchiectasis?

American Academy of Allergy, Asthma & Immunology.Nasal polyps.

Martin C, Hamard C, Kanaan R, et al.Causes of death in French cystic fibrosis patients: The need for improvement in transplantation referral strategies!.J Cyst Fibros. 2016;15(2):204-12. doi:10.1016/j.jcf.2015.09.002

Singh VK, Schwarzenberg SJ.Pancreatic insufficiency in cystic fibrosis.J Cyst Fibros. 2017;16 Suppl 2:S70-S78. doi:10.1016/j.jcf.2017.06.011

Cystic Fibrosis Foundation.Cystic fibrosis-related diabetes.

MedlinePlus.CFTR gene.

Cystic Fibrosis Foundation.Fertility in men with CF.

Neemuchwala F, Ahmed F, Nasr SZ.Prevalence of pelvic incontinence in patients with cystic fibrosis.Glob Pediatr Health. 2017;4:2333794X17743424. doi:10.1177/2333794X17743424

Mcphail GL.Coagulation disorder as a presentation of cystic fibrosis.J Emerg Med. 2010;38(3):320-2. doi:10.1016/j.jemermed.2007.10.038

Sathe M, Houwen R.Meconium ileus in cystic fibrosis.Journal of Cystic Fibrosis. 2017;16:S32-S39. doi:10.1016/j.jcf.2017.06.007

Schram CA.Atypical cystic fibrosis: Identification in the primary care setting.Can Fam Physician. 2012;58(12):1341-1345, e699-704.

Zvereff VV, Faruki H, Edwards M, Friedman KJ.Cystic fibrosis carrier screening in a North American population.Genet Med. 2014;16(7):539-546. doi:10.1038/gim.2013.188

Bush A, Sly P.Evolution of cystic fibrosis lung function in the early years.Curr Opin Pulm Med.2015;21(6):602-8. doi:10.1097/MCP.0000000000000209

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