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Management of Cystic Fibrosis in Kids

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Cystic fibrosis is a genetically inherited disorder where the body causes mucus. This article will discuss further its causes, diagnosis, and management.

Written byDr. Porwal Himanshi Avadheshkumar

Medically reviewed byDr. Kaushal Bhavsar

Published At February 3, 2023
Reviewed AtJuly 6, 2023

Introduction:

Cystic fibrosis is a chronic disorder caused by genetic inheritance. It is a lifelong disease causing a myriad of health-related complications in both males and females. As it is an autosomal recessive genetic disorder, the children need to have two genes inherited from each of the parents. On the other hand, if the child is a carrier of only one gene instead of two, he is a silent carrier of the disorder. Which means the child will not show any symptoms of the disorder. This disorder affects saliva, sweat, tears, mucus, and the digestive fluid of the body. These fluids help to lubricate the glands and their opening to function normally. On the contrary, cystic fibrosis causes these secretions and fluids to be abnormally thick and sticky. As a result, the glands and openings are clogged, affecting their functions. This affected function further causes health-related complications in organs such as the lungs and pancreas.

What Are the Causes of Cystic Fibrosis in Kids?

There are the following etiological factors that play a part in causing cystic fibrosis:

  • Cystic fibrosis is an autosomal recessive genetic mutation. This means to have this disorder; a child has to have two mutated genes from each parent. However, the carrier with one mutated gene is not affected by it.

What Are the Symptoms Associated With Cystic Fibrosis in Kids?

Children with cystic fibrosis show symptoms immediately after birth. For instance, a child with CF has meconium ileus. It is an intestinal blockage. Depending on the severity, the followings are the signs and symptoms associated with cystic fibrosis:

  • Lung infections.

  • Pneumonia.

  • Constipation or diarrhea.

  • Wheezing.

  • Shortness of breath.

  • Salty-tasting skin.

  • Recurrent cough.

  • Delayed and poor growth.

  • Low weight.

  • Frequent bowel movements with greasy and smelly stools.

How to Diagnose Cystic Cystic Fibrosis in Kids?

There are the following diagnostic methods used for diagnosing cystic fibrosis:

  • Sweat (Chloride Test): During this test, the skin is stimulated with mild electric current, and the forearm is placed in solution attached to electrodes. This causes sweat excretion from the body, which is further collected to check the chloride level.

chloride-levels-in-sweat

  • Genetic Testing: This test shows if a child has inherited the genes from parents by testing scrapped cells from the cheeks or blood samples.

  • Blood Test: This evaluation method is used to check the pancreatic function of the body.

  • Chest X-rays: This evaluation method is used to check the normality of the internal organs.

  • Stool Culture: By taking this sample, the fat level is tested. If the sample has a high level of fat, it suggests abnormal digestion.

What Are the Complications Associated With Cystic Fibrosis in Kids?

Cystic fibrosis has the following health-related complications associated with it:

  • Meconium Ileus: It is the first sign of cystic fibrosis, where the child’s first stool (meconium) causes obstruction in the small intestine (ileum). This can further cause upper respiratory complications, too.

  • Osteoporosis: It is a condition where the bone is weak and brittle.

  • Vitamin Deficiencies: Children with this disorder have a deficiency of fat-soluble vitamins that are vitamins A, D, E, and K.

  • Malnourishment: Children with CF have digestive problems that further cause a deficiency of nutrients in the body.

  • Gastroesophageal Reflux: It is the reflux due to the constant flow back of the acid in the body.

  • Low Weight: Due to malnourishment and deficiencies of nutrients, the child with CF has low weight and growth.

  • Liver Disease: Due to mucus obstructing the bile duct, children with CF often have liver diseases such as multilobular cirrhosis or neonatal cholestasis.

  • Diabetes: It is a health condition where either the pancreas is unable to produce enough insulin or the body is unable to use enough insulin.

  • Joint Pain: There are many reasons behind this condition. For instance, arthritis, injury, bursitis, or muscle pain.

  • Bronchiectasis: Due to the reduced ability to fight back the bacteria in the body, bacterial colonization increases. This further causes chronic infection and tissue destruction that causes bronchiectasis.

  • Continuous muscle ache.

How to Manage Cystic Fibrosis in Kids?

Cystic fibrosis is a lifelong disorder, and children have to deal with it throughout their lives. There are the following approaches to managing cystic fibrosis in children:

Management of the Lung Obstruction and Its Complications:

  • Physical Therapy of the Chest: If a child is at a certain age, lung-clearing exercises are very effective in clearing the mucous blockage. There are options such as postural drainage of the mucous, using devices such as a percussor or flutter. These devices clear the mucus blockage by causing vibration on the chest wall. Moreover, other physical therapy can be done manually or with the help of other devices.
  • Medications: Medications such as using inhalers, bronchodilators, anti-inflammatory drugs, or antibiotics can be of great help too.
  • Immunization: A child with this disorder is more likely to develop an infectious condition. As a result, doctors are more cautious and make sure that a child gets pneumococcal and influenza vaccination shots on time.

  • Management of Digestive Complications: There are several options available to manage the digestive health of the child, such as proper diet, using pancreatic enzymes to aid digestion and nutrition, and vitamin supplements.

  • Management of Psychological Complications: Children with this disorder need constant reminders and motivation that this can be managed. Because with age, they might have to face complications such as infertility, sexuality coping, or independence. Not only children but also parents sometimes face difficulty in managing regular care of their child too.

Conclusion

Cystic fibrosis is a lifelong disease that needs constant care throughout a child’s life. It causes not only physical complications but also psychological complications too. Even though cystic fibrosis can cause life-threatening complications, there are many options available to manage the symptoms associated with this disorder.

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Frequently Asked Questions

Children born with cystic fibrosis in recent years lead a normal life. About half the children can live up to 40 years of life. Advancements in treatment methods such as pancreatic enzymes, airway clearance techniques, and anti-staphylococcus and antibodies have improved patients’ survivability.
Patients with cystic fibrosis are often diagnosed at a median age of six to eight months. Most of the patients are diagnosed within one year. Symptoms differ from person to person and age. The disease can affect different parts of the body.
Most newborns are screened at birth or are diagnosed within one year of life. In children suspected of cystic fibrosis, the doctors suggest a sweat test or genetic test to confirm the diagnosis. The sweat test is the most common and painless test useful in cystic fibrosis diagnosis.
Cures for cystic fibrosis currently do not exist. However, treatment is provided to control symptoms, prevent or reduce complications, and improve patients’ quality of life. Frequent monitoring and aggressive early intervention can slow the disease progression and prolong life.
Pain is considered a clinical complication of cystic fibrosis. Pain is experienced by 70 percent of patients who are affected by cystic fibrosis. The symptom hampers patients' quality of life and ability to follow treatment procedures. The most common symptoms are headache, gastrointestinal, and chest pain.
The signs of cystic fibrosis develop at birth or early childhood. The main symptoms include:
- Repeating and frequent chest infections.
- Difficulties in gaining weight.
- Wet coughs are frequent.
- Diarrhea.
In some patients, wheezing and shortness of breath are present.
Patients with cystic fibrosis are also at risk of developing diabetes, osteoporosis, and liver problems.
 
Sleep disturbances are common in patients with cystic fibrosis. Almost half of the newborns with cystic fibrosis experienced trouble with sleeping. Hence, the patients sleep less than normal individuals. The cause may be due to cystic fibrosis's effect on lung health and comorbidities.
The first sign of cystic fibrosis appears as difficulty in newborns to pass their first bowel movement (meconium). It is caused by the thickening of the meconium that disturbs their passage through the intestine, resulting in blockage. In some other patients, respiratory symptoms may develop first.
Newborns are screened for cystic fibrosis with a heel prick test that is done shortly after birth. If the results suggest cystic fibrosis, then additional tests like sweat and genetic tests are required. The sweat test includes the measurement of the amount of salt in sweat that is abnormally high in patients with cystic fibrosis. A genetic test involves the analysis of blood or saliva to detect faulty genes responsible for cystic fibrosis.
 
Most children with cystic fibrosis are diagnosed within two years of life. However, children with a mild form of cystic fibrosis are not diagnosed until their teenage years or adulthood. A sweat test or genetic test is suggested by doctors to confirm the diagnosis.
Cystic fibrosis cannot be prevented. Newborns with two copies of abnormal genes have  developed cystic fibrosis in the pancreas and liver at birth. However, respiratory symptoms develop at birth. Genetic testing of couples with family history can limit disease severity. Studies are ongoing to utilize gene therapy to prevent lung involvement in cystic fibrosis.
Children born in recent times with cystic fibrosis can live up to 40 years of life. Patients experience mortality due to lung complications. Patients live longer and healthier lives due to advancements in medical treatment. However, life expectancy is dependent on disease severity.
Common symptoms of patients with end-stage cystic fibrosis include fatigue, anxiety, dyspnea, pain, and cough. The lungs are frequently affected along with the intestine, liver, pancreas, and endocrine system. Palliative care is provided to obtain relief from symptoms. Active treatment for the disease is combined with palliative care to improve the patient’s quality of life.
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Dr. Kaushal Bhavsar from iCliniq
Dr. Kaushal Bhavsar

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