Cystic Fibrosis In Children – Causes, Symptoms & Treatment

Written by K. Masthoff

Last Updated on Feb 11, 2016

Cystic-Fibrosis-In-Children---Causes,-Symptoms-&-Treatment

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Cystic fibrosis is a condition that many know little about. And, if your pediatrician diagnoses your little one with cystic fibrosis, you’ll probably be panicking. You might wonder what this condition is and how it will affect your child. Well, you can stop wondering! MomJunction opens up the world of cystic fibrosis so that it doesn’t remain a conundrum. Armed with the right knowledge and information about this genetic condition, you will be able to take an informed decision on how to treat your little one and ensure he grows up properly.

Read our post here to learn more about cystic fibrosis in children.

What Is Cystic Fibrosis In Children?

Cystic fibrosis is a genetic condition, which adversely affects the lungs and digestive system [1]. The condition results in the production of very thick, sticky, glue-like mucus in the body which, in turn, blocks, the bronchial tubes or airways.

The disease occurs due to some changes or mutations in a particular gene, which is inadvertently passed down through generations. But, it can only be passed on to your child when you and your partner have this altered gene. The good part is doctors today are equipped to diagnose cystic fibrosis in children at a very early stage.

Cystic fibrosis can make your little one more susceptible to lung diseases. He may also have frequent bouts of breathing troubles. You need to remember that this condition can worsen over time, and your child may have issues with growth, proper nutrition and digesting food. Thankfully, doctors, today can easily treat cystic fibrosis in kids. It’s why life expectancy rates among kids are increasing.

Cystic Fibrosis In Children- Causes:

A faulty or changed gene called the CFTR gene is responsible for cystic fibrosis [2]. The gene typically produces protein, which displaces water and salt from a cell. Now, if this CFTR gene doesn’t do its job properly, the airways and passages get accumulated with a sticky, glue-like and thick mucus.

The thick mucus causes damage to the digestive system, lungs, and organs in the body. It leads to swelling or inflammation of vital organs and repeated lung infections.

This sticky and ultra-thick mucus makes it tough for the body to keep its organs healthy, safe and clean. Most people are unaware they carry the mutated gene. So if your child is diagnosed with cystic fibrosis, don’t blame yourself. You were not responsible for activating the mutated gene or causing the mutation. It just happened.

[ Read: Genetic Disorders In Children ]

How Does The Gene Pass Down Through Generations?

For a child to get cystic fibrosis, he needs to inherit one CFTR gene from each parent. If the child has one instance of the gene, he will simply become a carrier of the condition, but will not actively have cystic fibrosis. When he has kids, he will pass on the gene to his offspring.

United Kingdom data estimates that 4 out of 100 people carry the CFTR gene. Thus, it comes as no surprise that the country classifies cystic fibrosis as a common genetic disorder. Remember that the carrier of the CFTR gene does not exhibit any cystic fibrosis symptoms and is quite healthy. If both parents carry the CFTR gene:

There is a slight chance (around 25%) that your child may inherit this changed gene and may not be a carrier.

Alternatively, there is a chance that your child will receive a single copy of the CFTR gene from his mother or father, or may carry the gene himself. There is a 50% chance of him not being afflicted by the condition.

There is also a chance that your child may get two copies of the CFTR gene – one from his father and the other from his mother. This will lead to the child developing cystic fibrosis.

[ Read: Breathing Problems In Children ]

Symptoms Of Cystic Fibrosis In Children:

Children with cystic fibrosis tend to exhibit different symptoms. The symptoms are not always the same for every child. However, some of the common signs and symptoms of the condition include:

Extreme saltiness of sweat and skin

Bouts of diarrhea

Slow growth compared to other children

Birth defect like blockage of small intestine which stops the baby from passing stool easily (also known as meconium ileus)

Bad smelling stools

Constipation

Frequent cough

Infection of the lungs

Experiencing wheezing and sudden loss of breath

Experiencing sinus attacks and growth of polyps or lumps of flesh in the nose

Flattened or rounded fingers

Stuffed nose or inflammation of the nasal passage

Protrusion of a part of the rectum from the anus (Rectal prolapse)

Intolerance for exercise [3]

Cystic fibrosis thickens the mucus in the body, which blocks the tubes that carry digestive enzymes from the pancreas to the small intestine. The main function of these enzymes is to help in the absorption of nutrients. This glue-like mucus also creates problems for the tubes that carry air to and from your kid’s lungs.

Today, newborn screening allows doctors to diagnose cystic fibrosis the moment the kid is born. Although, every child exhibits different symptoms depending on the severity of cystic fibrosis. These symptoms may get better or worse as your little one grows older. In some cases, the child may not show symptoms of cystic fibrosis until he turns into an adolescent or hits adulthood.

One way to tell if your child has the condition is by the sweat produced by your little one. A child with cystic fibrosis tends to have higher salt content in the perspiration, making the sweat extremely salty. In children, the condition causes issues for digestive and respiratory systems, but when your child turns into an adult, he may develop diabetes and pancreatitis, and in some cases, infertility.

Cystic Fibrosis In Children – Diagnosis And Tests:

Based on the results obtained from conducting various tests, doctors can accurately determine whether your little one has cystic fibrosis. Some of the tests that allow physicians to diagnose this genetic condition are:

1. Prenatal Genetic Test:

If you are pregnant, and you have a genetic predisposition to cystic fibrosis, you can opt for prenatal screening to determine whether your baby has the condition. The screening includes performing a chorionic villus sampling (CVS) and amniocentesis. Both tests have certain risks involved. It is best to understand these risks and make an informed decision.

2. Sweat Test:

If your child’s blood test points towards cystic fibrosis, the doctor will recommend the sweat test. The test measures salt content in the sweat.

The doctor will rub a specially-created chemical on your child’s skin to produce sweat. Then a mild electric current is passed through the sweat with the help of an electrode. Finally, the doctor collects the sample, which is tested for the presence and level of salt. Usually, the doctor will perform the test twice to ensure accurate results.

[ Read: Stomach Flu In Children ]

3. Cystic Fibrosis Carrier Testing:

If you or your partner has a family history of CFTR, and you are worried about your child inheriting the condition, you should get a cystic fibrosis carrier test. Here, the doctor takes samples of your saliva and blood to check whether you and your partner carry the mutated gene.

This is a particularly accurate test, and if you are stressed about your child getting the condition, the doctor will recommend it.

There are a few other tests the doctors may perform on your little one to conclusively diagnose the condition:

1. Lung Test:

This is a lung function test that determines the quantity and speed at which your kid breathes air in and out. It also checks how efficiently your child’s lungs transfer oxygen to the blood.

2. Chest X-ray:

This X-ray helps doctors find out your little one’s lungs are trapping air, whether the lungs are scarred or inflamed or they have a bacterial infection.

3. Sputum Culture:

The doctor will take a sample of your child’s spit or sputum and analyze it to see if bacteria are thriving in it. A bacterium known as mucoid pseudomonas can result in serious complications. If this bacterium is found, your little one will put through aggressive treatment.

4. Sinus X-ray:

The X-ray helps the doctor determine whether the block sinuses that your child has is due to cystic fibrosis. In fact, sinusitis is a common complication arising due to this genetic condition.

Cystic Fibrosis In Children – Complications:

There are so many complications that result from having this genetic disorder. Cystic fibrosis causes many problems related to the digestive system, respiratory system, and even the reproductive system.
Some of the complications that your child may develop in childhood or adulthood include:

1. Hemoptysis Or Coughing Blood:

As time passes, cystic fibrosis causes thinning of the airway walls. This results in small amount of blood in the mucus. Your little one may also cough up blood due to a hemorrhage in the airways.
If your child coughs more than 200 ml of blood, it is major hemoptysis and requires immediate medical attention.

2. Pneumonia Or Bronchitis:

The presence of thick, glue-like mucus in the lungs and sinuses creates a conducive environment for fungi and bacteria to thrive. As a result, your little one gets more susceptible to chronic infections, like pneumonia and bronchitis. Both require treatment from a qualified medical specialist.

[ Read: Bronchitis In Children ]

3. Damage To The Respiratory System:

If your little one has a severe case of cystic fibrosis, he could suffer from acute lung tissue damage. If the damage is grave, it can adversely affect the functioning of the lungs, leading to permanent respiratory problems, such as shortness of breath and inadequate oxygenation of blood.

4. Bronchiectasis Or Damage To The Airways:

Another serious complication due to cystic fibrosis is bronchiectasis, which is the medical term for damage to the airways. More often than not, cystic fibrosis leads to bronchiectasis, making it tough to clear the mucus from the bronchial tubes. This prevents the movement of air in and out of your child’s lungs.

5. Pneumothorax:

Pneumothorax refers to collapsed lung due to air leaking from the lungs and accumulating between the lung and chest wall [4]. This air then pushes against the lung, causing it to collapse. It causes shortness of breath and severe pain in the chest.

6. Nasal Polyps:

Nasal polyps are soft, fleshy lumps of tissue growing in the nasal passage. It is common among patients with cystic fibrosis. These polyps disrupt sleep and develop when the nose becomes swollen or inflamed.

7. Obstruction Of The Intestine:

Obstruction of the intestine is a major complication arising from cystic fibrosis. This obstruction can be partial or complete. While obstruction of the intestine can occur at any age, children with cystic fibrosis tend to get a condition known as intussusceptions. This refers to the intestine folding like an accordion, leading to a blockage of the intestine.

8. Diabetes:

Scientific studies reveal 20 percent people suffering from cystic fibrosis go on to get diabetes. This is because the condition affects the pancreas, which is responsible for producing insulin.

[ Read: Diabetes In Children ]

9. Vitamin And Protein Deficiency:

Cystic fibrosis blocks the tubes that transfer digestive enzymes from the pancreas to the intestines. As a result, your little one is unable to absorb proteins and vital nutrients, leading to a deficiency. This can inhibit your kid’s growth and development.

10. Blockage Of The Bile Duct:

Sometimes cystic fibrosis leads to the blockage and inflammation of the duct that carries bile from the gallbladder and liver to the small intestine. This can result in gallstones and even occasional liver problems.

11.Dehydration And Imbalance Of Electrolytes:

If your child has cystic fibrosis, you already know that he has very salty perspiration. You child may suffer from dehydration and imbalance of minerals and electrolytes in the blood. He may also have low blood pressure, complain of constant fatigue and have a rapid heart rate.

12. Osteoporosis:

As your child grows older, he is at a higher risk of developing osteoporosis, a condition in which the bones become weak and brittle and fracture easily [5].

It is important you speak to your child’s pediatrician to understand the complications your child can develop due to cystic fibrosis. The physician will give you advise on how to deal with these complications if they occur and what measures you can take to prevent or delay the onset of these complications.

Cystic Fibrosis In Children – Treatments & Solutions:

Since cystic fibrosis is a genetic condition, it does not have a cure. However, when your child gets diagnosed early in life, you can take steps to improve the quality of your little one’s life and also prevent complications from occurring. While your baby is being treated, you and the doctor will monitor him constantly. It is prudent to remember cystic fibrosis requires specialty treatment, and there are clinics that offer this type of treatment.

1. Digestive Enzymes:

Once the doctor diagnoses cystic fibrosis, your little one will be put on digestive enzymes and vitamins to help him gain weight normally and not suffer from vitamin deficiency. These enzymes help the digestive system to digest and absorb nutrients properly.

2. Nutritional Therapy:

Your little one will require a special diet to ensure he gets the nutrients for proper growth and development. With the help of a doctor and trained nutritional therapist, a diet plan consisting of high amounts of calories and fats will be created. Also, your child may also have to take other supplements to ensure there is no deficiency.

The treatment for cystic fibrosis in children is aimed at clearing mucus, and minimizing lung and digestive system infections.

3. Antibiotics:

As your little one will be prone to lung infections, he will be put on antibiotics to ward off the infections. This can be in the form of pills, capsules, liquids or nebulizer. If your child gets a severe infection, the antibiotics will be given intravenously.

Usually, your child will have to take an antibiotics course, even if he does not have an infection, and this could go on for a few years.

2. Pulmozyme:

This is a type of enzyme that is inhaled through a nebulizer. It helps dispel and dislodge the thick, sticky mucus so that your little one can cough it up. The nebulizer does not have any side effects.

3. Hypertonic Saline:

Hypertonic saline may be prescribed in place of Pulmozyme. Just like Pulmozyme, the salt solution makes the mucus runny, which your child can cough up.

4. Insulin:

If your little one develops diabetes due to cystic fibrosis, he will have to depend on insulin to maintain blood sugar levels. Doctors will show you how to give your child insulin, which is available as an injectable and pill.

5. Bisphosphonates:

As mentioned earlier, cystic fibrosis can result in underdeveloped bones. So the doctor will put your little one on bisphosphonates to maintain prevent fractures and boost bone density.

6. Corticosteroids:

If the airways of your child swell up and prevent him from breathing properly, the doctor may recommend corticosteroids. Also, sprays and nasal drops help treat nasal polyps.

7. Bronchodilators:

In rare cases, doctors may recommend bronchodilators to make it easier for your child to breathe. The medication relaxes the lungs and chest muscles and helps dilate and open up airways.

8. Flu Vaccination:

Since your child will be prone to lung infections, it is important he receives annual flu vaccination for his own good. It will protect him from complications that may arise due to an infection [6].

Cystic Fibrosis In Children – Prevention:

Cystic fibrosis is a genetic condition; you cannot prevent it. It just happens. However, you can take steps to improve the quality of your little one’s life by adopting certain measures:

Breathing exercises or activities to improve lung function.

Giving vitamin supplements to prevent vitamin deficiency.

Ensuring your little one eats a diet rich in proteins and calories

Keeping your home clean and free of dust, smoke and allergens to prevent your little one from developing breathing difficulties.

Helping him stay physically active.

Making him take pancreatic enzymes with each meal to ensure optimal absorption of proteins and fats.

After getting permission from your child’s doctor, you can try postural drainage to clean the mucus from the bronchial tubes of your kid.

Make sure your child drinks a lot of water to help drain the mucus and prevent dehydration and electrolyte imbalance.

You and your kid can enroll in cystic fibrosis support groups. It is good for your child to know that he is not alone battling this condition.

If a child has cystic fibrosis, he remains in good health till he reaches adulthood. So you child will be able to attend school, college and perhaps, even find a job. However, the average lifespan of a person with cystic fibrosis is 37 years. So you need to be prepared for any eventuality.

Cystic Fibrosis In Children – Home Remedies:

There are home remedies that you can use to ease the symptoms of cystic fibrosis. However, make sure you first consult your child’s doctor and get a go-ahead from him. This is because some home remedies may interfere with the medications your little one is taking.

1. Herbs For Improved Immune Function:

You can use herbs like red clover, astragalus, nettle and cleaver to help improve your child’s immunity. Usually, these herbs are brewed into a tea and then given to the child.

2. Herbs To Thin Mucus:

Herbs like comfrey, burdock root and lobelia are great for thinning the mucus that prevents your child from breathing properly. Once the mucus thins, your little one will cough it up.

3. Herbs To Improve Digestion:

Dandelion flowers and leaves are great for improving your child’s digestive function. You also can use other herbs, like oatseed and yellow dock.

4. Herbs For Bacterial Infection:

Papaya has antibacterial properties and can help your little one fight bacterial infections. If your angel has a drug-resistant bacterial infection, you can give him some green tea sweetened with honey. This home remedy helps in fighting off pseudomonas aeruginosa strain of bacteria. Alternatively, you can add some cinnamon powder in your child’s drinking water.

5. Herbs For Lung Inflammation:

Turmeric powder has anti-inflammatory properties. The curcumin is what makes turmeric so effective in dealing with inflammation. You can add turmeric powder in the food you prepare for your child, or you can add it to milk and give it to your child to drink.

It is a good idea to involve your child’s specialist when you want to use home remedies, as all herbs may not be suitable for kids.

In Conclusion:

It is not easy as a parent caring for a child with cystic fibrosis. However, you too can benefit from joining a local cystic fibrosis support group. Also, make your child’s doctor into your confidante and speak to him freely about your worries and concerns. He will be the best person to guide you properly and allay your fears.

Do you have a child or family member with cystic fibrosis? How do you deal with this chronic genetic condition? Please comment below.