Cystic Fibrosis

Symptoms of CF can be very different from person to person. Sometimes symptoms show up at birth, and sometimes they show up later. Also, in some cases, your child may have few symptoms, or very mild symptoms when they are younger, but get more or worse symptoms when they are older. Sometimes your child may have few symptoms, and other times your child’s symptoms may be more severe.

Some of the early symptoms include an inability to have a bowel movement shortly after being born and a salty taste on your baby’s skin.

• Other CF symptoms tend to happen later in your child’s life, and may include respiratory, digestive, or other symptoms.

Respiratory symptoms

Your child’s respiratory symptoms may include:

• Lung infections, typically caused by unusual or uncommon germs that don’t respond to regular antibiotics
• Frequent, persistent cough that brings up thick mucus that is sometimes bloody
• Frequent sinusitis [sie-nuh-SIE-tis], which is inflammation (swelling) of the sinuses
• Frequent bronchitis [bron-KIE-tis]
• Regular cases of pneumonia [noo-MOH-nee-uh]

Digestive symptoms

Your child’s digestive symptoms may include:

• Frequent foul-smelling, greasy stools
• Ongoing diarrhea
• Severe constipation
• Stomach pain and discomfort
• Gas or bloating
• Poor weight gain and growth

Reproductive symptoms

Later in your child’s life, they might have reproductive symptoms including:

• Infertility in males, as they are born without a vas deferens (the tube that transports sperm to the penis)
• Difficulty getting pregnant in females due to mucus blocking the cervix or other complications
• Once pregnant, women with CF may be at a higher risk of developing gestational diabetes (diabetes during pregnancy)

Other signs and symptoms of CF are generally due to an imbalance of nutrients and minerals in the blood. CF causes your child’s sweat to become very salty, which causes them to lose a lot of salt when they sweat. Your child may become dehydrated more easily than other children.

Have your child see a doctor if you suspect they have CF. It is important to get a diagnosis and start treatment as soon as possible. CF can have serious complications, and may also cause more severe conditions such as:

• Pancreatitis
• Diabetes
• Liver disease
• Gallstones
• Lung damage

CF is an inherited disease, which means it is passed from parent to child. Even if you do not have the symptoms of CF, if you have the damaged genes that causes CF, you can still pass CF on to your child.

You can get tested to find out if you have the genes that cause CF, which may provide insight as you think about having children. If you and your partner are both carriers of CF, it is more likely that your children will have CF. If you and your partner both have CF, your child will have CF.

There are many different ways to test for CF. These tests include:

• Blood tests. Also called newborn tests because they are often done when your child is born. This test will show if a newborn’s pancreas is working properly.
• Gene test. A gene test is also often performed at childbirth, and shows whether a newborn has certain damaged genes.
• Sweat tests. If your child’s blood test or genetic test suggests CF, your child’s doctor will use a sweat test to confirm the diagnosis. A sweat test takes a sample of your child’s sweat and measures the amount of salt in it.

If your child tests positive for CF, your child’s doctor may order other tests to monitor the progression of the illness. These other tests may include:

• Lung function tests, which will measure how much air your child can breathe in and out, and help show how strong your child’s lungs are and how fast they can bring oxygen to the blood.
• Chest x-rays, which will create a picture of your child’s lungs and show whether the lungs are inflamed (swollen), scarred, or trapping air.
• Sinus x-rays, which will show if the sinuses are inflamed.
• Sputum (spit) cultures, which your child’s doctor will test for bacteria.

There are many different treatments to help ease your child’s symptoms, depending on the severity of your child’s case of CF:

• Medications, such as antibiotics, anti-inflammatories, and enzymes to help your child’s digestive tract absorb nutrients.
• Physical therapy to help loosen the mucus in the lungs.
• Breathing techniques to help loosen the mucus in the lungs and strengthen them.
• Pulmonary rehabilitation programs, which are programs that can help reduce your child’s symptoms with healthy lifestyle choices.
• Vest therapy, which is where your child wears a vest that vibrates to help loosen the mucus in the lungs.
• Oxygen therapy, where your child is given pure oxygen to inhale to help prevent high blood pressure in the lungs.
• Endoscopy, which is where your doctor inserts a tube into your child’s lungs to suck out the mucus.
• In some cases, surgery may be needed if your child gets a severe intestinal blockage, or has nasal polyps that stop them from breathing.
• In very severe cases, a lung transplant may be required. CF does not come back in the transplanted lung. If your child has other symptoms, such as sinus infections or digestive problems, they will still remain after a lung transplant.

It is important to catch CF as early as possible so that you can begin treating your child early. Treatment goals include:

• Getting rid of mucus in the lungs and other organs
• Stopping infections in the lungs and sinuses
• Giving your child a special diet since they might not absorb nutrients from food the right way
• Preventing intestinal blockages, and treating them as needed

There is no known cure for CF, although most children with CF go on to live long lives with proper treatment.

CF is a genetic disorder, and there are no known prevention methods at this time. However, you may be able to have yourself tested to find out if you are a carrier of the disease, which may provide some insight about whether or not you could pass this on to your child.

Cystic fibrosis (CF) [SIS-tic fye-BROH-sis] is a genetic disease, which means a disease that is passed from parent to child. Most of the time, CF affects the mucus lining in your child’s liver, intestines, lungs, pancreas, sinuses, and sex organs. CF affects your child’s mucus and sweat glands, making the mucus thick and sticky.

Mucus helps protect the body. Healthy mucus is usually slippery, but slightly thicker than water. In children who have CF, their mucus can become so thick that it clogs the lungs, making it hard to breathe. Children that have CF can also have frequent lung infections, which can cause lung damage.

The thick mucus can also block the tubes in your child’s pancreas, which prevents digestive enzymes from getting into your child’s intestines. This makes it difficult for them to absorb nutrients, such as fats and proteins, from their food.

Although it can affect a few different organs, most often, the thick mucus that CF creates affects how your child breathes and digests food.