The impact on the Body when you have Cystic Fibrosis

The lungs and pancreas are most often affected by the disease. This leads to problems with breathing and the digestive tract. If a person has cystic fibrosis, mucus can still trap bacteria, but it has difficulty moving the mucus of the lungs. The end result is the bacteria can remain in the lungs which can lead to serious infections.

The pancreas produces a protein called enzymes. This helps the process of digestion. When someone has cystic fibrosis, the ducts or path between the pancreas and the intestines become blocked by sticky mucus. Those important enzymes are prevented from digesting fats and proteins from reaching the intestines. The end result, a patient with cystic fibrosis has difficulty properly digesting food and getting the nutritional value they need from it. The pancreas maintains the sugar levels in the blood, so a percentage of patients with cystic fibrosis can also develop diabetes (Type 1) also known as insulin-dependent diabetes.

Other anatomy such as the intestines, liver, sweat glands, and reproductive organs are also damaged in certain people with the disease.

Many males with cystic fibrosis become infertile because the tube connecting the testes and prostate gland can become blocked. New medical advancements now give hope. There are certain fertility methods and procedures that may sometimes make it possible for these males to experience fatherhood. Females with cystic fibrosis can be less fertile than others, many females with cystic fibrosis are able to conceive and have a successful pregnancy.
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Children and Cystic Fibrosis – the symptoms

Symptoms can be varied from one person to another, depending on how severe the disease is. In one example, a child with the disease can have respiratory issues but not have digestive issues, while in another example, the child may have both. Also, the signs and symptoms can vary with age of the child.

In certain newborns, usually very first indication of cystic fibrosis can be having difficulty to pass a first bowel movement. This happens when the meconium becomes thick to the point that it won’t transport through the intestinal tract, causing a blockage. A parent may later recognize their child is not increasing in weight or growing as expected. The stools of the baby can be very bulky, foul-smelling, and greasy since the digestion of fats are poor.

Other signs in newborn babies can include:

• Common respiratory infections
• Coughing & wheezing
• Sweat that tastes salty

Patients with cystic fibrosis can to have up to five times the normal amount of sodium chloride (salt) in their sweat glands. A parent can notice this symptom of cystic fibrosis, since they will taste the salt when they kiss the child.

Cystic fibrosis in children may also have the following symptoms:

Delays in growth, children with cystic fibrosis tend to be smaller than other children their age.

• Shortness of breath or difficulty breathing
• Challenges performing exercise
• Constant coughing and or wheezing
• Common chest & sinus infections
• Recurring pneumonia
• Recurring bronchitis
• very thick phlegm (babies and young children usually swallow the phlegm they cough up)

Diagnosing Cystic Fibrosis

When symptoms begin to appear, cystic fibrosis may not be a physician’s initial diagnosis. As indicated above, there are a wide variety of symptoms, and not every patient with this disease presents with all of them. Another important factor is that cystic fibrosis may range from mild to severe in different patients. Age at which symptoms first appear can vary. Certain patients with cystic fibrosis were diagnosed as newborns, while other patients are not diagnosed with the disease until they become older. If cystic fibrosis is mild in the beginning, a patient with cystic fibrosis may not experience issues until reaching their teen years – or even when they become an adult.

Newborns are usually tested for the disease if they are born with an intestinal blockage, this is called meconium ileus. In certain states in the US, doctors can screen all newborns for the disease with what’s known as a blood test.

The sweat chloride test or (sweat test) analyzes a patent’s sweat for the level of salt. To perform this test, a physician or medical technician stimulates sweating on a person’s body – usually in a small area on the person’s arm. The physician or medical technician does this with an odorless sweat-producing chemical being applied to the area. This sends a very weak electric current through an electrode attached. This mild current will stimulate sweating and then creates a warm tingling sensation, but it does not hurt. After a few minutes, the sweat is collected from the area that was stimulated and is sent to the lab for analyzation.

This sweat chloride test is performed two times; if their is a consistent high level of salt, this usually indicates that cystic fibrosis is present. The sweat chloride test is the gold standard test for cystic fibrosis, but it may not always be useful in newborn children. This is because newborns may not produce the necessary sweat for an accurate diagnosis in the first month of the baby’s life. For this very reason, physicians normally do not perform a sweat chloride test until the infant is at a few months old.

In certain cases, the physician can perform a gene analysis test of a blood sample to confirm the diagnosis of the disease. Almost 90 percent of patients with cystic fibrosis can be detected through DNA testing or genetic analysis.

Other tests can measure how well the lungs, pancreas and liver are performing to better determine the extent and severity of the disease as soon as the diagnosis is confirmed.

These tests include but are not limited to:

• • Chest X-rays
  • Testing Pulmonary lung function
  • Pancreatic function testing

Since cystic fibrosis is a genetic disease, the physician can suggest testing the sisters or brother of a patient with cystic fibrosis, even if they are asymptomatic.

Treatment for Cystic Fibrosis

As of today, there is no cure for cystic fibrosis. However, many medical treatments are available to manage this disease. The main goals of treatment for someone with this disease is to prevent and treat infections, keep the airways and lungs as open as possible, and maintain sufficient nutrition.

To achieve these objectives, medical treatments for cystic fibrosis in children can include:

Childhood immunizations. Cystic fibrosis doesn’t directly affect the immune system, however a child with cystic fibrosis is more likely to develop complications when they become ill. Physicians are mainly concerned with making sure that a child with cystic fibrosis receives the pneumococcal vaccination and influenza vaccine.

Antibiotics medicine. Some of the newest antibiotic treatments can more effectively fight the bacteria causing lung infections in patients with the disease. Antibiotic treatments are now usually available on an outpatient basis. Antibiotics that are a aerosolized can be inhaled. A good example is tobramycin thats allow the medication to reach direct into a person’s airways. The setbacks to long-term use of antibiotic medication include the resistance of certain bacteria to antibiotics.

Other medications. The main goal of these medications is to help maintain clear airways. Some medications that might be prescribed include:

• • • • Theophylline
      • Steroidal inhalers
      • Nonsteroidal anti-inflammation drugs
      • Drugs that thin the mucus making it easier to cough up
      • Albuterol which is a Bronchodialator
      • Proventil

Regular exercise and staying hydrated are other ways to loosen the mucus. Physical therapy of the chest also plays an important role in the treatment of this disease.

Patients with cystic fibrosis require a way to remove thick mucus from their lungs. When lying down in a position that helps the mucus drain from the lungs, the person may need a parent or caregiver to help. Carefully bang or clap on their chest or back to loosen the mucus. Some patients may use a mechanical percussor better known as an electric chest clapper. Certain patients can use a vest that inflates with a machine attached that has high frequency vibration to assist coughing up secretions. Adults and children with this disease need to have bronchial airway drainage at least two times per day for up to 30 minutes. Teens and adults can learn to do this on their own, especially if using aids such as vests and mechanical percussors.

digestive problems, people with cystic fibrosis can take enzymes orally with food in order to better digest their food and get the nutrition they need. The physician can also prescribe vitamins and a specific diet.

Insulin shots are only needed if the pancreas stops producing insulin by itself.

Oxygen therapy treatment may be required as the disease of cystic fibrosis progresses.

Transplants of the lung and/or liver. In certain cases, a lung transplant has extended the life of people with cystic fibrosis. Although it is rare, a liver can also be beneficial.

Although there is no cure for cystic fibrosis, there definitely is hope. In the last few decades, advancements in treatment and technology has come along way. A major breakthrough in cystic fibrosis research came about in 1989, when medical researchers identified the defective gene that causes cystic fibrosis. Today, Medical Researchers are currently exploring new gene therapy advancements to slow down the progression of cystic fibrosis, or potentially cure it.

Decades ago, if a child had cystic fibrosis, they would die before reaching their teen years. Now, with new advancements and breakthroughs in treatments available, more than 50% live well into their 30s, and have a better quality of life.

In today’s society, children with cystic fibrosis can go to school, participate in sporting activities and have an active social life.

If you or a loved has cystic fibrosis or are interested in learning more, contact Miami Pulmonary Specialists today.