Cystic Fibrosis | Diagnosis & Treatments

Cystic fibrosis diagnosis

All newborns in Massachusetts — and most other states — are screened for cystic fibrosis. If doctors suspect that a child has this disease, they typically test a sample of the child’s sweat to determine how much salt (sodium and chloride) is in it. A high level of salt indicates cystic fibrosis. The test takes about 40 to 45 minutes from start to finish.

How is cystic fibrosis treated?

At the present time, there is no cure for cystic fibrosis, but with medical and psychosocial support, many children and adolescents with the disease can cope well and lead a productive life. The goal of treatment is to ease severity of symptoms and slow the progress of the disease.

Cystic fibrosis treatments

Early treatment for cystic fibrosis can improve a child’s quality of life and increase their life expectancy.

A child's treatment is likely to include:

  • chest physical therapy to help loosen and clear lung secretions
  • exercise to loosen mucus, stimulate coughing, and improve overall physical condition
  • medications, such as bronchodilators and anti-inflammatory medications, to reduce mucus and help breathing
  • antibiotics to treat infections
  • management of digestive problems, which may involve appropriate diet, pancreatic enzymes to aid digestion, vitamin supplements, treatments for intestinal obstructions, and dietary supplements
  • ongoing screening to detect early-stage liver disease and intervene as needed
  • psychosocial support to help deal with issues such as body image, feeling different, and managing the condition in a school setting

Managing cystic fibrosis-related diabetes

If a child has cystic fibrosis-related diabetes (CFRD), they may need to see an endocrinologist in addition to their cystic fibrosis care team. Children with CFRD usually need to be treated with a combination of insulin, diet, and exercise.

Insulin helps glucose enter the cells of the body so it can be used to produce energy. Typically, insulin is injected through a short needle into the fatty layer just under the skin of the belly, leg, or arm.

Children with CFRD should continue to eat a high-calorie diet high in protein and salt, however, they will need to monitor the amount of carbohydrates they eat. Physical activity, such as jogging or playing sports, helps maintain your child’s lung function and regulate blood sugar.

Organ transplant for cystic fibrosis

An organ transplant won't cure cystic fibrosis. However, in severe cases, a transplant may enable a child to live a longer and healthier life.

  • Lung transplant — usually a double-lung transplant — may be an option if a child has end-stage lung disease. A lung transplant replaces the diseased lungs with a healthy pair of lungs.
  • A child may need a liver transplant if their liver becomes so scarred it can no longer function

Avoiding cross-infection

It’s important for children with a cystic fibrosis diagnosis to stay a minimum of 6 feet away from others with cystic fibrosis. This is because people with this disease are at high risk of spreading dangerous germs to each other. Also known as cross-infection, this can be a very serious concern.