Cystic Fibrosis Treatment

When it comes to treatment, research and quality improvement, there are a number of things to bear in mind. This will ensure the furthering of a cure, the aid of others, and the health of you or your child.

~It can be scary, but don’t be afraid to ask questions. Further your understanding of what you are dealing with. There will be many people involved with you or your child’s care. It’s important to know what’s going on.

~First, find out what you are taking. Read the label when you get it. Is it the right stuff? Then, tell your CF physician about all the things you were or are currently taking. They need to know if these things or combinations of them affect progress adversely. Your care center will prescribe and act in a more effective manner if this is done.
~Have a plan. You need to work with a CF healthcare team to formulate a course of action.

~Don’t overdo it. What is the purpose of this test? Why another treatment? More might not always be better.

~Follow up with the care center after a test or procedure is done. Just because you didn’t hear from them does not mean that there isn’t news. Test results get lost in the mail. Phone numbers can get written down incorrectly. This also goes with asking questions. Find out when and how test results will be received. No news doesn’t always mean goon news.

~Stay abreast on advances in treatment and scientific discovery. There are over 1,000 mutations of the CF gene. Not all cases/symptoms are the same. There is not a single form of treatment.

Common Treatments of Cystic Fibrosis (CF)

When someone is first diagnosed with cystic fibrosis (CF), they might spend a short time in the hospital to receive diagnostic tests to measure their breathing as well as the functioning of the lungs.  They might also have a nutritional assessment. Doctors will want to be sure their lungs are clear and that they are on the right track with proper vitamins and dietary enzymes to help them maintain a normal weight.

After preliminary testing and a prescription of treatments, the patient will usually follow up with the doctor every one to three months. Treatments will vary from one child to the next depending on the severity of the condition. Cystic fibrosis is a genetic disease that is inherited when both parents are carriers of the CF gene.  Because there are hundreds of CF gene mutations, one child's symptoms may vary from other patients, and some conditions of cystic fibrosis will be worse than others.

Therapy Treatments for Cystic Fibrosis

There are two main therapies given to cystic fibrosis patients - pulmonary therapy and nutritional therapy.  Pulmonary therapy is a treatment to help clear mucus from the lungs through exercise, physical therapy, and medications. Chest therapy includes bronchial drainage.  This is accomplished by putting the patient in a certain position that promotes lung drainage. While in this position, the chest or back will be clapped and vibrated to help dislodge and move mucus.

There are mechanical aids available today that help perform this therapy. Inhaled medications are often prescribed to help with breathing. These can include mucolytics to help thin the mucus, bronchodilators for widening breathing tubes, and decongestants that can reduce the swelling in the breathing tube membranes. A new treatment that is being researched is an inhaled spray that contains normal CF genes. The normal genes can enter the lungs through the inhaler to help repair lung damage. Also, there is a protein repair therapy that repairs the defective protein in the body called CFTR.

Medications and Other Treatments

Antibiotics are usually administered to patients with cystic fibrosis for lung infections or other types of infections. These are often taken orally or in the form of aerosol. They can also be injected directly into a vein.

Nutritional Therapy

To help with digestive problems, the patient is often given a manageable diet that is high in calories and low in fat. The diet is also usually high in protein.  Pancreatic enzymes are prescribed to help with digestion. These enzymes are not habit-forming and will not alter behavior. Enzymes help the body to absorb the necessary nutrients from food.  They also help reduce the number of stools, amount of bulkiness in the stool, distension, and abdominal pain. Nutritional supplements given are vitamins A, D, E, and K.  To help with obstructions of the intestines, mucolytic agents and enemas are used.

Patients might also be told to keep salty foods handy. Their body releases excessive amounts of salt through sweat, and the body needs to be replenished of salt throughout the day.

Though these treatments cannot cure cystic fibrosis, they can help the patient better cope with the affects of the disease and live a healthier, fuller life.