Management and Prognosis of CF

Prepared by: Ji Keon, LOOI

in consultation with the resources below:

http://www.med.monash.edu.au/paediatrics/resources/cf.html
http://www.cysticfibrosis.org.au/treatment/Physiotherapy/
http://www.cff.org/treatments/

Due to the enormous amount of information on the management of cystic fibrosis, the information deemed important is highlighted in red.

Physiotherapy

Airway Clearance Techniques
Chest Percussion
· aids clearance of secretions up and out of the lungs and increasing the amount of air entering the lungs.
· helps to prevent the thick, sticky lung secretions from blocking the air tubes. This helps to reduce infection and prevent lung damage.
· A cupped hand is used to clap the chest firmly (it is more comfortable through clothing or a towel).

Vibrations
· done in a drainage position in alternation with percussion.
· "Vibes" are a gentle shaking or vibrating of the chest whilst breathing out.
· This creates mini bursts of air flow which dislodge the secretions, moving them up and out of the airways. They are usually followed by a huff or cough in combination, to clear secretions from the lungs.
Breathing Techniques (The Active Cycle of Breathing Techniques)
· By controlled deep and shallow breathing to move mucus up through the airways.
· help re-inflate any areas of the lungs which may have been deflated because of mucus blockage of the airways.
Flutter Therapy
This technique uses a handheld oscillating positive pressure device through which you breathe out against an alternating resistance. This causes back pressure which results in expansion of the small airways, keeping them open for longer, whilst mini bursts of air flow move secretions out of the small airways and into larger airways where they can be cleared with a huff of cough in combination.Positive Expiration Pressure (PEP)
Involves breathing out though a mask or mouthpiece where a backpressure is created in the lungs by only allowing air out through a small hole.
This back pressure expands the small airways and opens side channels (collateral ventilation) to get behind secretions, moving them to larger airways where they can be cleared.
Autogenic Drainage
This technique was developed in Belgium and is a controlled method of breathing that requires no equipment. It involves training to breathe at three lung volumes:
low-lung volume to 'unstick' mucus
mid-lung volume to 'collect' mucus
high-volume maneuvers to 'expel' mucus
This technique requires extensive training and frequent practice.
Always ask your doctor before considering any change in your treatment
Medication
Cystic Fibrosis affects the lungs and the digestive tract - these areas are likely to require medication.
Lungs
Medication can be administered in various ways: inhaled into the lungs using nebulisers, taken orally, or taken intravenously.These drugs treat the lungs in the following ways:
Bronchodilator drugs open the airways by relaxing the surrounding muscle. They relieve tightness and shortness of breath.
Antibiotics help to treat or control persistent infection.
Steroids reduce inflammation in the airways.
Digestive System
Cystic Fibrosis affects the pancreas, so enzyme replacement capsules should be taken with meals and snacks to replace pancreatic enzymes and enable people with CF to gain more energy from the food they eat.
Pancreatic Enzyme Insufficiency
Pancreatic enzyme insufficiency is one of the common symptoms of CF. The CF gene defect results in a thickening of the pancreatic secretions. The accumulation of thick secretions causes obstruction and eventually leads to damage of the pancreas. Pancreatic enzyme insufficiency prevents digestion and absorption of nutrients. Large, bulky, smelly stools containing undigested nutrients are a direct result of a lack of pancreatic enzymes.Pancreatic enzyme insufficiency is treated with pancreatic enzyme replacement capsules, which should be taken with every meal or snack. Dosing can be based on the fat content of the food items. Foods containing only carbohydrate, such as fruit, juices and boiled lollies will not require pancreatic enzyme replacement capsules.
Pancreatic Enzyme Replacement Therapy (PERT)
Specially coated enzyme beads (microspheres) have been a treatment breakthrough for people with CF. Effective and acceptable enzyme replacement therapy has been a significant factor in maintaining health for those with CF. For infants and younger children, who cannot swallow capsules whole, the capsules can be opened and the enteric coated microspheres mixed with fruit gel. This is then fed to the infant before and during their feed. Older children can have the microspheres mixed with yoghurt or pureed fruit.
Dosage Of Enzymes
The physician and dietitian will monitor pancreatic enzyme therapy dosing. The dietitian will provide information on the correct dosage of enzymes for different foods.
Enzyme Storage
Australia has a unique climate which is not suited to preserving the potency of enteric coated microspheres. Parents and adults with Cystic Fibrosis need to be aware that this is costly medication and needs to be handled carefully. The Pancreatic Enzyme Replacement Capsules need to be stored below 250C, and kept in the dark and dry. They do not like to be left in the car. During summer, the preparations can go off quickly.Enzymes available in Australia are:
CREON® - Solvay Pharmaceuticals (available in several strenghts)
COTAZYM® - Organon
PANCREASE - Janssen-Cilag - Discontinued in Australia December 2005
Your dietitian or doctor can advise you on the appropriate type/dosage of enzyme supplement.
Other Organs
Bones can be effected by a lack of minerals, which can cause osteoporosis (weak/brittle bones). Bisphosphonates, which are used to treat osteoporosis in post-menopausal women, have been shown to be beneficial for the treatment of osteoporosis in CF too. Research is being carried out to investigate the benefits of high doses of vitamin D and calcium.
If someone with CF is having liver problems, they will be treated in the same way as other people with this condition. Promising results have been reported following early treatment with ursodeoxycholic acid.
Nutrition
Nutrition in a person with CF plays a very important role in the treatment and management of their condition. Research has shown there is a link between weight gain and survival. People with CF are required to consume between 120-150% of the daily amount of food recommended for a person of the same age and size that does not have CF. This is needed to ensure they:
Increase their chance of absorbing the nutrients needed for normal and healthy growth (in children) and general sustained health
Increase their body weight in order to fight off infections particularly in times of exacerbation of lung illness
The diet for a person with CF is high in calories, high in fat, high in salt, and high in sugar. Often when children or adults are sick they lose their appetite and will lose weight. Having a good body weight helps them when they are ill as appetite is often reduced and they can lose weight.They need to ensure they maintain a healthy body weight to assist in fighting infection and to help them through when they get sick.Cholesterol is NOT an issue in CF. Increased salt is required to replace that lost through sweat. Good nutrition is important for people with CF and a diet with fruits, vegetables, proteins and fats is required to maintain health. Other Supplements
People with Cystic Fibrosis have difficulty absorbing some vitamins, especially those that are fat-soluble. Often they will need to take supplements of vitamins A, E and K.People with Cystic Fibrosis have sweat that is five times saltier than people without Cystic Fibrosis. This puts them at risk of dehydration and salt depletion in hot weather or with increased activity. The high salt loss means the body's thirst mechanism does not trigger, so the person does not feel thirsty as they lose fluid. To make sure they do not dehydrate, they need to drinks lots of sports drinks (like Isosport) or Glucolyte, take salt tablets and maintain water intake. Salt input needs to be distributed across the day as the very salty sweat means salt loss can be very rapid. Lack of salt and fluid can lead to dehydration which can cause irritability, cramps, headaches, lethargy and fatigue.
Exercise
Research demonstrates a clear link between regular exercise, weight gain, body mass, lung function and survival in CF.
Exercise is an important part of the daily treatment routine for people with CF as it assists with airway clearance and builds up muscle mass and strength.
Coughing is a natural part of having CF. People with CF are encouraged to cough and should not suppress this important airway clearance mechanism. However, it is not convenient to expirate in the pool or gym and therefore airway clearance should be done prior to exercising.
During sporting activities people with CF may experience coughing, wheezing and/or breathlessness.
If any of these symptoms are experienced by a person with CF, it does not necessarily mean that they need to give up their sporting activity. If in doubt individuals should consult their doctor and physiotherapist. Some may need to use a bronchodilator before exercising.
People with CF are encouraged to drink plenty of water during and after exercise to avoid dehydration especially in hot weather.
Salt replacement tablets may be needed during periods of exercise as people with CF lose excessive amounts of salt in their sweat. Speak to your dietitian or doctor.
CF specialist clinics should be consulted to advise gym instructors and physical education teachers if they have any concerns regarding the participation of a person with CF in specific physical activities.

Transplantation
The following general information has been provided as a basic guide to people with CF. For information that relates to their personal health and situation people are encouraged to discuss the issue with their CF specialist.
Type of lung transplantation
In CF the method of lung transplantation is bilateral sequential transplant where both lungs are removed and replaced with the donated lungs. This removes the risk of a new lung being infected by the bacteria in the remaining lung.
Does CF “go away” after transplant?
The transplanted lungs come from people who do not have CF, so the new lungs do not have and will not develop CF. However, after transplant the person still has CF in the pancreas, sweat glands, sinuses and reproductive tract. The person still has to take enzymes with food and to help absorb the anti-rejection medications.
The transplanted lungs come from people who do not have CF, so the new lungs do not have and will not develop CF. However, after transplant the person still has CF in the pancreas, sweat glands, sinuses and reproductive tract. The person still has to take enzymes with food and to help absorb the anti-rejection medications.
Challenges
Transplanted lungs are susceptible to infection, rejection and other complications that require treatment. Following transplantation the immune system is suppressed to help prevent organ rejection and this will decrease the ability to fight infections. Therefore people must become vigilant about their drug treatment in that they need to take their drugs at the same time every day. This ensures that the most constant levels of immunosuppressive drugs is maintained. At first, this, along with the number of drugs, can be quite overwhelming. However, people with CF tend to adjust easily as they are used to taking daily medication. They are swapping one lot of treatment for another. They are also used to attending outpatient clinics and generally taking an active role in the management of their treatment. In addition their carers and families are well rehearsed at providing much needed support.
The immunosuppressive drugs may cause side effects such as diabetes, kidney problems, cancer-like tumours and osteoporosis. Your transplant centre will be monitoring your progress.
It is important to maintain contact with your CF Clinic post transplant as they are skilled in the total management of CF which still affects you, apart from your lungs.
Waiting
There is a shortage of donor organs in Australia and the wait for lungs is (on average) between 9 – 12 months. The waiting time is very difficult for most people as they struggle to maintain their weight and level of physical activity in an atmosphere of uncertainty. Acceptance on the active waiting list does not guarantee that suitable lungs will be found in time. When suitable donor organs become available the best matched recipient will be contacted. The length of time waiting for new lungs makes little difference to priority on the list. The person with the most compatible blood group and size will be selected. If there is more than one person considered to be suitable then the sickest person gets priority.
Life expectancy after lung transplantation
Currently in Australia over 85% of all people (not just with CF) undergoing bilateral, sequential lung transplants survive at least one year and 60% are still alive five years after the transplant.
Benefits
After recovery from the surgery many people with CF find they have more energy, can gain weight more easily and are more active than they were before.