CF - psychosocial issues

again not my area, but just my food for thought on the part D cliff hanger...

Issue in Pt D: Georgia (15y/o) has stopped her treatments and exercises.

Effect of CF on a adolescents:

• Adolescents will find it difficult to maintain a good social life due to their physical conditions and the fact that they are being controlled by their parents. Parents with children who have CF tend to be overprotective about their child doing certain things.
  These parents may fell that their chronically ill child is more susceptible to the dangers of the world.
• keeping up with school work can be difficult when you have to stay in the hospital for months at a time.
• If your friends have don't know you have Cystic Fibrosis, they will annoy you with questions about everything. They may wonder about your cough or all the pills you take.

this is a quote from the video i watched:

“Growing up has a lot of ups and downs when you have CF. The hardest thing for CF people (and me) I think, is that they want to be a normal person. No vitamins, aerosols, enzymes, Iv's, hospitalizations -- wouldn't that be great. It's hard for a lot of CF kids and even adults to remember that you have to do all those things to stay healthy. No matter what disease or problem you have, you still are a normal person. Another hard thing for me was not growing up as fast as everyone else. I'm not sure if that effects every CF person. But, for me, it sucked. In 8th grade, I only weighed 87 pounds. To me, I felt like a shrimp-- not just in 8th grade, up till about 10th grade. I didn't mature as fast as everyone else either. I hated that. I think I didn't have a lot of self confidence in myself at the time or maybe it wouldn't have mattered.
I always felt nervous taking my pills around other people. Even know when I go out to eat, I wonder what people think (It does not bother me as much now). I know it shouldn't matter, but I think every CF person wonders about it once in a while.”

Shantz

CF - clinical features

This wasn't really my area but I decided to post it up since I mentioned a couple of points during my presentation and just to expand a bit on what Vivian has done since Dr. Laksmi asked me to connect the pathophisiology with the signs and symptoms and complications...

So as mentioned earlier, the concentration of sodium and chloride within the ECF and ICF is disrupted because of the failure of opening of the chloride channel in response to elevated cAMP in epithelial cells (CFTR is a chloride channel). Consequences of this:

• In the sweat glands the chloride ions can not be reabsorbed from the sweat before it is expelled onto the skins surface, this also inhibits the reabsorbing of sodium ions. This results in significant loss of these ions, altering the overall balance within the body and which can cause abnormal heart rhythms.
• In the pancreas the inability to move chloride ions out of the cell and into the ducts also prevents the movement of water in the same fashion as the water normally followed the chloride ions via osmosis. These results in digestive enzymes not being carried out into the intestines through the water, the enzymes instead digest the pancreas triggering inflammation and mucus build up (90% of CF patients have pancreatic damage)
• The mutant CFTR also triggers mucous build-up in the lungs, causing the most noticeable symptoms of Cystic Fibrosis. The mucus can partially block parts of the lungs and most importantly is an excellent growing medium for bacteria, leading to chronic respiratory infections that inevitably result in lung damage.
• nutritional effects - malnutrition due to malabsorption and maldigestion
• delayed puberty and skeletal maturity
• males are always almost infertile owing to failure of development of the vas deferens and epididymis. Females are able to conceive but often develop secondary amenorrhoea as the disease progresses

~shantz~

Pathophysiology of CF

CF (Also known as mucoviscidosis or 65 roses)

• autosomal recessive inherited disorder (recap: both parents must be carriers; 25% chance for child to have the disease; 50% chance of being a carrier)


• single gene disorder


• gene mutation along long arm of chromosome 7 (7q21.3 to 7q22.1)


• There are at least 500 different genetic mutations associated with the disease, approximately 70% of the mutations are found to be deletion at position 508 (delta F508) in the amino acid sequence on chromosome 7, making it the most common CF mutation


• the gene codes for a membrane protein in epithelial cells which regulates chloride transport- cystic fibrosis transmembrane regulator (CFTR). Mutation results in defective CFTR


• In 20-30% of cases the secondary and tertiary structure of the protein is altered making the protein non-functional and leading to the chloride channels failing to open in the epithelial cells. The other 70-80% of cases involve the cells quality control processes destroying all the CFTR produced as it is ‘defective’, preventing the ion channels from even forming. Both result in an inability of the cell to control the concentration of ions in the intra and Extracellular fluid.
• So in summary (see diagram below):

xoxo~Shantz

source: Kumar and Clark and www.childsdoc.org

Addendum: Management of Cystic Fibrosis

Prepared by: Ji Keon LOOI

This is the very informative summary that I promised to upload.

From http://www.childsdoc.org/fall97/cf/fig2.jpg

Below is the discussion on the antibiotics prescribed to Georgia. Only relevant information is included to avoid overloading our meddies with an avalanche of information which might inevitably cause the burnout rate to increase.

Cannot be Emphasised More (Must know-lah)

Pseudomonas aeruginosa is the major culprit in causing complications in patients with cystic fibrosis.

Nice-to-know
Tobramycin

• Tobramycin sulfate is an aminoglycoside antibiotic used to treat various types of bacterial infections, particularly Gram-negative infections.


• Patients with cystic fibrosis will often take an inhalational form (Tobi®) for suppression of Pseudomonas aeruginosa infections. Tobramycin is also combined with dexamethasone as an ophthalmic solution (TobraDex®).

Ticarcillin

• Ticarcillin is a carboxypenicillin. It is almost invariably sold and used in combination with clavulanate as Timentin®.


• Because it is a penicillin, it also falls within the larger class of beta-lactam antibiotics. Its main clinical use is as an injectable antibiotic for the treatment of gram negative bacteria, in particular, Pseudomonas aeruginosa.


• Ticarcillin's antibiotic properties arise from its ability to prevent cross-linking of peptidoglycan during cell wall synthesis when the bacteria tries to divide, causing death.
  

Ciprofloxacin

• Ciprofloxacin is a broad-spectrum antibiotic that is active against both Gram-positive and Gram-negative bacteria. It functions by inhibiting topoisomerase, an enzyme necessary to separate replicated DNA, thereby inhibiting cell division.
  
• The major adverse effect seen with use is gastrointestinal irritation, common with many antibiotics. Because of its general safety, potency and broad spectrum activity, ciprofloxacin was initially reserved as a drug of last resort for use on difficult and antibiotic-resistant infections.

The information for the above drugs is all derived from http://en.wikipedia.org.

Learning Objectives for PCL 7

Here's a recap on the objectives for this week:

1) Describe the causes and symptoms of cystic fibrosis
2) Understand the process of diagnosing cystic fibrosis
3) Understand the need for multidisciplinary teams in managing this type of chronic illness
4) Review the principles of breaking bad news
5) Understand the difficulties, including family pressures, when managing adolescent patients

Hope this helps. =)

Diagnosing and managing cystic fibrosis in the developing world

This is the link to the summary of the article that talks about the existence of cystic fibrosis in the developing world.

Paediatric Respiratory Reviews, Volume 7, Supplement 1, 2006, Pages S147-S150

Diagnosing and managing cystic fibrosis in the developing world
Sushil K. Kabra, a, , M. Kabraa, S. Shastria and R. Lodhaa aDepartment of Pediatrics, New Delhi 110029, India

The full article is, unfortunately, inaccessible to us. Hope that the summary will be useful.

John

Epidemiology of Cystic Fibrosis

Cystic Fibrosis -the most common autosomal recessive disease for individuals of European
descent
-1 in 2500 newborns are affected with the disease, consequently 1 in 25
individuals are carriers.

• life expectancy -1959 (6 months)
• life expectancy -2006 (38.6 yr)
  -based on data compiled by Cystic Fibrosis Foundation - survival depends largely on the access to health care.
• 1989- the discovery of CF gene
• 1990- scientists successfully made copies of the normal gene, and added them to the CF cells in the laboratory dishes, which corrected the defective cells.
• 1993- the first experimental gene therapy treatment was given to a patient (modified common cold virus was used as carriers or delivery vehicle-carrying the genes to CF cells in the airway)
• the first drug therapy , 1993 =Pulmozyme (mucus-thinning drug) f(x)= improved respiratory infections and lung function.
• 1995, ibuprofen was found to reduce lung inflammation in children with CF, under controlled condition and in high doses.
• in late 1997, tobramycin solution for inhalation=reformulated version of antibiotics which was found to improve lung function and reduce no of hospital stays.
• other treatment strategies to correct the protein product of the gene are currently being tested in clinical trials.
  
• what makes these drugs unique is that the researchers appear to be treating the causes of CF not just the symptoms.
• in Malaysia, approximately 16 patients were diagnosed with CF from 1997 to 2003.

references: Cystic Fibrosis Foundation and National Peadiatric Institute

Respiratory Disorders similar to Cystic Fibrosis

Bronchiectasis

Bronchiectasis is defined as a permanent dilation of bronchi and bronchioles caused by the destruction of the muscle and elastic supporting tissue resulting from or associated with chronic necrotizing infections. It is not a primary disease but one that is secondary to persisting infection or obstruction caused by a variety of conditions, including cystic fibrosis. These include:

1. Bronchial obstruction: common causes include tumours and foreign bodies
2. Congenital or hereditary conditions:
- Cystic Fibrosis: obstruction and infection caused by secretion of abnormally muscid mucus
- Immunodeficiency states (e.g. hypogammaglobulinemia - lack of 1 or more specific antibodies): increased susceptibilty to repeated bacterial infection
3. Childhood pertussis or measles, TB and pneumonia may predispose to bronchiectasis.

Two processes are critical and interwined in the pathogenesis of bronchiectasis: 1. obstruction and 2. chronic persistent infection. Either of these may come first. Normal clearance mechanisms are hampered by obstruction, so secondary infection soon follows. If the obstruction is chronic, the infection persists and causes damage to the bronchial walls, distending the airways permanently; conversely, chronic infection results in obstructive secretions and inflammation throughout the bronchial walls, distending the airway and leading to irreversible dilation.

Clinical Features : -severe, persistent cough
- expectoration of mucopurulent, sometimes fetid (foul smelling), sputum
- sputum may contain flecks of blood
- clubbing may develop
- in widespread bronchiectasis, significant obstructive ventilatory defects occur leading to hypoxemia, hypercapnia, pulmonary hypertension and eventual heart failure (rarely).

Clinical features of common Obstructive Respiratory Diseases

Some of the clinical features of bronchiectasis are similar to other obstructive respiratory diseases, namely asthma, chronic bronchitis and emphysema, and may confuse the physician. Taking a thorough history from the patient (smoker? exposed to air irritants? dyspnea relieved by bronchodilators?) as well as having a good knowledge of the defining features of the common obstructive respiratory diseases will aid in clearing up this confusion and lead to a correct diagnosis.

Now, let us go through the clinical features of common obstructive respiratory diseases.

Asthma:
- severe dyspnea and wheezing during asthmatic attacks
- attacks last from 1 to several hours and subside either spontaneously or with bronchodilators and corticosteroids (obstruction is reversible)
- intervals between attacks are generally free from respiratory defects but there may be persistent, subtle respiratory defects detected by the spirometer.

Classic Emphysema (without chronic bronchitis):
- dyspnea, begins insidiously but is slowly progressive
- hyperventilation
- commonly known as 'pink puffers'
- barrel-chested

Classic Chronic Bronchitis (without emphysema):
- persistent, productive cough for at least 3 consecutive months in at least 2 consecutive years
- production of sputum may persist indefinitely without ventilatory dysfunction
- in severe cases, there may be significant outflow obstruction leading to hypercapnia, hypoxemia and cyanosis

COPD (chronic bronchitis with emphysema):
- features fall somewhere between those found in classic emphysema and classic chronic bronchitis
- gradual development of pulmonary hypertension which may lead to right heart failure, respiratory acidosis, hypoxia, cyanosis and peripheral oedema
- commonly known as 'blue bloaters'

Source: Robins, chapter 13 Basic Pathology, 7th ed

Contributed by John Lee

CAM options for Cystic Fibrosis

By: Chris

Sources:
http://jpepsy.oxfordjournals.org/cgi/content/abstract/24/2/175 Oxford Journals
http://www.umm.edu/altmed/articles/cystic-fibrosis-000045.htm University of Maryland Medical Center

Nutrition and Supplements
Following these nutritional tips may help reduce symptoms:
• Eliminate potential food allergens and foods that increase mucous production, including dairy (milk, cheese, sour cream, and ice cream), wheat (gluten), soy, corn, potatoes, cabbage, bananas, sugar, preservatives, food additives and excessive salt and meats. Your health care provider may want to test for food sensitivities.
• Eat more foods that decrease mucous production, including garlic, onions, watercress, horseradish, mustard, parsley, celery, rose hips tea, pickles, lemon, and anti-inflammatory oils (nuts, seeds, cold-water fish).
• Eat more foods containing digestive enzymes, such as papaya and pineapple.
• Avoid refined foods such as white breads, pastas, and sugar.
• Eat fewer red meats and more lean meats, cold-water fish, tofu (soy, if no allergy) or beans for protein.
• Use healthy cooking oils, such as olive oil or vegetable oil.
• Reduce or eliminate trans-fatty acids, found in commercially baked goods such as cookies, crackers, cakes, French fries, onion rings, donuts, processed foods, and margarine.
• Avoid coffee and other stimulants, alcohol, and tobacco.
• Drink 6 - 8 glasses of filtered water daily.
• Exercise moderately, for 30 minutes daily, 5 days a week.

You may address nutritional deficiencies with the following supplements:
• Omega-3 fatty acids, such as fish oil, 1 - 2 capsules or 1 tablespoonful of oil twice daily, to help decrease inflammation and improve immunity. Cold-water fish, such as salmon or halibut, are good food sources.
• A multivitamin daily, containing the antioxidant vitamins A, C, D, E, the B-vitamins and trace minerals, such as magnesium, calcium, zinc, and selenium.
• Digestive enzymes, 1 - 2 tablets three times daily with meals.
• Coenzyme Q10, 100-200 mg at bedtime, for antioxidant and immune activity.
• N-acetyl cysteine (NAC), 200 mg daily, for antioxidant effects. Alternative health care practitioners may use higher dosages. Check with your health care provider.
• Probiotic supplement (containing Lactobacillus acidophilus) , 5 - 10 billion CFUs (colony forming units) a day, for maintenance of gastrointestinal and immune health. You should refrigerate your acidophilus products.
• Grapefruit seed extract ( Citrus paradisi ), 100 mg capsule or 5 - 10 drops (in favorite beverage) three times daily, for antibacterial or antifungal activity, gastrointestinal health, and immunity.
• Methylsulfonylmethane (MSM), 3,000 mg twice a day, to help decrease inflammation.
• Whey protein, 10 - 20 grams daily mixed in favorite beverage, for support of immunity and weight gain, when needed.


Herbs
Herbs are generally a safe way to strengthen and tone the body's systems. As with any therapy, you should work with your health care provider to get your problem diagnosed before starting any treatment. You may use herbs as dried extracts (capsules, powders, teas), glycerites (glycerine extracts), or tinctures (alcohol extracts). Unless otherwise indicated, you should make teas with 1 tsp. herb per cup of hot water. Steep covered 5 - 10 minutes for leaf or flowers, and 10 - 20 minutes for roots. Drink 2 - 4 cups per day. You may use tinctures alone or in combination as noted.
• Ginkgo ( Ginkgo biloba ) standardized extract, 40 - 80 mg three times daily, for inflammation and as an antioxidant.
• Green tea ( Camellia sinensis ) standardized extract, 250 - 500 mg daily, for antioxidant and immune effects. You may also prepare teas from the leaf of this herb.
• Cat's claw ( Uncaria tomentosa ) standardized extract, 20 mg three times a day, for inflammation, immune and antibacterial or antifungal activity.
• Milk thistle ( Silybum marianum ) seed standardized extract, 80 - 160 mg two to three times daily, for detoxification support.
• Bromelain ( Ananus comosus ) standardized extract, 40 mg three times daily, for pain and inflammation.
• Ground Ivy ( Hedera helix ) standardized extract, 50 mg three times daily, to decrease mucous production and to loosen phlegm.


Homeopathy
Although very few studies have examined the effectiveness of specific homeopathic therapies, professional homeopaths may consider the following treatments to alleviate respiratory symptoms (such as those experienced from cystic fibrosis) based on their knowledge and experience. Before prescribing a remedy, homeopaths take into account a person's constitutional type -- your physical, emotional, and psychological makeup. An experienced homeopath assesses all of these factors when determining the most appropriate treatment for each individual.
The following treatments should be used under the guidance of a licensed, certified homeopath in addition to standard medical care provided by a medical doctor:
• Antimonium tartaricum -- for wet, rattling cough (although the cough is usually too weak to bring up mucus material from the lungs) that is accompanied by extreme fatigue and difficulty breathing. Symptoms usually worsen when the person is lying down.
• Carbo vegetabilis -- for shortness of breath with anxiety, chills, and bluish skin discoloration.


Acupuncture
Acupuncture may alleviate symptoms of cystic fibrosis. Acupuncture may help enhance immune function, normalize digestion, and strengthen respiratory function.


Massage
Therapeutic massage can help drain mucus from the lungs. Parents may reduce anxiety levels by massaging their children with cystic fibrosis and their children may benefit from receiving massage by having less anxiety and improved mood, which in turn may facilitate breathing.

Investigation of Cystic Fibrosis

Chest X ray of Cystic Fibrosis
Sweat Test

• For the past forty plus years, sweat testing has been the most effective, and therefore the most popular, test for detecting cystic fibrosis.

How sweat test is performed

• The sweat test is performed by using an electrode filled with pilocarpine
• A current is run through the electrodes delivering the pilocarpine under the skin.
• The electrodes are removed and the arm is left to rest for about a one-half hour with the filter paper.
• The filter paper will soak up any sweat released from the person's skin. Once the one-half hour is up, the filter paper is placed into a flask and rinsed to release the sweat from the filter.
• A digital chloridometer is used to measure the concentration of chloride ions in the fluid. If more than 60 mmol/liter is detected, cystic fibrosis is diagnosed.

note:The sweat test may not work well in newborns because they do not produce enough sweat. In that case, another type of test, such as the immunoreactive trypsinogen test (IRT), may be used. In the IRT test, blood drawn 2 to 3 days after birth is analyzed for a specific protein called trypsinogen. Positive IRT tests must be confirmed by sweat and other tests.
Heel Prick Test

• is a common procedure for taking a blood sample from the heel of newborn infants.
• Infants with cystic fibrosis 1 to 2 weeks of age show increased levels of immunoreactive trypsin in the plasma in the neonatal heel-prick test.
• The test is positive if immunoreactive trypsin is greater than 80 mcg/litre. It is currently the best screening test for cystic fibrosis but cannot be done after the first few weeks of life since the immunoreactive trypsin falls as pancreatic insufficiency develops.

Genetic Testing

• The diagnosis of cystic fibrosis can also be confirmed by genetic testing in a person who exhibits one or more typical symptoms or has a history of cystic fibrosis in a sibling.
• Finding two abnormal cystic fibrosis genes (mutations) confirms the diagnosis. However, because genetic testing can confirm only a small percentage of the more than 1000 different kinds of cystic fibrosis mutations, failure to detect two mutations does not exclude a diagnosis of cystic fibrosis.
• The disease can be diagnosed prenatally by performing genetic testing on the fetus using chorionic villus sampling or amniocentesis

Pulmonary Function Test

• may show that breathing is compromised and are good indicators of how well the lungs are functioning.
• reduced VC(Vital Capacity) suggest reduce of lung volume (restrictive lung disease),which is present in many patient with CF with advanced lung disease in whom lung parenchyma is destroyed due to chronic lung infection.Strictly speaking, restrictive lung disease may only be diagnosed is TLC(total lung capacity) is reduced
  
• reduced FEV1 suggest narrowing of airway (obstructive lung disease) which is invariably present in patient with CF
  

Chest X-ray & CT Scan

• chest x-rays and computed tomography may be helpful to document lung infection and the extent of lung damage.
• reduced VC(vital capacity) suggest reduction of lung volume (restrictive lung disease),which is present in many patient with CF with advanced lung disease in whom lung parenchyma has been destroyed due to chronic lung infection.Strictly speaking, restrictive lung disease may only be diagnosed if TLC(Total Lung Capacity) is reduced.
• reduced FEV1 also suggest narrowing of airway (obstructive lung disease) which is almost invariably present in patient with CF
  

Characteristic of CF in Chest X-Ray

• Atelectasis

  • Discoid, segmental, lobar with RUL predominance

• Mucoid impaction

  • Nodular and fingerlike densities along bronchovascular bundle

• Cylindrical or cystic bronchiectasis

  • Peribronchial cuffing

• Hilar adenopathy

• Pulmonary arterial hypertension and cor pulmonale

• Recurrent pneumonias, particularly Staph, Pseudomonas and P. cepacia

• Clubbing and hypertrophic osteoarthropathy can occur

• Recurrent pneumothorax is common

Contributed by Lawrence Oh

Sources:
http://pediatrics.about.com/cs/conditions/l/bl_cf.htm
http://www.merck.com/mmhe/sec04/ch053/ch053a.html

Cystic Fibrosis - Signs & Symptoms

Note: Specific signs and symptoms vary depending on the severity of the disease in each child and age

Signs & Symptoms:
1. Blockage of intestines (meconium ileus) – meconium becomes so thick it can’t move through
the intestines (tarry, greenish-black stools normally passed by infants in the first day or two
after birth)
2. Failure to thrive (weight loss and malnutrition)
3. Steatorrhea (bulky and greasy stools)
4. Salty taste to the skin – higher amounts of sodium chloride in their sweat
5. Frequent respiratory infections
6. Thick sputum – parents tend to overlook this because young children tend to swallow their
sputum rather than cough it up
7. Coughing or wheezing
8. Frequent chest and sinus infections with recurring pneumonia or bronchitis
9. Polyps in the nasal passages
10. Rectal prolapse – protrusion of part of the rectum through the anus caused by stools that
are difficult to pass or by frequent coughing
11. Blockage in the bowel
12. Displacement of one part of the intestine into another part of the intestine (intussusception)
in children older than 4
13. Clubbing of the fingertips and toes
14. Enlargement of the right side of the heart
15. Pancreatitis
16. Diabetes related to chronic pancreatitis
17. Cirrhosis of the liver due to inflammation or obstruction of the bile ducts
18. Male infertility (obstructive azoospermia)

Sources:
http://www.mayoclinic.com/health/cystic-fibrosis/DS00287/DSECTION=2
http://www.labtestsonline.org/understanding/conditions/cystic_fibrosis-2.html

(Posted by: Vivian)

Breaking Bad News

Right, it's time for breaking news. Breaking bad news, that is.

(Note from your friendly neighbourhood slacker: You can simply read the bold, coloured, numbered points and skip the rest)

1. Do it in a private place
And by this, we don't mean the janitor's closet. Don't break bad news right in the corridor of the hospital, or in front of a roomful of people. Take the patient aside, bring them to your office (if you are lucky enough to have one), or to an empty room.

2. As soon as possible
Don't wait til you receive a call from the nearest mental hospital stating that your patient has just been admitted due to a nervous breakdown thanks to waiting for ten days for that ominous phone call about whether they tested positive or not. ASAP people. In this world, nobody likes waiting.

3. Both parents together
When breaking bad news regarding children, don't give part A to Dad and part B to Mum. Get them both together, and impart your news to them then.

4. In clear and appropriate language
Don't speak Greek. And don't rattle off medical jargon just because you know your stuff, show off! Explain in clear and concise everyday English Language, as how you would explain it to your grandparents, or parents, who are NOT in the medical field, mind you. And by everyday English Language, we would think it would exclude words like 'dude', 'stuff', 'uh', 'erm' and 'you know'.

5. Appropriate emotions
Don't go in there with a big grin on your face and say, "Sorry matey, seems like you've got CF afterall!" 'Nuff said.

6. Give time for the patient (and parents or other caregivers/loved ones) to ask questions
You are not going to liftoff in 10 seconds to a russian space station. Don't rush through your news and then leave. That self-directed latte can wait!

7. Answer questions in an empathic and non-judgmental manner
I suppose we can all be trusted to behave like the fine medical students we are, right? Right?!

8. Provide supplementary written material (when possible)
Fact: If all else fails i.e. you suddenly have a temporary mindwipe thanks to that all-nighter you pulled before the OSCE, remember this point, and you will be fine. Examples of supplementary written material would be brochures, flyers, booklets, what not. In these days, the supplementary material would also extend to informative websites. NOT wikipedia.org, mind you (no offense to the kind folks who contribute to it).

9. Follow-up consultation for soon after
Fact: very little (only about 10%) of the 1st consultation is absorbed by patients/caregivers. They probably heard: "I'm really sorry, but you have tested positive. Blah blah blah... moo...moo...bahh..bahh..." So set up a follow-up consultation for soon after the first visit. Give the patient time for the information to sink in, for them to read through your supp. written material, and come up with other questions.

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.

Tasks For PCL 7

1. Pathophysiology of CF (Shantini)
2. Signs and Symptoms of CF (Vivian)
3. Investigations of CF, including clinical examination, sweat test, heel prick test. (Lawrence)
4. Management and Prognosis of CF (Ji Keon)
5. Respiratory disorders similar to CF - Problems in diagnosing CF (John)
6. Breaking of Bad News (Madhura)
7. Psychosocial issues faced by CF patients , consider support groups available in Australia for CF patients (Christine)
8. Epidemiology of CF, including developments in management of CF since 1969 and on-going research on treatment of CF (Sri)
9. CAM treatment options for CF patients (Chris)

Apologies to Christine, Sri and Chris for allocating tasks to you all without first asking your opinions. Hope you all are alrighht with it. If you have any questions about your tasks, please feel free to approach your friendly chairman for this week.

John