What is Cystic Fibrosis (CF)

Where does CF come from?
You cannot "catch" CF, it is not contagious.
CF is not caused by anything the parents did, or didn't do, during pregnancy. The only way to get CF is to inherit 2 abnormal CF genes - one from each parent. Everyone has 2 genes when they are born that determine whether or not they have cystic fibrosis. Both of these genes need to be abnormal for someone to have cystic fibrosis. Cystic Fibrosis is very common among white (caucasan) people, and is the most commonly inherited life-shortening disease.

Statistics
One in 30 Americans are symptomless carriers of the abnormal CF gene
CF is not gender specific, boys and girls can inherit cf equally.

When two abnormal-gene carriers have children, there is a :

50% chance the child will be a carrier of the defective gene
25% change the child will have CF
25% chance the child will be unaffected (no cf, and not a carrier)

*based on recessive-gene statistics (and remember, statistics have no memory)

Symptoms of Cystic Fibrosis
Cystic fibrosis is present from birth, yet signs may not show up for weeks, months, or years afterward. People with CF can have a variety of symptoms, including:

• very salty-tasting skin
• persistent coughing, most likely with phlegm
• frequent lung infections
• wheezing and/or shortness of breath
• poor growth/weight gain in spite of a good appetite
• frequent and/ or difficult bowel movements

So, What is Cystic Fibrosis?

Cystic Fibrosis (CF) is a life-shortening/threatening, inherited (genetic), progressive lung disease, which affects the way salt and water move into and out of the body's cells. People with CF make mucus and secretions that are extra-thick, sticky, dry and hard to clear.
CF does not affect the brain or nervous system.

In cystic fibrosis patients, this thick mucus causes blockages of small tubes and ducts in the body. This mostly affects the lungs and the digestive system. They may develop chronic lung infection and disease, and they are unable to break down and absorb nutrients.

When someone has cystic fibrosis, chloride (part of what makes up salt) cannot pass through the cells normally. And more sodium (the other part of salt) may be pumped through the cells than usual—at least in the lungs. Both of these problems may cause lung mucus and fluid to be thicker and stickier than normal and sweat to be saltier than normal.

The lung problem can lead to progressive blockage, infection, and lung damage, and even death if there is too much damage (see The Cycle below), while the pancreatic blockage causes poor digestion and poor absorption of food, leading to poor growth and a failure to thrive.

The Cycle of Lung Damage:

More than any other factor, the lungs determine the health and life span of the large majority of patients with CF.

There is no such thing as a "mild" case of CF. Some may have no lung involvement at first, but if not maintained with treatments, damage can occur. Even with some lung involvement a person with CF can lead a normal active life. However, the progression of the disease makes it more difficult to take in oxygen, and, release carbon dioxide, therefore causing chronic fatigue and chronic lung infections, thereby altering one's life style to varying degrees.

Infection ----> Inflammation ----> Lung Damage -----> Infection ----->....

• White blood cells are sent to kill bacteria
• White blood cells release different chemicals to attack and destroy the bacteria
• These chemicals themselves cause inflammation
• These chemicals cannot distinguish between bacteria and airway tissue. It can also damage the cells lining the airways

In recent years, we've come to understand that this damage from inflammation from our own white blood cells is just as harmful as, or even more harmful than, the harm from the bacteria. If the damage to the airways continues, it can weaken their walls so that they become floppy. If the lung damage progresses, it can lead to permanent changes, such as scar tissue.

Progressive:
The progression of lung damage is most often very slow and subtle, but can be relentless. This is why the lung disease of CF is often referred to as "progressive" - if left to its own (and even in the majority of cases with treatment), the cycle continues and lungs progressively get worse. If this progression of infection, inflammation and lung destruction continue uninterrupted for too long, it will eventually reach a point where there is no longer enough healthy lung to bring oxygen into the body or to eliminate carbon dioxide.

How long to live?

There is NO C URE for cystic fibrosis

It is impossible to predict how long a single patient will live. However, with advancement in technology and medicines, there are better and more effective ways to manage CF. Those with cystic fibrosis are living longer and healthier than when it was first recognized in 1938. Just a few decades ago, nearly all children with CF died before the age of two.

By 1995, the average survival had improved to nearly 30 years, and longer. In the last 30 years, many new antibiotics have become available, making treatment more effective. The key to managing CF is treating the symptoms before lung damage occurs.

Pulmonary Function Tests (PFT's) are performed annually (or more) to determine and monitor the health status of the lungs. The very last resort for someone with extensive lung damage is a lung transplant. However, an individual has be close to death, since the transplant procedure itself can be risky.

Let's make C.F. stand for Cure Found
........................................................
Source: Cystic Fibrosis A Guide For Patient and Family, by David M. Orenstein (second edition)

CF Take Aways

The Basic Defect

1. The main problem with the cells that make up the lungs, pancreas, and sweat glands in those with CF, is that chloride cannot pass through the cells normally
   
2. Another problem is that sodium may be pumped through the cells more than normal
   
3. Both of these problems probably cause lung and pancreatic mucus and fluid to be drier than normal, and sweat to be saltier than normal
   
4. The cells work abnormally in different ways in people with CF, depending on which CF gene mutation they have
   
5. Possibly, new treatments can be designed to get around these cell abnormalities
   
   

Making the Diagnosis

1. Genetic testing is one method for testing for CF. However there are over 1,300 known mutations, and more unknown, therefore it’s not possible to detect every mutation
   
2. The sweat test is a better method for testing for CF. It measures the amount of salt the body produces
   
   

The Respiratory System

1. The lungs are the most important part of the body in people with CF, and they cause most of the sickness, and more than 95% of the deaths from CF
   
2. Thick mucus blocks the bronchial tubes in people with CF, causing infection and inflammation
   
3. The lung problem is progressive, meaning it keeps getting worse as time goes by
   
4. With very good treatment, the progression of the lung disease can be slowed dramatically, and the lungs can be kept relatively healthy for long periods of time
   
5. Regular treatment to keep the airways clear of mucus and infection is extremely important
   
6. New or increased cough is usually the first sign of worsened infection and inflammation. If cough increases, you should call your CF doctor for treatment
   
   

The Gastrointestinal Tract

1. Most patients need to take pancreatic enzymes with their meals to help digest their food
   
2. Patients who skip their enzymes (or whose enzymes are not working right) will have abdominal pain and frequent (abnormal) bowel movements, and will have trouble gaining weight
   
3. Some patients have intestinal blockage that needs to be treated promptly. The signs of this problem are “stomachaches” and fewer bowel movements than normal. Blockages can be life threatening and some require surgery
   
4. Some patients have reflux
   
5. Some patients have serious liver problems
   
   

Nutrition

1. Nutrition is important for people with CF for growth
   
2. Good nutrition for patients has 3 parts:
   High-calorie diet
   Enzymes with every meal & every snack
   Vitamins
   
3. Some require tube feedings if normally prescribed diet fails
   
   

Hospitalization and other special treatments

1. Some patients need to be hospitalized to treat worsening lung infections
   
2. Hospitalizations usually last 14 days, but can be shorter or longer
   
3. During hospitalizations, patients get antibiotics through and IV, and have blood tests and chest xrays
   
4. Some can have their IV’s at home
   
   

Transplantation

1. Transplantation does not cure CF. It simply gives the patient a new set of healthy lungs.
2. Transplantation is taking a healthy organ from one person and putting it in another person, usually to replace a damaged organ. In cf patients, this has been done with the lungs, liver and heart
   
3. Transplantation is very difficult and expensive, and requires as much or more care afterward than CF itself. Getting a transplant is much like trading one disease for another
   
4. Some patients have done very well after lung transplants, and some have had months or years of problems, and some have died
   
5. Medicines to prevent the body from attacking the transplanted organ are important after transplantation; getting too little of this type of medicine can cause rejection, while too much can allow infection to set in
   
6. Stats: /About 75% of patients who get a lung transplant will be alive 1 year later; /about 50-60% after 2 years. /More than 80% of children with CF who receive liver transplants will be alive 3-5 year later
   

Exercise

1. Exercise is good for virtually all patients
   
2. Patients lose more salt than normal with exercise, and therefore can get dehydrated and fatiqued quicker
   
3. Physically fit patients live longer than those who are sedentary
   
4. An active lifestyle should be encouraged from childhood
   
   

Genetics

1. CF is inherited. In order to be born with CF, you have to get two abnormal CF genes (one from each parent.)
   
2. People with just one abnormal CF gene are called “carriers,” and do not have CF
   
3. Each time two carriers have a baby, the chances are 1 in 4 that their baby will have CF, even if the couple has already had one or more babies with CF
   
4. It is possible to test during pregnancy whether the baby will have CF
   
5. It is possible to test to see if someone is a CF carrier, but the test will miss some carriers
   
6. Gene therapy may some day be possible to cure CF, though damaged lungs will remain damaged
   
   

The Family

1. Hearing that a child has CF is very stressful for parents
   
2. As parents learn more about CF, and participate in their health, the stress lessens
   
3. The CF center welcomes questions. They know that it takes a long time to learn all there is to know about CF
   
4. Children with CF should be treated normally; they need to do homework and chores, and should be allowed to participate in all normal childhood activities and socialize. Children treated this way grow up healthier emotionally and physically
   
   

The Teenage Years

1. The teen years are a crucial time for CF patients’ health. If the lungs are neglected, they can easily be damaged irreparable; with good care, they can often remain very healthy
   
   

Cystic Fibrosis and Adulthood

1. Most patients live well into adulthood
   
2. Most adults are able to study, work, date, marry and do most of the other things that adults do
   
3. Adults may have more health problems than younger cf patients
   
4. Adults have to deal with some difficult questions, including what kind of work they can do, whether they can or should have children, an how long they will live
   
   

Death and Cystic Fibrosis

1. Many cf patients think about death and most will die of their CF, and not of old age
   
2. People with CF do not choke to death on their mucus
   
3. Death is seldom sudden or unexpected for people with CF
   
   

Research and Future Treatments

1. CF research has increased our understanding of CF, and has provided new treatments
   
2. Basic researchers (researchers working in the laboratory) are now looking into the different CF gene mutations, how they CF cells interfere with salt and water transfer, how CF cells might be made to act like they didn’t have CF, and how to cure CF cells with gene therapy
   
3. Clinical researchers (researchers working with patients) are looking into new ways of fighting infection, decreasing airway inflammation, thinning CF mucus, and transferring healthy genes into CF patients’ cells
   
4. Both researchers and patients have responsibilities to continue CF research
   
5. New treatments are coming to market at an increasing rate
   
   

The Cystic Fibrosis Foundation

The CF Foundation is the national organization that raises money to support CF research.

The CF Foundation has been very helpful in increasing our understanding of CF and should lead to better treatments

Treating Cystic Fibrosis - a DAILY task

There is NO cure for Cystic Fibrosis, only ways to minimize the symptoms.

Treating those with Cystic Fibrosis is best done by a multidisciplinary team of specialists. The team consists of a combination of, or all of the following:

• Pulmonary Physician
• Nurse Clinician
• Nutritionist
• Pharmocologist
• Social Worker
• Respiratory Therapist
• Gastroenterologist
• Genetic Counselors (for those wishing to have children)
• Physical Therapist

The Foundation for cystic fibrosis has developed guidelines by which all accredited cf centers must follow. One recommendation is that each patient been seen by their cf team at least once every 90 days, and more if needed. The main purpose is to monitor the patients overall lung health by spirometry tests - a decline can signal a flare-up of infection

The goals of multidisciplinary treatment are maintenance of adequate nutritional status, prevention or aggressive treatment of pulmonary and other complications, encouragement of physical activity, and provision of adequate psychosocial support. With appropriate support, most patients can make an age-appropriate adjustment at home and school. Despite myriad problems, the occupational and marital successes of patients are impressive.

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The two major components in treating those with cystic fibrosis include treating the respiratory system and treating the digestive system.

Managing Respiratory - Airway Clearance --->Open, Thin, Clear

Daily breathing treatments are necessary in attempt to keep the cycle of lung damage from progressing too quickly.

Step 1 - The first step in the breathing treatment routine is the Nebulized Medicines - liquid solutions which are turned into a mist that can be more readily absorbed into the airways. This step can take 10 - 45 minutes and include one or all of the following:

• Bronchodilators - Medications such as albuterol which help open and keep open, the airways in the lungs which allow clearing mucus easier a task.
• Mucus Thinning Drugs - DNA, the basic material in your white blood cells, is released when these cells attack bacteria in your airways, making the mucus in the airways thicker.
• Antibiotics - Antibiotics are crucial to treating CF lung infections The benefits of nebulized antibiotics are that they go directly to the site of a lung infection. They can minimize the number of flare-ups requiring hospitalizations.

Step 2 - The second step in the breathing treatment routine is Chest Percussion Therapy (CPT). CPT is performed for a minimal of 30 minutes each treatment.

• Bronchial Airway Clearance - Since the medications only aren't powerful enough to complete the job, the person with cf needs a way to physically remove thick mucus from their lungs. Daily CPT, also known as percussion and postural drainage, helps to loosen lung secretions and stimulates coughing. In times past, the lungs were "percussed" by manual clapping with cupped hands. Today the patient can perform CPT without assistance with the investion of The Vest.

Standard reccomended therapy requires these two steps performed twice daily, although it's done more frequently when the person has an active lung infection, or in progressed lungs. Regular exercise also helps to loosen and move secretions.

Additionally, some patients benefit from a maintenance dose of anti-inflammatories such as ibuprofen, which help to reduce inflammation or swelling of the body tissues. People with CF have chronic inflammation in their lungs, and it's now known that this is one cause of lung damage.

Management of Digestion

The digestive problems in Cystic Fibrosis are typically less serious and more easily managed than those in the lungs.

To enhance digestion, supplemental pancreatic enzymes are taken before every meal and snack, and extra enzymes to help digest high-fat foods. Those with severe digestive impairment may require supplemental feedings either with a feeding tube or by vein. Patients who fail to take these enzymes with each meal can cause life threatening intestinal blockages.

People with CF must eat a well-balanced, high-calorie, high-protein diet. Their reduced absorption of nutrients often means that as children, they must consume up to 150% of the daily recommended allowances to meet their growth needs. Multivitamins and supplements of the fat-soluble vitamins (A, D, E, and K) are also important.

As you can see, CF is relentless. Management can slow, without guarantees, the progression of lung damage, but it requires dedicated and aggressive efforts, both by the patient, their family, and their health care team.

The Cost of CF

CF is a systemic disease

It is not confined to the lungs and digestion

Systemic - of or pertaining to a system
–adjective
Physiology, Pathology:
a. pertaining to or affecting the body as a whole
b. pertaining to or affecting a particular body system

Therefore, the ramifications vary widely from patient to patient, as not all those with CF suffer the same secondary issues or to the same degree.

So, here is MY cost of CF:

• $ 5,938.00 - monthly Rx's
• $ 1,000.00 - each 14 day stay - room/bed only - no tests, meds, etc
• $ 110 - 310.00 - each CF appt - just for signing in (not including labs/tests at visit) minimal of 4 visits a year, but usually more.
• $ 355 + 380.00 - annual testings
• $ 75.00 + $1,100 - monthly, for in-home medical equipment
• $ 120 - 280.00 - each breathing test - minimal of 4 times a year, but usually more

These figures do not reflect all labs, xrays etc; diabetic (& eye), allergy or psychological care... all secondary components to the CF.

This of course, would be the cost, if not for insurance. Between insurances nearly everything I need and require is covered. Thank God for insurance.

What is out-of-pocket: insurance premiums, extra groceries, fuel for appts*, & pet-sitting fees during hospitalizations.

* 180 miles round trip to care center, of which I have 7 diff dr's - none of which who's schedules aid in planning for combined visits. grrr

What is a Tune-Up

What is a CF Exacerbation?

From Dictionary.com:

ex.ac.er.ba.tion

• to increase the severity, bitterness, or violence of (disease, ill feeling, etc); aggravate
• to embitter the feeling of (a person); irritate; exasperate

- Synonyms: intensify, inflame, worsen.

----------------------------------------

• An exacerbation is when the CF kicks it up a notch. You feel lousier, you have a harder time doing things, you cough more, you cough up more, the bacteria starts fighting harder/growing more, maybe you lose more weight than usual. Any combination of these would compromise an exacerbation.
• An exacerbation is a worsening of symptoms that leads to needing a tune-up. Sometimes we call them exasperations because they're so frustrating and each one makes it more difficult to make a come-back.

What is a Tune-Up?

Just as our cars need regular maintenance to run efficiently and effectively, so do the bodies of those with cf. They refer to this maintenance as a "tune-up"A Tune-Up is recommended based on a few factors:

• increase in cough/"lung junk" and/or a decline in spirometry (pulmonary function test)
• weight loss
• elevated blood sugars (for those with cf related diabetes)
• increased fatique
• increase in fevers

As you can see, increase is the key here. Due to the chronic infection in the lungs of those with cf, it's possible to experience all of these symptoms on a daily basis, depending on the progression of their lung disease, and worsening with lung damage. However, when a bug is growing and multiplying, all symptoms pick up speed.

What is involved in a Tune-Up?

• minimum of 14 days on i.v. antibiotics (ab's), and sometimes longer depending on how well the body responds. Typically a combination of ab's are used to fight the different "bugs."
• aggressive respiratory treatments (rt), a minimum of 4 times a day, and some require more. (see Treatment of CF to learn what's involved in this process.)
• physical therapy - anything from treadmill walking to stretching exercises
• all the food they can eat - there is a proven connection between weight and lung function in those with cf. Tests show that a drop in weight results in a drop in pft's. When the body is fighting infection, it burns more calories and so not only is it important to keep the weight up, it needs extra for just those times of infection

Most begin their I.V. antibiotic therapy in the hospital. Some remain there for their entire 14 day course, while others choose to finish their therapy at home after an initial stabilization period. Most certified cf centers would rather see their patients remain in the hospital for several obvious reasons:

• They do all the work while allowing the patient's body to rest. As stated previously, a combination of ab's are typically used, which can mean 'round-the-clock dosing schedules. Some antibiotics are dosed every 4 hours, other every 6, or 8 or 24 hours. While hospitalized, nurses can begin the next ab w/o waking the patient, allowing them to get better rest. If the patient is at home, their sleep (or a family member's sleep) is disrupted.
  *It's important for someone who is ill to get plenty of quality rest. While in the hospital, the patients aren't disturbed if sleeping, for vitals or miscellanous tasks.
• At home, responsibilities call which makes it easier to put off caring for oneself. On the contrary, while in the hospital, they do all the work - the cooking, the cleaning, gathering all the medicines, and scheduling the breathing treatments - everything is brought to the patient. The patient is able to concentrate on getting well, rather than preparing dinner or seeing that the laundry is done.

Graph - Sally's Tune-Up Trends

Click on Graph to see in Full View

This chart graphs the frequency of tune-ups, how many days spent on I.V antibiotics per tune-up, and how many days between tune-ups.

• My fastest turn-around was this last one in February at 124 days between
• My longest stretch was April '07 at 285 days between

* incomplete data records to chart before February 2003

Sally's Current Health Summary - February - Obstacle, Hurdles & Hoops

First and Foremost:
FEV1 = 39%
Based on Lung Health Grading, I am at stage 3 of 3 - YIKES!

-----------------------

Weight - let's begin with the good news:

Since there is a proven connection between Weight and FEV1 (lung health measurement), these are important factors to the person with cf, and their cf team. A drop in weight = a drop in fev1. Therefore, eating to keep weight up is another task the cf'er must address in their daily treatment routine.

According to my BMI, my weight isn't bad. But there's always room for improvement. Everyone, including myself, would love to see a 5-10 lb gain for good measure, leaving room for time of infection and stress.

Putting weight on:

Cf makes it hard, infection makes it harder. When an infection flares up, the body burns more calories fighting infection and, more calories are burned in labored breathing - both of these can cause weight to drop quickly.

For me, eating is an ardous task, given my lack-of-appetite. Eating is something I must work at, like my breathing treatments - everyday and often.

Lung Function - the not-so-good news:

My fev1 (see What are PFT's) has hovered around 39%. (fairly crappy if I might add). To top that off, in February my pft number was about 25% below that 39 marker. Grrr. Needless to say, it was requested I schedule a tune-up.

However, of that 39% remaining working lung, I'm exchanging 92-94% good oxygen. (This is refered to as O2 sats.) So, this is a plus in the swimming pool of numbers, since supplemental oxygen is required when oxygen saturations are 88% and below. This means, when I'm not fighting infection, I don't need supplemental oxygen; yet when I AM fighting infection, my sats do drop to about 88%.

View graph (trends - under construction) and you will see that my overall lung health have been on the decline over the few years of tracking.


Lung Cultures:

I still culture the most common bugs:
Staph - staphylococcus aureus
Pseudo - pseudomonas aeruginosa


Blood work:

In February I learned that my Vitamin A level is extremely low, at 18, whereas normal-range low is 38. I was reminded that this vitamin is key to fighting infection. So, this is actually good news to me. If we can bring that number up, it could possibly but not necessarily mean less hospitalizations.

Taking in more Vitamin A, or D, or E, or K is difficult for someone with CF. In order for these vitamins to be absorbed into the system, they require fat; and due to the CF patients' trouble digesting and absorbing fat, the bloodstream levels of these patients are often low. Specificaly formulated CF-vitamins are required everyday.

Diabetes - another factor in my health:

I am a type-I diabetic, this means I am insulin-dependent. Keeping blood sugars controlled is AS important as everything else I do on a daily basis. High blood sugars "feed" infection, and infections "feeds" the sugar. It can be a vicious cycle and a frustrating battle. To add to the battle, there are other factors that affect blood sugar numbers:

• poor appetite - regular meals help keep blood sugars from rollercoastering
• unknowns - such as infection, or, medicines. Both can contribute to unpredictible highs, making management difficult.

The good news here is that my A1C's (a 3-month blood sugar average) has been coming down, and is just shy of normal-range. I hope to see less infection as a result.


...so, this is just a little bit of the Crap I put up with on a daily basis.
I find CF challenging these days. CF Sucks.


....but my husband I like

Graph - Now that Sally is retired, what does she do with her free time?

Click on graph to see full view

Per year, this chart graphs how many days were spent going to my CF doctor, how many days were spent going to non-CF health professionals, and how many miles were driven to get there.

Whew!

Medications for the person with CF

The ramifications of cf vary from patient to patient. Here is a list of the possible medications / treatments that a patient might take on a regular, on-going basis:

Respiratory System medications (Lungs)

• Antibiotics
  
• Bronchodilators
  
• Theophyllines
  
• Anti-inflammatories
  
• Mucolytics
  
• Oxygen
  
• Cough medicines
  
  

Upper Airway to treat:

• Polyps
  
• Sinuses
  
• Allergies
  
  

The Heart

• Diuretics
  
• Digitalis
  
  

Gastrointestinal and digestive system medicines

• Enzymes
  
• Acid reducers
  
• Antireflux
  
• Anticonstipation
  
• Antibloating
  
• Liver medicines
  
• Vitamins

• Probiotics
  
• Growth and appetite stimulants and supplements
  
  

Transplant related drugs

• Anti-rejection drugs

CF FAQ's

Is CF contagious?
No, CF is not contagious and is strictly genetic.

Can a healthy person catch the same bugs the person with CF is infected with?
No. “Healthy people don't get sick when exposed to the bacteria of a person with CF. What is more likely is for the person with CF to contract what others are carrying, which would result in a flare-up requiring hospitalization. Therefore, it's possible to see someone with CF wear masks when they are in close contact with the public. The mask is in place to protect their own deeply compromised lungs, since a simple cold germ could put the person with cf in bed for a month."

Why are CF’ers advised to keep distance from one another?
The bacteria that infects the lungs of those with CF are easily contracted by another patient with CF, so based on the recommondation of the CF Foundation, patients are advised to keep a minimum safe distance of three feet between each other at all times. If a bacteria is passed from one CF sufferer to another, the consequences can be deadly.

CF patients generally start off with comparatively mild infections like staph, but as the disease progresses, the strains that take root become progressively more virulent. Even with regular checkups, one can never be absolutely sure what kind of bacteria is growing at a given moment, and if a person with a particularly vicious bacteria passes it to another CF person who doesn't have it yet, they can inadvertently quicken the other's decline."

Do you share a room while in the hospital?
No, Never, not even with a patient without CF.

So, are you stuck in your room?
No. Patients are encouraged to get as much exercise as possible. They are seen walking the halls in the wee hours of the morning, as most suffer from insomnia.

What do you do for 14 days in a hospital?
Most patients bring necessities from home which make their stay a little more like home - family photos and other interests posted on the entrance to their room, their favorite boxes of cereal and such, laptop computers, ipods, cell phones, xbox - you name it.

How often does Sally get Tune-ups?
See Graph - Tune-Up Trend
Currently, I have an average turn-around of 6 months, sometimes sooner. At one point in my life, I never needed "tune-ups". But due to the nature of this disease, lung health has deteariated and I get sick more frequently. Every flare-up of infection leaves the lungs less able to fight the next one. Seeking appropriate treatment at the first sign of a flare-up keeps the lungs healthier longer. I learned this the hard way.


What causes CF?
Cystic fibrosis (CF) is caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene makes a protein that controls the movement of salt and water in and out of the cells in your body. In people with CF, the gene does not work effectively. This causes the thick, sticky mucus and very salty sweat that are the main features of CF.

What is the average life span for someone with CF?
The median age survival for CF patients in 2005 is 36.8 years.
This is up from 2004’s figure of 35.1 years.

And so, what is the longest a person with CF has lived?
The oldest person with CF was 76 years of age

Is CF visible?
Yes, it can be. Besides the respiratory symptoms of coughing, wheezing and shortness of breath, some patients also show other signs such as: clubbed fingers, barrel chest, and/or flared nostrils.

Does CF affect everybody the same?
No, CF affects every patient differently. Even siblings in the same family who have the same CF mutations will have a variation in their complications. Research is currently being conducted and investigated by the CF foundation to discover why this is.

How is Lung Health Graded?
In general, medical guidelines suggest the following with regards to the severity of lung damage, based on fev1 %’s:

Mild – 90% - 70%
Moderate - 70% - 40%
Severe - 40% and below


Are there different levels/severities of CF?
CF can affect patients differently. There are more than 1,300 mutations- or versions- of the defective CF gene. These mutations impact people differently. Some people have more severe lung disease at an early age, and others have more problems with their digestive system.

What is a CF mutation?
Genes are strung together on Chromosomes, rodlike sturctures found in the nucleus of each cell. Human beings have 46 chromosomes, arranged in 23 pairs. In each pair, one chromosome is contributed by the mother, the other by the father. The gene involved in CF is found on chromosome 7. Each person has two copies of the gene, one on each chromosome. Because this gene is recessive, a person must inherit two genes containing a mutation-one from the mother, one from the father-to experience the symptoms of CF. If only one gene has a mutation, the person will be a carrier, who can pass the gene on but will not have symptoms of the disease.

What are CF probabilities?
Probabilities can differ depending on whether or not each parent is a carrier or is affected with CF.

If both parents are carriers of the defective gene and have children, there is a:
25% chance unaffected (no CF, no carrier)
50% chance unaffected carrier
25% chance affected (CF)

If one parent is a CF carrier and one parent has CF:
50% chance affected (CF)
50% chance unaffected carrier

If one parent has CF and one is a noncarrier:
100% chance unaffected carrier

If one parent is a carrier and one is a noncarrier:
50% chance unaffected (no CF, no carrier)
50% chance unaffected carrier


What is a nebulizer?
A Nebulizer is a mechanism that is used to turn a liquid medication into a mist for easy inhalation. The liquid is turned into mist with the use of pressurized air generated by the nebulizer. A nebulizer may be hand-held or larger ones that require a mask to be strapped around the face.

Is coughing up blood common in individuals with CF?
Coughing up blood is referred to as Hemoptysis. A small case of it is common amongst CF patients. Chronic or severe hemoptysis can cause death.


What is 65 Roses?
"65 Roses" is what some children with cystic fibrosis (CF) call their disease because the words are much easier for them to pronounce.


Mary G. Weiss became a volunteer for the Cystic Fibrosis Foundation in 1965 after learning that her three little boys had CF. Her duty was to call every civic club, social and service organization seeking financial support for CF research.

Mary's 4-year-old son, Richard, listened closely to his mother as she made each call. After several calls, Richard came into the room and told his Mom,


"I know what you are working for." Mary was dumbstruck because Richard did not know what she was doing, nor did he know that he had cystic fibrosis. With some trepidation, Mary asked, "What am I working for, Richard?" He answered, "You are working for 65 Roses." Mary was speechless. He could not see the tears running down Mary's cheeks as she stammered, "Yes Richard, I'm working for 65 Roses."


Since 1965, the term "65 Roses" has been used by children of all ages to describe their disease. But, making it easier to say does not make CF any easier to live with. The "65 Roses" story has captured the hearts and emotions of all who have heard it. The rose, appropriately the ancient symbol of love, has become a symbol of the Cystic Fibrosis Foundation. 65 Roses® is a registered trademark of the Cystic Fibrosis Foundation.

Being Normal

"What would you give to be normal?" she asked
Looking me straight in the eye
I paused a moment in order to give
A completely honest reply

What would I give to be normal
The question, itself, wasn't new
Quick to mind came all the things
I could now no longer do

The list was long, and the list was sad
Of that I was painfully sure
I could sit a while, with my "losses list"
And continually add to it more

I returned my thoughts to the question
What did it really mean
And decided the answer was simpler
Than at first, to me, it seemed

I faced my well intended friend
My response, I knew, was true
"In every way that matters the most
I'm just as normal as you

"My world is restricted and narrow
Compared to where you reside
But it doesn't mean my spirit or soul
Need lessen in worth or size

"No matter how weakened my body
I'll persist at any length
Not to allow my frailties
To negate my inner strengths

"I wish, I hope, I dream, I pray
Quite often, I sit and cry
As long as there's laughter and love in my life
I'll know I'll continue to try

"As children of our creator
We are equally valued the same
We're not defined by health or wealth
Position, power, or fame

"I may never be fully, physically well
While here upon our earth
But normal or not, I'll do my best
Not to let it determine my worth"


~ Saundra Sharp ~

Please Don't - a poem by a cf author

Do not tell me that I'm fragile:
I've withstood storms so powerful
they could have blown my house down,
shattered my world,
and I am yet not broken.

Do not tell me that I'm weak:
I've fought enemies
invisible yet all the more violent
for their lack of size,
and I have not yet been slaughtered.

Do not tell me that you're worried:
I've made decisions, right and wrong,
steered my life and held its course
through many twisted paths,
and I have not yet lost my way.

Do not tell me that you pity me:
I find joy in every play of the light
against a window, and every breath
no matter how hard won,
and I am not yet ready to give up my joy.

Above all, please do not tell me that because my life may scare you
that I am somehow weaker,
somehow more afraid,
more easily broken.

Do not try to turn your fear into my weakness.

I am both a lover
and still a fighter.
So please, Don't.

~Piper - 26 yr old, lawyer, w/cf

Don't Assume (a poem my an unknown CF author)

Don't assume that because I look well, that I feel well. Looks can be very deceiving.
Many days I look great but feel terrible.

Don't say, "I know how you feel." No one knows how anyone else feels.
We all have varying thresholds of pain, and pain cannot be measured.

Don't tell me about your Aunt with so and so disease or ailment and how she managed in spite of it.
I am doing the best I can.

Don't tell me, "It could be worse."
I don't need to be reminded.

Don't decide what I am capable of doing.
Allow me to make those decisions.
There may be times I'm wrong, but I'll know soon enough.

Don't be upset that you cannot ease my problems.
It won't do any good for both of us to be miserable.

Don't ask me how I feel unless you really want to know.
You may hear a lot more than you are prepared to listen to.

Don't assume because I did a certain activity yesterday that I can do it today.

Don't tell me about the latest fad cure.
If there is a legitimate treatment, my doctor will let me know.

Do realize that I am angry and frustrated with the disease, not with you.
Do let me know that you are available to help me when I ask.
Do offer me lots of encouragement.
Do understand why I cancel plans at the last minute. I never know from one day to the next how I will feel.
Do continue to invite me to activities.
Just because I am not able to bike along with the gang
does not mean that I can't meet you for the picnic at the end of the trail.

Please let me decide.
Thank you.

~Author Unknown

Risks

To Laugh is - to risk appearing the fool
To Weep is - to risk appearing sentimental
To Reach Out for another is - to risk involvement
To Expose Feelings is - to risk exposing your true self
To Place your Ideas, your Dreams before the crowd is - to risk their loss
To Love is - to risk not being loved in return
To Live is - to risk dying
To Try is - to risk failure

But risks must be taken, because the greatest hazard in life is to risk nothing. The person who risks nothing, does nothing - has nothing. He may avoid suffering and sorrow, but he simply cannot learn, feel, change, grow, love, live.

Chained by his certitudes, he is a slave.
He has forfeited freedom.

Only a person who risks, is free.

Lessons From Geese (dedicated to George H)

As each bird flaps it’s wings, it creates an “uplift” for the bird following. By flying in a V formation, the whole flock adds 71% greater flying range than if the bird flew alone.

Lesson: People who share a common direction and sense of community can get where they are going quicker and easier because they are traveling on the thrust of one another

Whenever a goose falls out of formation, it suddenly feels the drag and resistance of trying to fly alone and quickly gets back into formation to take advantage of the “lifting power” of the bird immediately in front.

Lesson: If we have as much sense as a goose, we will stay in formation with those who are headed where we want to go and be willing to accept their help as well as give ours to others

When the lead goose gets tired, it rotates back into the formation and another goose flies at the point position.
Lesson: It pays to take turns doing the hard tasks, and sharing leadership. With people, as with geese, we are interdependent on each other

The geese in formation honk from behind to encourage those up front to keep up their speed. (Cheer them onward).

Lesson: We need to make sure our honking from behind is encouraging, and not something else

When a goose gets sick or wounded or shot down, two geese drop out of formation and follow it down to help and protect it. They stay with it until it is able to fly again or dies. Then they launch out on their own, with another formation, or catch up with the flock.

Lesson: If we have as much sense as geese, we too will stand by each other in difficult times as well as when we are strong

Moving day draws closer for Earthlings

By Dale McFeatters - published 04/03/08

mcfeattersd@shns.com

For your planning purposes, scientists have determined that Earth will end in 7.59 billion years.

Billion? For a second you had me scared there. I thought you said million. These kinds of huge numbers should be left to deep-space astronomers and the gnomes who draw up the federal budget.

If you must know, a gaseously expanding sun will suck Earth out of its orbit and engulf it in a lethal fiery embrace. Earth, so to speak, will be toast.

Earth could survive. We won't, but our planet, now reduced to a lifeless cinder, could be kicked into a higher orbit where it would circle what little is left of the sun to the end of time, serving as a warning to passers-by: Pick your solar system very, very carefully.

In fact, we'll go mucher earlier.

The science section of The New York Times, reporting on the end of days, says, "About a billion years from now, the sun will be 10 percent brighter. Oceans on Earth will boil away." And that can't come soon enough for the global-warming crowd, who see immolation as a small price to pay for telling the rest of us, "We told you so but you wouldn't listen."

The real trouble begins in about 5.5 billion years, when the sun transforms itself into a red giant. The fun-loving astronomers who calculated all this, Klaus-Peter Schroeder of the University of Guanajuato in Mexico and Robert Connon Smith of the University of Sussex in England, say, that as a red giant, the sun will be 256 times as big as it is today and 2,730 times brighter. That's beyond sunblock, people.

But, face it, it would be fun to watch - and we might be able to. While you were trying to figure out how long 1 billion years really is, your government and the astronomy community have not been idle.

About 15 years ago, no one had seen a planet outside our solar system and many believed one might not exist. Since then, according to NASA's Web site, 277 extra solar planets have been discovered, most of them gas giants, not the most desirable real estate in the heavens.

But as the search techniques have improved, astronomers have begun finding smaller, more Earth-like planets, some very likely with water and organic compounds. NASA's featured planet, Gliese 581c, is only five times the size of Earth, reasonably convenient at 20.5 light-years away and in the "habitable zone" of its sun, although NASA's idea of "habitable" is not quite the same as yours and mine. However, its "year" is only 13.5 days, a little too whirlwind for our tastes.

Viewed another way, we have 999,999,999 years to find another nice planet in a solar system whose sun won't incinerate us and arrange a move there. Start looking.

Jack and I

'Never give up!' aging Jack told budding biographer Ethan

BY DENNIS MCCARTHY, ColumnistArticle
Last Updated: 09/09/2007 12:27:57 AM PDT
http://www.dailynews.com/news/ci_6840964




"My buddy's name is Jack Etlin. He was born in 1918 in Pennsylvania. His father was a shoemaker and his mom stayed home. Jack doesn't have a religion. His childhood was fun. Jack played football and did wrestling. His hobby was reading books. He wanted to become a newspaper editor. His teachers didn't have any influence on who he is today. Jack doesn't wish he was a child again because he outgrew it. What has helped Jack the most in life is `never give up, finish what you start.' This has helped him because he succeeded in things and had fun."

Nine-year-old Ethan Jackola put down his pen and got ready for bed. The first chapter in the life of his 89-year-old new buddy, Jack, was done.


He'd start on the next chapters after his fourth-grade class at Balboa Magnet School made its next visit to the Jewish Home for the Aging to see their new buddies again.

His teacher, Angela Bronson, was right. The visits were fun. For 12 years, she'd been taking her class every year to the Reseda home once a month and letting the kids pick one buddy to write a short biography about. She does it to honor her own grandmother, who lived in a nursing home before she died. Bronson said she knew how lonely it could be for the residents when no one came to visit.


Ethan and Jack hit it off right away. Sure, Ethan had to repeat himself a lot because Jack was hard of hearing, but that was OK. Jack was always smiling, always glad to see him and answer any questions Ethan had. He couldn't wait to hear what Jack had to say in the next few chapters of his life.

"The only thing Jack remembers about the Civil Rights movement is Martin Luther King Jr. because where he lived there wasn't as much struggle as in the southern states. What Jack remembers about segregation is that blacks weren't treated fairly. Jack remembers that before the women's rights movement the women didn't have the right to vote and the men did. Jack thinks that women should be treated equally and given the same rights as men because they should be able to vote. Women shouldn't be inferior."


Sharon Jackola was proud of her son. She had never seen him so excited about anything as he was about writing his book, "Jack and I," for his new buddy. “When he talked about Jack, there was a special tenderness in his voice," she said. "He truly enjoyed and looked forward to seeing and being with him. Ethan would take him outside in his wheelchair and give Jack a big hug before leaving. He was so excited about giving him the book after finishing the last chapter."

"I made Jack feel happy. I know this because once when I had to go get Jack from his room, he was hanging his head down. When he saw me, he lifted his head and brightened up. That was a special moment for me and this made me know that I am special to him. At first I didn't hug Jack, but then I started hugging him when I left. If I could give Jack anything, I would give him a good book because he loves to read.


"Jack's advice to me was to do a good job with my work, never give up, and when you do a job, do a good job."


Ethan had done a great job. He couldn't wait for his next visit to give Jack his book before school ended last year. “All the kids walked in with the books they had written about their buddies," Sharon said. "Ethan and I went looking for Jack, but he wasn't in his room. He had been moved the week before by his son to the Jewish Home for the Aging in San Francisco to be near his family. While all the other kids gave their buddies their books, Ethan just sat in the corner alone. He looked so sad I almost started crying. We mailed Jack his book."


The letter from Craig Etlin, one of Jack's sons, arrived a few weeks ago.


"Dear Ethan,
With great sadness I am writing to tell you that my father, and your buddy, Jack, passed away last Saturday. He died peacefully in his sleep.
I wanted to let you know how much my dad, my children, and I have enjoyed the book you wrote about my dad. We kept it in his room, and every time I took my children to visit him, they wanted me to read the book to them.

Whenever I would get to the parts where you wrote `never give up,' my dad would look up and say, `Never give up! Never give up!'

"I hope you can hear his voice saying that as you read this, as I can hear it as I am writing this. Your friendship was a blessing to an elderly man in the last year of his life, and your book is a gift to my family that we will cherish in all the years to come."



Ethan said he felt sad when he heard Jack died, but not as sad as the day he walked into his room last year to give his buddy his book and found he was gone. "I knew he was pretty old and would die soon," Ethan said Friday. "I just wish I could have given him the book myself. I wanted to see him smile as he read it. I think he would have liked it."

Jack Etlin loved it.

dennis.mccarthy@dailynews.com

Dear CF

(warning: some offensive language)

I hate you. I hate what you've turned my life into - waiting on you to catch up with me.
Taking time away from what I want to do, what I wish I could do.

What would I be doing? I don't really know, I've forgotten what it was that I enjoyed

.....but, I certaintly never expected being on the disabled list, especially at this age.

Growing up, I wasn't sick. I took my health for granted, like most.
I didn't know the cycle, the progression;

I didn't know that although I was healthy then, I could be sick, today.
And behold, here I am...cursing you.

It was easier to deal with you when it was "just" constant stomach aches, though that was no picnic in the park.

But I was used to it, it was familiar.

Then you snuck in and started working on my lungs, my breathing....leaving me me less able to exercise, leaving my heart and muscles weaker, leaving me slowed down even more.

And now as the days pass, I can relate more to those cf'ers I once thought I would never be.

It's never ending. It's tiring, ...it's old.

My epitaph will read: I'm sick and tired of being sick and tired

You made me quit work - to do what?
You've turned my life into a mental fight with doctors, with insurance, and with myself.

Some wonder why I can't work.

Those are the some that don't live with me, don't see me during my coughing fits, don't see my thermometer, don't see the daily maintenance, don't see me getting short of breath from normal, low key activity.

I can't help what others believe about what they think I should be able to do, I know what my limitations are, I respect them, now.

My epitaph will read: I told you I was sick

By God's grace, I've been able to deal with it fairly well.

But, it was a long road to get here.
Yet, you still nag me everyday, and now, more often - you are more relentless.

I can deal with the hospital visits (I know I'm there to feel better), it's the day-to-day that is more draining - when the tiredness, achiness and fevers return not long after.

How disheartening.
That's worse.
CF, You suck.

Who knows how long I will live. (Who knows how long anyone will live)
The fact is - I'm not afraid of you, I'm afraid for my loved ones and their fear of you.
I can live with you, I can die with you, but what about them?
Show your face!
You taunt them everyday, with every fever, with every hospital visit.

It's not fair that you rob my (our) resouces, time and energy.

"but you don't look sick" they say, when inside I feel {aged}.
They say I look good, ...when I feel like shit because I've spent the morning in coughing fits, looking in the mirror at my gaunt pale face, sad still when I remind myself that gaining weight won't be easy - I must work at it, like I work at my breathing treatments, ...sad again when I've climbed the stairs 'too fast' and am short of breath, ..and when my body aches from chronic inflammation, ...when I wonder if it's time to go back in the hospital, again.

No one can see what I go through - they can't see my lungs, my thoughts, my heart. They can't see what it does to me, on the inside. ..how I loathe what's become of me.
They can't see the depression. oh sure, I can mask all my frustrations with chemicals, but it doesn't go away. For the sake of saving face, I put on a smile. Because, dispite you, I try.

You make taking care of myself mandatory, not an option - a chore, not a luxury.
You make living tedious. You made me a slave to you.
If I quit trying, you'll take me, eventually- so, for everyone else's sake, I keep trying. Somedays more, somedays less...but I keep trying, even when I've failed, I keep trying.

But my hopes are in the future...
Some day we'll kick your ass
Some day, there will be a cure, or on a lesser scale, better treatments

Until then
CF, F*ck you !

Respectfully, Your Slave
Sally