everyone has a story, here is mine:

I have Cystic Fibrosis, diagnosed at birth by Meconium Ileus (an intestinal blockage requiring surgery). Although considered healthy most of my life (with no lung involvement), I've always dealt with fatigue, digestion complications and sinus issues.

As a child it was thought I was miraculously spared the devasting effects of CF - it was believed I had a mild case of CF, and wouldn't develop the typical complications of a person with Cystic Fibrosis. Respectively, my health was taken for granted, and I wasn't taught the importance of lung maintenance.

So, when I woke up sick one January morning in 2002,
I simply thought I had the flu, and didn't feel the necessity to go the doctor. Two weeks later after no improvement, I found myself in the hospital, on I.V. antibiotics and oxygen for no less than 14 days.

Not until just recently have I come to understand that what I experienced was my first CF exacerbation, which, sadly, caused irreversible lung damage by waiting for treatment.
So much for being "healthy".

Although I tried to return to work, it proved to be too stressful, too demanding, and too exhausting.
So, that October I retired and started my "life with CF".
And here we are today....

Monday, February 18, 2008

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

Posted by Sally at 2:09 PM

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