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For my 2007 Diary, click on the '2006 Diary' button to left and scroll all the way to the bottom of the 2006 Diary page. My new 2007 Diary begins there.
Why am I considering a lung transplant? I have Cystic Fibrosis (CF) Cystic fibrosis is a genetic disease affecting approximately 30,000 children and adults in the United States. CF causes an abnormally thick, sticky mucus, due to the faulty transport of sodium and chloride within cells lining the lungs and pancreas. People with CF typically suffer from chronic respiratory infections and digestive disorders. Symptoms of CF can include salty-tasting skin; persistent coughing, wheezing or pneumonia; excessive appetite but poor weight gain; and stinky stools. Most people with CF develop symptoms early in life, however, some people are diagnosed in their teenage and adult years. The sweat test, which measures the amount of salt in the sweat, is the gold standard for diagnosing CF. A high salt level confirms CF. With over 1250 CF mutations discovered so far, about 85% of those with CF have 2 CFTR mutations which helps confirm the diagnosis. However, some mutations are rare and can not be found, so with 2 positive sweat tests and clinical symptoms, the diagnosis of CF can often be made. Abnormal movement of salt in and out of the cells lining the lungs causes secretion of thick, sticky mucus. This mucus is not cleared from the airways and as a result, bacteria thrive in the moist and damp regions of the lungs. Pseudomonas aeruginosa is the most common bacterium, causing lung infections in people with CF. By the age of 17, nearly 70% of people with CF have Pseudomonas aeruginosa in their lungs. Chronic infection leads to a vicious cycle. Pseudomonas aeruginosa infections People with CF experience acute flare-ups (exacerbation’s) of their pulmonary symptoms. These exacerbation’s often lead to more rapid decline in lung function. Exacerbation’s are the primary reason for hospitalization for cystics. In most people with CF, chronic lung infections cause gradual destruction of lung tissue. This leads to progressive lung disease, and eventually, respiratory failure. In fact, the Cystic Fibrosis Foundation estimates 95% of people living with CF die of respiratory failure. I do chest physical therapy 4 times a day for 30 minutes each time using the ThAIRapy Vest, which vibrates and percusses my chest to move the sputum out of my lungs. Antibiotics are used to treat lung infections in most instances. I also inhale liquid medicines directly into my lungs (Albuterol, Tobi & Colistin) to help control lung infections. Does having a double lung transplant cure CF? NOPE! But it means you won’t die from CF lung disease. The most frequent misconception regarding have a transplant is that once the transplant is done, the patient returns to a normal life. This is often not true. Rejection of the new transplanted organ is a never-ending threat, only partially controlled by great quantities of medications daily, which have to be taken for the rest of your life. Even if episodes of rejection remain minimal and caught early, the side effects from the medications are often so severe that that a return to normalcy is virtually impossible. The major immunosuppressants, combined with steroids, have caused, among other problems, kidney failure, diabetes, hypertension, cataracts, severe mood swings, osteoporosis and lymphoma.
...”A transplant won't solve Lori’s medical problems, merely change them”.
Since the patient now has a severely weakened immune system as a result of taking a combination of these medications, minor infections like the common cold can become life threatening. Yet the value of transplantation is certain. Hundreds of people with CF have been returned to productive lives because of transplants. Children have enjoyed the opportunity to experience adolescence and beyond. Young adults have gone off to college, married and started careers. Other adults with CF are growing older and living to play a role in their grandchildren’s lives. I have yet to meet any person with CF who, after having received their new lungs, voiced any regret of their decision. They have all said they would do it over again. How do you get CF? One in 25 Caucasians is an unknowing, symptom less carrier of the defective CF gene. A person must inherit a defective copy of the CF gene - one from each parent - to have CF. Each time two carriers conceive a child, there is a 25% chance that the child will have CF; a 50% chance that the child will be a carrier; and a 25% chance that the child will be a non-carrier. So in my case, I lost out in the genetic lottery of life. Both my parents carried the gene for CF and when they had me, I inherited both their defective CF genes. The span of ages of those with CF goes from birth to the oldest living survivor who is currently 73 years old! This is indeed rare. The average life expectancy has slowly crept up to 33 years old (but don’t tell that to the 73 year old adult!). Still, far too many young people die in their 20s, when they are just beginning to experience life. Which is where transplant comes in. It is a life prolonging treatment which can allow for a renewed quality of life and a chance to do normal things again. On average, 1 in 4 people waiting for new lungs gets a suitable donor, while the others die waiting for a donor that never came. Let me caution the reader: it’s a trade off--you give up one disease (CF) to take on the complications of dealing with being an immunocompromized transplant patient. It’s not all smooth sailing, they tell me. The bright sunshine of it is that of many who have gone the route of transplant, I have not heard one person tell me they had regrets. Frustration, discouragement and anxiety, yes, but regrets, no. What Next? If I knew those answers, I would be a genius. You’ll have to keep reading to find out the ongoing adventures of my life while I am waiting for lungs.
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cause lung inflammation, which causes more mucus secretion. More mucus secretion and lung damage leads to further lung obstruction, encouraging the growth of more bacteria, causing additional deterioration of lung function. Although the rate of decline in lung function FEV1 varies from person to person, it is estimated to be between 1.4% and 4% per year. With this continued decline, the possibility of a double lung transplant becomes an option.
