Living with Cystic Fibrosis and aspergillosis

by Hal Solof

CYSTIC FIBROSIS is a genetic disease, and is common throughout the world, but primarily in Europe and North America. As efforts to detect the disease are not prioritized, there is no absolute statistic concerning the number of people it effects. The American Cystic Fibrosis Foundation estimates that approximately 30,000 Americans have the disease. Their figures are based only on the number of patients at Cystic Fibrosis clinics who are reported by those clinics. Adults with CF rarely attend those clinics as the doctors are usually pediatricians. The CFF also estimates the median age of survival of the disease is 30 plus years, but once again, there are survivors in their sixties and seventies who do not attend CF clinics, and possibly thousands of persons with CF who have gone undiagnosed.

Cystic Fibrosis (CF) was discovered in the United States in 1938-39 in New York, when thousands of babies were brought to emergency rooms suffering from dehydration, difficulty in breathing, and refusing nourishment during one of the hottest summers on record. The babies usually had salt encrusted foreheads and other body parts, and low salt levels in their blood. Although I was eight years old at the time, I spent that summer in the hospital in Dallas, Texas, dehydrated, unable to hold down solid food, and fed via IV. After I spent several days in a cool pool of water, I was finally able to feed myself. I remained on IVs for saline and glucose. As my sister had been born that April, the doctors attributed my illness to jealousy of my new sister. Quite a burden for an eight year old. At age four, a child psychiatrist had told my parents that my illness was a means of my trying to get attention. Fortunately, I survived the doctors.

Scientists have established that there are approximately 900 mutant genes that cause Cystic Fibrosis. A person with only one of those genes is called a "carrier." A person with two mutant genes has Cystic Fibrosis. As it is a genetic disease, both parents must be carriers. Males with the disease have only a 1% chance of being able to father a child, as their vas deferens is usually clogged, or non existent. Females with the disease often have trouble conceiving due to poor health. Artificial means of extracting male sperm to fertilize female eggs most often is not successful. My sister, born in 1939, died at age 20, six weeks after giving birth to a baby girl, as the physical strain was too much for her body to survive. Her husband later remarried, and they raised the child who now has two daughters of her own. My niece and her daughters are probably carriers.

CF is insidious. Due to a malfunction of the pancreas, the lungs, sinuses, bowels, and other areas of the body become clogged with thick mucous preventing the proper absorption of food, breathing, and other body functions. The mucous is a haven for bacterial and fungus infections. It effects those with the disease in different ways. Some who have CF have severe sinus problems, but no digestive symptoms. Some have severe lung damage, and do not have sinus problems. As the health of those involved is usually at risk of infection, survival depends on access for the person with the disease to medical care and medications. Few doctors are trained to recognize or treat CF, and even fewer are trained to treat adults with CF. Most of us with CF take enzymes to help our digestive system, as well as vitamins, and some take prophylactic antibiotics.

It is estimated by scientists who are familiar with CF, that approximately 90% of those with the disease will have Aspergillus infections at one time or another. Persons with CF often have allergies, and as Aspergillus is a common fungus, an allergic reaction as well as invasive Aspergillus infection would not be unusual. Unfortunately, for many years, CF doctors did not take Aspergillus seriously if detected in their patients. It has only been recently that CF doctors have begun to treat Aspergillus as a threat to their patients here in the States. A friend with CF was the exception, as she was diagnosed with Aspergillus in 1988. She was not treated until it was found again in 1991. She was treated with Itraconazole (Sporanox)and then Amphotericin B for several years, and has not cultured the fungus since. I was diagnosed with Aspergillus almost a year before my pulmonary specialist decided to treat it with Sporanox. At first, the level of the drug was so low, it had no effect. My CF specialist raised the level, and I am beginning to respond. I requested information from others who have CF about their Aspergillus, and was informed that Sporanox and prednisone are the standard treatments, and all complained of gaining weight due to the prednisone. As persons with CF have a problem gaining weight due to absorption problems, rarely is there such a complaint. Most infants and children with CF are given special diets and protein enhancement to help them maintain a healthy weight level. I must have saved thousands of dollars not having to spend money on diet fads, and even today, at almost age 70, I'm asked how I stay so slim! As I also have hypoglycemia, I watch my diet. Unfortunately, I believe the trend is changing for me, as I have let my belts out a notch lately.

When Aspergillus invades the lungs of a person with CF, another medication used is budesonide, which is a steroid inhalant. CF lungs are usually so damaged, that inhalations with antibiotics, as well as IV antibiotics are required almost on a regular basis. Hospitalizations for "clean outs" whereby IV antibiotics, steroids, and other medications for some are done at least annually. Lung transplants as well as other organ transplants are done more frequently now, and survival rates continually improve every year. As there are too few organ donors in the States, many CF patients have their names placed on several waiting lists, but many die waiting. Those who are interested in becoming a donor can find further information from Donate Life America

The symptoms of those who have CF and those who suffer from Aspergillus are so similar, that it might be worthwhile to have all those with Aspergillus given the standard tests for CF. The usual testing method is by using electrodes attached to the patient's arms, which are wrapped with plastic to determine the level of salt in the sweat. It's a painless test. Other tests include swabbing the mouth of the patient, and the ultimate means of detecting CF is by a DNA study. I have had two DNA studies done by two different laboratories, and although I was psychologically inclined most of my life not to believe I had the disease due to my longevity, the DNA studies were conclusive. The weight of the accusations early in my life that my illness was psychological finally was removed.

Due to Cystic Fibrosis, I have had to make several adjustments in my life. I have had many sinus surgeries, and know that I will have to continue to need them. I married before I was diagnosed with CF, and though I had many illnesses, I had learned to get on with my life to a degree, that illness was just a part of it. I was an attorney with a New York Stock Exchange firm when I was diagnosed. When the doctors explained the disease to me, and the possible consequences, I did not take them seriously. When my sister died of the consequences of CF, I knew I had to be realistic. As my wife was a teacher, she encouraged me to go back to grad school, and I became a teacher. I taught at a private secondary school for twenty five years, and as an adjunct faculty member on the undergraduate and graduate levels at two colleges. I retired in 1990, and my wife retired in 1992.

We adopted two children when they were infants. Our son is an international fashion model agent in New York. He travels the world looking for beautiful people. Our daughter, tired of cold weather, recently moved to Hawaii, where she is a guidance counselor at a high school in Hawaii Kai. We have traveled extensively in Europe, Canada, and the United Kingdom, usually taking our children with us. We spent a year's sabbatical at the University of London, and lived in Greenwich. My wife and I are Associates of the University of London. We return to our "home away from home," Britain, almost every year, where we have many friends and relatives. My wife has had surgery for cancer twice, the last time in 1990, so we have no illusions about our health and mortality, and make the best of our lives, health permitting. Aspergillus has caused us to cancel a few trips, but as soon as the medications have it under control, we'll be on our way again.

Harold A. Soloff, Doctor of Jurisprudence, Master of Science, Professional Diploma in Education, Associate, University of London, Attorney at Law (ret.)

Any questions on CF? Email Hal by clicking here

The International Cystic Fibrosis Support Group web page address is: http://cf.conncoll.edu