CF Diagnosis & Treatment

DIAGNOSIS: Most people with CF are diagnosed in early childhood. Jim was not diagnosed until he was 33 years old. He has always had problems with coughing, lung infections, and digestion but no one ever put them together to realize he had CF. He had extreme difficulty gaining weight. We started pursuing fertility treatment in 1998 after 4 years of marriage. We pursued many types of testing. When we bought our house on Ashland Avenue in Virginia, Jim pulled out a bunch of insulation from the basement ceiling. He got a very bad lung infection that persisted for months, and he was coughing up blood. He got referred to a pulmonologist who did an exhaustive panel of tests, including an MRI of his chest. He determined that Jim's bronchi were spongy, instead of hard as is needed to expel mucous from the lungs. He also showed some abnormality of the liver. His diagnosis was COPD (Chronic Obstructive Pulmonary Disorder) which basically means "there's something wrong with your lungs but we don't know what." At that time his lung function was in the 60th percentile. (I think it was 64% FEV-1 but can't remember for sure.) Several more months passed and Jim's infection did not go away. He went back to the pulmonologist's office and his regular specialist was on vacation so he saw his partner. The partner walked into the room, looked at Jim's chart, and said, "So, howlong have you known you had Cystic Fibrosis?" Jim said, "I do?" ??!!!! The doctor thought that Jim already knew, based on all of the test results that he saw in the record. Apparently this doctor had seen a lot of children with CF as lung transplant patients in his previous practice. He sent Jim to the Children's Hospital in Norfolk for the Sweat Test and it was positive for Cystic Fibrosis. We had just started packing up our house as we had accepted an offer to sell it a few weeks earlier.


TREATMENT: God is very good. We were already in process of moving to Madison, Wisconsin, where there is an excellent CF Adult Treatment Center at the UW Hospital. There were no adult treatment centers in the state of Virginia! (Now there are 2 - one in Richmond and one in Charlottesville.) Soon after arriving in Wisconsin, Jim was seen by Dr. Dopico, the director of the Adult CF Center. Their treatment is comprehensive: their staff is caring and thorough, and they also provide a social worker and senior caseworker. He is seen by a series of people on a regular basis. They also refer him to specialists in nutrition, diabetes, fertility, and osteology (bones), when they feel it is necessary as these are all issues that people with CF will commonly deal with.

They ordered The Vest which we received in October 2003. The Vest is an appartatus that Jim straps on his body each morning and each night. The Vest™ system uses a technology called high-frequency chest wall oscillation (HFCWO). The system has an inflatable vest connected by tubes to a generator. During therapy, the vest inflates and deflates rapidly, applying gentle pressure to the chest wall. This works to loosen and thin mucus and to move it toward the larger airways, where it can be cleared by coughing or suctioning. Jim's doctor prescribes the amount of time and pressure that he should be doing on the Vest daily. (Usually in 3 increments of 10 minutes each - repeated in morning and at night.) Jim also does inhaled daily doses of Pulmozyme (a genetically designed medicine that breaks down the mucus in his lungs making it easier to expel.) Every other month he does nebulized treatments each morning and night of Tobi (an inhaled antibiotic to treat the lung infections directly.) We are very grateful for these medicines which help Jim so much. He is amazingly patient and disciplined in doing his treatments daily.

We also attend CF Connection, a monthly support group for adults affected by CF, which is organized by the CF Clinic's case manager and psychologist. Every other month, spouses come to the meetings. This is the first group of its kind in the country, and it has been wonderful to meet with other people who deal with the same types of issues that we do.

We had several meetings with a fertility specialist and discussed family planning with Dr. Dopico and the CF Psychologist, Damion. There are some fertility treatments for CF patients. At this time we feel that they are too uncertain and too expensive for us. We have decided to adopt a child instead. But we both feel that in a few years the price of some of these treatments will go down, and the pregnancy rates will go up so we are looking forward to perhaps experiencing pregnancy in the years to come.