Chase
McGowen's
Transplant and Medical Information Web Site
Chase's
Illness:
Introduction
In this section, we will attempt to very simply, briefly, and in layman's terms, describe Chase's illness. More information general to the conditions is available by selecting the buttons on the left.
Disclaimer: Apologies in advance to medical personnel. We have been dealing with
this for 13 years, but we don't pretend to be more knowledgeable than
we actually are. No doubt some mistakes may creep in here or there.
If you would like to make suggestions of things to change, please e-mail
us.
Chase McGowen
is 13 years old. He has been battling Cystic Fibrosis his entire life.
His normal life includes multiple breathing therapy sessions per day,
taking enzymes with everything he eats (to aid digestion), being attached
to a night-time feeding tube, taking multiple medications and being tethered
to an oxygen generator when at "home" and using tanks of oxygen
when away from home. Chase is no stranger to hospitals. He has been in
and out of hospitals 2 to3 times each year for "tune-ups" where
they beef him up and maximize his breathing capabilities. Within the past
year, Chase's health has deteriorated. He has been in and out of Brackenridge
Hospital in Austin over 10 times for collapsed lungs. Each of these hospital
stays has been critical. Chase even stopped breathing once in ICU but
was resuscitated. The doctors classified Chase as having chronic lung
failure and recommended that he get on a transplant list.
The decision was made to move Chase to Houston and put him on the Lung transplant list at the Children's Medical Center. After evaluation by the transplant team, it was determined that Chase also needs a liver.
About Cystic Fibrosis
The #1 genetic killer of children in the United States
Quick Facts from the Cystic Fibrosis Foundation
- Cystic fibrosis is a genetic disease affecting approximately 30,000 children and adults in the United States.
- CF causes the body to produce an abnormally thick, sticky mucus, due to the faulty transport of sodium and chloride (salt) within cells lining organs such as the lungs and pancreas, to their outer surfaces. The thick CF mucus also obstructs the pancreas, preventing enzymes from reaching the intestines to help break down and digest food. Specialized treatments are available to address these problems at CF Foundation-supported care centers across the country.
- CF has a variety of symptoms. The most common are: very salty-tasting skin; persistent coughing, wheezing or pneumonia; high calorie need, poor appetite and poor weight gain; and bulky stools. The sweat test is the standard diagnostic test for cystic fibrosis. This simple and painless test measures the amount of salt in the sweat. A high salt level indicates that a person has CF.
- The treatment of CF depends upon the stage of the disease and which organs are involved. One means of treatment, chest physical therapy, requires vigorous percussion (by using cupped hands) on the back and chest to dislodge the thick mucus from the lungs. Antibiotics are also used to treat lung infections and are administered intravenously, via pills, and/or medicated vapors which are inhaled to open up clogged airways. When CF affects the digestive system, the body does not absorb enough nutrients. Therefore, people with CF may need to eat an enriched diet and take both replacement vitamins and enzymes.
- One in 31 Americans (one in 28 Caucasians) - more than 10 million people - is an unknowing, symptom-less carrier of the defective gene.
- An individual must inherit a defective copy of the CF gene ﾑ one from each parent ﾑ to have cystic fibrosis. Each time two carriers conceive a child, there is a 25 percent chance that the child will have CF; a 50 percent chance that the child will be a carrier; and a 25 percent chance that the child will be a non-carrier.
How our Lungs Work
From the American Lung Association
In mechanical terms, our lungs can be described as the site of gas exchange:
Oxygen--the fuel all the cells and organs of our body need to function--is extracted there from the air we inhale and infused into the bloodstream, to be distributed to other organs and tissues. With each exhalation, we dispose of the carbon dioxide that is the by-product of our bodily processes. In our lungs, in the course of a single day, an astonishing 8,000 to 9,000 liters of breathed-in air meet 8,000 to 10,000 liters of blood pumped in by the heart through the pulmonary artery. The lungs relieve the blood of its burden of waste and return a refreshed, oxygen-rich stream of blood to the heart through the pulmonary vein.
The lungs are internal organs. Yet they are, uniquely, constantly exposed to our external environment--a direct interface with the world outside. With each breath, a host of alien substances enter our bodies--pollens, dust, viruses, bacteria; the constituents of the air in our homes and offices and factories, ranging from animal dander and tobacco smoke to radon and airborne lead; the toxic chemicals spewed into our atmosphere by smokestacks and tailpipes.
The lungs, with their tiny air sacs called alveoli, have sometimes been simplistically compared to sponges. They are actually far more complex than many other organs. The heart, for example, is a relatively uncomplicated muscular pump designed, with one-way mechanical valves, for one purpose: to keep the bloodstream flowing in one direction. The lungs must play multiple roles--supplier of oxygen, remover of wastes and toxins, defender against hostile intruders. They contain at least three dozen distinct types of cells, each with its special tasks and abilities. Some scavenge foreign matter. Others, equipped with delicate, hair like cilia, sweep the mucous membranes lining the smallest air passages. Still others act on substances crucial to blood-pressure control, or serve as sentries to spot invading agents of infection. And the roles of many others remain mysteries, posing challenges to researchers.
What CF Does To the Lungs
CF causes the body to produce an abnormally thick, sticky mucus, due to the faulty transport of sodium and chloride (salt) within cells lining organs such as the lungs. The thick CF mucus obstructs the airways and provides an environment where opportunistic bacteria thrives. Chronic infections set in, and the lungs eventually loose their affectivity.
Lung Transplantation
Facts About Lung Transplantation (from the Cystic Fibrosis Foundation)
When is it time for a lung transplant? What is evaluated? Is the evaluation process "standard" for all centers, or does each transplant center have individual requirements?
A primary care physician may refer an individual with CF for evaluation at a lung transplant center when the individual develops lung complications that may lead to death in two to three years. The evaluation involves measurement of lung, heart, and renal functions, infectious disease studies, and psychological examination. Most components of the evaluation are standard, but each center has some particular requirements. Acceptance as a transplant candidate is specific to each center. The patient may change his/her chosen center, although some new evaluation is usually necessary.
Once a CF patient has received a lung transplant, does CF "go away?"
The transplanted lungs came from individuals who do not have CF. These "new" lungs are initially disease free, however, CF does remain in the sinuses, pancreas, intestines, sweat glands, and reproductive tract after the lung transplant.
What does the body go through after transplant?
Each individual has an immune system that protects against foreign material, including microbes. Transplanted organs are foreign to the recipient and the immune system reacts against them in a process called "rejection." Immuno-suppressive drugs are given daily for the life of each transplant recipient to reduce the immune response and protect the transplanted organs from rejection. Immuno-suppressive drugs may increase one's susceptibility to some infections, and cause side effects such as diabetes, decreased kidney function, and osteoporosis (thinning of the bones). The doses of such drugs are adjusted to maintain adequate immuno-suppression and minimize these side effects.
What is the difference between a living donor lung transplant and a cadaveric lung transplant? Are there differences in success rates and recovery times?
A cadaveric double lung transplant for cystic fibrosis entails removing both lungs from a brain-dead organ donor and surgically implanting them, one at a time, into the recipient. The success rate is measured as the percentage of transplant recipients alive at certain intervals after the operation. For transplant recipients with CF, the rates are as good as those following lung transplantation for other diseases. The time to discharge from the hospital ranges from 16 days to months, depending on the complications.
A much less common technique is the living donor transplant. This entails surgically removing a right lower lobe from one donor and a left lower lobe from another donor, and the sequential implantation of them into the recipient.
This procedure is more complicated because three individuals have major surgery. Each donor must be significantly larger than the recipient so that the two lower lobes are big enough to fill the recipient's chest cavity. Also, the surgical connections of the airways and blood vessels are more difficult. The success rate is about as good for living donor transplants as for cadaveric transplants, and the complications are equivalent. Donors must be healthy enough to tolerate the operation. They will have a modest decrease in their maximal lung function, but this should not limit normal activities.
Can a CF patient be on more than one transplant list?
The lung transplant community has agreed that a candidate can only be on one transplant list at a time to distribute the likelihood of matching with acceptable donor organs fairly.
Once a patient is listed, how is priority determined?
Donor lungs are allocated on the basis of blood type, size, and accrued time on the transplant waiting list. Seriousness of condition is not currently a criterion; however, plans are underway at the federal level to change this policy.
Once a patient is listed, do they have to be "on call"? Are they required to live within a certain distance from the center, or required to establish residency in the center's state?
The residency requirements are different at each transplant center, although all centers require patients to be able to arrive at the center within a certain time limit (usually a few hours). Transplant candidates begin "call" status when they are close to the top of a waiting list. This may be several months to two years after initial listing. Legal residency in the state of the transplant center is not always required, although some centers require living within a short distance of the hospital once "on call" status is determined.
Links To More Information
National Transplant Assistance Fund [NTAF]
United Network for Organ Sharing [UNOS]
Texas Children's Hospital in Houston
Health and Human Services Organ Donation Page