January/February 2010 Issue

Cystic Fibrosis Social Work — Support on a Changing Journey
By Nadine M. Hasenecz
Social Work Today
Vol. 10 No. 1 P. 22

Living with cystic fibrosis is a challenging journey no one should take without the support, education, and teamwork social workers can provide.

Social workers who serve patients with cystic fibrosis (CF) on hospital interdisciplinary teams help them deal with myriad psychosocial issues. Because CF is an inherited chronic disease with no cure, it remains with a person from birth until death, and a social worker encounters issues that change as a patient ages.

Because CF is characterized by thick, sticky mucus that clogs the lungs and causes shortness of breath; a persistent, phlegmy cough; and lung infections, it is often thought of as a lung disease. However, the mucus also affects the digestive system, preventing digestive enzymes from properly breaking down food and the body from absorbing nutrients. The result can be a malnourished, underdeveloped individual who suffers with troublesome bowel movements, including greasy, bulky stools.

Approximately 30,000 individuals in the United States and 70,000 worldwide have CF, according to the Cystic Fibrosis Foundation, a nonprofit organization that accredits more than 115 U.S. CF care centers, 95 adult care programs, and 50 affiliates.

Living With Cystic Fibrosis
Perhaps the biggest challenge for a CF social worker is ensuring a patient’s adherence to the medical regimen, which consumes several hours of patient and caregiver time each day. Although it varies by patient, a typical medical regimen for treating CF consists of taking at least one antibiotic, such as Zithromax or TOBI, in a pill, nebulized, or inhaled form; taking at least one medication to thin mucus, administered via a nebulizer or an inhaler; taking enzymes with all meals and snacks to aid digestion; engaging in aerobic exercise for 30 minutes per day at least three times weekly to build strength and appetite and help with airway clearance; taking vitamins A, D, E, and K; and utilizing at least one airway clearance technique for at least 20 minutes twice daily or more to loosen mucus and move it out of the chest.

One airway clearance technique involves a caregiver using his or her hands to pound or “clap” on a patient’s chest and back. (In the case of a child from birth to approximately the age of 2, the caregiver may use a handheld rubber device to “bop” the chest and back.) The most popular method of airway clearance is having a patient wear a mechanical vest that vibrates the chest and back. Patients may also blow into products such as acapella or FLUTTER, both handheld devices that create vibrations that help clear breathing passages and are small enough to be taken to school, work, and on overnight trips and vacations.

While patients may be advised to clear their airways twice daily during healthy times, increasing treatment to three to four times daily is advised whenever patients become ill or experience increased coughing or sputum production.

In some cases, an individual with CF may choose to remain at home rather than be hospitalized. Some must be fitted with a gastronomy feeding tube to battle malnutrition or may be required to take intravenous antibiotics to combat exceptionally resistant bacteria.

In any case, patients and caregivers can often struggle to find the time and energy to accomplish all that is prescribed as part of the medical regimen while balancing it with the hectic demands of everyday life. Marcy Odell, LMSW, a social worker in the pediatric pulmonary medicine department at Golisano Children’s Hospital at Strong at the University of Rochester Medical Center, recalls a single mother of two who cried about her daily struggle to handle the recommendations for her 13-year-old daughter with CF. The child’s after-school activities and the mother’s work schedule left little time to prepare and eat dinner and perform airway clearance. While it’s generally believed that a CF patient can accomplish certain sedentary tasks such as doing homework while wearing the mechanical vest, Odell says this particular child and several other individuals have attested to its difficulty. Odell arranged to have dinner delivered occasionally to ease the family’s evening routine.

Many patients wake up at 4:30 am or 5 am to do their treatments, take care of hygiene, and eat breakfast before leaving for work or school, according to Bill Taub, MSW, LCSW, of the pediatrics department at Duke University Medical Center in Durham, NC.

Children then “come home from school at 4 o’clock, and they’ve been up since 5 am. The first thing they have to do is another set of treatments, have dinner, then homework, and come around 8 pm, they’re starting to get tired. But they don’t want to go to school without their homework,” he says.

Thus, social workers are often called on to meet with teachers, principals, and school counselors to discuss reducing a student’s homework assignments. Taub recalls a case in which a middle school student was granted permission to complete only every other math problem. He notes that getting the student—let alone school officials—to agree to such an arrangement can be challenging because children don’t want to be perceived as different than their peers.

But homework is only one issue social workers face when dealing with a patient’s school experience. If a student is hospitalized, for example, a social worker must ensure that he or she is not penalized for missed work and allowed adequate time to make it up. Or school policy may dictate that only a nurse can administer medication and, therefore, the child must make regular trips to the nurse’s office.

Something as simple as taking enzymes during lunch may cause angst. “If they can find a way to pocket them [the enzymes], they’ll do it,” says Mary Jo Chambers, LCSW, MSW, a supervisor in the social work department at Arkansas Children’s Hospital CF care center, a sister institution to the University of Arkansas Medical Sciences.

Taub adds, “Other teens will say, “‘What’s that you’re taking? Does it make you high? Will you sell it to me?’”

However, even more embarrassing than taking enzymes in front of one’s classmates is the immediate abdominal cramping, flatulence, and diarrhea that results from skipping them, notes Chambers.

“Some patients try to go a whole day at school without using the bathroom,” Taub says, “because they fear they’ll be teased if they leave the bathroom stinky or take too long.”
In addition to liberal restroom privileges, a student should be permitted to keep drinking water at his or her desk, Chambers says, because the secretions caused by CF can cause excessive thirst.

Preventing Isolation
Due to the possibility of patients with CF transmitting bacteria to each other, students with CF in the same school should be separated into different lunch periods and classes, according to Chambers. When separate rooms are not an option, the students with CF must remain on opposite sides of the room. Chambers teaches her patients and their families what she calls the “3-ft rule,” meaning that people with CF should always remain at least 3 ft away from each other.

In fact, CF is an isolating illness that requires patients to avoid the very people who share their experiences and understand their struggles. Camps created exclusively for kids with CF are relics of the past.

“We feel really badly about the fact that we no longer do support groups and no longer have patients in the hospital getting together, having lunch, and going into each other’s rooms and having conversations,” says Taub. “But we know we’re doing the right thing diminishing transmitting organisms.”

Instead, socialization among patients with CF should be confined to the telephone, postal mail, e-mail, online chat rooms, and Web sites. Odell says she refers patients to online social networks and is in the process of organizing such a network through her own hospital’s Web site.

Tracey Hartmann, MSW, LCSW, a pediatric medical social worker in the clinical social work department at St. Louis Children’s Hospital, which is in research collaboration with Washington University School of Medicine, is involved in similar activities. “Adolescent patients are interested in meeting and knowing others who have CF,” she says. “Our team makes every effort to connect patients to other teens via e-mail or Web sites for patients with CF. We are getting a Facebook page started and are notifying patients of this resource.”

Although the Internet can be positive in facilitating communication among individuals with CF, Taub urges his patients to approach it with care not only because of the possibility of encountering false information about CF but also because of the possibility of being on the receiving end of communications from a patient with CF who is angry or in denial about the illness.

“We want to talk to them [patients] about what they’re hearing to make sure they’re receiving any information over the Internet with a good, open mind,” he says.

Other Issues
Body image can be an issue for patients with CF who, due to nutrient malabsorption, can be physically underdeveloped compared with their peers. Individuals with CF wonder whether others will find them attractive or whether their coughing will turn people off since it often produces mucus that must either be spit out or swallowed. And just as with exercise, sex can cause coughing. Furthermore, an adult or young adult with CF may worry that a potential long-term partner will refuse to become involved out of fear that the partner with CF will die prematurely.

When they reach school age, children begin to question when they will die from CF, according to Hartmann. Taub says he’ll discuss death with a child as young as 5 years old unless the parents dictate otherwise. “If a child is young enough, you have to respect parents’ decision not to [discuss death],” Taub says. “But I will argue if a kid is approaching the teens. I’ve lost an argument or two over the years. I’ll say, ‘Your son knows what’s happening, and he’s trying to protect you.’”

Due to medical advances, patients with CF “can now expect to live into their 30s, 40s, and beyond,” according to the Cystic Fibrosis Foundation’s Web site. This forecast contrasts that of the 1950s, when “few children with cystic fibrosis lived to attend elementary school,” the organization’s site indicates.

Sex and family planning must be seriously considered. Both parents must be carriers for a child to develop CF, but an individual can be a symptomless, unknowing carrier.

Although vaginal mucus makes it difficult for sperm to reach an egg in a woman with CF, pregnancy is possible. Therefore, a social worker may ask a female patient and her partner to contemplate the manner in which a pregnancy and delivery may affect her health and how the lack of energy to raise a child may strain the marriage.

While a man with CF produces sperm and experiences an erection and ejaculation, most are infertile because the vas deferens, by which the sperm enters the semen, is at least partially missing, causing the sperm to be obstructed. However, men with CF can have their own biological children via an expensive procedure in which sperm are surgically removed and implanted into the partner.

In addition, social workers must broach genetic counseling with a CF patient’s parents. “If both parents are carriers, with every pregnancy there’s a one-in-four chance of the child having CF. That doesn’t mean if you have four kids that only one has CF. It is a one-in-four chance with every pregnancy,” says Chambers, who knows of a family of four siblings, three of whom have CF.

She says she helps parents think about what their lives would be like with more than one child with CF. There’s not only the additional time spent administering treatment but also the possibility of leaving a sick child alone in the hospital because another is at home.
In fact, leaving a child alone in the hospital can be an issue even when a sibling doesn’t have CF. Parents need to consider several issues, such as the fact that even a healthy sibling may be too young to stay home alone and that at least one parent risks losing employment because of absences due to hospital visits.

Just as parents of patients with CF worry about losing their jobs due to work absences, so do CF patients themselves. In fact, some wonder whether it’s worth it to pursue a career or even college. In early adulthood, a CF patient may consider higher education but worry about the inability to follow through if he or she grows increasingly ill.

Keeping CF patients motivated and forward thinking is a large part of a social worker’s job. “I try to mail out all the scholarship information that I know about to patients that are in college or planning to go to college,” says Amy Purdy Plachta, LMSW, of the Cystic Fibrosis Center at St. Vincent Catholic Medical Center in New York City. “We discuss what accommodations might need to be made when one of our patients goes to college.”

Even when an individual wants to continue working, it may become too difficult, and he or she may be left with no choice but to consider disability and all of the questions that it introduces, such as what life will be like without a job. “Every CF patient who is healthy enough to have a career reaches that point,” says Taub. “Whether at 27 or 47, they’re sitting in their living room wondering, ‘How long do I keep working and when do I go on disability?’”

Most, if not all, CF patients consider lung transplantation at some point in their lives, says Taub, because it’s the only way to survive. In addition to the usual risks of surgery, the possibility of organ rejection, and the expense, a patient must be made aware of the possibility that the desired result may not be achieved and that he or she may continue to experience a sickly and difficult life.

“People need to go into the process with their eyes open,” Taub says. “The last thing I want is a person six months later, dying, saying, ‘Why didn’t you tell me about the possibility [of problems occurring]?’ That would be unconscionable to me.”

— Nadine M. Hasenecz is a freelance writer who lives in Bethlehem, PA.