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It had been the coarse sheets, blue, and tightly
tucked under me
It had been the steady beep of machinery lulling
and tranquil that carried me off to sleep
It had been the red tubes running into my arms
and allowing someone else's blood to flow into me
It had been the packaged hot chocolate
my mother and I could not consume enough ofIt had been the summer of 2003, the day after my graduation
The day after my final steps as a Jr. Higher
The day after my warm goodbye bid to my
naive self and decided to become the mature
person I looked up to
The day after my mother's smile blossomed fullyThe day after my graduation, it was somewhat
amazing how swiftly everything happened
Somewhat amazing how fast one dizzy night
could swim in and out of your head
Somewhat amazing how quick I could be
carted off in family's care leaving the dessert
plates spinning where we left them
Somewhat amazing how rapid a mother's smile could turnSomewhat amazing how long nine days could
seem in the hospital
In the hospital with the nice nurses and hot doctors
In the hospital with friends stopping by and
family always around
In the hospital with A Fish Called Wanda, with
The Gods Must be Crazy one and two, with
White Oleander, and a comedy we couldn't finish
In the hospital with my mother as a devoted
room mate and we talked about everythingIn the hospital it had been the coarse sheets,
blue, and tightly tucked under me
It had been the steady beep of machinery lulling
and tranquil that carried me off to sleep
It had been the red tubes running into my arms
and allowing someone else's blood to flow into me
It had been the packaged hot chocolate my
mother and I could not consume enough ofIt had been the worst and best week and a half
of my life and I can pull from it
a great many things...
I remember my childhood nightly ritual. I knelt down besides my bed, bowed my head and clasped my hands together. The Children's Bible we asked Mama and Dad for Christmas lay open on the bed, and I had just finished reading the story of the locust swarms. "Please dear, God, please let there be a cure for CF." I tightened my hands together and squinted extra hard when I said please, as if the harder I emphasized the word the more God would listen to me. "Please, I can handle anything else that happens, but please, please let me live a long, long time. Please find a cure for CF. Thank you for everything else that I have and all the love in my family. Amen." I finished my prayer as quickly as possible, just in case Ryuta barged in, because praying pissed him off and he usually hit Ana or I if he caught us in the act.
In 1988, the gene for Cystic Fibrosis was discovered by Francis Collins and Lap Tsu Chui on the seventh chromosome of the human genome. I remember our friends Akemi and Naomi running upstairs when they came over one day with a newspaper clipping announcing the discovering. "Look, Isa! Look, Ana! There's going to be a cure for your disease!!" Akemi cried, and we celebrated all together. The article said that finding the gene was a major breakthrough and that a cure could be expected within the next ten years. So, it was all right. All those prayers, the wishes I made every time I blew out the candles on my birthday cake (double wishes because I know Ana made the same one), the wishes I made when I spotted the first star at night, when I broke a wishbone, when I saw a shooting star, or when I dropped a shiny penny into a well; all I ever wished for was a cure for my disease, so I could live happily ever after. It was a secret wish shared by all members of the CF club, one that remained unspoken for fear that it would not come true if revealed. Year after year of passing wishes and finally there was a glimmer of hope that the light was at the end of the tunnel. But the light was faint, because behind our jubilation with our friends was a hidden terror of wondering whether Ana or I would still be alive in ten years to reap the benefits of the cure. At the time, it seemed highly unlikely.
Years passed, and in the nineties and the start of the millennium, the kids and then young adults at CF support groups and camps shared their musings about the so-called 'cure'. With their striking maturity forced onto them by circumstance, they possessed a well- balanced assessment of candid realism and hopefulness.
"I know I need to keep myself healthy until the cure comes."
"That's a crock. The science is so much more complicated than they thought; it'll be years before they find any cure."
"There's not going to be any real cure. There's be a control for CF, like with diabetes, but I doubt there'll be a magic pill that will cure it."
"You see, I'm confident. I think the cure is right around the corner."
"I am way too sick for a cure. I just don't think I'll be around then."
"What the hell is taking so long? I mean, there is so much fundraising and still no cure."
"Yeah, the idea of a cure is just to give parents of young kids hope. We're too old to benefit from it, because we adults have way too much lung damage to fix our lungs."
"My version of a cure is to get a lung transplant. Maybe those will be more successful some day."
And with each passing year, there were more complex articles in the CF newsletters about the gene and protein and sophisticated but yet successful techniques for gene therapy, but most basic science research remained obscure and unattainable and largely clinically irrelevant. A decade after the gene discovery, the impatient parents who attended the CF conferences would protest and cast about bitter accusations to the researchers who always ended their presentations with "… and further research needs to be done…" But in the 1990's new drugs were in fact developed; a mucolytic called Pulmozyme, a concentrated form of inhaled tobramycin (an anti-pseudomonas drug), improved digestive enzymes to increase nutritional health, and second and third and fourth generation antibiotics that helped to treat the pseudomonas lung infections that were the primary cause of death in people with cystic fibrosis. So with each new treatment, came more minutes and hours of nebulizer treatments, more pills to swallow, more intravenous antibiotics to infuse, more time and energy to treat the disease that was supposed to shorten our lives. There were better treatments but still no cure.
So the wishes continued with each passing birthday cake, until one day, I suddenly woke up, around my 25th birthday, when I realized the number of candles could barely fit on my cake, and it hit me; I was living the cure. It wasn't something sitting on the horizon, way off into the distance. It wasn't something that I was in an eternal state of reaching for, desperately trying to grab. The cure was happening right now. I was riding the wave of technology that was prolonging the lives of people living with cystic fibrosis. There was the CF generation behind me whose waves had crashed, leaving them drowning in the rip tide; there was the CF generation ahead of me, whose waves were taller so perhaps they could see the distant cure on the sandy beaches ahead. But for me and my generation of comrades with CF, there was no magic bullet cure, but the timing was right, and my prayers for a long, long life were still being answered.
Here's Angel Mammino's victory song: "Pomp and Circumstance" bleated out on plastic purple kazoos.
Unusual? Yes. But Mammino, 18, has had an unusual path to graduation. Diagnosed with cystic fibrosis at age 2, the teenager has divided her education between public schools in Hollister, where she lives, and a little-known school on the third floor of Lucile Salter Packard Children's Hospital at Stanford -- her "second home."
On Thursday, she graduated in an intimate ceremony on the hospital's third floor. She was the only graduate of the class of 2002, and well-wishers included family and friends, patients and hospital employees.
"Everybody who knows me is here, if they can make it," Mammino said, her tongue ring glinting as she spoke.
She said her family was happy to see that she had made it this far. Her cystic fibrosis-afflicted poker buddies -- their ante was bubble gum cigars -- have all died. Her best friend in the hospital, Adam Oneto, died last year.
"I lost a lot of people in my life," said Mammino, who created a bulletin board for patients to post notes and photos after Adam died and confidentiality rules kept many patients from learning about his memorial.
Angel plans to attend Gavilan College in Gilroy and be a disc jockey.
"I'm so proud of her. She worked so hard to get here," said Melissa Guarino, her best friend from Hollister.
The hospital school, which is affiliated with the Palo Alto Unified School District, has existed in some form since 1924. Cammy Sunde, the school's head teacher, estimates that two-thirds of the hospital's school-age children have attended the school, some on a long-term basis and others just during a short stay. Mammino has attended the hospital school since the first grade.
The school has graduated seven students in its history, and four have taken equivalency exams. For a child with CF, high school is a big deal because many don't make it to college.
Cystic fibrosis (CF) is the number one genetic killer. Psychological adjustment through social support groups and education may directly affect compliance with medical treatments. The newly proposed Cystic Fibrosis Foundation (CFF) cross-infection guidelines were created to help prevent the spread of pathogens between CF patients. The aim of the guidelines, and reason for the great restrictions, is to keep the progression of CF among patients, minimal for as long as possible. These guidelines discourage all social contact between CF patients. They do not allow for CF patient-to-CF patient social support and as a result, may cause social isolation and possibly other detrimental psychological affects. Further examination of the effects of the cross-infection guidelines should be studied, to discover if the biological benefits outweigh the possible negative psychological implications.
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Cystic fibrosis (CF) is a genetic disease affecting approximately 30,000 Americans. CF is a recessive genetic disorder, and the number one genetic killer. CF affects the exocrine glands, causing the most prominent complications in the respiratory tract, and digestive system. Treatment of CF includes a regimen of pills, physiotherapy, a high calorie diet, and for some, routine IV antibiotics. Symptoms of CF generally are seen as chronic lung infections, difficulty digesting fatty foods, a large appetite combined with a failure to gain weight, and salty tasting skin. Approximately 900 babies are born with CF in the United States each year (Hopkins, 1998).
Thick mucus clogs the airways in an affected CF patient. Daily administration of physiotherapy as well as nebulized broncho-dialators and antibiotics are used to combat infection. Therapy can be administered anywhere from 1 to 4 times daily, depending on the severity and progression of the illness. The chronic lung infections also harbor bacteria; the most common is Pseudomonas aeruginosa. Most CF patients contract this bacterium some time in their lives, from the environment. More harmful and destructive bacteria, known as B. cepacia or multi-antibiotic resistant strains of bacteria can also be contracted from the environment as well as from other CF patients (Hopkins, 1998).
The thick mucus in the pancreas, affects the digestive system by preventing proper secretion of digestive enzymes. To treat this symptom, supplemental enzymes, along with many other vitamin supplements are prescribed. A high calorie diet is often recommended in order to consume the needed amount of nutrients. Many other pills are also prescribed to help in the inflammation of the lungs along with oral antibiotics and a variety of other supplements.
There is no cure for this disease. However, treatments are progressing and research is currently being conducted in hopes of a potential cure. The possibilities are very hopeful. The life expectancy has increased dramatically in the past two decades. Average life expectancy is 30-32 years. Thirty seven percent of CF patients are over 18 years old (Saiman, Siegel, et al., 2002). CF is no longer only a pediatric disease. As the life expectancy increases, advances are made to help patients maintain good health, for longer periods of time.
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Growing up with a chronic disease presents the individual with many different complications in their life. The average CF patient does not go through the typical adjustment phases that occur in other chronic illnesses with late onset. Quality of life is a priority in CF patients. The life of a CF patient is dedicated to surviving and controlling CF. They are continuously bombarded by treatment regimens, pills, and frequent doctor visits for their entire lives. Living and coping with the disease is their life, and they must find ways to maintain normalcy in their day-to-day life, despite the treatment regimens.
Many CF patients do not experience denial as a part of their adjustment to their chronic illness. This may be because many people are diagnosed at a young age, and life as they know it, revolves around living with Cystic fibrosis. These patients may however, experience anxiety. Cystic fibrosis is under constant supervision, continually trying to maintain a baseline, with the goal of keeping progression of the disease to a minimum. The overwhelm experienced by the medical treatments and tests can provide a large amount of anxiety upon a patient with CF. The medical procedures, tests and even side effects of medications can be a large component in they anxiety they may experience. Depression may also be a factor in the psychological development of a CF patient. During times of continuous illness, and downward progressions, hope may be lost. This is a critical time when depression may potentially set in. Awareness of the psychological well-being is crucial in the adjustment of a CF patient.
Knowledge of the illness is necessary in healthy adjustment with Cystic fibrosis. Knowing the details of the disease, and what it entails for the future is essential in creating realistic goals and attitudes towards life. It helps to prepare for and understand what is happening to the body, as well as what they need to do to control the disease. Causation of CF is an important fact to understand. Realization that the disease is genetic can help to take the blame off the patient, and also facilitate an understanding that no one is at fault. Finally, beliefs about control of the disease will help to encourage self-efficacy. The treatment the patient is using, along with the knowledge of how if effects the disease helps to determine self worth, and putting in the most effort possible to control the disease. Strong self-efficacy helps the patient continue more treatments and as a result, possibly live longer.
The knowledge of a shortened life span can potentially cause psychological harm, if not dealt with in a rational manner. The acceptance of life as it is given is essential to be a well-adjusted individual for the duration of their life. The acceptance of death does not mean loss of hope; it can function as the opposite by creating hope and motivation for the time the patient is alive.
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As a result of CF being such a time consuming disorder, there is potential for interference in the social life of patients. This can be especially prominent in the event of severe illness as well as very high compliance, "an extreme high level of involvement that entails rigid compliance with the child's medical regimen may be regarded as a substitute for positive marital and social interactions. Conversely, maintaining what physicians consider to be high levels of compliance with the CF treatment regimen may interfere with a patient's ability to maintain satisfactory marital and social relations. Given the complexity and time-consuming nature of CF treatment, this later explanation seems quite detrimental" (Geiss & Hobbs, 1992, p.102). Children should also be kept in a "normal" environment as much as possible. Encouraging the child to develop social support, regular school attendance and physical activities as much as possible (Taylor, 1999), can help to foster healthy psychological adjustment with CF.
Cystic fibrosis impacts all aspects of the patient's life, and quality of life may be compromised. Compliance with medical treatments is necessary to survive and maintain good health. CF is a continuous battle of treatments and procedures and keeping up hope and optimism. Despite the treatments, coughing, and illness they must also work to maintain a sense of normalcy in their lives by keeping up with the "real world". Attention must be drawn to the emotional side of Cystic fibrosis. The possibility of early death, and the stress, anxiety and possible depression must be expressed to maintain psychological health. The most important aspect of psychological health in CF patients, as well as in other chronic illnesses, is the need for connections with others who are living with CF and having the same experiences. Education as well as knowledge and understanding can come from interactions with other CF patients. However, as medicine has advanced barriers have been presented to make the social interaction between CF patients virtually impossible, and highly discouraged. This concept has emerged as a result of research findings; certain strains of bacterium are contagious and can be contracted through patient-to-patient contact. The Cystic Fibrosis Foundation (CFF) has proposed new guidelines for cross-infection, which completely discourages social contact of any kind with other CF patients. These restrictions may cause problems in many areas such as, isolation, fear and a sense of loneliness. Personal connections with other CF patients are no longer possible and a lack of support, education, and psychological well-being may be compromised as a result of the new CFF guidelines.
Research on the benefits of social support among chronic illnesses has not been documented in mass amount. There is little evidence to describe the significance of group therapy and social support groups in Cystic fibrosis simply because of the lack of research. However, there has been documentation of the benefits of other chronic diseases such as AIDS and Cancer.
The research by Stewart and Gregory (1996), examined six content themes through the study of gay men with AIDS in long-term (5 year) support groups. The themes studied were: marginality, making choices, coping with the emotional roller coaster, premature confrontation of life issues, living with a chronic illness vs. dying with a terminal disease, and death and dying. Membership of the group continually changed. There was a mix of new members along with the old. The average attendance was 5.18 members at each session with membership averaging 9.56 over the five-year history (Stewart & Gregory, 1996). The confidentiality of the group was strongly enforced, at the time of this study people with AIDS were being fired from jobs, denied housing and insurance, and shunned from friends and families. Co facilitation was also strongly encouraged for these support groups. "Support group members fell within areas such as having a safe place to come and talk about living with AIDS, being heard and validated, being reassured that one is not along in the struggle with AIDS, receiving AIDS-related information, problem solving, participation in social activities with other group members, telephoning members during the week, and being visited when hospitalized" (Stewart & Gregory, 1996, p.289). The men shared their experiences through storytelling, which helped the members to get to know each other intimately. Empathy was a crucial source of support within the group settings as well.
When people feel that they matter, they no longer feel marginal. "In times of stressful transitions, individuals are in the process of redefining themselves. Part of one's personal identity is defined through relationships with others. Identity clarification often occurs through interacting with those who are similar to one's self. If one is sharing a similar stressful situation with another such as a chronic or terminal illness, it is more likely that a bond of trust and communication will form, creating an environment conducive to self-exploration and understanding" (Stewart & Gregory, 1996, p.291).
The support group members helped each other to let go of the "shoulds" in life, and helped to prioritize personal choices. The older members helped with their experiences of symptoms and medications to predict how it may affect the other members, helping them to anticipate appropriate therapeutic choices. Validation for their emotions and feelings helped to cope with the emotional roller coaster associated with AIDS. A greater emotional support is associated with less emotional distress. Members also were able to borrow successful coping strategies such as visualization, affirmations, and relaxation exercise to manage their emotions. The group also discussed ways to deal with daily life struggles and coping with all components of the disease. The men with HIV conceptualized their diagnosis by defining AIDS as a chronic, rather than terminal illness. Finally in their discussions about death and dying, the men expressed hope for a peaceful death. They were able to discuss their fears and concerns in these contained, confidential support groups (Stewart & Gregory, 1996).
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The research conducted by Classen, Butler, Koopman, Miller, et al., (2001), studied 125 women with metastatic breast cancer. They were divided into 64 women in the intervention condition, where they received one year of weekly supportive-expressive group therapy and educational materials. The other 61 women comprised the control condition; these women only received educational materials, no group support. During the first year the women were assessed every 4 months. It is estimated that 22% to 50% of all patients with breast cancer meet the criteria for psychiatric diagnosis of depression (Classen, et al., 2001). The aims of this research was to discover if supportive-expressive group therapy would decrease mood disturbance as well as if the supportive-expressive group therapy would also reduce trauma symptoms of intrusion and avoidance.
The primary analysis indicated that participants in the intervention condition showed significantly greater decline in traumatic stress symptoms on the Impact of Event Scale, compared with the control condition. There was no difference however, in the profile of mood status in the total mood disturbance. The secondary analysis showed a significantly greater decline in total mood disturbance and traumatic stress symptoms for the treatment condition compared with the control condition.
Through the sharing of experiences, group members in the intervention condition became role models for each other, teaching coping strategies they found effective in their own lives. A major purpose of the therapy sessions was to create a close-knit group that would serve to counter feelings of isolation and enhance social support. This expanded their social network, provided role models for coping with the illness, and enhanced self-esteem through their providing concrete help to others in a similar situation (Classen, et al., 2001).
From these two studies, evidence suggests that there are benefits of social support. This evidence is shown for two different chronic diseases, AIDS and Cancer. The evidence is not directly related to CF, but this is enough evidence to lead to the possible conclusion that social support and group therapy can be significant in the lives of people living with Cystic fibrosis.
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Education about the disease is important for any chronic or even acute disease. The more a patient knows, the more control they will feel they have, as well as an understanding of what is happening to their body as a result of the disease. Health care professionals through pamphlets, education classes, and dialogue can facilitate education. If more information is needed than the medical professional can supply, they should refer the patient to other sources to obtain the information they desire. It is important to be realistic in the education of a chronic illness.
Simply receiving the information from a written source makes it less believable and even overwhelming to the patient. Education from other individuals who have CF can make treatment options and medications seem like a reality, rather than an overwhelming burden. Hearing real feedback from someone who has gone through the same struggles and is experiencing the same life situation, such as living with CF is very important for the patient. "Introducing younger children to well-functioning older CF patients may be helpful as these older patients could serve as positive role models and positive life forces for the younger patients" (Gudas, Koocher, & Wypij, 1991, p.240-41).
Education, along with the link to social support helps to encourage compliance among CF patients. Speculation on this concept leads to the assumption that social support, in the form of role models, can lead the patient to feel more hope about their illness. Witnessing CF elders who have survived can deeply affect the CF patient and motivate them. Seeing the outcome of compliance is a strong motivator because no immediate outcome is seen in their everyday, mundane medical therapies. Education can be directly linked to compliance; the more the patient knows about the disease, its effects and treatments, the more options and control over the disease they will have. Compliance can also be influenced by optimism, as seen in the study by Gudas, Koocher, and Wypij, (1991), "Although the illness of children in our sample was perceived to be quite serious, the children's levels of optimism remained high, reflecting resilience and an overall hopeful attitude despite their debilitating, fatal illness. The high degree of optimism also suggests that this group of CF children had generally effective coping styles, were not globally depressed, and may well have been using adaptive denial as a defense against CF-related anxieties." Keeping optimism high as well as hope can be fostered and nurtured by the social support of others. Seeing their successful, and not so successful results, "Self help groups may help victims especially cope with the stigma associated with certain disorders, such as cancer or epilepsy, and such groups may help patients develop the motivation and techniques to adhere to complicated treatment regimens" (Taylor, 1999, p.359).
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With the knowledge of the importance of social support among CF patients, and the benefits it has for their lives, we enter into the ironic situation that has recently arisen. The new circumstances create a barrier for the essential psychological aspects needed to promote adjustment and well-being in the chronically ill patients.
CF patients can potentially be contagious to one another. The bacteria harbored in the lungs are the potential contagion. The most common infections are Pseudomonas aeruginosa and staphylococcus aureus. The latter bacteria do not prove to be highly contagious or resistant to antibiotics. These forms of infection are expected to develop in most, if not all CF patients at one time in their lives, and for the most part they can be kept under control (Hopkins, 1998). Burkholderia Cepacia (B. cepacia), as well as multi-antibiotic resistant strains of bacteria proves to be very harmful and contagious among CF patients. B. cepacia is the exception, not the rule in CF sputum cultures. Approximately 3.5% of all people with CF receiving care at a CF Foundation accredited care center were infected (Cystic Fibrosis Foundation, n.d.). It is because of these "bad bugs" that CF patients must be cautious, and take precautions not to cross-infect each other.
For the protection of all CF patients, the CF Foundation has recently created a new and very restrictive set of guidelines. "During the past two decades, cystic fibrosis (CF) patient-to-CF patient transmission of pathogens has been documented with increasing frequency. As a result, infection control policies have been developed within individual CF centers to prevent transmission among patients. However, policies vary from CF center to CF center. These policies can generate controversy and anxiety among members of the CF community including patients, their families, and their medical care team, particularly if care is received at different locations with different practices" (Saiman, et al., 2002, p.10). Because of the rarity of B. cepacia, these strict guidelines cause concern about their social implications and consequences on the CF community. "The purpose of this document is to provide a summary of the relevant data and evidence-based recommendations for infection control practices for CF patients to standardize care across CF centers" (Saiman, et al., 2002, p.11).
The suggestions for waiting area behaviors in clinics from Saiman, et al., (2002) are as follows, combined with hand washing procedures when you enter and leave the clinic,
Despite solid research to back up their information, the guidelines also contain directions to discontinue all CF-specific camps and overnight CF educational retreats. The suggestion instead is to substitute this void by having the CF patient participate in other camps and sports with non-CF individuals. This concept is like trading apples for oranges. The importance of CF camps and CF retreats are for the psychological benefits of support and education. The fact that everyone involved knows about or has CF is the essence, and reason of importance for camps or retreats. Trying to fill that void may actually make CF patients feel more isolated. They would be the only person at the non-CF camp or sporting event who has CF. This would be no different than everyday life. These substitutions do not qualify as a justification, or even a second option.
The psychological impact of these guidelines is going to eventually take away any source of group support that CF patients currently have been able to develop for themselves. "It is critical to acknowledge the psychosocial impact of infection control guidelines for CF patients, but at present, there is a paucity of studies in this area. Acceptance of recommendations to segregate CF patients from each other has led to a shift among CF patients in their friendships and support groups away from other CF patients to non-CF patients" (Saiman, et al., 2002, p.41). This acknowledgement of the psychological impact is expected, but by the means that it is so quickly disregarded is concerning.
The cross-infection guidelines affect every aspect of life: hospital behavior, school, home and family, social and educational. They will surely cause a sense of isolation among the patients who must abide by these strict rules. However, the physicians at the Danish CF center, "acknowledge the psychological consequences of isolation precautions, but stated that reducing the risk of chronic infection outweighed the negative impact of social isolation" (Saiman, et al., 2002, p.41). The standards used to determine the impact of social isolation verses the risk of chronic infection are not stated. A physician rather than a psychologist makes the decision. The main concern of a physician is to maintain physical health as best as possible. However, because the body and mind prove to function together, this does not obviously indicate that biological impact outweighs the negative effects of social isolation. The effects of social isolation listed by Saiman, et al., (2002) are separations from friends, loss of familiar hospital surroundings, guilt, depression regarding worsening prognosis, paranoia about acquiring B. cepacia and changes in social activities among patients with CF. These implications seem to stand as a strong warning sign. The physicians must be aware of the negative psychological impact this new criteria may have on the people of the CF community.
The physician should not only be concerned with the CF patient's longevity and keeping the patient healthy, but they should also show concern and compassion for the quality of life. The possibility of a CF patient-to-CF patient relationship may also benefit the individual equally, as maintaining strict cross-infection may on the biological health. The shared experiences of love, compassion and camaraderie can be just as healing as the medicine the patient takes. The power of this type of social support, group therapy, and camaraderie is difficult to measure. Medical data is easy to create and quantify. Love, compassion and the psychological benefits from others who share the same experiences are immeasurable. The psychological side of human beings, especially chronically ill patients, are often undermined by medicine. For patients who currently have shorter life spans, they need to focus on their quality of life. The idea of social support is not for everyone. However, the guidelines presented and the forbidden social interaction may possibly prove to have long-term detrimental effects for some. For many of these guidelines and regulations there is little solid evidence. Ironically, and against all odds, medicine must work to prolong life and increase longevity at any expense.
Future research should be conducted to follow the psychological implications of the cross-infection guidelines. With data to support the benefits of the restrictive cross-infection, the CF community may be able to more appropriately incorporate the new lifestyle into their lives. Until documentation is made, the implications are still unknown and will likely cause a disturbance amongst the CF community.
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Despite all these boundaries and regulations, technology has allowed for a moderate amount of continued "appropriate" contact between CF patients. There is the option of joining a chat board, where individuals with CF or people directly involved with the community can post information, daily comments, keep in touch with friends, make new friends and reach out to others in the community. Another option is the possibility of educational outreaching by videotapes, and even video-conferencing. There are now a few options and hopefully more will develop as time goes on. There is no person who can deny that computers and technology cannot fill the void of the human connections and relationships people form from one on one contact.
People are meant to be together. As a species, humans are social beings. Taking the appropriate precautions cannot guarantee the absence of cross-infection between CF patients, but it can limit the chances. The guidelines were created to protect the CF community but could potentially harm them in the end. The question is if the chance of catching a rare form of bacteria when all precautions are taken, is really worth cutting off all social connections with the real, breathing, thinking, feeling, loving, CF community. It may be the answer for some but others cannot deny the need for connection and the power of the human spirit.
References
Classen, C., Butler, L.D., Koopman, C., Miller, E., DiMiceli, S., Giese-Davis, J., et al. (2001). Supportive-expressive group therapy and distress in patients with
metastatic breast cancer. Archive of General Psychiatry, 58, 494-500.
Cystic Fibrosis Foundation, (n.d.). FAQ about burkholderia cepacia and the cf
foundation's participation policy. Retrieved March 5, 2002, from
http://www.cff.org/bcepaciafaq.htm
Geiss, S.K., Hobbs, S.A., Hammersley-Maercklein, G., Kramer, J.C., & Henley, M. (1992). Psychosocial factors related to perceived compliance with cystic fibrosis
treatment. Journal of Clinical Psychology, 48, 99-103.
Gudas, L.J., Koocher, G.P., & Wypij, D. (1991). Perceptions of medical compliance in children and adolescents with cystic fibrosis. Developmental and Behavioral Pediatrics, 12, 236-242.
Hopkins, K. (1998). Understanding cystic fibrosis. Jackson, Ms: University Press.
Saiman, L., Siegel, J., & the Cystic Fibrosis Foundation Consensus Conference on
Infection Control Participants. (2002, January 10). Infection control
recommendations for patients with cystic fibrosis: Microbiology, important
pathogens, and infection control practices to prevent patient-to-patient
transmission. Unpublished manuscript.
Stewart, G.M., & Gregory, B.C. (1996). Themes of a long-term AIDS support group for gay men. The Counseling Psychologist, 24, 285-303.
Taylor, S.E. (1999). Health psychology. Fairfield, PA: McGraw-Hill.
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I Can Get By by Lauren M. Catron
I can get by
I can get by
I can get by
I can get by
I can get by
I can get by |
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I can't get by
with the thought of the many cooped in the hospital watching their lives go by without them
or the thousands that have died still hoping for a cure.
This is why I pray for a cure every day
and thank God for research studies......
for lives that can't get by.
As a newborn, our third child, Brian, had an unusually soft cry. Friends and family laughed when I seemed concerned. And in truth, it didn't seem to be a big deal. Nearly two months later, though, I heard a faint cough coming from Brian's small frame. Barely audible, it was noticed only by me. A cough at this age just didn't seem right for a breastfed baby who stayed at home.
Brian's cough steadily grew worse. Soon anyone within earshot would say, “ What a cough!” But he had no other symptoms, not even a fever. Stronger and stronger medications did not help. His cough worsened. His wheezing increased. Soon Brian was gasping for air and we were rushed to the hospital in an ambulance.
This experience was completely foreign to me. My only hospital stays were to give birth. All three times we had opted for natural deliveries and chose to go home as soon as possible. Now I was strapped to a stretcher holding my baby as the emergency medical crew rushed around us. We all wondered how a child could be so sick and not even have a fever.
Upon admittance to the hospital, I soon learned to identify the different nurses, physicians, residents, medical students, and respiratory therapists who came to care for Brian. They each offered a different opinion, but their comments consistently began with the exclamation, “What a cough!” and concluded with the statement, “He's too happy to be so sick.” All I could do was nod my head and wonder why no one could figure out what was wrong with my bright-eyed little boy.
We followed a course of treatments for pneumonia and a collapsed lung. Brian was given potent antibiotics and medicated breathing treatments followed by chest physiotherapy. Still, his cough remained.
Meanwhile, the weeklong hospital stay was tearing my family apart. Our two-year-old daughter cried for me to tuck her into bed at night; all her little animals would say, “I miss my mommy.” Brian had so many tubes coming out of him that our four-year-old was frightened of his tiny brother. My husband was overburdened; he was tending to the older kids, running back and forth to the hospital, and trying to get at least some work done for his employer. I desperately wanted to go home, but I couldn't leave Brian for more than three hours at a time.
At last, Brian was weaned from the oxygen tube. Since I could give Brian the medication and breathing treatments myself, this meant we could come home. I faithfully cared for Brian, but the cough remained and his breathing grew worse. Less than a week later, Brian was readmitted to the hospital. I was crushed.
Tests were given and re-given to Brian. Each one came back negative. One afternoon he was given a sodium chloride sweat test to determine whether or not he had cystic fibrosis. Electrodes were placed on his lower arm to force the skin on his arm to sweat; the liquid was collected, and the chloride content measured.
Later that day a resident walked into the room and handed me a tape measure. Puzzled, I looked up at her as she turned away and told me the test would be redone tomorrow. A moment later I understood: the tape measure had been meant to divert me. I demanded to know if the results had been positive. She was not supposed to tell me, but she could not refrain. Brian had cystic fibrosis.
As I looked down at my baby, I cried, I questioned, and I prayed. A short time later my husband Tim arrived, but I couldn't even acknowledge him, and when he asked about my red eyes, I shrugged my shoulders. Then Tim asked about the test results, but I could only nod my head in disbelief.
That night I couldn't sleep; I had the worst headache of my life. A visit from our pediatrician the next morning helped a little. We learned that cystic fibrosis is a genetic disorder which causes digestive problems and frequent lung infections. In the past, those with this disease lived only a year or two. Today, life expectancy is much longer but still limited. With proper care, children can be bright and energetic, but they live with difficult treatments and life-threatening illnesses.
We grieved for Brian; we feared for our other children. We prayed for strength and courage to face each coming day. As news spread, the phones at home and at the hospital didn't stop ringing. We were overwhelmed by the love showered on us by friends, neighbors, and co-workers.
We experienced some relief when the results of the sodium sweat test for our other two children were completely normal. We could learn to care for Brian. We could bring our family back together again.
I quickly learned that being Brian's mom meant I had to acquire a whole new set of skills. The following two days I had intense training sessions with the nurses and respiratory therapists. I had to demonstrate competence at certain skills before they would discharge Brian from the hospital. I learned to give Brian medication intravenously through a semi-permanent tube, called a central line, which was surgically inserted into an artery near his heart. I learned how to clean the dressing and create a sterile environment right in Brian's crib. I even managed to suction mucus from his chest by inserting a small tube through his nose to the top of his trachea.
These intense procedures lasted only about two weeks, at which point we were reasonably sure that the medication had been able to remove the bacteria in Brian's lungs. However, there is a great deal of routine care required to keep a child with CF healthy. To clear his lungs, Brian needs breathing treatments followed by chest percussion, a clapping of the torso to dislodge mucus plugs, which result from the thick, sticky mucus his body produces. All this must be done several times a day. To aid in his digestion of fats, Brian takes enzymes with each meal or snack.
Brian's care seemed so overwhelming at first, yet after much reading, learning, and adjusting of our lifestyle, it has become a regular part of our lives. If Brian acquires certain infections, he will need to be hospitalized. To keep him as healthy as possible, he will also need periodic hospital stays known as “tune-ups.” These too, I'm sure, will become a normal part of our family's life.
What I thought was tearing our family apart has actually drawn us closer together. We are honored to be Brian's parents. We are cheered by Brian's contagious smile and encouraged by his resilient spirit. Brian's older sister adores him, his big brother loves to entertain him and make him laugh. Together we are learning to live with cystic fibrosis in one we dearly love.
When will Brian stop coughing? Probably never, but now I understand that this cough is part of the way Brian was created, and he is a beautiful child. If his genes were different, he would no longer be Brian. As we celebrate Brian's first birthday, we celebrate with thanksgiving. And in the years ahead, we will be thankful for every day God blesses our lives with this precious little boy.
We hear it all the time: “I don't care if I have a boy or a girl, as long as the baby is healthy!” But rarely do we ask, “And what if the baby isn't healthy?” When our third child was three months old, I had to ask that question. Our son Brian had just been diagnosed with cystic fibrosis.
With the results of a single test, motherhood became much more demanding than I had ever imagined. I had known it would take a lot of love, patience and sacrifice. I didn't know I would need to be a nurse, a nutritionist and a respiratory therapist! Brian needs several medications given at timed intervals. He needs a high-calorie diet rich in fats, salts and vitamins. He needs medicated breathing treatments followed by chest physiotherapy several times a day.
Brian's special needs have placed his brother and sister in extraordinary situations. They have witnessed serious medical intervention at hospitals and in our home. They have been forced to give up some of our time together to Brian's treatments. As their understanding of the situation deepens. they will have to face mortality and the untimely loss of a brother they adore.
But just as a baby's health is more important than gender, so there are character traits that I feel are more important than health. I don't want Brian or our family to be characterized by his medical condition. I hope all of our children will be known for their sincere hearts. We pray each one grows to be considerate, thankful, courageous and faithful. Health definitely enables a child to grow and do many good things. but even children with extra physical needs can enhance the lives of those around them. Our greatest hopes for Brian involve him being an inspiration to others.
Just one year old, Brian already demonstrates determination. When he faces a new challenge, he gives it his all. Brian doesn't dwell on the obstacles, he presses forward. On the playground, he puts all he can into mastering throwing a ball. Our family takes great joy in seeing him go through all the normal stages of toddlerhood.
Concerning his medical care, Brian is resilient. For example, one medical test needed a culture taken from deep in his throat. Another time he had a probe in his stomach for twenty-four hours; his arms were splinted so he couldn't pull it out. And then there are the endless blood tests. He gets through these grueling medical tests and then puts the experience behind him.
Brian also brings happiness to others. His smile is contagious. He enjoys laughing especially when others join in. He has a sweet way of lightening your heart.
Instead of taking away from our lives, our child with special needs has greatly enriched us. However long he lives and however much care he requires, I have no doubt that Brian's life has value. Brian can inspire people to accept their own strengths and weakness, focusing them on the truly meaningful things in life. Brian's challenges also give others the opportunity to show their love and compassion. I did not know the depth of love our friends and family had for us until they stood with us through these times of need.
The very act of giving has value to the giver. Brian offers me many opportunities to give. I am making an immeasurable impact on the life of another person - my son. He also causes me to grow in many ways. His treatments challenge my physical strength (and his!). I need to be emotionally strong for the stability of our family. As I help Brian endure various tests and treatments,I have to be creative in amusing him.
I may not get a lot of recognition outside of our home, but I am given many priceless moments. Sometimes Brian expresses his love to me in a special way: he comes behind me and claps his hands against my back, just as I do on his during therapy to clear his lungs. While these moments are dear to my heart, I did not become a mother for such fleeting times. It is selfless giving, intense pain and immense joy that make a mother's love. What would love be in a world without needs?
Now when I hear a pregnant woman say, “I just want a healthy baby,” her words tug at my heart. I sincerely hope she has a strong, healthy child; but if that child comes with a special challenge, I hope she can welcome the child along with the challenge.
Hi! My name is Gianna. I am 10 years old and have had a port on the left side of my chest for two years. A port is a place where I can get IV medications without having to worry if my veins will last. I have CF so that's why I need to have IVs sometimes when I get sick. I wanted to tell my story because I really didn't want to get a port, but now that I have it I think it is totally worth it.
I have always been really active. I like to wrestle and play soccer and football, and I love to go surfing and to swim. I really love the beach, especially The Cove at Santa Cruz. I also like drama. I like to sing and play the piano, and I want to learn to play the guitar. Oh, and I also want to take Kung Fu.
But before I got my port I was getting sick all the time, and when I would come into the hospital I couldn't get all the medicine I needed because my veins kept closing, and they couldn't even get PICC lines in. I was getting sick way too much, and I couldn't do all the things I love to do. So everybody tried to talk me into getting a port, but I was scared and didn't want it. I even talked to somebody who already had a port, but I was still scared. Finally, I agreed to get it, but for the first couple of months after I kept thinking, “I can't believe they made me do that.” But now it's so much easier! There's no poking around to get IVs started or worrying about whether they'll last.
So what was it like to get a port? Well, I had to go to surgery. I couldn't eat for a really long time before, in fact, I didn't eat all day because they didn't get around to doing me until really late and so I couldn't eat. But when I went to surgery they gave me Versed, a medication that makes you forget everything, so all I know is that I went to sleep and it took about 45-60 minutes to put the port in place. But I know what they did. They make an incision and put the port right under your skin, and then they string the tubing through a vein into your heart. Then they stitch you up and it's over! And you don't even have to have the stitches pulled out because they dissolve. The port will last for about four years. That's a lot longer than any IV or PICC line! And when they use my port to give me IVs they only have to change the tubing once a week!
It's not that hard to take care of a port, either. I have to come into clinic about once a month to have it flushed with heparin so it won't clog up with things like blood or medicines, but there's nothing else to do. You can't see it because it's totally under my skin. It did hurt a bit to walk when it was first in, but only for the first three days. Now I hardly notice it. For the first two to three months after it was in I couldn't swim or use the trampoline or do the active stuff that I love, but now I can do it all.
Using the port is better than an IV, too. Putting Emla cream on the site two hours before it is accessed numbs the site, and then you feel only pressure. Well, sometimes you feel a little poke, sort of like you're pushing your fingernail into it. I've done so much better since I got the port. It's totally worth it!
So if you're thinking about a port, listen to this. You may not want it, but you'll be glad you did!
There is a lot to learn about Cystic Fibrosis. It is a complicated disease that affects different people in different ways. Scientists found the CF gene back in 1989, but there is a long way to go before a cure is found. Medical people are constantly searching for new treatments and new medications to help control CF, but they can't do it alone. It takes the cooperation of people who have CF to really find out if the new treatments or new medications work.
I consider myself to be a very healthy person. Yes, I have Cystic Fibrosis, but I've been lucky. Sure, I've had my share of hospitalizations, and medications, and treatments, but that is all part of staying healthy when you have CF. I think that one reason God put me here is so that I can help people who may be sicker than I am. And I realize that participating in research studies is one way that I can help people with CF.
I've been at the CF Center at Stanford for a long time, and I know the staff here really well. And they know me. They know that I have a positive outlook, that I'm friendly, and that I am the kind of person who likes to help others. I think that is why they asked me to participate in some of the research studies. I've been in the Gene Therapy study, and CFODACT, and a few other studies in the past.
Gene Therapy is probably the most dramatic study I've been in. When the staff approached me to enroll, I was pretty scared. But they explained everything that I would do and everything that would be done to me in as much detail as I needed to fully understand what would happen. There was a written consent form that I had to read, understand, think about, and then sign after I was certain I wanted to do this. And I knew I could change my mind at any time if I decided I didn't want to continue for any reason. After I thought about the fact that Gene Therapy might point the way toward controlling or even curing CF, I knew I wanted to do it. But I guess you don't lose your fear of the unknown that easily. After my first treatment I can remember thinking, “What have I done?”
Safety issues are always a concern in any kind of research. That is why it is so important to really understand just what the research is about, and what the long-term effects could be. But I felt I really knew and trusted the staff at the CF Center. They wouldn't let me get hurt.
Time is another issue. If you agree to be in a research study, you have to do things at certain times. Some studies are done during a hospital stay, and it's just like any other hospitalization. But some studies involve coming to clinic for longer or extra visits, or you have to take medicines or treatments at home at certain times. You must follow the study protocol, so it's important to know if the study will fit into your time schedule.
Research into CF is important for everyone who has CF. If you think being involved in research is something you would like to do, you don't have to wait until the staff asks you. Ask questions; be interested in CF; ask if you would be a candidate to do some research. It takes all different kinds of people with all different degrees of CF at all different ages to do the many different research projects that are out there. Stanford is doing a lot of research all the time. Ask if you meet the criteria for any of the studies. Maybe the reason you haven't been asked to do research is that you are healthy and don't come into clinic very often. That might just mean that you are the perfect candidate for a study. Look into the studies carefully. Know what you are getting into. Read and understand the consent. Getting involved in research is a gift that only you can give to the CF community.
