301November/December 2003

ORIGINAL ARTICLE

PHCABSTRACTIntroduction: Addison’s disease is arare chronic illness caused byadrenocortical insufficiency. Under-standing the process of family adap-tation to this chronic illness in achild will help practitioners en-hance the ability of the child andfamily to cope with this stressful di-agnosis.Method: A qualitative descriptivecase study methodology was usedto explore the process of familyadaptation to this chronic illness.Eight interviews were conductedwith a newly diagnosed 11-year-oldWhite girl with Addison’s disease,along with her parents, grandpar-ents, and siblings. Participant obser-vations were made in the family’shome. Drawings by the girl withAddison’s Disease were included inthe data analysis, along with an in-terview with the child .Results: The overall theme of adap-tation to this chronic illness is livinga normal life. The family noted thatafter reaching some acceptance,they began learning how to managethe disease. This process was en-hanced by seeking informationabout the disease and receiving thesupport of others; in addition, thechild was affected by the accep-tance of her peers.Discussion: Recognizing factors thatmay influence the family’s adjust-ment to the diagnosis of Addison’sdisease in a child will help to directeducational and support efforts bypractitioners.J Pediatr Health Care. (2003). 17,301-310.

Adaptation to Addison’s Disease

in a Child: A Case Study

Bonnie Gance-Cleveland, PhD, PNP, RNC

The National Health Interview Survey on Child Health conducted bythe National Center for Health Statistics estimates that 31% of childrenyounger than 18 years of age, or almost 20 million children, have a chronicillness (Jackson, 2000). These chronic illnesses do not have a cure; therefore,“The goal of care is to minimize the manifestations of the disease and max-imize the child’s physical, cognitive, and psychosocial potential” (Vessey &Mebane, 2000, p. 27). Practitioners provide care to these children and theirfamilies to achieve this goal. Knowledge of the family’s adaptation to thechronic illness can increase the practitioner’s ability to intervene with thefamily as they learn how to cope with chronic illness.

One of the more rare chronic diseases affecting children is Addison’s Dis-ease. When a child is diagnosed with this disease, the child and family mustlearn to cope with a chronic disease. Because of the rarity of this condition,there is a paucity of information available to families and to the health careproviders who care for them.

Addison’s Disease, or primary adrenal insufficiency, is a rare disordercharacterized by chronic adrenocortical insufficiency resulting in defi-ciency of glucocorticoids and mineralocorticoids (Sabol, 2001). Addison’sDisease affects females two to three times more often than males, and onsetmost often occurs between the ages of 30 to 40 years, with 4 out of 100,000people affected by the disease. Although the disease occurs rarely in chil-dren, it can occur at any age. Historically, the cause of Addison’s diseasewas tuberculosis infection; however, since the introduction of antibiotics,the main cause of Addison’s Disease has been autoimmune destruction ofthe adrenal gland (Sabol, 2001). Autoimmune adrenalitis is relatively morefrequent in children than adrenal insufficiency as a result of infections,

Bonnie Gance-Cleveland is Assistant Professor, University of Colorado Health Sciences Center, Denver.

Reprint requests: Bonnie Gance-Cleveland, PhD, PNP, RNC, University of Colorado Health Sciences Center,4200 East Ninth Ave, Denver, CO 80262; e-mail: Bonnie.Gance-Cleveland@uchsc.edu.

Copyright © 2003 by the National Association of Pediatric Nurse Practitioners.

0891-5245/2003/$30.00 + 0

doi:10.1016/mph.2003.46

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although occasionally the etiology mayinvolve parasitic infections, cytomegalo-virus, fungal infections, amyloidosis(deposits of waxy substance called amy-loid in vital organs), and adrenoleuko-dystrophy (fat molecules collect in theadrenal glands) (Sabol, 2001).

The disease results in a lack of the glu-cocorticocoids that aid the body’s abilityto handle stress (O’Donnell, 1997). Glu-cocorticoids are involved in carbohy-drate, protein, and fat metabolism, carbohydrate and glucose storage, de-creasing inflammation, and suppressionof the immune response. Often a defi-ciency in mineralocorticoids also exists,which leads to increased renal sodiumloss and potassium reabsorption, re-sulting in decreased intravascular vol-ume, vascular tone, cardiac output, andrenal perfusion. These symptoms in turnresult in lower arterial pressure, posturalhypotension, and compensatory tachy-cardia, which, if left untreated, leads tovascular collapse and coma (O’Don-nell, 1997).

The adrenal glands are controlled bythe pituitary gland, which increases theproduction of adrenocorticotropic hor-mone (ACTH) to stimulate the adrenalglands. Diagnostic findings for patientswith Addison’s Disease include lowplasma cortisol and high plasma ACTHlevels (Gance-Cleveland, 1999; O’Don-nell, 1997).

The increased ACTH stimulates pig-ment-producing cells in the skin, result-ing in darkened scars, skinfolds, andmucous membranes of the lips, mouth,rectum, vagina, and nipples and blackfreckles appearing over the face, fore-head, neck, and shoulders. In additionto changes in pigmentation, early symp-toms of the disease may include fatigue,decreased appetite, weakness, and hy-potension. Children may exhibit a de-creased level of activity and a drop inweight and a corresponding shift on thegrowth charts. Undetected, the diseasemay lead to anorexia, nausea, vomiting,and diarrhea, which in turn can lead todehydration, dizziness, syncopal epi-sodes, small heart, decreased cold toler-ance, fever, abdominal pain, hypo-glycemia, apathy, and mental confusion.Prolonged delay in diagnosis and treat-ment may lead to developmental issueswith an impact on school performancebecause of decreased attendance andlethargy when in attendance. Adrenalcrisis or acute adrenal insufficiency can

occur if a child remains undiagnosed orthe child is given inadequate amountsof cortisol to deal with stress such as infection, exposure to cold, burns,trauma, surgery, or sodium loss result-ing from perspiration (O’Donnell, 1997).Symptoms of adrenal crisis include hypotension as a result of decreasedintravascular volume, severe renal in-sufficiency, electrolyte disturbances,metabolic acidosis, and hypoglycemia(Lenzer, 1998).

Complications of Addison’s Diseaseinclude acute adrenal crisis, psychoticreactions, hyperkalemic paralysis, andexacerbation of the disease with preg-nancy. Other complications includethyroiditis, hypoparathyroidism, per-nicious anemia, and ovarian failure,probably caused by autoimmune disor-ders.

Treatment of the disease involves re-placement of the missing hormones,most often hydrocortisone (Cortisol)and fludrocortisone acetate (Florinef)with a daily maintenance rate. Childrentake hydrocortisone, 12 to 15 mg/kg/day in two doses with two-thirds of thedose in the morning and one-third ofthe dose in the evening to duplicate thenormal circadian rhythm (Gance-Cleveland, 1999). Addisonian crisis canoccur with any stress; therefore, thedose of hydrocortisone is increasedwith any illness, emotional stress, phys-ical stress, trauma, or surgery. Parentsare taught to double or triple medica-tion in anticipation of a stressful event(eg, tests, travel, and field trips) and toincrease medication in the event of any

illness (eg, fever, vomiting, and diar-rhea). Families are told to repeat thedose if the child vomits within 1 hour oftaking the medication. The dose of fluo-hydrocortisone is 0.05 to 0.2 mg per day,and parents are instructed to increasethe dose with exposure to heat and in-creased sweating. In addition, familiesare given hydrocortisone sodium succi-nate (Solu-Cortef) injections to have onhand at home and school in an emer-gency. Injections are indicated in a childwho is unable to take medicationsorally or has an injury resulting in lossof consciousness (Gance-Cleveland,1999).

Knafl and Deatrick (2002) describeda process undergone by families of chil-dren with a chronic illness that, overtime, led family members to come toview both the child and their life as nor-mal. This normalization was defined asacknowledging the condition and itspotential to threaten lifestyle; adoptinga normalcy lens for defining the childand family; engaging in parenting be-haviors and family routines that areconsistent with normalcy lens; and in-teracting with others based on a view ofchild and family as normal. As notedearlier, little information is available onfamilies’ adaptation to Addison’s dis-ease in a child. The purpose of this casestudy is to explore the process of adap-tation to this childhood chronic illnessand come to an understanding of thesimilarities and differences in normal-ization when a child has Addison’s Dis-ease. In this case study, a child with Ad-dison’s Disease and her family memberswere asked to provide information ontheir adaptation to this chronic illness.Drawings by the child with Addison’sDisease provide additional insight intothe experience.

Ryan-Wenger (1998) supports the useof children’s drawings as a projectivetechnique, based on the assumptionthat drawings reflect a child’s self-con-cept, attitudes, and conflicts and repre-sent emotions or thoughts that childrenare unable or unwilling to verbalize.Spontaneous drawings provide impor-tant insights into the child’s world;drawings requested by the nurse are aform of communication. The inclusionof the child’s drawings provided in-sights into her experience with the di-agnosis of Addison’s Disease thatmight not have been fully understoodwithout the drawings.

Gance-Cleveland

Addisonian crisis can

occur with any stress;

therefore, the dose of

hydrocortisone is

increased with any illness,

emotional stress, physical

stress, trauma, or surgery.

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METHODThis qualitative descriptive case studywas undertaken to explore the processof family adaptation to Addison’s Dis-ease. The research question addressedin this case study was, “What was theprocess of family adaptation to the di-agnosis of Addison’s Disease in achild?” Tape recorded, unstructured in-terviews lasting 15 to 75 minutes wereconducted with members of a familywith a newly diagnosed 11-year-oldWhite female child with Addison’s dis-ease. See the Box for questions guidingthe unstructured interviews with thechild, parents/grandparents, and sib-lings. Eight interviews were conductedwith an 11-year-old school-aged youthand her parents, grandparents, and 9-and 13-year-old siblings. Consent wasobtained from parents and grandpar-ents, and verbal assent was obtainedfrom each child prior to the interview.Interviews were conducted in thehome. As the interviewer was leavingthe home at the conclusion of the inter-view, the child with Addison’s diseaseoffered to share several pictures and apoem about the disease. Asecond inter-view was scheduled with that child toobtain an explanation of each drawingand the poem. Participant observationswere conducted to obtain informationon family interactions and the interac-tions of siblings.

The data were transcribed verbatimand placed on the Ethnograph, a soft-ware data management program that fa-cilitates sorting and analyzing the data(Seidel, Kjolseth, & Seymour, 1988) formanaging and sorting the data. The datawere analyzed by using the constantcomparative method (Lincoln & Guba,1985). The central theme that emergedwas the family’s ability to adapt to thechild’s illness and live a normal life. Fac-tors that made this adaptation possiblewere identified. A review of the relevantliterature on chronic illnesses in childrenwas used to compare and contrast theemerging concepts from this study andtheir relationships. These emerging con-cepts were discussed with the familiesand further modified until the investiga-tor, informants, and a colleague foundthem credible.

RESULTSThe overall theme in adapting to thischronic illness for this family was beingable to live a normal life. The family de-

scribed a process of adjusting to the diag-nosis that was influenced by the severityof the child’s condition and involved agrief process similar to that described byKubler-Ross (1969). After reaching alevel of acceptance, the family began aprocess of learning to manage the diseasethat was influenced by seeking informa-tion about the disease and receiving thesupport of others; the child was also in-fluenced by the acceptance of peers (seeFigure 1). A final theme that unfoldedduring interviews with siblings was theimpact of the disease on them.

Adjusting to the DiagnosisThe process of adjusting to the diagno-sis of Addison’s disease in a child wasdescribed by family members using

concepts similar to those that Kubler-Ross (1969) described in her work withterminally ill patients. The child andher family described denial regardingthe significance of the disease. Onefamily member said, “The hardestthing for me is some of my family mem-bers don’t seem to want to accept thefact that this is a lifelong thing…theysay she might outgrow it.”

A parent shared this story depictingthe child’s denial:

One day when they only had a halfday of school and she was runninglate, I told her to go take her medi-cine. She said she didn’t have timeand that she only had a half day andshe would take it when she gothome…When she got home she hada headache, was crabby, and didn’tfeel very good. Then she knew shehad to take (her medicine) in themorning when she gets up.

The family reported feelings of angerand guilt when they took the child tothe emergency room for the third timeprior to the diagnosis of Addison’s Dis-ease.

Sometimes some of the nursesmade me feel like I did somethingwrong. They always used to ask mewhy I let her get so sick and how

Gance-Cleveland

BOX Guidelines for unstructured interviews

Interview of Child with Addison’s Disease• If you met a child who just found out that he or she had Addison’s Disease, what

would you tell him or her that it is like to have this disease?• What is the hardest thing about having Addison’s Disease?• How have you learned to take care of yourself with this disease?• What have other people done that has been helpful to you since you found out

that you had Addison’s Disease?• What is the biggest change that you have noticed since you got Addison’s Disease?

Parent Interview• What has it been like to have a child diagnosed with Addison’s Disease?• How did you and your family learn to cope with this disease in your child?• What was the most helpful to you and your family?• What was the hardest part of having a child with Addison’s Disease?

Sibling Interview• What is it like to have a sister with Addison’s disease?• What has been the hardest part of having your sister get Addison’s Disease?• If you had a friend who just found out that her sister had Addison’s Disease,

what would you tell her it is like?• Is there anything that you would tell that friend that might help her?

The central theme that

emerged was the family’s

ability to adapt to the

child’s illness and live

a normal life.

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come she was so skinny. They wouldwant to know what she was eating,and when she had pneumonia theywanted to know why we waited un-til she was so sick until we broughther in.

The grandmother also reported feel-ing angry:

One time I was in the emergencyroom with her because her motherwas unable to get there, and thenurses wanted her to stand on thescale to be weighed and she was toosick to stand up; she would get dizzy,and so I was holding on to her, andthey said don’t hold on to her. I wasreally mad they put her through that.

A parent also described bargainingwhen her child was very sick and theywere not sure of a diagnosis: “I thoughtif they could just figure out what waswrong I could deal with it, but notknowing was driving me crazy.”

The parents described their child’speriod of depression after the diagno-sis: “The only time she has ever been re-ally upset was the day that they told her(the diagnosis), and then she cried andtold me that it wasn’t fair and that shedidn’t want to have a disease.”

Depression was also evident in thepicture this child drew of herself in thehospital (Figure 2). She described thedrawing: “This is the picture I drewwhen I got out of the hospital one timebefore they knew what was wrong with

me. I was sad because I didn’t want togo there (the hospital). I was scared. Ididn’t know what they were going todo.”

Afactor influencing the family’s abil-ity to accept the diagnosis was theseverity of the child’s condition. Thechild was hospitalized in the intensivecare unit in critical condition before thediagnosis of Addison’s Disease waseven considered, and they experiencedmultiple hospitalizations and manypainful procedures (Figure 3). The fam-ily experienced the stress of the possibledeath of the child prior to the diagnosisand initiation of therapy. The diagnosisprovided a sense of relief for this family.“She was in the hospital five differenttimes before they diagnosed her (withAddison’s Disease), and every time shewas more seriously ill. She was in in-tensive care two different times withpneumonia and dehydration.”

This family had been told there was agood chance the child had anorexia nervosa or a brain tumor, and manyother serious diseases had been ruledout prior to the diagnosis. Therefore,Addison’s Disease seemed less threat-ening after the family had worriedabout these other diseases and hadstruggled for months with the un-known etiology of the child’s frequent,severe illnesses.

The child also was aware of theseverity of her condition, as evidencedby the drawing in Figure 4, which thechild explained as the following:

That is the picture of the ambu-lance that I rode to the hospital in be-cause I was too sick for mom to takeme in the car. I woke up one morningwhen I had just gotten out of the hos-pital and I didn’t feel good at all. Itold my mom take me to the doctorright now. I went and laid down inmy mom’s car and she carried meinto the doctor’s office. The nursecouldn’t get my blood pressure andso she hurried out to get the doctorand she took one look at me and toldmy mom to come out into the hall.They came back in and the doctorsaid that I think you know that youhave to go back to the hospital and Isaid, “Yes.” But she said, “You haveto go in an ambulance because I wantyou to get there fast,” and I said,“Can you get me a helicopter, cuz Ijust wanted to get there fast.”

Gance-Cleveland

FIGURE 1 Process of adaptation to Addison’s Disease.

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Acceptance of the diagnosis is alsoreflected in this statement: “I felt muchbetter (after being diagnosed with Ad-dison’s Disease) because I thoughtmaybe they could help it and maybe Iwouldn’t have to come to the hospitalso much.” Acceptance was also re-flected in the series of drawings doneby this child. In the first drawing, doneprior to diagnosis, the girl is crying andupset at being sick (see Figure 2). Afterdiagnosis and stabilization, the picturesdepict a girl in her hospital room watch-ing television and visiting with clowns(see Figure 5).

Adjusting to the diagnosis of Addi-son’s Disease appeared to be a complexprocess for this family. They explainedthat their ability to adapt was enhancedby the resources available to them (viaonline information and the NationalOrganization of Rare Diseases) and thecoping behaviors they were able to use,such as looking at the positive aspectsof the situation.

Seeking information about the dis-ease. Another step toward adapting toAddison’s Disease was obtaining infor-mation about the disease and what itmeant. The child relied on her parentsfor information or interpretation of theinformation that health care profes-sionals provided:

When you first find out that youhave Addison’s Disease, it’s hard tounderstand until someone explains itmore, so you have your parents ex-plain it a little more. They were usingbig medical terms for it and I didn’tunderstand…. One time I had a CTscan and the man (a medical resi-dent) goes, “Well it’s just a simpleprocedure; it’s just going to cutthrough your brain” (her eyes arelike huge saucers as she is recallingthe situation)…then I kinda gotscared and my dad told me what hewas talking about. That they’re justgoing to take pictures of your brain.

The parents also expressed a need forinformation to increase their ability tocope with the diagnosis and care for achild with Addison’s Disease. They de-scribed obtaining information fromother parents, medical professionals,the public library, a hospital library, andthe National Organization of Rare Dis-eases. The mother said, “It’s probably

Gance-Cleveland

FIGURE 2 Adjusting to the diagnosis: Depression.

FIGURE 3 Accepting the diagnosis influenced by severity of illness.

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been easier for me to accept that she hasit because I have a friend whose son hasit. The sickest he’s ever been was beforehe was diagnosed and he’s had it foreight years.” Much of the informationfamilies obtained did not come fromhealth care professionals.

Learning to manage the disease. Themother described her fears when her

child was first ill, shortly after being diagnosed with Addison’s Disease.

The first time she got sick she wasrunning a fever of 101°…her energylevel was wiped out and she curledup in a little ball and went to sleepand it scared me, and I tripled hercortisone and within 4 hours she wasfine. And I guess since I saw the fast

turnaround with just tripling hermedicine I realized that she was go-ing to be okay.

The child also recognized her respon-sibility in managing her care. The girl ex-plained learning to manage the diseasein another one of the pictures (Figure 6):

That’s me taking my medicine athome. That pill box is pretty big, butI wanted you to see it. The red thingis my pill crusher, the other thing isthe pill box. The pills were hard tocrush between two spoons, so mydad got me the pill crusher. I crushthe pills and mix it with applesauceso I can take them because I can’tswallow pills too good. My dad isbuying me a pill crusher to keep atgrandma’s house. She has frozen ap-plesauce and I mix it with that.

Need for SupportOne facet of the coping process identi-fied by this family was a need for sup-port for the child with Addison’s Dis-ease and the family. The motherreported, “Just the moral support, Iwould have never made it without mysister being there all the time.”

Her child reported, “Well, my momhad a best friend and her son has it andhe kinda told me how to accept it, so itwasn’t as hard. He helped me a lot.”

Need for Acceptance by PeerGroupThe child with Addison’s Disease alsodescribed a need for acceptance by thepeer group. She said:

Gance-Cleveland

FIGURE 4 Severity of diagnosis: Emergency hospitalization.

FIGURE 5 Acceptance of the diagnosis.

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One thing that bugs me is I wonderwhy kids would treat me different justbecause I have some disease. It kindagets me upset…I don’t think its rightto do cuz I know if I had a friend (witha chronic illness) I wouldn’t do that…some of the kids treat me different cuzthey think it is contagious or some-thing. Cuz I had a friend who was re-ally close and then when I came back(from the hospital) and she found out(about the Addison’s Disease) we’renot that close. When I’d go up to hershe’d just kinda say hi, and walk awaywith a different friend. That didn’tmake me feel too good.

Living a Normal LifeAn additional factor that became ap-parent during the interviews was acommitment to do whatever was nec-essary to ensure that the child wouldlive as healthy and normal a life as pos-sible. The mother of this recently diag-nosed child said she was able to acceptthe diagnosis when she understoodthat her child could live a normal lifewith good management.

And that’s another thing that Iread …that really made me feel morecomfortable about accepting it all, itsaid that if you have good doctor-patient relationship and you followthrough on your therapy that she canlive a perfectly normal, full life. Thatit’s the people who don’t do what thedoctors tell them to do that haveproblems.

The child’s parent describes the im-portance of living a normal life: “I reallytry to make her feel like she can do any-thing she did before.” Adaptation to thedisease was also influenced by thechild’s perception of their ability to livea normal life. The child explained theimportance of living a normal life: “Iwould tell another kid…that you justact normal, it’s nothing. All you have todo is take medicine. It’s not like differ-ent diseases, and if they ever needed afriend to talk to they could talk to me.”

The child with Addison’s Diseaseadapted to this chronic illness when shediscovered the ability to control her dis-ease, which allowed her to live a nor-mal life. The family’s stories indicatededication and commitment alongwith confidence in their ability to en-sure their child would live a normal life.

Impact of the Disease on SiblingsA final theme that emerged was the im-pact of the disease on siblings. Al-though the parents did not think the ill-ness had an impact on the siblings,interviews and observations of the sib-lings revealed worry about the siblingwith Addison’s Disease, guilt whenplaying too rough, an increased level ofresponsibility, and jealousy over specialtreatment for the Addison’s Disease pa-tient.

The siblings said that they worriedabout their sister with Addison’s Dis-ease: “I was worried when she was inthe hospital that she might die, becauseshe looked so bad and stuff.”

Increased responsibility was evidentin this sibling’s statement: “Now everynight before bed I ask her, ‘Did you takeyour cortisone?’ Cuz she gets crabby

when she doesn’t get her medicine, andI don’t want her to get sick again.”

Sibling rivalry or jealousy emergedin the family: “It ticked me off whendad bought her a new bike and he saidhe would buy her new softball stuff andnot me.”

FINDINGS COMPARED WITHEXISTING LITERATUREKnafl, Breitmayer, Gallo, and Zoeller(1996) described five family manage-ment styles (FMS) in families with aschool-aged child with a chronic ill-ness. The five FMSs differed in the fam-ily’s definition of the illness, manage-ment goals, and approach and illnessconsequences (see Table). The FMSs(thriving, accommodating, enduring,struggling, and floundering) representa continuum of difficulty families expe-

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FIGURE 6 Learning to manage the disease.

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rience in managing a child’s chronic ill-ness. Findings from this case study sug-gest that the support and educationprovided to the family in the adapta-tion process may influence the family’sability to adopt a more favorable FMS.Learning to manage the disease wasconsistent with a proactive manage-ment approach versus the reactive,compliant, or uninvolved manage-ment. The child’s role in learning tomanage the disease was consistent withcompetent self-care behaviors de-scribed by Knafl et al. The theme of liv-ing a normal life is consistent with par-ents viewing the child’s identity asnormal and child’s perception of theconsequences of the disease as no bigdeal (see Table).

The findings from the current studyabout the impact of the severity of theillness on adjusting to the disease areconsistent with Horner’s (1997) de-scription of overwhelming uncertaintywhile waiting for a diagnosis and reliefwhen a diagnosis was made. The cate-gories that emerged within the themeof adjusting to the diagnosis—denial,anger, bargaining, depression, and ac-ceptance—are consistent with thestages of grief as described by Kubler-Ross (1969) in her work with terminallyill adults and also observed in familieswith severely ill children (Kubler-Ross,1999). In contrast to the literature onadults with chronic illnesses who arefrequently faced with isolation (Burk-hardt, 1987), the child with Addison’s

Disease said that coping was influ-enced by her peer group’s acceptance.

Families in this study described seek-ing information as the next phase afteraccepting the diagnosis. Similarly,Shepard and Mahon (2000) suggest thathaving a diagnosis leads to a search forinformation about causes, treatments,

and effects of the child’s condition. Alsoconsistent with the findings of thisstudy was Craig’s (1983) model of cop-ing behavior in chronic illness, whichincludes the use of both personal andextrapersonal resources.

Gibson (1988) used Lazarus and Lau-nier’s theory on stress and coping as a

framework for understanding parentalresponse to coping with a chronic ill-ness such as cystic fibrosis. Althoughthe parents experienced degrees ofstress in meeting the illness-related de-mands, they coped with it through thehelp of available resources and action-oriented and intra-psychic behaviors.Findings from the current case studyare consistent with Gibson’s theory offamily coping with chronic illness in achild. Coping depended upon the re-sources the family had available andthe behaviors they employed. In thecurrent case study, resources that facili-tated coping included information andsupport of friends and family. Also, fo-cusing on the positive as a means ofcoping was evident in interviews withboth the child and parents: the diagno-sis was better than not knowing, it wasbetter than some of the diseases thathad been ruled out, the child was grate-ful the medication could be taken orallyinstead of by injection, and they wererelieved the child could live a normallife. Learning to manage as describedby these families is consistent with Gibson’s (1988) description of cop-ing, which involved drawing on theirown resources and feeling less over-whelmed. Another aspect of the studyconsistent with Gibson was the parent’scommitment to and love for the child asa strong motivating force to do one’sbest to deal with the difficulties. Familymembers who believe they can do whatis needed are less likely to be threatened

Gance-Cleveland

TABLE Relationship between adaptation to Addison’s Disease in a child and Knafl’s defining, managing, and consequences of childhood chronic illness

Themes* Subthemes Case study findings

Defining themesParental views of child Normal, problem, tragic Living a normal lifeIllness view Life goes on, ominous, hateful, uninformed Learning to manageChild’s view of health More healthy, equally healthy, less healthy Living a normal life

Managing themesParenting philosophy Accommodative, sheltering, minimizing, inconsistent Learning to manageManagement mindset Confident, burdened, inadequate, observer Accepting the diagnosisManagement approach Proactive, reactive, compliant, uninvolved Learning to manageSelf-care behavior Competent, deficient, unconventional Learning to manage

Consequence themesParental mutuality Present or absent Adjusting to the diagnosisIllness dominance Yes or no Need for acceptance by peersFuture dread Present or absent Not addressed in case studyChild consequences No big deal, intrusive, worry Living a normal life

*Themes and subthemes obtained from Knafl & Deatrick, 2002.

The role of the child in

learning to manage this

disease and the need for

acceptance of his or her

peer group are important

aspects of care for children

with Addison’s Disease.

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or to feel helpless or hopeless in stress-ful situations (Gibson, 1988).

In contrast to the findings in the pre-sent study, Shepard and Mahon (2000),in their discussion of chronic childhoodillnesses, stated that coping patterns,concurrent stressors, and culturally de-termined values about health and ill-ness affect the family’s response to thediagnosis of a chronic illness. They sug-gest that visibility of the condition, po-tential for functional limitations, andpresence of mental disability influencea family’s ability to cope. In the presentcase study, the family with a child withAddison’s Disease did not mention cul-tural influences, the visibility of thecondition, or the presence of mentaldisability as influential factors. How-ever, similar to living a normal life,Shephard and Mahon suggest that ex-pectation of suffering for the child, un-certainty about prognosis, previous ex-perience with chronic illness, andfamily perceptions about the conditioninfluence coping in the family.

In addition, learning to manage thedisease is consistent with the frame-work by Strauss et al. (1985) for under-standing chronic illness, which delin-eates key problems in living withchronic illness, including prevention ofmedical crisis, management of crises ifthey occur, control of symptoms, andcarrying out prescribed regimens.

Finally, consistent with the presentstudy, Barbarian (1995) reports that sib-lings experience a variety of stressorsand that parents have difficulty in accu-rately assessing the coping of siblings,and Morrison (1997) reports that mostsiblings are stressed during the chroni-cally ill child’s hospitalization.

DISCUSSIONMany consistencies were found be-tween the findings of this case studyand the literature on chronic childhoodillnesses; however, because of the rarenature of Addison’s Disease in a child,literature on the adaptation to Addi-son’s Disease could not be found in theliterature. This case study suggests thatthe family’s experience with this rarechronic disease is similar to the experi-ence of other families with otherchronic conditions. This family re-ceived care from nurses who were notfamiliar with the disease and thereforeprovided very little information orguidance. Information from this case

study can be used to enhance the prac-titioners’ information on this diseaseand the family’s adaptation to the Ad-dison’s Disease.

Much of the information that thefamilies in this study obtained did notcome from health care professionals, inpart because this rare disease had notbeen seen in a child before by many ofthese providers. Practitioners need tobe aware of the sources of informationthat patients are using and the accuracyof that information, and they need tounderstand the process of adaptation toAddison’s Disease for a family. Thiscase study revealed the need for infor-mation so the family could adapt tothe illness. Lack of familiarity with

Addison’s Disease may interfere withthe practitioner’s ability to provide in-formation and support families at thisdifficult time. Thus, familiarity withsources of information and identifyingfactors that may influence the family’sacceptance of the diagnosis and theprocess of adjusting to the diagnosiswill direct education and support ef-forts. In addition, the role of the child inlearning to manage this disease and theneed for acceptance of his or her peergroup are important aspects of care forchildren with Addison’s Disease. Thenurse practitioner may want to discusswith the child how he or she is manag-ing the disease, as well as what friends

have been told about the disease andhow supportive they have been. Aprac-titioner who recognizes the family’sneed for support and the child’s needfor acceptance may provide the familywith information on support groups orput them in contact with another familythat has a child with Addison’s Disease.Referring families to the National Orga-nization for Rare Diseases (www.nord.org) is a good way to increase theirknowledge about the disease. With in-formation from families about the im-portance of the child’s ability to live anormal life, the practitioner can rein-force the positive prognosis with goodmanagement.

Finally, the practitioner should beaware of the impact of the disease onsiblings and the parents’ probable lackof awareness of this impact. Duringwell-child examinations, youth shouldbe screened regarding chronic illness intheir families and the impact of the dis-ease on them. Visits with chronically illpatients should also include an assess-ment of the family’s response to thechild’s chronic illness.

REFERENCESBarbarian, O.A. (1995). Siblings adaptation to

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Burkhardt, C. S. (1987). Coping strategies of thechronically ill. Nursing Clinics of North America,22, 543-550.

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Gance-Cleveland, B. L. (1999). Addison’s disease.In D. Robinson (Ed.), Primary care across thelifespan (pp. 25-31). St. Louis: Mosby.

Gibson, C. (1988). Perspectives in parental copingwith a chronically ill child: The case of cystic fi-brosis. Issues in Comprehensive Pediatric Nurs-ing, 11, 33-41.

Horner, S. D. (1997). Uncertainty in mothers’ carefor their ill children. Journal of Advanced Nurs-ing, 26, 658-663.

Jackson, P. L. (2000). The primary care providerand children with chronic conditions. In Pri-mary care of the child with a chronic condition (pp.3-19). St. Louis: Mosby.

Knafl, K., & Deatrick, J. (2002). The challenge ofnormalization for families of children withchronic conditions. Pediatric Nursing, 28, 49-53,56.

Knafl, K., Breitmayer, B., Gallo, A., & Zoeller, L.(1996). Family response to childhood chronicillness: Description of management styles.Journal of Pediatric Nursing, 11, 315-326.

Kubler-Ross, E. (1969). On death and dying. NewYork: Macmillan.

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Practitioners need to be

aware of the sources of

information that patients

are using and the accuracy

of that information, and

they need to understand

the process of adaptation

to Addison’s Disease

for a family.

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310 Volume 17 Number 6 JOURNAL OF PEDIATRIC HEALTH CARE

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