Pain and Suffering in an Adolescent with Neuroblastoma

Case Study

MS was a male African Americanwho was 15 years old when he re-ceived a diagnosis of right retro-orbital olfactory neuroblastoma.MS received surgery, chemo-therapy, and radiation therapy.After completing the initial treat-ment, he was readmitted to thehospital repeatedly for anticancertreatments and symptom control ofright facial pain, mucositis, andnausea and vomiting. Eight monthsafter receiving the diagnosis, thepatient was admitted to the hospi-tal for severe mid-back pain andlower extremity weakness. A diag-nostic workup revealed wide-spread bone metastases involvingthe thoracic spine and impingingon the spinal cord. Treatment in-cluded therapy with corticoste-roids, radiation, pamidronate, andintensive rehabilitation.

Three months later, MS was ad-mitted to the hospital for severepain, primarily in the right facialarea. He was treated with bolus

injections of intravenous hydro-morphone, per his preference, thatwere preceded with injections ofintravenous diphenhydramine torelieve pruritus associated withthe opioid. The patient requestedthat the drugs be injected quicklyinto the central line to ensurerapid onset of action. He often wasfound to be in a fetal position, cov-ered by the blankets in a darkroom. MS’s parents requestedthat he be admitted to our facility,despite the fact that our hospitalis an acute-care setting that doesnot include pediatric care beyondinfancy.

One month later, the patient wasadmitted to the hospital with pro-gressive cancer, complaints ofpain, and need for symptom con-trol. The Palliative Care ConsultService was asked to see MS, andhe was transferred to the PalliativeCare Unit. Although the patientcontinued to receive the intrave-nous regimen initially, gradually hewas converted to oral long-actingmorphine with immediate releasefor breakthrough pain. Adjuvantanalgesics were added to the regi-men. Other symptoms included alo-pecia, severe pruritus predomi-nantly over the right facial area,odor from the mouth and nose re-sulting in taste changes, and loss ofvision in the right eye. Urinary con-trol problems arose, and a bladder

catheter was placed. Bowel inconti-nence occurred intermittently.

Staff were conflicted regardingan appropriate plan of care. Thepatient was perceived as infantileand demanding, frequently callingout for assistance, yet refusing toparticipate in his care. Dissent re-volved around whether limitsshould be set or whether the pa-tient should be allowed to havecomplete control (eg, not bathingand taking medications when hewanted) and have all his requestsmet (eg, special food and videogames). The staff often felt helplessand frustrated. The parents visitedinfrequently, even when money andpasses were given to defray thecost of transportation or parking.

With aggressive teamwork,symptoms were better managed,MS’s anguish and anxiety lessened,and his family became open to hisadmission to our home hospiceprogram. Less than 3 weeks afteradmission to the hospice, the pa-tient returned to the Palliative CareUnit with increasing shoulder andabdominal pain. Because of diffi-culty in swallowing, the oral mor-phine was converted to continuousintravenous morphine infusion,supplemented with bolus doses ofmorphine. MS’s condition continuedto deteriorate, and he died 3 daysafter transfer with his pain havingbeen relatively well controlled.

Department Editors: Laura J. Hilder-ley, RN, MS; Ryan R. Iwamoto, RN, CS, MN;and M. Tish Knobf, RN, PhD, FAAN.

Queries and contributions for Mul-tidisciplinary Rounds may be sent to theDepartment Editors at Cancer Practice,American Cancer Society, 1180 Avenue ofthe Americas, New York, NY 10036.

S4 CANCER PRACTICE May/June 2002, Vol. 10, Suppl. 1© American Cancer Society 1065-4704/02/$15.00/S4 S4–S8

Judith A. Paice, PhD, RNResearch Professor of Medicine

Northwestern UniversityMedical School

Division of Hematology-OncologyPalliative Care and

Home Hospice ProgramNorthwestern Memorial Hospital

Chicago, Illinois

Response 1

As an oncologist, I first met MS 3 monthsbefore he was referred to the PalliativeCare Unit. My role was that of “service”attending (ie, 2 weeks of daily inpatientrounds with housestaff for all inpatientson the oncology floor). Directing hiscare were his attending oncologist and athird-year oncology fellow who washighly involved. Spinal cord compres-sion and widespread bony metastaseshad been diagnosed in MS about 1 weekbefore my starting on service. When thehousestaff presented him to me, I wasmost impressed with the degree of alien-ation and frustration that they felt. It ap-peared that the entire focus for bothhousestaff and nursing staff on the oncol-ogy floor was the inappropriateness ofadministering boluses of intravenous di-phenhydramine and hydromorphone ev-ery 2 hours.

When I first went into the room Ifound a relatively healthy-looking, 15-year-old boy in a completely dark roomwho seemed almost unarousable letalone able to engage in conversation. MSdenied any pain but immediately re-quested reassurance that his medicationswould be given on schedule. A large TVset and many video games were in theroom, but there were no flowers, cards,or pictures. Initially, I felt enormous dis-tress and anger: How could this hospitalaccept a pediatric case when there wereno supportive services? How could I carefor a child that was only 4 years youngerthan my own son? How could I addressthe staff’s concerns about the pain regi-men? These questions continued tohaunt me during and after this firstmeeting.

Attempting to deal first with symp-tom control, MS told me that his symp-toms were completely controlled andthat he would accept no changes. Hisattending oncologist, oncology fellow,housestaff, and nurses each loudly pro-claimed a “solution” to the problem. Un-

fortunately, these solutions ran thegamut from no change (“he’s dying any-way”) to switching him abruptly to oralacetaminophen with codeine, and theyreflected the general feeling of helpless-ness that all of us were experiencing.Nevertheless, we had to prepare MS fortransfer to the rehabilitation facility atwhich he could not receive parenteralopiates. I spent a significant amount oftime explaining to the patient that thecombination therapy of oral short-actingand long-acting opiates would provideequal pain control but without the“peaks and valleys.” His response wasthat he would “die” if anything werechanged. I will never forget his tears andbegging during this time.

The next time we met was when Iwas acting as the palliative care consul-tant after his attending thought that fur-ther anticancer treatments were unlikelyto help. He was immediately transferredto the Palliative Care Unit where thelevel of conflict among our team quicklyescalated. The two polarities—let himbe, give him whatever he wants, he’s dy-ing versus he deserves the same oppor-tunities of optimal symptom control, clo-sure, maturation, and dignity that weoffer to all our adult palliative care pa-tients—split us as individuals and as ateam. The staff nurses, particularly, weredistressed over his unconventional andsuspicious need for receiving intrave-nous hydromorphone and diphenhy-dramine every 2 hours, which led to al-most continuous sleeping. Suggestionsfor placebo administration led to agoniz-ing ethical debates.

As a team, we were able to transformmultiple diverging opinions, which weresubtended by shared feelings of sadness,empathy, and frustration, into a mutuallyagreed upon plan of care. With the helpof more experienced team members, webegan to appreciate the many layers anddimensions of MS’s pain. We could un-derstand how his basic and normal hu-man and teenage needs at the end of lifewere being suppressed and thwarted bythe intravenous regimen. Through ourexplanation, MS began to feel that byswitching to a simpler oral regimen, hewas lessening his mother’s burden.When MS left the hospital this time,there was anxiety, but no begging ortears.

As a physician with considerableadult oncology and palliative care expe-rience, but no background in or desire to

practice in pediatrics, I initially felt help-less when confronted with MS’s situa-tion. Only an interdisciplinary approachcould allow me to rediscover my capac-ity both to understand a child’s sufferingand to participate in providing effectivepalliative care to MS, his family, and ourteam.

James Brandman, MD

Attending Physician

Northwestern University

Medical School

Division of Hematology-Oncology

Palliative Care and

Home Hospice Program

Northwestern Memorial Hospital

Chicago, Illinois

Response 2

There were several significant and chal-lenging issues involved in this case. All ofthese affected the way MS’s pain wasmanaged. First, although MS was 15years old when he presented to ourteam, developmentally he was muchyounger. Developmental delays are notuncommon in children with life-threatening illnesses, as they may resultfrom treatment effects as well as changesin role expectations as a consequence offamily members protecting the childfrom perceived harm.1 Our team on thePalliative Care Unit had little experiencein caring for children, and conflicts aroseregarding the appropriate direction ofcare that should be provided. Statementssuggesting countertransference werecommon. Some team members were see-ing their own child in MS and got overlyinvolved. As a result, some staff boughtpizzas, ice cream, video games, andother gifts whenever the patient re-quested these.

Other team members believed thatchildren need boundaries to providesupport, and that this ultimately makesthe child feel more secure. These teammembers believed MS should be encour-aged out of his isolation by having himplay with video games in the PalliativeCare Unit’s family room rather thanalone in his hospital room. It wasthought that the real meaning behind hisexcessive use of the call light was hisunspoken plea for aid in helping him ver-balize his feelings. Handing MS a newtreat was perhaps the easy way out ofpacifying him to be a “good patient” anddid not address his underlying need toverbalize his feelings.

Multidisciplinary Rounds S5

In my role as a social worker on theteam, I needed to support the staff sothat we could provide optimal care toMS and his family. I worked with teammembers to address the countertransfer-ence, first by identifying the origin ofsome of these feelings.2 Some felt terri-bly guilty that this was an African Ameri-can patient living in poverty, who wasfeeling horrible pain and suffering. Therewas even anger and frustration at thefamily and at ourselves that he was achild on an adult unit and that we did notknow how best to care for him. I re-framed the issue of the child being in ourhospital. His mom was being a strongadvocate for him. She believed his carewas better here than at the children’shospital. We invited a child life specialistwho had expertise in the care of chil-dren with cancer to speak to our staff.This outside expert described childhooddevelopment and how this is often af-fected by illness. Educating the staff thatterminally ill children typically remain fu-ture-oriented, unlike adults who will of-ten reflect back on their lives, was help-ful. This expert also listened to staffconcerns, acknowledged the difficulty incaring for children with life-threateningillnesses, and provided specific strategiesthat could be implemented.

I worked with team members to ad-dress their motivation for gift giving. Wasthis to enhance the patient’s coping andstrengthen his support systems? Orrather, was the gift giving done to ad-dress their own feelings of helplessnessand guilt about the patient’s illness? Itwas also observed that gift giving wasbeginning to play a major role of distract-ing MS and his family from facing thetask of dealing with this terminal disease.Both the patient and the family werepleading with the staff to purchase itemsthat were both very expensive and diffi-cult to find. The patient’s mother wasadamant that her son not be told of histerminal diagnosis out of fear that hewould lose all sense of hope, yet MS’sbehavior indicated that he really didknow what was happening. MS partici-pated in his mother’s need to hide thisinformation from him by insisting thatthe staff only speak with his motherabout what was happening and not in-clude him in the conversation. Instead ofhelping MS learn to cope with his unspo-ken knowledge of his terminal illness,the mother would shift her concern onthe next new toy he needed. What

worked was having an outside agencyset clear parameters on gifts they couldprovide and this allowed our unit to fo-cus on all aspects of his care.

Another major focus of my interven-tions was directed toward relieving MS’spain, which had me trying several cogni-tive-behavioral techniques. The patienthad a low frustration tolerance, andguided relaxation exercises were ineffec-tive. Distraction in the form of videogames was successful, and it took effortto gain his trust so that he would becomemotivated to allow other forms of dis-traction, including humor, art, and con-versations. I would encourage him totalk about his favorite holidays and todescribe these in detail. One of thenurses, whom he accepted as a motherfigure, read to him each night as he waslosing his vision. (This bond grew to thepoint where MS was even able to discusshis feelings and cry.) During these peri-ods, his requests for pain medicationsand reports of pain diminished.

The third major focus of my interven-tions was MS’s mother. When MS was inthe hospital, he would receive signifi-cant doses of opioids and other medica-tions. While at home, these doses dimin-ished greatly, the patient would haveescalating pain, and he would need to bereadmitted to the hospital. Through dis-cussions with MS’s mother, it became ap-parent that they were withholding painmedications, not because of fears of ad-diction or tolerance, but rather becauseMS would frequently dream of friendsand family members who were dead.These dreams had been attributed byone of the physicians to the pain medi-cines and were disturbing to MS’smother. I educated the patient’s motherabout the role of dreams and on the factthat perhaps MS was beginning to pre-pare himself for his death. It was begin-ning to become apparent to me that MS’sprotective stance for his mother partlystemmed from his need to support hismother’s need to protect him. My con-versations with his mother later ledto her realization that she needed tostart emotionally preparing MS for hisdeath.

James Cappleman, LCSW

Clinical Social Worker

Palliative Care and

Home Hospice Program

Northwestern Memorial Hospital

Chicago, Illinois

Response 3

This case represents so clearly that painis a sensory and emotional experienceand that the relief of suffering is as cru-cial as the pharmacologic managementof pain.3 MS had obvious reasons for sig-nificant pain, including a tumor affectingthe entire right sinus, palate, and oro-pharynx, as well as multiple, diffusebone metastases. However, emotionalfactors, including existential distress,fear, uncertainty, and a need to protecthis mother, contributed equally to theoverall pain phenomenon. As an ad-vanced-practice nurse pain specialist, mygoal was to provide the optimal pharma-cologic and nonpharmacologic manage-ment of the patient’s pain, while assist-ing the team in understanding the originof some of the pain-related behaviorsthat we all found so troubling. This wasparticularly challenging for me in that Ihave no pediatric experience.

This African American patient’s fre-quent requests for pain medication, andthe method of delivery (injected fast andinto the central line), reminded some ofthe team of the behavior of patients withsickle cell disease. The conflict was be-tween our beliefs that this was a poor,dying child with a devastating cancerand the concern that his behaviors sug-gested some hidden motive. This sce-nario illustrates the concept of pseudo-addiction, where behaviors suggestive ofuncontrolled pain and fear (eg, demand-ing behavior, “clock-watching”, andseeking additional medications) are mis-interpreted as addiction.4 MS was at highrisk for undertreatment as a young Afri-can American man coming from a house-hold of poverty and family memberswho had substance use histories.5 Hehad likely been treated with suspicionregarding opioid requests at some pointduring his illness. Furthermore, it wasclear that MS was attempting to treat hisexistential distress with opioids. Weworked together as a team to identifyour discomfort with MS’s responses andto understand the concept of pseudoad-diction. The nurses on the inpatient unitworked hard to provide the medicationon a regular basis, injecting the drug asthe patient requested and in a nonjudg-mental fashion. This helped to establishtrust so that MS could eventually acceptchanges in the regimen.

Clearly, intermittent hydromorphoneadministration provided inconsistent re-

S6 CANCER PRACTICE May/June 2002, Vol. 10, Suppl. 1

lief and would have been a significantburden for MS’s mother to administeronce he returned home. In evaluatinglong-acting oral medication options,methadone was considered to be a use-ful alternative. In addition to binding toopioid receptors, methadone also is be-lieved to act as an N-methyl-D-aspartateantagonist.6 The advantage of this actionis in relieving neuropathic pain. MS hadsignificant burning in the facial area andin the palate. Furthermore, methadone isinexpensive, which was a considerableconcern because the family had not yetdecided on hospice care. However, MSand his parents had major concernsabout the use of methadone, a drug withsignificant stigma in their community.The decision was made to convert thetherapy with hydromorphone to a long-acting oral morphine, 400 mg orally ev-ery 12 hours, with immediate-releasemorphine, 60 to 90 mg every hour asneeded. This titration occurred gradu-ally, with some intermittent injections ofhydromorphone allowed during the firstfew days. The purpose of the gradualconversion was to build trust with thepatient that oral medications would beeffective.

Other pharmacologic therapies in-cluded enhancing adjuvant analgesics.The patient was being treated with dexa-methasone, and the dose was increasedto 8 mg daily; omeprazole, 20 mg, wasadded to provide ulcer prophylaxis.Therapy with gabapentin, 100 mg threetimes daily, was begun for neuropathicpain symptoms.7 Alprazolam, 0.25 mg,was administered twice daily to treatanxiety. Two tablets of a laxative soft-ener were given twice daily to preventconstipation, and this was titrated tothree tablets twice daily. Lactulose wasadded later. A saline nasal spray was ad-ministered several times during the dayto cleanse and soothe the nasal area andsinuses.

During the time that MS was in homehospice, his pain was generally wellmanaged, as he received two to threedoses of immediate-release morphine, 90mg, usually at night. Because he com-plained of more pain at night, the long-acting morphine dose was changed to400 mg each morning and 600 mg eachnight. When questioned, MS usually re-ported having no pain to mild pain. Afterreadmission to the Palliative Care Unit,the therapy with oral opioids was con-verted to a continuous intravenous infu-

sion because MS could no longer swal-low. Using a standard equianalgesictable, the dose was calculated to be mor-phine, 20 mg/h, with bolus doses of 10mg (50–100% of the hourly rate) every15 minutes as needed. The patientwould have intermittent periods of cry-ing out, but he denied the presence ofpain. He told his mother “I have got togo,” and he died later that day.

Judith A. Paice, PhD, RN

Research Professor of Medicine

Northwestern University

Medical School

Division of Hematology-Oncology

Palliative Care and

Home Hospice Program

Northwestern Memorial Hospital

Chicago, Illinois

Response 4

My first visit with MS was only 3 weeksbefore his death, although chaplainsfrom the inpatient setting had workedwith him and his family while he hadbeen hospitalized. As so often happens,the admission to hospice was delayed be-cause of fears that treatment would bewithheld and the hope that his prognosiswas not so grave. My spiritual assessmentbegan by talking with the family and ob-serving their interactions with MS andwith each other. Neither parent worked,and several of the older children wereinvolved in gang activity. According toMS’s mother, he was the “good” son, at-tending school, staying out of trouble,helping his mother in the home, andfinding diversion primarily in videogames. The home was an apartment in acity-subsidized development wherecrime was high, roaches were prevalent,and only one bed was available for sleep-ing. There were clear financial deficitsand limits in their coping mechanisms.However, MS’s mother and father bothobviously loved their son and had suf-fered greatly as a result of his illness.

My assessment identified a strongnonphysical component to the patient’spain. In part, MS’s reports of pain werethe only way he knew to give voice tohis underlying emotional and spiritualpain. His need to feel safe and lovedmanifested itself somatically, as is oftenthe case in cultures in which displays ofemotions are discouraged or when thereare scant resources to meet the emo-tional needs of all. Clearly MS had physi-

cal pain, but when he was scared or sawhis parents distressed, his complaints ofpain and requests to return to the hospi-tal increased. At the hospital he felt thesafest, and he felt his parents would beprotected.

MS’s physical pain, in turn, causedacute spiritual pain. Both the patient andhis family asked why God would punishan innocent child by making him suffer.MS would ask periodically, “Wasn’t Igood? What did I do wrong?” His motherwould often shift the blame to herself, acommon response of parents with chil-dren who have life-threatening illnesses.(She was convinced she had caused bothhis seizure disorder and his cranial tu-mors by dropping him on his head as ababy.) The family prayed for a miracu-lous healing, and when that was notforthcoming, their faith faltered. Attimes, they felt utterly abandoned byGod.

In part because of the patient’s dete-riorating condition, but also because herneeds were greatest, my care was pri-marily directed to MS’s mother. MS’smother was his primary caregiver in thehome and would rarely let others assist.The hope was that, by relieving her suf-fering, she could better address both thespiritual and physical pain of her son.And if MS knew that someone was caringfor his mom, he would feel safe. Despiteher spiritual crisis, I was able to win hertrust quickly through my role as a pastor.This is a role held in high regard in theAfrican American community. Further-more, I made myself an ally, not chal-lenging her decision not to tell her sonhis prognosis. She was protective of him,especially being reluctant to disclose tohim what was happening, although itwas clear to the team that he was awareat some level of his prognosis.

My pain-related interventions in-cluded affirming that she was doing ev-erything possible, encouraging her roleas an advocate for her son, and support-ing her presence with him (somethingthat had been very difficult for her). Iencouraged her to cuddle him and to useprayer to calm him. When MS felt safeand calm, his pain diminished and herspiritual suffering lessened. Yet, shewanted to remain totally connected toMS and would withhold pain medica-tions in the belief that they were makinghim sedated. At times she would awakenhim from a restful, pain-free sleep so shecould hear his voice and know he was

Multidisciplinary Rounds S7

alive. With intensive grief and spiritualcounseling, she came to believe thatmother and son shared a primal, spiritualbond that allowed for communicationand connection even if MS was unable tospeak. Over time, I suggested that amother’s love is bigger than her need tohave him awake (yet in pain). At the end,she was willing to allow MS to receivepain medications.

When MS’s mother lost her faith, Iallowed her to express her feeling of in-justice, senselessness, and abandonment.I reassured her that God does not sendsuffering nor punish a child for a moth-er’s failings. Through words and prayersand by mediating divine care in my roleas a pastor, I offered an alternative expe-rience of a loving and present God. MS’smother needed to believe in God and inHeaven so that she could be reunitedwith MS again in another life. She oftencalled him “her little angel”. Her use ofprayer with MS, and his calmness as aresult of better pain control, slowlyhelped to rebuild her faith. Before MSdied, she was able to tell him his prog-

nosis, albeit indirectly, by reading sec-tions from scripture about the promiseof eternal life. I had the honor of offici-ating at MS’s funeral, and I continue towork with his mother 9 months afterMS’s death. She is making slow but sig-nificant progress in rebuilding her faithin God and in herself.

M. Jeanne Wirpsa, MA

Chaplain/Counselor

Palliative Care and

Home Hospice Program

Northwestern Memorial Hospital

Chicago, Illinois

References

1. Hinds PS, Oakes L, Furman W. End-of-life decision making in pediatric oncol-ogy. In: Ferrell BR, Coyle N, eds. Text-

book of Palliative Nursing. New York,NY: Oxford University Press; 2001:450–460.

2. Wellisch DK. Family issues and pallia-tive care. In: Chochinov HM, BreitbartW, eds. Handbook of Psychiatry in

Palliative Medicine. New York, NY:Oxford University Press; 2000:275–289.

3. Cassell EJ. The nature of suffering andthe goals of medicine. N Engl J Med.

1982;306:639–645.4. Weissman DE, Haddox JD. Opioid

pseudoaddiction: an iatrogenic syn-drome. Pain. 1989;36:363–366.

5. Cleeland CS, Gonin R, Hatfield AK, etal. Pain and its treatment in outpatientswith metastatic cancer. N Engl J Med

1994;330:592–596.6. Davis MP, Walsh D. Methadone for re-

lief of cancer pain: a review of phar-macokinetics, pharmacodynamics,drug interactions and protocols for ad-ministration. Support Care Cancer.

2001:9:73–83.7. Nicholson B. Gabapentin use in neuro-

pathic pain syndromes. Acta Neurol

Scand. 2000;101:359–371.

The Department Editors encourage thesubmission of your challenging case studies toCancer Practice. Your contributions continueto make Multidisciplinary Rounds a valuableresource for the entire oncology team. Sendemail to journals@cancer.org

S8 CANCER PRACTICE May/June 2002, Vol. 10, Suppl. 1