SURVIVING CATASTROPHIC PEDIATRIC ILLNESSES
From the 50th Annual Scientific Assembly of the American Academy of Family Physicians, October 3-6, 2007,
Chicago, IL
| INTRODUCTION —Edward G. Zurad, MD, Clinical Assistant Professor of Family Medicine, Temple University
School of Medicine, Philadelphia, PA
|
| Origin of topic: article by KC Oeffinger in New England Journal of Medicine indicating many survivors of pediatric
catastrophic illnesses not well followed and often develop late effects of illness or other serious medical problems
|
| Big picture: >10 million cancer survivors in United States; nearly 300,000 of those survivors diagnosed with cancer at
<21 yr of age; ≈80% of children who develop cancer now survive ≥5 yr after diagnosis (many more children with serious
neonatal problems and infectious diseases also now surviving); many cancer survivors particularly vulnerable to
other lifelong problems
|
| Patient who developed neuroblastoma as child: underwent bone marrow transplantation; when seen by speaker
at 26 yr of age was 4’11”, weighed 90 lb, had no health insurance, could not sustain job due to lung and cardiac problems,
and presented with osteonecrotic disorder (ie, all teeth falling out) and chronic severe pain
|
| Pertinent points: majority of survivors of catastrophic pediatric illnesses seek family physicians for continuing care;
pediatric cancer survivors 8 times more likely than siblings to develop potentially life-threatening or disabling conditions;
75% of pediatric cancer survivors report ≥1 serious health conditions if they live 30 yr after cancer diagnosis
|
| Components of care for pediatric cancer survivors: screen for secondary cancer, cardiovascular and pulmonary
disease, and renal dysfunction; family physician must—develop sensitivity for survivors; deal with fertility issues,
body image concerns, and depression; look for breast cancer in patients who have undergone central body
irradiation; check for cardiomyopathies and osteoporosis; identify those not receiving adequate follow-up care and focus
on their needs
|
| Evidence-based recommendations: adult survivors of childhood cancer should be followed closely by family physicians,
since one-third of them suffer from psychologic problems; survivors 3 times more likely to develop chronic health
conditions than their siblings; childrensoncologygroup.org good source for guidelines for long-term follow-up of survivors
of cancer and other catastrophic childhood illnesses
|
| Issues to consider in pediatric cancer survivors: emotional and educational issues; educational problems include
1) 10 to 20 point drop in IQ, 2) attention-deficit/hyperactivity disorder, and 3) difficulties with science and
mathematics courses; difficulties in finding and paying for health care; diet and exercise; eye problems (eg, early-onset
glaucoma or cataracts); hearing problems (eg, high-frequency loss); growth hormone deficiency (common); central
adrenal insufficiency (big issue); hyperprolactinemia; hypopituitarism; osteonecrosis (significant problem in people
who underwent head and neck irradiation; can lead to dry mouth and loss of teeth); thyroid disorders (eg, hyperthyroidism,
hypothyroidism); cardiac disorders (eg, congestive heart failure, cardiomyopathy in patients who received
anthracyclines); pulmonary problems (patients treated with bleomycin vulnerable to early-onset pulmonary fibrosis);
liver disorders (eg, steatosis, fibrosis, cirrhosis); splenic loss (appropriate immunizations indicated); kidney and bladder
problems (especially for those with neurologic-based cancers); infertility; neuropathy (may lead to proprioception
problems and inability to maintain job); chronic pain; Raynaud’s phenomenon; secondary cancers (especially for patients
who underwent central irradiation)
|
| Observations: childhood cancer survivors require close long-term follow-up care from physicians “who know what
these patients have been through and what kinds of complications to look for in the future”; “complications demand
level of knowledge that is beyond the range of the general internist or family doctor” wrote PM Rosoff in New England
Journal of Medicine editorial accompanying Oeffinger article; however, Dr. Rosoff failed to realize that family
physicians provide care for majority of geriatric patients with multiple medical problems and that they “are
masters of concurrent priorities and can manage these things”
|
| SURVIOR OF STAPHYLOCOCCAL EPIDEMIC IN NURSERY —Andrea V. Phillips, Lancaster, PA
|
| Speaker’s story: birth 50 yr ago perfectly normal; exposed to infections (ie, Staphylococcus, Salmonella, paratyphoid
fever) in nursery; mother suspected something wrong at time infant discharged from nursery because infant unable
to move left side of body and had erythema at joints; family physician felt there was no major problem, and
simply recommended formula changes; hospital called 3 wk after birth to advise that 2 babies in nursery had died; by
that time speaker’s infection had spread on left side of body from shoulder to hip and spinal cord; parents told she
might not survive; when she did survive, parents told she would be crippled for life and require braces
|
| Follow-up care: involved series of operations (starting at 3 wk of age) and fitting of leg brace; through church connections,
parents met Washington, DC, physician who provided considerable hope and performed first operation (including
bone graft) on patient at 3.5 yr of age; by 4 yr of age, she was able to walk independently; at 7 yr of age,
surgery done to stunt growth of right (“good”) leg, to allow left leg to catch up; for several years patient wore special
lift shoes on left foot; at 15 yr of age patient able to wear regular shoes for first time
|
| School years: elementary school—very difficult; while parents “always encouraged me, they never pitied me;” she
was constantly made fun of and shunned by most classmates; became good friends with black girl who also was rejected
by classmates; middle school—body image big issue; teachers and guidance counselors failed to understand
that physical disabilities do not necessarily equate to intellectual impairment, and tried to discourage her from preparing
for college; she was placed in classes with “average” students and found it frustrating and boring; parents remained
her advocates, and she eventually took series of tests to prove she had intellectual ability to go to college; high
school years—body image remained big issue; one of her very close friends developed cancer and died at 21 yr of
age; her father died from massive myocardial infarction when she was in 11th grade; patient met her future husband in
senior year and finally came to realization that people had to accept her for who she was and she had had to accept herself
|
| Marriage: resides in home with elevator; despite previosly being told she could never have children, she gave birth to
3 healthy children; first delivery vaginal, but there were concerns about possible infection; second conception induced
by infertility therapy and resulted in twins; fertility process difficult experience; she eventually had to explain to children
why one of her arms shorter than other
|
| Message to physicians: be more concerned about “whole picture” of patient, including psychologic issues; inquire
about problems at school and body image issues and offer support
|
| HUSBAND’S PERSPECTIVE —Spencer Phillips, MD, Private Practice, Lancaster, PA
|
| Opening remarks: one has to avoid certain activities (eg, roller skating, camping) with handicapped spouse; wife had
first “modern” hip replacement in 1981, while he was nearing completion of medical school; following surgery, she
was in bed 1 mo with no weight bearing allowed; during recovery she developed pulmonary embolus; recovery period
included specialized physical therapy and use of walker with built-up cane
|
| Pregnancy issues: unable to get pregnant for first 6 yr of marriage; although first delivery vaginal, speaker made sure
option of cesarean delivery available on same hospital floor; second pregnancy, utilizing menotropins (Pergonal) and
terbutaline injections, gamete intrafallopian transfer and reinjection of eggs, quite difficult (including premature labor
3 mo before delivery date), but resulted in twins
|
| Ongoing concerns: risk for infection; Staphylococcus present on one of blood cultures during second hip operation,
necessitating intravenous (IV) antibiotics for ≈1 mo; numbness and chronic pain in left arm
|
| What family physicians can do: once patient diagnosed with major problem, strive to be that patient’s lifelong
health care provider and lifelong friend
|
| Issue of medical records in survivors of catastrophic pediatric illness (Dr. Zurad): do not discard
records when 7 yr elapse or patient reaches adult status; those records can be valuable later in life
|
| SURVIVOR OF PINEAL GERMINOMA DURING TEENS —Matthew B. Pfenninger, Midland, MI
|
| Summation of speaker’s story: treated for brain cancer (pineal germinoma) that recurred twice; underwent bone
marrow transplantation, resulting in multiple complications
|
| Observations: all people who go through difficult illness require case manager; case managers require skills to
communicate effectively with patient and physicians; physicians need “to apologize and seek forgiveness” for mistakes;
family physicians in best position to fight for patients and be their advocates
|
| What physician-father did: extensively researched type of cancer his son had and explored cutting-edge technologies
to treat it; assisted in logistical issues to help son, including enrolling him on Medicaid when $2 million insurance
coverage used up; suggested that son save sperm before treatment
|
| Role of family physicians: act as case managers and fight as advocates for their cancer patients because they are
in best position to treat “whole patient”; physicians deal with insurance companies almost every day, whereas most
patients rarely know how to deal with them; physicians can provide map for patients to guide them through their
journey; speaker and parents clueless at times as to what was going on in his care; patients look to physicians for
hope and clarity; family physicians must interpret results of specialists for their patients; communication not just
talking and explaining, but also listening to patients and understanding their needs and fears; speaker was more
scared of living with major defects or disabilities (eg, reduced intelligence, double vision, raspy voice) than of dying;
role of physician to help patients “through the whole thing, not just treating them for disease”
|
| Speaker’s treatment experience: hospitalized for 7 mo, weak and delirious most of time; physicians communicated
well with parents
|
| What patient requires: interpreter to explain what is being done; physician that patient can trust and communicate
with; remarks—speaker considers self symbol of hope and believes in miracles, but also realizes many things improvable
|
| Issues involving physicians: can make mistakes—neurosur-geon forgot to test cerebrospinal fluid [CSF] for tumor
markers; another physician forgot to adequately tighten G-tube after inserting, and speaker developed peritonitis and
intestinal adhesions, and nearly died; in removing lung tumor, thoracic surgeon caused vascular problem in right lung
that was discovered few days later, necessitating removal of upper lobe of right lung; failure to provide adequate and
truthful information—neurosurgeon said he had checked CSF, even though he had not, and told patient CSF negative
for tumor markers; ability to admit mistakes and apologize—thoracic surgeon apologized immediately after realizing
he made mistake; “you guys were trained to be perfect, but you are not it takes a special person to admit it”; admission
by physician that he or she cannot do it all—physicians also have own personal life issues that require
attention
|
| Concluding comment: “physicians are super human, have special powers, and the power to heal—use it”
|
| FATHER’S PERSPECTIVE —John L. Pfenninger, MD, President, National Procedures Institute, Midland
|
| What son did not say: in hospital 1.5 yr over 3-yr period, last time for 7 mo; during course of hospitalization, father
received 5 calls indicating son would not make it through night
|
| Points: physicians have no idea of power they have and its importance; essential for primary care physician to help coordinate
care for patients in hospital
|
 | Regarding son’s case: no one to coordinate care; over 3-yr period, son had 4 family physicians; comments—case
manger very important; family physicians should be case managers; physicians must look at entire patient; give patients
hope, and tap into all available resources to help them (eg, drugs, x-rays, surgery, prayer, saving semen [in
this case, son’s semen lacked sperm])
|
| Woman with ovarian cancer: stated on national television that her oncologist would not help her save her eggs;
physician claimed her business was only to treat problem, not whole patient; woman took necessary steps and eventually
conceived on own; points—1) family physicians have to take care of whole person and family (ask patients how
they feel; “look at the whole family because they are hurting”); 2) physicians can have problems too (ask colleagues,
“how are you doing? how do you feel? anything I can do to help out?”); 3) physicians must be concerned about future
|
| CLOSING COMMENTS —Dr. Zurad
|
| Responsibilities of primary care physicians: 1) include self in at least some of initial treatment decisions; 2) procure
appropriate follow-up schedule from oncologist or other specialist when treatment completed; 3) provide continuity
of care; 4) attempt to ascertain what looms ahead for those who have had significant illnesses; 5) watch for late
untoward effects and utilize continuity of care to full advantage; 6) treat beyond cure; 7) remember “big picture” for
remainder of patient’s life
|
Suggested Reading
Ariz NM et al: Comprehensive long-term follow-up programs for pediatric cancer survivors. Cancer 107:841, 2006;
Bertini G et al: Staphylococcus aureus epidemic in neonatal nursery: a strategy of infection control. Eur J Pediatr
165:530, 2006; Bratu S et al: Community-associated methicillin-resistant Staphylococcus aureus in hospital nursery
and maternity units. Emerg Infect Dis 1:808, 2005; Cimolai N: Staphylococcus aureus outbreaks among newborns:
new frontiers in an old dilemma. Am J Perinatol 20:125, 2003; Denmark-Wahnefried W et al: Survivors of childhood
cancer and their guardians. Cancer 103:2171, 2005; Hudson MM et al: Health status of adult long-term survivors
of childhood cancer: a report from the Childhood Cancer Survivor Study. JAMA 290:1583, 2003; Ness KK et al:
Limitations on physical performance and daily activities among long-term survivors of childhood cancer. Ann Intern
Med 143:639, 2005; Nguyen DM et al: Risk factors for neonatal methicillin-resistant Staphylococcus aureus infection
in well-infant nursery. Infect Control Hosp Epidemiol 28:406, 2007; Oeffinger KC et al: Chronic health conditions
in adult survivors of childhood cancer. N Engl J Med 355:1572, 2006; Oeffinger KC et al: Health care of
young adult survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. Ann Fam Med 2:61,
2004; Oeffinger KC et al: Programs for adult survivors of childhood cancer. J Clin Oncol 16:2864, 1998; Oeffinger
KC, Hudson MM: Long-term complications following childhood and adolescent cancer: foundations for providing
risk-based health care for survivors. CA Cancer J Clin 54:2089, 2004; Oeffinger KC, Robison LL: Childhood
cancer survivors, late effects, and a new model for understanding survivorship. JAMA 297:2762, 2007; Oeffinger KC:
Childhood cancer survivors and primary care physicians. J Fam Pract 49:689, 2000; Oeffinger KC: Longitudinal
risk-based health care for adult survivors of childhood cancer. Curr Probl Cancer 27:143, 2003; Ressler IB et al:
Continued parenteral attendance at a clinic for adult survivors of childhood cancer. J Pediatr Hematol Oncol 25:868,
2003; Rosoff PM et al: Response rates to a mailed survey targeting childhood cancer survivors: a comparison of conditional
versus unconditional incentives. Cancer Epidemiol Biomarkers Prev 14:1330, 2005; Rosoff PM: The two-
edged sword of curing childhood cancer. N Engl J Med 355:1522, 2006; Sunga AY et al: Care of cancer survivors.
Am Fam Physician 71:699, 2005; Van Howe RS, Robson WL: The possible role of circumcision in newborn outbreaks
of community-associated methicillin-resistant Staphylococcus aureus. Clin Pediatr 46:356, 2007; Yeazel MW
et al: The cancer screening practices of adult survivors of childhood cancer: a report from the Childhood Cancer Survivor
Study. Cancer 100:631, 2004; Zebrack BJ et al: Health care for childhood cancer survivors: insights and perspectives
from a Delphi panel of young adult survivors of childhood cancer. Cancer 100:843, 2004.
Educational Objectives
| The goal of this program is to improve the detection and management of adverse consequences that survivors of catastrophic
pediatric illnesses may encounter. After hearing and assimilating this program, the clinician will be better able
to:
|
| 1. Screen for the development of secondary cancers and other serious medical problems in pediatric patients who
have had cancer.
|
| 2. Provide life-long care for survivors of life-threatening infections during their pediatric years.
|
| 3. Realize that the majority of survivors of severe pediatric illnesses are not receiving appropriate follow-up care.
|
| 4. Serve as a case manager for patients with serious illnesses, particularly cancer.
|
| 5. Develop sensitivity for survivors of catastrophic illnesses who may have fertility issues, body image concerns,
and depression.
|
Faculty Disclosure
In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and planning committee
members to disclose relevant financial relationships within the past 12 months that might create any personal conflicts of
interest. Any identified conflicts were resolved to ensure that this educational activity promotes quality in health care
and not a proprietary business or commercial interest. For this program, the following has been disclosed: Dr. Pfenninger
is president and owner of the National Procedures Institute. Drs. Zurad and Phillips, Ms. Phillips, Mr. Pfenninger, and
the planning committee reported nothing to disclose.
Acknowledgements
All speakers were recorded on October 3, 2007, at the 50th Annual Scientific Assembly of the American Academy of
Family Physicians in Chicago, IL. The Audio-Digest Foundation thanks the speakers and the Academy for their cooperation
in the production of this program.
|
