Social Work Perspective

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Social

Work

Perspective

SUPPLEMENT 903

Sandra

Hernandez,

MSU

From the Department of Pediatrics, St Lukes-Roosevelt Hospital, New York

The ultimate objective of newborn screening for sickle cell disease should be twofold. The first es-sential step is the identification of the infants at risk. This has been effectively done in New York state as of 1975 through the New York State New-born Screening Program. However, identifying these children is not enough. Second is the much more complicated task of providing comprehensive follow-up care for families whose children are af-fected by the disease, including the much needed psychosocial services. This area continues to be sorely neglected.

The increased risk of death due to overwhelming

infection in the first 3 years oflife for children with

sickle cell disease has been noted in the literature.

When there is no specialized care, 15% to 20% do

not survive. Therefore, it is essential for

knowl-edgeable staff to make contact and begin to develop

a trusting relationship as soon as possible with

parents of infants born with sickle cell disease.

Prophylactic penicillin and pneumococcal

vaccina-tion can reduce mortality during the early years.

Family involvement with a consistent, available

team of health care providers is pivotal in

under-standing this chronic illness and coping effectively

with this extraordinary stress. Our staff is available

by telephone for consultations with patients or other medical staff during clinic and emergency

room visits and hospitalizations.

One element that is clear in our experience at the

St Luke’s-Roosevelt Hospital Sickle Cell Center in New York City is that adjustment to this chronic illness is a lifelong process. As with any process, time is essential. One or two counseling sessions at

the time of diagnosis are not sufficient to enable

families to fully understand the information given

or to realize the impact of having a child with a

chronic illness. We see the infants every 2 months

for the first 6 months and every 3 months after

until 2 years of age. We provide well-baby care as

well as follow-up for sickle cell disease. After 2 years of age, visits vary from every 3 to 6 months, depending on the need. At each visit, staff inquires about the baby’s progress and the family’s adjust-ment. Concerns are explored, anxieties are allayed, and practical advice is given. From the initial visit, the process of education about the disease begins.

The initial invisibility of the disease encourages

the denial that is the normal human reaction to

psychologic trauma. The baby looks fine, so how

can he or she be sick? The variability of the disease

in sickle cell anemia augments the problem of de-nial and coping. It is impossible to predict when or

how the disease will show itself or if it will show

itself the same way all the time in the same patient. These uncertainties make preparation for handling the critical first episodes difficult at best. Concep-tualizing or believing what is not seen is often

difficult for some medical personnel, ie, believing

there is pain with no objective signs in a crisis. One

can imagine the difficulty some parents may have

believing their child is ill before the first episode

occurs. By providing information gradually, we try

to prepare the parent for the first crisis or infection. Parents are informed of the risks of infection for

children with sickle cell disease. They are asked to

look for changes in the infant’s behavior, loss of

appetite, or fever. They know to obtain immediate

attention at the first signs of infection. This

mes-sage, at times, needs to be reemphasized. For

ex-ample, the mother of a 19-month-old baby informed

the clerk at our emergency room registration desk

that her daughter had sickle cell disease. At this initial visit, this information did not afford her the priority treatment customary for patients with

sic-kle cell disease in the emergency room. On her

second visit to emergency room, she neglected to

tell the pediatrician the child had sickle cell disease.

The pediatrician examined the child, who had a

temperature of 39.3#{176}C(102.8#{176}F), prescribed

anti-biotics for an ear infection and was about to dis-charge the child. The mother then informed the

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904 DISEASE AND CARE

doctor that the child’s regular care was provided by

our center. The doctor immediately admitted the

child to the hospital. Our center staff needed to reemphasize to their mother that, regardless of whether the clerk realized the need for certain

precautions with her child, she needed to be aware

and insist on priority treatment for her daughter. Sharing the child’s diagnosis with the doctor is

essential. The two children who died in 1986 were

in their usual state of health six hours before their

death from overwhelming sepsis. We cannot afford

the risk of silence or ignorance.

Parental vigilance’ is needed without

overprotec-tion. Not every fever, infection, ache, and pain is sickle cell related. Sickle cell disease should not become the focus of the patient’s life. Children will

experience common medical problems during their

growing years. However, these parents need to be

alert more often than parents whose children do not have sickle cell disease. Striking a balance in

each family in this area is an individual task and is

usually accomplished by trial and error. Some as-sistance from the health care team can facilitate

this process. They can help the parent sort out

when to treat a child at home and when to bring the child to the hospital. They can set guidelines

for parents to experiment with or when to

appro-priately allow dependency in their children and

when to encourage self-reliance. One of our adoles-cents wanted to get an after-school job. The mother

needed support in sorting out which concerns were

appropriate, eg, the type ofjob, and which concerns

were not, eg, her son is sick and should not work.

The boy, who is severely anemic and has a

chroni-cally enlarged liver, also needed help in accepting sensible limits on the job while still encouraging his desire to experiment and master an area of

com-petency essential for healthy development.

Healthy coping patterns can be taught to parents

early and eventually passed on to the children.2 In

treating approximately 100 adult patients in the past 4 years previously treated at other medical clinics, our staff is continuously amazed at the misconceptions adult patients have about their dis-ease. We are frustrated at the maladaptive coping patterns developed by some of those patients.

First hospitalizations are usually the first real encounters for parents of the realities of their child’s chronic illness. Support from and

availabil-ity of the team is of paramount importance. Fre-quently, it is the family’s first encounter with the

medical system outside the sickle cell center staff.

The encounter may be with medical staff members

who are not as knowledgeable or as sensitive as the

center staff. This adds additional pressure on

par-ents and children. But this is often the beginning of a realization of what the disease is about and of

its acceptance. Feelings of inadequacy, guilt, and

anger are raised in parents and staff. Fears of

abandonment and the beginning experiences with

pain, confusion, and helplessness are stirred up in

children. At this time, parents need support,

clan-fication of the medical situation, and advocacy.3

Being able to relate to medical staff that is trusted

and has been available before the first

hospitaliza-tion can make the first major illness bearable. It

can also afford the health team the opportunity to

further bond with the family. Our center staff

knows our patients in times of health as well as in

illness and can provide a sense of perspective for

patients when they are ill and not forget there are

times of health and joy.

When the process of acceptance of the illness is

on the way, the process of coping with this

addi-tional family stress intensifies. There are pressures

between couples and among siblings and parents

brought about by the addition of a chronically ill

child. This is a fertile time for staff to intervene in

helping to establish adaptive coping mechanisms

for patient and family. A chronic illness does not

have to mean a low self-image. The literature

in-dicates the desirability of children to be

partici-pants in the management of their disease.’ At the

center, before routine blood specimens are drawn,

the procedure is explained to the child. Parents are

discouraged from lying to children, eg, telling them

it will not hurt and it is nothing. The child is told

it will hurt initially and he or she can say ouch as

many times as he or she wants. Crying is okay too.

He on she is given the option of holding someone’s

hand and squeezing hard if he on she wants. For

toddlers, we have found it effective to ask them to

help us by asking them to tell their blood to come

out. For the few moments it takes to draw the

blood, the child repeats “Blood, come out,” giving

the child some sense of control and involvement in

what is being done. They are praised for those

aspects of their behavior that are helpful, including

standing still.

Findings indicate that how children feel about

themselves plays an important part in their coping

with their illness.4 No one likes to deal with a

demanding, dependent, whining individual, yet

many times medical staff and parents reinforce this

behavior. Cooper and Viera2 have addressed this

issue. Our response toward patients is usually to

make them dependent and relieve them of

respon-sibility. The medical establishment is

authonitar-ian. It likes patients who listen, are obedient, do

not complain, get better, and go away. The reality,

however, is most of our patients do not listen, are

not obedient, do complain, will not be cured, and

always come back. The system often insists on

ignoring the emotional/spiritual part of the

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SUPPLEMENT

905

ual. This is a fatal mistake. We can form an alliance with this part of an individual when all palliative measures have failed.

Sometimes the effect of a hospitalization can

mean not only temporary physical discomfort but

lingering emotional pain at seeing your dreams shattered. We have had a couple of young adults go into deep depressions because they had to drop out of college because of repeated hospitalizations. Me-peridine and intravenous fluids did not help make them feel any better. A recognition of how painful this can be is helpful but not sufficient. They need encouragement and reminders from family and staff that, yes, it might take them 6 months, or a year or two longer, to achieve their goal, but their goal is obtainable.5 Currently, one of those individ-uals is a laboratory technician at St

Luke’s-Roose-velt Hospital; the other is back in engineering

school and doing well.

Our families do not live in a vacuum. Along with

a child who has sickle cell disease, they must often

deal with the problems ofpoverty and racism. Their

experiences with many social systems that affect

their lives, including welfare, educational, housing, legal, and medical systems, have usually been

neg-ative.6 Our older patients often have difficulty in

obtaining adequate pain medication from medical staff. There are often comments made about drug

addiction and manipulative behavior. The medical

system often displays the same disrespect and

hu-miliation exhibited by other social systems. Part of

our job at the sickle cell center is to advocate for

patients in these situations.

Families can be given information about the

dis-ease, taught to become knowledgeable consumers

of health care, and self-advocates; all important in

adjusting to the lifelong process of living with

chronic illness.

Self-acceptance for all of us takes a lifetime. We

all know the pain of having something we do not

want or wanting something we do not have. We

need to accept our limitations before we can do the same for others. If a trusting relationship can be

established early with parents, this message of

ac-ceptance can be communicated to the parent and

in turn to the child. If the parents can believe their

child has the potential to become a functional

in-dividual, not necessarily without struggles, then the child can believe it, too. All too often our focus is illness. We, like our families, are influenced by the shadow cast by a chronic illness such as sickle cell disease. It is important to look beyond the illness.

We become too immersed in pain and forget the joy

of the human spirit that can see us through the most difficult situations. In the end, the goal is to

help our patients achieve their potential, in spite of

the additional burden of a chronic illness. The earlier the intervention, the more likely the success.

Eighty-four percent of the children initially

screened through the New York state program

through our hospital continue to be followed by the sickle cell center. Overall compliance with medical appointments is high, and our patients tend to have

a positive working relationship with our staff.

Future efforts need to focus on developing the

comprehensive team approach in working with the

chronically ill and their families. Ongoing educa-tion, reinforcement, telephone contacts, support

groups, and counseling need to be an integral part

of any program dealing with chronically ill

individ-uals, especially infants with newly diagnosed dis-ease.

REFERENCES

1. Cooper D, Viera CT: Adaptive interactional styles for pa-tients, families and practitioners, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psychosocial Issues. Chicago, University of Illinois Press, 1986, pp 94-105

2. Collins R: Psychosocial variables and interventions in the patient population, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psychosocial Issues. Chicago, University of Illinois Press, 1986, pp 62-74

3. Hurtig AL, White LS: Children and adolescents: The unex-plored terrain of emotion and development, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psy-chosocial Issues. Chicago, University of Illinois Press, 1986,

pp 24-40

4. Moise JR: Toward a model of competence and coping, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psychosocial Issues. Chicago, University of Illinois Press, 1986, pp 7-22

5. LePontois J: Adolescents with sickle cell anemia: Develop-mental issues, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psychosocial Issues. Chicago, University of Illinois Press, 1986, pp 75-84

6. Nash KB: Ethnicity, race, and the health care delivery system, in Hurtig A, Viera CT (eds): Sickle Cell Disease-Psychological and Psychosocial Issues. Chicago, University of Illinois Press, 1986, pp 131-146

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1989;83;903

Pediatrics

Sandra Hernandez