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While considering the diagnosis and treatment of hypoplasia of the left heart, it is important also to look at the psychological, develop- mental, and social problems that the children and their families face. As previous chapters have discussed diag- nosis and treatment, this chapter discusses the needs of the family, especially the parents, during the initial diag- nosis and treatment of the condition, explores the prob- lems confronting the children as they develop, and examines some of the issues that affect those with special needs living within the community.
INITIAL DIAGNOSIS
To most parents, the realisation that their child has hypoplasia of the left heart, be it diagnosed antenatally or postnatally, is a devastating shock. The expectant family is anticipating a normal child. The pregnancy, until diagnosis, will have been normal, with the baby growing as expected, and the mother feeling healthy.
All the early tests done during pregnancy will have been negative, reassuring the family that all is well.
Nowadays, of the 200 babies diagnosed in the United Kingdom each year with hypoplasia of the left heart, approximately half are diagnosed at the ultrasonic scan performed at their obstetric centre between 18 to 20 weeks’ gestation, and then further confirmed at their regional fetal cardiac centre.1 At this stage, the most difficult problems for parents and families are trying to understand the diagnosis and the options for treatment, whilst at the same time trying to cope with the emo- tional trauma that goes with their feelings of loss. Many families exhibit the same emotions as families who are bereft. They often become angry and frustrated,
denying the diagnosis and demanding a second opinion.
Then they show signs of depression and weepiness.
One mother described the experience as “being in a pot of treacle—the more you struggled to the edge, the harder the treacle pulls you down.”
Amongst all this turmoil, the expectant couple are expected to make a decision about whether to continue with the pregnancy or interrupt it. Many families ask to be led through this mire of feeling. Although it is extremely important to support and inform them, fami- lies must make the decisions themselves. During this time, therefore, it is important that families are offered the chance to talk through the diagnosis and options for treatment more than once, and with different clinicians involved in their care, for example, cardiologists, spe- cialist nurses and midwives, fetal medical obstetricians, and possibly surgeons. Families welcome the use of visual aids during these consultations, ideally ones that they can keep for future use. They should always be given the opportunity to return with the questions that such discussions generate. To help complete their per- sonal information jigsaw, they also need to be offered the opportunity to talk to other couples and families that have had to make the same choices. Two charities based in the United Kingdom, namely Little Hearts Matter (www.lhm.org.uk), a support and information service for families of children in whom a single func- tioning cardiac ventricle disorder has been diagnosed, and Arc (www.arc-uk.org) who offer support to families that have received antenatal results and have been given choices for treatment. These and other organisations offer information geared to nonmedical readers and nonjudgemental support from diagnosis onward.
Having made their choices with regard to treatment, couples must be supported, whatever decision has been
THE PSYCHOSOCIAL PROBLEMS FACED BY THE FAMILIES OF CHILDREN WITH HYPOPLASIA OF THE LEFT HEART
Suzie Hutchinson
made. Those who have opted for pregnancy termina- tion must be allowed the opportunity to grieve for their baby, with nonjudgemental support provided through- out. Those who decide to continue with the pregnancy need support and more information about the possible options for the treatments available at birth. Many of these families, although stressed and frightened, have the opportunity to learn and understand more about what is to follow. They build mechanisms for support, drawing on the many professionals and families that they have had the opportunity to meet. These parents have time to collect the information necessary to make an informed decision about consent for surgery. After the birth of one such baby, his mother said to me, “I can now love my baby and not waste any of our time.” Little Alex, unfortunately, died. But because his family had the opportunity to plan, they have many mementos of his short life. He is part of their family.
DIAGNOSIS AFTER BIRTH
The opportunities to understand the disorder and to start to prepare for the treatment to come are not avail- able to those families for whom the diagnosis has not been made before birth. For these families, the trauma is magnified, since they swing from the joy of what seems to be a normal delivery and a healthy baby to the devastating discovery that the baby has a noncorrectable condition for which only very complex and risky pallia- tive surgery is available, with the great chance that, if their baby lives through the first 5 years, he or she will probably face heart transplantation as a teenager.
These families may well have had to travel many hun- dreds of miles for this information. The mother will be sore and tired following a delivery. The father will be trying to keep things together, whilst possibly juggling other children, work, and his own emotional turmoil.
They will need to make a decision as to whether to opt for the palliative surgical options or not. They have none of the advantages of time that are now offered the families in whom the diagnosis is made antenatally.
They will be feeling even more bereft, confused, and very frightened. One mother described this experience as being “in a fast moving river. Rocks and branches jut out into the water.” These “rocks and branches” were the ever-increasing difficulties for their child that they were learning about as the clinicians explained the con- dition, treatments, and life expectancy. The mother said she could hear the waterfall at the end of the river.
Unable to free herself from the water, she was hurtling toward the drop. Suddenly, she was flung into the air, falling a vast distance into a pool of deep cold water, which engulfed her. Eventually, arms stretched towards
her, and she was able to pull herself free, never quite losing the bone-deep coldness that the water had caused.
This metaphor provides us with an insight into the feelings of the parents, their lack of control, and above all, the impression of inevitability. It is important, there- fore, that these families, like the antenatal group, are offered many sources of support and information. The cardiac liaison nurses are able to bridge the gap between the medics and the parents, offering informed explana- tions and a caring word. The families also need the opportunity to speak to other families who have had similar experiences.
Some families decide that treatment is not for them.
They may wish to take the baby home, and be sup- ported until the baby dies, so-called comfort care.
Others prefer the support of the hospital, but may like to travel back to their original centre of care. Families need the opportunity to spend as much time with the baby as they can. Other children should be encouraged to visit, and grandparents and friends should be allowed to be part of the baby’s short life. The religious needs of the family should be considered, with every opportunity for the family’s own support network to visit and be part of care. During this short time with the baby, the family needs to build up memories that they can keep with them forever.
Those families that have opted for treatment now ride the roller-coaster of the surgical progression for pal- liation of hypoplasia of the left heart, with all the traumas described in Chapter 7. Families now begin to use the resources that they have previously been offered; they look for more information and gradually look forward to what may lie ahead. It is important that they are offered consistent support, building their rela- tionship with the medical and nursing team, and using parent-to-parent support as their way to understanding the life of a child with this complex condition. Often the professional teams have to help a family sift through the information they collect. Access to the Internet can cause as many problems as it solves, with families often subjecting themselves to sensationalised research and journalism that uses language that is difficult to under- stand and interpret. Directing parents to recognised Web sites and documentation ensures that they have access to information written for them. Later, when there is a fuller understanding of the condition, they will be able to explore the Net without leaving themselves open to misunderstanding.2–4 As the baby’s condition improves, parents need to be introduced gradually into care. This helps them to stay involved, reducing their worries of detachment.
“George wasn’t mine whilst he was on intensive care.” As this mother’s comment indicates, there are
great difficulties in parent bonding when a child is seri- ously ill. Encouraging a mother to express breast milk, which can be used in supplemental feeding, helping a father learn how to do eye care, and suggesting the keeping of written and pictorial diaries can all help families to feel included. The bedside and liaison nurses do a great deal to observe and solve problems of parent–child interaction, working as a team to find com- fortable ways to work through any problems. Many fam- ilies express the concern that, if they get too close to the child and he or she subsequently dies, it will be harder for them to cope. Gently, they need to be encouraged that the reverse applies, that their feelings of loss will be harder to cope with if they fail to establish any relation- ship with their child.5
For some families, especially those from abroad, it is impossible for the parents to travel with their child for treatment. For these parents, there is a great need to record their child’s activity. Using pictures, poems, and text, translated if necessary, diaries can be kept so parents have a little piece of the time they were away from their child. Saving identification bands, bootees, and even ventilator hats can help parents feel that they have a memory that otherwise would be lost to them.
During treatment for this condition, there are risks that the child might die at anytime. Families need to be offered the opportunity to express the fears that this knowledge generates. Once parents have talked through these worries, they often make a positive deci- sion that they will lead as normal a life as possible, facing any problems as they arise rather than worrying constantly. Other families become stressed and insular.
All the family activity revolves around the sick child, and any normal childhood differences are blown out of pro- portion, making it difficult for families to remain united.
There is evidence that, in some communities, more than two-thirds of families with a chronically disabled child undergo major marital stress leading to divorce.6
As parents face the child’s discharge, it is important that they take more and more responsibility for the day- to-day needs of their child. It is only then that any worries about their confidence or ability will be observed. Gradually, they will learn to feed, bathe, dress, and medicate their child. This needs to be sup- ported by the experienced ward staff, with time being given for families to express their fears and worries, especially if there are still concerns about weight gain, cardiac function, or oxygenation.
Hand-held records or information folders should be used to back up the verbal instructions that are being given. All information should be written in an easily understandable form to ensure that all members of a family can use it. Empowering families with knowledge concerning their child with hypoplasia of the left heart
will help to ensure better observation and reaction should the child become sick. This helps families approach day- to-day care with confidence, whilst also providing a better, more logical, response when needed.7
LIFE AT HOME
Discharge from the hospital to home causes many stresses and strains on the family of a child with hypoplasia of the left heart. Adjusting family life to any new arrival is difficult, but when the child has a life- threatening disorder the adjustments may take a great deal of time, and for some it will never quite happen.
Finding themselves miles from their centre for tertiary treatment, within a community where the medical team knows little or nothing about the condition of their child, increases significantly the level of stress placed on the family, making it even harder to cope at home.
Finding the balance between sensible concern about their child and overpowering worry is very difficult for many parents. Often, the period between the first and second stages of palliative surgery, that balance is never reached. After the second operation, if things have gone well, a more settled daily routine emerges, inter- jected with only brief periods of hospitalisation and treatment. There are many areas of life at home, nonetheless, that remain to be explored. Below, I high- light some of the most common parental worries, offer- ing some ideas as to how the problems might be alleviated.8
THE COMMUNITY TEAM
As previously mentioned, one of the greatest fears expressed by parents is that their family practitioner and visiting nurse do not understand their child’s condition.
In this respect, it is important to remember that it would be unreasonable to expect every community doctor and nurse to understand the extremely complex and rela- tively rare condition of hypoplasia of the left heart. They are trained to look after healthy children who may occa- sionally become ill, rather than children who have complex congenital cardiac disease requiring long-term medical support.
To help the transition from hospital to home, the hospital liaison and the nurses based on the ward of the tertiary centre should make contact with, and work with, the community team, ensuring a basic understanding of the condition and providing a route through which they can express concerns. The use of hand-held records or information sheets helps to ensure continuity of care.
On the child’s discharge from the hospital, families are
advised to make a routine appointment with both their family practitioner/local paediatrician and their visit- ing/community nurse, to introduce these professionals to the new baby, and to permit them to take a baseline set of observations, for example, weight, height, colour, respiratory rate, and a list of current medications. It also gives both the parents and the medical staff the oppor- tunity to express any concerns that they may have about community care. They can then build a structure for ongoing support and access to swift medical help should it be required. There are many areas of care that parents have to learn to take on themselves following the child’s discharge from the hospital.
Heart Failure
Having been surrounded by clinical support within the hospital, many parents worry that they will not know if their child’s condition has deteriorated once they have been discharged from the ward. Understanding the signs to look for, and knowing what to do about them, can help families cope with the feelings of medical iso- lation at home. Before discharge, the signs of heart failure should have been explained to parents with the use of hand-held records and information sheets. There are various publications now available to help parents understand the diagnosis and treatment of these complex conditions, and details are available on the Web site of Little Hearts Matter (www.lhm.org.uk). It is often reassuring to provide contact numbers that parents can call if they have any worries. There may be many calls in the first few weeks, but gradually, as the child settles and parents become more confident, the need to call decreases. The family practitioner and the visiting nurse should be used as contact points, since any ailments might be simple and easily treated by them.
Diet and Weight Gain
For many parents, and their community team, diet is the area of care at home that causes the most consterna- tion. Children with hypoplasia of the left heart have a very high nutritional requirement. Their bodies run inefficiently, so they need many calories just to maintain the system. To ensure growth, they need added calories.
However, they tire easily when feeding, and they also need careful fluid balance to ensure that they do not tip into uncontrolled heart failure. In this respect, parents need a great deal of support during their first few months at home, with lots of encouragement with feeding even if their baby gains only small amounts of weight. There is no reason to dissuade mothers from breast-feeding if they and the baby are happy with it.
Extra calories can be given by mouth just before
feeding, or supplementary feeds can be provided if the baby is not gaining weight.
Mixed feeding often ensures satisfactory gains in weight over the first few months. Oral feeding can be encouraged in the day, with overnight continuous naso- gastric or gastric feeding helping to raise calorie intake at a time of least activity. Although charts for weight are used for children with hypoplasia of the left heart, they are only one way of assessing the gain, and should not be used to compare weights with the “normal” scale for that age group. Often parents feel so responsible for their baby’s failure to gain weight that they lose the joy of having their child at home. Meals become a battle, and families become extremely tired and tense. This adds to the strain, making the circle of feeding a vicious one. It is important that the hospital and community teams work together with families, listening to the needs of parents, grandparents, and siblings, encouraging and praising even the smallest weight gains. Realistic targets can be set to ensure that plans for feeding fit into family life.
After the second stage of palliative surgery, feeding often settles down. Children wean at this stage, and so it is easier to give them more calories. They will gradu- ally have more energy and become less breathless because of the surgical rerouting of blood to the lungs.
These children are very unlikely ever to be overweight, but gradually they will settle into a feeding pattern of a small amounts of food at regular intervals, eating higher calorie foods, and enjoying eating with their family.
Development
It is very difficult in the early days of childhood to make judgements as to how a child will develop. When those days may have been interrupted by hospitalisation, bypass surgery, and periods of time under artificial venti- lation, development becomes even more difficult to assess. The majority of children, nonetheless, appear to have developed normally. They are able to walk, talk, ride bicycles, and climb ladders. Occasionally, there are prob- lems with either gross or fine motor skills, or social devel- opmental delay. The evidence at present is that these cases are few and far between (see Chapter 13).
Some children experience increasing fears on hospi- talisation. These often manifest themselves as phobias against needles or doctors. Much can be done to allevi- ate these fears. One option is to ensure that parents have a good understanding of treatments, so that they have as much control through understanding as they can, thus alleviating some of their stress and conse- quently reliving some of their child’s fears. It also helps to prepare children before a hospital admission through the use of books and games, coupled with a preadmis-
sion visit offering the opportunity to discuss their fears, thus greatly reducing the extreme reactions to hospital- isation experienced by some children.
Financial Concerns
For many families, the financial strain of having a child with hypoplasia of the left heart is an added stress. Often there is a need for parents to take extended periods of leave from work, which adds to the extra expenses created by visits and stays in the hospital, as well as the expenses of day-to-day living with a disabled child. To help alleviate this worry, they may be able to apply for various government or state allowances. They often need a great deal of professional help to make a claim, since the application system is complex and off-putting.
Allowances vary by country. Some disability living allowances are available, as are allowances that finan- cially support the caregiver and help with mobility where physical ability is restricted. Individual grants are available to help families that are unable to purchase the medical equipment necessary to keep the child safe or well cared for, or even to acquire a telephone or washing machines or to take driving lessons. It is impor- tant that families are encouraged to apply for such allowances, as acknowledgement of a disability can help open doors of opportunity for a child, as well as helping to alleviate the financial strain discussed above.
Education
The thought of their child moving from the safe envi- ronment of home into nursery, and then full-time edu- cation, fills many parents with dread. Is the child going to be safe? Will people understand his or her needs? Will the child achieve at the same rate as other children? A majority of children with hypoplasia of the left heart cope well with normal mainstream education. They mix well with other children, and develop as children of their age should. Most children need simple support, for example, easy access to the toilet, the opportunity to stay inside if the weather is bad, extra time to get from class to class, and extra care during physical education.
In elementary and junior high schools, the goal is to treat the children as normal individuals. The needs of children with hypoplasia of the left heart are met easily within the school structure. The needs of some children, nonetheless, go beyond the bounds of normal support, and provision of a classroom assistant or specialist edu- cational support is required.
To ensure that individual needs are met, a statement of educational needs is written, including basic physical and educational assessment, as well as observations from the parents and teachers about the ability of the
child. The assessment gives the education team the information needed to meet the individual needs of each child. Many families choose to have the assessment done early in the educational life of their child, as it makes it easier to ensure that support continues throughout their education. Schools in the United Kingdom, for example, are unable to fund extra class- room support without the backing of the education authorities, so “statementing” provides for the individual needs of each child.
THE FUTURE
It is difficult to predict the future for these children with any certainty. From the experience gained from chil- dren undergoing surgery in the United States, the evi- dence is that, ultimately, children with hypoplasia of the left heart may need cardiac transplantation, perhaps in their late teens or early twenties. Some children will require this treatment earlier should their condition dete- riorate. For many families, the unsure future for their children is very difficult to live with. Many families have successfully gone through the three stages of palliative surgery, have been able to lead a normal life, and have experienced the fun and games of normal childhood. It is difficult for these children and their parents to face the fact that life may not always be so easy, and that there are many obstacles still to face.
Growing through adolescence, experiencing the beginnings of adult life, and feeling invincible, makes the timing of increased heart failure seem unfair.
Professionals who have been working with children with congenital cardiac disease for many years have seen these problems before. However, it will be a new hurdle years from now for many of the children with hypopla- sia of the left heart currently in our care.
Acknowledgements
This chapter was written with the aid of comments from cardiac liaison nurses, cardiac nurses, cardiologist and surgeons, community health care teams, and most importantly children and their families who live every day with the diagnosis of hypoplasia of the left heart.
References
1. Brackley KJ, Kilby MD, Wright JG, et al. Outcome after prenatal diagnosis of hypoplastic left heart syndrome: a case series. Lancet 2000;356:1134–1147.
2. Kanneh A. The need to communicate. Nurs Standard 1990;5(5):19–20.
3. Hutchinson SE. Getting to the heart of the matter. Nurs Times 1999;10:55–57.
4. Tucker Swanson L. Treatment options for hypoplastic left heart syndrome: a mother’s perspective. Crit Care Nurs 1995;15(3):70–79.
5. Klaus MH, Kennell J. Parent and Infant Bonding. St.
Louis: Mosby, 1982.
6. Hayes VE, Knox JE. The experience of stress in parents of children hospitalised with long-term disabilities. J Adv Nurs 1984;9:333–341.
7. Coombes R. From parent to expert. Child Health 1995;2:237–240.
8. Macdonald M. Children discharge from hospital—what mothers want to know. Nurs Times 1988;84(16):63.
Further Reading
NAWCH Charter for Children in Hospital, Action for Sick Children. London: NAWCH, 1984.
Court SDM. Fit for the Future: Report of the Committee on Child Health Services, London: HMSO, 1976.
Department of Health. Child Health in the Community: A Guide to Good Practice, London: Department of Health, 1996.
Department of Health. The Welfare of Children and Young People in Hospital, London: Department of Health, 1991.
Rodin J. Will This Hurt? London: Royal College of Nursing.
Sreeram N. Hypoplastic left heart syndrome: current treat- ment options and outcome. Curr Paediatr 1996;6:156–161.
