It is very important for everyone to appreciate that the burden of care and the effects of the disease on the child necessitate adjustments in virtually all aspects of the lives of all family members.
THE IMPACT OF LENNOX-GASTAUT SYNDROME ON FAMILIES
Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that not only has a major impact on the health-related quality of life (HRQoL) of the affected children, but on their caregivers as well. The primary caregivers of these patients are their family members, and their mothers usually play the most important role along with support from fathers and siblings.
Patricia A. Gibson, MSSW, DHL, ACSW, Director of Epilepsy Information Service and Associate Professor in the Department of Neurology at Wake Forest University School of Medicine, explains the impact of LGS on family members of patients, and in particular, the parents. It is very important for everyone to appreciate that the burden of care and the effects of the disease on the child necessitate adjustments in virtually all aspects of the lives of all family members.
Similar to epilepsy, the impact of LGS on the health-related quality of life of patients and family members depends on many factors, including the severity of the disease, other handicapping conditions, the complexity of the management of the disease, restrictions in the activities of children and families, and the level of social support and resources available. The physical, cognitive, behavioral, and social effects of LGS have a major impact on the HRQoL of the affected child. The physical impact is the result of frequent, severe seizures that can cause injuries throughout the entire body, most importantly, in the brain. Approximately 90 percent of children with LGS have intellectual impairment. In addition, the underlying problems in the brain cause many children with LGS to have severe behavioral problems. Mothers of children with intractable seizures and especially serious behavior problems are far more likely to have greater maternal stress.
IMPACT OF LGS
Physical Impact: Both the frequency and severity of seizures result in LGS having a major physical impact on patients. Many children with LGS are unable to walk and therefore must be carried or transported via wheelchair. As children become older and therefore heavier, lifting and maneuvering them becomes physically more difficult for caregivers. As a result, caregivers often report experiencing back and shoulder pain.
Children with LGS that are able to walk are often unsteady and tend to fall easily. Also, patients can fall during a seizure, resulting in frequent injuries and emergency room visits. Caretakers can also be hurt trying to intervene during falls. Additionally, they often experience physical exhaustion due to the constant vigilance required to prevent injury, and the sleep loss that occurs when the child has a nighttime seizure.
Surveys we have conducted at Wake Forrest have produced results that are similar to those obtained during a limited number of studies examining the physical impact of chronic diseases or disabilities, including LGS, on caregivers of children. For example, in a focus group study of 40 parents/caregivers of children with disabilities, more than half indicated that their physical and emotional health was negatively impacted by the demands of caregiving, and most also reported experiencing chronic fatigue and sleep deprivation. Many caregivers linked negative physical and psychological impact to the combination of the daily tasks required for caregiving and anxiety about the health and future of their children.
Emotional Impact: Unfortunately LGS has a major emotional impact on the entire family. The constant vigilance required when caring for a child with uncontrolled seizures and developmental
disabilities that progress over time is emotionally taxing. The fact that the onset of seizures is unpredictable while the emotional impact is tremendous was well described by Susan Usiskan, a parent who said, “My life between seizures is like walking on a series of trapdoors, any one of which may open any moment and throw you to the ground.”1
Because of the threat of continued and unpredictable seizures, parents, children with LGS, and their families experience a loss of control. Such a loss results in constant anxiety among parents, who are especially concerned by the possibility of a seizure at any given point in time as well as the future of their children. Parents also report feeling anxiety about the potential for injury, cognitive decline, or death of their children, as well as the very realistic financial burden of the disease. Some parents have also described a sense of loss and unfilled expectations as a consequence of LGS.
Social Impact: LGS also has a major social impact on the family of patients. In our experience, following a diagnosis there is typically a decrease in the amount of recreational activities for not only the affected children but for all family members. Although the condition has an immediate negative impact on patients and families, most tend to adjust and eventually come to terms with the new diagnosis and what it means to them. However, due to the burden of care, parents of children with LGS report limitations on the time available for their own leisure and social activities. Additionally, because it is not possible to predict when seizures will occur, parents often find it extremely difficult to attend social events. Both routine childcare and respite care can be difficult to acquire because non-caregivers and immediate family members frequently do not want to tend to a child with frequent seizures, especially those that can result in sudden falls.
Despite great advancements in social attitude over the last generation, LGS, as well as epilepsy, continues to be a stigmatized condition, and many families suffer as a result of these public attitudes. As one parent remarked in the 2010 study by Gallop et al.,2 “There is a huge stigma against seizures, and people freak out about them even though there is no sense in it, but you cannot get past that.” One mother, after a number of negative encounters with the public, now carries with her a card that explains the condition of her child that she can simply hand to unsympathetic bystanders (see sample card below).
IMPACT ON FATHERS
Due to the fact that the primary caregivers of children with LGS are often mothers, most studies assessing the impact of the condition on families have included limited numbers of fathers. As a result little is known about the specific effect LGS has on fathers of children with LGS. However, a 1999 study performed by Katz et al. in Israel3 found that fathers of children with chronic illnesses experienced a greater number of stressful life events and expressed feelings of lower self-esteem; no significant differences between fathers and mothers were reported for social support, marital satisfaction, and involvement in the care of the child.
IMPACT ON SIBLINGS
In our observations at the Epilepsy Information Service, siblings are greatly affected by their brothers or sisters with LGS. Despite parental efforts to spare them, siblings are well aware of the fears, concerns, and worries of their parents. Because of their anxiety and fears, many parents pay more attention to their children with LGS, and therefore siblings can be resentful and angry, and act out their frustration. As a result of their roles during their childhood, many siblings assume caretaker duties early in life by necessity and not infrequently end up in such professions, including medicine, special education, physical therapy, etc.
IMPACT ON MARRIAGES
Our experience has also enabled us to learn that LGS has a major impact on marital relationships. The many physical and emotional demands in caring for children with LGS often result in changes in family dynamics, roles, and lifestyles. These changes may have negative or positive consequences. For example, the primary caregiver may resent, even subconsciously, giving up a career or other life options to care for the child. Conversely, caring for children with LGS can bring a family closer together.
MY CHILD HAS SPECIAL NEEDS
Please be understanding. My child developed epilepsy at 18 months and has severe developmental delays, much like an autistic child. My child is NOT a bad child, and I am NOT a bad parent. My child is having a meltdown and it is common in children who are frustrated and cannot communicate with us. You cannot imagine what it is to live with this situation every day and whispers and stares do not help. Please spare a thought for the child who struggles to stay calm and regulated and for the parents who are constantly stared at, judged, and criticized. Please educate yourself before you judge. Parents and families like us need all the support we can get. Please visit this website (personal website or Facebook) for more information on our story, or visit these websites for additional information: www.lgsfoundation.org or www.epilepsyfoundation.org.
A chronic disease of any sort has major financial implications, and this is especially true for LGS since treatment of seizures alone can be very expensive. A recent study by Dr. Andrew Wilner compared costs of those with epilepsy to controls without epilepsy enrolled in a managed health care program and found that the medical costs of those with epilepsy were much higher.4 In our
experience with working with families of LGS patients, some of the equipment needed, such as helmets, may not be covered by insurance or Medicaid. Available data suggest that career opportunities of primary caregivers may be negatively affected by LGS, and there may be immediate unanticipated costs and reduced family income, as well as long-term financial concerns that contribute to emotional stress and anxiety. •
ABOUT THE AUTHOR:
Ms. Patricia Gibson is Director of the Epilepsy Information Service at Wake Forest University in Winston-Salem, North Carolina, a nationwide telephone information line for patients and their family members. She also serves as Associate Director of the Comprehensive Epilepsy Program at Wake Forest University, where she provides individual, group, and family counseling and is actively involved in medical student, nursing, and patient education.
Acknowledgements: Writing support for this manuscript was provided by Aric J. Fader, PhD, of MedVal Scientific Information Services, LLC, and was funded by Eisai Inc., which did not have a role in the development or review of this article. This manuscript is an original work and was prepared according to the International Society for Medical Publication Professionals’ Good Publication Practice for Communicating Company-Sponsored Medical Research: The GPP2 Guidelines.
Disclosure of Conflicts of Interest: Ms. Gibson was a member of an expert committee for Eisai Inc. and received an honorarium for her participation. Ms. Gibson also discloses that she has participated on the scholarship committee of UCB Pharma and has received reimbursement for this; she has been a consultant with Eisai Inc. in the development of two patient educational videos for which she received payment.
1. Trimble MR. Women and epilepsy. New York: John Wiley & Sons, 1991.
2. Gallop K, Wild D, Verdian L, et al. Lennox-Gastaut syndrome (LGS): development
of conceptual models of health-related quality of life (HRQL) for caregivers
and children. Seizure. 2010;19:23-30.
3. Katz S, Krulik T. Father of children with chronic illness: Do they differ from
fathers of healthy children? J Fam Nurs. 1999;5:292-315.
4. Wilner AN, Sharma B, Soucy A, Krueger A. Healthplan paid cost of epilepsy in
2009 in the US. Epilepsy Behav. 2012; 24; 412-416.