Role of Pediatric Palliative Care and Down’s Syndrome

Down’s syndrome or Trisomy 21 is a genetic disorder caused due to the presence of an extra copy of chromosome 21. It occurs in approximately 1 in 830 live births, manifested at birth through characteristic physical features. In the 1980’s, the average life expectancy of a person with Down’s syndrome was only 25 years of age. Today, the average life expectancy for people with this condition is nearly 60 years (in developed countries), which continues to follow an increasing trend.

The severity of symptoms in people with Down’s syndrome varies across a wide spectrum. Some patients will only require minimal support in the form of regular pediatrician visits and multidisciplinary team follow-ups. Such patients will need concentrated efforts from Occupational therapists, Speech Language Pathologists, Physiotherapists and other allied health care professionals to support and manage their cognitive delay, developmental needs, feeding issues and delayed motor milestones. Some others might need palliative care services right from when they are diagnosed with a complex heart disease or leukemia. These children may also have comorbidities due to the presence of cardiac defects, immune disorders, Spinal and gastrointestinal defects, to name a few.

Childhood mortality is often associated with congenital heart defects and leukemia. The risk of developing childhood acute leukemia is 10-20 times higher in children with Down’s syndrome than in the general population, while the risk of early death and persistence of morbidities remain through their lifetime.

Palliative care can provide holistic care for these children. Children with Down’s syndrome and their care givers, often tend to experience unpredictable disease trajectories which requires robust care planning, which includes attention to not only the physical toll the disease causes, but to also the psychosocial and spiritual issues that the child and the family deal with. While the psychiatric disorders experienced by this population will need to be assessed and managed, the Psychosocial issues for this population varies according to the age of the child; for example, adolescents may experience moderate to severe emotional and psychological distress when compared to their healthy peers.

In certain cases, palliative care will need to be provisioned right from the initial stages when there is a medical crisis. For example, when the baby has severe respiratory distress due to an underlying cardiac ailment, the pediatric palliative care team will be called in for immediate symptomatic management. Once the child stabilizes and undergoes a curative surgery successfully, the pediatric palliative care team will slowly step back as needed. Those children who did not need palliative care teams to be involved at the time of diagnosis, may end up needing it when they develop Acute leukemia. Involving the palliative care team in such cases will provide an extra layer of support for both the child and the caregiver.

With increasing rates of survival and prolonged life spans for this population (due to the significant advances in the field of medicine), a well-planned transition to adult palliative care services will be needed as and when appropriate. As these patients progress into adulthood, there is an increased incidence of Early Alzheimer’s -like dementia, which warrants the definite involvement of palliative care teams to support such patients and their caregivers and ensure the best quality of life possible for them, be provided.

To conclude, we would like to reiterate the below:

Not all children with Down Syndrome require Palliative Care.
Not all children with Down Syndrome requiring Palliative Care, will need it as a full-time support. The need will vary based upon the needs of the child and the family.
Care co-ordination between several specialties, Genetic and family counselling, advocating for the child are the other components of care, that will be addressed by a Palliative Care team.

About the Authors:

Dr Mohammad Ishak Tayoob is a Pediatric Palliative Care Consultant at the Pain Relief & Palliative Care Society, Hyderabad, who provides Palliative Care for Children with Cancer & Non cancer conditions.