How to counsel caregivers on their child’s LGS diagnosis

Lennox-Gastaut syndrome (LGS) is a developmental and epileptic encephalopathy with a high rate of drug resistance.^[] LGS is usually diagnosed in early childhood. The prognosis is generally poor and often associated with developmental regression.

Patients with LGS can live into their 60s, but due to the high association with other disorders, they are more likely to die prematurely.^[] 

The initial conversations

Fortunately, bedside manner has become a stronger area of focus for medical schools across the country. Many schools now teach their students best practices for approaching difficult conversations, giving physicians a framework on which to rely. 

One such framework, the ABCDE rule, helps to simplify an approach.^[] The breakdown is as follows:

A—Advanced preparation

This simply means that you are ready to discuss details of the patient’s specific case: you have arranged for adequate time, and you are prepared physically and emotionally for the conversation.

B—Build a therapeutic environment/relationship

Determine how much information the parents or caregivers are capable of hearing and interpreting in the initial visit.

C—Communicate effectively

Begin by asking what the parents already know about their child’s diagnosis. Try to avoid medical jargon, answer questions appropriately, and always summarize the conversation at the end.

D—Deal with patient and family reactions

Be empathetic but do not argue with or criticize colleagues.

E—Encourage and validate emotions

Explore the parents’ or caregivers’ emotional response to the diagnosis. Offer reasonable hope, and discuss treatment options. During this difficult encounter, be conscious of your own emotions. Remember that difficult conversations often happen over many visits, not just the first.

Supporting caregivers

Shared decision-making involves collaboration between the physician and family in discussions of evidence-based solutions that reflect a patient’s (and family’s) goals, values, and preferences.^[] This involves developing a strong connection with the patient and their family, employing active listening, and finding common ground.

Recognizing and acknowledging the skills and knowledge of parents in the care of their child is of utmost importance to avoid power struggles and to empower parents.^[]

Preparing loved ones for possible challenges

Given the poor prognosis of LGS, disease-specific counseling is important. This should not be approached in one conversation alone, but rather continuously throughout the course of the patient’s disease. 

The mortality rate at diagnosis is between 3% and 7% in 8 to 10 years of follow up.^[] Frequently, death can be from accidents. Parents need to be counseled on the possibility of drug resistance and what to do if their child experiences an uncontrolled seizure.

Patients with LGS may require 24/7 support and will often benefit from an interdisciplinary team approach, including nursing, physiotherapy, occupational therapy, and speech therapy. Connecting parents with the LGS Foundation and the Epilepsy Foundation may be quite beneficial, as they offer a wealth of resources covering most aspects of an LGS diagnosis. 

Comprehensive treatment plans developed with a team of clinicians—including a pediatrician, neurologist, psychiatrist, neuropsychiatrist, and possibly a surgeon—are often needed to ensure the patient is receiving the best care. Treatment plans can be complex, so patients with LGS require a lot of special care and attention from parents and family. As well, the frequency of uncontrolled seizures and resultant intellectual disability means counseling is essential.