Care Across the Lifespan

The patient journey starts with a diagnosis and often involves pediatric-to-adult transition, clinical follow-ups and multidisciplinary disease management. In recent years, the care model for neuromuscular disease has evolved into a patient-centric treatment approach, in which individuals are driving their own care decisions.

This approach was the focus of the Care Across the Lifespan session at MDA’s Clinical & Scientific Conference. The session revealed that many people with neuromuscular disease are living longer lives with higher quality of life, in particular because of improvements in the diagnostic process, a robust drug development pipeline with increased clinical trial opportunities, the increased availability of multidisciplinary MDA Care Centers and improved management of disease symptoms.

The incorporation of professionals with experience in nutritional, respiratory, psychosocial and mobility aspects of neuromuscular illness means that many everyday problems can be addressed. As part of this, patients are better able to have difficult but important conversations with their providers — specifically with respect to sexual health and end-of-life care.

Talk About Sex

Sarah Stoney, M.S.W., L.S.W., a licensed clinical social worker at Children’s Hospital of Philadelphia, spoke about an underdiscussed topic: sexual health for individuals with neuromuscular disease. She addressed issues surrounding body image, challenges in advocating for sexual health, and ways patients and care teams can normalize these conversations.

Young adults with progressive illnesses often have a complex relationship with their body image that is compounded by a lack of representation in the media. Many patients report feeling they may not be able to have sex; are weak, asexual or infertile; that they may not ever fit the role of spouse or parent; or that their medical needs outweigh their sexual needs.

Family planning is also an underdiscussed topic for women with neuromuscular disease. Pregnancy and childbirth with these conditions can be more complex, and many women find themselves underprepared because of a lack of information.

Individuals with physical disabilities and neuromuscular disease are entitled to a positive sexual identity, Stoney asserted. She encouraged the care teams of individuals with neuromuscular diseases to acknowledge and validate the importance of healthy sexuality. Sometimes, the best resource is a peer group that can offer information and support.

Plan Ahead

Often, when patients are referred to hospice care, they haven’t been asked about their preferences. Members of the care team tend to delay the conversation because they don’t want to take away hope or contribute to feelings of depression. However, as Nancy Glass, M.D., M.B.A., of Texas Children’s Hospital, discussed at the conference, failure to plan can create more distress, not less.

Dr. Glass emphasized that individuals with progressively degenerative diseases are aware of their decline and likely have end-of-life issues on their minds already — so clinicians need to talk about it. Discussing advance directives, do-not-resuscitate orders and hospice plans actually puts patients in control and helps identify those who can make decisions.

A vital piece of the discussion is providing accurate information about the patient’s options. There is an important difference between palliative care and hospice care. Palliative care is part of the treatment plan and should be discussed early on to help decide the goals of care, irrespective of the prognosis. Hospice care is for patients who are reasonably expected to live no longer than six months. Recent legislation has ensured that Medicaid patients in hospice care are able to receive active therapy, which means there is no reason to delay a referral to hospice. An earlier referral allows the hospice team to form a relationship with the patient and provide support.

A conversation about the goals of care is essential to ensure the best quality of life for a patient. This conversation should take place in a calm setting, not during a health crisis. If a patient does experience a health crisis, previously discussed goals can guide the care team in its decisions.

Parting Thought

Difficult and uncomfortable conversations are essential in advancing the patient-centered care model. Care teams should approach these conversations with the idea that all patients are entitled to the dignity of the full human experience — and patients deserve the opportunity to access information and address these areas.

Lucja Grajkowska, Ph.D., is MDA’s medical education content manager.