Dr. Sheelah Connolly, Research Fellow, was kind enough to do an email interview to tell me more about an Irish study that’s just getting underway. The study takes a comprehensive look at the situation any family finds itself facing in the aftermath of an ALS diagnosis. Dr. Connolly and Dr. Miriam Galvin are the lead investigators. For more about the study design, see the information at the end of this article.

Marylee: Briefly explain what happens now in Ireland when a patient is diagnosed with ALS. Is there immediate support provided to the family in terms of visiting nurses or nurses aides or does the burden of care all fall on the family’s shoulders?

Sheelah: For those patients diagnosed within or referred to the ALS multidisciplinary clinic, the consultant neurologist will refer the patient to the appropriate health services in their locality, as well as arranging a follow-up visit to the clinic, usually within 6 to 8 weeks. The clinical nurse specialist (CNS) is available to patients to answer queries, provide advice and do home visits where appropriate. In addition the CNS will liaise with the community based service providers.
It is less clear what happens to those who are not referred to the multidisciplinary clinic.
In addition, the Irish Motor Neuron Disease Association (IMNDA) – a charitable organisation – provides support for people with ALS, their caregivers and families. This can entail home visits by a specialist ALS nurse, financial assistance towards home care help and the supply of specialised equipment on loan to those with ALS. Upon registration with the association, ALS patients can avail of the services provided by the association free of charge.

Marylee: One focus of your study will be “caregiver burden.” Does that refer to the number of hours the caregiver is “on call” and the anxiety of caregiving, or does it refer to the physical demands?

Sheelah: Caregiver burden will be assessed in a number of ways in the study. Caregivers will be asked the number of hours of care provided as well as the type of care. Caregiver burden will be quantified using the Zarit burden interview, which includes 22 statements about care giving with respondents asked to choose one of five responses ranging from never to nearly always.  Some open ended questions will explore both positive and difficult aspects of the caregiving experience, as defined and perceived by the caregivers themselves.

Marylee: In addition to asking patients about depression and anxiety, will you be asking caregivers about that, too?

Sheelah: Yes, depression and anxiety will be measured in patients and caregivers using the Hospital Anxiety and Depression scale.

Marylee: Your study will address try to determine the financial implications of ALS on the household. Will you include renovations needed to make the home more accessible, such as adding a downstairs bath, widening doors, and adding wheelchair ramps?

Sheelah: Yes, the cost analysis will take a societal perspective and include costs associated with health and social care usage (as well as the costs of accessing these services including travel and parking), aids and appliances, modifications to the home and car, any increase in household bills (for example increased heating costs) as well as any alternative or complementary medicine.

Marylee: Will you also ask about the economic impact on caregivers of lost hours from work, lost wages, and possible work furloughs needed when patients are in the final stages of the disease?

Sheelah: Yes, the questionnaire will ask about economic status and hours worked before the onset of caregiving as well as at various stages in the caregiving process. It is anticipated (and seems to be backed up by interviews already completed) that some caregivers are reducing the number of hours worked and/or giving up work altogether as the disease progresses. The questionnaire also has some questions on hours of work missed due to the caregiving role.

Marylee: What about families where there are young children? Are any questions specifically designed to learn if they have different needs than families where the household is just a husband and wife or two siblings?

Sheelah: We do not specifically address the issue of families with young children in our study though it would be an interesting line of enquiry and at least one of our current study participants has children under the age of five.

Marylee: Right now in Ireland, are there many patients living with mechanical ventilation (trach tubes), and if so, who pays for round-the-clock caregivers?

Sheelah: There are very few ALS patients with mechanical ventilation in Ireland; in general it is not recommended by medical professionals and anecdotal evidence would suggest that relatively few patients want it. Of those in receipt of mechanical ventilation, cost is likely shared between the health service and patients family.

The study hopes to develop an integrated program of palliative care that will serve as a “best practices” model for how patients with ALS and other neurodegenerative diseases will be treated in the future. As things stand right now, are families pretty much on their own, or does care depend on whether the patient comes from a rural or urban community?

We are planning on examining differences in care by geographical location within the study. There is, as you suggest, an urban/rural divide in Ireland with people in urban areas generally better served than those in rural areas, especially in relation to the provision of hospices. However, the situation is probably more complicated with not all urban areas having sufficient facilities.

The research team consists of Dr. Miriam Galvin (Research Fellow), Dr. Sheelah Connolly (Research Fellow), Mr Iain Mays (Research Assistant), Professor Orla Hardiman (Consultant Neurologist/Head of Academic Unit of Neurology, Trinity College Dublin)

Here is a brief description about what the study hopes to find out.

The project – A population based analysis of needs, services and outcomes in motor neuron disease (MND)/amyotrophic lateral sclerosis (ALS) – is a prospective study tracking the patient and caregiver journey through ALS. The study will estimate the economic and welfare impact of the condition on patients, their caregivers and the health system, and is part of a larger project which aims to generate a framework of care for ALS patients.

The study aims to identify and recruit 100 ALS patient and their primary caregiver attending the only multidisciplinary ALS clinic in Ireland. Patients and caregivers will be interviewed a maximum of three times over an 18 month period. A wide range of data will be collected on both the patient and caregiver. Face to face interviews in the patient home (where appropriate) will be used to elicit details of the patient demographic and socio-economic situation as well as the health and social care resource use associated with their condition. In addition, quality of life and depression and anxiety will be measured using standardised measures at the home visit. Clinical data on the patient, including details of their diagnosis and progression over time, will be obtained from their medical records. The primary caregiver will also be interviewed in the patients’ home (where possible separate from the patient). Caregivers will asked about their demographic and socio-economic situation including any change in employment status/hours due to their caregiver role.  The same standardised measures as used with the patients will assess caregiver quality of life and depression and anxiety with an additional measure used to evaluate caregiver burden. These will be tracked over time in order to assess how the caregiving experience changes as the disease progresses and a series of open ended questions will explore aspects of the caregiving experience.

Data collection began in the summer of 2013 and at present (January 2014), 42 patients and 39 associated caregivers have been recruited and completed time one data collection. Time two data collection is also now underway.