C58 INDIVIDUAL ALS CARE IN THE JAPANESE ‘NANBYO’ CARE MODEL: COMPARISON WITH PALLIATIVE CARE APPROACHES IN ACHIEVING BEST QUALITY OF LIFE
Nakajima T
Niigata National Hospital, Niigata, Japan
E‐mail address for correspondence: [email protected]
The concept of ‘nanbyo’ in Japan can be traced to the administrative measures of the Japanese Ministry of Health, Labor and Welfare (MHLW) in 1972. Nanbyo (meaning, nan: difficult and worried, byo: illness in Japanese) refers to diseases that are incurable and intractable in physical and psychosocial aspects. Three pillars of the measures, including research programmes on nanbyo, a care system for patients with nanbyo, and reimbursement of medical expenses to individual patients with nanbyo started in 1972. At the beginning, the administrative definition of nanbyo included Behçet's disease, myasthenia gravis, systemic lupus erythematosus, cancer in children, nephritis in children, bronchial asthma in children, hemodialysis patients, severe physical and mental disability in children, and progressive muscular dystrophy; ALS was added in 1974 according to the annual revision.
At present, funding by MHLW for nanbyo researchers provides 2 billion yen for 63 research groups. Each has missions to survey and study its own focused diseases including ALS. Clinical data including history, signs and symptoms, treatments, and activities of daily living are collected in exchange for reimbursement of medical expenses. This accounts for 70 billion yen.
Research and clinical practice in ALS have made dramatic progress under the measures of MHLW since 1974. In the early1980s, a small number of ALS patients started tracheostomy ventilation in hospital. Multi‐ and inter‐disciplinary care is thought to be necessary for improving quality of life of patients with ALS. Although the nanbyo care program together with the elderly care insurance system and disabled patients' support system worked very well, it was not enough for the patients and their family in reality, and their burden was apparently heavy. Even in this care environment some patients with ALS and their families published books or articles about their happy lives with ventilators. Many ALS patients were encouraged to live longer with a ventilator. In the mid‐1990s home mechanical ventilation started to be officially supported by Japanese health insurance. Relatively new techniques such as respiratory physiotherapy, mechanically assisted coughing, and PEG gradually became available in ALS care in Japan.
Although it may be valuable that non‐invasive ventilation and tracheotomy ventilation are both available in Japanese ALS care, psychological and spiritual conflicts sometimes occur in patients and their families in the area of informed consent to each palliation. It may now be possible that using individual QOL assessment methods such as SEIQoL and a spiritual care approach derived from UK hospice care will help diminish these conflicts. Although ALS is still incurable and intractable, patients living with ALS can sometimes establish a new meaning of life and can find new perspectives of life. We think this may be one of the goals of ALS care.
C59 WHAT CAN WE LEARN ABOUT LIVING WITH ALS/MND FROM PERSONAL ILLNESS NARRATIVES POSTED ON THE INTERNET?
O'brien MR1, Clark D2
1Edge Hill, Ormskirk, Lancashire, UK, 2Lancaster University, Lancaster, UK
E‐mail address for correspondence: [email protected]
Background: Experiences reported in internet narratives, as opposed to those appearing in other media, are more likely to be written as they occur and rarely undergo reflective editorial processes. Hence, they are often written with a degree of poignancy and intensity not found in other media.
Objectives: To determine: 1) What PALS/PwMND write about their illness experiences; 2) Why PALS/PwMND write about their illness experiences; 3) What the benefits of writing are.
Methods: The process of identifying internet narratives by people with ALS/MND has been reported previously (1). This process ultimately generated a sample of 64 electronic narratives, which were subjected to a content and thematic analysis, aided by Nvivo software.
Results: A number of key themes emerged. Most narratives contain a strong emphasis on regular condition updates, often including tips on how to manage particular symptoms and disabilities. Several authors, despite increasing loss of function, stress the importance of what can still be done, rather than what has been lost. Many refer to this as “living with, not dying of ALS/MND”. There is a sense that authors have remained ‘whole’ in their thoughts, despite their physical deterioration. Frequent comments are made about the impact on and changes to relationships with others, usually partners/spouses and referred to as their “loss of life”. There are numerous reports of searches for alternative and complementary therapies, which help to provide a sense of personal control in a life that is otherwise “out of control” and generates a feeling that they are doing something for themselves when conventional medicine has little to offer. Most authors display altruistic motivation for writing; they share their experiences (including the testing of unproven treatments) and knowledge of the disease to lessen the burden on others. They provide resources for others, in terms of advocacy, information and support; several raise awareness and educate, many inspire, offer hope and provide an example to other sufferers of how to cope with the diagnosis of ALS/MND.
Authors view the narrative writing process as therapeutic, and for some, helping to remove the final barrier to admitting their diagnosis by “going public”. Readers report being comforted, supported and enlightened. Publishing on the internet provides an opportunity to communicate with other PALS and reduce isolation. By being visible, authors act as a beacon for others and a source of invaluable information for patients, carers and health professionals.
Discussion and conclusions: Illness narratives enable individuals to document how illness has impacted on their lives and the lives of those closest to them. The electronic narratives of illness identified in this study comprise a rich source of data about issues which people with ALS/MND regard as important.
References
- O’Brien MR, Clark D. Personal experiences of living with ALS/MND: illness narratives posted on the “Web”. Amyotrophic Lateral Scler Other Motor Neuron Disord. 2005;6:160.
C60 HOUSE CALLS: MULTI‐DISCIPLINARY OUTREACH MEDICINE FOR THE HOME BOUND ALS PATIENT
Allred PL, Simpson EP, Appel SH
Methodist Neurological Institute, Houston, Texas, USA
E‐mail address for correspondence: [email protected]
Background: As ALS progresses, activities of daily living become more difficult for patients to perform. Furthermore, advanced disease often renders the ALS patient unable to access medical therapy, participate in clinical trials, and receive the support of a multi‐disciplinary team. Currently, home health services are not available to most patients. If available, these services are usually limited to restricted nursing care and/or therapy services and do not adequately meet the needs of the homebound ALS patient.
Objective: To assess the feasibility of implementing multi‐disciplinary outreach care to homebound ALS patients in order to provide a continuum of care for the duration of the disease.
Methods: The ALS Home Outreach Team consists of a neurologist, physical therapist, social worker, and a patient services coordinator from the Muscular Dystrophy Association. Patients are visited every three to six months and each visit lasts approximately one to two hours. The neurologist performs a full physical/neurological examination, assesses respiratory and nutritional status, and updates prescriptions. The physical therapist assesses the patient's functional status, updates the home exercise program, performs family and/or caretaker education, and assesses equipment utilization and needs. The social worker assesses the patient's current financial and insurance status, advises on changes in disability or insurance policies, and performs patient and family counselling as needed. The patient services coordinator facilitates obtaining needed equipment and/or other services that are identified during the visit.
Results: Multiple benefits of the House Calls Program have been identified since its inception. The medical care team is updated on the patient's status such that they feel comfortable discussing medical needs over the phone and/or refilling prescriptions even though the patient has not been in the clinic for many months. The medical team also has the benefit of assessing patients in their home environment where the logistics of day‐to‐day activities can accurately be addressed. Patients have a full medical examination and assessment without having to leave their home and are able to maintain a connection to their medical team. Required follow‐up visits from study protocols can be performed, thus minimizing drop‐out rates. Caretakers receive in‐home support and education and have the opportunity to problem‐solve difficult tasks.
Discussion: Our experience suggests that multi‐disciplinary ‘house call’ care is successful as a mechanism to maintain a continuum of care when patients are no longer able to leave their homes for medical care. Future benefits with this intervention include reduced hospitalizations, increased completion rates for required study visits, increased compliance with equipment/exercise recommendations, and improvements in quality of life for homebound patients and their caretakers.
C61 A STUDY OF COVARIATES OF FATIGUE IN ALS
Simpson EP, Allred MP, Yen AA, Appel SH
The Methodist Neurological Institute, Houston, Texas, USA
E‐mail address for correspondence: [email protected]
Background: The symptom of fatigue has been recognized in the ALS population. However, the relationship between fatigue and disease severity or other clinical features has not been well characterized.
Objective: To determine the relationship between fatigue in ALS and other disease variables and covariates of survival.
Methods: We performed a cross‐sectional study of probable or definite ALS patients compared to normal, non‐caregiver controls. We measured fatigue with the Multidimensional Fatigue Inventory‐SF (MFSI‐SF), depression with the Beck Depression Inventory (BDI), hypersomnolence with the Epworth Sleepiness Scale (ESS), and quality of life with the McGill Quality Life Scale. Regression and χ2 analyses were performed to study the relationship between fatigue and disability, progression, FVC, frontal lobe function, and comorbidity/medication use. Statistical significance was set at p⩽0.05. Results are presented as mean±SD.
Results: We evaluated 52 ALS patients (ALS) and 32 non‐caregiver, normal controls (NC). Measures of fatigue (ALS: 29±16 vs. NC: 6.4±6.6; p<0.0001) and depression (ALS: 12±8.6 vs. NC: 5.0±8.6; p<0.01) were significantly elevated above controls, with prevalence of 80% and 26%, respectively. Quality of life scores were lower (ALS: 7.1±1.0 vs. NC: 8.4±1.4; p<0.01). ALS patients showed a strong trend toward increased hypersomnolence (p = 0.059). Fatigue severity showed a significant relationship with disability as measured by the Appel ALS score (R = 0.4), but not with disease progression (R = 0.09). There was a trend toward a significance with FVC (R = −0.24). Fatigue showed the strongest correlation with depression (R = 0.74) and an inverse relationship with quality of life (R = −0.54). In the majority of cases subjects who scored within the normal range on the BDI, MFSI‐SF (fatigue) scores were still significantly higher in the ALS patients (p<0.001). There was no relationship with comorbidities or concomitant medications, although there was trend toward significance for frontal lobe dysfunction and riluzole therapy.
Conclusions: Fatigue is highly prevalent in the ALS population and its presence appears to be independent of disease progression and disability. Its high prevalence in patients without depression suggests that fatigue may also be independent of such non‐motor symptoms. Future studies will be directed at investigating other potential variables for fatigue, including frontal lobe dysfunction, respiratory function, and riluzole therapy which all showed an association trend towards significance in this study.
C62 INTRATHECAL BACLOFEN PUMP FOR TREATMENT OF MEDICALLY REFRACTORY RIGIDITY AND SPASTICITY IN PATIENTS WITH MOTOR NEURON DISEASE
Rosenfeld J, King R, Blythe A
Carolinas Neuromuscular/ALS Center, Carolinas Medical Center, Charlotte, North Carolina, USA
E‐mail address for correspondence: [email protected]
Background: The medical management of rigidity and spasticity is often incomplete due to intolerance to one or more of the currently available medications. Most commonly oral antispasmodic medications such as baclofen, dantrolene and tizanidine result in fatigue or perceived weakness and these symptoms may offset a gain in functional ability.
Objective: We report here the experience of 20 consecutive patients with motor neuron disease, in our clinic, who received intrathecal (IT) baclofen as an alternative to medically refractory rigidity and/or spasticity.
Results: All patients who received the IT baclofen pump were resistant or intolerant to at least two available medications. Ten of the 20 patients we implanted had tried three medications without clinical benefit at maximum tolerated doses. Among the 20 patients we report here, one patient was unable to tolerate any dose of baclofen, 19 patients reached their maximum tolerated dose without a significant benefit and two of those patients received no benefit at all. Sixteen patients received dantrolene, with three unable to tolerate the medication and 13 unable to achieve a satisfactory benefit from maximum tolerated dose of dantrolene. Twelve patients were treated with tizanidine, five received no benefit, two were unable to tolerate it, and four were receiving sub‐optimal benefit at maximum tolerated dose.
Patients received the intrathecal baclofen pump with initial dosing of 50 mcg per 24 h period. This dose was increased incrementally until the maximal benefit was reached. The maximum dose adjustment we used was 50 mcg per titration step. The optimal therapeutic dose of IT baclofen varied greatly by patient (75 mcg to 1450 mcg daily). Intolerance to oral baclofen was not a predictor of IT dose or tolerance. IT dose delivery protocol also varied among our patients. Continuous steady delivery of the drug throughout the day was optimal in 65% of the patients, while periodic bolus delivery was needed in 35% of the implanted patients.
Oral anti‐spasmodic medication was used by seven patients after IT pump placement to achieve optimal benefit; however, significantly lower doses were used and side‐effects were not problematic. Most patients noted a decrease in fatigue and sedation with the decrease in oral medications. One patient noted an initial benefit from the IT pump, but did not note a long‐term benefit. No patients experienced complications from the surgery.
Conclusions: Clinical improvement (less rigidity and/or spasticity) was measurable in all patients implanted. Subjective improvement was reported in 90% (18/20) patients by 3.3 months (4–365 days) after implantation.
Overall, the IT delivery of baclofen via surgically implanted intra‐abdominal pump was well tolerated and beneficial to most refractory patients with rigidity and spasticity. Clinical benefit was usually realized by three months after implantation. This procedure should be considered as an aggressive treatment option in the appropriate clinical setting.