MIBRELA - Brazilian Interactive Manual of ALS

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the progressive loss of motor neurons in the cortex, brainstem and anterior horn of the spinal cord, leading to atrophy and generalized muscle weakness.

The ALS patient, as Spinal Muscular Atrophy (SMA) patient, faces a disease of unknown origin, unlikely cure and uncertain prognosis. Recurrently, there is frustration with the speed of muscle loss and increased disability, emerging the need for effective guidance by a multidisciplinary team for the patient and family.

The progression of the disease causes a profound impact on people’s lives. There are numerous precautions to be included in the daily lives of these patients, for example, the use of prescribed drugs, changing habits related to activities of daily living (ADL), adapting to the new socioeconomic circumstances, coping with emotional consequences and participating in decisions associated with treatment. On the other hand, it is noteworthy that the health system is responsible for preparing patients for this care, being one of the strategies used to invest in their education to guide, encourage and support the appropriate care.

Health education, facing a nosological process, can result in benefits in several ways, such as improving clinical symptoms, maintaining and adapting physical and functional limitations resulting from it, and also in the emotional aspect, facilitating the acceptance of the disease, which may favorably interfere with lifestyle. Additionally, the patient education programs promote decrease of the direct and indirect costs of the health system, by reducing the need for medical appointments and the use of hospitals.

However, the patient education method for a more efficient caregiver is not yet clear. This educational guidance is usually given through verbal communication, which is often not fully understood and/or assimilated. Written information represents an important adjuvant in this process, as long as it is clearly understood by the target population. The study of other educational strategies that could provide a greater number of access to information in the different stages of the disease is necessary to investigate which method could facilitate greater adherence to the guidelines.

Addressing complex diseases, such as Spinal Muscular Atrophy (SMA), Amyothophic Lateral Sclerosis (ALS) and other Neuromuscular Disorders (NMD), requires effective communication for the transmission of reliable information and health care. Patients are treated, usually in an outpatient setting, and the service time may not be enough to effectively transmit information about the disease and its management by the entire multidisciplinary team involved.

It is widely known that the caregiver is a family member without previous healthcare training who assumes responsibility for the patient with SMA/ALS/NMD. This caregiver faces the challenge to search and select the necessary information and knowledge to anticipate the required care activities.

The elaboration of the MIBRELA product (interactive manual of amyotrophic lateral sclerosis), that is, a Brazilian website/platform for guidance for patients with amyotrophic lateral sclerosis, was created from the need to provide reliable and selected information by specialized professionals for patients and family caregivers.

The manual contains 47 descriptive information on energy conservation techniques, indications for adaptations, guidelines for the proper use of upper limb orthotics, ergonomic changes in the home and prescription of postural adjustment systems.

This information is organized into seven domains, and may or may not be checked by the therapist according to the individual need of each patient:

• Power: thicken the cutlery, bend the spoon handle, raise the plate to reduce the angle between the table and the mouth, use a light cup preferably made of plastic and with a large handle,
• Hygiene: raise the toilet seat, install support bars near the toilet and inside the shower box, use a chair for hygienic bath, use an electric toothbrush, install bidet or specific showers on the toilet, thicken a toothbrush and razor, stretch and/or thicken a comb or hairbrush handle, sit down to brush teeth or shave with support elbows on edge of sink, use mirror behind the faucet, sit down to take a shower - energy consumption is lower, put the "pool spaghetti" in the back of bath chair, to enlarge the torso and neck support surface, place the soap inside the pantyhose (cut of a leg) and tie it to the shower or bath chair;
• Clothing: put "key rings" on the zippers, replace buttons with Velcro, use extender (80 cm wood, "plant hook", magnet, shoehorn), use sock placer, use two chairs to assist in putting on and taking off the clothing, start removing and putting the blouse on the side that has more difficulty, use elongated shoehorn, replace shoelaces with other models;
• Communication: writing pad, everyday figures, adapter to indicate figure, use of biblical support to support the book (cushion, reading position facing the horizon);
• Leisure and computing: adapters for keyboard and mouse (customised), use of virtual keyboard (computer program), increase the keyboard sensitivity (computer program), use deck support, use adjustable and/or cut-out table in half-moon with support for the elbows (for several activities), thicken or adapt pen and/or brush, use key adapter;
• Positions: raise the bed, sofa or chair, sit close to the wall to support the head on the pillow, use cervical collar (look at the time indication), use purse, pockets or chair for upper limb support, not leaving the upper limbs without support (joint protection technique), upper limb orthoses (use two hours in the morning, two hours in the afternoon, to sleep all night and for specific activities);
• Locomotion (detailed description of the locomotion).

Use a straw, cut a half-moon in the cup to avoid hyperextension of the neck, nail two nails on a wooden board to facilitate to cut food, leave all necessary objects within reach;

 Besides this set of information, the website will offer a series of digital services for users. Among these services, we have a social network to encourage the exchange of experiences and interaction. The system will also host a marketplace for the sale and donation of assistive equipment, since the rapid progression of the disease demands the acquisition of such equipment.

To ensure the safety of users, the access will occur through IPG authorization for people with SMA/ALS/NMD diagnosis, relatives and caregivers.

Neuromuscular disorders (NMD) such as ALS and SMA causes a great impact on patients and their families. However, it is possible to minimize the impact supporting families through knowledge about caregiving. It is widely known that the caregiver is a family member without previous healthcare training who assumes the responsibility for the patient with ALS, SMA or other NMD. This caregiver faces the challenge to search and select the necessary information and knowledge to anticipate the required care activities.

In recent years, increasingly easy access to the internet has led patients and families to search and obtain health information, thus representing an interactive alternative. However, it is very important to emphasize that health information available on the internet can often be distorted, false or simply not applied to that patient. Moreover, it can be very complex to choose a direction to follow in the massive volume of information on the internet.

Further, patients need a lot of support from assistive technologies and these resources are not easily available for the population. Thus, patients and family members should seek appropriate guidance and help. In turn, the rapid progression of the disease makes the possibility of exchanging and donating equipment very dynamic. Therefore, the project aims to create a safe environment to promote these interactions.

IPG is a Civil Society Organization of Public Interest, a non-profit philanthropic entity with certification as an entity for the Defense of Human Rights of the State of São Paulo, founded in 2005, acting as a Reference Center for Knowledge, Research and Care on Spinal Muscular Atrophy (SMA) an more recently other Neuromuscular Disorders. 

Our mission is to develop humanization programs and projects, disseminate information that help to improve the quality of life of people with ALS and other NMD, qualify the multidisciplinary care network and gather investments in scientific research to find a cure for these diseases. Our vision is to be recognized as a Research Center and reference in ALS/NMD in Brazil and the most effective way to strengthen our values is through our basic principles of preserving the legacy left by its creator Paulo Gontijo, with honesty, respect, engagement, responsibility, transparency and, above all, love for others. The Institute has more than one thousand registered people receiving our assistance and 1.3 million people impacted by our actions.

Besides support actions to offer some services, hope, attention and affection for people with the aforementioned diseases and their families, IPG also provides free social guidance and specialized service, with the aim of promoting access to the benefits of the Rights Guarantee System. The Social Guidance.

IPG is prepared to meet legal guidelines and demands presented by patients and their families to access and enforce their social rights related to their condition. For this, individuals with SMA, ALS and NMD must be assisted and registered by the Institute's Social Assistance team, and after screening and in specific cases, they are referred for free legal assistance.

IPG thinks about the well-being of people diagnosed with SMA, ALS and NMD in case of vulnerability, and so that people with a diagnosis can interact with their families and loved ones, it maintains equipment loan lending, including: wheelchair, hospital bed, assistive technology equipment, breathing apparatus, among others. Thus, it is possible to allow people, even in advanced stages of the disease, to have a little better quality of life and even to communicate, interact with the world, breathe with ventilatory support equipment and live better.

Many of these equipments are of pivotal importance, remembering it even help people to leave house.

The Volunteer Project of Paulo Gontijo Institute is formed by committed people who work in several actions, promoting hope, dedicating attention and care to patients diagnosed with NMD and their families. Currently, more than 70 people work on different fronts, such as organizing and holding events, fundraising, legal advice, multidisciplinary care at the patient’s home and various services at the IPG headquarters.

Our goals are related to:

NMD disease awareness.

Welcoming and assisting, free of charge and without any discrimination, people diagnosed with SMA/ALS/DNM and their families, in order to support access to social policies and their rights.

Encourage scientific researches that investigates new treatments and discoveries for the cure of SMA/ALS/DNM.

Provide assistance bringing technology, medical and digital devices to them.

We already have a website with important tips for patients and caregivers/relatives of patients who are in severe clinical condition of disease progression. We understand that this is an important first step whose focus is guidance and education envisioning the best quality of life for patients and families. However, unfortunately our public health system cannot meet all the demands that these patients have and we want to expand our project by providing assistance to the most vulnerable patients, bringing technology, medical and digital devices to them.

Currently our website is open to the public and anyone can access it and get directions for free. We have about 700 patients registered in our association and most of them don't have complete assistance and access to multidisciplinary care and access to technologies due to financial vulnerability. Our results report is available at the link: RELATORIO IPG_2021

We want to share this idea with as many people as possible, because we believe that this way it can be possible to join forces to enable the necessary resources for our patients in vulnerable situations, not only for the conditions of the disease that is progressive and fatal, but also because of the scarcity of family resources and the limitations of our public health system to meet all demands. There is still a very large limitation of access to technological tools not only for patients, but also for health professionals who care for these patients. We want to be able to give patients access to technologies that facilitate day-to-day activities for them and their families, such as communication tools for patients who can no longer speak, tools that allow some movement and the handling of the bedridden patient, among other needs.

We have a technological partner (Interactive Spiral) and with this we guarantee content with digital accessibility for all, promoting inclusion to patients who no longer have mobility to type, for example. Our team is diverse, we do not distinguish color, race, age, disability, sexual orientation, social class, etc.

IPG assists patients with Amyotrophic Lateral Sclerosis and other progressive and debilitating neuromuscular diseases and also supports the families of these patients. We rely on the work of social workers, psychologists and health professionals to assist patients and families in their demands. We have a very important educational and care role.

Our results report is available on RELATORIO IPG_2021.

IPG - Instituto Paulo Gontijo

We are always attentive to possible notices for application, whether public or private, national or international. We have the support of a person who does fundraising projects.
We mediate donations of equipment and Devices from one patient to other patients.
We work transparently and make our reports available to the population.