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Yimaitong edited and sorted, please do not reprint
without authorization.
Xiaonong Unlike other diseases that only affect a single organ, SMA can cause dysfunction in multiple systems such as respiratory, digestive, and bone in addition to motor dysfunction, involving almost all organs
.
Multidisciplinary collaboration is the key to SMA management, which helps to assess the condition comprehensively and individualize the treatment of patients, so as to better improve the treatment effect and quality of life
.
As an important part of multidisciplinary comprehensive management, rehabilitation therapy can intervene and delay the progression
of the disease through regular physical therapy, correct use of braces or orthoses, and regular exercise training.
Professor Kaishou Xu
"Expert Consensus on Rehabilitation Management of Spinal Muscular Atrophy" is to enable SMA patients to receive standardized rehabilitation management and improve the efficacy
.
In recent years, consensus on multidisciplinary diagnosis and treatment of SMA has been released at home and abroad, but there is no consensus on guidelines for SMA rehabilitation management, and the release of this consensus fills this gap
.
At present, there are many and widely distributed SMA patients in China, but the medical conditions and levels in different regions of China are uneven, and the formulation and release of this consensus provides a standardized guidance for hospitals in different regions, as well as clinicians and rehabilitation professionals, to better evaluate and treat
diseases.
.
Water exercise is recommended and active movement of the upper limbs is promoted; ➤For those who can sit alone, maintain recovery or improve function as much as possible, improve mobility ability, prevent or reduce muscle atrophy, joint contractures and spinal deformities
.
Strength training for the waist and abdominal trunk muscles and resistance muscle strength training are recommended; ➤For those who can walk alone, it is necessary to maintain or improve function, mobility and joint range of motion, and prevent secondary muscle atrophy and skeletal deformity
.
more emphasis on balance training and aerobic exercise; ➤ If the patient has other discomfort, it should be recommended to transfer to a department or invite the corresponding specialist medical staff for treatment
.
Professor Kaishou Xu
Xiaonong ➤ In order to make more rehabilitation doctors aware and understand how to manage the disease, we have done a lot of training work
.
At the same time, it is hoped that rehabilitation doctors will learn and communicate with other specialists, improve their professional level, and better serve
SMA patients.
➤SMA requires more professional rehabilitation management
than other diseases.
Therefore, it is hoped that each professional institution has at least 2~3 professionals responsible for managing patients, including assessors, therapists and nurses
.
➤Due to the particularity of SMA rehabilitation treatment, patients after diagnosis should be transferred to the rehabilitation department as soon as possible after intrathecal injection treatment to form a seamless docking
.
At the same time
, the rehabilitation treatment room should evaluate and train patients according to the guidance of expert consensus.
➤Family rehabilitation guidance is essential for SMA patients, and rehabilitation training should be integrated into daily life to form a model of institutional rehabilitation combined with family training
.
of the efficacy of current drugs combined with rehabilitation therapy.
➤Secondly, it is necessary to build a professional SMA follow-up registration system to facilitate professionals, parents and community workers to carry out rehabilitation management
throughout the life cycle.
➤In addition, it is necessary to further develop relevant rehabilitation equipment and management technologies to provide scientific basis for clinical practice and better improve the efficacy
of rehabilitation.
D.
, professor, doctoral supervisor, director of the rehabilitation department of Guangzhou Women and Children Medical Center
without authorization.
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder
characterized by progressive muscle weakness and muscle atrophy.
In addition to affecting motor function, SMA can also lead to multi-system dysfunction such as respiration and digestion, and long-term standardized rehabilitation management is essential to improve the prognosis of children with SMA1
.
In 2019, the world's first SMA disease-modifying treatment (DMT) drug nocinaxen was approved in China, breaking the dilemma
that there is no drug available for SMA patients in China.
With the clinical application of DMT drugs, a new SMA phenotype that is different from the natural course of the disease has emerged, which brings new challenges to rehabilitation management.
At the same time, more patients are benefiting from current treatment drugs, extending their lives and preserving their functions, highlighting the importance of rehabilitation1
.
In this context, the Rehabilitation Group of the Pediatrics Branch of the Chinese Medical Association and the Physical Therapy Special Committee of the Chinese Association of Rehabilitation Medicine recently issued the Expert Consensus on the Rehabilitation Management of Spinal Muscular Atrophy to further standardize the rehabilitation assessment and treatment of SMA and give full play to the therapeutic role
of rehabilitation combined with DMT drugs.
To this end, Yimaitong is honored to invite Professor Xiao Nong of the Children's Hospital Affiliated to Chongqing Medical University and Professor Xu Kaishou of Guangzhou Women and Children's Medical Center to conduct exclusive interviews, and the two professors shared their insights on SMA rehabilitation treatment with us, calling on more doctors to understand, pay attention to and actively participate in SMA rehabilitation treatment, and build a long-term benefit path
for SMA patients.
SMA is a progressive genetic disease that originates in the central nervous system and endangers multiple systems throughout the body, and in addition to drug therapy, multidisciplinary comprehensive management is also essential
for patients with SMA.
What are the benefits of multidisciplinary management for patients?
Xiaonong Unlike other diseases that only affect a single organ, SMA can cause dysfunction in multiple systems such as respiratory, digestive, and bone in addition to motor dysfunction, involving almost all organs
.
Multidisciplinary collaboration is the key to SMA management, which helps to assess the condition comprehensively and individualize the treatment of patients, so as to better improve the treatment effect and quality of life
.
As an important part of multidisciplinary comprehensive management, rehabilitation therapy can intervene and delay the progression
of the disease through regular physical therapy, correct use of braces or orthoses, and regular exercise training.
Professor Kaishou Xu
Because SMA involves multisystem impairment and complications, multidisciplinary integrated management is essential
for patients with SMA.
At present, it is recommended to establish SMA multidisciplinary joint outpatient clinic to provide one-stop services
for patients.
Multidisciplinary management avoids SMA patients from moving to multiple departments, thereby improving the diagnosis and treatment experience and reducing the financial burden
of patients to a certain extent.
Among them, rehabilitation training is indispensable in multidisciplinary management, and studies have shown that rehabilitation training can benefit SMA patients in muscle strength and function
.
At present, in the mode of SMA DMT drugs combined with targeted rehabilitation training, the survival time of SMA patients can be further prolonged, and the quality of life has been further improved
.
Yimaitong: With the gradual improvement of access to SMA treatment drugs, what are the changes in the needs, objectives and strategies of rehabilitation treatment for SMA patients in the new diagnosis and treatment environment?
The advent of DMT drugs has changed the natural course of SMA, and this change has prompted many departments to participate in the management of the whole course of the patient's
disease.
Rehabilitation also goes from behind the scenes to the foreground, from assessment to diagnosis to treatment, and then to post-treatment assessment and guidance, throughout the whole process
of disease management.
The purpose of rehabilitation treatment for SMA patients is mainly to reduce or prevent SMA complications, delay disease progression, optimize drug treatment effects, and improve the quality of
life.
With the clinical application of DMT drugs, the life expectancy of SMA patients has been extended, the function has been preserved, the demand for rehabilitation treatment has also increased, and new problems
have emerged.
In order to further standardize the rehabilitation evaluation and treatment of SMA and give full play to the therapeutic role of rehabilitation combined with DMT drugs, we formulated the Expert Consensus on Rehabilitation Management of Spinal Muscular Atrophy, in order to standardize and guide the rehabilitation assessment and treatment
of children with SMA.
At the same time, with the joint help of China Primary Health Care Foundation and Biogen and other institutions, a large number of SMA professional assessment tools have been introduced for the diagnosis and treatment of patients, which is of great help
to patients.
At present, more SMA patients can be seen in the clinic for diagnosis and treatment, and in the past, most type 1 SMA patients will reach the end of life within 2 years old, so it is rare in clinical practice
.
Secondly, patients with type 1 SMA may not have complications such as scoliosis or joint dislocation before reaching the end of life because there is no drug to treat in the past, but SMA is a progressive disease, and as the disease progresses, complications such as scoliosis and joint contracture may occur in the future, and the need for rehabilitation management has changed
.
Therefore, not only patients with type 2 and 3 SMA need to pay attention to the rehabilitation management of scoliosis and joint contractures, but also need to carry out corresponding rehabilitation treatment
such as orthosis use, motor function training and positioning for type 1 SMA patients.
Rehabilitation can improve motor function, muscle strength and endurance in SMA patients, improve joint range of motion, and make musculoskeletal biomechanics better
.
In the era of DMT treatment, it is more necessary to combine rehabilitation management to make drugs play a better effect, and at the same time improve patient compliance, thus forming a virtuous circle
of disease management.
Yimaitong: The publication of the Expert Consensus on Rehabilitation Management of Spinal Muscular Atrophy will further standardize the rehabilitation treatment of SMA, please talk about how to better improve the value of rehabilitation treatment for SMA patients?
The treatment of SMA is inseparable from the whole process of rehabilitation management, so it is necessary to let the rehabilitation doctors and other department doctors involved in patient management know that before treatment, an objective and effective comprehensive assessment of SMA patients should be carried out to clarify the stage of the patient's condition and the degree of damage to each organ system, which will help to formulate an individualized rehabilitation treatment plan
.
SMA has the particularity of muscle fatigue, so SMA rehabilitation characteristics are different from
other diseases.
First of all, rehabilitation should be done when the patient is happy and willing; Secondly, when choosing rehabilitation items in functional activity training, attention should be paid to easy first, then difficult, gradually increase exercise intensity, and avoid excessive
exercise.
In addition, in addition to regularly going to the hospital for intrathecal injection, SMA patients spend more time at home, so it is more necessary to guide patients on how to recover at home and parents on how to conduct care assessments
.
At present, with the help of institutions such as the China Primary Insurance Foundation and Biogen, we are also compiling rehabilitation guidance manuals for medical institutions and families, guiding relevant rehabilitation therapists to carry out treatment and help parents better care for patients
.
"Expert Consensus on Rehabilitation Management of Spinal Muscular Atrophy" is to enable SMA patients to receive standardized rehabilitation management and improve the efficacy
.
In recent years, consensus on multidisciplinary diagnosis and treatment of SMA has been released at home and abroad, but there is no consensus on guidelines for SMA rehabilitation management, and the release of this consensus fills this gap
.
At present, there are many and widely distributed SMA patients in China, but the medical conditions and levels in different regions of China are uneven, and the formulation and release of this consensus provides a standardized guidance for hospitals in different regions, as well as clinicians and rehabilitation professionals, to better evaluate and treat
diseases.
Medical Pulse: The clinical manifestations of SMA are heterogeneous, and the functional status and disease severity of different patients are different
.
Can you talk about how to manage the rehabilitation of motor function for patients with different conditions?
In terms of positioning and assistive device applications:
➤ For those who cannot sit alone, most of the time is in the lying position, the goal is to improve function, alleviate obstacles, and optimize tolerance in different positions.
Water exercise is recommended and active movement of the upper limbs is promoted; ➤For those who can sit alone, maintain recovery or improve function as much as possible, improve mobility ability, prevent or reduce muscle atrophy, joint contractures and spinal deformities
.
Strength training for the waist and abdominal trunk muscles and resistance muscle strength training are recommended; ➤For those who can walk alone, it is necessary to maintain or improve function, mobility and joint range of motion, and prevent secondary muscle atrophy and skeletal deformity
.
more emphasis on balance training and aerobic exercise; ➤ If the patient has other discomfort, it should be recommended to transfer to a department or invite the corresponding specialist medical staff for treatment
.
Professor Kaishou Xu
The clinical manifestations of SMA are heterogeneous, and SMA is divided into 4 types
from severe to mild according to the age of onset and the maximum motor function obtained.
Patients with SMA of different types have different functional status
.
At the same time, whether the treatment is treated or not and whether the treatment is standardized also affects the functional status
of the patient.
Based on the different rehabilitation goals of different functional states, it is necessary to carry out targeted positioning and assistive device application for patients to improve their postural tolerance
.
Secondly, targeted exercise training is required, including active exercise, anti-gravity exercise, water exercise, intelligent robot-assisted walking training, walking, cycling, and neuromuscular electrical stimulation that does not cause fatigue
.
In addition, corresponding swallowing and respiratory training is also required in daily life, such as manual chest physiotherapy, to promote airway clearance and avoid suffocation
.
Yimaitong: You have rich clinical experience in the field of pediatric rehabilitation, please talk to us about how to better improve SMA rehabilitation management, and the development direction of SMA rehabilitation management in the future?
Xiaonong ➤ In order to make more rehabilitation doctors aware and understand how to manage the disease, we have done a lot of training work
.
At the same time, it is hoped that rehabilitation doctors will learn and communicate with other specialists, improve their professional level, and better serve
SMA patients.
➤SMA requires more professional rehabilitation management
than other diseases.
Therefore, it is hoped that each professional institution has at least 2~3 professionals responsible for managing patients, including assessors, therapists and nurses
.
➤Due to the particularity of SMA rehabilitation treatment, patients after diagnosis should be transferred to the rehabilitation department as soon as possible after intrathecal injection treatment to form a seamless docking
.
At the same time
, the rehabilitation treatment room should evaluate and train patients according to the guidance of expert consensus.
➤Family rehabilitation guidance is essential for SMA patients, and rehabilitation training should be integrated into daily life to form a model of institutional rehabilitation combined with family training
.
At the same time, with the participation of the multidisciplinary diagnosis and treatment team, a targeted forward-looking rehabilitation strategy is formulated, and in the "hospital-community-family" rehabilitation model, the community and family can continue the rehabilitation after discharge, and patients receive long-term standardized professional rehabilitation management and home care
.
We hope to provide SMA patients with more professional, effective, humanistic, economical and convenient services, and maximize the functional status and quality of life of patients, which is also our aim!
SMA rehabilitation management is a new challenge we have faced in recent years, and it is also a core issue of disease management:
➤First of all, it is necessary to carry out scientific education on diseases for patients and their families, so that they can have a correct understandingof the efficacy of current drugs combined with rehabilitation therapy.
➤Secondly, it is necessary to build a professional SMA follow-up registration system to facilitate professionals, parents and community workers to carry out rehabilitation management
throughout the life cycle.
➤In addition, it is necessary to further develop relevant rehabilitation equipment and management technologies to provide scientific basis for clinical practice and better improve the efficacy
of rehabilitation.
Introduction by experts
Professor Xiaonong
From June 2004 to May 2005, he was sponsored by China Scholarship Council to do neuroimmunology research at the Institute of Neurobiology, Munich Marx-Planck-Institute, Germany
Standing Director of Chinese Rehabilitation Society, Leader of Rehabilitation Medicine Group of Pediatrics Branch of Chinese Medical Association
Director of the Chinese Anti-epilepsy Association, Vice Chairman of the Rehabilitation Assessment Professional Committee of the Chinese Association of Rehabilitation Medicine
Director of China Association for the Rehabilitation of the Disabled, Vice Chairman of the China Children's Neurorehabilitation Committee
Standing Committee Member of Chinese Rehabilitation Medicine Burn Treatment and Rehabilitation Branch
Chairman of Chongqing Rehabilitation Society, Vice Chairman of Physical Medicine and Rehabilitation Society of Chongqing Medical Association, Chairman of Children's Rehabilitation Special Committee of Chongqing Rehabilitation Association
Editorial Board Member of Chinese Journal of Physical Medicine and Rehabilitation, Executive Editorial Board Member of Journal of Pediatric Pharmacy, Reviewer of Chinese Tissue Engineering Research and Clinical Rehabilitation, Neural Regeneration Research, Psychiatry Research, Journal of Paediatrics and Child Health
Introduction by experts
Professor Kaishou Xu
D.
, professor, doctoral supervisor, director of the rehabilitation department of Guangzhou Women and Children Medical Center
The first batch of outstanding young medical talents in Guangdong Province and key medical talents in Guangzhou
He presided over 16 national natural and provincial and municipal science and technology projects
Presided over and participated in the formulation of 14 children's rehabilitation guidelines and expert consensus
He has won 4 provincial and municipal science and technology awards, 5 national patents and 2 computer software copyrights
He has published more than 60 papers in domestic and foreign journals such as Brain as the first or corresponding author
He is the chief editor of 4 monographs including "Clinical Work Guide for Rehabilitation Therapists - Physical Therapy Techniques for Children's Diseases"
Deputy leader of the rehabilitation group of the Pediatrics Branch of the Chinese Medical Association
Vice Chairman of the Physical Therapy Special Committee of the Chinese Association of Rehabilitation Medicine and Chairman of the Pediatrics Group
Chairman of the Community Rehabilitation Branch of Guangdong Medical Association
Chairman of the Pediatric Cerebral Palsy Rehabilitation Committee of Guangdong Disabled Persons' Association
References:
1.
Rehabilitation Group of Pediatrics Branch of Chinese Medical Association, Physical Therapy Special Committee of Chinese Rehabilitation Medical Association.
Expert consensus on rehabilitation management of spinal muscular atrophy[J].
Chinese Journal of Pediatrics,2022,60(09):883-887.
)
