Product information and disease description Place of product in therapy Approaches to treatment Current treatment options and standard of care

Multidisciplinary supportive care

5 evidence topics · 5 sources

Guideline scope and recommendation for multidisciplinary symptomatic treatment

“This guideline summarizes frequently encountered medical problems in the disease course of patients with classic and variant ataxia-telangiectasia, in the domains of neurology, immunology and infectious diseases, pulmonology, anaesthetic and perioperative risk, oncology, endocrinology, and nutrition.” (opens the source at this quote in a new tab)
“No curative therapy is available for ataxia-telangiectasia.” (opens the source at this quote in a new tab)
“Given the complexity and severity of the disorder, patients should receive optimal symptomatic treatment in the setting of a dedicated and experienced multidisciplinary team of health care professionals.” (opens the source at this quote in a new tab)

Supportive care and the specialties involved

“There is no cure for ataxia-telangiectasia.” (opens the source at this quote in a new tab)
“Supportive care to improve quality of life, maximize function, and reduce complications is recommended. This ideally involves multidisciplinary care by specialists in (pediatric) neurology, pulmonology, immunology, pediatrics (for children) and internal medicine (for adults), rehabilitation medicine, and professionals in physical therapy, speech-language therapy, occupational therapy, and nutrition.” (opens the source at this quote in a new tab)
“Most of the guidelines recommended for the management of health-related problems in individuals with ataxia-telangiectasia (A-T) are expert and evidence based, due to a lack of clinical trials” (opens the source at this quote in a new tab)

Manifestations that respond to treatment and those that do not

“The management and treatment of A-T is symptomatic and supportive.” (opens the source at this quote in a new tab)
“Treatment of the neurologic problems associated with A-T is symptomatic and supportive, as there are no treatments known to slow or stop the neurodegeneration. However, other manifestations of A-T, e.g. immunodeficiency, pulmonary disease, failure to thrive and diabetes can be treated effectively.” (opens the source at this quote in a new tab)

Status of the United Kingdom clinical guidance for children

“There are currently no fully-researched and evidence-based clinical guidelines on the treatment of people with A-T published anywhere.” (opens the source at this quote in a new tab)
“What this document does aim to do is to provide suggestions for care based on the experience and current state of knowledge of doctors and therapists with considerable experience of seeing patients with A-T at what is the world’s longest-established multi-disciplinary A-T clinic.” (opens the source at this quote in a new tab)

Effect of supportive and multidisciplinary care on survival and quality of life

“Earlier diagnosis, supportive care and a multidisciplinary approach to patient follow-up can improve survival and quality of life.” (opens the source at this quote in a new tab)