The Burden of Primary Caregivers of Spinal Muscular Atrophy Patients and Their Needs
Condition: Spinal Muscular Atrophy; Caregiver Burden
MeSH terms: Muscular Atrophy; Muscular Atrophy, Spinal; Atrophy; Caregiver Burden
Study type: Observational

Summary: 
      The purpose of this study is to assess carer burden, needs, and expectations of Spinal
      Muscular Atrophy Parents
    

Detailed description: 
      Spinal muscular atrophy (SMA) is a group of neuromuscular diseases involving the spinal cord
      anterior horn cells and brain stem motor nuclei. It is followed by programmed cell death. It
      progresses with symmetrical weakness and atrophy of voluntary muscles throughout the body. It
      leads to a decrease in mobility with progressive loss of power, and pulmonary function
      impairment with the involvement of the respiratory muscles.

      Families who are confronted with these problems that arise after the birth of the baby or
      shortly after birth have a serious adaptation problem. When the course of the disease and its
      incurability are explained, patients and their families are under severe stress. Physical and
      emotional health is worse than normal healthy growing children's parents.

      They need information, psychological and social support. The role of the family is very
      important in the life of children with disabilities. Family-oriented care; has been developed
      to facilitate the care process of children with special needs and to help their families. The
      main characteristics of the family-oriented approach are that families know their children in
      the best way, that each family is unique and different, and that family and community support
      is provided for the child's functionality. The assessment of family function helps the
      planning and management of treatment according to the concerns of the family. The education
      of the family, the socio-cultural structure and the psychological approach of the parents
      play an important role in the development of the child with SMA.

      The aim of this study was to evaluate the care burden, needs and expectations of SMA parents
      and to reveal the problems clearly; so that this information can be used in rehabilitation
      planning and interpretation of results.
    

Intervention name(s): Zarit Burden Interview Scale; Family Needs Survey (FNS); Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND); Hammersmith Functional Motor Scale Expanded (HMFSE)
Intervention type(s): Behavioral; Behavioral; Other; Other
Intervention description(s): It is a questionnaire consisting of 22 items. A minimum score of 0 and a maximum score of 88 can be obtained. The higher the scale score, the higher the difficulty experienced.; It is a questionnaire consisting of 35 items prepared to determine the requirements. In the FNS survey; The points given to the items can be directly collected and the amount of family needs can be determined over the total score. The lowest score that can be obtained from FNS is 29 and the highest score is 87. As the scores obtained from the scale and subscales increase, it can be said that the families' level of necessity increases.; CHOP-INTEND uses a scale of 0-64 points. Higher scores indicate better motor function. It is used to reliably measure natural motor function decline in infants with SMA Type 1.; It is an extended version of the 20-item HFMS. Based on another scale known as GMFM, 13 items were added and expanded HFMS to include these elements. Therefore, HFMSE is a scale used to evaluate motor functions in types 2 and 3 SMA. It contains 33 items with a total score of 66 on a scale of 0, 1, 2.
Observational model: Cohort
Time perspective: Prospective
Sampling method: Probability Sample
Study population: 
        Patients with SMA type 1, type 2 or type 3 between the ages of 0-18 and their caregivers.
      
Keywords: Spinal Muscular Atrophy; Caregiver Burden; Disabled persons; Caregivers