Psychological interventions to control emotional stress can help relieve the anxiety that parents and children with spinal muscular atrophy (SMA) type 1 feel during Spinraza administration through spinal taps, an Italian study reports.
Researchers recommend that similar interventions become part of the standard care in infants and children with SMA during Spinraza treatment.
The study, “Intrathecal administration of Nusinersen in type 1 SMA: successful psychological program in a single Italian center,” was published in the journal Neurological Sciences.
The study stemmed from the experience of a medical team at a center in Messina, Italy, while accompanying the first SMA type 1 patients who started intrathecal (via the spinal canal) administration of Spinraza (nusinersen), after Italy urged its access under Biogen’s expanded access program to young children with this severe form of SMA.
The team investigated the effects of an anxiety-relieving program given to children and their parents while the children underwent the stressful process of repeated Spinraza intrathecal administrations.
This type of delivery requires periodic spinal taps, or lumbar punctures, to inject the medicine into the spinal canal. From here, Spinraza enters the cerebrospinal fluid reaching motor neurons, the primary cells affected in SMA.
The study enrolled 16 children and teens with SMA type 1 (11 boys, five girls), ranging in age from from 2 months to 14 at the start of Spinraza therapy.
The first four doses of Spinraza were administered at day one, day 15, 30, and 60; thereafter, a maintenance dose was administered once every four months. During treatment 14, parents and seven children (ages 6 and older) were screened for anxiety using specific measures.
For each Spinraza administration, patients were admitted to the hospital the day before and left the day after. Before the fifth treatment, researchers initiated a psychological intervention plan based on cognitive-behavioral therapy and consisting of several activities before, during, and after the spinal tap.
For the youngest patients, the plan included telling fairy tales and playing their favorite cartoon songs, and older children were taught coping skills and solved fun riddle quizzes. Parents and children were also accompanied by a psychologist throughout the process.
In the first four treatments, before the psychological plan was initiated, emotional stress, anxiety, and fear increased in parents and their children. Although distress decreased after Spinraza administration, it remained heightened above normal for most individuals.
Some children even developed negative reactions to stress such as sleeping disturbances, irritability, and separation anxiety.
But after the psychological support plan started, before the fifth treatment, both parents’ and childrens’ behavior changed. Parents became less anxious and worried and begun to feel more self-confident and secure.
Childrens’ anxiety levels were also closer to normal, and they cooperated more with medical procedures both before, during, and after the spinal tap.
After the psychological plan was implemented, “children became able to use their coping skills in a better way than before and referred greater serenity since the night before [lumbar puncture], less agitation and tension, and a general state of well-being,” researchers wrote.
“Most children showed pleasure and interest during hospitalization and, when leaving the hospital, they wished to return soon to continue playing,” they added.
Although more studies are necessary to confirm the results, the authors drew attention to the potential benefits of such types of psychological interventions, which can contribute to “a better quality of psychological health of type 1 SMA patients, also in light of the commercial availability of Nusinersen in many countries and the need of recurrent [lumbar puncture].”