Some Neurodevelopmental Challenges Faced by Students
Despite the lack of consistency in the intellectual experiences of the students with BTHS, parents would do well to prepare for the kinds of neuro-developmental and learning issues their child(ren) might face. Some of the issues could be direct results of the symptoms of BTHS, and some of the issues might be caused by the medications the students have to take.
Students with BTHS often struggle to stay focused in classes when there is much movement and noise to distract them. Some teachers have noticed dramatic improvements in learning when students go from distracting classrooms to one-on-one lessons. Staying on task is another commonly noted challenge for students. Teachers should keep students with BTHS close to the front of the room where they can keep an eye on their progress on assignments and where the distractions are reduced.
A sizable portion of parents who responded to the educational survey noted that their children struggle with short and long-term memory issues[i]. Such problems might very well be a consequence of the fatigue, which would place a strain on anyone’s capacity for retaining information. Another possible cause for memory difficulties could be found in the effects of the medications that many students with BTHS take. Parents should always consult with their children’s doctors about the possible side effects of medications on their child’s memory and other cognitive functions. Ultimately, however, there are no clear answers yet for the cause of memory difficulties in individuals with BTHS.
Reading comprehension and retention are also noted by some parents as challenges for their children. Again, for many students, challenges in reading might be caused more by fatigue than by discernible learning difficulties, but some students do seem to have weaknesses in the verbal area. In the United States, once students with BTHS are identified as other health impaired, they are able to receive special education services, and this allows them to receive recorded texts through mobile apps such as Learning Ally (see references).
Listening to readings through an app, as opposed to reading the texts directly usually takes longer, but easily fatigued students might expend less energy in getting the work done. Each student needs to explore his options and choose the method of reading or listening to the text that best suits him. Easily distractible students might have an even harder time digesting material that comes to them orally as opposed to material that they read themselves.
Mathematics is an area of difficulty for many students with BTHS, but very little data exist to explain why this is. Possibly, it stands to reason that students with focus and short-term memory difficulties will struggle with the logical sequencing necessary in mathematical operations. Dr. Michele Mazzocco began investigating cognition patterns in BTHS in 2000. She published a preliminary paper in 2001[ii] that discussed the first evidence ever found that boys with BTHS might share some learning patterns as a result of BTHS. Further research demonstrated those with BTHS to have age appropriate cognition, vocabulary skills and reading skills[iii].
Dr. Mazzocco and her team characterized those with BTHS to have below-average performance in mathematics and selective difficulties in visuo-spatial skills that is not linked to impaired motor functioning from myopathy. Math difficulties are not evident in preschool but do emerge in most during kindergarten[iv]. In the Barth Syndrome Registry study, twenty-two of forty-six males older than age seven years reported some form of “learning disability”[v]. An estimated 33% of those diagnosed with BTHS require some type of educational accommodations[vi].
If parents find that their child is having substantial difficulty with any part of the learning process, they should consider having their child tested for his cognitive learning styles, strengths, and weaknesses. The testing does not commit parents to any course of action, but the results could supply helpful information as well as essential evidence as parents work with educators to find the best educational environment for their child. There are two options for parents in the United States: parents have the right to ask their local public school to test their child at no cost, or parents can find a private psychologist to do independent testing, which might be covered by insurance. Costs for a comprehensive round of tests and a full written analysis is generally thousands of dollars. So, it pays to be the squeaky wheel to try to get these costs covered through school or insurance.
If significant cognitive challenges are found in the testing, a student in the American public school system might be eligible for legal protection under the Individuals with Disabilities Education Act (IDEA). Protection under the IDEA supplies a student with an Individualized Education Plan (IEP), which is a written plan that outlines how the school is going to best meet the needs of an individual student. Sometimes, parents are afraid of having their child labeled as different, but these protections are more about providing students with accommodations than they are about labeling students. The vast majority of parents who filled out the educational survey noted their child had an IEP in place for his education.
[i] Cole, LK, Kim JH, Amoscato AA, et al. Aberrant cardiolipin metabolism is associated with cognitive deficiency and hippocampal alteration in tafazzin knocknown mice. Biochim Biophys Acta Mol Basis Dis, 2018 Oct;1864(10):3353-3367.
[ii] Mazzocco MM, Kelley RI. Preliminary evidence for cognitive phenotype in Barth syndrome. Am J Med Genet, 2001 Sep 1;102(4):372-8.
[iii] Mazzocco MM, Henry AE, Kelley RI. Barth syndrome is associated with a cognitive phenotype. J Dev Behav Pediatr. 2007;28(1):22–30.
[iv] Raches D, Mazzocco MM. Emergence and nature of mathematical difficulties in young children with Barth syndrome. J Dev Behav Pediatr. 2012;33(4):328–335.
[v] Roberts AE, Nixon C, Steward CG, et al. The Barth Syndrome Registry: Distinguishing disease characteristics and growth data from a longitudinal study. Am J Med Genet A. 2012;158A(11):2726–2732.
[vi] Clarke SL, Bowron A, Gonzalez IL, et al. Barth syndrome. J Rare Disord. 2013;8(23):1–17