Relevancy to SLP Practice


“A speech-language pathologist is responsible for the diagnosis, prognosis, prescription, and remediation of speech, language, and swallowing disorders” (“Definitions of the Professions," 2014). Speech-language pathologists (SLPs) are educated and trained to look at a person’s behavioral issues, communication abilities, their style of learning, academic achievements, and other organic variables to determine a logical diagnosis of a speech, language, or swallowing disorder if one is present. SLPs should be aware of the characteristics of Velocraniofacial Syndrome (VCFS) accompanied by the presence of cleft palate and recognize the appropriate signs when they are present (Carneol, Marks, & Weik, 1999).

VCFS causes numerous speech, language, hearing, and swallowing deficits. Hypernasal speech is almost always a prominent feature of this disorder. Speech delay is also often apparent, especially referring to expressive language (Pike & Super, 1997). According to Morrison (n.d.), some children with VCFS may be non-verbal until they are two or three years of age. Although expressive language delay is usually higher, receptive language, as well as IQ scores are also reduced. Due to incompetency of the velopharyngeal mechanism, many articulation errors can be seen. (Fullman & Boyer, 2012). Quite often, other phonological or articulation disorders can also be seen in clients with VCFS, such as dyspraxia of speech, dysarthria, a high pitched voice, and phonological disorders or delays. Hearing loss due to otitis media may also affect clients with VCFS from time to time (Carneol et al., 1999). Speech-language pathologists provide remediation for all of these types of disorders by using different therapy approaches. 

VCFS also causes swallowing and feeding difficulties. Carneol et al. (1999) reported that 90% of those who have VCFS also have pharyngeal hypotonia. This lack of movement in the pharyngeal mechanism makes it difficult for these clients to swallow efficiently. Clients with VCFS may also have other difficulties that may adversely affect their ability to swallow or feed effectively. Some of those may be vascular anomalies, airway compromise, irritable temperament, palatal and pharyngeal anomalies, chronic constipation, and slow gastric emptying (Fullman & Boyer, 2012). SLPs should be involved in the treatment of swallowing and feeding difficulties for these clients. Some techniques that may be recommended are using a botulinum toxin A injection in order to relax the upper esophageal sphincter, adjusting behavior, or adjusting diet, perhaps by adding more strongly flavored foods in order to compensate for hyposensitivity within the pharynx. Most importantly, an SLP should work closely with a team of professionals, as well as with the client’s family in order to provide the best care possible (Fullman & Boyer, 2012).

One of the primary symptoms of VCFS is cleft palate. Because of malformations in the oral cavity, hearing loss, and repeated invasive surgeries, babbling in babies with cleft palate is typically delayed or reduced (Chapman, Hardin-Jones, Schulte, & Halter, 2001). Any child with a cleft is likely to have a resonance, language, voice, or articulation disorder (Sharp, Daily, Moon, 2003). Children with cleft palate may also suffer from pragmatic or social delays due to their other cognitive and language issues. Younger school-aged children may show adequate ability to keep up with their studies, but as the children get older and the academics become more difficult, these children tend to fall behind (Hardin-Jones & Chapman, 2011).

SLPs play a role in the treatment of cleft palate related to VCFS. They provide feeding strategies or make recommendations to other professionals on their team. One treatment strategy that may be used is maximizing feeding by adjusting an infant’s position. Sitting in an upright position during feeding allows gravity to help the food go where it is supposed to. This keeps food away from the nasopharynx and Eustachian tubes. Because children with cleft palate do not have an efficient seal in their oral cavity, air is often taken into the stomach. Having an upright feeding position can help to mobilize this air, if combined with breaks for burping (Glass & Wolf, 1999). Shaw et al. (1999) reported that using modified equipment such as an orthodontic nipple combined with a compressible bottle and parental counseling has moderate to strong evidence to support its success in helping babies with cleft palate to gain weight (as cited in Reid, 2004). Brine et al. (1994) also wrote about the success of using modified equipment such as a rigid bottle with a crosscut teat and a Mead Johnson cleft palate feeder along with a nutrition intervention protocol to help infants gain weight during their first 18 months (as cited in Reid, 2004). SLPs could recommend and implement these treatment strategies for their clients.

An SLP’s main role, in regards to children with cleft palate due to VCFS, is to correctly assess and provide early intervention and therapy services. The areas of communication, feeding, gross motor exercises, and social-emotional areas should all be addressed during therapy (Boyer, Fullman, Bruns, 2012). Integrating communication into a client’s daily play or feeding activities allows intervention strategies to be both functional and generalizable. An accurate diagnosis, as well as early intervention, can allow for great improvements of social-emotional, feeding, gross oral motor, and communication abilities in a client with cleft palate due to VCFS.



References
  
Boyer, V. E., Fullman, L. I., & Bruns, D. A. (2012). Velocardiofacial syndrome and 
     early intervention Providers. Infants & Young Children, 25(1), 83–94. 
     doi:10.1097/IYC.0b013e31823cff6b.

Carneol, S., Marks, S., & Weik, L. (1999). The speech language pathologist: key role in the
     diagnosis of velocardiofacial syndrome. American Journal of Speech-Language Pathology, 8,
     23–32. Retrieved from http://ajslp.pubs.asha.org/article.aspx?articleid=1774642.


Chapman, K.L., Hardin-Jones, M., Schulte, J., & Halter, K.A. (2001). Vocal 
     development of 9-month old babies with cleft palate. Journal of Speech, 
     Language, and Hearing Research, 44(6), 1268-1283. doi: 1092-4388/01
     /4406-1268.

“Definitions of the Professions.” (2012). In American Speech-Language Hearing 
     Association. Retrieved from http://www.asha.org/div40/definitions.htm.

Forsyth, A., & Morrison, M. (2008). 22ql1 Deletion Syndrome (Velocardiofacial Syndrome), ACQ, 2(1), 50–3.

Fullman, L., & Boyer, V. (2012). Velocardiofacial syndrome and early intervention.   
     Contemporary Issues in Communication Science and Disorders, 39, 21–29. 
     doi: 1092-5171/12/3901-0021. 

Glass, R.P., & Wolf, L.S. (1999). Feeding management of infants with cleft lip and palate and 
     micrognathia. Infants & Young Children, 12(1), 70–81. doi:10.1097/00001163-199907000-00010.

Hardin-Jones, M., & Chapman, K.L (2011). Cognitive and language issues associated
     with cleft lip and palate. Seminars in Speech and Language, 32, 127-140. doi: 
     http://dx.doi.org/10.1055/s-0031-1277715.

Pike, A.C., Super, M., (1997). Velocardiofacial syndrome. Postgrad Med Journal  
     73, 771-775. doi: 10.1136/pgmj.73.866.771.

Reid, J. (2004). A review of feeding interventions for infants with cleft palate. The Cleft Palate-      
     Craniofacial Journal: Official Publication of the American Cleft Palate-Craniofacial 
     Association, 41(3), 268–78. doi:10.1597/02-148.1.

Sharp, H.M., Daily, S., Moon, J. (2003). Speech and language development disorders in infants and 
     children with cleft lip and palate. Pediatric Annals, 32(7), 476-480.

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