Hereditary Sensory and Autonomic Neuropathy Type IV (HSAN
IV) is a rare disease that is without cure and therefore the only measure we
can take is to prevent and handle before and after the disease. There are
several management methods that are tailored for the symptoms shown by the
child or individual with HSAN IV.
First of all, individuals with HSAN IV have to be constantly under the
inspection of a team of medical specialist of different fields. This team of
specialists has to join effort to work out a comprehensive and systematic plan
to help treat or manage the affected child. Plus, dermatologists, dentists,
neurologists and orthopedists could be an example of personnel in the
specialist team (National
Organization for Rare Disorders, 2014). This method is to promote the awareness
of symptoms of the disease to their family members as well as to guide them on
the ways to handle such child at home or at public area.
Secondly, to prevent consequences due to the
inability to feel hot or cold stimuli, parents or guardians should be alert
always on the affected child’s body temperature and body condition. Whenever
fever exists, parents or guardians could provide medication such as ibuprofen
and acetaminophen to help subside the fever within their bodies. Not only that,
parents could also bring their affected child to have a cold bath or cover them
with a cooling blanket to lower their body temperature (National Organization for Rare Disorders, 2014).
Third, various orthopedic and dental measures need
to be taken to help them recover from severe unknown injuries and to avoid
self-mutilation. Orthopedic measures are usually to treat abnormalities
affecting one’s bones and joints, procedures such as braces or orthopedic
devices. In addition, they should also use dental procedures to protect the
child from self-harming. For instances, prevent any mouth injuries by smoothing
or grinding down the sharp edges of the child’s tooth, using a night guard or
extracting a tooth (National
Organization for Rare Disorders, 2014).
Next, affected child or individual needs behavior
conditioning as well as the other variety supports they can obtain. The
behaviors of the affected individuals need to be conditioned so that they would
not harm themselves unknowingly especially when their parents or guardians are
not beside them (National
Organization for Rare Disorders, 2014). Behavior modification techniques such
as operant conditioning concept should be introduced to the parents and
encouraging the child to recognize dangerous objects through cues beyond than
senses stimulation. Operant conditioning is a concept that connects certain
responses or actions by using a certain stimuli to trigger the intention of
behaving the desired behavior or to eliminate unfavorable behavior (Huitt &
Hummel, 1997). A child could learn whether a behavior is desired or expected
from the reinforcement and punishment system implemented by their parents or
guardians. To reinforce a behavior would increase the frequency of that action.
On the other hand, to punish a child due to his or her behavior would reduce
the occurrence of that undesirable behavior (Huitt & Hummel, 1997).
Besides learning and conditioning favorable
behaviors, psychosocial support plays a great yet crucial role for the affected
children and individuals (National
Organization for Rare Disorders, 2014). Children are a growing social being. In
order to be able to fit into the society, they need to step out from their home
and start being independent. However, independency is a task with high
difficulty level for them as they could not sense danger, especially to extreme
temperature and pain. Thus, a supportive and cooperative social networking is
definitely required by the affected group. The society should increase their
understanding towards this disease through research or acquiring information
from professionals. A helpful psychosocial support could help sustain the life
quality of the affected group. It would be better if the affected ones are able
to find a trustworthy friend or partner to assist them.
Most importantly, this group of people needs
regular and daily monitoring or inspection the most. This is an important
measure to prevent any unwanted accidents from happening at home or outside
before they could achieve independency fully. With regular monitoring, any
unrealized and unknown injuries could be found out as quickly as possible to
inhibit the wounds or hurting from worsening (National Organization for Rare Disorders, 2014).
This inspector has to be extremely alert and cautious in order to sense the danger
that is unknown to the affected individuals.
References:
National
Organization for Rare Disorders. (2014). Hereditary sensory and autonomic neuropathy type IV. National
Organization for Rare Disorders. Retrieved September 22, 2016 from http://rarediseases.org/rare-diseases/hereditary-sensory-and-autonomic- neuropathy-type-iv/
Huitt, W. &
Hummel, J. (1997). An introduction to operant (instrumental) conditioning. Educational Psychology Interactive. Valdosta, GA: Valdosta State University. Retrieved October 10, 2016 from http://www.edpsycinteractive.org/topics/behavior/operant.html
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