FEBRUARY 2019 ISSUE - Digital Edition

adapted exceptionally well and managed to
regularize his life style as well as his glucose
levels with the help of the pump. This HbA1c
levels improved from 13% to 8.2% between
June 2017 to February 2018.
To ensure appropriate glucose monitoring
in addition to insulin delivery, the patient was
started on a continuous glucose monitoring
system. This is a cell phone app based realtime
glucose monitoring system that provides
continuous interstitial glucose profiles
directly on the cell phone. This system sends
regular alerts on the cell phones based
app, effectively preventing hyperglycemic or
hypoglycemic episodes and allowing for even
better glucose level control. Other benefits of
using such a continuous monitoring system
include a reduction in checking interstitial
glucose to twice a day only, and that too only
wolframin result in improper functioning of the endoplasmic
reticulum, and consequently, cell death. Prominently, insulinproducing
beta cells are affected, which results in diabetes
mellitus; and a gradual loss of the cells and the optical nerve
eventually results in blindness. Cell death in other organs may
cause a variety of other signs and symptoms associated with
Wolfram syndrome.
Currently, there is no cure for this syndrome and longterm
prognosis is completely dependent on the affected
organs. Several therapeutic options, including drugs that can
balance calcium levels in the ER or improve protein folding
and trafficking in the ER, and stem cell therapy to regenerate
the destroyed cells are being explored. However, as of now,
treatment is only supportive and based on the symptoms in
each individual. Most individuals with Wolfram syndrome have
an average lifespan of only 30-40 years.
The patient and his parents were extremely worried
because of the nocturnal hypos which significantly affected
their quality of life. The patient was already being treated with
multiple daily injections of insulin. However, this treatment
was not effective and resulted in unpredictable episodes of
hyperglycemia and hypoglycemic without any recognizable
warning signs. The patient was consequently afraid of taking
the insulin as the effect was unpredictable and even small
doses could result in dangerous hypos. To provide a more
continuous treatment strategy, Dr Menon started him on an
insulin pump. The continuous subcutaneous insulin infusion
(CSII) allows for constant, continuous infusion of insulin under
the skin. The insulin pump was effective in immediately
stabilising the glucose levels. However, a major concern was
whether the patient would be able to manage the pump with
his visual and hearing impairment. Surprisingly, the patient
THE PATIENT WAS AFRAID OF
TAKING THE INSULIN AS THE
EFFECT WAS UNPREDICTABLE
AND EVEN SMALL DOSES COULD
RESULT IN DANGEROUS HYPOS
for calibration purposes. As of September
2018, the patients HbA1c levels were at 7.3%.
While these are still higher than normal,
they are much improved compared to a year
ago, and the patient is able to manage his
glucose levels much more effectively and is
witnessing far fewer hypoglycemic episodes.
While diabetes and related conditions
are dramatically increasing worldwide,
technology is also advancing by leaps and
bounds. Several monitoring and drug delivery
devices are available now, even as others are
in the pipeline, to make it simpler to control
diabetes and continue retaining good quality
of life. Dr Menon is optimistic for the future:
“With the technological advances, it is now
possible to get real-time glucose profiles for
a better understanding of how glucose levels
fluctuate in each individual, and treatment
should be tailored accordingly.”
DR SHIVANEE SHAH
FEBRUARY 2019 / FUTURE MEDICINE / 47