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so what that tells us is that the role of the registered dietitian is critical
in the management of patients with cystic fibrosis.
Hello everyone my name is Caroline Weeks and I'm a registered dietitian and nutritionist who works in
the areas of cystic fibrosis, gastroenterology and endocrinology. I am
here on Kim's YouTube channel today to talk about the medical nutrition therapy
for cystic fibrosis. I will discuss cystic fibrosis as a disease- what it is,
how it's diagnosed from infancy; I'm going to discuss common medications that
these patients need on a day to day basis some of which I'm involved in
dosing and then I'm going to talk about some long-term complications of the
disease specifically related to GI and nutrition so here we go let's get
started. Cystic fibrosis is an autosomal
recessive genetic disease of the lung, but particularly it's a genetic mutation
of the CFTR protein or the cystic fibrosis transmembrane conductance
regulator protein and what this protein is involved in is it's basically
expressed in every tissue in the body so think lungs pancreas GI tract other
organs. These patients excrete sodium through their skin -salt much more
readily if you kiss them on their on their face you can taste salt so that's
one aspect of the disease basically the system is becoming more dehydrated which
can lead to a thick mucus being produced in the lungs but also different tracts
and ducts of the GI system so CF is first diagnosed now on a newborn screen
now there have been recent and huge I might add
advancements in the pharmacological management of cystic fibrosis originally
this was thought to be a pediatric disease however because of the work
that's been done on the pharmaceutical side of things the mean life expectancy
for a patient with CF is now 47 years of age which is huge. The CF Foundation puts
out an annual report and there is a plethora of research linking optimal BMI
and improved BMI with overall lung function as measured by fev1 percent
predicted so what that tells us is that the role of the registered dietitian is
critical in the management of patients with cystic fibrosis. The typical diet
that is prescribed for these patients and I'll get into more details soon is a
high-fat, high-calorie, high sodium diet now it's my personal opinion that we can
achieve that in much healthier ways than others for example increased intake of
mono and polyunsaturated fats as opposed to trans fats and saturated fats but
that's generally the overall diet prescription. When it comes to BMI the
goal or optimal BMI for pediatric patients is at or above the 50th
percentile for women it's at or above a BMI of 22 and for men it's 23 now that
being said energy requirements for those with cystic fibrosis are generally 1.5
to 2 times that DRI and protein requirements are about 1.5 to 2 times
the DRI as well so that being said it can be very difficult to get adequate
calories by mouth so oftentimes my patients have a gastrostomy tube in
place and I'm involved the management of g-tube regimens I'm
constantly bombarding patients with medical nutrition therapy and ideas for
increasing calories whether that's through oral nutrition supplementation
or just adding oils butter things like that to their meals on any given day. One
of the greatest aspects of medical nutrition therapy for patients with
cystic fibrosis is the dosing of pancreatic enzyme replacement therapy or
PERT and these are little enzyme capsules that I like to think of almost
as insulin. Pancreatic enzyme replacement therapy needs to be taken with every
meal every snack from infancy so with breast feeding or formula feeds
all the way until adulthood this is very very important for the absorption of
protein and fat from our foods and it's essential to prevent deficiencies of fat
soluble vitamins A,D,E, and K now I'm involved in the dosing and management of
these enzymes they come in a variety of different units of lipase and strengths
if you will that is dosed one of two ways you can dose it based on the
patient's weight or based on how many grams of fat the patient is consuming at
any given meal or snack now these enzymes are entericly coated so that
there is better absorption in the proximal duodenum these enzymes and fat
absorption occurs better in a more basic environment now without this enzyme
therapy patients run the risk of experiencing steatorrhea or oily stools
basically fat malabsorption they can also experience bloating abdominal pain
loose stools in general and we run the risk of becoming
deficient in vitamin D, vitamin E, K, and A all of which can lead to long-term
health problem I said that I would discuss some of the other complications
more nutrition based complications of CF and the first one is constipation
because our GI tract it's becoming more dehydrated our stool becomes thicker and
runs the risk of becoming impacted along the colon and small intestine now
unfortunately a mixture of both fluid and fiber in this setting is not enough
and so oftentimes I push bowel regimens with the use of miralax or some other
non stimulant laxative a second GI related complication and CF is GERD or
gastroesophageal reflux and though this is somewhat misunderstood we think it's
due to altered gastric emptying and a reduced lower esophageal pressure and
also reduce primary peristalsis in the setting of cystic fibrosis also for
normal patients typically a high-fat fried food diets can cause reflux so we
think there could be a link between that as well
additionally long term effects such as gall bladder disease, cholestasis and
drug-induced liver disease can sometimes be seen in cystic fibrosis. One of the most common complications of cystic
fibrosis is cystic fibrosis related diabetes also known as type 3 diabetes
because it shares characteristics of both type 1 and type 2 in the fact that
there's insulin resistance and insulin deficiency in our patients annual labs
and screens we make sure they get an oral glucose tolerance test in addition
to other screening factors such as vitamins and minerals
and pancreatic function tests. A final complication that can occur in cystic
fibrosis are skeletal complications mainly being osteoporosis and there are
a number of factors that can lead to osteoporosis but the main one is
long-term vitamin D deficiency as a result of steatorrhea and fat malabsorption
other factors include overall poor nutritional status, circulating
pro-inflammatory cytokines, respiratory acidosis and overall physical inactivity
a DEXA scan is also included in our patients overall labs that we get
usually this happens when they are adults to begin with but my role as a
dietitian with the dosing of pancreatic enzymes is something that is very
critical to preventing this long term complication from half so in a nutshell
the role of the registered dietitian in CF is very very critical to their
overall health I'm involved in teaching patients how to increase overall
calories in their diet ensure that they are well hydrated through adequate
fluids and addition of adequate salt in their diet because they excrete salt
more readily through their membranes I'm involved in overall reflux therapy and
dealing with kind of picky eating behaviors and things and school-aged
children sometimes I work directly with my GI provider to dose appetite
stimulants such as cyproheptadine in young kids because they need an extra
boost to just overall eat enough so I feel blessed and very lucky to be
working in this specialty area I feel like it's challenging every day is
different and I am forced to keep on my toes
the latest drug therapies and overall nutrition therapies for this
these patients because as I said at the beginning of the video things are
rapidly changing and evolving in cystic fibrosis so thank you so much for
watching please feel free to comment and leave any questions you have below I by
no means covered to all extents of the ins and outs of MNT for CF but
hopefully I gave you a good overview and again I'm happy to answer any questions
you may have thanks for watching
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