Saturday, May 24, 2014

Colorado Vaccination Legislation

Colorado House Bill 1288 was signed into law this week.  This is the update from Children's Hospital Colorado on the legislation:

Under Colorado law today, all children enrolled in licensed child care and schools (public and private) must be immunized against a series of vaccine-preventable diseases like measles and pertussis. Parents can elect to exempt their children from these requirements for medical, religious, or personal belief reasons. This bill requires schools and child care centers to disclose their immunization and exemption rates, and also directs the Public Health Department to create an online, evidence-based education module about the benefits and risks of vaccines.
Why it’s good for kids: Requiring schools to disclose their immunization and exemption rates will arm parents with good information about choosing the right child care center or school for their child, which is especially important for medically fragile kids who are at higher risk for vaccine preventable diseases.
Why it still needs more work: Currently, a one-time parent signature is the only requirement to elect a personal belief exemption. This bill initially included a provision that would have required parents electing a personal belief exemption to either get a medical provider’s signature or complete an online education module addressing the risks and benefits of vaccines. This requirement would help spread knowledge in our communities and ensure our children don’t get sick from preventable diseases. The hospital and a strong coalition of advocates will continue to work with parents, health experts, educators, and lawmakers to improve Colorado’s vaccine requirements. This is important policy at any time, but it is especially vital now, as Colorado is in the midst of a serious outbreak of pertussis (whooping cough).

I'm excited that by the time the law goes into effect, I will be able to know the vaccination rates of the preschool that LB will attend, but I'm with Children's Hospital on wishing the law went just a little further in requiring parents to have a documented conversation with a medical provider or view scientific information about vaccinations before putting their child (and those that come into contact with their child) at risk of vaccine preventable illness.  Maybe next year?

Saturday, May 17, 2014

Editing Our Genes?

A study published in Nature Biotechnology and also described in this article from The Independent, seems very exciting for genetic disorders!

Scientists used a genome-editing technology called Clustered Regularly Interspaced Short Palindromic Repeats (CRISPR) to cure adult laboratory mice of an inherited liver disease called type 1 tyrosinemia.  This condition can be found in both animals and humans (it affects about 1 in 100,000 people and is part of newborn screening panels in some states) and is related to liver metabolism.  Affected individuals are unable to break down the amino acid tyrosine and require a special diet and medication to prevent liver, kidney and brain damage.

The CRISPR technique enables researchers to snip out a mutated piece of DNA and replace it with the correct sequence.  In this instance, the researchers were able to correct a single “letter” of the genetic alphabet which had been mutated in the gene involved in liver metabolism.  They altered the genetic make-up of about a third of the liver cells in the mice through a high pressure intravenous injection and the researchers effectively cured the mice.

The fact that this technique can be used successfully in an adult animal could lead to the ability to use the technique in humans (probably after lots of ethics committees and institutional research boards weigh in on it and, of course, oodles of clinical trials are performed).  It might be a long wait, but it sure is exciting to think about how a future gene therapy might fix the genetic defect in people with MCADD that prevents them from making the enzyme that allows them to break down medium chain fats.

Science is amazing!

Saturday, May 3, 2014

Dietary Fats Explained!

While skimming through my alumni e-newsletter this weekend, I happened upon an interesting article about dietary fats.  While the main point of the article was to inform generally healthy people about the role of fats in their diet and which fats are healthy, there were some interesting facts in the article that helped me understand my son's MCADD a little bit more.

Some things that I learned:

First, I learned that fats have 4 main roles in our bodies...
  1. Energy source and reserve–Fat provides 80-90 percent of the energy requirement at rest. One gram of pure lipid contains about 9 calories of energy, more than twice the energy available from carbohydrate or protein.
  2. Protection of vital organs– The body’s internal fat (about 4 percent of the total) protects against trauma to vital organs like the heart, liver, kidneys, spleen, brain, and spinal cord.
  3. Thermal insulation–Fat keeps us warm!
  4. Vitamin carrier and hunger suppressor–Approximately 20g of daily dietary fat provides a sufficient source and transport medium for the four fat-soluble vitamins: A, D, E, and K. Severely reducing lipid intake depresses the body’s level of these vitamins and may lead to a deficiency.
Although we aren't on a low-fat diet, #4 on the list makes me wonder whether MCADD kids on the low-fat diet may have vitamin deficiencies as a result of not having enough fat to transport vitamins and fully reap their benefits?

Second, I learned that fatty acids have a chain length categorized as short to very long.  This becomes important since the different chain lengths undergo different metabolic fates.  Short-chain and medium-chain fatty acids are quickly available for use as energy. Long-chain fatty acids, in contrast, undergo more complex processes and are deposited as fat in the liver and other body fat cells. Thus, the short- and medium-chain fatty acids often are considered “good” fats since they are easily metabolized and don’t necessarily contribute to fatty livers.

The four different types of fatty acids include: 

  1. Short-chain fatty acids = <6 carbons. These are typically found in butter and some tropical fats.
  2. Medium-chain fatty acids = 6-12 carbons.  You’ll find these in coconut oil, palm kernel oil, and breast milk.
  3. Long-chain fatty acids = 13-21 carbons. These are found in animal, fish, cocoa, seeds, nuts, and vegetable oils. (Chain lengths of 16-18 carbons are most common)
  4. Very-long-chain fatty acids = >22 carbons. Examples include omega-3 and omega-6 fatty acids found in fatty fish (omega-3) and some processed vegetable oils
This explains the common information in the MCADD community about avoiding coconut oil and avocado, the only food regularly mentioned to be high in medium chain fats.

Anyway, if you're interested in a deeper dive into fats, I'd encourage you to read the article and, of course, GO BLUE!