Sickle Cell Disease

Sickle Cell Disease

Sickle-cell is an inherited disease. It is a blood disease caused by inheriting the gene from both your mother and father. A person is thus born with Sickle-cell disease.

You can be a carrier of the gene and not have the disease. If only one parent has the gene they can pass the gene on to their children. A child who inherits only one gene for Sickle-cell becomes a carrier of the disease but does not have the actual disease.

Sickle-cell is when the red blood cells are misshapen. Rather than round concave, convex smooth shape the red blood cells are in the shape of a sickle or half-moon.

This abnormal cell shape impairs the blood cells’ ability to carry oxygen throughout the body. The abnormally shaped cells can get stuck inside the blood vessels and cause intense pain throughout the body. When our organs such heart, liver, kidneys and our tissue in general does not get adequate oxygen it leads to very intense pain.

Sadly, there is no cure for Sickle-cell disease.

Some of the ways of managing sickle-cell disease:

  • high fluid intake
  • heathly diet
  • folic acid supplements
  • pain medication
  • vaccination and antibiotics for the prevention and treatment of infections
  • Blood transfusions when anemia is severe and life threatening

via WHO | Sickle-cell disease and other haemoglobin disorders.

Here are some things that you also might want to know about Sickle-cell disease:

1. Sickle cell disease is the most common genetic disorder in the United States.  World wide it affects  about 500,00 babies a year, about 100,0oo Americans have sickle cell disease. In the US as many as 2 million people carry the sickle cell gene.

2. Sickle cell disease is chronic but treatable and is not a death sentence. Sickle cell disease is a chronic illness and can be debilitating but with treatment people are living longer into their 40’s and 50’s. Twenty years ago, in many cases children did not live to be adults.

3. Sickle cell disease affects people of many different races.  This is not an African-American disease…it affects other races as well, including Mediterranean, Indian, and Middle Eastern heritage.

4. Patients with sickle cell disease require comprehensive care.   “A lot of day-to-day care can be done by a generalist,” says Dr. George Buchanan, professor of pediatrics at UT Southwestern Medical Center at Dallas. “But it is important to have a team of experts who work together and are in ongoing consultation with a large institution or center that specializes in sickle cell disease.”

via Sickle Cell Disease – 5 Things to Know – NYTimes.com.

Pediatricians refuse unvaccinated kids

Today I heard a disturbing piece of news regarding children and vaccinations.

Apparently, an infant who was not old enough to receive the pertussis vaccine was exposed to pertussis in a pediatrician’s office. This baby wound up extremely sick and hospitalized due to his exposure to pertussis, a potentially fatal, contagious disease.

This situation poses a dilemma.

Some pediatricians are now refusing patients whose parents are not allowing them to be immunized .

Where is the balance here??? I am not quite sure.

Personally, I would not want my child exposed to these diseases because as a pediatric nurse I have seen how they can ravage a healthy child and in some cases steal their life.

I am also aware of  and empathize with the parental fears that surround childhood vaccinations. These vaccinations have been associated with autism. Even though the evidence that at first supported these fears has been sited as flawed, the fear is still there.

So, how do we work with the parents and children who are not vaccinated against these childhood diseases and at the same time protect infants who are not yet vaccinated?

Is refusing to treat the unvaccinated the ethical way to approach this? I don’t think so.

Perhaps, there is a way to separate those who refuse to be vaccinated by making specific office hours when these children can be seen by the pediatrician…this would be expensive but could be an option.

What do you think as a parent…and how does this problem affect you?

Related links: No shot, no doc: Pediatricians refuse unvaccinated kids – TODAY Health – TODAY.com. http://parentingintheloop.wordpress.com/2011/01/08/more-on-vaccines-and-autism-from-march-of-dimes/   http://parentingintheloop.wordpress.com/2011/01/24/a-century-of-vaccine-scares-nytimes-com/