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You have Dominic, an African-America nine year old boy, admitted to your floor with significant pain to his hands, feet and abdomen. He has been in before for sickle cell anemia and is experiencing another crisis.

1. What is sickle-cell anemia?

2. Why did sickle-cell anemia evolve?

3. Why does this disorder cause pain?

4. What are the risks with sickle cell anemia?

5. What is the main treatment?

6. How can you prevent another crisis?

7. Dominic needs pain control. He weighs 92 pounds. The Morphine Sulfate (MS) is ordered at 0.1mg/kg. What is Dominic's dose?

Answer 1

1). Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances.

2). When someone inherits two mutant copies of the hemoglobin gene, the abnormal form of the hemoglobin protein causes the red blood cells to lose oxygen and warp into a sickle shape during periods of high activity. ... This is sickle cell anemia.

3). Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones. ... Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.

4). Sickle cell anemia can lead to a host of complications, including:

Stroke. A stroke can occur if sickle cells block blood flow to an area of your brain.

Acute chest syndrome.

Pulmonary hypertension.

Organ damage.

Blindness.

Leg ulcers.

Gallstones.

Priapism.

5)Bone marrow transplant, also known as stem cell transplant, offers the only potential cure for sickle cell anemia. It's usually reserved for people younger than age 16 because the risks increase for people older than 16. Finding a donor is difficult, and the procedure has serious risks associated with it, including death.

As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. Babies and children age 2 and younger with sickle cell anemia should make frequent visits to a doctor. Children older than 2 and adults with sickle cell anemia should see a doctor at least once a year, according to the Centers for Disease Control and Prevention.

Treatments might include medications to reduce pain and prevent complications, and blood transfusions, as well as a bone marrow transplant.

Medications

Medications used to treat sickle cell anemia include:

Antibiotics. Children with sickle cell anemia may begin taking the antibiotic penicillin when they're about 2 months old and continue taking it until they're at least 5 years old. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to an infant or child with sickle cell anemia.

As an adult, if you've had your spleen removed or had pneumonia, you might need to take penicillin throughout your life.

Pain-relieving medications. To relieve pain during a sickle cell crisis, your doctor might prescribe pain medications.

Hydroxyurea (Droxia, Hydrea). When taken daily, hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. Hydroxyurea seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells.

Hydroxyurea increases your risk of infections, and there is some concern that long-term use of this drug might cause problems later in life for people who take it for many years. More study is needed.

Your doctor can help you determine if this drug might be beneficial for you or your child. Don't take the drug if you're pregnant.

6). Taking the following steps to stay healthy may help you avoid complications of sickle cell anemia:
Take folic acid supplements daily, and choose a healthy diet.
Drink plenty of water.
Avoid temperature extremes.
Exercise regularly, but don't overdo it.
Use over-the-counter (OTC) medications with caution.

7). 4.1mg is morphine dose.