Health Information Management

National Sickle Cell Awareness Month: Can you code it?

Coding Educator, September 1, 2008

by Jennifer Avery, CCS, CPC, CPC-H

I had a friend who played in the percussion section at band camp. One year, he had to leave camp at the beginning of the week. We all wondered where he was and why he had to leave. Later, I learned that he suffered from sickle cell anemia. He left band camp early because he had a sickle cell crisis. At age 15, I wasn’t sure exactly what all of this meant. Over the years, I’ve heard many stories from people who suffer from the disease as well as from those who have the trait. 

September is National Sickle Cell Awareness Month and this is the perfect opportunity to address coding for sickle cell disease, sickle cell trait, and sickle cell crisis.
 

Sickle cell anemia, also known as sickle cell disease, is a chronic hemolytic anemia occurring almost exclusively in African Americans and characterized by sickle-shaped red blood cells. Normal red blood cells are disc-shaped—much like a donut without a hole—and they contain hemoglobin A. The abnormal hemoglobin S and hemoglobin C are characteristic of sickle cell disease. For more information, visit the Sickle Cell Disease Association of America Web site. 

Normal red blood cells move easily through the body’s blood vessels. Sickle-shaped blood cells don’t move easily. They’re sticky, stiff, and tend to form clumps. These clumps get stuck in the blood vessels and impede blood flow to other areas of the body. The result is pain, serious infections, and organ damage.  

Sickle cell disease sufferers inherit the disease from their parents. It affects millions of people worldwide. It’s most common in people from Africa, South or Central America, the Caribbean Islands, Mediterranean countries, India, and Saudi Arabia. In the United States, sickle cell disease affects approximately 70,000 people, most of whom are African American. About 1 out of every 500 African Americans has sickle cell disease. Additionally, 1 out of every 36,000 Hispanic Americans is born with sickle cell disease.    
 
There are different types of sickle cell disease. The most common are HbSS, HbSC, HbS beta thalassemia, and HbAS. There are also a few rare types of sickle cell disease such as HbSD, HbSE, and HbSO Arab. Sickle cell anemia (HbSS) is the most common of the sickle cell diseases. People who have sickle cell anemia inherited two sickle cell “S” genes—one from each parent. 
 
If you inherit only one copy of the sickle cell gene you will not have sickle cell anemia, nor can you develop the disease. The presence of one sickle cell gene is known as sickle cell trait. People who have sickle cell trait have can pass on the sickle cell gene to their children. If two parents each have sickle cell trait, their children can inherit sickle cell anemia. Click here for an illustration of how sickle cell trait works.  
 
Although sickle cell anemia is present at birth, it does not manifest symptoms for at least four to five months. This is due to the presence of “baby” hemoglobin. Since the “baby” hemoglobin consists of enough hemoglobin A, it prevents the red cells from forming “sickle” shapes. Once the “baby” hemoglobin is replaced with “adult” hemoglobin, the cells begin to sickle and symptoms can manifest.
 
Symptoms of sickle cell anemia can vary depending on the severity of the disease.  Patients are often hospitalized for treatment. The signs and symptoms of sickle cell anemia are similar to regular anemia and include pain, fatigue, shortness of breath, dizziness, headache, coldness in the hands and feet, pale skin, and chest pain. 
 
Sudden pain throughout the body due to sickle cell anemia is known as sickle cell “crisis.” Sickle cell crisis often affects the lungs, bones, abdomen, and joints. This pain can last for hours, days, or even weeks to months at a time. A sickle cell crisis is debilitating and may severely limit daily activities. Most patients will suffer a “crisis” at some point in their lives, while other patients may suffer several in one year. The cause of these “crises” is unknown, but physicians can control some of “crisis” precursors.
 
Often, the physician documents the “crisis” to identify its location such as “splenic crisis.” One complication of sickle cell crisis is hand-foot syndrome which is characterized by pain, swelling, and fever. Acute chest syndrome is another common crisis that is life-threatening. Similar to pneumonia, an infection or a sickle cell “clump” trapped in the lungs causes acute chest syndrome. Other complications that can occur include infections, pulmonary arterial hypertension, delayed growth and puberty in children, strokes, eye problems, priapism gallstones, ulcers on the legs, and multiple organ failure.
 
Currently this is no cure for sickle cell disease but physicians can treat complications. The goals of treatment include pain relief, infection prevention, eye damage prevention, stroke prevention, and complication control. Physicians can treat pain with simple over-the-counter medications. Other patients may require hospitalization to control pain. In these cases, treatment consists of fluids and medications such as narcotics.
 
Physicians sometimes give blood transfusions when a patient’s symptoms and complications worsen. Bone marrow transplants and gene therapy are two other possible treatments options. Prognosis of the disease is on a case by case basis. Here are some coding tips for sickle cell disease: 
  • Code sickle cell anemia, not otherwise specified (NOS), with ICD-9-CM code 282.60.
  • Code sickle cell “crisis,” NOS, with ICD-9-CM code 282.62.
(Note: There is parenthetical note under code 282.62 that directs coders to use additional codes when identifying the type of crisis, such as acute chest syndrome (517.3).)
 
Code the various types of sickle cell anemia as follows:
  • If documented as Hb-SS, report disease code 282.62,
  • If documented as Hb-SS, with or without crisis, report code 282.63
  • If documented as HB-C, report crisis code 282.64. (This includes Hb-S/Hb-C disease with crisis or with vaso-occlusive pain.) 
  • If documented as “other sickle cell disease,” report code 282.68,
  • If documented as “other sickle cell disease without crisis or with crisis, report code 282.69. (This includes Hb-S/Hb-D, Hb-S/Hb-E, sickle cell/Hb-D, and sickle cell/Hb-E.)

As we celebrate National Sickle Cell Awareness Month, keep in mind those who suffer from this debilitating disease. Early detection by way of newborn screenings, continued studies on gene therapy, and improved bone marrow transplants offer hope.

Editor’s note: Contact Jennifer Avery, CCS, CPC, CPC-H, at javery@hcpro.com.

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