Discussing Sickle Cell Anemia with Dr. Corey Hebert.

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(ThyBlackMan.com) Sickle cell anemia is a disease we don’t here very much about anymore, but it was once a diseased that plagued African American communities and resulted in early death. Elders knew of the diseased and often asked questions about it before marrying. This is a lost conversation to many that needs to resurface in our community. Sickle cell anemia disproportionately effects the black community, and more awareness is needed to understand the disease, and how to help those we love that live with it. ThyBlackMan.com spoke Dr. Corey Hebert who took the time to shed some like on sickle cell anemia and one of the treatment options. Dr. Hebert is an assistant professor, and he is in private practice in New Orleans, LA. He is the former Chief Medical Officer for New Orleans public school district, and the current Chief Medical Officer for Dillard University.

Many of our readers have probably heard of sickle cell anemia, but it seems there isn’t an understanding of the illness. Could you expand on what this disease is, and how it affects the body?
Sickle cell anemia is the most common inherited blood disorder. In simple terms, it is a condition in which red blood cells are abnormally shaped. Whereas normal red blood cells are round, in patients with sickle cell anemia, they are half-moon-shaped and stiff, causing them to become stuck in the blood vessels, blocking blood flow. These blockages can cause crippling pain and organ damage, which may require hospitalization. Thus, the disease can significantly reduce quality of life and potentially decrease life expectancy by 25 to 30 years. Although it is considered a “rare disease” sickle cell anemia affects nearly 100,000 Americans.

Does having a sickle cell trait mean your children will inherit the disease?
For a baby to be born with sickle cell anemia, both parents must carry a sickle cell gene. Those who inherit the gene from only one parent are known as carriers. They have sickle cell trait, but not sickle cell anemia. Although carriers generally don’t have any symptoms of the condition, they can still pass the gene on to their children.

We’ve often heard sickle cell anemia is a black person’s illness. Are black people the only group that has to battle this illness?
Although the majority of sickle cell anemia patients are of African ancestry, a minority are of Hispanic or Southern European, Middle Eastern or Asian Indian descent.

How can we better help family members cope with, and manage, sickle cell anemia?

Building a strong relationship with their pediatrician/HCP can help families understand the disease, as well as the risks and benefits of available treatment options.
Early treatment is key. Children with sickle cell disease are at increased risk of infection and recurrent painful episodes during childhood. These may lead to multi-organ damage, associated with poor prognosis and early mortality. Therefore, as recommended by the National Institutes of Health, it is important to start disease-modifying therapy with hydroxyurea as early as possible.
Hydroxyurea dosing in sickle cell anemia patients is based on patient body weight and clinical response. These must be closely monitored so that dosing can be adjusted accordingly. This is particularly important in the case of children, whose weight changes frequently.
Until recently, the only way to prepare customized doses of hydroxyurea was by compounding (mixing/altering/combining ingredients), which is a time-consuming and expensive process. It is also important to note that compounded drugs are not approved by the FDA.
Fortunately, hydroxyurea now comes in a pill specifically indicated for pediatric patients. Siklos® (hydroxyurea) is the first and only FDA-approved prescription medicine that is used to reduce the frequency of painful crises and reduce the need for blood transfusions in children, 2 years of age and older, with sickle cell anemia with recurrent moderate to severe painful crises. Available in 100 mg film-coated tablets and 1,000 mg triple-scored (4 x 250 mg) tablets, Siklos® makes it easier to tailor dosing to a patient’s individual needs. These tablets are even dissolvable for those who have trouble swallowing them whole. Siklos® has a Boxed Warning regarding low blood cell count and cancer.
It is also critical that patients who need hydroxyurea have access to the medication. Fortunately, Medunik USA, the company that markets the Siklos® tablets, offers savings programs and patient assistance programs to make treatment affordable.

What can we do to bring about more awareness where sickle cell anemia is concerned?

Educating the community is key to raising awareness of the disease. Awareness is important not only to ensure that patients and their families are well informed about their disease, but also to create public awareness to help secure funding for research into treating it. While education should be ongoing throughout the year, a variety of events and campaigns, hosted by advocacy groups, healthcare centers and patient organizations can help increase public awareness. For example:

Annual awareness days and months: World Sickle Cell Day (June 19) and Sickle Cell Awareness Month (September)
Advocacy campaigns (e.g., engaging elected officials for proclamations, hosting awareness events, distributing educational information to dispel the myths about sickle cell disease, and lighting public spaces, buildings and landmarks red, etc.)
Fundraisers and appeals organized by various healthcare centers and patient associations (e.g., walk-a-thons, etc.)
Annual national conventions (e.g., SCDAA Annual National Convention, conferences, seminars, etc.)
Community forums, candle lighting ceremonies, etc.

As we acknowledge Sickle Cell Awareness Month please take the time to talk to your family about sickle cell anemia. It is important to know if you carry the trait and remember to ask your significant other as this can greatly affect your children. This is not a disease of the past… it is one that many in our community still face. Below you can read more information about Siklos, provided by Dr. Hebert, as a treatment option you can share with loved ones, and members of the community that suffer with sickle cell anemia. Continuing the conversation will always be necessary when confronting this disease, and its silent effect on our community.

Please read Important Safety Information below for further information about Siklos®.

Siklos® (hydroxyurea) tablets, for oral use

WHAT IS SIKLOS®?

Siklos® is a prescription medicine that is used to reduce the frequency of painful crises and reduce the need for blood transfusions in children, 2 years of age and older, with sickle cell anemia with recurrent moderate to severe painful crises.

It is not known if Siklos® is safe and effective in children less than 2 years of age.

IMPORTANT SAFETY INFORMATION

WARNING: LOW BLOOD CELL COUNT and CANCER

See full prescribing information for complete Boxed Warning.

Low blood cell counts are common with Siklos®, including low red blood cells, white blood cells, and platelets, and can be severe and life threatening. If your white blood cell count becomes very low, you are at increased risk for infection. Your healthcare provider will check your blood cell counts before and during treatment with Siklos®. Your healthcare provider may change your dose or tell you to stop taking Siklos® if you have low blood cell counts. Tell your healthcare provider right away if you get any of the following symptoms: fever or chills; shortness of breath; body aches; unusual headache; feeling very tired; bleeding or unexplained bruising.
Some people have developed cancer, such as leukemia and skin cancer, after taking Siklos® for a long time. Your healthcare provider will check you for cancer. You should protect your skin from the sun using sunblock, hats, and sun-protective clothing.

WHAT IS THE MOST IMPORTANT INFORMATION YOU SHOULD KNOW ABOUT SIKLOS®?

Siklos® can harm your unborn baby.
For females taking Siklos® who can become pregnant:
You should talk with your healthcare provider about the risks of Siklos® to your unborn baby.
You should use effective birth control during treatment with Siklos® and for at least 6 months after treatment with Siklos®.
Your healthcare provider will perform a pregnancy test before you start treatment with Siklos®. Tell your healthcare provider right away if you become pregnant or think you may be pregnant.
For males taking Siklos®. Siklos® can affect your sperm. If you have a female sexual partner who can become pregnant, you should use effective birth control during treatment with Siklos® and for at least 6 months after treatment.
Siklos® may cause fertility problems in males. Talk to your healthcare provider if this is a concern for you.

WHO SHOULD NOT TAKE SIKLOS®?
Do not take Siklos® if you are allergic to hydroxyurea or any of the ingredients in Siklos®. See the Medication Guide for a list of the ingredients in Siklos®.

WHAT SHOULD YOU TELL YOUR HEALTH CARE PROVIDER BEFORE TAKING SIKLOS®?
Tell your healthcare provider about all of your medical conditions, including if you:

have kidney problems or are receiving hemodialysis
have liver problems
have human immunodeficiency virus (HIV) or take HIV medicines. Taking Siklos® with certain HIV medicines can cause serious reactions and may lead to death.
have increased levels of uric acid in your blood (hyperuricemia)
have a history of receiving interferon therapy or are currently receiving interferon therapy
have leg wounds or ulcers
plan to receive any vaccinations. You should not receive “live vaccines” during treatment with Siklos®.
are pregnant or plan to become pregnant. See “What is the most important information I should know about Siklos®?”
are breastfeeding or plan to breastfeed. It is not known if Siklos® can pass into your breast milk. Do not breastfeed during treatment with Siklos®.

Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

WHAT ARE THE POSSIBLE SIDE EFFECTS OF SIKLOS®?

Siklos® may cause serious side effects, including:

See “What is the most important information I should know about Siklos®?”

Skin ulcers, including leg ulcers have happened in people who take Siklos®. This has happened most often in people who receive interferon therapy or have a history of interferon therapy. Your healthcare provider will decrease your dose or stop treatment with Siklos® if you develop any skin ulcers.
Enlarged red blood cells (macrocytosis). Macrocytosis is common in people who take Siklos® and can make it difficult to detect a decrease of folic acid. Your healthcare provider may prescribe a folic acid supplement for you.

The most common side effects of Siklos® include: