Sickle Cell Disease Awareness

A candle loses none of its light by lighting another candle!

To the Congress of the United States:

A second targeted disease for concentrated research should be sickle cell anemia--a most serious childhood disease which almost always occurs in the black population. It is estimated that one out of every 500 black babies actually develops sickle cell disease.

It is a sad and shameful fact that the causes of this disease have been largely neglected throughout our history. We cannot rewrite this record of neglect, but we can reverse it. To this end, this administration is increasing its budget for research and treatment of sickle cell disease fivefold, to a new total of $6 million.

Richard Nixon XXXVII President of the United States: 1069-1974

63 - Special Message to the Congress Proposing a National Health Strategy, February 18th, 1971

http://www.presidency.ucsb.edu/ws/index.php?pid=3311

"About Sickle Cell Disease " -  which is in every race!

 This page includes:

Sickle Cell Disease is not just about Race; it's also about the Region

 

Red Cell Defenses Against Malaria

 

The What and Where of Sickle Cell Disease and Trait

 

Did You Know?

 

SICKLE CELL DISEASE AND TRAIT from CHLA

 

Prevention of Sickle Cell Anemia/Disease

 

Test your knowledge about Sickle Cell

 

Sickle Cell on Film

 

 

Articles:

2009 ~ Our Weekly -The silent disease

2008 ~ BBC News UK-Call to treat sickle cell better

2007 ~ SCD News Update-From Senators Thomas R. Carper (D-DE) and Benjamin L Cardin (D-MD)

2002 ~ New York Times-A CONVERSATION WITH: JOSEPH GRAVES; Beyond Black and White in Biology and Medicine

CDC's info about Sickle Cell Disease (SCD) Visits to Emergency Departments (ED)

References

Estimated amount of people in each race affeted by SCD

Chart coming soon!!

 

SICKLE CELL Disease is not just about Race;

it‘s also about the Region where our ancestors came from.

That is why:

People of all races are diagnosed with one of the many forms of Sickle Cell Disease. 

From the mouth of a child:

http://www.youtube.com/watch?v=L4bB4QUV_wY 

10 year old Giovanna Poli, the 2009 Sickle Cell Disease Association of America, (SCDAA) Poster Child Contestant (who is NOT a Black/African American).

Or Visit

 http://www.facesofscd.org/

"When I was advised by the doctor that I carry the thalassemia trait and my children's father carried a sickle trait, I told them they were wrong.  I was positive they had read the wrong blood test because my husband had blonde hair and blue eyes.

The only thing I'd known about sickle cell disease was what I'd learned in school, and that was that you were born with it only if you were of African-American descent.  This is what most of society believes, including many in the medical profession."

or

Latino Florida Girl With sickle Cell Disease Suffers Thrid Stroke at Age 7, Brother Age 3 Also Diagnosed with Sickle Cell!

Find article on Flyers in PDF Page

 

We hope to use our website as a means to educate the public and medical profession at large.  ( Families like Giovanna are NOT African American/Black! )  Nita T.

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 On May 22, 2010 during the Sickle Cell Workshop: Enhancing Skills, Assessment and Pain Management we recently learned that soon Hispanics will soon out number African Americans/Blacks in the USA that have Sickle Cell Trait and diagnosed with the Disease!                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                    

                  Red Cell Defenses Against Malaria

Chart can be copied from PDF Page

The What and Where of Sickle Cell Disease and Trait

Just keeping it real ~ it matters to me because people have it so twisted and Sickle Cell Disease is so much more than a pain crisis!

---Nita T.

 

 

 

Sickle Cell Disease:

Sickle cell disease is a condition which affects red blood cells and relates to all types of sickle cell SC, SE, SD, etc. except for SS which is called Sickle Cell Anemia.  Both Sickle Cell Anemia and Sickle Beta-Thalassemia are also included when you talk about Sickle Cell Disease.

It is a "tropical disease" where the red blood cells mutated to protect the body from Faliciparum Malaria.  Those individuals have the Sickle Cell Trait.  ( Evolutionary and Historical Aspects of the Burden of Malaria  http://cmr.asm.org/cgi/content/full/15/4/564)

When two people with the Sickle Cell Trait have a child, there is a one (1) in four (4) chance that each child they have will be diagnosed with Sickle Cell Disease. 

People with Sickle Cell Trait can fight off malaria better than those without the trait, but people diagnosed with Sickle Cell Anemia/Disease usually can't fight off malaria and get very sick or die.

The disease causes the normal biconcave red blood cell to change to a crescent shaped or a deformed red blood cell when it gives off its oxygen. This can cause the blocking of blood vessels (picture a pileup and traffic jam on a major freeway or expressway during rush hour traffic, where not even the emergency vehicles can pass).  This type of blockage leads to triggering pain; strokes, pulmonary or heart challenges; the need for hip replacements; eye surgery, partial to full blindness, and many other problems.   

The disease is called Sickle Cell, however all of the abnormal blood cells DON"T look like a sickle.  They are abnormal blood cells, so they no longer look round and are no longer flexible.  Soon we will have a page dedicated to you just how the abnormal blood cells look like.

Sickle Cell Disease (SCD) affects more people in the world than any other genetic disease, was the first genetic blood disease to be understood and has served as a model in research to assist with the identification and treatment of other conditions.

It affects about 72,000 people in the US and affects millions of people worldwide!  It kills an estimated half-million people worldwide annually. Globally, almost one-half million babies die from SCD each year.

 

Within the US SCD affects more people of African descent, but the facts are being updated world wide because SCD is being found in large numbers among Hispanics, Native Americans and Caucasians. 

Throughout the world sickle cell affects people of various racial and ethnic backgrounds.   SCD affects a large percentage of people in México, Central America as well as significant numbers in the Caribbean and in South American countries such as Brazil. In the Mediterranean countries, SCD is found in people of Portuguese and Spanish descent, the French Corsicans, Sardinians, Sicilians, mainland Italians, Greeks, Turks and Cypriots. It is increasingly seen in the new births of European countries like France, Germany, and the UK,  also because of the migration from African countries. Sickle cell disease also affects large numbers of people in Middle Eastern countries of Pakistan, India, Saudi Arabia, and Asia. Although approximately 70 percent of the global number of SCD cases are in Africa (also because Africa is so large) SCD is the most frequently occurring genetic disease on earth.

Events that can lead to the closing up or clogging up of the blood vessels causing a sickle cell pain crisis/episode include:

Dehydration • Infections • Fatigue • Emotional stress • Overexposure to cold or heat • Cold water • High altitudes • Physical overexertion • Trauma, physical injury.

Sickle Beta-Thalassemia:

In this condition, the patient has inherited a gene for hemoglobin S from one parent and a gene for beta-thalassemia from the other. The severity of the condition is determined to a large extent by the quantity of normal hemoglobin produced by the beta-thalassemia gene. (Thalassemia genes produce normal hemoglobin, but in variably reduced amounts). If the gene produces no normal hemoglobin, b0-thalassemia, the condition is virtually identical to sickle cell disease. Some patients have a gene that produces a small amount of normal hemoglobin, called b+-thalassemia. The severity of the condition is dampened when significant quantities of normal hemoglobin are produced by the b+-thalassemia gene. Sickle/beta-thalassemia is the most common sickle syndrome seen in people of Mediterranean descent (Italian, Greek, Turkish) and Latin descent (Mexico, Central America, South America).  Beta-thalassemia is quite common in this region, and the sickle cell gene occurs in some sections of these countries. Hemoglobin electrophoresis of blood from a patient with sickle/b0-thalassemia shows no hemoglobin A. Patients with sickle/b+-thalassemia have an amount of hemoglobin A that depends of the level of function of the b+-thalassemia gene.

Sickle Cell Trait:

Millions of people in every race globally have Sickle Cell Trait and about 2 million people have sickle cell trait in the US.  Although we may be protected from malaria, there is concern about the effects of sickle cell trait as it relates several known situations.

First, persons especially athletes who become overly fatigued and dehydrated due to over exercise in extreme hot temperatures are at risk. Research has identified that dehydration and extreme physical challenges/exercise during exposure to very hot weather can result in death. 

Second, concerns with the eyes when people with SC trait go to high elevations.  Those visits have resulted in eye surgery, partial to full blindness.

Third, Pulmonary Hypertension is now being looked at as an issue not only for SCD patients but for those of us who have the trait.

*A Mutation Story

**Sickle Cell Trait Howard University School of Medicine Center for Sickle Cell Disease

***http://sickle.bwh.harvard.edu/hemoglobinopathy.html

FYI ~

Beta thalassemia disease is not a form of sickle cell disease, but it is a serious lifelong illness. People who have beta thalassemia disease do not make enough hemoglobin. The amount of hemoglobin produce, determines whether a person is diagnosed with:

  • Beta Thalassemia Intermedia
  • Beta Thalassemia Major (also known as Cooley’s anemia).

 

 

 

 

Did You Know?

Children born diagnosed with Sickle Cell Disease are considered to have a Pre-Existing Condition.  Did you know in California there are Health Care Options for Children with Pre-Existing Conditions? Please click on to view: 
files/file/id/zkrjha7eb790hb/0/0?_c=zkwx8utoooh4o1

CHLA ~ SICKLE CELL DISEASE AND TRAIT

SICKLE CELL DISEASE AND TRAIT from CHLA

What is Sickle Cell Disease?                                                             Sickle Cell disease is a group of inherited red blood cell disorders. It is the most common genetic disease in the US.

Over 70,000 Americans have sickle cell disease.

Normal Red Blood Cell

Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen.  

Sickled Red Blood Cell

Sickle red blood cells become hard, sticky and shaped like sickles used to cut wheat.

When these hard and pointed red cells go through the small blood tubes, they clog the flow and break apart.

This can cause pain, damage and a low red blood cell count (anemia). This blockage of blood flow by stiff red blood cells cuts off the supply of oxygen and nutrients.  This can result in life threatening problems, severe pain and early death.

What makes the red cell sickle?                                                                        There is a substance in the red cell called hemoglobin that carries oxygen inside the cell. One little change in this substance causes the hemoglobin to form long rods in the red cell when it gives away oxygen. These rigid rods change the red cell into a sickle shape.

How do you get sickle cell anemia?                                                      You inherit the abnormal hemoglobin from both parents who may be carriers with sickle cell trait or parents with sickle cell disease. You cannot “catch it” like a cold. You are born with the sickle cell hemoglobin and it is present for life.

Is Sickle Cell only in African Americans?                                             Sickle cell is in many nationalities including African Americans, Arabs, Greeks, Italians, Latin Americans and people from India. All races should be screened for this hemoglobin at birth. In the US, 1 out of 10 African Americans have sickle cell trait and 1 out of 625 newborns have the disease.

How can I be Tested?                                                                          A simple blood test called the hemoglobin electrophoresis can be done by your doctor or local sickle cell foundation. This test will tell if you are a carrier of the sickle cell trait or if you have the disease.


Newborn Screening                                                                               Most States now perform the sickle cell test when babies are born. The simple blood test will detect sickle cell disease or sickle cell trait. Other types of traits that may be discovered include: Hemoglobin C trait, Hemoglobin E trait, Hemoglobin Barts - which indicates an alpha thalassemia trait.

What is sickle cell trait?                                                                             Sickle cell trait is a person who carries one sickle hemoglobin producing gene inherited from their parents and one normal hemoglobin gene. Normal hemoglobin  is called type A. Sickle hemoglobin is called type S. Sickle cell trait is the presence of both hemoglobin A and S. Patients almost never have problems because of trait alone. Other hemoglobin traits common in the United States are AC and AE traits.

Are there different types of sickle cell disease?                                   There are three common types of sickle cell disease in the United States.

1.       Hemoglobin SS or sickle cell anemia

2.       Hemoglobin SC disease

3.       Hemoglobin sickle beta-thalassemia

Each of these can cause sickle pain episodes and complications, but some are more common than others. All of these may also have an increase in fetal hemoglobin which can protect the red cell from sickling and help prevent complications. The medication hydroxyurea also increases fetal hemoglobin.

 

Where can I get more information?

Childrens Hospital Los Angeles                                                                            323-361-SCD0 (7230)    www.chla.org                                    http://www.youtube.com/childrensLA             http://www.facebook.com/ChildrensLA

Center for Cancer and Blood Disease New Referral Line                        323-361-4100

Sickle Cell Disease Foundation of California                              http://www.scdfc.org    info@scdfc.org                                                             Phone (310) 693-0247 Toll free (877) 288-CURE (2873)

Sickle Cell Disease Association of America                                                      (800) 421-8453   scdaa@sicklecelldisease.org

Prevention of Sickle Cell Anemia/Disease

 First, know if you have Sickle Cell Disease or Trait!!! 

It is simple, have your blood tested! 

 In California, starting in 1990 newborn screening tested for Sickle Cell Disease and Trait.  Other states started in different years. 

If your parent's don't have your paperwork or remember once the child turns 18 years, they can directly contact their states Department of Health and Human Services "New Born Screening Program" to find out!

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SC Prevention links

http://apps.who.int/gb/ebwha/pdf_files/WHA59/A59_9-en.pdf

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Prevention of Sickle Cell Anemia/Disease

http://www.mayoclinic.com/health/sickle-cell-anemia/DS00324/DSECTION=prevention

By Mayo Clinic staff

If you carry the sickle cell trait, you may wish to see a genetic counselor before trying to conceive a child. A genetic counselor can help you understand your risk of having a child with sickle cell anemia. He or she can also explain possible treatments, preventive measures and reproductive options.

There is an in vitro fertilization procedure that improves the chances for parents who both carry the sickle cell gene to have a child with normal hemoglobin. This procedure is known as preimplantation genetic diagnosis. First, eggs are taken from the mother. Then, sperm is taken from the father. In a laboratory, the eggs are fertilized with the sperm. The fertilized eggs are then tested for the presence of the sickle cell gene. Fertilized eggs free of the sickle cell gene can be implanted into the mother for normal development. However, this procedure is expensive and not always successful.

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How Can Sickle Cell Anemia Be Prevented?

http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_Prevention.html

You can't prevent sickle cell anemia because it's an inherited disease. However, you can take steps to reduce its complications. (For more information, see "Living With Sickle Cell Anemia.")

People who are at high risk for sickle cell anemia and are planning to have children may want to consider genetic counseling. A counselor can help you understand your risk of having a child who has the disease and help explain the choices that are available to you.

You can get information about genetic counseling from health departments, neighborhood health centers, medical centers, and clinics that care for people who have sickle cell anemia.

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*A More Excessive and Radical Action for the Preventative Spread of Sickle Cell Anemia/Disease:

Sterilization for people who are diagnosed with Sickle Cell Anemia/Disease and Trait carriers

Termination of pregnancy

* We, the African American Blood Drive and Bone Marrow Registry for Sickle Cell Disease Awareness do not advocate for this excessive and radical action!  I/We don’t believe that anyone has the right to force people to not have children because of perhaps becoming a special needs parent.  And I/we certainly don’t believe that babies should be killed before born or at birth because they are diagnosed with Sickle Cell!

- - - Nita T. - - -

Test your knowledge about Sickle Cell

Visit these sites and take the tests to see just how much you know about SCD.  Weight the information with the newest discoveries!

http://www.nhlbi.nih.gov/health/dci/quiz/quiz_sickle.html 

http://sicklecell.starlightprograms.org/index.html

Sickle Cell on Film

3 Movies about Sickle Cell Disease

1.  A Warm December (73): Director: Sidney Poitier

Go to http://www.amazon.com/gp/offer-listing/6303922198/ref=dp_olp_3 to order your copy!

Dr. Matt Younger (Poitier) and his daughter (Curtis) arrive for a month-long visit to London for dirt-bike racing and unexpectedly, a new romance for the widowed Dr. Younger. His new love interest is the beautiful and playful Catherine (Anderson) who seems to enjoy eluding Dr. Younger as much as she enjoys eluding the mysterious men who are following her. Their romance develops gradually as does the viewers' understanding of what is going on with this charming woman and the complications* she brings with her.

*These complications are due to the fact that Catherine has sickle cell anemia, and the mysterious men following her are hired by her very protective father.

2. To All My Friends On Shore (72):  Bill Cosby's idea

Go to http://www.publicdomaintorrents.com/nshowmovie.html?movieid=148 to order your copy!

Remember "The Cosby Show"? Ever wonder what Bill Cosby did afterwards? Here's just one of his projects. Cosby plays Blue, a man doing everything he can to buy his family a house and move them out of the ghetto. His wife Serena (played by Gloria Foster who also played Oracle in "The Matrix") works hard, too. Then their child falls ill with sickle-cell anaemia, and everything falls apart. The ending is truly, truly special. This film was made in 1972. The plot was Bill Cosby's idea. He acted in it, produced it, wrote the music for it. Don't think for one moment that it's a comedy - it isn't. It is a powerful, moving film; a tribute to the enduring power of the human spirit. How on earth it never won even just one award is a puzzle which I shall never solve. Do yourself a favor - download this movie, then share the information that this gem is available for free with others.

3. Layla a romantic movie within the darkness of sickle cell  (We are doing my best to locate this movie!)

 http://www.womengateway.com/enwg/Art+and+Culture/art+and+culture84.htm

Article by-Suad Hamada (Women Gateway)

Cinema has stepped in the fight against sickle cell disease with a movie about the story of a nurse who fall in love with a patient.

Layla is the name of one of the biggest Bahraini movies that pays tribute to 18,000 citizens infected with the incurable inherited blood disease. It is produced by Orman Media and Bahrain Sickle Cell Patients Society and financially supported by the Ministry of Social Development.

The movie that its shooting is almost over tells the love story between a sickle cell patient and his nurse. The movie also gives realistic melodramas about the sufferings of those patients and their families.

Chairman of the Omran Media, Omran Al Musawi said in a statement on Tuesday that the movie was the second the company’s produced about aliments as the first one tackles the plights of kidney patients. “We want to send strong message through – Layla- the movie to society, so the public could accept and understand sickle cell patients better.”

Omran said that arts and social activities should step in to help as medical services weren’t the only solution to combat the disease. He said that in two decades cypress through joint efforts succeeded in eliminating such new cases.

Most of the actors are newcomers to bring freshness to the story of the movie. It tells the tale of Ali and his family with the disease and how he tried to keep his aliment as a secret from the nurse he loves.

Layla is the fourth movie produced by the company that started its operations with Maryami a film that won the best scenario award at the Second Gulf Cinema Festival.

The movie is directed by Ali Al Ali and written by Hussain Mahdi.  

Articles:

Los Angeles June 2009

 

 

By Shirley Hawkins | OW Staff Writer | 04.JUN.09

The silent disease

They are brave survivors who valiantly battle an illness that impacts the lives of thousands of Americans in the United States—and its name is sickle cell disease (SCD).

While diseases such as diabetes and breast cancer are well known among the general population, sickle cell disease—an inherited blood disorder that causes the red blood cells to become crescent or sickled-shaped, cluster together and cause blockages in the blood stream, is still relatively unknown by many in society.

Although the disease affects one in 400 African Americans, the disorder is also seen among Asians, Latinos and others with Mediterranean or African ancestry.

Approximately 130 babies are born in California with sickle cell disease every year. The disease is passed to the child when both parents have the sickle cell trait. According to Sickle Cell Disease Association of America, Inc. “sickle cell trait is an inherited condition, (but it) is not a type of sickle cell disease. People with sickle cell trait are generally healthy.

“Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets…one from each parent.” When this happens with SCD, that both parents have the trait and each passes on one set, there is a 25% chance that their child will have the disease.

When the sickle shaped cell is unable to pass through tiny blood vessels, this can result in tissue and organ damage. Symptoms can be fatigue and strokes—even as young as age three, and include chronic pain, shortness of breath, pain in organs or joints, infections, pain and swelling in hands and feet, chest pain, fever, and the need for regular blood transfusions for severe anemia.

A sickle cell crisis can last anywhere from hours to days. Some people with sickle cell disease have only one crisis every few years, while others have many crises in a year.

Blood transfusions play a major role in treating the illness. The transfusions increase the number of normal blood cells in the body and help correct anemia. Blood transfusions are best tolerated when the donor blood closely matches the patient’s.

Los Angeles resident Morgan Todd, 28, has lived with sickle cell his entire life.

After pain entered his arms one evening, Todd traveled to the clinic at Midway Hospital Medical Center in the Fairfax district to see his doctor. Like many sickle cell patients who are diagnosed with the disease at a young age, clinics and hospitals become like a second home for sickle cell sufferers.

“Having sickle cell brings undeniable pain,” nodded Todd, who talked to Our Weekly via cell phone while waiting in his doctor’s office to receive an intravenous dose of saline fluids and Dilaudid, a pain medicine.

Like most sickle cell recipients, Todd must grapple with the day-to-day physical trials of living with the disease. The result is that living a normal life is a challenge.

As a child, Todd dreamed of joining the ranks of the National Basketball Association as the first player to be officially drafted into the league with sickle cell. But the strain of living with the disease dashed his hopes of joining a professional team. “I could just be having a regular day, and then suddenly my body starts to hurt,” said Todd, who said that the excruciating pain from sickle cell can occur at any time. “Sometimes the pain strikes the whole body, and sometimes it’s in the arm, leg or chest. Imagine the pain of breaking an arm. The pain of sickle cell is 10 times worse-and then imagine if you’ve endured that pain for 28 years.”

Todd’s family has been especially supportive. Brandon Norwood, 24, Todd’s younger brother, said that the family tries to keep Todd’s spirits up. “We take him back and forth to the emergency room when he needs treatment,” he said.

You don’t hear news about people with sickle cell,” Norwood pointed out. “A lot of people in the Black community are not aware of the disease. There needs to be more awareness.”

Yolanda Allen, who serves on the board of directors of the Sickle Cell Disease Foundation and is the mother of 17-year-old sickle cell patient Jackie Allen, said that raising a child with sickle cell poses special challenges. “It’s a hard road,” admitted Allen. “The biggest challenge is that the disease is so spontaneous, you never know when the symptoms are going to occur. Jackie can be fine one minute and go into crisis the next—and it can happen in the middle of dinner or a conversation.”

Allen said that children with sickle cell often have to forego normal activities. “Jackie wants to go out and do things, but as a parent, you have to put your foot down. I tell her, you have to get your rest today because she can’t get overtired or dehydrated.”

Allen is also aware that sickle cell patients are often misunderstood by the medical community.

There is not enough being done for the parents, the patients or the families grappling with sickle cell,” maintains Allen. “For example, when you go to the emergency room, many times the nurses don’t know enough about how to treat a patient with sickle cell disease. They’ll have us sitting there for hours when my daughter or other sickle cell patients are in dire need of hydration or pain medication.”

Pausing, Allen added, “The most serious complication for sickle cell patients is acute chest syndrome which is one of the leading causes of death in sickle cell patients. It can cause death within two to three hours.”

Like Norwood, Allen said that society is still not aware that sickle cell is affecting people and they have yet to find a cure (for everyone). “People say, ‘I thought sickle cell was cured,’ or ‘We don’t hear anything about it,’ but people don’t know that sickle cell is the most prevalent genetic disorder in the country.”

Roger Brown, director of development for the Los Angeles based-Sickle Cell Disease Foundation of California, the oldest sickle cell disease organization in the country, has witnessed firsthand the devastation caused by the disease.

Brown said it is not unusual for sickle cell patients to experience strokes, heart attacks, joint degeneration, and infection. “There is also organ and joint degeneration, sepsis or infection, leg ulcers, and a large occurrence of strokes. Currently, there is no cure for sickle cell, with the exception of bone marrow transplants.”

Brown said that the agency services 750 families throughout the state and offers counseling and other supportive wraparound services. He maintains that it is difficult for sickle cell patients to live a normal life. “Sickle cell disease can hit the carrier any time of the day or night, and it doesn’t affect any two people the same way.”

Where once sickle cell disease patients were faced with a short life expectancy, advanced medicines are allowing sickle cell patients to live longer lives. “Because of the advances in medical care, people are living longer,” he pointed out. “Quite frankly, many of the hospitals and medical care facilities are not equipped to deal with adult patients with sickle cell. They didn’t think these people were going to live long.”

The excruciating pain that sickle cell patients endure means that they often need pain reducing medication such as morphine and Demerol. “When they go into the emergency room to get these drugs, doctors and nurses often think the patients were drug users,” said Brown. “These doctors and nurses have to apologize later when they realize how wrong they were.”

The director said that maintaining a job or finishing school are often challenging for sickle cell patients. “In many cases, kids do not graduate from high school on time,” reported Brown. “Sufferers have a difficult time completing college or maintaining a job. The stress of living with sickle cell means their ability to compete in society is compromised. It affects them on a physical, social and emotional level.”

Trish Peterson, a pediatric nurse practitioner in the hematology program at Children’s Hospital Los Angeles (CHLA), said that mandatory screening for the disease in newborns began in 1990. Peterson said that early diagnosis is critical in order to identify or prevent SCD’s myriad symptoms and complications.

Peterson said that CHLA, home to one of the nation’s premiere centers for treating sickle cell disease, also offers a battery of wraparound services.

A tour of the facility reveals bedridden children who have been receiving treatment at Children’s Hospital since birth. Most are there to receive intravenous transfusions of blood or fluids. “We have a whole team approach that looks at how sickle cell is affecting their school performances and family,” said Peterson, adding that sickle cell advocates frequently visit schools to educate school nurses about the disease.

Blood drives are especially important in combating the disease. “SCD patients who receive frequent transfusions require specially matched blood, which further increases the need for donors. CHLA said that it needs 400 donors a month for patients. “We have been trying to reach out to the African American community,” said Peterson,” who said that CHLA regularly holds blood drives to solicit blood. “There are patients who need blood every two to three weeks, which are our chronic transfusion children. Then we have others who have complications from the disease such as when they have a stroke or acute chest syndrome and they need fresh supplies of blood.”

Charles Taylor, 45, was diagnosed with sickle cell at birth.

Although he maintains a positive outlook, the disease has taken a toll on Taylor’s employment. “When I was young, I wanted to be a police officer, but because I had the disease, it hindered me from passing the physical. I was eventually hired by the Los Angeles County Office of Education (LACOE) as a behavioral management instructional assistant, and I told the interviewers I had sickle cell disease,” said Taylor. “They understood that this was a particular challenge that I had. Despite having the disease, I was hired.”

Taylor, who has worked with LACOE for 18 years, said that he frequently has had to miss days each week from his job. “In every paycheck, I’ve had a deduction due to the fact that I had an episode with sickle cell. It took 15 years before I could even have a vacation because I had used up all my sick time for sickle cell.”

Noting the unpredictable nature of crises, Taylor added that, “One time I was driving down the freeway and I had to swerve away from another car to avoid an accident,” recalls Taylor. “My entire body went into panic mode and I experienced one big cramp from head to toe. That happened a number of times. I would have to drive myself to the hospital for treatment.”

Todd, who regularly visits Central Juvenile Hall to talk to youths about being tested for sickle cell, feels it is vitally important to educate the community about sickle cell disease. “Many Black people aren’t aware that this disease exists. If you ask them about leukemia, lupus, or cancer, everybody knows about those diseases,” he pointed out. “But when it comes to sickle cell, Black folks don’t know anything about it. It saddens me.”

Currently, bone marrow transplants have proven to be the only known cure for sickle cell disease, although complications from a transplant can be life threatening. As reported by the Sickle Cell Information Center at the Grady Health System in Georgia, “bone marrow transplants have been used to treat sickle cell disease for 20 years. . .The treatments—high doses of chemicals that knock out the patient’s own marrow before the transplant—are so toxic that adults with sickle cell-induced organ damage would be unlikely to survive them.” According to a Nov. 14, 2008 article in U.S. News and World Report, “bone marrow transplants rely on heavy doses of chemotherapy prior to a transplant in order to destroy a recipient’s bone marrow so it won’t reject the donated marrow. Finding a perfect bone marrow transplant is another obstacle, since the bone marrow must be an exact match. Family members are often tested for matches, especially siblings and parents of the patient.

When it comes to combating the disease, doctors recommend that people with sickle cell take good care of themselves and see a doctor regularly.

They also recommend that sickle cell patients maintain a healthy diet, get enough sleep and rest, avoid overexertion, stress, extremes of hot and cold, drink adequate amounts of water every day, get a flu shot and other vaccinations to prevent infections, and see a dentist regularly to prevent loss of teeth.

Allen said that the Sickle Cell Disease Foundation is pushing for changes in legislation to fund programs and services for sickle cell disease on the state and national level. “The Sickle Cell Disease Treatment Act of 2003 was supposed to be signed into law by President George W. Bush, which would have brought more resources for sickle cell patients, but it was never signed,” said Allen. “We are trying to get our state legislators to support our federal legislators to reauthorize $10 million in funding for that act, which would help to fund 10 comprehensive treatment centers nationwide. It would also support 40 demonstration projects to improve the prevention and treatment of sickle cell disease.”

Todd’s advice to other sickle cell patients is never give in to the disease. “Keep fighting,” he said. “Dealing with this disease is really hard, but I thank God that I’m alive and still battling this illness.”

To contact the Sickle Cell Disease Foundation of California, call (310) 693-0247.

The UK May 2008

Call to treat sickle cell better

http://news.bbc.co.uk/2/hi/health/7408179.stm

More than 12,000 people have sickle cell disease in the UK

Treatment of sickle cell anaemia is compromised by health workers' lack of knowledge, a report warns.

The first national survey of the disease found seriously ill patients were not offered support from sufficiently experienced staff.

Patients' use of painkillers was not effectively monitored, in some cases leading to in fatal overdoses, it said.

The inherited genetic disease affects the ability of the red blood cells to carry oxygen around the body.

It can cause severe pain and damage to the organs.

Not enough support

The charity, the National Confidential Enquiry into Patient Outcome and Death, carried out the research into the deaths of 55 patients with sickle disease or the related condition thalassaemia.

Of 19 patients in the study who had complained of pain and who subsequently died in hospital, nine had been given excessive doses of painkillers and five of those patients died because of complications due to overdose.

The report "A Sickle Crisis?" also found the cause of death of some patients was unclear.

Doctors and nurses need to be more familiar with what needs to be done if patients' vitals signs become abnormal
Dr David Mason, study author

Professor Sebastian Lucas, one of the study authors, said: "We were surprised that our review found such a high number of cases where we did not know the actual cause of death.

"This is a wake-up call to the clinical community. Sickle cell disease is as common as cystic fibrosis, yet less is known about the severe complications that can lead to death in sickle cell disease patients."

The report also found the take-up of vaccinations by sickle cell disease patients at their GP practice was low.

Dr David Mason, co-author of the study, said: "Doctors and nurses need to be more familiar with what needs to be done if patients' vitals signs become abnormal.

"We need a multidisciplinary approach to acute pain management and regular reviews of therapy to control pain adequately."

People it affects

Around 12,000 people in the UK have sickle cell disease and it is one of the most common reasons for hospital admissions.

Sickle Cell mainly affects people of Afro-Caribbean, African, Eastern Mediterranean, Middle East and Asian origins. Thalassaemia mainly affects people from Asia, the Mediterranean and the Middle-East.

The demographics of where patients live have changed. At one time sickle cell disease was mostly found in London and the West Midlands, but it is now across the UK.

A Painful Condition

·  Sickle cell anaemia patients carry abnormal haemoglobin in their red blood cells

·  Normal red blood cells are doughnut-shaped and can bend and flex and travel easily along the narrowest of blood vessels

·  Those carrying sickle haemoglobin have a sickle shape destroying the cells' natural flexibility, making them less able to squeeze through small blood vessels

·  This can lead to blockages in the vessels, preventing oxygen from getting through to the tissues and causing severe pain and damage to the organs

·  When a blood vessel becomes blocked this can trigger an attack of pain know as a crisis.

Dr Mason said "We need a supporting health network from GP practice to hospitals to expert centres, to ensure that their care is managed well regardless of where they are in the UK."

Professor Lucas said it is now vital to set up the national database of sickle and thalassaemia patients proposed by the Department of Health because of the scale of the problem.

"Only when we have this framework for the uniform collection of clinical and outcome data, can we then audit patient outcomes and treatments.

"Then we will understand the disease better and so improve the management and quality of acute and chronic care."

Dr Asaah Nkohkwo, chief executive of the Sickle Cell Society helped with the NCEPOD study.

He said: "I am appalled by the findings. They show how much of a gap exists in the delivery of the care services for sickle cell patients including the lack of knowledge of health professionals and the consequences that families affected have had to face as revealed by the autopsy reports."

Dr Nkohkwo said the research has been used in the development of the NHS standards of care document for adults with sickle cell disease which is being launched at the House of Commons next month.

Story from BBC NEWS:
http://news.bbc.co.uk/go/pr/fr/-/2/hi/health/7408179.stm

Published: 2008/05/20 23:50:28 GMT

© BBC MMX

USA Senators September 2007

To read the 2 page article, please go to Flyers in PDF page under SC Articles.

New York Times Interview January 1, 2002

Friday, November 27, 2009

Health Guide

 

http://health.nytimes.com/health/guides/disease/sickle-cell-anemia/news-and-features.html

 

 

A CONVERSATION WITH: JOSEPH GRAVES; Beyond Black and White in Biology and Medicine

 

By LINDA VILLAROSA
Published: Tuesday, January 1, 2002
http://www.nytimes.com/2002/01/01/health/a-conversation-with-joseph-graves-beyond-black-and-white-in-biology-and-medicine.html?pagewanted=1

Despite the American obsession with race, growing numbers of experts believe that it is a meaningless concept, at least as far as medicine goes. Dr. J. Craig Venter, the president of Celera Genomics, which recently completed a sequence of the chemical letters in the human genome, has said it bluntly: ''It is disturbing to see reputable scientists and physicians even categorizing things in terms of race.''

But some still see a place for race in medicine, including a number of African-American scientists and medical experts.

Dr. David Satcher, the surgeon general, who is black, has said that ''compelling evidence that race and ethnicity correlate with persistent, and often increasing, health disparities among U.S. populations demands national attention.''

Under his leadership, the government has directed millions of dollars toward eliminating racial health disparities by 2010. More recently, the Association of Black Cardiologists agreed to sponsor a clinical trial enrolling blacks only, to test a heart medicine designed for African-Americans.

In his new book ''The Emperor's New Clothes: Biological Theories of Race at the Millennium'' (Rutgers University Press), Dr. Joseph L. Graves Jr., a professor of evolutionary biology and African-American studies at Arizona State University, argues that races do not exist and that race is simply a social and political construct that the world would be better without.

Racism, he says, is fueled by the idea that human beings can be separated by genetics into races.

Dr. Graves, who is 46 and lives in Glendale, Ariz., discussed genes, race and health recently while in New York City to be on a panel sponsored by the Gene Media Forum, a nonprofit organization promoting dialogue about genome research.

 

***Q. Doesn't race, in the social and cultural definition, have an impact on health? For instance, isn't sickle cell disease much more common in blacks than whites?

 

A. Contrary to popular belief, sickle cell anemia is not a black disease nor did it originate in West Africa. The gene responsible for sickle cell provides protection against malaria, so it is present wherever we find malaria. That includes Greece, Yemen, India, East and West Africa and the Middle East, where it originated. The only reason we think of it as a black disease in America is because the slaves came from West Africa. If the slaves who worked the cotton fields of America had come from Yemen or Greece, then we would have seen it as a Yemeni or a Greek disease.

 

Q. It is still true that blacks suffer from significant health disparities. How do we combat that?

A. No one in America, regardless of socially constructed race, should be getting sick. Studies show that 53 percent of toxic waste sites in America are located in communities that have greater than 75 percent minority composition. Toxic materials are known to have disease causing effects, and mutations can be passed on from generation to generation. So the bottom line is that if we clean up the environment, African-Americans and other minorities will benefit. But so will everyone else. If we also give people access to education and improve the economy, we will begin to see the end of racial disparities in health. These are things we should be doing anyway. It's a win-win situation.

Joseph L. Graves Jr., an evolutionary biologist, holds that biological races do not exist. (Tom Story for The New York Times)

 CDC REPORT: USA SC ER Visits Between 1999 and 2007

http://www.cdc.gov/Features/dsSickleCell_ED_Visits/

Sickle Cell Disease (SCD) Visits to Emergency Departments (ED)

Between 1999 and 2007, approximately 197,333 sickle cell disease ED visits occurred each year.

 

Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS), a survey of hospital ED and outpatient visits, for the years 1999–2007 revealed estimates of approximately 1,475,000 (95% CI = 1,214,000, 1,736,000) and 1,776,000 (95% CI = 1,484,000, 2,068,000) emergency department visits made by patients with a primary or any diagnosis of SCD, respectively. The estimated yearly average number of emergency department visits by patients with any diagnosis of SCD was 197,333. ED visits by patients with SCD were identified using the International Classification of Disease, 9th Revision, Clinical Modification (ICD-9-CM) codes, and nationally weighted estimates were calculated.

 

Reasons for ED Visits

Reasons for ED visits included chest pain (11%*), other pain or unspecified pain (67%), fever/infection (6%), and shortness of breath/breathing problem/cough (5%) among other reasons. Substantial numbers of ED visits occur among persons with SCD. The most common reason for the ED visits is pain symptoms. The findings of this study can help to improve health services delivery and utilization among patients with SCD.

Characteristics of ED Visits

The mode of arrival for most emergency department visits by patients with SCD was walk-in (79%), followed by ambulance (14%; Table 1). The mode of arrival was walk-in for 87% of visits by patients aged 0–19 years, and 76% of visits by patients aged ≥20 years. The age groups with largest percentage of ED visits were 20-29 years and 30-45 years, and the age group with the fewest visits was >45 years.

 

The expected source of payment for emergency department visits by patients with SCD was private insurance, Medicaid/State Children’s Health Insurance Program (SCHIP), Medicare, and other or unknown for 14%, 58%, 14%, and 15% of visits, respectively. Among visits by patients aged 0 –19 years, the expected source of payment was private insurance for 20% and Medicaid or SCHIP for 70%. Among visits by patients aged 20 years and older, the expected source of payment was private insurance for 11%, Medicare for 19%, and Medicaid for 53%.

The percentage of visits indicated to be initial visit, follow-up visit, and unknown as to the episode of care were 66%, 23%, and 11%, respectively (Table 1). Among visits by patients aged ≥20 years, the episode of care was indicated as a follow-up visit for 27% of the visits. Approximately 29% of emergency department visits by patients with SCD resulted in hospital admission. This was 37% among visits by patients aged 0–19 years, and 26% among visits by patients aged ≥20 years. The difference by age group in the percentage admitted to the hospital came close to but did not reach significance (p=0.059).

*All statistics listed in this feature under Reasons for ED Visits and Characteristics of ED Visits have been rounded to the nearest whole number. Co statistics can be found in the charts on this page.

Data Source

Yusuf HR, Atrash HK, Grosse SD, Parker CS, Grant AM. Emergency Department visits made by patients with sickle cell disease: a descriptive study, 1999-2007 . Am J Prev Med 2010;38(4S):S536–S541.

More Information Visit: http://www.cdc.gov/Features/dsSickleCell_ED_Visits/

· Sickle Cell Disease (SCD)

· National Hospital Ambulatory Medical Care Survey

· Sickle Cell Disease: 10 Things You Need to Know

· Listen to Sickle Cell Disease: What You Should Know  ( 6:36 mins)

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