Imagine having to constantly watch your child endure excruciating pain. A pain that has been described as feeling like someone is repeatedly stabbing you in the joints with a butcher knife. Or making sure your child takes the many daily medications. Or watching your child watch other children play in a pool they can’t play in because it’s too cold for their body.
|TAKING A STAND—Andrea Williams, director of the CSCF, stands with her son Jonathan, a child dealing with sickle cell disease.
When Tanya D. Davis’ daughter Tatiana was born she found out that she had the trait for sickle cell. Her daughter is now six and since being diagnosed has had her spleen and gallbladder removed, suffers severe episodes of pain called crisis and has been through five to six blood transfusions. And is constantly in and out of the emergency room.
Both Cadiadra Kendrick and her husband were carriers of the sickle cell trait. When she got pregnant with twins it was suggested by her doctor that she get an amniocentesis because the percentage of her children developing the condition increases. After finding that one of her twins had sickle cell, there was a suggestion of a termination, but for Kendrick that was not even an option. Today her son is 12 and even though he has his ups and downs, he still has a positive attitude.
These are just a few of the things that families and children with sickle cell endure on a daily basis. But it is the Children’s Sickle Cell Foundation Inc. that is trying to make it easier for families to cope with this disease.
“This is an organization formed for the parents by the parents (of children with sickle cell disease),” said Andrea Williams, executive director of CSCF. “The foundation was formed in 2002 and prior to that there were no organizations devoted to children with sickle cell disease.”
According to the CSCF website, sickle cell disease is an inherited blood disorder that affects red blood cells, causing the cells to change their shape. The red blood cells contain hemoglobin, a substance that carries oxygen from the air in the lungs to all parts of the body. Normal red blood cells contain hemoglobin “A,” they are soft and round and can easily pass through tiny blood vessels.
People with sickle cell disease have red blood cells that contain mostly hemoglobin “S,” an abnormal type of hemoglobin. The cells become sickle or crescent shaped, stiff and sticky and have difficulty passing through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what can cause complications of sickle cell disease.
According to the Centers for Disease Control and Prevention, there are 70,000 people in the United States with sickle cell, two million have the trait and it occurs in one in every 500 African-American births.
Dr. Lakshmanan Krishnamurti, director of the comprehensive sickle cell program at Children’s Hospital, said there are approximately 450 individuals, both children and adults, in the region that have been diagnosed with the disease. He also stated it is unknown whether the disease is increasing or declining due to the lack of statistics.
“We do not know whether the disease has increased or decreased over there years. There is no registry and no national reliable statistics out there, only estimates,” Krishnamurti said. He added there has been a recent effort by national health administrations to get more concrete statistics.
Krishnamurti said the lack of statistics also comes from a lack of access to health care, especially in the African-American community. A lot of people go undiagnosed because they do not have health care. “If an individual does not have access to health care, how can they find out if they have disease?” He said there is not enough awareness out there and not enough information.
Williams is not only the director, but the parent of a child with sickle cell disease. Both she and her husband are carriers of the trait. Her two other children carry the trait and her son Jonathan has the disease.
Williams said it is hard to watch her son suffer through this disease. He is typically a normal child, but there are things he he has to do that other children don’t have to. He takes daily medications, has to constantly carry a water bottle to make sure he does not get dehydrated. She said she tries to teach him everything about the disease and the medications.
“I had to teach the principals and teachers about the disease (and how to react). And that he is allowed to take as many water breaks as he needs and even in gym class, he is allowed to rest when he needs to,” Williams said. “I encourage him to participate, but I also teach him to know his limits and when he needs to rest.”
Williams said the main goals of the foundation are to give families dealing with this disease support and to educate people. She said through education comes understanding, especially when it comes to the myths.
There are a lot of misconceptions about sickle cell disease. Williams many people think the disease is contagious and if you are found to have the trait it means you have the disease. Both of these are not true. But the greatest misconception is that this is a disease that only occurs in African-Americans. And while it affects a greater number of people of African descent, it can be found in other ethnicities, such as those of Mediterranean and Middle Eastern descent.
There are several programs that the CSCF offers for individuals and families affected by sickle cell. The foundation, in partnership with the Pittsburgh Public Schools, designed the Education Support Program, where staff go to the schools and talk to the principals, teachers and sometimes the students to discuss the disease. There is also a coordinator who works with the school to pick up assignments due to missed days.
Kendrick said the foundation has been very helpful. Her son, Denzelle, 12, has missed more than 40 days of school due to his illness, but managed to pass with good grades because of help from the foundation that made sure he stayed current on his schoolwork.
The foundation also offers a swim program, where kids can swim in heated pools to decrease stress levels, exercise and work on strength and flexibility; a Read 2 Lead and Math 4 Masters program, which helps kids to look at math and reading in a different way; and their Saturday SMASH 4 Success program.
But it’s the foundation’s Family Support Program that makes the most difference. “The foundation has helped me cope,” Davis said. “They make you feel at home. When I needed to learn things about the disease, they taught me. I have friends and family that are supportive, but it’s nice to have people who are going through what I am. Sometimes I may be going through something that someone else has been through and can help me and vice versa.
“We are so close knit. Everyone knows each other. You do not get that from the national sickle cell foundation because it is too large. They do not know you and my son is more than a number.” Kendrick added. “The foundation has made it a lot easier. It keeps me sane.”
With the activities that the foundation provides, Williams said they always try to make it a family event. “Everything we do, we include the siblings, because we feel this disease affects the entire family, not just the person going through the disease.”
Besides the Pittsburgh Public Schools, some of the foundation’s other partnerships are the Children’s Hospital, the Children’s Institute and the YMCA Allegheny.
The CSCF is now in the process of developing a program called the Parent Education Advocacy and Transition Tool Kit Program. It will help parents learn about the needs and changes that will come as their children grow older with this disease through age-appropriate modules. For instance, as a child grows, the medicine and doses change, so parents will learn what is to come and how to address it.
It will also have something for teens and young adults. They will learn about taking care of themselves, not only health wise, but educational and career wise. “We have a portion that will help to prepare teens and young adults for the future. For instance, we have them choose a career that they’d like to go into and then we work to show them what will be needed to get them to that point. For instance, we show them what they need to take in high school and college,” Williams said.
Williams said the foundation’s next step is talking to legislators and discussing the success of the programs and the need for funding.
“We want funding for research. It’s been lax. We need to raise awareness of the lack of research being done,” she said. “Sickle cell has been around for a long time and there is only one medication, whereas HIV/AIDS has been around a shorter time and there are numerous medications available.”
The medication that Williams is referring to is Hydroxyurea. It reduces the frequency of severe pain. The FDA approved it in 1988.
She said that everywhere she goes she sees fund-raisers and collections taken for various causes, but never for sickle cell and that hurts her.
“It hurts my heart. This disease did not die off, it is very much here and alive and more needs to be done,” Kendrick said.
She said she created this foundation to help people. “The long nights and writing grants is all worth it to know that it’s making a difference. When I go to speaking engagements I am not just speaking for me, but for all the families affected. There are 111 kids in the program and 250 kids in Allegheny County that have this condition. We want to help them all. We no longer want to be the best kept secret.”