SICKLE CELL RAPPER
Fall, 1999 The Children's Sickle Center, San Antonio, Texas
Sickle Cell Awareness Month
September is here again. The children are back in school, and families have once again begun that busy daily schedule. Everyone is looking forward to Labor Day, the last summer holiday weekend.
We would like to remind our readers that September is designated as the month to raise public awareness about Sickle Cell Anemia. Many people are unaware of the potentially devasating genetic disease and its long term effects on the individual and their family. If you are a family living with sickle cell disease, please take the time to encourage family and friends in their child-bearing years to talk with their doctor, and seek screening and counseling regarding their genetic status. Those with the disease should seek and maintain good health care follow-up with a hematologist, and utilize resources that contribute to a better quality of life.
- Yvonne Shannon R.N., M.S.N.
South Texas Sickle Cell Center
Sickle Cell Disease Overview and History
In the United states sickle cell disease is one of the most common genetic disorders and affects approximately 50,000 individuals, including three to four thousand Texans. The disease occurs in one of every 350 African Americans born in Texas. About one in every eight African American newborns in Texas has sickle cell trait.
Sickle cell disease is an inherited disorder of the red blood cells. A person with the disease has abnormal red blood cells that lose their normal, round shape, and take on a sickle shape that impedes the flow of oxygen in the blood. The disorder includes sickle cell anemia (SS), the sickle beta thalassemia syndromes (S-beta +/0 Thal), and hemoglobinopathies in which Hb S is combined with other abnormal hemoglobin in sufficient concentration to enable red blood cells to sickle. Examples of the later are hemoglobin SC disease Hb SC), hemoglobin SD disease (Hb SD), and hemoglobin SO Arab (Hb SO Arab).
The person with sickle cell disease, has inherited a sickle gene from one parent and a sickle gene or another abnormal gene from the other parent. The individual with sickle cell disease has no adult normal hemoglobin (Hb A), or she/he has more of the abnormal hemoglobin that Hb A.
The individual with sickle cell trait has inherited one sickle gene (Hb S) from one parent and one normal gene (Hb A) from the other parent. A child conceived by two parents with sickle cell trait has a one in four chance of having a child with sickle cell anemia, with each pregnancy.
Sickle cell anemia was first identified as a disease of the red blood cells by Doctor Herrick in 1910. It was not until sixty years later that many people became aware of sickle cell disease. In 1970 a campaign to initiate sickle cell screening along with educational programs emerged and reached its climax with President Nixon's health message to the 92nd congress. It called for a vast increase in federal expenditure to deal with sickle cell research, education, and screening programs. This led to the passage of the National Sickle Cell Anemia Control Act on May 16, 1972.
During the past twenty-five years, much progress has been made in the identification and care of the client with sickle cell disease. The most dramatic progress has been in the area of early identification and treatment in children with sickle cell disease.
On November 1, 1983, Texas began screening all newborns for sickle cell disease. At tis time there were only two other states that were screening all newborns for sickle cell disease, New York and Colorado. Currently forty-six of the fifty states have some type of program in place to screen newborns for sickle cell disease.
Some occurrences that have improves the identification, care, and treatment of sickle cell patients are as follows. 1) June of 1986, The New England Journal of Medicine published the penicillin study. This study clearly demonstrated that prophylactic penicillin reduced the incidence of pneumococcal sepsis by 84%. 2) The consensus statement from the National Institute of Health (niH) was published in April of 1987. This statement concluded that every child should be screened for hemoglobinopathies to prevent the potentially fatal complications of sickle cell disease during infancy. 3) In 1987, the federal government provided supplemental funding to states for the development of hemoglobinopathy screening programs or to expand existing programs. These funds were available through the Maternal and Child Health Special Projects of Regional and National Significance (SPRANS) program. Through these grants, many states were able to develop hemoglobinopathy screening programs in combination with their existing newborn screening programs, or expand existing programs. 4) January 1995 the National Heart, Lung, and Blood Institute (NHLBI) announced the use of hydroxyurea to reduce the frequency of painful episodes or "crises" in adult patients with sickle cell anemia. A multi-center, double blind study showed administering daily doses of the drug hydroxyurea, reduced the frequency of painful episodes and hospital admissions for painful episodes by approximately 50%. In addition, hydroxyurea therapy significantly reduced the frequency of acute chest syndrome, a life-threatening complication of sickle cell anemia characterized by chest pain, fever, prostration, and abnormal chest x-rays. Currently hydroxyurea is also being used in treatment of children with sickle cell anemia.
Although the only known cure for sickle cell anemia is bone marrow transplant, many positive advances are being made. Thirty years ago, it was estimated that 50% of all sickle cell patients died before the age of twenty. Now about 90% of the sickle cell patients survive past the age of twenty. Recent studies indicate that the mean age at death for Sickle cell anemia patients is 42 years for males and 48 years for females. Most patients with sickle C disease and Sickle beta + thalassemia can be expected to reach age 70 years and older. Many patients live a full life, and patients age 50 years now constitute a large segment of the adult population with sickle cell disease.
Today with newborn screening and follow-up, prophylactic antibiotics, new medicines, and advanced procedures the client with sickle cell disease has a life expectancy much better than thirty years ago.
Mae Wilborn, BSN, MAHS
Newborn Screening Program, Texas Department of Health
South Texas Regional Children's Sickle Cell Program
The staff of the regional center would like to take this opportunity to introduce our program to our readers who may not know the services and scope of the program.
We serve the patient, the family and the community. Our aim is to provide and manage family oriented, integrated, quality care for infants and children identified with sickle cell anemia.
Listed below are the specific aims and goals of the program:
Specific Aims of the Program
- To implement the following services:
newborn follow-up, homevisits, education of medical professionals, education of school personnel, education of nursing students, community education and referral for developmental/psychosocial assessments and other needed services.
- To integrate primary preventive care and special hemotological care according to medical management protocols for sickle cell anemia.
- To maintain computer based database for tracking patients.
Goals of the Program
- To increase the life expectancy and quality of life for infants and children in South Texas with sickle cell disease.
- To improve access and integration of medical services for infants and children in South Texas with sickle cell disease.
- To provide medical information regarding medical treatment of infants and children to medical students, resident, fellows, nurses and other professionals in South Texas.
- To promote utilization of existing systems to enhance comprehensive services for families and patients of sickle cell anemia in South Texas.
|South Texas Comprehensive
Sickle Cell Program
|Community Agencies Network
||Other Medical Professionals Network
||Selected Services Proovided to Network
|Sickle Cell Association
||Primary Care Providers and Clinics
|Head Start Day Care Centers
||Parent Support Group
||Adolescent Support Group
||Area Emergency Rooms
|Local Health Department
- Medical and Nursing
- Patient Care including:
- Patient Education
|School of Nursing
||Adult Patient Coordination
Yvonne Shannon, R.N., M.S.N.
Program Nurse Coordinator
South Texas Children's Sickle Cell Center
Chimerism...Cells of different genetic makeup living together
Presently we know that the only cure for Sickle Cell Anemia is a bone marrow transplant from a tissue matched donor. A brother or sister who have the same parents. During the usual procedure, the patient's bone marrow is generally wiped out to allow space within the marrow for the donor's cells to grow. The result is that no sickle cells are left, and if the marrow "takes", the patient produces only normal hemoglobin A. The period between the marrow wipe out and when the new graft takes hold, may last 1-2 weeks, and the patient may be quite sick and even die. New research suggests that it may not be necessary to do this marrow wipe out. Instead, they would simply provide the patient with marrow to produce hemoglobin A, along with hemoglobin S, and thus convert the patient to a sickle cell trait. Sickle trait is a condition not associated with sickness. Sufficient time has not passed to know how long this side by side production might work, but it is another attempt toward producing a cure and reducing the risks. We shall see.
-Howard A. Britton, M.D.
South Texas Children's Sickle Cell Program
When "Doing Anything" Becomes "Doing Everything"
It is not unusual to hear the parent of a child with a chronic illness say "I would do anything to help my child. I would take their place if I could." While we can't take the place of someone else, this sentiment sometimes becomes translated into doing everything for the child. Parents, grandparents, and even doctors and nurses sometimes try to protect children from life's bumps and bruises by taking over life's chores and responsibilities. Yet, those bumps and bruises are often life's mose effective way for a child to learn. Not getting things "perfect" teaches us that we aren't infallible. This lesson teaches us that people still love us even if we aren't perfect. Making a mess and having to clean it up teaches us to be responsible for ourselves-and even to plan ahead so we don't make such a mess next time. Falling down on roller skates teaches us how to keep our balance. Children often learn one step at a time. If we take the first 10 steps for them, the eventual fall is likely to be much harder.
-Judith Grant, Ph.D.
Camp Sky 1999 was a raving success! Sixty-three campers from Beaumont to El Paso attended Camp For All July 5-9, 1999. Camp Sky was a week of fun filled activities. Campers, ages 6-13, boarded chartered buses at the Sickle Cell association on Monday morning. Campers enjoyed exciting activities such as swimming, water games, canoeing, fishing, archery, art & crafts, basketball, horseback riding, mountain bikes, hayride, mini-Olympics, talent show and campfire. Even Education was fun! A jeopardy-like game help campers learn about sickle cell disease. Skull sessions gave campers the opportunity to discuss issues or problems they face every day.
The Camp For All facility is especially designed for children with disabilities. Our own Camp Sky physician, and two nurses kept tabs on the campers. Regularly scheduled meds and/or any other minor crises that may arise are dealt with at the on-site infirmary. Local hospitals and pharmacies in the area are educated about sickle cell disease and put on alert during our stay. They are also provided educational material and notified of any meds that may be needed. Once again Texas Children's Hospital staff, Prairie View Nursing students and community volunteers formed our medical team.
Our volunteer counselors were great! Veterans from last year and newly trained volunteers helped us meet our counselor camp ration 1-3. After sixteen hours of sickle cell disease education and training, including Psychosocial issues, CPR certification and medical symptoms, volunteer were well prepared. The air-conditioned cabins made scheduled afternoon rest periods a refreshing break. Campers drank plenty of fluids which helped them stay out of the infirmary.
Family Day, Thursday July 8, brought 88 family members to Camp Sky. Speakers shared important information on what it means to live with sickle cell disease and resources for parents. Thanks to SCA board members, Isiah Sanders and Borris Miles, the families received free transportation to camp. Family day is open to anyone wanting to visit/tour the camp or simply want to be informed about sickle cell disease.
Camp is very important for children with disabilities. For many children with sickle cell disease, attending camp might seem impossible due to chronic health problems and overwhelming treatment related expenses. Today, however, there is no reason a child should miss the experience of Camp. Camp Sky emphasizes wellness, promotes self-esteem, self confidence and provides the opportunity for campers to meet others with sickle cell disease, as well as, increased personal growth and independence. Local Chapters and the State Association work to secure sponsors for interested campers.
Please consider signing your camper up next year for a positive and rewarding experience!
-Adrian LeBlanc, SWA
Camp Educational Coordinator
My name is La Rae, I have Sickle Cell. This year was the first year that I went to camp, and I had a blast. I went swimming, rode horses, and went canoeing. I met a lot of people that have sickle cell. We had a talent show and we won first place. We had a dance and I really enjoyed the dance. I had fun, and I was not shy because the kids all understood me, because we all have things in common with sickle cell. Nobody was different.
We played a game called skull session, where you learn about sickle cell. The game had categories, T.V., movies, sickle cell, sports. Sickle cell category had the most points. You are learning about sickle cell and having fun at the same time. We also cooked a cookie pizza, climbed a wall, and went fishing. I had such a good time, I didn't want to leave camp.
La Rae Brown, age 13
South Texas Sickle Cell Center
Christus Santa Rosa Children's Hosp
San Antonio, Texas
P.S. I scored 95% on the Sickle Cell Disease Review, and I know my hemoglobin type.
CHRISTUS SANTA ROSA
The Children's Sickle Cell Center
519 West Houston Street
San Antonio, Texas 78307-3198
(210) 704-2187 (800) 227-3618
(After hours, call 704-2011 and ask for Hematologist on call.)
Anne-Marie Langevin, MD
Chief, Division of Pediatric Hematology/Oncology-UTHSC-SA
Howard A Britton, MD, FAAP
Medical Director, Pediatric Hematologist/Oncologist
Reginald Moore, MD
Associate Medical Director, Pediatric Hematologist/Oncologist
Javier R. Kane, MD
Anthony Infante, MD, PhD
Pediatric Hematologist/Oncologist Immunologist
Paul J. Thomas, MD, FAAP
Director, Pediatric Oncology Clinical Services
Shafqat Shah, MD
Leanne Embry, PhD
Psychology Fellow/Assistant Professor
Elisa Ornelas, LSW
Sickle Cell Social Worker
Yvonne Shannon, RN, MSN
Sickle Cell Disease Nurse Coordinator
Editor of the Sickle Cell Rapper