The Sickle Cell Disease Association of America estimates more than 70,000 people in the United States have sickle cell disease. As an inherited disorder primarily among African Americans that affects the red blood cells, there are estimated to be more than 200 sickle cell patients in New Orleans.
As the city repopulates after Hurricane Katrina, local emergency rooms are seeing more patients seeking help for the pain caused by the debilitating disease. The new Tulane University Health Science Centers Sickle Cell Day Hospital offers specialized care and new treatment options.
Sickle cell anemia or disease is an lifelong condition with no cure. Normal red blood cells are smooth and round and can move easily through the blood vessels to carry oxygen to all parts of the body. Disease carriers have abnormal hemoglobin strands that cause the cells to form sickle shapes. As the sickle cells begin to pile up in parts of the body, they prevent blood flow and can cause infections, lung tissue damage, stroke and priapism in men.
The life expectancy of those afflicted with the disease is 42 years, but with careful care and new treatments, some patients can live longer. Since there is no cure, patients spend most of their time trying to control and manage the extreme pain that occurs when sickle cells build up and stop the flow of blood to and from various parts of the body.
Most of the pain tends to be in the arms, the legs and in the back. You can get it anywhere though. It is absolutely excruciating. Most people try to treat it at home with medications and fluids but it often gets out of hand, said Rebecca Kruse-Jarres, MD.
In post-Katrina New Orleans, a lack of primary care physicians means that it can often take up to a month to get a doctors appointment. As a result, many of the citys sickle cell patients have been coming to the emergency rooms seeking treatment. Kruse-Jarres said that in the EDs, few doctors and nurses have specialized training or knowledge in the treatment of sickle cell. The new Tulane day hospital will meet a need by offering a place where patients can get regular care and treatment from a staff that specializes in the disease.
The day hospital has five beds and a full-time staff consisting of a nurse, a nurse practitioner and a social worker. Open during the daytime hours to those over the age of 18, the hospital works with patients to create a multi-disciplinary program of care and can treat patients when the pain gets out of control. A social worker will also work with the hospital to help patients manage the pain and to help them secure insurance or coverage for medical bills.
Were not just treating pain as everyone does and letting that be the end of the story. Were trying to understand the pain, the different types and how they have to be treated. The social worker will be in once a day to do assessments on the patients. Weve really incorporated the psychological aspects of pain management, said Kruse-Jarres.
She also said that the sickle cell day hospital will work in conjunction with personnel in the emergency room to develop guidelines for seeing and admitting sickle cell patients.
The hospital is being subsidized by a $420,000 grant from Baptist Community Ministries that will cover the nurse practitioner and social worker for three years. Tulane offered space and a nurse to the program. The idea for a sickle cell day hospital was based on the sickle cell clinic at Montefiore Medical Center in New York. The Adult Bronx Comprehensive Sickle Cell Center there has been a national leader in treatment and research on the disease.
Every time patients go to the emergency room they see different doctors but at the Tulane day hospital they will now be able to see the same practitioners. Dana Sylvester, RN, who is staffing the hospital, said that she has seen the trouble with sickle cell treatment first hand through the eyes of a good friend.
She has been in and out of the hospital. Ive personally gone to the emergency room many times and waited for hours and hours. There were so many people that just did not understand [the disease] and were reluctant to care for her. It seems like theres sometimes a stereotype against treating those with sickle cell, said Sylvester.
Nikita Ussin, a student a Xavier University of Louisiana who suffers from the disease, says that pain crisis can make normal routines and life difficult. She considers herself fortunate in that she has been able to manage the disease and is only admitted to the hospital a few times per year.
At the day hospital, Im just really looking forward to talking to someone who knows about sickle cell. Many people in the emergency rooms or other doctors just dont know the disease, said Ussin.
Kruse-Jarres also says that one of the best aids in coping with and caring for sickle cell is that patients understand and take ownership of their disease. By educating themselves on their ailment and learning to listen to their bodies, they can substantially decrease the onset of pain by the accumulation of sickle cells in various parts of the body. Some of the best ways to avoid pain is for them to stay well-hydrated, avoid extreme temperatures, stay away from stress and not engage in extreme exercise.
Sickle cell patients need continuous treatment, even when they are not experiencing a pain crisis. Kruse-Jarres said it is not uncommon for sickle cell patients to come to the hospital a few times a week or even on a daily basis in search of help. Although there is no cure, the purpose of treatment is to manage the systems and prevent the onset of pain. Many patients try to treat themselves at home by consuming lots of fluids to flush the sickle cells through the vessels.
The way you want to treat them is get them lots of fluids to flush it through, get them some pain medication because its just excruciating. You want to give them oxygen and some blood with some normal hemoglobin to dilute their sickle cells, said Kruse-Jarres.
Long-term TreatmentsDana Sylvester, RN, and Luann Wenthold, LPN, serve as the primary staff for the day hospital and are learning everything they can about sickle cell.
There is no cure for sickle cell disease. Long-term treatments include hydroyurea, a medication that increases the hemoglobin F content in the blood and has been shown to reduce pain crisis and increase life expectancy to those who are responding.
Another treatment is to transfuse or exchange a patients blood once a month and keep the sickle cells from forming and blocking vessels. The final treatment is a bone marrow transplant, but it is rarely performed on sickle cell patients because of the risks and high mortality rates.
Complications from sickle cell are usually prevented or minimized by proper nutrition, good hygiene, lots of rest, hydration and observance of how the body reacts to different situations.