Sickle Cell Anemia Patient Advocate, Navigator or Health Advocate
The concept of patient advocacy in its current form was developed in 1950’s as the treatment of patients grew more and more technically complicated, as a means to make the voice of the patient more readily heard. In today’s world of constantly evolving medical techniques, treatments, and services, the average individual cannot be expected to be thoroughly acquainted with any and all this material. So it becomes the function of the sickle cell anemia patient advocate to perform a variety of services to ease the burden of the patient, and their family, as they learn to deal with every aspect of the disease.
The primary concern of the sickle cell anemia advocate is the support of the patient in all aspects of life with the disease, whether they be physical, emotional, or financial. An advocate can assist the patient in investigating available treatment options and educating the patient on the efficacy of each of them, be it pharmacological or surgical. By educating the patient, the advocate can be of great assistance in helping them make an informed decision about their course of action, and the possible side effects and outcomes. Support may also involve recommending counseling services for the patient, and their family members, to help them cope from day to day. Sickle cell anemia patient advocates may also be of assistance in helping the patient, and their caregivers, deal with any physical debilitation resulting from the disease, and its treatment.
Another aspect of sickle cell anemia patient advocacy is assistance in navigating the financial aspects of treating the disease. An advocate can act as a liaison between the patient and his or her insurance company, assuring that the patient receives any and all benefits to which they are entitled. When one is dealing with a serious disease, it is quite easy to lose track of other aspects of your life. An advocate can help to seek out sources of assistance which can ease the financial burden of the patient.
Education is also a major function of a health advocate. Both the patient, and their caregivers, are often in need of information about how the disease will affect their lives, both during its progression, and in its aftermath. Patients must be informed about how their condition will affect their mobility, the ability to care for themselves, and if they will be left with any permanent impairment to their lifestyle. Caregivers need to be instructed in mechanisms, or techniques they must use to cope with the patient’s daily care, such as injections, proper care of surgical dressings, etc.
The patient, above everything else, needs to know that there is someone in their corner. Someone who will help them find their way through the confusing, and sometimes daunting, world of modern medicine. Someone who will have their best interests in mind as they face challenges presented every day in their struggle to find, secure, and finance the best treatment available.
What is Sickle Cell Anemia?
Sickle cell anemia is one of the several types of blood disorders collectively called haemoglobinopathies. In this disorder, there is an inherited disorder in the hemoglobin genes that makes the red blood cell to deform (becomes sickle-like) when under stress. When this happens, the deformed cells get trapped in the blood capillaries so that there is blood flow blockage and extensive destruction of the cells. With time anemia develops. Common stresses that make these blood cells to sickle include:
· Hypoxia (low circulating oxygen in the body)
· Infections due to bacteria, viruses or protozoal.
· Sometimes there is no obvious cause of the sickling episode which can be so severe that it is referred to as ‘sickle cell crises’.
Symptoms of sickle cell anemia
· Symptoms associated with anemia: dizziness, faintness, pallor on the conjunctiva, tongue and other mucous membranes as well as hands.
· Recurrent pains in parts where the sickled cells have occluded blood capillaries.
· Recurrent infections
· Poorly healing and recurrent skin sores.
· A man may come with a persistent and painful erection – priapism.
· Swellings on hand and feet.
· Poor growth and development in children
· Impaired vision where blood supply to the retina has been impaired
· Swollen abdomen.
· Hotness of the body.
· Weakness of certain parts of the body may be a pointer to an affected brain by the occluding sickled cells.
· There may be yellowness of the eyes – jaundice.
The disease is an inherited disorder of the gene that help the red blood cells maintain their shape even under stress. They do this by means of hemoglobin which is the carrier of oxygen throughout the body. The inherited and abnormal hemoglobin makes the cell misshapen, sticky and rigid. A normal blood cell will spring back to normal shape even after being distorted while passing through small capillaries.
· Having one parent who is a carrier of the defective gene.
· Having two carrier parents
· Having a parent who has sickle cell disease
· Although sickle cell can be found in all races, it is more common in Blacks than in White in the U.S. The incidence in Africa, Asian countries and Mediterranean countries is higher.
A detailed history taking and examination is useful before engaging in more invasive testing methods. These tests include:
· Testing blood for hemoglobin S. Other tests are done to confirm whether a person has both sickle cell genes or has just one (sickle cell trait.)
· A complete blood count and other hematological tests are also done.
· Pre-natal screening for sickle cell genes in high risk babies may also be done.
The treatment used depends on the current state of the patient and the disease. It may involve:
· Management of concurrent infections
· Treating symptoms of inflammation as a result of vascular occlusions.
· Treating complications
· Specifically treating the sickle cell disorder itself.
Currently there is no cure readily available for all patients but measures to make life more bearable for patients are there. They include:
· Medications. Common ones used are Hydroxyurea (so far the most used), sodium thiocyanate, Nicosan, Senicapoc, Lexican (Regadenoson) and decitabine. Other medications may be analgesics and anti-inflammatory agents and antibiotics. In malaria endemic regions, prophylactic antimalarial agents are given.
· Bone transplant has been used successfully to reverse sickle cell but the main problem is to get compatible donors.
· Gene therapy is a new approach to the management of sickle cell and other inherited disease. The technology though promising is still in its infancy stage.
· Nutritional supplements like multivitamins and essential fatty acids are also helpful.
· Sometimes blood transfusions are inevitable.
U.S. Statistics for Sickle Cell Anemia
· Estimates from the CDC show that there are about 90 000 to 100 000 Americans affected.
· The rate among Blacks and African-Americans is 1:500 births.
· Among the Hispanic-Americans it is 1:36000 births
· Sickle cell trait is found in 1:12 African-Americans and Black population.