Sickle Cell Disease

TREATMENT OPTIONS

There is NO cure for sickle cell disease, although new stem cell research is promising. Bone marrow transplant is a potential cure, however finding a matched sibling donor is difficult. Therefore treatment is primarily focused on management of symptoms and prevention of pain episodes. Hydroxyurea (a chemotherapeutic medication) is the only recognized treatment option for the prevention of vaso-occlusive crisis in SCD. It is a prescribed drug for adult patients who experience more than three pain episodes causing hospitalization, as well as two or more life threatening complications. The drug reduces the frequency of pain crisis and potential complications related to SCD. Alternatively, cognitive behavior therapy (CBT) is a psychological intervention that assists patients in developing coping strategies for pain symptoms. Some coping mechanisms include thought monitoring, distraction, relaxation and activity rescheduling. In a study done by Cummins & Anie (2003) it was shown that patients treated only using CBT experienced more pain crises but fewer hospitalizations than patients taking hydroxyurea therapy. However, they expressed a more positive overall health outlook. Blood transfusions are sometimes administered to patients in specific circumstances such as cerebrovascular accident (stroke), acute chest syndrome, or when sickle cell crisis are so recurrent that damage to the organs occur (Brown, 2012).
Managing the social effects of the disease should comprise of a solid social support group where patients, families and healthcare professionals can benefit from an open forum of communication, fostering education about the illness for all contributing members (Jisieike, 2007).
ADDITIONAL RESOURCES

For more information about sickle cell disease or to provide your support to the SCD community, please contact:
The Canadian Sickle Cell Society
6999 Ch. De La Cote-Des-Neiges #33,
Montréal QC,
H3S 2B8
514-735-5109

If you are a caregiver or family member of someone living with Sickle Cell Disease please visit the following websites for additional information.
Sickle Cell Awareness Group of Ontario http://sicklecellanemia.ca/ Iron Health Alliance http://www.ironhealthalliance.com/disease-states/sickle-cell-disease.jsp -------------------------------------------------

“YOU HAVE SICKLE WHAT?”

Sickle cell disease (SCD) is the most common genetic condition worldwide (Brown, 2012) primarily affecting people of the African and Caribbean descent. It is prominent also in the Middle Eastern and Mediterranean regions. It is an autosomal disorder inherited by an infant when both parents pass on one copy of a faulty β-globin chain.
The disease is characterized by a malfunction in haemoglobin synthesis resulting in poor oxygen transport via red blood cells. The deoxygenated red blood cells then become shaped similar to a sickle (it is after this farmer’s tool that the disease is named). As the cells change shape they no longer flow easily through blood vessels and consequently block oxygen and nutrients from supplying tissues. This results in vaso-occlusive crises VOC.
During a VOC, the patient experiences severe pain in the abdomen, extremities, chest, and/or back. These pain episodes are unpredictable but can be precipitated by stress, dehydration, smoking, alcohol use, and weather changes. In many case the pain is so intractable that the patient requires hospitalization, where they are given opioid analgesics to manage the pain and intravenous fluids for rehydration.
Complications from SCD include stroke, acute chest syndrome, and damage to vital organs, any of which can potentially lead to a fatality.

EFFECTS OF SCD

In the absence of pain episodes the sickle cell patient is often considered asymptomatic. The invisible nature of the disease can lead to the common belief that people who suffer from SCD are healthy when they are not experiencing a VOC. However, it is important to consider the biological, cognitive and social effects that can negatively impact the quality of life of adults living with SCD.
According to Erikson’s 8 stages of development, adults over thirty years old must resolve the crisis of generativity versus stagnation mostly by giving back to the future generation, starting a family, finding success in a career, or helping others (Boyd et al., 2012). Adults living with SCD are likely to have had unresolved growth and development difficulties originating in early childhood and increasing in adolescence. These impediments include delayed reticular formation and decreased visuomotor skills in early childhood, and poor self-esteem, and delayed sexual development in adolescence. As a result of these developmental shortcomings, they will often withdraw from social relationships and consequently experience a lack of intimacy. By adulthood, sickle cell patients often find it difficult to maintain stable employment due to unpredictable pain episodes. Often times they will not tell their employers that they have SCD for fear of being let go due to possible missed work days (Jisieike, 2007).
Fatigue is another symptom of SCD affecting job performance. After pain, fatigue is considered to be the most significant feature of SCD. According to Ameringer & Smith, hypoxemia and inflammation are key contributors to SCD fatigue (pain, stress, depression and anxiety are also associated). Adults experiencing fatigue associated with SCD describe having low energy and an inability to carry out activities of daily living. This can lead to “interference with cognitive function, social withdrawal, modified daily activities, and decreased psychological well-being” (Ameringer & Smith, 2011).
EFFECTS OF SCD

Perhaps the most destructive psychosocial effect of SCD in adulthood is the disturbance of social relationships. It is not uncommon for adults experiencing pain or fatigue due to SCD to have to rely on a spouse or family members to act as caregivers. This often puts a strain on relationships because it creates and imbalance in the relationship and according to Butler and Beltran (1993) it leads to the patient becoming dependent on others to carry out their responsibilities. In terms of childbearing, passing the disease on to future offspring is of great concern. New born screening is now standard in all hospitals across North-America, however choosing a partner that is not a carrier remains a concern for many adults with SCD.
Sickle Cell Disease is a debilitating disease that affects the physical, cognitive, and social domains. Participation in an adult social support group is crucial to providing patients with an outlet to communicate with others facing the same or similar circumstance. It allows SCD patients to relate to others and discover ways in which some cope with the effects of the disease on their quality of life.