Sickle cell anemia

 

Sickle cell anemia is an inherited genetic disorder and involves the abnormality in the oxygen-carrying protein hemoglobin in the red blood cells. Hemoglobin normally carries the oxygen into the capillaries throughout your body to diffuse the tissues and organs with oxygen.

 

In this disease, the normally shaped red blood cells take on a crescent or sickle shape and this can lead to various symptoms, some of which can be life-threatening. This disease is most common in people of African, Indian, or Arabian descent, and there are many cases of Sickle cell anemia in the US for these nationalities. Adequate genetic testing of parents who have this disease can prevent the spread to offspring, and therefore limit the spread of this disease.

It is certainly advisable that patients who have this disease rely on regular medical care from an experienced provider who understands this disorder and familiar with treating the symptoms. The severity of symptoms can vary from person to person and can be chronic or acute. Acute symptoms are life-threatening and require immediate medical care or hospitalization.

Patients with this disorder can enter a crisis phase in addition to normal daily discomfort and chronic pain which they may also experience. Because the red blood cells in this disease are shaped abnormally they can get stuck in your circulatory system, particularly in the capillaries. This can lead to a vaso-occlusive crisis where the affected organ can have impeded blood flow and lack of oxygenation, and suffer from avascular necrosis. These painful episodes are treated with hydration, analgesics, blood transfusions, oxygen therapy, or pain control using self-delivered opioid delivery systems. Some milder crisis with this disease can be treated with over the counter meds and Nsaid’s, Tylenol, hydroxyurea, ibuprofen or anti-inflammatories.

 

In severe cases, the spleen, chest, or kidneys may be impacted with life-threatening consequences if left untreated. Often a blood transfusion with fresh normal red blood cells is needed to restore proper balance, or strong antibiotic therapy to prevent infection, as well as other supportive care measures.

 

The role of cannabis in this disease is strictly palliative to help with the adverse symptoms, and chiefly to help the patient combat pain and discomfort. Some cannabinoids have been shown to be effective as anti-bacterial and anti-inflammatory agents, and some cannabis methods of use can help to combat the chronic pain and discomfort of this disease on an ongoing basis. For flair ups, narcotic medications may be used along with high CBD cannabis to potentiate the narcotic and require a lower dose or less frequent dose of the narcotic. Many patients who have this disease are also more likely to already use cannabis, as studies recruiting patients have some difficulty in finding naïve users who have never used cannabis. It appears that many are self-medicating to allow them to feel better and mitigate some of the negative feelings that are associated with this disease by already using cannabis.

 

Explicit inclusion of sickle cell disease as a qualifying condition for medical marijuana might reduce illicit marijuana use and related risks and costs to both persons living with sickle cell disease and society. Among users of cannabis with this disease, a majority used cannabis for pain, anxiety, mood, sleep, and appetite control. There is a strong rationale for the use of medicinal cannabis and/or its constituents in sickle cell disease and anemia. However, the ultimate cause of the disease and the necessary treatment does not specifically benefit from cannabinoids, they are best at only treating the symptomatology and in no way offer a cure. Some patients may receive a cure through bone marrow transplants, but these patients are limited.

 

The use of illicit marijuana/cannabis places persons living with sickle cell disease at risk for both exposure to poisonous contaminants and arrest and incarceration for breaking the law. More widespread allowance of sickle cell disease as a qualifying condition for medical cannabis might reduce the personal and social costs of illicit cannabis use in this group of patients, as well as allow these patients the freedom to use cannabis if they feel that it helps them deal with their disease!