Chronic pain, health complications and frequent trips to the emergency room are everyday concerns for an estimated 10,000 New Yorkers living with sickle-cell disease. Together, these individuals account for 10 percent of the country’s population diagnosed with the inherited blood disorder. Despite this geographic concentration, patients, advocates and health providers say that funding and overall quality of care continues to fall short in New York State. In 2017, the state allocated $250,000 to sickle-cell in its annual budget. Legislation to boost that funding has been introduced in both the New York State Assembly and Senate, asking for a budget of $3 million. Related content

Through a small academic setting survey, researchers at Yale University have discovered that sickle cell disease (SCD) patients are using marijuana to relieve symptoms related to the condition. How the substance is affecting these users and whether SCD should be added to the list of conditions that may be legally treated with medical cannabis is still in question. While medical marijuana in the US is legal to alleviate pain caused by several health conditions, only three states (Connecticut, Ohio, and Pennsylvania) include SCD as an approved condition. The Yale survey showed that SCD patients typically use marijuana as an alternative to opioids or supplement other pain relief treatments. The survey included 58 SCD patients (age 21 and older), seen at an academic medical center, who were anonymously surveyed for their use of marijuana, cocaine, and phencyclidine (angel dust) for pain relief. Of the total study population, 42% reported using marijuana within the past two years; most patients said they used it for medicinal purposes to help with pain, anxiety, appetite, mood, and sleep. In addition, 79% of these patients reported taking less of their pain medications after marijuana use. Related content

One of the first things nurses learn about pain is that it is “whatever the person experiencing it says it is.” Yet when individuals with sickle cell disease (SCD) report severe pain, they are frequently met with disbelief and their pain is often inadequately managed. Also, most individuals with SCD are black (though the disease can affect any race or ethnicity) and many report experiencing discrimination during such interactions. In fact, race-based discrimination for patients with this disease contributes to negative provider attitudes, longer emergency department (ED) wait times, insufficient treatment, and lack of research funding. SCD describes a group of inherited blood disorders that often cause hard, sticky, and sickle or C-shaped red blood cells. It affects 90,000-100,000 Americans and millions globally. The most prevalent clinical complication of SCD is sickle cell crisis, an episode of severe, acute pain caused by occlusion of small blood vessels by the sickle-shaped cells. These account for approximately 230,000 ED visits and over $1.5 billion in health expenditures annually. Related content

It’s such an important topic and so little attention is given to it! Besides being weighted every time I go to hospital (appointments, examinations, admission), neither doctors or healthcare personnel ever mention any nutrition guidelines specific to SCD. Only that good hydration and a balanced diet are very important. I have gathered some information available on the Web but what is your experience? What is beneficial and what should you avoid to stay clear from a crisis? Foods. Good nutrition, while essential for anyone, is critical for patients with sickle cell disease. Some dietary recommendations are included here. Related content

The most common complication of sickle cell disease (SCD) is a vaso-occlusive crisis. The sudden nature of these crises, marked by intermittent, unexpected episodes of severe pain, leads most SCD patients to turn to the emergency department (ED) for care. In a 1997 study, for example, researchers found that 85.7% of hospital admissions for SCD came through the ED. And not much has changed since then. A more recent study out of California found that out of a cohort of 4,636 patients with SCD, 88% had one or more treat-and-release visits to an ED during 2005 and 2014. And in 2005 alone, 35% of those patients had one to three treat-and-release visits, 9% had four to 10 visits, and 3% had >11 visits. Related content

For most of us, the summer represents fun: playing outdoors, cooling off in a pool and enjoying a family vacation. But for many children, teens and adults living with sickle cell disease, these activities can trigger an extremely painful “crisis” episode – the most common manifestation of this genetic, chronic, and deadly disease. Crisis has been described as a severely debilitating pain: sharp, intense, stabbing, throbbing, and more uncomfortable than post-surgical pain or even childbirth. Worse, crisis can occur at any moment without warning. Enjoying a summer day at the pool can end up in the hospital if you’re not aware of the crisis triggers. Crisis is the leading cause of hospital admissions among people with sickle cell disease. And, this recurring condition hospitalizes sufferers for 4-5 days on average, but may last a week or longer. Each year, there are about 100,000 crisis hospitalizations.   Related content

Sickle cell disease affects ~70,000 to 100,000 people in the United States, and ~10 million worldwide. The disease was first described by James B. Herrick [Herrick 1910]. The molecular basis was partly elucidated by Linus Pauling and Ibert C. Wells [Pauling 1949], and the exact causal amino acid substitution (E6V) was later identified by V.M. Ingram [Ingram 1956]. This missense variant causes hemoglobin to form long polymers which deform red blood cells into a “sickle” shape. Related content

Sickle cell disease (SCD) is a perfect candidate for gene editing. It is perhaps the best understood single-gene condition, due to a substitution of a single DNA base in the gene that encodes the beta subunit of hemoglobin, the protein that carries oxygen in the blood. Red blood cells, which bend into the sickle shape in the disease, obstructing circulation and causing excruciating pain, descend from hematopoietic (“blood-forming”) stem cells (HSCs) in the bone marrow. So theoretically, a patient’s stem cells can be removed, the mutation replaced in them, and then the fixed cells infused back into the patient, circumventing an immune response. Related content

Sleep is a complex process and impairments can occur at different stages of the process. Sleep impairments may include decreased total sleep time (sleep duration), an increase in the amount of time it takes to fall asleep (sleep onset latency), or an increase in the amount of wakefulness after sleep onset (sleep fragmentation) experienced by an individual (Mezick, 2013). Studies broadly suggest that sleep impairments can be directly linked to increased pain (Finan, 2013) and it is this relationship that our group chose to explore further in adults with sickle cell disease (SCD) (Moscou-Jackson, 2015). Related content

The doctors and nurses that I trusted so much and that I felt were like a family, apparently did not feel the same way about me as I thought. Their frustrations with their inability to understand and address the reasons why my disease is so progressed led them to begin to question me! For some reasons as many of you know, my hospitalizations last for months on time but they began to push for my discharge days into my admittance into the hospital. Why? Because they wanted to get rid of me as soon as they could regardless of whether I was still in pain. Related content