Sickle cell disease (SCD) affects 12 000–15 000 individuals in the UK. Whilst homozygous SCD (sickle cell anaemia — HbSS) is the most common and severe genotype, and is where most of the evidence exists, this guidance should be used for all genotypes of SCD. Acute chest syndrome (ACS) is defined as an acute illness characterized by fever and/or respiratory symptoms, accompanied by a new pulmonary infiltrate on chest X-ray (Charache et al, 1979; Ballas et al, 2010). This definition encompasses cases both where an infective organism is isolated and where no infective cause is identified. It is unique to SCD but in some cases ACS may appear to be similar to bacterial pneumonia in a patient without SCD. ACS may have a severe clinical course and can progress rapidly from mild hypoxia to respiratory failure and death. The presence of hypoxia is not included in the definition, but in clinical practice, hypoxia is a useful predictor of severity and outcome (Vichinsky et al, 1997, 2000). Historically, ACS is one of the most common causes of death in patients with SCD (Platt et al, 1994; Lucas et al, 2008), although mortality is improving with improved medical management (Fitzhugh et al, 2010). ACS can also be associated with significant morbidity, including long-term parenchymal lung damage, pulmonary vascular abnormality and neurological sequelae. Patients may present to hospital acutely unwell with ACS or ACS may develop during a hospital admission following a painful crisis or post-operatively. Abstract source Full text

Shamonica Wiggins is a 30-year-old sickle cell warrior and a native of Dallas, TX. Although she has sickle cell SS, which is commonly referred to as the most critical form of the illness, she rarely allows it to get in the way of her chasing her dreams. This is evident in the work she did as creator and former CEO of the non-profit organization, #BoldLipsForSickleCell. Shamonica was awarded the 2018 National Sickle Cell Advocate of the Year by Sickle Cell 101. Ms. Wiggins is a trailblazer in the sickle cell community and strives to be a voice of one, speaking for many. When she's not busy fulfilling her passion of spreading sickle cell awareness you can find her on the sidelines of the soccer field cheering on her 8-year-old daughter Roree. Shamonica prides herself as a mother and her daughter is a big reason why she refuses to give up. She hopes that her advocacy work leads to a big breakthrough for the sickle cell community and she's not going to stop being a voice for her people until she see the changes that sickle cell warriors so desperately need.

This guidance represents the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, healthcare professionals are expected to take this guidance fully into account. However, the guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer. Commissioners and/or providers have a responsibility to implement the guidance, in their local context, in light of their duties to have due regard to the need to eliminate unlawful discrimination, advance equality of opportunity, and foster good relations. Nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties. Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible. Abstract source

With advances in medical care, the majority of children with sickle cell disease are surviving to adulthood. Patients, families, and providers now face the need for this growing population to move from pediatric- to adult-focused care. In order to facilitate a successful transfer to adult health care, and prepare young adults for greater independence, it is recommended that all pediatric patients with sickle cell disease receive transition preparation. ASSOCIATION POSITION: As the professional voice of pediatric hematology/oncology healthcare practice, the Association of Pediatric Hematology/Oncology Nurses (APHON) and the American Society of Pediatric Hematology Oncology (ASPHO) recommends that the discussion of transition begin early and is presented as part of the natural process of becoming an adult; that patients, providers, and families are all involved in creating a transition plan and assessing transition preparedness annually; and that transfer of care involve direct communication between the pediatric team and the accepting adult provider. Abstract source Full text

Nancy is the grandmother of a child with Sickle Cell Disease. She has been active in the sickle cell community since the birth of her grandson, participating in fundraising and attending conferences. She has attended Sickle Cell in Focus at NIH and testified at the FDA as an advocate for those with sickle cell disease. As a career educator and a retired high school principal, Nancy has special interests in helping parents and educators make appropriate decisions for students with sickle cell disease.

Trained at St John’s University, College of Pharmacy and Health Sciences in New York, Marie Ojiambo is a Formulation Scientist by profession specializing in pre-clinical Pharmaceutical Research. She is also a Sickle Cell warrior and a global advocate for Sickle Cell Disease Awareness. Diagnosed at the age of 1, she uses her knowledge and personal experiences to raise awareness about the disease, mentor young warriors and consult with Pharma and other CBO’s on matters Sickle Cell Disease. Currently consulting with Pfizer Inc, the Sickle Cell Diseases Associations of America and heading the Consortium’s Clinical Trials Initiative work group, Marie uses her experiences of living with the disease to inform research, advocacy and various other stakeholders within the Sickle cell disease space on strategies to advance the healthcare and treatment options available to sickle cell warriors. Birthed in the year 2013, Marie founded the Sickle Strong Initiative, a Kenyan based NGO whose mandate is to raise awareness around sickle cell disease and advocate for better health care opportunities for patients suffering the Disease in Kenya. Through SSI, she has been able to host medical training and awareness campaigns for patients suffering the disease in Kenya. Marie is also the proprietor of the annual sickle cell public awareness forum, Ongea (which means to ‘speak out!’ in Swahili). Ongea is an annual public forum that provides a platform for sickle cell warriors to speak out about their condition, share and interact with each other. It also provides an opportunity for patients to engage with and challenge the Government, Pharma, Insurance companies, the public and other stakeholders on what is being done to improve the health care and environment that patients are exposed to. An estimated 650 plus warriors and their families have benefited from Ongea. Marie also holds beauty pageant titles of Miss Kenya 2014/15, The People’s princess Miss Africa USA 2014/15 and is currently 1st runners up Face of Kenya USA 2016/17, platforms that she has used to actively and vocally raise awareness around the disease.

Shirley Miller has over 20 years of experience in program development, comprehensive care management, clinical trials consortium and care coordination among physician specialties for children and adults living with sickle cell disease.  Shirley knows the importance of patient and provider education and is a skilled trainer, facilitator and public speaker.  Ms. Miller was named National Patient Advocate by the Sickle Cell Disease Association of America in 2016 and presented the keynote address at the national conference.  Ms. Miller was the 2016 spokesperson for the FDA initiative on increasing diversity in clinical trials and her advocacy was featured on a front- page article in the June 10, 2017 issue of “Heme Onc Today”. She has served in advisory positions with various pharmaceutical companies to help design clinical trials that enhance the patient experience and currently serves on the FDA Oncologic Drug Advisory Committee (ODAC) to review and approve new drugs being considered for use in sickle cell disease. Ms. Miller is a published author of the book entitled “The Stranger Within Me”, a story of her journey living with sickle cell disease.  She helped to facilitate meetings in which national patient stakeholders developed a peer mentoring protocol to facilitate transition of young adults with sickle cell disease to adult care and currently serves as a patient advocate and mentor at Carolinas HealthCare System Comprehensive Sickle Cell Program.  Shirley is a past board member of the Sickle Cell Disease Association of America and, also currently serves as the coordinator for the Sickle Cell Adult Provider Network (SCAPN).  She holds a Bachelor of Business Administration and a Masters in Psychology.