Palliative Care in Sickle Cell disease

Dr. Pankaj Singhai and Dr. Swati Patel Jain, Indore

Arun (name changed)16-year-old male child, a known case of sickle cell anaemia, presented to the hospital with acute painful crisis. Arun was diagnosed at 5 years of age, was not on regular follow up and was taking hydroxyurea irregularly. He presented with fever, pain in hands and legs for 2 days. Arun was managed onIV fluids and IV antibiotics. His pain was managed with the collaborative efforts of the haematology team and the palliative medicine team. Once he was out of his pain crisis, his analgesics were slowly stopped and he was discharged on oral hydroxyurea and supportive drugs.

Sickle cell disease is one of the commonest hemoglobinopathy worldwide. It is an autosomal recessive inherited haemoglobin disorder, in which there is the replacement of valine in place of glutamine on the beta globin chain, causing a structural modification in the shape of RBCs and thus leading to the sickle like shape of Red Blood Cells (RBCs). These sickle red cells are prone for haemolysis and are “sticky”. The sticking together of sickle RBCs, or “sickling” leads to microvascular thrombosis, inflammation, haemolysis and vascular blockage.

Sickle cell disease is highly prevalent in certain parts of India, especially the Central Deccan region, which is popularly known as the “Sickle Belt”. One fifth of the population in Madhya Pradesh consists of tribal population. It is estimated that the prevalence of sickle cell gene within the tribal community, ranges from 10% to 33 %. Sri Aurobindo Institute of Medical Sciences, Indore, caters to a large number of sickle cell disease patients from the nearby districts.

Most often, the patients from these tribal populations are uneducated, poor, and reside in interior areas which make it difficult for them to access healthcare as they do not have the capacity to afford frequent doctor visits or access even simple drugs like hydroxyurea etc. All of these factors lead to delayed diagnosis, poor drug compliance and ultimately to patients landing up with complications of sickle cell disease.

Patients with sickle cell disease live with significant morbidity due to pain which adversely affects their quality of life. This pain can present as either acute, acute on chronic or persist as chronic pain. The incidence and severity of pain usually increases with age as complications of the disease advances. These episodes of pain can occur due to various reasons such as inflammation, tissue hypoxia and reperfusion injury, ischemia due to capillary occlusion and many other neuropathic pain mechanisms like central sensitization.

Pain control in these patients usually requires individualized, multi-modality approaches including pharmacological, Non-pharmacological and Interventional pain treatments. In acute pain, it is recommended to have a rapid and frequent assessment of pain and titrate the analgesics accordingly. Analgesics are advised as per the WHO Analgesic ladder. NSAIDS like Ibuprofen and Ketorolac are preferred first line analgesic agents. However, these drugs should be avoided for long term with fear of end organ toxicities. Corticosteroids may reduce opioid requirement in acute pain, but generally not recommended in sickle cell disease related acute pain as cessation has been associated with rebound pain and other complications.

Adjuvant analgesics like Gabapentinoids, Tricyclic antidepressants and SNRI may be useful in chronic pain with or without other causes eg. Avascular necrosis. Hydroxyurea has proven to be useful in preventing recurring episodes of pain. IV hydration, chronic transfusions and erythrocytopheresis are to be considered based on the opinion of the haematologist.

Opioid medications can be used in acute pain either as intermittent or continuous infusions based on the frequency of pain. The pain controlled analgesia option can be preferred in acute and varying pain conditions. Chronic use of opioids is usually discouraged unless pain is refractory to other modes of treatment. In these patients, opioids are required for long term, however, it should be prescribed in the lowest effective dose with frequent behavioural risk assessments. In patients with opioid resistant pain, subanaesthetic infusion of ketamine in dosage of 0.1mg/kg/hr upto 1 mg/kg/hr can be considered. Regional anaesthesia like epidural or peripheral nerve blocks can be considered based on anatomical location of pain in frequent acute or persistent pain conditions. Orthopedic surgical interventions like decompression coring procedures, osteotomy may be required for pain relief as well as joint preservation in avascular necrosis (AVN) of femoral head. AVN often causes chronic pain which may require total joint replacement for amelioration of symptoms.

Other Non-pharmacological measures like TENS, Yoga, guided relaxation techniques, cognitive behavioural therapies, mindfulness treatments, can be useful in pain and distress related to sickle cell disease. These therapies can be individualised as per the need.

Although pain is an overtly visible symptom in these patients’, the chronicity of this illness affects various domains of overall health and growth of young people. Dropping out from education, delayed growth and bony abnormalities affect the social wellbeing of such patients’. Recurrent, severe infections and ischaemic events may lead to sudden unexpected death.

Palliative Care can help such patients’ to

  • cope with the disease
  • understand the course of the illness andit’s treatment options
  • provide psychosocial support to patients’ and their caregivers

On the occasion of World Sickle Cell Awareness Day (19th June) our message to all is that, “Suffering in these diseases can be reduced by adopting an integrated and a holistic palliative care approach which can be provided along with primary treatment”.

About the Authors

Dr. Pankaj Singhai completed his MD Palliative Medicine training from Tata Memorial Hospital, Mumbai, and currently working at Sri Aurobindo University, Indore. He is also the national faculty of the IAPC and is associated with various training programs.

Dr. Swati Patel is a Pediatric Hematologist and Oncologist, who is currently working as an Assistant Professor in the Department of Medical Oncology at Sri Aurobindo Medical College and Postgraduate Institute, Indore. Dr Swati is particularly interested in the field of sickle cell anemia, and is involved with formulating and overseeing community projects for awareness, screening and management of sickle cell anemia in districts surrounding Indore.

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