Management of pain in sickle-cell disease (2022)

  • Journal List
  • J R Soc Med
  • v.95(9); 2002 Sep
  • PMC1279994

Management of pain in sickle-cell disease (1)

Journal of the Royal Society of Medicine

J R Soc Med. 2002 Sep; 95(9): 456–458.

PMCID: PMC1279994

PMID: 12205212

(Video) Principles of pain management and warning signs of sickle cell disease webinar

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The acutely painful episodes that characterize sickle-cell disease were described in 1872 by Africanus Horton1, though the mechanism remained uncertain until, nearly thirty years later, James Herrick observed the sickling deformity of red cells that causes vaso-occlusion and tissue infarction.

Sickle-cell disease (SCD) is the commonest globin gene disorder: across the world, about 300 000 children are born with it each year3. The pain of sickle-cell crisis is excruciating and, in global terms, a major health problem.

TYPES OF PAIN IN SICKLE-CELL DISEASE

Pain caused by sickle-cell disease can be acute, chronic or a mixture of the two. The acute pain of tissue infarction, in skeletal or soft tissue, tends to be sudden, unpredictable in onset and intense. After resolution of sickle-cell crisis, it usually stops. Chronic pain in SCD is not simply a continuation of the pain of vaso-occlusion: it is usually secondary to avascular necrosis of bone at various joints—the hips, shoulders and ankles, in decreasing order of frequency. Also, avascular necrosis commonly develops in the spine, causing chronic back pain and displaying the well-known ‘fish-mouth’ appearance on X-rays. The knees are seldom involved. Individuals with SCD are not, of course, immune to other painful acute and chronic disorders unrelated to the haemoglobinopathy. Abdominal pain may be a manifestation of sickle-cell crisis affecting the abdominal viscera, but it may also reflect a surgical emergency such as perforation. Similarly, chronic joint pain in people with SCD can be caused by rheumatoid arthritis, osteoarthritis or other forms of degenerative joint disease: one memorable individual turned out to have tuberculous arthritis in one hip and avascular necrosis in the other. In a person with a previously recognized cause of chronic pain such as hip necrosis, acute exacerbations can result either from new vaso-occlusive events in the same site or from movement-induced injury to the damaged joint. Previously satisfactory measures for pain relief may then become inadequate. Somewhat easier to recognize and manage is the development of generalized painful crisis in an individual who formerly had chronic pain at one or few anatomical sites. Resolution of a crisis requires a switch away from medications that are suitable only for the relief of acute pain.

TREATMENT OF ACUTE PAIN

The methods of pain relief in SCD depend on whether the pain is acute, chronic or a mixture of the two types and on whether the patient is opioid-naïve or opioid tolerant. The guiding principle is to use a stepwise approach, akin to that used for hypertension. When the patient has not been seen before, he or she can be asked what types and doses of analgesics have in the past been effective; but a pitfall of this approach is that people with drug-seeking behaviour may exaggerate the intensity of their pain or the effective doses of analgesics so as to obtain more medication. Our protocol for treating acute (crisis) pain caused by SCD is summarized in Table 1. Regular analgesia is given for acute pain. The standard dosing interval for morphine injections and rapid release preparations is 4-6 hours, but we find that some individuals become so tolerant to opioids that doses are needed 2-hourly. Every effort is made to prevent such tolerance developing in new patients because there is a limited choice of injectable opioids that can be used in acute painful episodes. By combining analgesics with different mechanisms of action, such as paracetamol or diclofenac, the dose of opioids can be kept to a minimum.

Table 1

Treatment of acute pain

SeverityOpioid naïveOpioid tolerant
Mild/moderate Dihydrocodeine tablets 30 mg/4 h or co-codamol 2 tablets/4 h or co-proxamol 2 tablets/4 h Immediate-release morphine sulphate tablets 10-40 mg/4 h or hydromorphone tablets 1.3-3.9 mg/4 h
Severe Diamorphine Diamorphine
2.5-5 mg/4 h s.c. 10-20 mg/2-4 h s.c.

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(Video) ASH Guidelines on SCD: Pain Management

The non-steroidal anti-inflammatory drugs (NSAIDs) are effective in relieving the inflammatory component of infarctive (vaso-occlusive) bone pains. Diclofenac by mouth, 50 mg three times daily or 75 mg twice daily, is added to the analgesic regimen if the patient has no contraindications such as peptic ulcer, asthma or renal impairment. For those who are vomiting or cannot take the drug orally, diclofenac by suppository (100 mg daily) is an alternative. One complication of SCD is nephropathy—characterized by proteinurea, ranging from microalbuminuria to massive excretion (with nephrotic syndrome)4, 5. The nephropathy can be worsened by NSAIDs, so treatment with these agents should be stopped after a week at the most. Patient-controlled analgesia (PCA) is reported to be as safe and effective as intermittent opioid injections6, and in our centre PCA consists of diamorphine subcutaneous infusions. PCA is used for patients who prefer it to intermittent injections.

If pain is not controlled, the amount of opioid is increased in small increments (e.g. diamorphine 2-3 mg) to avoid the risk of central nervous system depression. When the acute pain begins to resolve, the dose is tailed off gradually rather than stopped abruptly, so as to avoid withdrawal symptoms, which can mimic those of sickle-cell crisis. These strategies apply to intermittent injections or oral administration of opioids, not to PCA. In patients started on diamorphine less than 10 mg/injection, the opioid is stopped when the crisis resolves. If intermittent injection is started with a dose more than 10 mg a switch from parenteral to oral analgesics is made when the dose of diamorphine is less than 10 mg per injection. The equivalent doses of other opioids are shown in Table 2. In deciding the dose to be prescribed, one should be guided by the degree of opioid sensitivity observed clinically in the patient. If the prescribed dose is based solely on the theoretical equivalent amounts, an individual who is very sensitive to opioids runs a risk of overdosage, with respiratory depression.

Table 2

Equivalent doses of opioids

Ratio
Subcutaneous diamorphine 400 mg/24 h Oral immediate release morphine sulphate 130 mg/4 h 1:2
Oral immediate-release morphine sulphate 130 mg 4-hourly Oral slow-release morphine sulphate 400 mg twice daily 1:1
Oral immediate-release morphine sulphate 130 mg 4-hourly Subcutaneous diamorphine 400 mg/24 h 2:1
Oral dihydrocodeine 30 mg 4-hourly Oral immediate-release morphine sulphate 3 mg 4-hourly 12:1

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(Video) Understanding Sickle Cell Pain

Diamorphine has replaced pethidine as the analgesic of first choice for acute pain in SCD. Of about 800 adults with the haemoglobinopathy who receive treatment in our centre, only 3 (who reacted seriously to diamorphine) still receive pethidine. There are several reasons for preferring diamorphine. The pethidine metabolite is excitatory to the nervous system, and causes seizures. Diamorphine has a longer duration of action, and mass for mass is a more potent analgesic. Whereas diamorphine is soluble enough to be given subcutaneously, pethidine has to be injected into muscle. Repeated intramuscular injections of pethidine cause muscle fibrosis and contractures; absorption from the injection site becomes less and larger doses are needed—causing further muscle fibrosis and, far more serious, increasing the likelihood of drug dependence or addiction.

When opioids are used as part of pain management in SCD, their side-effects must be prevented or treated. Constipation is treated with agents such as sodium docusate 100 mg three times daily, lactulose 10-15 mL twice daily or senna 2-4 tablets daily. Nausea/vomiting can be relieved with metoclopramide 10 mg or cyclizine 50 mg 8-hourly, orally or by injection. Many of our patients get pruritus when given morphine, and this commonly responds to oral hydroxyzine 25 mg twice daily. Pruritus does not imply allergy to morphine and does not warrant stopping the drug or switching to pethidine. We find that pruritus is more frequent in black patients than in other ethnic groups. The most serious side-effect of opioids is respiratory depression, which sometimes requires treatment with an opioid antagonist such as naloxone.

TREATMENT OF CHRONIC PAIN

In our centre, the approach to chronic pain caused by SCD is multidisciplinary, including the use of analgesic drugs, nerve block, physiotherapy, orthopaedic intervention or surgery, and cognitive behaviour therapy. Mild chronic pain is relieved by dihydrocodeine or co-proxamol (dextropropoxyphene/paracetamol). Any pain not controlled by two tablets 4-hourly is considered moderate/severe, and we then step up to morphine. Slow-release oral morphine, taken 12-hourly, is used for long-term analgesia, with smaller amounts of rapid-release oral morphine for breakthrough pain. The alternatives are slow-release and rapid-release hydromorphone. For the reasons given earlier, we discourage long-term use of NSAIDs for chronic pain in SCD. A switch from morphine to hydromorphone, or viceversa, is made when tolerance develops to one or other drug; tolerance (the need for increasing doses to maintain the same effects) is a feature of long-term opioid therapy.

In a patient whose chronic pain is severe enough to warrant opioid therapy, supplementary approaches may be applicable. For example, the pain of avascular necrosis of the hip, shoulder or intervertebral joints can be lessened by nerve block, with benefit lasting up to 12 weeks for each injection (Box 1). Physiotherapy can lessen joint pain, prevent muscle contracture and lessen joint stiffness and physical disability. Cognitive behaviour therapy helps the individual to develop strategies for coping with pain and other psychological disturbances caused by SCD7. Orthopaedic devices for back support, or for raising the foot to make up for differences in length between the legs, help reduce chronic pain in the hips or back. In some cases of avascular necrosis, orthopaedic surgery is the only treatment that effectively relieves pain, and should be performed as early as possible. For other orthopaedic procedures such as total hip replacement, the duration of benefit is limited8 and there is a strong argument for deferring operation until the pain becomes intolerable.

Box 1 Nerve block for chronic hip pain

A man aged 23 with sickle-cell disease complicated by avascular necrosis of the left femoral head developed methicillin-resistant Staphylococcus aureus (MRSA) infection of the left hip joint after left femoral osteotomy. Wound healing was delayed and he continued to have chronic hip pain uncontrolled with opioids. Further surgery was judged inadvisable because of the likelihood of reactivating MRSA infection. Left hip nerve block, performed by anaesthetists, yielded profound benefit and allowed effective pain relief with hydromorphone 16 mg twice daily. A repeat nerve block was required after 4 months to maintain analgesia.

DEPENDENCE AND ADDICTION

Unfortunately, opioid therapy can lead to dependence or addiction. Dependence—the occurrence of an abstinence syndrome (withdrawal) after abrupt reduction in the dose of a drug or after administration of an antagonist—can develop after just a few days of repeated administration. More serious is addiction—a psychological and behavioural syndrome in which there is craving for a drug, compulsive use, and strong tendency to relapse if the drug is withdrawn. Addiction affects only a small percentage of SCD patients9, 10. Out of about 800 adults with SCD registered in our centre, only 4 have been addicted to opioids. However, such patients do take a disproportionate amount of time and resources.

Healthcare personnel have a duty to ensure effective relief of pain—which is broadly defined as an unpleasant sensation and emotional experience that occurs in association with actual or potential damage to part of the body. In the absence of objective measures, assessment must be based on what the patient says. However, they must also be alert to the possibility that prescriptions of opioids or other addictive drugs such as temazepam exceed the medical needs of an individual. Pointers to dependence are a patient's insistence on determining the dose and timing of an addictive drug without caring as much about antibiotic or other therapy, incessant objections to dose reduction considered medically appropriate, and frequent demands for dose increases especially after working hours. An addicted person may acquire drugs illegally, or unlawfully obtain materials used for drug injection. Also, an occasional SCD patient who is neither dependent nor addicted will dispose of prescribed drugs for personal gain. One strategy to obtain excess supplies is to register with more than one general practitioner or hospital; another is to use different personal details such as name, address or date of birth.

If dependence or addiction is in the differential diagnosis, referral to a drug dependency unit is advisable. Healthcare personnel should prescribe only the amounts of drug they judge necessary for control of pain.

References

1. Africanus Horton JB. The Diseases of Tropical Climates and their Treatment. London: Churchill, 1874

2. Herrick JB. Peculiar elongated and sickled red blood corpuscles in a case of severe anemia. Arch Intern Med 1910: 517-21

3. Serjeant GR. Sickle cell disease. Lancet 1997;350: 725-30 [PubMed] [Google Scholar]

4. Ataga KI, Orringer EU. Renal abnormalities in sickle cell disease. Am J Hematol 2000;63: 205-11 [PubMed] [Google Scholar]

5. De Jong PE, De Jong-Van Den Berg LTW, Sewrajsingh GS, et al. The influence of indomethacin in sickle cell anaemia. Clin Sci 1980;59: 245-50 [PubMed] [Google Scholar]

6. Gonzalez ER, Bahal N, Hansen LA, et al. Intermittent injection vs patient controlled analgesia for sickle cell crisis pain. Comparison in patients in the emergency department. Arch Intern Med 1991;51: 1373-6 [PubMed] [Google Scholar]

7. Thomas VJ. Cognitive behavioural therapy in pain management for sickle cell disease. Int J Palliat Nurs 2000;6: 434-42 [PubMed] [Google Scholar]

8. Moran MC. Osteonecrosis of the hip in sickle cell hemoglobinopathy. Am J Orthoped 1995;24: 18-24 [PubMed] [Google Scholar]

9. Brookoff D, Polomano R. Treating sickle cell pain like cancer pain. Ann Intern Med 1992;116: 364-8 [PubMed] [Google Scholar]

10. Porter J, Jick H. Addiction is rare in patients treated with narcotics. N Engl J Med 1980;302: 123 [PubMed] [Google Scholar]

(Video) "Managing Pain in Sickle Cell Disease: Challenges and Opportunities" by Drs. A Brandow & P Kavanagh

FAQs

How do you manage sickle cell pain? ›

How to Manage a Pain Crisis
  1. Drink water or other fluids when your symptoms start. Staying hydrated can help you head off the worst of an attack.
  2. Use a heating pad or take a warm bath.
  3. Try a massage, acupuncture, or relaxation techniques.
  4. Do something to take your mind off your pain.
21 Aug 2022

Why is pain management so important for sickle cell crisis? ›

Pain causes significant morbidity for those living with sickle cell disease (SCD) and has a profoundly negative impact on patients' health-related quality of life (HRQOL).

What is the best pain medication for sickle cell? ›

Diclofenac and ibuprofen are commonly used NSAIDs in sickle cell anemia patients. If the pain persists, an opioid can be added. Moderate-to-severe pain usually is treated with opioids. For moderate pain, a weaker opioid such as codeine may be sufficient.

What type of pain is sickle cell pain? ›

Sickle cell pain includes 3 types: acute recurrent painful crises, chronic pain syndromes, and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the emergency department.

How do you deal with a pain crisis? ›

What Should I Do If I Have a Pain Crisis?
  1. Try relaxation techniques and distraction.
  2. Apply warm compresses where it hurts (never use ice or cold packs).
  3. Drink lots of water and other non-caffeinated beverages.
  4. Take over-the-counter pain medicines, like ibuprofen or acetaminophen.

Why do sickle cell anemia patients experience pain? ›

Pain. Pain is the most common complication of SCD, and the top reason that people with SCD go to the emergency department or hospital. Sickled cells traveling through small blood vessels can get stuck and block blood flow throughout the body, causing pain.

How long does a sickle cell pain crisis last? ›

Episodes of pain known as sickle cell crises are one of the most common and distressing symptoms of sickle cell disease. They happen when blood vessels to part of the body become blocked. The pain can be severe and lasts for up to 7 days on average.

What is a pain crisis? ›

Pain crises are recurrent episodes of pain that range in severity from mild to severe, usually occur very abruptly and are often localized around joints. Pain crises are caused by vaso-occlusions in the vascular bed of the bone marrow, leading to necrosis, edema and increased pressure.

Which drug is not recommended for treating pain during sickle cell disease episodes? ›

Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD.

What are 3 treatments for sickle cell anemia? ›

Treatment
  • Hydroxyurea (Droxia, Hydrea, Siklos). Daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. ...
  • L-glutamine oral powder (Endari). ...
  • Crizanlizumab (Adakveo). ...
  • Voxelotor (Oxbryta). ...
  • Pain-relieving medications.
9 Mar 2022

How painful is sickle cell pain? ›

Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. The pain varies in intensity and can last for a few hours to a few days.

Is Tramadol good for sickle cell crisis? ›

In conclusion, tramadol infusion i.v. (0.25 mg x kg(-1) x h(-1)) combined with nonopioids was effective to relieve moderate to severe pain due to vaso-occlusive crisis and can be recommended before using morphine in a pediatric sickle cell crisis.

Does sickle cell cause chronic pain? ›

Chronic pain in sickle cell disease (SCD) refers to pain present on most days lasting over six months. It can start during childhood and the prevalence increases with age. By adulthood, over 55% of patients experience pain on over 50% of days; 29% reporting pain on 95% of days.

Can sickle cell patient take paracetamol? ›

Based on the pain pathophysiology, physicians frequently choose non-steroidal anti-inflammatory drugs (NSAIDs), paracetamol and opiates to treat sickle cell painful crisis. These are the three main classes of analgesics that prove to be most effective in painful crisis.

Can sickle cell patients take ibuprofen? ›

Diclofenac and ibuprofen are frequently used to treat mild chronic pain in sickle cell disease.

What is the treatment for a patient in acute sickle cell crisis? ›

For sickle cell crisis, when the severity of the episode is assessable, self-treatment at home with bed rest, oral analgesia, and hydration is possible. Individuals with SCD often present to the emergency department (ED) after self-treatment fails.

How do you help someone with sickle cell crisis? ›

Sickle cell patients need to drink a lot of water. If your friend is able to drink, give them water. If they have access to pain relief, they may need help administering it, depending on the location of the pain. Also, some patients find that heat pads, ice packs, or massaging the affected area minimizes the pain.

What should a sickle cell patient avoid? ›

avoid very strenuous exercise – people with sickle cell disease should be active, but intense activities that cause you to become seriously out of breath are best avoided. avoid alcohol and smoking – alcohol can cause you to become dehydrated and smoking can trigger a serious lung condition called acute chest syndrome.

What are the four types of sickle cell crisis? ›

Four major types of crises are recognised in sickle cell anaemia: aplastic, acute sequestration, hyper-haemolytic, and vaso-occlusive crises.

What are the 3 main causes of anemia? ›

Hemoglobin is an iron-rich protein that gives the red color to blood. It carries oxygen from the lungs to the rest of the body. Anemia has three main causes: blood loss, lack of red blood cell production, and high rates of red blood cell destruction.

What are the 3 types of sickle cell? ›

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle Hemoglobin-C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

What are the common triggers for sickle cell crisis? ›

Common sickle cell crisis triggers include:
  • sudden change in temperature, which can make the blood vessels narrow.
  • very strenuous or excessive exercise, due to shortage of oxygen.
  • dehydration, due to low blood volume.
  • infections.
  • stress.
  • high altitudes, due to low oxygen concentrations in the air.
  • alcohol.
  • smoking.
1 May 2018

Why does a sickle cell crisis last 5 7 days? ›

That's when you have a sickle cell crisis. The stuck cells slow or even totally block blood flow, so some parts of your body don't get the oxygen they need. That can cause intense pain that lasts anywhere from a few hours to a few weeks.

How many years do sickle cell patients live? ›

With a national median life expectancy of 42–47 years, people with sickle cell disease (SCD) face many challenges, including severe pain episodes, stroke, and organ damage.

What happens if sickle cell crisis is not treated? ›

If it's not treated quickly, damage can cause problems with getting erections later in life. Stroke: Sickle-shaped cells can block small blood vessels in the brain, causing a stroke. Signs can include headache, seizure , weakness of the arms and legs, speech problems, a facial droop, or loss of consciousness.

What blood type carries sickle cell? ›

HbAS. People who have sickle cell trait (SCT) inherit a hemoglobin “S” gene from one parent and a normal gene (one that codes for hemoglobin “A”) from the other parent.

What antibiotics are used for sickle cell anemia? ›

Preventing infections if you have sickle cell disease

Most people need to take a daily dose of antibiotics, usually penicillin, often for the rest of their life.

Does heat help sickle cell? ›

Extreme temperature changes can trigger a sickle cell crisis. Changing from a hot to a cold environment can constrict our blood vessels, which slows the flow of blood and causes sickled red blood cells to stick together.

Why should sickle cell patients not take iron? ›

Too much iron is extremely dangerous and causes damage to blood vessels, red blood cells, liver, hormone producing glands and heart. It is very difficult to know what damage due to iron overload in sickle cell patients because the sickle cell disease itself causes organ damage to the same organs affected by iron.

What is the difference between sickle cell disease and sickle cell anemia? ›

Sickle cell disease (SCD) is a serious group of conditions which are inherited (genetic). It affects the red blood cells in the blood. Sickle cell anaemia is the name of a specific form of SCD in which there are two sickle cell genes (see below).

What is the name of sickle cell test? ›

The best way to check for sickle cell trait or sickle cell disease is to look at the blood using a method called high-performance liquid chromatography (HPLC). This test identifies which type of hemoglobin is present.

How does sickle cell affect the body? ›

These cells do not bend or move easily and can block blood flow to the rest of your body. The blocked blood flow through the body can lead to serious problems, including stroke, eye problems, infections, and episodes of pain called pain crises. Sickle cell disease is a lifelong illness.

What famous person has sickle cell anemia? ›

Musician and actress Jordin Sparks started advocating for those with sickle cell disease when she learned about her stepsister's diagnosis. "We need to end the stigma that can come with sickle cell disease. No one should feel alienated because they are in pain."

Can sickle cell patients take aspirin? ›

Both non-aspirin NSAIDs and aspirin should be used cautiously in sickle cell patients, especially those with renal, GI, and cardiovascular risk factors. Aspirin may have a better cardiovascular and renal toxicity profile.

What is VOC medical? ›

What is a vaso-occlusive crisis? A vaso-occlusive crisis, or VOC, occurs when sickled red blood cells block blood flow to the point that tissues become deprived of oxygen. This in turn sets in motion an inflammatory response as the body tries to rectify the problem.

What are the side effects of oxycodone? ›

Common side effects
  • constipation.
  • feeling or being sick (nausea or vomiting)
  • stomach discomfort.
  • feeling sleepy or tired.
  • feeling dizzy and a sensation of spinning (vertigo)
  • confusion.
  • headaches.
  • itchiness or rash.

Can sickle cell be cured permanently? ›

Sickle cell disease can be cured with a donor bone marrow transplant but use of this therapy has the best chance of success in patients who have a closely matched sibling donor, which is only a minority of patients.

Can bone marrow transplant cure sickle cell? ›

BMT, also known as bone marrow transplant or stem cell transplant, is the only known cure for SCD. It replaces the unhealthy blood-forming cells (stem cells) with healthy ones.

Why are platelets high in sickle cell anemia? ›

The platelet hyperactivity of the sickle-cell steady state therefore reflects an increased circulating population of young, metabolically active platelets resulting from previous autosplenectomy.

How long does a sickle cell pain crisis last? ›

Episodes of pain known as sickle cell crises are one of the most common and distressing symptoms of sickle cell disease. They happen when blood vessels to part of the body become blocked. The pain can be severe and lasts for up to 7 days on average.

How do you help someone with sickle cell crisis? ›

Sickle cell patients need to drink a lot of water. If your friend is able to drink, give them water. If they have access to pain relief, they may need help administering it, depending on the location of the pain. Also, some patients find that heat pads, ice packs, or massaging the affected area minimizes the pain.

Can sickle cell patients take ibuprofen? ›

Diclofenac and ibuprofen are frequently used to treat mild chronic pain in sickle cell disease.

What are 3 treatments for sickle cell anemia? ›

Treatment
  • Hydroxyurea (Droxia, Hydrea, Siklos). Daily hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations. ...
  • L-glutamine oral powder (Endari). ...
  • Crizanlizumab (Adakveo). ...
  • Voxelotor (Oxbryta). ...
  • Pain-relieving medications.
9 Mar 2022

What are the 5 sickle cell crisis? ›

Conclusion: There were six types of crises seen in the sickle cell disease subjects namely vaso-occlusive, sequestration, infarctive, aplastic, haemolytic and bone pain crises. Vaso-occlusive crisis was the most common and haemolytic crises the least.

What are the four crisis of sickle cell? ›

Four major types of crises are recognised in sickle cell anaemia: aplastic, acute sequestration, hyper-haemolytic, and vaso-occlusive crises.

What are the 3 types of sickle cell? ›

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle Hemoglobin-C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

What can trigger a sickle cell crisis? ›

Common sickle cell crisis triggers include:
  • sudden change in temperature, which can make the blood vessels narrow.
  • very strenuous or excessive exercise, due to shortage of oxygen.
  • dehydration, due to low blood volume.
  • infections.
  • stress.
  • high altitudes, due to low oxygen concentrations in the air.
  • alcohol.
  • smoking.
1 May 2018

What is the most common type of sickle cell crisis? ›

The most common is the vasoocclusive ('painful') crisis. Vasoocclusive crisis has sudden onset, usually lasts 5–6 days, and may be localized in one area of the body or generalized.

Why is hydration important for sickle cell patients? ›

Staying hydrated can prevent you from having vaso-occlusive crises, pain crises, strokes and infections associated with sickle cell disease. Drinking eight to 10 eight-ounce glasses of water a day can help with controlling some of your pain.

What drugs should sickle cell patients avoid? ›

Morphine is considered the drug of choice for the treatment of acute sickle cell pain (Table 2), whereas meperidine (Demerol, Sanofi-Synthelabo) should be avoided because of the increased risk of seizures in patients with renal dysfunction, which can occur in patients with SCD.

How painful is a sickle cell crisis? ›

A sickle cell crisis is pain that can begin suddenly and last several hours to several days. It happens when sickled red blood cells block small blood vessels that carry blood to your bones. You might have pain in your back, knees, legs, arms, chest or stomach. The pain can be throbbing, sharp, dull or stabbing.

Is aspirin good for sickle cell disease? ›

Both non-aspirin NSAIDs and aspirin should be used cautiously in sickle cell patients, especially those with renal, GI, and cardiovascular risk factors. Aspirin may have a better cardiovascular and renal toxicity profile.

What vitamins are good for sickle cell? ›

Supplementing with vitamin C may help correct a deficiency. Antioxidant nutrients protect the body's cells from oxygen-related damage. Many studies show that sickle cell anemia patients tend to have low blood levels of antioxidants, including carotenoids, vitamin A, vitamin E, and vitamin C, despite adequate intake.

What is the difference between sickle cell disease and sickle cell anemia? ›

Sickle cell disease (SCD) is a serious group of conditions which are inherited (genetic). It affects the red blood cells in the blood. Sickle cell anaemia is the name of a specific form of SCD in which there are two sickle cell genes (see below).

What is the name of sickle cell test? ›

The best way to check for sickle cell trait or sickle cell disease is to look at the blood using a method called high-performance liquid chromatography (HPLC). This test identifies which type of hemoglobin is present.

Videos

1. Managing pain in sickle cell crisis with Dr. Ifeyinwa Osunkwo
(MDedge: news and insights for busy physicians)
2. Acute Sickle Cell Pain Crisis - Internal Medicine Residency Series
(THE USMLE CHANNEL.)
3. Music Therapy in the Management of Sickle Cell Disease and other Painful Conditions
(Sound Health Network)
4. Management of Pain in Sickle Cell Patients Session for Junior Doctors
(West London HCC)
5. Sickle Cell NATURAL Healing Daily Regimen | Avoid Crisis, Transfusions or Surgeries! | CiCi Moya
(CiCi Moya)
6. Correct IV Fluids For A Sickle Cell Crisis.
(Sickle Cell Medical Advocacy by Dr Simone)

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