What Causes Sickle Cell Disease
Sickle cell is an inherited disease caused by a defect in a gene.
- A person will be born with sickle cell disease only if two genes are inheritedone from the mother and one from the father.
- A person who inherits just one gene is healthy and said to be a “carrier” of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier.
For parents who are each carriers of a sickle cell gene, there is a 1 in 4, or a 25 % chance of having a child with sickle cell disease.
What Is The Follow
Considering the many body systems involved and the likelihood that sickle cell crisis will occur time and time again, strong consideration should be given to follow-up with a hematologist .
Most uncomplicated cases of sickle cell crisis can be treated in community emergency departments. People with this condition can be safely sent home when their pain is under control and their dehydration is eliminated. A short observational period in the emergency department helps to prevent acute relapse and admission for pain and rehydration.
Outpatient treatment centers on the control of infection, reduction of pain, and prevention of dehydration. The use of narcotics is often necessary and should not be limited for fear of turning someone with sickle cell disease into a drug addict.
When To Call The Doctor For Sickle Cell Disease
If certain conditions develop in a person with sickle cell disease, the person must contact a physician. If the physician is not quickly available or cannot see the person right away, the person with sickle cell disease may choose to go to a hospital’s emergency department. Contact the physician in the following cases:
- Many people with sickle-cell disease have pain with enough frequency that they need to take pain medications at home. If the pain is unrelieved by the medication, or the pain is significantly different from previous episodes, contact the health care provider.
- If experiencing nausea, vomiting, and diarrhea losing a lot of fluid and having inability to drink and keep it down, the person with sickle cell disease is in danger of becoming dehydrated. This is a serious concern with sickle cell disease. The physician or the hospital may provide IV fluids to replace the lost fluids.
- It is important to control infection. If it appears that a person with sickle cell disease is getting an infection, even if using antibiotics to prevent infection, contact the physician immediately.
A sickle cell crisis can often be managed efficiently and quickly in a hospital’s emergency department with fluids and pain medicines. A person with sickle cell disease should not delay going to the hospital. Delay can only make the condition worse and might require hospitalization for treatment.
Go to a hospital’s emergency department if these conditions develop:
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Enhancing Healthcare Team Outcomes
Patients with sickle cell crises are best managed by an interprofessional team that also includes ICU nurses. The key is rapid hydration and pain control. In addition, oxygenation should be monitored. It is important to find and treat the trigger of the crisis to prevent a recurrent crisis.
Despite optimal treatment, the quality of life of most patients with sickle cell is poor, marked by repeated admissions. The pharmacist should ensure that the patient is compliant with hydroxyurea because it has been shown to reduce the adverse effects of the disease.
What You Need To Know:
Sickle cell disease causes your RBCs to be sickle shaped. The sickle shape is caused by abnormal hemoglobin attached to the RBC. Hemoglobin carries oxygen to all tissues in your body. Sickle-shaped RBCs can get stuck to the walls of blood vessels. This can stop or slow blood flow, and prevent oxygen from getting to tissues. When this happens, it is called a sickle cell crisis. SCD may also cause low red blood cell levels .
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Data Management And Analyses
Data were entered into Excel Spreadsheets for transport into SAS for Windows. The Windows applications of SAS have the specialized statistical procedures necessary for analyzing multilevel data sets with repeated measures. All data were double entered and compared to eliminate any errors in data entry.
How Is Sickle Cell Disease Treated
Your doctor will consider your age, overall health and other factors when determining the best treatment for you.
Early diagnosis and prevention of complications is critical in sickle cell disease treatment. Treatment aims to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:
- Pain medications. This is for sickle cell crises.
- Drinking plenty of water daily . This is to prevent and treat pain crises. In some situations, intravenous fluids may be required.
- Blood transfusions. These may help treat anemia and prevent stroke. They are also used to dilute the sickled hemoglobin with normal hemoglobin to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
- Vaccinations and antibiotics. These are used to prevent infections.
- Folic acid. Folic acid will help prevent severe anemia.
- Hydroxyurea. This medication helps reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for blood transfusions. The long-term effects of the medication are unknown.
- Regular eye exams. These are done to screen for retinopathy.
- Bone marrow transplant. Bone marrow transplants can cure some people with sickle cell disease. The decision to have this procedure is based on the severity of the disease and ability to find a suitable bone marrow donor. These decisions need to be discussed with your doctor and are only done at specialized medical centers.
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Sickle Cell Crisis: How To Manage Pain Prevent Crisis
A sickle cell crisis is those complications, mostly pain that comes with the sickle cell anaemia. The pain can start suddenly and last from hours to several days. The pain might be felt anywhere from the back, chest, knees, legs, arms or stomach.
Sickle cell disease or Sickle cell anaemia, is a common medical condition of the blood that arises as a result of a hereditary disorder, where by the red blood cells are destroyed whenever the body is subjected to stress or illness.
This condition are seen in those individuals who have a haemoglobin genotype of SS. These individuals inherit an S gene each from the mother and the father who me would be either AS or SS.
Sickle cell diseases comes with a lot of complications, known as Crisis. You can read more about sickle cell disease here.Today, we would be talking about how to manage the complications that comes with sickle cell crisis.
Daily Diary Of Pain Health
The daily diary was a simple form that adolescents completed each day. It was modeled after the diary used in our prior study of adults . Adolescents were trained in how to complete the diary by an investigator in person who helped them to complete sample diaries. Each adolescent was given a folder with personalized training materials to refer to, including definitions for common SCD complications. Adolescents were given daily prompts to complete the diaries by a programmable wristwatch and were provided with stamped addressed envelopes in which to return diaries on a weekly basis. Weekly telephone contact was maintained to reinforce diary completion and to answer any questions.
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Sickle Cell Disease Pain Other Pain And Health
The first section of the diary asked about SCD pain and health-care use. These questions were modified from the Structured Pain Interview and Daily Self-Monitoring Record developed by Gil . Participants were asked if they had SCD pain today that was located anywhere in the body and that had no known cause other than SCD. They then rated their average pain level for the day on a 100 mm visual analogue scale . A pain day was defined for the analyses as any day on which the child reported yes to the question Are you having an episode of sickle cell pain today? Adolescents were also asked to indicate pain duration, whether they called or visited their doctor, went to the emergency room , were admitted to the hospital, or took prescription medication. Reliability and validity of the items have been reported in studies of children and adolescents with SCD . The items were found to have satisfactory test-retest reliability and adequate interrater reliability. Positive correlations were found between health-care use data obtained from this self-report format and documented health-care contacts recorded in the medical record.
Other Therapy For Sickle Cell Disease
Chronic therapy: New developments in regularly scheduled transfusion therapy have shown promise in decreasing the following:
- Symptoms of acute chest syndrome
- Incidence of stroke
- Severity of pain crises
- Bone marrow transplantation holds promise for a very small percentage of people with sickle cell disease. Discuss this with the physician.
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Lagged Associations Between Stress Mood And Scd Pain Ratings
In order to investigate whether stress and mood temporally preceded pain, we conducted exploratory analyses investigating the association between stress and pain ratings and between mood and pain ratings on the 2 immediately subsequent days, that is, on pain for the next day and for 2 days later. Most of these analyses were not significant or not in the expected direction. In other words, there was no evidence that higher levels of stress and negative mood predicted pain on the next 2 days, or that positive mood was protective.
What Are The Symptoms Of Sickle Cell Disease
The following is a list of symptoms and complications associated with sickle cell disease. However, each person may experience symptoms differently. Symptoms and complications may include:
The symptoms of sickle cell disease may look like other blood disorders or medical problems. Always consult your health care provider for a diagnosis.
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What Causes Sickle Cell Disease In A Child
Sickle cell is present at birth. It is inherited when a child has 2 sickle cell genes, 1 from each parent.
A child who has only one sickle cell gene is healthy. But he or she is a carrier of the disease. If two carriers have a child, there is a greater chance their child will have sickle cell disease.
Once parents have had a child with sickle cell disease, there is a 1 in 4 chance that another child will be born with sickle cell disease. There is also a 1 in 2 chance that a child will be a carrier, like the parents.
Which Factors Can Precipitate The Sickling Process That Prompts A Crisis In Sickle Cell Disease
The sickling process that prompts a crisis may be precipitated by multiple factors. Local tissue hypoxia, dehydration secondary to a viral illness, or nausea and vomiting, all of which lead to hypertonicity of the plasma, may induce sickling. Any event that can lead to acidosis, such as infection or extreme dehydration, can cause sickling. More benign factors and environmental changes, such as fatigue, exposure to cold, and psychosocial stress, can elicit the sickling process. A specific cause is often not identified.
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Sickle Cell Crisis: Tips On How To Decrease Crisis
What Should I Do If I Have A Pain Crisis
If you have a pain crisis, tell an adult what’s going on. To help ease the pain, you can:
- Try relaxation techniques and distraction.
- Apply warm compresses where it hurts .
- Drink lots of water and other non-caffeinated beverages.
- Take over-the-counter pain medicines, like ibuprofen or acetaminophen.
If these things don’t help, you may need prescription pain medicine. If you are still in pain, call your doctor or go to the ER. You may need to get stronger medicines and fluids in the hospital.
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When To Get Medical Advice
Make sure you know when to get medical advice and where to go, as sickle cell disease can cause a number of serious problems that can appear suddenly.
Problems to look out for include:
- a high temperature over 38C
- severe pain that’s not responding to treatment at home
- severe vomiting or diarrhoea
- weakness on 1 or both sides of the body
- changes in vision or sudden vision loss
Contact your GP or healthcare team straight away if you develop any of these symptoms.
If this is not possible, go to your nearest A& E. If you’re not well enough to travel to hospital yourself, dial 999 for an ambulance.
Make sure that the medical staff looking after you know you have sickle cell disease.
Experimental Setup And Study Protocol
All studies were performed in an ANS laboratory under strictly controlled settings.18 Neuropsychological stress was assessed at baseline using the State-Trait Anxiety Inventory questionnaire.23 The STAI Y-1 and Y-2 evaluate anxiety at this moment, aka state anxiety and how people generally feel, aka trait anxiety, respectively.
Following 5 minutes of baseline recording, the stress induction protocol was presented through psychological software . The protocol consisted of a memory task 24 and a conflict test ,2625 presented in a randomized order, followed by a pain anticipation test . During the N-back task, the subjects were asked to respond when the current letter matched the letter from n steps earlier in the sequence. During the Stroop task, the participants were asked to identify the font color of a word, not the written name of the word. We measured state anxiety between tasks. During the PA task, subjects read the following sentence on their computer screen: You will receive a maximum pain stimulus in one minute. When you cannot tolerate the pain any longer, say STOP and the device will cool down to normal level immediately. However, no pain stimulus was actually applied.
Figure 1.Time sequence of the study protocol. The subjects were randomly assigned to perform the N-back or Stroop test first. STAI: State-Trait Anxiety Inventory Y-1: Sate questionnaire Y-2 Trait questionnaire.
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Living With Sickle Cell Disease
Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications.
It is important to eat a healthy diet with lots of fruits, vegetables, whole grains, and protein, and drink lots of fluids.
Do not take decongestants because they cause constriction of blood vessels and could trigger a crisis.
Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and heavy physical labor.
Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams.
Testing The Study Hypotheses
All multilevel model analyses were generated using PROC MIXED in SAS . Binomial models were applied where appropriate . As recommended by Schwartz and Stone , to ensure that coefficients for within-person predictors remained unbiased, each model included the aggregated person-means corresponding to within-person diary predictors as control variables . However, because results corresponding to these control variables were not central to our hypotheses, only results for within-person associations are reported herein.
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Pain Patterns In The Vaso
A vaso-occlusive crisis most commonly involves the back, legs, knees, arms, chest and abdomen.4,5,12 The pain generally affects two or more sites. Bone pain tends to be bilateral and symmetric. Recurrent crises in an individual patient usually have the same distribution.4,5,13 It has been postulated that the symmetry of bone marrow necrosis can be accounted for by centrally mediated reflexes that direct blood away from the marrow in response to the cooler skin temperature.13 If a patient’s pain has a different than usual pattern, other causes for the pain should be sought.
An acute abdominal pain crisis often resembles an intra-abdominal process such as cholecystitis or appendicitis. Diagnoses that may require surgery and suggest a process other than vaso-occlusive crisis include pain in the absence of a precipitating event, pain that differs from the pain experienced in previous vaso-occlusive crises, and lack of pain relief within 48 hours despite hydration and oxygen therapy.14
Which Children Are At Risk For Sickle Cell Disease
Having a family history of SCD increases a childs risk for the disease. SCD mainly affects people whose families came from Africa, and Hispanics whose families are from the Caribbean. But the gene has also been found in people whose families are from the Middle East, India, Latin America, and Mediterranean countries. It has also been found in American Indians.
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What Is Sickle Cell Disease In Children
Sickle cell disease is a blood disorder that a child is born with. It’s passed down through a parents genes. Children with SCD make an abnormal type of hemoglobin. This is the protein in red blood cells that carries oxygen to all parts of the body. With SCD, the body organs and tissues dont get enough oxygen.
Healthy red blood cells with normal hemoglobin are round and move easily through blood vessels. When a child has SCD, the red blood cells are hard and sticky. They are shaped like the letter C . These damaged red blood cells clump together. They cant move easily through the blood vessels. They get stuck in small blood vessels and block blood flow. This blockage can cause pain. It can also damage major organs.
Sickle cells die sooner than healthy cells. Normally the spleen helps filter infections out of the blood. But sickle cells get stuck in this filter and die. Having fewer healthy red blood cells causes anemia. The sickle cells can also damage the spleen. Without a healthy spleen, children are more at risk for serious infections.
There are several complex types of the sickle cell gene. Some dont cause symptoms or severe problems, but others do. Talk to your childs healthcare provider about the specific form of sickle cell your child has.
Most children with SCD will start showing symptoms during the first year, often around 5 months.
