Sickle Cell Disease: Symptoms, Treatment and Prevention

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Introduction

Sickle cell disease is a problem that can be encountered by newly-born individuals. This disease has been explored by scholars and has been reported to shorten life expectancy. The current report is aimed at producing an approximate plan of action for a definite patient who has been diagnosed with sickle cell anemia at birth.

General Overview of the Disease: Symptoms and Occurrence

Sickle cell anemia is a disease diagnosed in patients of different age groups. According to Platt et al (1994), sickle cell disease or sickle cell anemia is an autosomal blood disorder that occurs on a genetic level and can recess during the life period of a patient. This disease is characterized by red corpuscles that acquire a sickle shape. This transformation reduces the cells flexibility and can cause numerous complications in case the problem is not approached adequately. Sickling is the result of a genetic mutation in blood cells. Life expectancy is shortened because of genetic transformations; besides, an average life expectancy equals 42 and 48 years in males and females respectively (Platt et al, 1994).

Stress experienced by most individuals with a sickle-cell-anemia diagnosis in the teenage is a frequent phenomenon that should be approached properly. These individuals face specific problems related to sickle cell anemia such as their body-image conflict. In this respect, psychological assistance should be provided to those patients as well as appropriately coordinated therapy. Sickle cell anemia is a recessive disease though it is also characterized by relapses that can cause some complications and damage the body of a patient. Counseling sessions as well as some support and encouragement received from family and friends can help individuals to cope with their psychological stress and uncertainty.

Treatment and Prevention

There are different approaches a young person can treat and/or prevent having a sickle cell crisis. The patient is advised to avoid certain types of medications such as decongestants because of the simple fact that they vasoconstrict blood vessels. When blood vessels vasoconstrict, some hemoglobin is altered to blood giving rise to the possibility the hemoglobin will sickle. As the scholars from the National Heart Lung and Blood Institute (NIH) report (2010), patients should avoid extreme heat or cold and also work to reduce overall stress levels. Patients also need to consider the possible early warning signs of a stroke.

These include numbness, one-sided weakness, and slurred speech patterns. The blood of a patient is transformed in the shortest time periods; so, it is necessary to provide a patient with a supplementary amount of folic acid to produce red corpuscles as suggested by the New York Times experts (2010). Patients will usually have a higher life expectancy by managing their health status.

Exams and Treatments

There are various types of treatment modalities that can aid the sickle cell patient in maintaining a healthy lifestyle. As suggested by the American Association of Clinical Chemistry (2009), life-threatening complications and specific episodes that occur in the process of treating a patient with sickle cell anemia can be approached with blood transfusions. Blood transfusions can also be helpful for children with sickle cell anemia in terms of improvements reached in height and weight indices (American Association of Clinical Chemistry, 2009). Approximately 8 g/dL is the hemoglobin level normal for patients with sickle cell disease. The hemoglobin level should not increase higher than 10 g/dL after transfusion which means that this level should be preserved. Routine lab work is also required and is basically part of the patients life.

Hospitals have now started performing sickle cell screenings on all newborn babies. This has become the routine and, with evidence supporting DNA testing, makes it easier to detect and treat sickle cell disease in a timely manner. According to the American Association of Clinical Chemistry (2009), hemoglobin electrophoresis has been used as a traditional method to define the presence of different variants of hemoglobin. Screening of hemoglobin variants is performed via HPLC and Hb S. Identifying the variants of hemoglobin that have the same electric potential is a common method for contemporary scholars (American Association of Clinical Chemistry, 2009).

Conclusion

A sickle cell disease is a diagnosis that decreases a life expectancy of a patient. However, this problem has been faced by contemporary health care specialists at the stage of a fetus which suggests new methods for preventing, diagnosing, and treating properly this disease. The most adequate approach lies through attending group therapy and maintaining consistent laboratory work, maintaining a healthy diet and avoiding certain factors that can trigger the onset of a sickle cell crisis. Understanding the disease and keeping to all prescriptions is essential for increasing life expectancy and preventing complications.

References

American Association of Clinical Chemistry. (2009). Sickle Cell Tests. Lab Tests Online. Web.

National Heart Lung and Blood Institute. (2010). Living with sickle cell anemia. Diseases and Conditions Index. Web.

New York Times. (2010). Sickle cell. Health Guide. Web.

Platt, O. S., Brambilla, D. J., Rosse, W. F., Milner, P. F., Castro, O., Steinberg, M. H., & Klu, P. P. (1994). Mortality in sickle cell disease. Life expectancy and risk factors for early death. New. England. Journal of. Medicine, 330(23), 16391644.

Appendix

Sickle Cell Anemia Intake Sheet

Personal Information

Patients information

  • Patients name: Albert Thompson
  • Gender: Male
  • Age: 10 years
  • Date of birth: September 10, 2000
  • Ethnicity: Brazilian/African American

Patients mothers information:

  • Mothers name: Isabel Thompson
  • Age: 32
  • Address: Jersey City, NJ
  • Occupation: Nurses Aide
  • Ethnicity: Brazilian

Patients fathers information:

  • Fathers name: Charles Thompson
  • Age: 35
  • Address: Jersey City, NJ
  • Occupation: Welder
  • Ethnicity: African American
  • Guardians information: (applicable if the patient does not live with his/her parents)
  • Guardians name: N/A
  • Age:
  • Address:
  • Occupation:
  • Ethnicity:
  • Relationship to the patient:

Family History

  • Number of patients siblings: Girls 1 Boys
  • Birth order of the patient: First
  • Marital status of parents:

    • Single parent
    • Married _X_
    • Divorced
    • Separated
    • Widowed
  • History of sickle cell anemia in either of the parents or siblings _None_
  • Parent/sibling death none
  • Parent substance abuse _None_
  • Sibling substance abuse _None_
  • Encounter with the criminal justice system (please specify) _None_
  • Living with immediate family members _Yes_
  • Living with extended family members _No_

School History

  • Current school: Joseph Smith Elementary School
  • School district: District #15
  • Grade level: _4_
  • Type of class: Regular Ed _X_ Special Ed Resource ED Behavioral unit
  • Current number of: Students _200_ Teachers _20_ Aides _8_
  • Does your child have a 1:1 Aide? _No_
  • Has your child had special education testing in school?
  • Psychological/Cognitive: _Yes_Date: November 2008
  • Academic: _Yes_ Date: March 2009
  • Speech/Language: _No_ Date:
  • Other: _No_ Date:
  • Is your child receiving any special education services at school? Yes - No _X_
  • Is your child on an IEP (Individual Education Plan)? _No_ If yes for what reason?
  • Please list all of the schools, including preschools, your child has attended:
  • Name of school Age/grade attended Hours per day Days per week
  • Saint Marys Preschool _Age 4-5_ _4 hours per day_ _5 days per week_

Medical History

  • What is the main health problem? _Sickle Cell Anemia_
  • When was it first discovered or diagnosed? _At birth_
  • How long has the patient lived with the condition? _10 years_
  • Are there any treatments/therapies that have been given to the patient? If yes, please list them
  • Treatment/therapy 1: _Extra fluids_
  • Treatment/therapy 2:
  • Treatment/therapy 3:
  • Others (please specify):
  • What was the outcome of the different treatments on the patient?
  • Treatment/therapy 1: _Patient is doing well with this treatment_
  • Treatment/therapy 2:
  • Treatment/therapy 3:
  • Others (please specify):

Symptoms

Has the patient showed any of the following symptoms?

Symptom Yes/No
Abdominal pain no
Bone pain no
Breathlessness minor
Stunted growth no
Fatigue minor
Fever no
Paleness yes
Rapid heart rate no
Ulcers on the lower legs no
Jaundice no
Chest pain yes
Extreme thirst no
Frequent urination yes
Poor eyesight no
Strokes no
Skin ulcers no

Treatments

  • Has the patient been treated with the following? Please check where appropriate:
  • Pain killers (please list them): Paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs)
  • Non-narcotic drugs: none
  • Narcotic drugs: none
  • Hydroxyurea: 500 mg daily
  • Antibiotics (please list them): Penicillin 125 mg daily
  • Blood transfusions (please list the frequency with which blood transfusions have been done): None
  • Dialysis: No
  • Kidney transplant: No
  • Drug rehabilitation: No
  • Removal of gall bladder: No
  • Surgery (please specify the type of surgery done): No
  • Care of wounds such as zinc oxide or surgery (please specify): No
  • Nutrition (please specify any special diets the patient is on): Daily multiple vitamin, Folic acid 1 mg.
  • Vitamin D:
  • Intravenous Fluid:

Vaccinations

Has the patient received vaccinations for the following conditions?

Vaccination Yes/No Year
Chicken pox yes October 2001
Mumps yes October 2001
Measles yes October 2001
Rubella yes October 2001
Small pox yes March 2001
Tetanus yes August 2005
Polio yes October 2004
Typhoid no -
Flu yes August 2010
Diphtheria yes August 2005
Pertussis yes August 2005
Influenza yes August 2010
Hepatitis B yes August 2006
Others (please specify) no 

Medical Tests

Has the patient undergone any of the following tests? Please check where appropriate

Tests Yes/No
Complete blood count (CBC) yes
Hemoglobin electrophoresis yes
Sickle cell test yes
Bilirubin no
Blood oxygen no
CT scan no
Magnetic resonance imaging no
Peripheral smear yes
Serum creatinine no
Serum hemoglobin yes
Serum potassium no
Urinary casts no
White blood cell count yes
Blood marrow/stem cell transplant no
Amniocentesis (in the patients parents) no

Side Effects

Has the patient suffered from any of the following side effects? Please check where appropriate and state the causal treatment:

  • Side effect Related treatment
  • Infections (please list them)
  • Rejection

Graft-vs-host disease

Complication Yes/No
Acute chest syndrome yes
Anemia yes
Vision impairment no
Brain and nervous system complications no
Multiple organ illnesses no
Drug abuse no
Gallstones no
Hemolytic crisis no
Infection no
Joint destruction No
Leg sores (ulcers) No
Dysfunctional spleen No
Parvovirus B19 infection No
Tissue death in the kidney No

Complications

  • Has the patient suffered from any of the following complications?
  • Patients signature: Date:
  • Parents/guardians signature: Date:

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