SICKLE CELL DISEASE

SICKLE CELL DISEASE

Sickle disease is the medical name for several similar but different conditions.

The 3 most common kinds of sickle cell disease are:-

1. Sickle cell anaemia
2. SC. Disease
3. Sickle bethathalassaemia disease sickle cell disease is inherited from the parents.

Types of haemoglobin

1.  Haemoglobin A (HbA) most common

2.  Sickle haemoglobin (Hbs)

3.  Haemoglobin C (Hbc)

4.  Beta –thalassaemia (Beta-thal or B-thal).  If you inherited 2 genes for haemoglobin A (HbA) you will have Hb AA (Hb AA) the normal Hb.

Types of Hb usually called for are:-

1.  Hb AA – Normal haemoglobin

2.  Hb AS – Sickle cell trait

3.  Hb AC – C trait

4.  Hb A Beta that thalassaemia trait

5.  HbSS – Sickle cell disease

6.  Hb SC – SC disease                                         Kind of sickle cell diseases

7.  Hb S Beta thal – Sickle beta thalassemia

How sickle cell Disease are Inherited

1.  SC Disease (Haemoglobin SC)

Father

Hb AS                                                Mother Hb AC

     Hb SC child (pt)

2.  Sickle cell anaemia (Haemglobin SS)

Father Hb AS                                    Mother AS

                                    SS

3.  Haemoglobin S – Betha -thal

     Sickle Beta –thalassaemia

Father Hb AS                     Mother

     Hb A Betatha

                                              

                                            HbS. Betathalassaemia

S.C. Disease (HbSC)

1.  It occurs when a child inherits sickle haemoglobin (HbC) from the other

2.  It is not as common as sickle cell anaemia

3.  It is milder than sickle cell anaemia

Signs and Symptoms

1.  These patients are less anaemic than those with sickle cell anaemia.  Their Hb may

      be   in the normal range.

2.  They have fever crises and suffer less severe infections e.g pneumonia.

Types of Hb usually called for are:-

1.  Hb AA – Normal haemoglobin

2.  Hb AS – Sickle cell trait

3.  Hb AC – C trait

4.  Hb A Beta that thalassaemia trait

5.  Hb SS – Sickle cell diseases.

6.  Hb SC – SC diseases                                              Kinds of sickle cell diseases

7.  HbS Beta –thal – Sickle beta thalassemia

How sickle cell Disease are inherited

1.  SC  Disease (Haemoglobin SC)

     Father                                 Mother

    HbAS                                  HbAS

HbSC child (pt)

2.  Sickle cell anaemia (Haemoglobin SS)

     Father                                    Mother

     HbAS                                    AS

 

                         SS

3.  Haemoglobin S – Betha - thal

     Sickle Beta – thalassaemia

                                               

Father                                      Mother

Hb AS                                     Hb A Betathal

                                               

                       

HbS Betathalassaemia

SC Disease (HbSC)

1.  It occurs when a child inherits sickle haemoglobin (HbS) from one parent

     and  haemoglobin C (HbC) from the other.

2.  It is not as common as sickle cell anaemia

3.  It is milder than sickle cell anaemia

Signs and Symptoms.

1.  These patients are less anaemic than those with sickle cell anaemia.  Their Hb may

      be in the normal range.

2.  They have fever crises and suffer loss severe infection eg. pneumonia.

3.  Because of high Hb their blood is thicker than those with sickle cell anaemia and

     this cause problems in the small vessels of the eyes sick ling in the eyes can

     cause partial or total loss of vision.

4.  Joint problems especially joints of hips and shoulders.

5.  Other problems are similar to those found in sickle cell anaemi.

Treatment.

1.  Same as for sickle cell anaemia

2.  Should drink enough fluids

3.  Takes folic acid and antimalarials

4.  Penicillin to prevent infection may be prescribed

5.  Good supervision during pregnancy and child birth.

SICKLE BETA – THALASSAEMIA

(Hbs Beta – thal)

This occurs when a child inherit sickle haemoglobin (Hbs) from one parent and thalassaemia (Beta-thal) from the other.

NB:  If you have sickle beta-thalassaemia disease you must inform the Doctor.  eg:-

(a)    Before anaesthesia

(b)    As soon as you are pregnant so that extra care can be given.

Treatment

Blood transfusions and other measures as for sickle cell anaemia.

Care of a patient with sickle cell anaemia

• In this type of anaemia many of R.B.C are sickle shaped and easily destroyed.
• This disease is due to inherited abnormal haemoglobin called haemoglobins (Hbs)
• Sickle cell anaemia usually present itself after 6 months of age as Hbs recedes
• Sickle cell anaemia is common to the negroes.
• Parents with Hb, As can have children with haemoglobin AA, and SS.
• People with Hb AS are carriers (trait).
• People with HbSS are patients of sickle cell anaemia.

Signs and Symptoms.

1.  Begin in infancy and the patient dies before 30 years.

2.  Anaemia

3.  Jaundice

4.  Enlarged spleen

5.  Crises of pyrexia

6.  Pain in the joints

7.  Intravascular clothing

8.  Between crises the patient is list less and depressed

9.  Chronic ulceration of the legs is common

10. Enlargement of the bones of the bones of the face and the skull; (prominent for

      head and high cheek bones)

11. The chronic anaemia leads to tachycardia and heart failure lacter on

      with cardiomegally.

12. Asptic femoral mecrosis, renal damage, pricipism and retinopathy.

Diagnosis

1.  Sickle cell test

§  Up to 60% of the R.B.C may be sickle in shape.

§  R.B.C are reduced to 3 million or even less per ml³ during crises.

Problems

• Anaemia, wekness, pain, fever.

Management And Nursing Care

1.  Bed rest during crises

2.  Take measures to reduce temperature

3.  Transfuse with packed cells.

4.  Splenectomy may help

5.  Administer oxygen to correct hypoxia.

6.  Relieve pain by analgesics

7.  Have identity card to show type of sickle cell disease you have.

8.  See doctor when pregnant for extra care close observation

9.  These patients should avoid hypoxia, dehydration, acidosis or cold as they lead

     to sickling.

10.  Prompt treatment and prevention of infection by ant malarial, penicillin etc

11. Treat crises by hydration correcting of precipitating factors etc.

12. Administer folic acid

13. During crises (sickling) encourage plenty of fluids.

14. Reassure and make them relaxed and feel safe as they have fear.

15. Patient should knows the type of sickle cell disease he have and inform the

      doctor incase of operation, anaesthesia, accidents etc.

16. Regular check ups.

17. Patient should discusses and ask question about the disease.

18. Strokes are prevented by blood transfusion every 4 – 6 weeks for 3 years.

19. Exchange transfusion are helpful.

20. Immunize children with sickle cell diseases.

21. At the time being no cure for sickle cell diseases.  Don’t give iron pills or tonics.

Care during Painful Crises Due To Sickling

• Inform doctor
• Make patient comfortable in bed.
• Give plenty of fluids
• Give pain killers
• Reassure as they are worried

When to seek Medical Advice

1.  Incase of fever over 38C (100oF)

2.  Severe pain in the body

3.  Persistent cough

4.  Bad headache, feeling sleepy, wekness on one side of the body.

5.  Diarrhoea and vomiting

6.  short of breath

7.  Have no energy (listless)

8.  Sudden paleness

9.  Jaundice

10.  Priapism

11. Swollen and tender abdomen in children (sickling in the spleen)

Causes Of Sickling

Predisposing Factors For Sickling

1.  Reduced supply of Oxygen

2.  Strenous exercises

3.  Anaesthetics

4.  Dehydration

5.  Infection

6.  Fever

7.  Pregnancy

8.  Sudden change in temperature

9.  Alcohol

10.  Emotional stress

Prevention of Sickling

1.  Prevent the above factors

Complications of Sickle cell anaemia

1.  Paiful swollen hands and feet.

2.  Painful crises

3.  Infections

4.  Anaemia

5.  Splenic Squestration

6.  Jaudince

7.  Frequent urination

8.  Bed wetting

9.  Dark urine and haematuria

10. Large abdomen due to enlarged spleen

11. Nose bleeding

12. Teeth stick out

13. Poor appetite

14. Strokes when suckled cells block the blood vessels

15. Growth retardation

16.  Sores and ulcers around the ankles

17.  Gall stones

18. Chest syndrome (sickling in the lungs)

19. Blurred vision and loss of sight

20. Kidney disease

21. Weak bones

22. Peptic ulcer

23. Painfull crises during pregnancy

24. Priapism

25. Anaesthetic cause suckling

Testing for sickle cell Disease

1.  The sickle test

2.  The solubility test

3.  Haemoglobin electrophoresis

Care of a patient at Home

 1.  Take plenty of fluids

 2.  Avoid too much exercises

 3.  No special diet

 4.  Express your worries freely

 5.  Don’t take yourself as a permanent invalid when you are well live as normal

      as possible.

How sickle cell Disease may affect the family

1.  Shock – or Denial parent don’t believe.

2.  Gruilt – parents feel quilt that they are the cause

3.  Blame – Parents blame each other

4.  Anger – Family may change its life style

5.  Fear – Parents are afraid of how to cope.

6.  Helplessness – Parents feel helplessness as they cannot help.

7.  Shame – Parents don’t want any body to known

8.  It is a curse – parents think some one doesn’t like their family and cursing them.

9.  Jelons from brother and sisters that the patient is loved more by parents

10. Jelous from child that why is he different from other brother sisters

Health Education

1. Avoid all factors leading to suckling

2.  Keep doctors appointment

3.  Prevent and treat infections early.

4.  Seek medical aid in crises

5.  Report to doctor when pregnant for extra care.

6.  Express worries freely to prevent mental stress.

7.  Carry with you your sickle cell disease identity card

8.  Immunize fully all children

9.  Inform your doctor that you have sickle cell disease prior operation; anaeshtesia etc.

10. have bed rest during crises

11. Have pain killer to relieve pain at home.

12. Live as normal as possible between crises

NB:  Read notes and add more health Education.