The Sickling Process
Sickle cell disease affects the hemoglobin part of red blood cells. Hemoglobin carries oxygen from the lungs to other parts of the body and gives blood its red color.
Normal red blood cells are round, soft, and flexible. They can squeeze through small blood vessels allowing the blood to flow easily. In sickle cell disease, red blood cells can change from round to half moon (or sickle shaped) when the hemoglobin gives off its oxygen. When red blood cells are shaped like a sickle, they are hard and inflexible and plug up small blood vessels. This causes the flow of blood and oxygen to be slowed down or stopped.
Pain, damage to parts of the body and anemia can result. When the blood flow stops or slows suddenly, the problem is called sickle cell crisis. The following chart (Sickling of Venous Blood) shows how some body organs and tissues may be affected by the plugging of blood vessels.
Sickling of Venous Blood
| ORGAN/TISSUE INVOLVED |
PROBLEMS CAUSED |
| KIDNEY |
* Enuresis
* Hematuria
* Nephrotic Syndrome
* Unconcentrated urine
* Urinary frequency |
| SPLEEN |
* Increased risk for serious infections
* Splenic Sequestration
* Abdominal pain |
| LUNGS |
* Pneumonia
* Acute Chest syndrome |
| BONES |
* Infection
* Aseptic Necrosis |
| BRAIN |
* Stroke
* Headache |
| SKIN |
* Slow healing ulcers |
| PENIS |
* Priapism |
| EYES |
* Sickle Cell Retinopathy |
| LIVER |
* Hepatomegaly
* Cholelithiasis
* Jaundice |
| • Not all these problems happen to everyone with Sickle Cell Disease. You need to know, however, that they can happen. Notify the parents/caregivers immediately if you think their child has any of these problems. |
Pain
Pain is the most common problem in sickle cell disease. Fever, infection, exposure to extreme cold, physical exhaustion, and/or unusual stress/anxiety may trigger the pain. Sickling in the venous system causes pain. It may occur at any time and in any part of the body. Each person differs in terms of severity, duration, and frequency of pain. The pain may be so mild that no analgesic (pain medicine) is needed, or may be severe enough to require hospitalization. Patients with sickle cell disease should be encouraged to take an active role in the management of their crises.
SPECIAL CARE IN SCHOOL SETTING
Treatment to relieve mild to moderate pain may include any or all of the following:
- Rest (bed-rest or a decrease in activity).
- Increased fluid intake with drinks such as caffeine-free soft drinks, juices, or bouillon.
- Analgesics as directed by the physician. There are slow-release preparations of morphine and codeine, which can be given every twelve hours, often obviating the need to give medication in school.
- Relaxation exercises to help relieve pain (if the child is familiar with these techniques).
- Avoid immersion in cold water.
- Avoid excessive exposure to cold temperatures.
*** Call the parent/caregiver and/or physician immediately if any of these symptoms are present. If the family is unavailable, call the child's health care provider or the Sickle Cell Treatment Center immediately.
Kidney
Children with sickle cell disease urinate frequently due to an inability to concentrate urine. This inability to concentrate urine is the result of damage to the kidneys due to repeated sickle cell crises. Children with sickle cell disease are also more prone to kidney infections.
- SIGNS AND SYMPTOMS
- dysuria
- hematuria
- more frequent urination than usual for this child
- SPECIAL CARE IN SCHOOL SETTING
- The child should be allowed to use the bathroom frequently.
- Notify the family if child has hematuria (blood in the urine).
- The child needs to get plenty of fluids throughout the day.
Fluid Requirements For Children With Sickle Cell Disease
| CHILD’S WEIGHT |
APPROXIMATE DAILY FLUID REQUIREMENTS |
| 25 lbs. |
1½ to 1¾ qts. |
| 50 lbs. |
2 to 2½ qts. |
| 75 lbs. |
2¼ to 3 qts. |
| 100 lbs. |
2½ to 3½ qts. |
Infection/Fever
Infection is a life-threatening problem in sickle cell disease. Children with sickle cell disease have less resistance to infection and develop infections more easily and more frequently than other children. Children with sickle cell disease are typically on prophylactic (preventive) penicillin until five years of age. Infection is the most common cause of morbidity and mortality in young children with sickle cell disease. Prophylactic penicillin has decreased, but not eradicated, this problem.
The child with a serious infection may not appear to be acutely ill in the early stages. Therefore, it is important to learn the early warning signs and to know when to contact the family and when to advise referral to the primary care provider, treatment center, or emergency room.
- SIGNS AND SYMPTOMS
- fever (temperature of 101 degrees or higher) is the most common symptom of infection
- lethargy (unexplained tiredness)
- SPECIAL CARE IN SCHOOL SETTING
- A fever in a patient with sickle cell disease of 101 degrees or higher is a medical emergency.
- These patients must be seen in an emergency setting as soon as possible.
- The child's family and physician should be contacted immediately.
Lungs
Respiratory complications are common in children with sickle cell disease. These can be secondary to either an acute lung infection (pneumonia) or sickling in the lung. Dyspnea (difficulty breathing) and chest pain are typical symptoms. A transfusion is often necessary. The combined symptoms are called Acute Chest Syndrome, which can be life threatening.
- SIGNS AND SYMPTOMS
- chest pain ***
- cough
- dyspnea
- tachypnea
- fever
- SPECIAL CARE IN SCHOOL SETTING
- Encourage deep breathing exercises i.e. use of an incentive spirometer (machine that measures the amount of inhaled and exhaled air) when recommended by child's physician (Sometimes pain may prevent the child from breathing deeply.)
- Encourage compliance with medications for asthma.
- Report any of the above signs and symptoms to the child's family or the Sickle Cell Treatment Center.
*** Recognize that chest pain is an emergency. Hospitalization of the child with acute chest syndrome is imperative.
Spleen
One of the spleen's functions in the body is to filter out bacteria from the blood stream and help the body fight infection. Due to sickling in the spleen, splenic dysfunction and eventual fibrosis develop. These children are therefore at a higher risk for developing infections.
Splenic sequestration is a possible complication of sickle cell disease that occurs most commonly in children under 5 years of age, but can occur at any age It occurs when there is a sickling in the splenic vein resulting in the sudden entrapment of a large amount of blood in the spleen. The spleen suddenly becomes very large and the child can have signs and symptoms of a very low blood count (he/she becomes very anemic) or shock in extreme instances. This is usually treated with hydration, analgesia, and red cell transfusion. In some children, a splenectomy (removal of the spleen) is necessary.
The occurrence of splenic sequestration is difficult to predict. It comes on suddenly, and immediate action is required for the reversal of symptoms. For some children/young adults with sickle cell disease, the spleen may always be palpable and this is normal for that individual. Again, it is important to know what is normal for the child. Many families are taught splenic palpation (feeling for the spleen) to help identify this complication at an early stage.
- SIGNS AND SYMPTOMS
- sudden weakness
- pallor (especially of lips, gums and nails)
- abdominal pain
- increase in abdominal girth
- enlargement of the spleen
- fever
- SPECIAL CARE IN SCHOOL SETTING
- Should you observe these signs and symptoms, notify the child's family and advise them to contact the child's physician or sickle cell treatment center immediately. ***
- These patients need to be evaluated in an emergency setting as soon as possible.
*** Prompt recognition and treatment is necessary. Hospitalization and a blood transfusion may be required.
Anemia
Children with sickle cell disease usually have anemia. They usually adjust and are asymptomatic (without symptoms). There are times, however, when their blood count experiences a substantial drop in red blood cells. Failure to adequately replace the red blood cells can cause pallor, fatigue, and loss of appetite. An acute, (sudden) drop in the red cell count is referred to as an aplastic crisis. This is discussed in detail in the next section.
Normal red blood cells live for approximately four months before they break down. Regeneration normally occurs within four months. Sickled red blood cells live less than one month. The body is unable to produce sufficient red blood cells for replacement, resulting in anemia. When red blood cells break down, bilirubin (the orange-yellow pigment in bile) is produced. In sickle cell anemia, excess bilirubin causes jaundice (yellowing) which can be seen in the sclera (white portion) of the eyes, the color of the urine, and the color of the skin. These are often normal findings in a child with sickle cell disease.
- SIGNS AND SYMPTOMS
- fatigue
- poor appetite
- pallor
- jaundice that is greater than normal for the child
- SPECIAL CARE IN SCHOOL SETTING
- Should you observe any of these signs and symptoms, notify the child's family
Aplastic Crisis
An aplastic crisis is a sudden severe drop in the hemoglobin. In other words, a person becomes more anemic than usual. This is typically due to an infection with parvovirus, also the cause of Fifth Disease in childhood. The bone marrow slows down and much needed red blood cells are not produced. The life expectancy of a sickled red blood cell is 15 to 20 days instead of the typical four months for normal red blood cells.
- SIGNS AND SYMPTOMS
- Sudden fatigue
- Pallor
- Lack of interest in play
- Shortness of breath
- Tachycardia (fast heart beat)
- SPECIAL CARE IN SCHOOL SETTING
- Should you observe any of these signs and symptoms, notify the child's family immediately.
- Shortness of breath and tachycardia should be evaluated in an emergency setting as soon as possible.***
*** Hospitalization and blood transfusion may be necessary.
Bones and Joints
Osteomyelitis
Often the signs and symptoms of this infection are similar to those of sickle cell pain episodes in the joint or bone.
- SIGNS AND SYMPTOMS
- pain
- swelling
- erythematous (red) joint
- fever
- warmth
Painful Crisis
Almost all children with sickle cell disease will have a painful crisis at some point in their lives. The pain and swelling occur as the result of vessels being occluded (blocked) by sickled cells. Some children have them very often. A fever or infection, dehydration, exposure to extreme heat or cold, physical exhaustion, and unusual stress or anxiety may trigger the pain. However, most often there is no obvious reason why a child gets a painful crisis. The pain can happen in any part of the body.
- SIGNS AND SYMPTOMS OF PAIN CRISIS
- Bone pain crisis
- pain
- swelling of the hands and feet in young children; long bones and back in older children
- fever
- Abdominal pain crisis
- abdominal distention
- dyspnea
- SPECIAL CARE IN SCHOOL SETTING
- Fluids - It is recommended that the child be given juices rather than water so as to replace the electrolytes lost due to frequent urination.
- Rest.
- Analgesics (pain medicine) as recommended by the child's treatment center.
Cholelithiasis (Gallstones)
Adolescents and young adults often have problems with cholelithiasis. This is due to excess bilirubin (product of blood breakdown) caused by red blood cell destruction. Excess bilirubin may also collect in the gallbladder and form stones.
- SIGNS AND SYMPTOMS
- abdominal pain
- vomiting
- fatty food intolerance
- SPECIAL CARE IN SCHOOL SETTING
- Notify the child's parents or Sickle Cell Treatment Center. ***
*** Hospitalization and surgery may be necessary.
Central Nervous System
Stroke is a serious complication of sickle cell disease. It occurs when part of the brain doesn't get as much blood as it needs. This is caused by sickle cells blocking a blood vessel in the brain. There is a lifetime incidence of approximately 10%. Once experienced, there is a tendency for recurrence.
*** If a stroke is diagnosed, the child/young adult should be hospitalized immediately for observation, testing, and treatment. Treatment is aimed at preventing a second stroke and this is achieved by giving transfusions of red blood cells on a chronic basis in order to maintain the percent of Hemoglobin S at less than 30%. There is an ongoing study to determine the benefits of screening for the likelihood of a stroke, enabling prophylactic (preventive) transfusion treatment.
This continues to be a controversial treatment option.
Frequently, the child/young adult who has a stroke may return to normal activity in several days. However, there may be physical weakness and/or disability requiring rehabilitation.
Priapism
Priapism (continued erection of the penis without sexual desire) occurs when sickle cells block blood circulation in the penis.
- SIGNS AND SYMPTOMS:
- Painful, prolonged swelling of the penis
- Sustained unwanted erection
- SPECIAL CARE IN SCHOOL SETTING
- Attempt to dispel embarrassment.
- Counsel adolescents regarding the importance of reporting signs and symptoms.
- Notify the family and physician IMMEDIATELY when symptoms are present. ***
*** Hospitalization may be required.
Aseptic Necrosis
When blood vessels supplying bone get blocked by sickle cells, the bone can become damaged. When sickling occurs repeatedly in the veins supplying bone, damage may occur in these tissues.
- SIGNS AND SYMPTOMS
- Chronic hip, extremity or lower back pain which does not resolve
- Afebrile
- Occurs most commonly in the hip bone
- Persistent pain in groin or buttock
- Gait (manner of walking) disturbance
- SPECIAL CARE IN SCHOOL SETTING
- Notify the child/young adult's family if these symptoms are present.
- Oral analgesics.
- Increased fluids
- Appropriate limitation of activities in physical education
- Rest
- Physical therapy may be necessary
- Surgery is necessary in severe cases
Sickle Cell Retinopathy
Children with sickle cell disease may develop sickle cell retinopathy, which can occasionally lead to blindness. This condition manifests slowly and rarely occurs before the age of ten years. Sickle cell retinopathy is more common in children with Hemoglobin SC disease.
- SPECIAL CARE IN SCHOOL SETTING
- Notify the child's family immediately of any vision problems or trauma to the eye.
- After the child reaches ten years of age, he/she should be seen annually by an ophthalmologist who is familiar with sickle cell disease.
Skin Problem
Some patients develop ulcers, especially around their ankles. This problem does not happen often and usually affects older children and adults.
- SIGNS AND SYMPTOMS
- Cut or wound that doesn't heal.
- A patch of dry itchy skin.
- SPECIAL CARE IN SCHOOL SETTING
- If the child has any ulcerations (skin sores), which are not healing normally, or look infected notify the parents.
- Keep the area clean.
- Put on a fresh bandage twice a day or as often as directed.
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