The sites most often affected by the blocking or stacking action of sickled cells are found in the lungs, liver, bone, muscles, brain, spleen, penis, eyes, and kidneys.
The immune system of a person with sickled cellsdramatically weakens. People with sickle cells arehighly susceptible to infections from certain forms of bacteria. Some of the most common infections are... from flu viruses, pneumonia, and salmonella (a type of bacteria).
Severe pain is the most common of sickle cell disease emergencies (acute sickle cellcrises). A personmay not know what brought on the pain, but one or more of the following situations may have contributed to the start of the painful sickle crisis:
Hypoxia (decrease in oxygen to body tissue)
Drug and alcohol use
Pregnancy and stress
Fourpatterns of an acute sickle cell crisis are now recognizable. They are based on the part of the body where the crisis occurs.
Bone crisis: An acute or sudden pain in a bone can occur, usually in an arm or leg. The area may be tender. Common bones involved include the large bones in the arm or leg: the humerus, tibia, and femur. The same bone may be affected repeatedly in future episodes of bone crisis.
Acute chest syndrome: Sudden acute chest pain with coughing up of blood can occur. Low-grade fevers can be present. The person is usually short of breath. If a cough is present, it often is nonproductive.Acute chest syndrome is common in a young person with sickle cell disease. Chronic (long-term) sickle cell lung disease develops with time because the acute and subacute lung crisis leads to scarred lungs and other problems.
Abdominal crisis: The pain associated with the abdominal crisis of sickle cell disease is constant and sudden. It becomes unrelenting. The pain may or maynot be localized to any one area of the abdomen. Nausea, vomiting, and diarrhea may or may not occur.
Joint crisis: Acute and painful joint crisis may develop without a significant traumatic history. Its focus iseither ina single jointor in multiple joints. Often the connecting bony parts of the joint are painful. Range of motion is often restricted because of the pain. Avascular necrosis of the hips can occur, causing permanent damage.
Many other organ systems are often injured orimpaired.
Central nervous system: Two-thirds of all strokes in people with sickle cell disease occur in children, at an average age of8 years. About 10% of people with sickle cell disease have strokes or other brain bleeding when younger than 8-10 years. As the population ages, the incidence of these events also increases. Repeat strokes occur in two-thirds of all survivors within 3 years of the first stroke. Blood clots affect the large vessels in the brain. Bleeding may occur in the small vessels damaged by sickle cell disease.
Eyes: The effectofsickle cell disease on the eyes comes from the increased viscosity, or "sludging," of blood and the narrowness of the eye's blood vessels. Retinopathy (disease of the retina in the eye) is common and causes problems with vision. Retinal detachment is frequent. Hyphemas, bleeding in the eye, occur at the same rate as the general population, but complications are more common because of the increased sickling effect that the waterlikefluid in the eye promotes.
Kidneys: Some amount of kidney damage occurs in nearly every person with sickle cell disease.
Genitals: Priapism (a constant erection of the penis) is common. It affects about 40% of all men with sickle cell disease. Severe episodes are a frequent cause of impotency.
Infections: People with sickle cell disease have weakened immune systems and are at increased risk for developing infection, especially in the lungs, kidneys, bones, and central nervous system.
Repeated crises damage the spleen, which over time, causes it to stop functioning.
Blood problems: People with sickle cell disease can develop anemia-a reduction in the number of red blood cells. Symptoms of anemia are shortness of breath (oxygen is not getting to tissues), lightheadedness, and fatigue.
Heart attacks can occur
The liver can also be affected by severe sickle cell crisis.
— with Shawns Testimony,
It may NOT be a HEADACHE/MIGRAINE!
Headaches in sickle cell anemia patients may
be caused by any of the common etiologies
or may be early symptoms of several life-threatening disorders, which are more
common in sickle cell anemia patients. These
include meningitis, subarachnoid
hemorrhage, or osteomyelitis of the jaw or
skull. Patients presenting with headache need
complete and thoughtful evaluation.
Present Illness. The headache must be
characterized as new or recurrent, unusual,
associated with fever, altered mental status,
location, radiation, character (steady,
throbbing, band-like), onset (day, night,
morning, evening), duration, aggravating/alleviating factors, aura, nausea, vomiting,
family history, stress, or trauma.
Review of Symptoms. Allergies, lacrimation,
nasal congestion, earache, toothache,
pharyngitis, neck pain or stiffness,
photophobia, neurologic symptoms (syncope,
ataxia, weakness, paresthesias,
dysesthesias), insomnia, anorexia, anxiety,
Past Medical History. Document hemoglobin
type, general health, past history of stroke,
hemorrhage, meningitis, seizures, recent
hospitalizations, or surgery, present
medications, drug allergies.
Vital Signs. Record temperature, blood
pressure, pulse, respiratory rate.
General. Determine mental status and
amount of distress.
HEENT. Note scalp tenderness, masses,
bruits, TMs, sinus tenderness, EOMs, optic
disk, nasal polyps, gums, pharynx, teeth.
Neck. SUPPLE?!, Define muscle spasm,
Chest. Seek signs of pneumonia.
Heart. Define new heart murmur or
change in heart murmur.
Abdominal. Record bowel sounds, liver/spleen size, tenderness.
Neurologic. Do a careful mental status
exam, document orientation x 3.
Meningeal signs - Stiff neck, Kernig’s,
CNs - Document II-XII are intact.
DTRs - normal active and asymmetric,
Muscle strength - asymmetric.
Sensation - intact to light touch.
Coordination - Normal gait, finger/nose,
CBC with differential and
Additional Lab. Chemistry pannel if patient is
hypertensive. Skull x-ray for point
tenderness/masses, fever, or trauma. Sinus
films for sinusitis symptoms and fever/tenderness. CT for severe headache, altered
mental status, or focal neurologic findings.
Lumbar puncture for fever and stiff neck,
severe or unusual headache, altered mental
status, or meningeal signs. MRI-MRA if not an
- Tension/Psychogenic. Bitemporal, base of
skull, band-like, last hours to days, steady,
pressure, tightness, does not disrupt sleep,
negative exam except for scalp and or neck
tenderness and increased tone in neck
- Sinusitis. Frontal and maxillary pain, around
eyes, throbbing, dull aching, positional, hours
in duration, rhinorrhea, itching eyes, nasal
congestion, exam negative except for
tenderness over sinuses, nasal congestion,
and low grade fever. Fever suggests acute
- Vascular/ Migraine. Headaches are
frequently initially unilateral, throbbing, may
be associated with nausea, vomiting, and
photophobia. Auramay be present and family
history is often positive. Precipitated by
stress, menstruation, BC pills. Exam usually
- Cluster. Headaches are unilateral, behind
eye, sharppain, of short duration, male
predominance, repetitive. Exam may show
redness, tearing, rhinorrheaon side of pain.
- Hypertension. Headaches are diffuse,
throbbing, worse in morning. Exam shows
elevated blood pressure and retinal changes.
- Meningitis/Encephalitis. Diffuse or posterior
with radiation down neck, severe, throbbing,
recent onset, chills or fever, lethargy,
vomiting, photophobia, irritability, and new
seizures. Exam may show alteredmental
status, fever, rash, or focal neurologic
findings. Laboratory may show leukocytosis
with left shift, leukopenia, and DIC. LP shows
leukocytosis with increased neutrophilsor
- Subarachnoid Hemorrhage. Anterior or
diffuse, down neck, acute onset, severe,
unremitting, vomiting, confusion, lethargy, or
seizures. Exam may reveal meningeal signs,
or focal neurologic findings. Patients are
usually afebrile. Many patients present only
with an unusual headache and no other
findings. CT usually (about 85%) positive. LP
usually positive for RBCs and/ or
xanthochromia. MRA or angiography may
demonstrate multiple aneurysms in adults.
- Osteomyelitis. Localized pain over skull or
jaw, fever, mass may be palpable. Lab reveals
leukocytosis with left shift. X-rays, bone scan,
or gallium scan are positive. Bone scan most
sensitive, gallium scan most specific.
- Bone Infarction. Localized pain over jaw or
skull, NO fever and a mass may be palpable.
Lab reveals normal white count. X-rays are
usually negative. Bone scan shows
decreased uptake for about a week the
normal or increased uptake. Gallium scan
may or may not be positive. Findings improve
Brain Abscess. Diffuse or localized,
intermittent or constant, progressive, fever,
history of sinus, ear, or lung infection. Exam
often shows focal neurologic findings and
source of infection. Lab findings include
leukocytosis with left shift. Brain MRI or CT
are positive. LP usually shows increased
protein, and leukocytosis.
Brain Tumor. Diffuse or localized to area of
mass, intermittent or constant, progression in
severity and duration, personality changes, or
new onset seizures. Exam shows no fever.
Focal neurologic findings and papilledema
Plan - Treatment
- Tension/Psychogenic. Treatment of non-crisis pain in sickle cell patients is
complicated by previous experiences of
complete pain relief with narcotics. Education
is required to assure patients that all pain
needs not be treated withnarcotics.
Treatment with plain acetaminophen or
aspirin is all that should be given for these
headaches. Relaxation techniques, mild heat
to neck, and massage will all help greatly.
- Sinusitis. Acute sinusitis with fever and
crisis requires admission for parenteral
antibiotics. Acute sinusitis with fever and no
crisis can be managed with ampicillin500
mg. P.O. q6h or bactrim DS i tab p.o. bid for 7
to 14 days , Actifed i tab p.o. q 6-8 hours PRN
congestion, and plain acetaminophen 900
mg. p.o. q4 h or aspirin 600 mg. p.o. q 4h
PRN pain. Warn patient about operating
machinery and drowsiness. Acute sinusitis
without fever, as above, without antibiotics.
- Vascular. Migraine should not be treated
with ergotamine, Sumatriptan (Imitrex) or
Zolmitriptan (Zomig) in this population
because of vasoconstriction. Tylenol #3 and
nonsteroidal antiinflammatory drugs
(NSAIDs) may be required to control pain.
Rest and relaxation techniques, propranolol
or Verapamil (Isoptin, Calan), 80 mg tid or qid
.may reduce frequency. Amitriptyline (Elavil)
or imipramine (Tofranil), 50 to 75 mg in
divided doses or at bedtime, provides
effective migraine prophylaxis for some
patients. Cluster headaches are treated as
above but oxygen inhalation at a flow rate of
7L/minute for 10 minutes is said to abort
about 80% of cluster headaches.
Hypertensive headaches respond to control
of the blood pressure.
- Meningitis/Encephalitis. Admit for I.V.
antibiotics based on gram stain.
- Subarachnoid Hemorrhage. Needs
emergency admission to a neurosurgery
service. Patient should have an exchange
transfusion in preparation for immediate
angiography and possible surgery. Exchange
must be done at isovolemia to prevent arterial
spasm making erythropheresis the method of
- Osteomyelitis. Should be
medicine with neurosurgery consult. If
osteomyelitis is suspected, biopsy for culture
should precede antibiotic administration.
- Brain Abscess. Should be admitted to the
neurosurgical or medical service with a
neurosurgical consult for diagnostic and
- Brain Tumor. Should be admitted to a
neurology or neurosurgical service. Exchange
transfusion should be initiated to prepare for
diagnostic procedures and surgery.
Patient/parent must be taught that severe,
uncharacteristic headaches can be early
signs of neurological problems; and that they
need to be evaluated by healthcare providers
for meningitis, encephalitis and subarachnoid
Patients with chronic benign headache
must be reassured about the nature and
proper management of their problems
Encourage patients totake medicine
before pain becomes too intense
Monitor patients for adverse reaction
Encourage rest and provide quiet
Assess history of sinus and ophthalmology
The main prevention strategy is aggressive
work-up of headaches to diagnose potentially
devastating complications early so that
treatment will be of maximum benefit.
Patient and Parent Education
Patients with benign headaches must be
reassured about the nature andproper
management of their problems. This is
difficult in patients with past episodes of
meningitis, encephalitis and subarachnoid
hemorrhage. Parents must be educated about
the need to have children evaluated for fever,
headache, or changes in mental status
Hemoglobin is the protein in RBCs that carries oxygen. It is made up of two alpha chains and two beta chains. These are made by the alpha and beta genes. The four main types of sickle cell anemia are caused by different mutations in these genes.
Hemoglobin SS Disease
Hemoglobin SS disease is the most common type of sickle cell disease. It occurs when you inherit copies of the hemoglobin S gene from both parents. This forms hemoglobin known as Hb SS.
Hemoglobin SC Disease
Hemoglobin SC disease is the second most common type of sickle cell disease. It occurs when you inherit the Hb C gene from one parent and the Hb S gene from the other. Individuals with Hb SC have similar symptoms to individuals with Hb SS. However, the anemia is less severe.
Hemoglobin SB+ (Beta) Thalassemia
Hemoglobin SB+ (Beta) thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made. If inherited with the Hb S gene, you will have Hemoglobin S Beta thalassemia.
Beta-Zero thalassemia is the second type of beta thalassemia. It has similar symptoms to Hb SS anemia. However, sometimes the symptoms of beta-zero thalassemia are more severe. It is associated with a poorer prognosis.
People who only inherit a mutated gene from only one parent are said to have sickle cell trait. They may have no symptoms or reduced symptoms.
WHY TRAIT IS DANGEROUS
Sickle Cell Trait Can KILL you!
TRAIT...You heard it right!
TRAIT...Yes..the same one they tell you not to worry about!
TRAIT...Yea..the same one that they brushed off when you weren't feeling too hot because YOU COULDN'T BE HAVING ANY PROBLEMS BECAUSE.. WHAT...YOU JUST HAVE TRAIT!
TRAIT...Yep..the same one that NOW after HOW MANY athletes DYING are taking precautions in the NCAA. But wait...WHAT ABOUT THE PEOPLE OUTSIDE OF THE NCAA? This is why people likeWhiskey Delta Charlie are so important to this community! He posted this in the midst of my crisis and I just had to share with you guys!
Sickle Cell Trait Toolkit
CDC, together with the American Society of Hematology (ASH) (http://www.hematology.org/) and the Sickle Cell Disease Association of America (SCDAA) (http://www.sicklecelldisease.org/), created the Sickle Cell Trait Toolkit. The toolkit is a collection of resources to increase understanding of sickle cell trait. Check back soon for more tools that will be added in the coming months.
Basic facts about sickle cell trait.
Learn why and how to get screened for sickle cell trait.
Did you know there’s more than one way to inherit sickle cell trait? Learn how it is inherited.
Learning some NEW things about Sickle Cell
Pulmonary veno-occlusive disease is an extremely rare disease that causes high blood pressure in the lung arteries (pulmonary hypertension).
In most cases, the cause of pulmonary veno-occlusive disease is unknown. The high blood pressure occurs in the pulmonary arteries, which are the lung arteries directly connected to the right side of the heart.
The condition may be related to a viral infection. It may occur as a complication of certain diseases such as lupus, or as a complication of leukemia, lymphoma, chemotherapy, or bone marrow transplantation.
The disorder is most common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins,pulmonary artery hypertension, and congestion and swelling of the lungs.
Exams and Tests
The doctor or nurse will examine you and ask about your medical history and symptoms.
The exam may reveal:
- Increased pressure in the neck veins
- Fingernail clubbing
- Bluish coloration of the skin due to lack of oxygen (cyanosis)
- Swelling in the legs
Your doctor may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.
The following tests may be done:
There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:
- Vasodilator drugs (drugs that widen the blood vessels)
- Drugs that control the immune system response (such as azathioprine or steroids)
A lung transplant may be needed.
The outcome is often very poor in infants, with a survival rate of just a few weeks. Survival in adults may be months to a few years.
- Progressive difficulty breathing
- Pulmonary hypertension
- Right-sided heart failure (cor pulmonale)
- Coughing up blood
When to Contact a Medical Professional
Call your health care provider if you have symptoms of this disorder.
Pulmonary vaso-occlusive disease