An amazing sicklecell power point!

A Sickle Cell Power Point

Why Cartilage Damage is WORSE than CRISIS

Connective Tissue Structure
and Support

Cartilage is connective tissue that is less rigid than bone and
less exible than muscles

Connective tissue is essential for our
bodies to function properly. Bone
connective tissue provides structure and
support, adipose (or fat) connective tissue
insulates and provides energy, and blood
connective tissue distributes oxygen to
our tissues and removes carbon dioxide.
Another integral type of connective tissue
is cartilage.
The main structural components of our
bodies are bone, muscle, and cartilage.
Bones are rigid, while muscles bend,
stretch, and are exible. Cartilage
connective tissue is the perfect halfway
point between these other tissues. It is
not as rigid or as hard as bone, and it is
also less exible than muscle. Therefore,
we nd cartilage in places where we need
some support and structure, but a bit of
exibility as well. This includes places
such as our joints, our ears, and our nose,
as well as in between the vertebrae in our
spinal column.

Chondroblasts and
Chondrocytes
Connective tissue is comprised of living
cells within an extracellular (or outside
the cell) matrix. The extracellular matrix in
cartilage is produced by specialized cells
called chondroblasts. Chondroblasts that
are caught in the matrix are called
chondrocytes. These cells lie in spaces
called lacunae. Chondrocytes, also called
chondrocytes in lacunae, determine how
'bendy' our cartilage is.
When looking through a microscope,
chondrocytes look similar to eyeballs
oating in goo. Have you ever served
eyeball soup for Halloween? This is the
perfect way to describe cartilage
connective tissue. The eyeballs are our
chondrocytes, and the soup is the matrix
they live in. That's the easiest way to
determine what type of cartilage you're
talking about - the number of 'eyeballs' in
the soup.

Elastic Cartilage
There are three types of cartilage found in
the human body. Elastic cartilage is the
most exible, which means it contains the
most chondrocytes. This is the type of
cartilage found in your ear. If you look at
this slide, which is what elastic cartilage
looks like under a microscope, you'll note
quite a few chondrocytes.

Fewer chondrocytes in hyaline cartilage make it less exible
than elastic cartilage

Hyaline Cartilage
Hyaline cartilage is the second most
exible, and this cartilage is found in your
nose and at the end of your ribs. Again,
note the chondrocytes in this tissue; you'll
notice there are fewer than in the elastic
cartilage tissue slide.

Fibrocartilage
Fibrocartilage is the cartilage with the
fewest number of chondrocytes, which
means it bends the least. This is the type
of cartilage found in your knee, as well as
in between the vertebrae in your spine.
Note how many chondrocytes are on this
slide - only three! This makes sense, right?

We have more than cartilage damage to worry about!

Connective Tissue of the Skeletal System

Connective tissues primary function in the skeletal system is to connect muscles to bone and to connect joints together.  This dense connective tissues is comprised of fibers called collagen.  Mature connective tissue has fewer cells than other tissues and needs less blood, oxygen, and other nutrients.  Each collagen bundle is comprised of several fibers, and these fibers contain fibrils.  The fibrils contain the actual collagen molecules.

Tendons

The Golgi Tendon Organ

Tendons connect muscles to bone and are an extension of the muscle fibers.  They are slightly more elastic than ligaments but cannot shorten as muscles do.  Within the muscles and tendons there is a built in sensory mechanisms called the Golgi tendon organ.  The Golgi tendon organ acts as a “safety valve” and provides feedback about the bodies position and protects the muscle and connective tissue.  This safety valve is called the feedback loop.  In essence, when tension becomes to great, greater than the brain can recall; the Golgi tendon organ’s signal inhibits the contraction stimulus and reduces the risk of injury.

Ligaments

Ligaments of the skeletal system connect bones to bones at a joint.  Ligaments contain collagen and also contain an elastic fiber called elastin.  Ligaments do have some elasticity to allow joint movement, but it is very limited.

Cartilage

Cartilage of the skeletal system is the firm, elastic, flexible, white material found at the ends of the ribs, between the vertebrae, at joint surfaces, and in the nose and ears.  Cartilage functions as both a shock absorber, and to provide structure.  Cartilage also functions as a lubricant in the working parts of a joint.  Unlike tendons and ligaments, cartilage has no blood supply of its own.  It receives oxygen and nutrients through diffusion.  Because of this, damage to cartilage takes a very long time to heal.

IT IS NOT IN YOUR HEAD...JUST YO CELLS.

Sickle cell disease may affect brain function in adults
who have few or mild complications of the inherited
blood disease, according to results of the first study to
examine cognitive functioning in adults with sickle cell
disease. The multicenter study, funded by the National
Heart, Lung, and Blood Institute (NHLBI), part of the
National Institutes of Health, compared brain function
scores and imaging tests in adult patients with few
sickle cell complications with results in similar adults
who did not have the blood disease.

Researchers report that the brain function scores in
sickle cell patients were, on average, in the normal
range. However, twice as many patients as healthy
adults (33 percent versus 15 percent) scored below
normal levels. Those who were more likely to score
lower were older and had the lowest levels of
hemoglobin, the protein in red blood cells that carries
oxygen in the blood, compared to sickle cell
participants who scored higher. Findings from brain
magnetic resonance imaging scans did not explain
differences in scores.

Researchers at 12 sites within the NHLBI-supported
Comprehensive Sickle Cell Centers conducted the
study. Their results are published in the May 12 issue of
the Journal of the American Medical Association. An
editorial accompanies the article.

"This study suggests that some adult patients who
have sickle cell disease may develop cognitive
problems, such as having difficulty organizing their
thoughts, making decisions, or learning, even if they do
not have severe complications such as stroke related to
sickle cell disease," said NHLBI Acting Director Susan
B. Shurin, M.D. "Such challenges can tremendously
affect a patient's quality of life, and we need to address
these concerns as part of an overall approach to
effectively managing sickle cell disease."

Researchers tested cognitive functioning of 149 adult
sickle cell disease patients (between the ages of 19 and
55) and compared them to 47 healthy study
participants of similar age and education levels from
the same communities. All of the participants were
African-American.

More sickle cell disease patients scored lower on
measures such as intellectual ability, short-term
memory, processing speed, and attention, than
participants in the healthy group. The sickle cell
disease participants did not have a history of end-organ failure, stroke, high blood pressure, or other
conditions that might otherwise affect brain function.

"We need to study whether existing therapies, such as
blood transfusions, can help maintain brain function, or
perhaps even reverse any loss of function," noted Elliott
P. Vichinsky, M.D., of the Children's Hospital &
Research Center Oakland, principal investigator of the
study and the lead author of the paper. "These effects
were found in patients who have clinically mild sickle
cell disease, which raises the question of whether
therapies should be given to all patients to help prevent
these problems from developing."

Researchers involved in this study are recruiting
patients with sickle cell disease into a clinical trial to
determine whether blood transfusions may help
preserve cognitive function. Participants will receive
transfusions every three or four weeks for six months
as part of the clinical study. Information about this
study can be found at www.clinicaltrials.gov, search for
NCT00850018.

Sickle cell disease affects about 70,000 Americans. At
one time, many children died from the disease, but new
therapies have enabled sickle cell disease patients to
live well into middle age or beyond. As more people
with sickle cell disease are living into adulthood, health
care providers are uncovering previously unrecognized
complications.

Studies of brain function in children who have sickle
cell disease have suggested that some children with the
disease, even if they have not suffered a stroke, have
experienced silent brain injury. Others without obvious
changes on brain scans may have some level of
cognitive dysfunction that seems to worsen with age.
Stroke is a common complication of sickle cell disease,
and can lead to learning disabilities, lasting brain
damage, long-term disability, paralysis, or death.

Sickle cell disease involves an altered gene that
produces abnormal hemoglobin. Red blood cells with
sickle hemoglobin that have too little oxygen become
C-shaped in addition to becoming stiff and sticky.
These crescent-shaped cells can clump to block blood
flow, causing severe pain and potential organ damage.
In the United States, the disease mainly affects those of
African descent, but it is also found in other ethnic
groups, including those of Hispanic and Middle Eastern
descent.

Sickle Cell and The Brain

 THIS INFO NEEDS TO BE IN THE HANDS OF PARENTS WHEN DISCUSSING WHY THE KIDS AND US ARE EXPERIENCING FORGETFULNESS/ MEMORY LOSS/ SHORT ATTENTION SPANS ETC...I THOUGHT I WAS GOING CRAZY BECAUSE I'M NOT SUPPOSED TO BE FORGETTING AT MY AGE. AND I QUESTIONED MYSELF ABOUT MY CHILD BEING RUDE OR IS HE GOING THROUGH WHAT I AM ‪#‎UFEELME‬?>>>> SICKLECELLAWARENESS:
HOW THIS ILLNESS AFFECTS OUR BRAIN

Sickle cell disease (SCD) is a blood disorder; however, the central nervous system (CNS) is one of the organs frequently affected by the disease. Brain disease can begin early in life and often leads to neurocognitive dysfunction. Approximately one-fourth to one-third of children with SCD have some form of CNS effects from the disease, which typically manifest as deficits in specific cognitive domains and academic difficulties. 
Patients who have sickle cell disease may
develop cognitive problems, such as
having difficulty organizing their thoughts,
making decisions, or learning, even if they
do not have severe complications such as
stroke related to sickle cell disease
We discuss SCD as a neurodevelopmental disorder by reviewing the mechanisms of neurological morbidity in SCD, the timing of these mechanisms, the types of cognitive and behavioral morbidity that is typical, and the interaction of social-environmental context with disease processes. The impact of the disease on families shares many features similar to other neurodevelopmental disorders; however, social-environmental factors related to low socioeconomic status, worry and concerns about social stigma, and recurrent, unpredictable medical complications can be sources of relatively higher stress in SCD. Greater public awareness of the neurocognitive effects of SCD and their impact on child outcomes is a critical step toward improved treatment, adaptation to illness, and quality of life.

BRAIN AND SICKLECELL AND CLOTS

The anterior communicating artery due to its location an aneurysm will go undetected because the person will be a systematic / has no symptoms at all.

Sickle cell disease may affect brain function in adults
who have few or mild complications of the inherited
blood disease, according to results of the first study to
examine cognitive functioning in adults with sickle cell
disease. The multicenter study, funded by the National
Heart, Lung, and Blood Institute (NHLBI), part of the
National Institutes of Health, compared brain function
scores and imaging tests in adult patients with few
sickle cell complications with results in similar adults
who did not have the blood disease.

Researchers report that the brain function scores in
sickle cell patients were, on average, in the normal
range. However, twice as many patients as healthy
adults (33 percent versus 15 percent) scored below
normal levels. Those who were more likely to score
lower were older and had the lowest levels of
hemoglobin, the protein in red blood cells that carries
oxygen in the blood, compared to sickle cell
participants who scored higher. Findings from brain
magnetic resonance imaging scans did not explain
differences in scores.

Researchers at 12 sites within the NHLBI-supported
Comprehensive Sickle Cell Centers conducted the
study. Their results are published in the May 12 issue of
the Journal of the American Medical Association. An
editorial accompanies the article.

"This study suggests that some adult patients who
have sickle cell disease may develop cognitive
problems, such as having difficulty organizing their
thoughts, making decisions, or learning, even if they do
not have severe complications such as stroke related to
sickle cell disease," said NHLBI Acting Director Susan
B. Shurin, M.D. "Such challenges can tremendously
affect a patient's quality of life, and we need to address
these concerns as part of an overall approach to
effectively managing sickle cell disease."

Researchers tested cognitive functioning of 149 adult
sickle cell disease patients (between the ages of 19 and
55) and compared them to 47 healthy study
participants of similar age and education levels from
the same communities. All of the participants were
African-American.

More sickle cell disease patients scored lower on
measures such as intellectual ability, short-term
memory, processing speed, and attention, than
participants in the healthy group. The sickle cell
disease participants did not have a history of end-organ failure, stroke, high blood pressure, or other
conditions that might otherwise affect brain function.

"We need to study whether existing therapies, such as
blood transfusions, can help maintain brain function, or
perhaps even reverse any loss of function," noted Elliott
P. Vichinsky, M.D., of the Children's Hospital &
Research Center Oakland, principal investigator of the
study and the lead author of the paper. "These effects
were found in patients who have clinically mild sickle
cell disease, which raises the question of whether
therapies should be given to all patients to help prevent
these problems from developing."

Researchers involved in this study are recruiting
patients with sickle cell disease into a clinical trial to
determine whether blood transfusions may help
preserve cognitive function. Participants will receive
transfusions every three or four weeks for six months
as part of the clinical study. I

LUNGS-Pulmonary Hypertension

Vasculopathy and pulmonary
hypertension in sickle cell disease:

Sickle cell disease (SCD) is an
autosomal recessive disorder in the
gene encoding the β-chain of
hemoglobin. Deoxygenation causes the
mutant hemoglobin S to polymerize,
resulting in rigid, adherent red blood
cells that are entrapped in the
microcirculation and hemolyze.
Cardinal features include severe painful
crises and episodic acute lung injury,
called acute chest syndrome. This
population, with age, develops chronic
organ injury, such as chronic kidney
disease and pulmonary hypertension. A
major risk factor for developing chronic
organ injury is hemolytic anemia, which
releases red blood cell contents into the
circulation. Cell free plasma
hemoglobin, heme, and arginase 1
disrupt endothelial function, drive
oxidative and inflammatory stress, and
have recently been referred to as
erythrocyte damage-associated
molecular pattern molecules (eDAMPs).
Studies suggest that in addition to
effects of cell free plasma hemoglobin
on scavenging nitric oxide (NO) and
generating reactive oxygen species
(ROS), heme released from plasma
hemoglobin can bind to the toll-like
receptor 4 to activate the innate
immune system. Persistent
intravascular hemolysis over decades
leads to chronic vasculopathy, with
∼10% of patients developing pulmonary
hypertension. Progressive obstruction
of small pulmonary arterioles, increase
in pulmonary vascular resistance,
decreased cardiac output, and eventual
right heart failure causes death in many
patients with this complication. This
review provides an overview of the
pathobiology of hemolysis-mediated
endothelial dysfunction and eDAMPs
and a summary of our present
understanding of diagnosis and
management of pulmonary
hypertension in sickle cell disease,
including a review of recent American
Thoracic Society (ATS) consensus
guidelines for risk stratification and
management.

SickleCell and joints

Joints the Skeletal System

A joint, or otherwise known as an articulation; is formed where two bones connect.  There are two major classifications of skeletal system joints: 1) synarthrodial joints, which is a union of two bones by fibrous tissues such that there is no joint cavity and almost no movement possible.  An example would be the skull, and 2) diarthrodial joints, which is a freely moving joint with an articular cavity (which holds the fluid inside the joint).

 

Joints of the Human Body

As a freely moving joint, the diarthrodial joint has an articular cavity encased in a ligamentous capsule, and synovial fluid lubricates the cartilage inside the capsule.  There are six categories of diarthrodial joints:

1) Arthrodial Joint (gliding): They comprise of two flat bone surfaces that press up against each other and allow a limited gliding movement.  They can be found in the wrist and the foot.

2) Ginglymus Joint (hinge): They provide a wide range of movement in one place. An example is the knee joint.

3) Condyloid Joint (ellipsoid): They provide movement in two planes without rotation.  An example is the wrist between the radius and the proximal row of carpal bones.

4) Enarthrodial Joint (multi-axial ball-and-socket): They permit movement in all planes.  An example is the hip joint.

5) Sellar Joint (saddle): This joint provides movement similar to ball-and-socket movement but without rotation. The thumb is the only saddle joint in the body.

6) Trochoidal Joint (pivot): This is a joint that moves by rotating.  One bone articulates just like the hinge on a door in such away that one bone rotates using the other as pivot. An example is the neck.

SickleCell and bones

Bones of the Skeletal System

Cross Section of a Human Bone

All of the 206 bones in our body consist of three layers: the bone marrow, compact bone, and the periosteum. Within the center section of our long bone is a central cavity which holds the bone marrow.  The are two different types of bone marrow: 1) red marrow produces platelets to assist in blood clotting, and red blood cells which fight against infection, and 2) yellow marrow which consists mostly of fat cells.  Surrounding the marrow is a dense rigid bone called the compact bone.  The ridge compact bone is honeycombed with thousands of tiny holes and passages that supply oxygen and nutrients to the bone.  This dense layer of compact bone supports the weight of the body and is mostly comprised of calcium and minerals.  Every bone is then covered by the periosteum which acts as the skin of the bone.  The inner layer of the periosteum contains cells the produce bone.

Our 206 bones of the skeletal system are divided into two categories: 1) the axial skeleton, which is comprised of our trunk and head, and 2) the appendicular skeleton, which is comprised of our arms and legs.  Our bones are further broken down into five main categories:

1) Flat Bones: Provide protection.  They include such bones as the ribs, sternum, and scapula.

2) Short Bones: Provide shock absorption.  They include such bones as the carpals, and tarsals.

3) Long Bones: They provide the structural support of our body.  They include such bones as the tibia, fibula, and ulna.

4) Sesamoid Bones: Provide protection and improve mechanical advantage of musculotendinous units (relating to or affecting both muscle and tendons).  They include such bones as the patella (knee cap).

5) Irregular Bones: Provide a variety of purposes throughout the body.  They include such bones as a vertebra.