Lecture 13 Flashcards
What is Hemophilia?
X-Linked Recessive Disorder
Impaired clotting capability
Pain
Reduced ROM and Strength in affected joints (bleeding occurs)
Hinge joints (knee, elbow, ankle) most typically affected
What is the prevalence of joint contracture in patients with severe form of hemophilia?
Between 50% and 95%
What are contractures a result of?
Recurrent hemarthroses and intramuscular bleeding episodes. Most frequently involve knee flexion, elbow flexion, ankle plantar flexion.
Are recurrent intramuscular hemorrhages common?
No, they are rare. A single, large hematoma can cause permanent joint contractue because the large volume of blood will cause localized necrosis.
As blood as reabsorbed, dead muscle fibers are replaced by fibrous tissue in a shortened position. This occurs in about 10% of all muscle hemorrhages and is most common in the plantar flexor and forearm muscles (multiple hemorrages with frequent immobilition can also result in muscle shortening around a joint)
As hemarthroses recur, what else might occur in the body?
Synovial hypertrophy and eventual articular cartilage destruction, bony changes (such as genu valgus), posterior subluxation of the tibia, and osteophytes.
Changes may restrict ROM permanently
Muscle weakness is usually found around affected joints.
See Campbell Ch E-3
How is Hemophilia treated?
i. Minimizing joint trauma and hemorrhages
ii. Proactive muscle strengthening
iii. Promote cardiovascular fitness in recreational activities
iv. Elastic pressure bandages and ice for muscle hemorrhages
v. Immobilization, gentle ROM for joint hemorrhages
vi. Adapt normal developmental tasks and challenges
vii. Medications
viii. Frequent hospitalizations
ix. Alterations in family relationships
Physiological aspects of the disease itself, medications and other forms of treatment, frequent hospitalizations, disruptions in daily activities alterations in family relationships are some of the potential factors that may restrict the normal development in chronically ill children!!
What is sickle cell anemia?
a serious disease in which the body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a “C.”
Normal red blood cells are disc-shaped and look like doughnuts without holes in the center. They move easily through your blood vessels. Red blood cells contain the protein hemoglobin
The clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs. Blocked blood vessels can cause pain, serious infections, and organ damage.
Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones.
The sickle cells also block the flow of blood through vessels resulting in lung tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen), stroke and priapism (painful prolonged erection).
It also causes damage to most organs including the spleen, kidneys and liver. Damage to the spleen makes sickle cell disease patients, especially young children, easily overwhelmed by certain bacterial infections i.e. they get very sick more easily; infection control measures are even more important
How is Sickle Cell Anemia treated?
i. Health maintenance!!
1. Early diagnosis, preferably in the newborn period
2. Penicillin prophylaxis
3. Vaccination against pneumococcus bacteria
4. Folic acid supplementation
ii. Treatment of complications!!
1. Antibiotics, pain management, intravenous fluids, blood transfusion, and surgery.
2. All backed by psychosocial support
3. Varied episodes of care
4. Best managed in a comprehensive multi-disciplinary program of care
5. Blood transfusions
a. help benefit sickle cell disease patients by reducing recurrent pain crises, risk of stroke and other complications
6. Eliminate iron overload
a. Because red blood cells contain iron, and there is no natural way for the body to eliminate it, patients who receive repeated blood transfusions can accumulate iron in the body until it reaches toxic levels. It is important to remove excess iron from the body, because it can gather in the heart, liver, and other organs and may lead to organ damage.
b. Treatments are available to eliminate iron overload
What kind of cancer makes up one third of childhood cancers?
Leukemia
What is the most common type of Leukemia?
ALL - Acute Lymphoblastic Leukemia
Children with this diagnosis are at an increased risk for Leukemia.
What is Down Syndrome?
What accounts for half of childhood cancers? What are the causes?
Leukemias and cancer of the brain and CNS account for half of childhood cancers. Causes are generally unknown
How was childhood cancers treated?
IMMUNOTOXINS THAT TARGET CANCER CELL WALLS! Also, surgery, radiation, chemotherapy, and Immunotherapy to improve immune system
What are common symptoms of Gastroesophageal Reflux (GERD)?
- Constant or sudden crying – or colic-like symptoms
- Irritability and pain
- Poor sleep habits typically with frequent waking
- Arching their necks and back during or after eating
- Spitting-up or vomiting
- Wet burp or frequent hiccups
- Frequent ear infections or sinus congestion
What are the key symptoms indicative of GERD?
Refusing food or accepting only a few bites despite being hungry – or the exact opposite requiring constant small meals or liquid
Food/oral aversions
Swallowing problems, gagging, choking
Apena
Respiratory problems - pneumonia, bronchitis, wheeing, asthma, night-time cough, aspiration
Poor weight gain, weight loss, or failure to thrive
Neck aching (Sandifer’s Syndrome)
See PPT for all symptoms
