Lecture 13 Flashcards
What is Hemophilia?
X-Linked Recessive
DisorderImpaired 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 (in reference to Hemophilia?
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
Osteophytes
Changes may restrict ROM permanently
Muscle weakness is usually found around affected joints.
See Campbell Ch E-3
How is Hemophilia treated?
Minimizing joint trauma and hemorrhages
Proactive muscle strengthening
Promote cardiovascular fitness in recreational activities
Elastic pressure bandages and ice for muscle hemorrhages
Immobilization, gentle ROM for joint hemorrhages
Adapt normal developmental tasks and challenges
Medications
Frequent hospitalizations
Alterations in family relationshipsPhysiological 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? Symptoms
Body makes sickle-shaped red blood cells. “Sickle-shaped” means that the red blood cells are shaped like a “C.” Clumps of sickle cells block blood flow in the blood vessels that lead to the limbs and organs.
Pain
Serious infections
Organ damage
Sickle cells are destroyed rapidly in the body of people with the disease causing anemia, jaundice and the formation of gallstones.
acute chest syndrome
stroke
Priapism (painful prolonged erection).
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?
Health maintenance
Early diagnosis, preferably in the newborn period Penicillin prophylaxis
Vaccination against pneumococcus bacteria
Folic acid supplementation
Treatment of complications: Antibiotics Pain management Intravenous fluids Blood transfusion Surgery 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 symptom 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
Food/oral aversions
Swallowing problems
Gagging
Choking
Apena
Respiratory problems - pneumonia, bronchitis, wheeing, asthma, night-time cough, aspiration
Poor weight gain
Weight loss,
Failure to thrive
Neck aching (Sandifer’s Syndrome)See PPT for all symptoms
What is Tracheosophageal fistula (TEF)?
An embryological malformation - a congenital or acquired communication between the trachea and esophagus.
TEFs often lead to severe and fatal pulmonary complications.
What is the incidence rate of TEF?
incidence of 1 case in 2000-4000 live births(bolded in the PPT. Good to know)
When are patients with TEF diagnosed?
Immediately following birth or infancy, usually in the neonatal period.
TEFs are often associated with life-threatening complications.
Infants in 3rd world countries die without the surgery—Survival rates of 100% can be achieved in infants who do not have severe associated congenital anomalies.
What is an Omphalocele?
Birth defect in which the infant’s intestine or other abdominal organs stick out of the belly button (navel).
It is a type of hernia
Intestines are covered only by a thin layer of tissue and can be easily seen.
An omphalocele can be clearly seen, because the abdominal contents stick out (protrude) through the belly button area. Sizes vary - different organs may stick out as a result
How are Omphaloceles treated?
Omphaloceles are repaired with surgery, although not always immediately!!! A sac protects the abdominal contents and allows time for other more serious problems (such as heart defects) to be dealt with first, if necessary.
To fix an omphalocele, the sac is covered with a special man-made material, which is then stitched in place.
Slowly, over time, the abdominal contents are pushed into the abdomen.When the omphalocele can comfortably fit within the abdominal cavity, the man-made material is removed and the abdomen is closed.
What is the cause of an Omphalocele?
An omphalocele develops as a baby grows inside the mother’s womb. The muscles in the abdominal wall (umbilical ring) do not close properly. As a result, the intestine remains outside the umbilical cord.
What % of babies with Omphalocele also have other birth defects?
Approximately 25 - 40% of infants with an omphalocele have other birth defects.
How is Omphalocele diagnosed?
Prenatal ultrasounds often identify infants with an omphalocele before birth. Otherwise, a physical examination of the infant is enough for your health care provider to diagnose this condition. Testing is usually not necessary.
What is the prognosis for the baby after surgery for Omphalocele?
Complete recovery, unless other birth defects exist.