Blood Disorders Flashcards
What is Sickle Cell Disease?
- group of inherited hemoglobinopathies in which RBC’s carry Hg55 instead of HgbAA
- severe chronic blood disorder
What do RBC’s look like with sickle cell disease?
elongated with shortened life span, rigid and sickled shape
Complications of Sickle Cell Disease
- pain crisis
- stroke
- sepsis
- acute chest syndrome
- splenic sequestration
- reduced visual acuity
- chronic leg ulcers
- cholestasis and gall stones
- delayed growth and development
- delayed puberty
- priapism
What occurs when the cells sickle?
- significant anemia
- blood becomes viscous
- hypoxia followed by ischemia and results in infarction
Sickle Cell crisis can be triggered by what?
stress, trauma, or illness
Therapeutic Management of Sickle Cell
- preventing sickling and infection
- prophylactic antibiotics in children
- administer appropriate immunizations
What antibiotic is given to children ages 2 months-5 years for Sickle Cell?
Penicillin VK
Nursing Management During Crisis
- focus on pain control
- oxygen administration
- adequate hydration
- monitor labs
- treat underlying conditions
S/S of Sickle Cell
- pallor
- lesions/ulcers
- jaundice of the skin
- scleral icterus
- decreased BP w/ severe anemia
- increased BP w/ nephropathy
What to do when managing Pain Crisis for Sickle Cell
- always believe report of pain
- moderate/severe pain may require opioid meds
- addiction to narcotics is rarely a concern
- treat underlying infection/injury
Why should you be careful about using cooling mattresses for a child w/ Sickle Cell?
Sudden temp change could precipitate sickling
Hemophilia
x-linked recessive disorders that result in deficiency in one of the coagulation factors in the blood
How is hemophilia transmitted?
By carrier mothers to their sons, so usually only males are affected
What is the primary goal for Hemophilia?
preventing bleeding
What activities are ok to encourage for a child w/ Hemophilia?
- swimming
- running
- tennis
- baseball
- basketball
What should be done if bleeding or injury occurs for a child w/ Hemophilia?
Factor administration is prescribed
Factor is given to a child w/ Hemophilia before what?
- surgery
- IM injection
- dental care
What is the most common renal tumor and the 4th most common solid tumor in children?
Wilm’s Tumor
What is the treatment of choice for Wilm’s Tumor?
Removal of tumor and kidney
Why should you avoid palpating the abdomen of a child with a Wilm’s Tumor?
Wilm’s tumor is highly vascular and soft so excessive handling may result in tumor seeding and metastasis
Neurofibromatosis
genetic disorders of the nervous system that primarily affect the development and growth of neural cell tissues
What is the most common type of neurofibromatosis?
Type 1
What is the hallmark sign of neurofibromatosis?
Multiple Café au Lait spots
Diagnosis of Neurofibromatosis is made from 2 or more of what signs?
- 6 or more café au lait spots
- 2 or more neurofibromas
- freckling in armpit/groin
- optic glioma
- 2 or more growths on iris
- abnormal development of spine, temple bone of the skull, or the tibia
- 1st degree relative w/ NFM
Turner Syndrome
- common abnormality of sex chromosome
- loss of all or part of the sex chromosome
- females
- no cure
Girls w/ Turner Syndrome are more prone to what types of problems?
- cardiovascular probs
- kidney and thyroid probs
- skeletal disorders
- hearing/eye disturbances
- learning disabilities
- obesity
S/S of Turner Syndrome
- short-stature
- webbed neck
- low posterior hairline
- wide-spaced nipples
- amenorrhea
- social skill difficulties
What is the standard of care for Tuner’s Syndrome?
Growth hormone
Nursing Management for Turner’s Syndrome
- mainly supportive
- education for family-need to understand short stature and infertility are likely
