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Pediatrics > Blood Disorders > Flashcards

Blood Disorders Flashcards

1
Q

What is Sickle Cell Disease?

A
  • group of inherited hemoglobinopathies in which RBC’s carry Hg55 instead of HgbAA
  • severe chronic blood disorder
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2
Q

What do RBC’s look like with sickle cell disease?

A

elongated with shortened life span, rigid and sickled shape

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3
Q

Complications of Sickle Cell Disease

A
  • 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
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4
Q

What occurs when the cells sickle?

A
  • significant anemia
  • blood becomes viscous
  • hypoxia followed by ischemia and results in infarction
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5
Q

Sickle Cell crisis can be triggered by what?

A

stress, trauma, or illness

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6
Q

Therapeutic Management of Sickle Cell

A
  • preventing sickling and infection
  • prophylactic antibiotics in children
  • administer appropriate immunizations
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7
Q

What antibiotic is given to children ages 2 months-5 years for Sickle Cell?

A

Penicillin VK

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8
Q

Nursing Management During Crisis

A
  • focus on pain control
  • oxygen administration
  • adequate hydration
  • monitor labs
  • treat underlying conditions
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9
Q

S/S of Sickle Cell

A
  • pallor
  • lesions/ulcers
  • jaundice of the skin
  • scleral icterus
  • decreased BP w/ severe anemia
  • increased BP w/ nephropathy
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10
Q

What to do when managing Pain Crisis for Sickle Cell

A
  • always believe report of pain
  • moderate/severe pain may require opioid meds
  • addiction to narcotics is rarely a concern
  • treat underlying infection/injury
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11
Q

Why should you be careful about using cooling mattresses for a child w/ Sickle Cell?

A

Sudden temp change could precipitate sickling

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12
Q

Hemophilia

A

x-linked recessive disorders that result in deficiency in one of the coagulation factors in the blood

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13
Q

How is hemophilia transmitted?

A

By carrier mothers to their sons, so usually only males are affected

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14
Q

What is the primary goal for Hemophilia?

A

preventing bleeding

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15
Q

What activities are ok to encourage for a child w/ Hemophilia?

A
  • swimming
  • running
  • tennis
  • baseball
  • basketball
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16
Q

What should be done if bleeding or injury occurs for a child w/ Hemophilia?

A

Factor administration is prescribed

17
Q

Factor is given to a child w/ Hemophilia before what?

A
  • surgery
  • IM injection
  • dental care
18
Q

What is the most common renal tumor and the 4th most common solid tumor in children?

A

Wilm’s Tumor

19
Q

What is the treatment of choice for Wilm’s Tumor?

A

Removal of tumor and kidney

20
Q

Why should you avoid palpating the abdomen of a child with a Wilm’s Tumor?

A

Wilm’s tumor is highly vascular and soft so excessive handling may result in tumor seeding and metastasis

21
Q

Neurofibromatosis

A

genetic disorders of the nervous system that primarily affect the development and growth of neural cell tissues

22
Q

What is the most common type of neurofibromatosis?

A

Type 1

23
Q

What is the hallmark sign of neurofibromatosis?

A

Multiple Café au Lait spots

24
Q

Diagnosis of Neurofibromatosis is made from 2 or more of what signs?

A
  • 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
25
Q

Turner Syndrome

A
  • common abnormality of sex chromosome
  • loss of all or part of the sex chromosome
  • females
  • no cure
26
Q

Girls w/ Turner Syndrome are more prone to what types of problems?

A
  • cardiovascular probs
  • kidney and thyroid probs
  • skeletal disorders
  • hearing/eye disturbances
  • learning disabilities
  • obesity
27
Q

S/S of Turner Syndrome

A
  • short-stature
  • webbed neck
  • low posterior hairline
  • wide-spaced nipples
  • amenorrhea
  • social skill difficulties
28
Q

What is the standard of care for Tuner’s Syndrome?

A

Growth hormone

29
Q

Nursing Management for Turner’s Syndrome

A
  • mainly supportive

- education for family-need to understand short stature and infertility are likely

Pediatrics (24 decks)

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