Hematologic Disorders 5

Question 1

Patient blood management develops an individualized plan for each person to minimize the need for transfusion

3 principles

Answer 1

• manage anemia
• minimize blood loss
• optimize hemostasis

Question 2

Why does pt blood management need to be developed to minimize the need for transfusion?

Answer 2

• Techniques acceptable for religious groups (Jehovah’s Witnesses)
• New infectious agents possible
• Risks associated with transfusions

Question 3

What needs to be done for pts who have had transfusions in the acute care, surgical, and post-op settings? (PT)

Answer 3

• Monitor lab values when treating patients

- Adjust treatment and intensity accordingly

Question 4

disorders of iron absorption =

Answer 4

hemochromatosis

Question 5

What type of disorder is hemochromatosis?

Answer 5

autosomal recessive hereditary disorder

Question 6

What causes hemochromatosis?

Answer 6

mutations in genes of any of the proteins that regulate the entry of iron into th eblood

Question 7

hemochromatosis severity depends upon:

Answer 7

variable, but thought to have an environmental component

• alcohol intake
• coexistence of Hep B/C

Question 8

What does hemochromatosis result in (liver + iron)

Answer 8

Excessive absorption of iron from small intestine

Question 9

hemochromatosis: sx onset

Answer 9

• present at birth

- onset of sx usually between 40-60 y.o.

Question 10

hemochromatosis: prevalence

Answer 10

• Equal between men and women

- Men experience sx 5-10x more often than women

Question 11

What are some of the sx associated with hemochromatosis?

Answer 11

• weakness
• chronic fatigue
• myalgia
• joint pain
• abnormal bronzing of the skin

Question 12

What is used to diagnose hemochromatosis?

Answer 12

• blood tests

- liver biopsy

Question 13

Medical tx for hemochromatosis (therapeutic phlebotomy)

Answer 13

Therapeutic phlebotomy until iron stores are at normal level

Question 14

initial tx for hemochromatosis (amts)

Answer 14

• may be 1 pint

- 1-2x per week

Question 15

maintenance therapy for hemochromatosis (amts)

Answer 15

• 1 pint

- every 2-4 months for life

Question 16

What can hemochromatosis lead to if left untreated?

Answer 16

• hepatomegaly
• pancreatic damage
• DM
• destructive arthritis
• liver failure
• cardiac myopathy
• CHF
• arrhythmias
• impotence in men
• decreased libido and sterility in women

Question 17

What is a major indicator with a pt who has hemochromatosis that distinguishes is from OA?

Answer 17

pt may report twinges of pain when flexing small joints of hands

ARTHROPATHY

Question 18

Arthropathy is present in ______% of cases of hemochromatosis

Answer 18

40-60%

Question 19

joint destruction with hemochromatosis

Answer 19

likely irreversible

Question 20

For pts who have hemochromatosis, what do PT interventions seek to address?

Answer 20

• flexibility
• strength
• assistive devices
• orthoses
• fall prevention