-
Pathophysiology (Adults)
- -More common in whites than any other races
- -Men and Women have equal chances of inheriting
-Inherited disorder in which fluid-filled cysts develop in the nephrons
-Kidney function is replaced with non-functioning cysts that look like clusters of grapes
-Kidneys become very large, possibly 2 to 3 times its nomal size which can displace other organs causing pain
-Fluid filled cysts are at an increased risk for infection, rupture, and bleeding
**Control of blood pressure is TOP priority to prevent further kidney damage
-
Key Features Include:
(Adults)
- -Abdominal or flank pain
- -Hypertension
- -Nocturia (peeing at night)
- -Increased abdominal girth
- -Constipation
- -Bloody or cloudy urine (infection)
- -Bright-red cola colored urine (cyst ruptures)
- -Kidney stones
*Pain is often the first manifestation
-
Uremic Problems:
-Urine biproducts in blood
- -Anorexia
- -Nausea and vomiting
- -Pruritis
- -Fatigue
-
Assessment: Patient History (Adults)
- -Parents or others in family w/ PKD?
- -Constipation?
- -Abdominal discomfort?
- -Change in urine color or frequency?
- -High blood pressure?
- -Headaches?
- -Family hx from a sudden death of stroke?
-
Assessment: Physical/Clinical Manifestations (Adult)
- -Pain is often the FIRST manifestation
- -Distended abdomen is common
- -Dull aching pain caused by increased kidney size or by infection of a cyst
- -Sharp intermittent pain occurs when a cyst ruptures
- -Bright red or cola colored urine= cyst ruptured
- -Cloudy or foul smelling urine w/ dysuria--> infection suspected
- -Nocturia is an early manifestation and occurs due to decreased urine concentrating ability
- -As renal function declines theres increased HTN, edema, uremic problems (anorexia, N/V, pruritis, and fatigue)
- -Berry Aneurysms
-
Intracranial Verry Aneurysm
-AKA saccular aneurysm
- -Sac like outpouching in a cerebral blood vessel
- -Once a berry aneurysm has formed it is very likely to rupture- causing a stroke
- -SERIOUS MEDICAL EMERGENCY, and should be treated ASAP
-
Assessment: Psychosocial (Adults)
- -Has seen this happen to other family members
- -Parent may have died with this disease
- -May be concerned for their children also
-
Assessment: Diagnostic (Adults)
- -UA shows proteinuria once the glomeruli are involved
- -Hematuria may be gross or microscopic
- -As kidney function declines: Serum creatine and BUN rise BUT creatinine clearance decreases
- -Changes in kidney handling of sodium may cause either sodium losses or sodium retention
-Diagnostic Tests: renal sonography, CT, MRI
-
Interventions: Acute and Chronic Pain (Adults)
- -Drug therapy and complementary approaches
- -NSAIDS used cautiously because of the tendency to decrease renal blood flow
- -Aspirin containing compounds are avoided due to risk of bleeding
- -If pain is from infection antibiotics are prescribed
- -*Bactrim, Septra, Trimpex or Cipro are prescribed because they enter the cyst wall
- -Monitor serum creatinine because antibiotics can be nephrotoxic
- -Apply dry heat to abdomin or flank area
- -If pain is severe cysts can be reduced by needle aspiration and drainage
- -Teach relaxation and comfort for using deep breathing, guided imagery, etc.
OVERALL GOAL: patient self management
-
Interventions: Constipation (Adult)
- -Teach to maintain adequate fluid intake
- -Increasing dietary fiber when fluid intake is greater than 2500 mL/24 hr
- -Exercise regularly
- -may need to advise about the use of laxatives and bulk agents including careful use of laxatives to prevent chronic constipation
- -Bowel management might change, particularly if renal failure also develops
-
Interventions: Hypertension and Renal Failure (Adult)
- -Restrict sodium to help control BP, may need antihypertensive and antidiuretic drugs
- -Teach to measure and record BP
- -Teach to monitor daily weights
- -Low sodium diet is often prescribed however some pts may have salt wasting and should not follow a sodium restricted diet
- -Protein may be restrictued to slow the development of kidney failure
- -Work with the nutritionalist and help the patient to understand the importance of diet planning
|
|