polycystic kidney disease

Question 1

What is PKD?

Answer 1

Hereditary, genetic kidney disorder –> fluid filled cysts develop in the nephrons

Cysts damage the nephron, reducing kidney function and causing hypertension. Kidney failure can occur over time.

Cysts are at increased risk for infection, rupture, and bleeding. Contribute to kidney stone formation.

Question 2

Most common form of PKD
Cysts begin to multiply at age 30

Answer 2

Autosomal dominant PKD

Question 3

More severe form of PKD
Cysts present at birth, w/ death typically occurring early in childhood

Answer 3

Autosomal recessive PKD

Question 4

Sx of PKD

Answer 4

Flank or abd pain
Distended abd
Enlarged, tender kidney on palpation
Changes in urine- hematuria, clarity, odor
Changes in pattern of urination
Dysuria
Vital signs- fever and hypertension require intervention
Edema
NV
Pruritus
Fatigue
Emotional responses

Question 5

Risk factors for PKD

Answer 5

Caucasian
Family hx of PKD

Question 6

Labs &
Diagnostics

Answer 6

• Urinalysis with findings of proteinuria and hematuria
• Urine culture and sensitivity if infection is suspected
• Serum creatinine and BUN to assess kidney function, Creatinine Clearance
• Renal sonography, CT scan, or MRI to assess the presence and size of cysts

Question 7

Meds

Answer 7

• Analgesics for comfort; use NSAIDs cautiously.
• Administer antibiotics such as trimethoprim/sulfamethoxazole TMP-SMX (Bactrim, Septra) or ciprofloxacin (Cipro) if a cyst infection is causing discomfort
• Nitrofurantoin
• Fosfomycin

Question 8

Procedures

Answer 8

• Needle aspiration of cysts

Question 9

Complications & Nursing Care

Answer 9

• HTN control & Fluid management (Major priority!!)
  o Antihypertensive agents, including ACE inhibitors, vasodilators, beta-blockers, and calcium channel blockers, as ordered
  o Diuretics, as ordered, to eliminate fluid overload
  o Daily weight to detect fluid-related weight gain
• Pain management
  o Apply dry heat to the abdomen or flank.
  o Teach relaxation or distraction techniques to self-manage pain and discomfort
• Infection prevention
• Constipation prevention - associated with fluid restriction and intestinal tract displacement from cysts with fiber intake and regular activity
• Diet therapy with dietitian consult to slow progression of kidney injury with fluid, sodium, and protein restrictions
• Provide counseling, support, and teaching about health maintenance to promote self-management