Emergency Signs

When to Call Emergency Services Immediately

Call emergency services right away if you experience any of the following:

• Sudden severe headache — possibly a ruptured intracranial aneurysm; often described as the "worst headache of your life."
• Severe flank or back pain with fever — possible cyst infection; may progress to sepsis if untreated.
• Gross hematuria with blood clots — significant cyst bleeding or rupture requiring evaluation.
• Anuria or severe oliguria — passing little or no urine; may indicate acute kidney injury or obstruction.
• Difficulty breathing — may signal fluid overload, pulmonary edema, or a cardiovascular event.
• Confusion or altered consciousness — may indicate severe hypertension, electrolyte disturbance, or intracranial bleeding.
• Severe vomiting or diarrhea with dehydration — can trigger acute kidney injury, especially in reduced kidney function.
• Sudden vision changes — may indicate severe hypertension or a vascular event.

Cyst Infection

Cyst infection is common in ADPKD. Delayed treatment can lead to sepsis. Prompt medical evaluation is essential; treatment typically requires antibiotics, and sometimes drainage. If you have fever with flank or back pain, contact your doctor promptly or go to the emergency department.

Cyst Bleeding / Rupture

Most cyst bleeding resolves on its own, but medical evaluation is needed to rule out other causes (such as stones or tumors). Heavy bleeding may cause hemoglobin to drop and require hospital observation. Seek medical care promptly if you see visible blood in your urine or experience sudden severe flank pain.

Intracranial Aneurysm Rupture

Approximately 8–10% of ADPKD patients have intracranial aneurysms; the rate is higher in those with a family history of aneurysm. A ruptured aneurysm is a life-threatening emergency — call emergency services immediately. Patients with a family history of aneurysm should discuss screening (MRA) with their doctor.

Acute Kidney Injury

Acute kidney injury (AKI) may result from obstruction (e.g. stones), severe infection, dehydration, or nephrotoxic medications (such as NSAIDs or contrast agents). Seek prompt medical care — delayed treatment can cause irreversible kidney damage. Always tell healthcare providers that you have ADPKD before any procedure involving contrast dye.

Severe Hypertension

A blood pressure of 180/120 mmHg or higher accompanied by symptoms is a hypertensive emergency requiring immediate medical attention. If your blood pressure is this high but you have no symptoms, contact your doctor promptly — this is a hypertensive urgency that still needs urgent evaluation.

What to Bring to the Emergency Department

If you need to go to the emergency department, bring the following if available:

• Recent lab reports — latest kidney function (creatinine, eGFR), blood counts, electrolytes, urinalysis.
• Medication list — all current medications, doses, and schedules, including tolvaptan, ACEI/ARB, and any supplements.
• Medical history — ADPKD diagnosis, known aneurysms, prior surgeries, allergies, and family history.
• Imaging reports — recent kidney ultrasound, CT, or MRI reports, and any MRA results if you have been screened for aneurysms.

What NOT to Do

• Do not self-medicate — never take new medications, especially NSAIDs or herbal remedies, without medical advice during an emergency.
• Do not stop prescribed medications without medical direction — stopping blood pressure medications or tolvaptan abruptly can be dangerous; follow your doctor's guidance.
• Do not force fluids if oliguric — if you are passing little or no urine, drinking large amounts of fluid can cause dangerous fluid overload; follow medical advice.