Organ/system involvement | Adjunctive/symptomatic therapy and preventative measures |
---|---|
Renal | |
Proteinuria and reno-protection | ACE inhibitor or ARB |
Dietary salt restriction | |
Statin therapy | |
Vitamin D replacement therapy if needed | |
Renal failure | Dialysis or kidney transplantation (donor screened negative for FD) |
Cardiac | |
Hypertension | ACEI or ARB (beta blockers should be used with caution and amiodarone avoided in patients receiving ERTa) |
Symptomatic bradycardia/chronotropic incompetence or significant AV conduction impairment | Permanent cardiac pacing |
Atrial fibrillation | Lifetime anticoagulation with maintenance of sinus rhythm |
Malignant arrhythmias | Implantable cardioverter-defibrillator |
Neurologic | |
Stroke prophylaxis | Antithrombotic agents (aspirin or clopidogrel) as secondary prevention; no data available regarding primary prevention |
Anticoagulants (warfarin or the new anticoagulant drugs in absence of kidney failure), when needed, e.g., patients with atrial fibrillation | |
Neuropathic pain management | First-line agents include anticonvulsants (e.g., carbamazepine, gabapentin, pregabalin); other drugs can be considered according to current international recommendations for neuropathic pain |
Pain crises | Opioid agonists (care needed to avoid worsening GI disturbances) |
Avoiding pain triggers | Lifestyle modifications (e.g., avoid temperature extremes, maintain proper hydration, use air conditioning, cooling vests, facial mist/spray) |
Psychiatric | |
Depression, anxiety | Psychiatric referral, pain center referral, serotonin reuptake inhibitors |
Gastrointestinal | |
Delayed gastric emptying | Metoclopramide |
Dyspepsia | H-2 blockers |
Dysmotility and diarrhea | Dietary changes (increased fiber intake, more frequent and smaller meals) and pharmacotherapy |
Pulmonary | |
Airway obstruction | Bronchodilators |
Ophthalmological | |
Difficulty in driving at night | Polarized glasses |
Artificial tears ointment | |
ENT | |
Hearing loss | Hearing aids, cochlear implants |
Vertigo-related nausea | Trimethobenzamide, prochlorperazine |
Dermatological | |
Angiokeratomas | Laser/cosmetic treatment (not proven effective) |
Lymphedema | Compression stockings |