Ehlers-Danlos Syndrome (EDS) is a group of inherited disorders primarily affecting connective tissue, which includes collagen. This tissue provides support and structure to the skin, joints, blood vessels, and internal organs. Abnormal collagen production leads to tissue fragility, joint hypermobility, and systemic involvement affecting nearly every organ system. Due to this complexity and tissue vulnerability, medication choices require extreme caution and individualized assessment to avoid compounding risks.
Medications Affecting Pain and Tissue Integrity
Non-Steroidal Anti-Inflammatory Drugs (NSAIDs), such as ibuprofen and naproxen, pose specific risks for individuals with EDS. These medications interfere with platelet function, increasing the risk of bleeding and bruising, which is already a concern due to fragile blood vessels. Furthermore, NSAIDs can cause gastrointestinal irritation and ulceration, which is problematic since many individuals with EDS already experience significant gastrointestinal issues.
Systemic corticosteroids require extreme caution, especially when used long-term or in high doses. Corticosteroids negatively affect collagen synthesis and metabolism, further compromising already fragile connective tissue. This effect can impair wound healing and worsen tissue integrity, which is a significant concern for EDS patients.
Localized procedures, including joint or trigger point injections, require careful consideration due to the increased risk of complications. Fragile tissues and vessels increase the risk of hematoma formation or nerve damage at the injection site. Poor wound healing and tissue fragility mean that even minor interventions must be meticulously planned to minimize trauma.
Fluoroquinolone antibiotics, such as ciprofloxacin and levofloxacin, are associated with severe adverse effects in people with connective tissue disorders. The FDA has warned that this class of antibiotics is associated with aortic aneurysm and dissection. These drugs weaken connective tissues, potentially leading to tendon rupture and life-threatening vascular events, making their use discouraged in the EDS population.
Special Precautions for Anesthesia and Sedation
EDS presents unique challenges in surgical and diagnostic settings requiring anesthesia and sedation. A notable phenomenon is “local anesthetic resistance,” often reported in those with hypermobile EDS (hEDS). This may be due to abnormal collagen structure affecting anesthetic diffusion, meaning higher doses or alternative techniques like nerve blocks may be required for adequate pain control.
General anesthesia carries heightened risks, especially concerning airway management during intubation. Cervical spine instability, common in hEDS, makes the neck vulnerable during positioning. The fragile nature of the trachea and laryngeal tissues means that smaller endotracheal tubes or specialized techniques, such as fiberoptic intubation, may be necessary to reduce the risk of tissue tearing or trauma.
Procedural measures, such as careful patient positioning, are paramount during surgery. Hyperflexible joints are prone to subluxation or dislocation when the patient is unconscious and muscle tone is relaxed. Operating teams must pad and secure joints to prevent nerve compression or injury from awkward positioning. Additionally, the use of blood pressure cuffs and intravenous line insertion requires gentleness due to the risk of bruising and fragile blood vessels.
Navigating Medications for Autonomic Dysfunction
Autonomic dysfunction, often manifesting as Postural Orthostatic Tachycardia Syndrome (POTS), is a common comorbidity in EDS. This condition involves autonomic nervous system malfunction, leading to issues with blood pressure (BP) and heart rate (HR) regulation. Medications that significantly affect these parameters must be titrated with precision.
Drugs that lower blood pressure, including certain antidepressants, alpha-blockers, or some beta-blockers, can exacerbate orthostatic hypotension or lightheadedness. While beta-blockers are sometimes used to manage the rapid heart rate of POTS, they must be used cautiously as they may drop the already low blood pressure. Stimulants, occasionally used for fatigue, may also worsen tachycardia or anxiety, requiring careful monitoring.
Management of dysautonomia often involves medications intended to increase blood volume or cause vasoconstriction. Vasoconstrictors (e.g., midodrine) and volume expanders (e.g., fludrocortisone) treat orthostatic intolerance, but dosage requires careful balance to avoid excessive BP spikes. Conversely, medications that cause significant vasodilation, such as nitrates, may be poorly tolerated as they can worsen blood pooling and orthostatic symptoms.
Medications that cause significant fluid loss, particularly diuretics, should be used cautiously unless specifically prescribed. Chronic dehydration and low blood volume often contribute to dysautonomia symptoms in EDS. Diuretics can deplete the body’s limited fluid reserves, potentially worsening symptoms like dizziness, fatigue, and lightheadedness.