EDS COMPLICATIONS AND TREATMENT CONSIDERATIONS
Joint preservation strategies. Although very difficult as this older man can attest, wear and tear osteoarthritis from joint-tissue flexibility and fragility is best combatted by “wise” exercise that chooses joint-protective activities like swimming, cycling, and low-weight-lifting over jogging. Remaining active is crucial with supine (recumbent bicycles, floor routines) or aquatic exercise best tolerated by those with severe dizziness and/or skeletal pain. Physical therapists, massage, and appropriately gentle chiropractors are helpful in guiding build-up of core-strength and protective muscles around joints (see DNA discussion describing the many muscle-related gene changes that are being found in EDS),4,5 It is essential to minimize cycles of inactivity that can exacerbate POTS, chronic fatigue or fibromyalgia, shown by the many EDS patients who first seek medical attention after being bedridden from illness, trauma, or surgery. Bracing susceptible joints--waist bands for back/sacroiliac constraint, shoe/arch supports for feet—plus strategies to help blood return (support hose) are helpful, and weight gain that is understandable in those with pain and fatigue is a major contributor to joint wear-and-tear. The RICE (Rest, Ice, Compression, Elevation) approach to injury can minimize ongoing joint damage, and attention to joint pain (growing pains usually occur at night) or injury/skeletal deformity with prompt orthopedic referral can optimize function.
Nutrition strategies. Low bowel motility/IBS is benefitted by smaller, more frequent meals, lower dairy/gluten, enzyme supplements like BetaGest or Vitazyme, and probiotics. Dietary and gastroenterology consultation should be considered since hiatal hernia, food intolerance similar to celiac disorder, and inflammation provoking gastritis, esophagitis, or Crohn diagnoses can occur. Supplements including vitamins (D at 2000 units, B12 at 1 milligram, C at 1 gram daily in addition to B complex) will offset frequent malabsorption and others may be needed to rectify low iron, ferritin, thyroid levels (post Hashimoto thyroiditis) that are common in EDS. Some find chondroitin-glucosamine preparations (e. g., Osteobiflex) and magnesium to be helpful among many anecdotal remedies for osteoarthritis. The chronic fatigue that can occur from IBS is greatly exacerbated by POTS, managed first by hydration (8-10 glasses of fluid per day), salt (absent hypertension), and high protein (to maximize intravascular volume and brain circulation). Mast cell activation ensures many dietary intolerances that are periodic and poorly identified by skin or antibody testing.
Common medication and subspecialty therapies. Cardiology evaluation with electro- and echocardiograms are essential for EDS, those like Drs. Suleman (adults >17) and Pearse (peds) in DFW with expertise in POTS being ideal. Mitral and other valvular regurgitations are common, aortic dilation or bicuspid aortic valve occurring in EDS as well as Marfan syndrome, and dilated veins may be coagulated or coiled (e. g., Dr. Chan of Precision Vasculature in DFW) to improve upper body blood return. Screening of all arteries for aneurysms is not out of place, though only 4 of my 1656 EDS clinic patients and 0 of 9 patients having vascular EDS-associated collagen 3 gene mutations had one.5 Impingement of lax ligaments on vessels as with median arcuate ligament or nutcracker syndromes can occur, and tilt-table testing can quantify POTS treatment including beta-blockers (Corlenar), Midodrine, and Clonidine at night. Antihistamine protocols (Zyrtec, Zantac, Singulair, Cromolyn sodium, etc.) can treat MCAD as coordinated by allergists (e. g., Dr. Aasia Ghazi in DFW) and atypical responses to medicines (not always predictable by pharmacogenetics) should be expected.
Surgery and delivery complications. These atypical medication responses include frequent needs for higher dental and general anesthetic doses that along with bleeding tendencies, suture dehiscence, and excessive scarring can complicate surgeries. When mothers and offspring (often at 50% risk) have EDS then the friable connective tissue in fetal membranes can cause premature labor. While fragile vessels can lead to post-op/postpartum hemorrhage I remember offhand only 3 of nearly 1800 (clinic and online EDS patients) with life-threatening surgery/pregnancy complications (one of many statistics that I need to quantify). Unless concerns about rare and severe diseases like vascular EDS or Loeys-Dietz have been raised, then surgeries and pregnancies in EDS should have good prognoses if anesthetic sufficiency, sutural care, and post-procedure monitoring of bleeding are ensured.
Orthopedics. Negative outcomes have followed surgeries for joint “tightening” or replacement, so conservative management by orthopedists familiar with EDS (e. g., Dr. Humeniuk in DFW) is important along with knowledge of EDS complications like plica band, avascular regions from injury, or deformations from gravitational and other forces on lax tissue (kyphosis, pectus, scoliosis, disc herniation/degeneration, toeing-in, flat foot). Injections and steroids should be used with caution because they can augment the lower bone density, compression, and stress fractures that are common in EDS.
Neurosurgery, neurology. Recurrent daily headaches including exacerbating of pain with sneezing or abdominal pressure can indicate increased intracranial pressure caused by Chiari deformation (acquired in EDS and different from the congenital Chiari malformation occurs in spina bifida). Upright head MRI with cervical extension-flexion studies are needed to recognize the low-lying or herniated cerebellar tonsils associated with Chairi and the craniocervical instability that along with cervical disc changes can cause severe occipital and neck pain in EDS. This is one of many areas where websites like EDS awareness—www.chronicpainpartners.com, www.ehlers-danlos.com, www.ednf.org, local EDS sites (e. g., Dallas, Houston, Austin) can direct patients to national experts familiar with EDS (e.g., neurosurgeon Dr. Greenfield in New York). Experience with EDS is important because neurosurgery may cause cerebrospinal fluid leaks from fragile dura, the low intracranial pressure being as devastating as high (pseudotumor cerebri, empty sella) from Chiari. Migraines often occur, sometimes diagnosed as occipital neuralgia, many EDS patients experiencing several types of headaches even in the absence of Chiari. Neurologists may collaborate in therapy for headaches and the frequent neuropathies (carpal tunnel, thoracic outlet syndromes) that occur, able to document frequent small fiber neuropathy if an EDS-dysautonomia (AAD) diagnosis is in doubt. The many EDS patients (>150) I am discovering with mitochondrial DNA changes or mutations formerly associated with muscle disease (myopathy)5 may justify more invasive tests like muscle biopsy, but none have chosen this procedure so far.
Rheumatology and pain management. Rheumatologists (e. g., Drs. Chowdhry, Dao, Rezazadeh, Syeda in DFW) are often the first to suspect EDS and are helpful in separating autoimmune from osteoarthritis by documenting autoimmune (ANA, anti-phospolipid, Sjogren antibodies) or susceptibility markers (HLA B27 with ankylosing spondylitis) that may warrant a trial of immunosuppressive medicines like Humira. Although 10% of my EDS patients have one of more of these markers, their predominant wear-and-tear osteoarthritis rarely responds to immunosuppressive medicines. Joint pain and instability are the most refractory problems in EDS and the depression without prior history experienced by a national pain expert during a year of back pain2 should be heeded by anyone caring for these patients. Fibromyalgia is a frequent symptomatic diagnosis because most complain of muscle tenderness, spasms, and aches (“whole body pain”) that in my personal experience are difficult to separate from arthritis and tendonitis. Ibuprofen and other NSAIDS are effective for joint pain but problematic in people with IBS. Celebrex and Flexeril are mentioned by many, but all medicines require cautious introduction and titration due to the intolerances and atypical responses mentioned above. It is important to remember that NSAIDS are more effective than narcotics in treating pain although the latter can lessen perceived distress, a likely factor in addiction.
Miscellaneous. Many patients have dry and itchy eyes, treated with lubricating and anti-allergic drops. They often have dry/scaly skin, transient rashes, eczema, and hives, the former perhaps related to frequent FLG gene mutations5 in EDS that were first associated with ichthyosis vulgaris (common fish-like skin), the latter three to mast cell activation. Most can manage with lotions and primary care for the atopic symptoms; need for dermatology has been rare. Bladder issues include interstitial cystitis, frequency, incontinence, and frequent urinary infections, and gynecology visits for discussion of dyspareunia and birth control regulation of menorrhagia with eventual endometriosis should be encouraged. Pelvic prolapse can occur with age and/or multiparity and vulvodynia can be severe in some women. Conventional medications for depression and anxiety can be helpful, especially if these disorders are recognized as secondary and the many medical therapies described above are also instituted.
Nutrition strategies. Low bowel motility/IBS is benefitted by smaller, more frequent meals, lower dairy/gluten, enzyme supplements like BetaGest or Vitazyme, and probiotics. Dietary and gastroenterology consultation should be considered since hiatal hernia, food intolerance similar to celiac disorder, and inflammation provoking gastritis, esophagitis, or Crohn diagnoses can occur. Supplements including vitamins (D at 2000 units, B12 at 1 milligram, C at 1 gram daily in addition to B complex) will offset frequent malabsorption and others may be needed to rectify low iron, ferritin, thyroid levels (post Hashimoto thyroiditis) that are common in EDS. Some find chondroitin-glucosamine preparations (e. g., Osteobiflex) and magnesium to be helpful among many anecdotal remedies for osteoarthritis. The chronic fatigue that can occur from IBS is greatly exacerbated by POTS, managed first by hydration (8-10 glasses of fluid per day), salt (absent hypertension), and high protein (to maximize intravascular volume and brain circulation). Mast cell activation ensures many dietary intolerances that are periodic and poorly identified by skin or antibody testing.
Common medication and subspecialty therapies. Cardiology evaluation with electro- and echocardiograms are essential for EDS, those like Drs. Suleman (adults >17) and Pearse (peds) in DFW with expertise in POTS being ideal. Mitral and other valvular regurgitations are common, aortic dilation or bicuspid aortic valve occurring in EDS as well as Marfan syndrome, and dilated veins may be coagulated or coiled (e. g., Dr. Chan of Precision Vasculature in DFW) to improve upper body blood return. Screening of all arteries for aneurysms is not out of place, though only 4 of my 1656 EDS clinic patients and 0 of 9 patients having vascular EDS-associated collagen 3 gene mutations had one.5 Impingement of lax ligaments on vessels as with median arcuate ligament or nutcracker syndromes can occur, and tilt-table testing can quantify POTS treatment including beta-blockers (Corlenar), Midodrine, and Clonidine at night. Antihistamine protocols (Zyrtec, Zantac, Singulair, Cromolyn sodium, etc.) can treat MCAD as coordinated by allergists (e. g., Dr. Aasia Ghazi in DFW) and atypical responses to medicines (not always predictable by pharmacogenetics) should be expected.
Surgery and delivery complications. These atypical medication responses include frequent needs for higher dental and general anesthetic doses that along with bleeding tendencies, suture dehiscence, and excessive scarring can complicate surgeries. When mothers and offspring (often at 50% risk) have EDS then the friable connective tissue in fetal membranes can cause premature labor. While fragile vessels can lead to post-op/postpartum hemorrhage I remember offhand only 3 of nearly 1800 (clinic and online EDS patients) with life-threatening surgery/pregnancy complications (one of many statistics that I need to quantify). Unless concerns about rare and severe diseases like vascular EDS or Loeys-Dietz have been raised, then surgeries and pregnancies in EDS should have good prognoses if anesthetic sufficiency, sutural care, and post-procedure monitoring of bleeding are ensured.
Orthopedics. Negative outcomes have followed surgeries for joint “tightening” or replacement, so conservative management by orthopedists familiar with EDS (e. g., Dr. Humeniuk in DFW) is important along with knowledge of EDS complications like plica band, avascular regions from injury, or deformations from gravitational and other forces on lax tissue (kyphosis, pectus, scoliosis, disc herniation/degeneration, toeing-in, flat foot). Injections and steroids should be used with caution because they can augment the lower bone density, compression, and stress fractures that are common in EDS.
Neurosurgery, neurology. Recurrent daily headaches including exacerbating of pain with sneezing or abdominal pressure can indicate increased intracranial pressure caused by Chiari deformation (acquired in EDS and different from the congenital Chiari malformation occurs in spina bifida). Upright head MRI with cervical extension-flexion studies are needed to recognize the low-lying or herniated cerebellar tonsils associated with Chairi and the craniocervical instability that along with cervical disc changes can cause severe occipital and neck pain in EDS. This is one of many areas where websites like EDS awareness—www.chronicpainpartners.com, www.ehlers-danlos.com, www.ednf.org, local EDS sites (e. g., Dallas, Houston, Austin) can direct patients to national experts familiar with EDS (e.g., neurosurgeon Dr. Greenfield in New York). Experience with EDS is important because neurosurgery may cause cerebrospinal fluid leaks from fragile dura, the low intracranial pressure being as devastating as high (pseudotumor cerebri, empty sella) from Chiari. Migraines often occur, sometimes diagnosed as occipital neuralgia, many EDS patients experiencing several types of headaches even in the absence of Chiari. Neurologists may collaborate in therapy for headaches and the frequent neuropathies (carpal tunnel, thoracic outlet syndromes) that occur, able to document frequent small fiber neuropathy if an EDS-dysautonomia (AAD) diagnosis is in doubt. The many EDS patients (>150) I am discovering with mitochondrial DNA changes or mutations formerly associated with muscle disease (myopathy)5 may justify more invasive tests like muscle biopsy, but none have chosen this procedure so far.
Rheumatology and pain management. Rheumatologists (e. g., Drs. Chowdhry, Dao, Rezazadeh, Syeda in DFW) are often the first to suspect EDS and are helpful in separating autoimmune from osteoarthritis by documenting autoimmune (ANA, anti-phospolipid, Sjogren antibodies) or susceptibility markers (HLA B27 with ankylosing spondylitis) that may warrant a trial of immunosuppressive medicines like Humira. Although 10% of my EDS patients have one of more of these markers, their predominant wear-and-tear osteoarthritis rarely responds to immunosuppressive medicines. Joint pain and instability are the most refractory problems in EDS and the depression without prior history experienced by a national pain expert during a year of back pain2 should be heeded by anyone caring for these patients. Fibromyalgia is a frequent symptomatic diagnosis because most complain of muscle tenderness, spasms, and aches (“whole body pain”) that in my personal experience are difficult to separate from arthritis and tendonitis. Ibuprofen and other NSAIDS are effective for joint pain but problematic in people with IBS. Celebrex and Flexeril are mentioned by many, but all medicines require cautious introduction and titration due to the intolerances and atypical responses mentioned above. It is important to remember that NSAIDS are more effective than narcotics in treating pain although the latter can lessen perceived distress, a likely factor in addiction.
Miscellaneous. Many patients have dry and itchy eyes, treated with lubricating and anti-allergic drops. They often have dry/scaly skin, transient rashes, eczema, and hives, the former perhaps related to frequent FLG gene mutations5 in EDS that were first associated with ichthyosis vulgaris (common fish-like skin), the latter three to mast cell activation. Most can manage with lotions and primary care for the atopic symptoms; need for dermatology has been rare. Bladder issues include interstitial cystitis, frequency, incontinence, and frequent urinary infections, and gynecology visits for discussion of dyspareunia and birth control regulation of menorrhagia with eventual endometriosis should be encouraged. Pelvic prolapse can occur with age and/or multiparity and vulvodynia can be severe in some women. Conventional medications for depression and anxiety can be helpful, especially if these disorders are recognized as secondary and the many medical therapies described above are also instituted.