Editing Ehlers–Danlos syndrome (section)

== Management ==
No cure for Ehlers–Danlos syndrome is known, and treatment is supportive. Close monitoring of the cardiovascular system, physiotherapy, occupational therapy, and orthopedic instruments (e.g., wheelchairs, bracing, casting) may be helpful. This can help stabilize the joints and prevent injury. Orthopedic instruments are helpful for the prevention of further joint damage, especially for long distances. People should avoid activities that cause the joint to lock or overextend.<ref>{{cite web|url=https://www.ehlers-danlos.org/what-is-eds/information-on-eds/physiotherapy-and-self-management/|title=Physiotherapy and self-management – The Ehlers–Danlos Support UK|website=www.ehlers-danlos.org|access-date=2018-04-17}}</ref>

A physician may prescribe casting to stabilize joints. Physicians may refer a person to an orthotist for [[orthotics|orthotic treatment]] (bracing). Physicians may also consult a physical and/or occupational therapist to help strengthen muscles and teach people how to properly use and preserve their joints.<ref name=Rombaut11>{{cite journal | vauthors = Rombaut L, Malfait F, De Wandele I, Cools A, Thijs Y, De Paepe A, Calders P | title = Medication, surgery, and physiotherapy among patients with the hypermobility type of Ehlers–Danlos syndrome | journal = Archives of Physical Medicine and Rehabilitation | volume = 92 | issue = 7 | pages = 1106–1112 | date = July 2011 | pmid = 21636074 | doi = 10.1016/j.apmr.2011.01.016 | url = https://www.academia.edu/20158514}}</ref><ref name="wristpr">{{ cite journal |vauthors=Woerdeman LA, Ritt MJ, Meijer B, Maas M |url= https://www.academia.edu/24725432 |title= Wrist problems in patients with Ehlers–Danlos syndrome|journal=European Journal of Plastic Surgery |volume=23 |issue=4 |pages=208–210 |year=2000 | doi=10.1007/s002380050252|s2cid= 29979747}}</ref>

[[Aquatic therapy]] promotes muscular development and coordination.<ref>{{cite journal | vauthors = Callewaert B, Malfait F, Loeys B, De Paepe A | title = Ehlers–Danlos syndromes and Marfan syndrome | journal = Best Practice & Research. Clinical Rheumatology | volume = 22 | issue = 1 | pages = 165–189 | date = March 2008 | pmid = 18328988 | doi = 10.1016/j.berh.2007.12.005| url = https://biblio.ugent.be/publication/432358/file/6811097 }}</ref> With manual therapy, the joint is gently mobilized within the range of motion and/or manipulations.<ref name=Rombaut11/><ref name="wristpr"/> If conservative therapy is not helpful, surgical joint repair may be necessary. Medication to decrease pain or manage cardiac, digestive, or other related conditions may be prescribed. To decrease [[Bruise|bruising]] and improve wound healing, some people have responded to [[vitamin C]].<ref>{{EMedicine|article|943567|Genetics of Ehlers–Danlos Syndrome|treatment}}</ref> Medical care workers often take special precautions because of the sheer number of complications that tend to arise in people with EDS. In vascular EDS, signs of chest or abdominal pain are considered trauma situations.<ref>{{Cite news|url=https://www.ehlers-danlos.com/emergency/|title=Vascular (VEDS) Emergency Information |work=The Ehlers Danlos Society|access-date=2018-04-17}}</ref>

[[Cannabinoid]]s and medical marijuana have shown some efficacy in reducing pain levels.<ref>{{Cite journal|url=https://tcapp.org/wp-content/uploads/2017/09/Pain-Management-in-Patients-with-Hypermobility-Syndrome.pdf|title=Topics in Pain Management: pain management in patients with hypermobility disorders: frequently missed causes of chronic pain|journal=Topics in Pain Management}}</ref>

In general, medical intervention is limited to symptomatic therapy. Before pregnancy, people with EDS may be recommended to have genetic counseling and to familiarize themselves with the risks pregnancy poses. Children with EDS should be given information about the disorder so they can understand why they should avoid contact sports and other physically stressful activities. Children should be taught that they should not demonstrate the unusual positions they can maintain due to loose joints, as this may cause early degeneration of the joints. Emotional support along with behavioral and psychological therapy can be useful. Support groups can be immensely helpful for people dealing with major lifestyle changes and poor health. Family members, teachers, and friends should be informed about EDS so they can accept and assist the child.<ref>{{Cite journal| vauthors = Giroux CM, Corkett JK, Carter LM |date=2016|title=The Academic and Psychosocial Impacts of Ehlers–Danlos Syndrome on Postsecondary Students: An Integrative Review of the Literature.|url=https://files.eric.ed.gov/fulltext/EJ1133767.pdf |journal=Journal of Postsecondary Education and Disability |volume=29|issue=4|page=414}}</ref>

=== Pain management ===
Successful treatment of chronic pain in EDS requires a multidisciplinary team. The ways to manage pain can be to modify pain management techniques used in the normal population. Pain is classified into several types. One is [[nociceptive]], which is caused by an injury sustained to tissues. Another is [[neuropathic pain]], caused by abnormal signals by the nervous system. In many cases, the pain individuals experience is an unequal mix of the two. [[Physiotherapy]] (exercise rehabilitation) can be helpful, especially in stabilizing the core and the joints. Stretching exercises must be reduced to slow and gentle stretching to reduce the risks of dislocations or subluxations. Usable methods may include posture reeducation, muscle release, joint mobilization, trunk stabilization, and manual therapy for overworked muscles. [[Cognitive behavioural therapy]] is used in many chronic pain patients, especially those who have severe, chronic, life-controlling pain that is unresponsive to treatment. It has not been checked for efficiency in clinical trials. The state of pain management with EDS is considered insufficient.<ref name="Chopra_2017" />

==== Medications ====
Nonsteroidal anti-inflammatory drugs ([[Nonsteroidal anti-inflammatory drug|NSAIDs]]) may help if the pain is caused by inflammation. However, long-term use of NSAIDs is often a risk factor for gastrointestinal, renal, and blood-related side effects. It can worsen symptoms of [[mast cell activation syndrome]], a disease that may be associated with EDS. [[Acetaminophen]] can be used to avoid the bleeding-related side effects of NSAIDs.<ref name="Chopra_2017" />

[[Lidocaine]] can be applied topically after subluxations and painful gums. It can also be injected into painful areas in the case of musculoskeletal pain.<ref name="Chopra_2017" />

If the pain is neuropathic in origin, [[tricyclic antidepressant]]s in low doses, [[anticonvulsant]]s, and [[selective norepinephrine reuptake inhibitor]]s can be used.<ref name="Chopra_2017" />

====Dislocation and subluxation management====
When a dislocation or subluxation does occur, muscle spasms and stress tend to occur, increasing pain and reducing the chances of the dislocation/subluxation naturally relieving. Methods to support a joint after such an incident include the usage of a sling to hold the joint in place and allow it to relax. Orthopedic casts are not advised as there could be pain if unrelaxed muscles are still trying to spasm out against the cast. Other solutions to promote relaxation are heat, gentle massaging, and mental distractions.<ref>{{Cite web |title=Dislocation/Subluxation Management |url=https://www.ehlers-danlos.com/dislocation-subluxation-management/ |access-date=2022-04-19 |website=The Ehlers Danlos Society}}</ref>

=== Surgery ===
The instability of joints, leading to subluxations and joint pain, often requires surgical intervention in people with EDS. Instability of almost all joints can happen, but appears most often in the lower and upper extremities, with the wrist, fingers, shoulder, knee, hip, and ankle being most common.<ref name=Rombaut11/>

Common surgical procedures are joint [[debridement]], tendon replacements, capsulorrhaphy, and [[arthroplasty]]. [[Aortic dissection]] and other such complications of [[Vascular Ehlers-Danlos syndrome|vascular EDS]] will mandate surgical intervention. After surgery, the degree of stabilization, pain reduction, and people's satisfaction can improve, but surgery does not guarantee an optimal result; affected people and surgeons report being dissatisfied with the results. The consensus is that conservative treatment is more effective than surgery,<ref name=Camerota14/> particularly since people have extra risks of surgical complications due to the disease. Three basic surgical problems arise due to EDS – the strength of the tissues is decreased, which makes the tissue less suitable for surgery; the fragility of the blood vessels can cause problems during surgery; and wound healing is often delayed or incomplete.<ref name=Rombaut11/> If considering surgical intervention, seeking care from a surgeon with extensive knowledge and experience in treating people with EDS and joint hypermobility issues would be prudent.<ref name="Recommendations for anesthesia and">{{cite journal | vauthors = Wiesmann T, Castori M, Malfait F, Wulf H | title = Recommendations for anesthesia and perioperative management in patients with Ehlers–Danlos syndrome(s) | journal = Orphanet Journal of Rare Diseases | volume = 9 | page = 109 | date = July 2014 | pmid = 25053156 | pmc = 4223622 | doi = 10.1186/s13023-014-0109-5 | doi-access = free}}</ref>

Local anesthetics, [[arterial catheter]]s, and [[central venous catheters]] cause a higher risk of [[haematoma|bruise formation]] in people with EDS. Some people with EDS also show a resistance to local anaesthetics.<ref>{{cite journal | vauthors = Parapia LA, Jackson C | s2cid = 7809153 | title = Ehlers–Danlos syndrome – a historical review | journal = British Journal of Haematology | volume = 141 | issue = 1 | pages = 32–35 | date = April 2008 | pmid = 18324963 | doi = 10.1111/j.1365-2141.2008.06994.x | doi-access = free}} {{free access}}</ref> Resistance to [[lidocaine]] and [[bupivacaine]] is not uncommon, and [[mepivacaine]] tends to work better in people with EDS. Special recommendations for anesthesia are given for people with EDS.{{Citation needed|date=February 2023}} Detailed recommendations for anesthesia and perioperative care of people with EDS should be used to improve safety.<ref name="Recommendations for anesthesia and"/>

Surgery in people with EDS requires careful tissue handling and a longer immobilization afterward.<ref>{{cite journal | vauthors = Shirley ED, Demaio M, Bodurtha J | title = Ehlers–danlos syndrome in orthopaedics: etiology, diagnosis, and treatment implications | journal = Sports Health | volume = 4 | issue = 5 | pages = 394–403 | date = September 2012 | pmid = 23016112 | pmc = 3435946 | doi = 10.1177/1941738112452385}}</ref>