Pain and discomfort that persists for an extended period of time are characteristic signs of Ehlers-Danlos syndrome. The condition usually involves joints, muscles, and nerves of the body. A significant amount of it can also cause stomach pain and headaches if eaten in excessive doses.
Before recently, the name Ehlers-Danlos Syndrome (EDS) was linked with a lack of significance and urgency in the field of chronic pain. However, this has since changed. Patients presenting to pain specialists across the United States are being diagnosed with EDS at an increasing rate, which is becoming more visible. According to the National Institutes of Health in the United States, one in every 10,000 to one in every 15,000 persons globally suffers from this disease. All pain practitioners need to be informed of the features and clinical symptoms of this illness so that they may be prepared to recognize, accurately diagnose and treat patients who are suffering from this condition.
Recognizing EDS Pain
EDS is the most prevalent hereditary connective tissue disorder that produces intractable pain and needs the use of high-dose opioid treatment (i.e., double-jointed). This disorder results in joint degeneration and degeneration, as well as degeneration and degradation of connective tissue in the eye, spine, gums and internal organs, teeth, and central nervous system (CNS).
It is unusual for patients who suffer from the most severe type of this genetic disease to live longer than fifty years after being diagnosed. Typically, patients reach a point where their connective tissue begins to fail in its ability and responsibility to remain linked to and support organs such as tendons, ligaments, cartilage, spinal cord, brain, eye, teeth, and peripheral nerves, all of which are vital to their survival. Excruciating micro-tears that form when the connective tissue deteriorates cause the organs to be damaged, resulting in severe anguish that may be felt from head to toe throughout the body. The consequence is unremitting anguish, which leads to a severe disability, to the point where people are restricted to their beds or sofas for the rest of their lives.
To get to this point, the assistance of a caregiver will be necessary. Furthermore, the connective tissue has commonly deteriorated to the point that it has damaged the patient’s overall health to the point where a catastrophic, life-threatening event may occur. For example, in patients with EDS, spinal cord tethering, adhesive arachnoiditis, cerebellar herniation, aortic or uterine rupture, cerebral vascular rupture, and visceroptosis are all predicted catastrophic consequences.
It has become more apparent that some patients who initially appear with a diagnosis other than EDS as the source of their symptoms may have had their diagnoses incorrectly diagnosed in the first place. Consequently, individuals are not obtaining the most appropriate and required pain management treatment. Many EDS patients may not even be aware that they are suffering from a disease that is giving them so much discomfort. All pain practitioners should become familiar with the signs and symptoms of EDS and be prepared to screen for the disease in their patients.
Fact: EDS demands the use of severe, high-dose pain medication.
Pain management that is aggressive and humanistic is the most effective therapy option for symptomatic patients.
Neck pain in EDS
Even the most complex cases of Ehlers-Danlos syndrome-related craniocervical instability provide a significant challenge. If you don’t get an appropriate diagnosis right away, it might make things more difficult. An incorrect diagnosis might drive patients on a years-long search for assistance that they are unable to obtain because they and their physicians are pursuing the wrong condition.
Dislocations and subluxations caused by laxity of the ligaments and capsular tissue lead to neck pain
The pathophysiological cause of peripheral neuropathy in Ehlers-Danlos syndrome is still unknown. In addition to dislocations and subluxations caused by ligament and capsular laxity, hypermobility has been linked to aberrant stretching of peripheral nerves or pressure on peripheral nerves, which can result in neuropathy/plexopathy or neck discomfort.
Fusion surgery to correct neck pain
Fusion surgery is the best possible solution to cure EDS-affected neck pain. Numerous individuals have been advised to have cervical fusion surgery performed. They are on the lookout for alternate solutions. The pain and symptoms of some patients who have previously had the operation, including those linked to adjacent segment illness above and below the fusion sites, are causing them to seek alternatives to an enlarged or revision fusion surgery. Cervical fusion surgery can be a highly effective procedure for specific individuals. Those who oppose it believe that cervical fusion is a method of correcting issues and associated symptoms and conditions and that replacing it was a lesser concern for the time being than the loss of future neck motion.
Use of high-dose opioid therapy to reduce EDS pain
Patients suffer from intractable pain due to a gradual concentrated breakdown in connective tissue, which leads to becoming unable to perform any normal function as a result of the breakdown. In the vast majority of instances, the desire for analgesia grows as well.
The quickest and most basic initial screening approach is to have the patient answer a brief questionnaire online. Upon receiving a favorable response from the patient to these questions, the next step would be to calculate the patient’s Beighton hypermobility score. Consider the following scenario: the score suggests that an EDS diagnosis is likely. Finally, the International Consortium for Ehlers-Danlos Syndrome and Related Disorders developed diagnostic criteria for Ehlers-Danlos Syndrome and Related Disorders, which were recently revised.
The patient must be in chronic pain and have a positive pain scale score, according to our observations, in order for these requirements to be satisfied 100% of the time.
In addition to the fact that more and more encouraging data shows that high-dose opioid medicine coupled with extra hormones (as needed on an individual basis) may be an effective approach to pain control, there are other compelling reasons to screen for and appropriately diagnose EDS. According to studies, hormone treatments appear to both delay and repair connective tissue degeneration and regeneration. Several hormones have shown promise in the last several years, including human growth hormone, human chorionic gonadotropin, oxytocin, and nandrolone, to mention a few examples.
Additionally, patients can be directed to various exercises and physical supports that have been designed particularly for EDS and that are usually made available through EDS support groups and websites, as well as through other means.
The use of medicine in treating pain for many people with Ehlers-Danlos syndrome can be a significant component of their overall pain management strategy.
People who suffer from chronic pain may find it necessary to use over-the-counter pain relievers such as acetaminophen (Tylenol) or ibuprofen daily to regulate their symptoms (Advil, Motrin). Prescription pain medicines may be necessary for the event of acute injuries.
If you are having other symptoms, your doctor may recommend that you take additional drugs. People suffering from vEDS, for example, may require blood pressure-lowering medications in order to reduce the risk of blood vessel ruptures in their legs.
EDS pain can be relieved by surgery.
In some cases, EDS surgery may be required in order to do the following:
- Heal joint damage that has occurred.
- Reconstruct blood arteries that have ruptured.
- Make a contribution to the overall joint stabilization
- Reduce the amount of pressure that is placed on the nerves.
The skin issues produced by Ehlers-Danlos disease, such as poor healing, make surgery a risky proposition, but cosmetic procedures are quite safe.
Necessary changes in lifestyle to cope with EDS pain
The following are some strategies that people with Ehlers-Danlos syndrome may find helpful in managing their pain symptoms:
- Prevent heavy lifting, contact sports, and other activities that impose an excessive amount of strain on the joints or increase the chance of injury
- When engaging in sports, wearing a protective helmet and supporting braces is recommended.
- Experiment with moderate workouts such as swimming, Pilates, yoga, and other activities to discover whether they are beneficial to you.
- It is vital to get adequate sleep and establish a good sleep schedule in order to avoid feeling tired.
- Avoiding hard meals and chewing gum, both of which enhance the chance of sustaining a jaw injury, is recommended.
- It’s important to take breaks during dental procedures to keep the jaw from being overworked.
You should avoid playing a wind or brass instruments because they may raise the risk of a collapsed lung, which is particularly dangerous in people with vascular EDS.
Support to manage pain in EDS
Living with EDS comes with its own set of challenges, and those who are impacted by the illness can benefit from the assistance of others in overcoming these challenges.
Individuals suffering from Ehlers-Danlos syndrome can seek help from a variety of sources, including the following organizations:
- A physiotherapist (physical therapist)
- Members of the family
- A co-worker
- A business owner or a teacher
- Any medical professionals
- A therapist
- Participants in face-to-face support groups
- A genetic counselor
- Members of an online support group
Patients with EDS have various degrees of discomfort, determined by the kind of Ehlers-Danlos syndrome they have and the intensity of the symptoms they are experiencing.
Even though Ehlers-Danlos syndrome can cause unpleasant symptoms, lifestyle changes and medical treatments can give relief, enhance the quality of life, and reduce the chance of damage or consequences from the condition.