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EDS is a collection of hereditary diseases that damage the body’s connective tissues. The severity of the condition can vary significantly, damaging the skin, blood vessels, and joints.
Fact: EDS treatment depends upon the type and symptoms of the condition
There are six major kinds of this illness (out of total 13), which are distinguished by their symptoms as well as the genes that are affected:
- Arthrochalasia: The disease known as arthrochalasia affects patients with congenital hip dislocation, a distinctive form of the illness.
- Classical: it is associated with organ fragility.
- Dermatosparaxis: This disease is an uncommon skin fragility characterized by significant bruising and extreme skin fragility.
- Hypermobility: This is the most prevalent kind of disorder, in which patients have loose joints and persistent joint pain. This is the most severe variety of the ailment.
- Kyphoscoliosis: In this case, the patient’s spine is affected by a rare kind of disease called kyphoscoliosis.
- Vascular type: It is possible to have a severe case of the disease, which can cause the walls of blood vessels, intestines, and the uterus to burst.
Typical Symptoms (Generally of all types)
The symptoms of EDS might vary depending on the type and severity of the disease. A patient may suffer some of the following general symptoms as a result of their condition:
- Joints that are too flexible
- Excessively elastic skin
- Brittle skin
- Lumps of fatty tissue around the knees or elbows
In many cases, patients with vascular EDS exhibit physically distinguishing features that are exclusive to the disease. These may include the following:
- Thin nose
- Thin upper lip
- Too small earlobes
- Prominent eyes
- Transparent skin
Vascular EDS can cause the weakening of the blood vessels in the body, increasing the risk of rupturing the aorta, which is the major artery that transports oxygen-rich blood from the heart to the rest of the body.
Causes and Risk Factors
Depending on the patient’s EDS, a genetic mutation might develop in one of eight distinct genes responsible for the condition. According to research, people with EDS have a 50 percent risk of passing the disease on to their offspring.
Several types of Ehlers-Danlos syndrome are hereditary, implying that a faulty gene can be passed down from one generation to the next through the parents. When it comes to Ehlers-Danlos syndrome, the kid’s kind will be the same as their parents.
National Organization for Rare Disorders estimates that the likelihood of an affected parent passing on the mutation to their child is either 25 percent or 50%, depending on whether the mutation is transmitted by dominant or recessive gene inheritance, respectively. There are many ways in which the different subtypes of EDS are descended from other parents.
In other cases, the abnormal gene may not have been handed down from either of the parents, as is the point in this case. An example of a probable cause of this disease is genetic alterations in the egg or sperm that are not passed down from one generation to the next.
Diagnosis
The diagnosis of EDS is generally made based on the patient’s symptoms, family medical history, and genetic tests. In addition, the physician may do a physical examination to establish whether the patient is experiencing any other symptoms linked with EDS.
The aorta or other blood vessels may be seen and assessed using imaging tests such as an MRI, CT scan, or chest X-ray if the disease has caused damage to the aorta or blood vessels in general. Angiograms may be performed if further information about the heart and its arteries is required. This treatment creates a map of the heart and the blood vessels around it by injecting a specific dye into the blood vessels, which causes the vessels to appear more clearly on X-rays. The procedure is performed under general anesthesia.
General EDS Treatment
Because EDS is a hereditary disease, there is currently no treatment available. Reduced stress on the aorta and other arteries will be the primary focus of treatment, as will the management of skeletal and joint problems and the relief of any pain connected with them.
The most frequent types of medication therapy include those used to treat pain, high blood pressure, and excessive cholesterol levels, among other things. This may consist of beta-blockers, which decrease the heartbeat and relax the cardiac muscle, allowing the heart to function more efficiently. Angiotensin II receptor blockers have been another type of medication that can be used to decrease blood pressure.
The current treatment options for EDS are primarily geared toward symptom management, which includes medication, surgery, and physical therapy, among other things. EDS is a connective tissue disease, and symptoms such as discomfort can be handled with over-the-counter pain relievers (acetaminophen, ibuprofen, and naproxen sodium), but weak joints and frequent dislocations are frequently controlled by surgery and physical therapy.
Physiotherapy
Physiotherapy can assist in the strengthening of muscles and the stabilization of joints. Physical activities can help to alleviate discomfort and minimize the likelihood of dislocations. Pain can be relieved by using heat or cold, massages, ultrasound, electrical stimulation, and acupuncture, among other treatment methods.
Use of Analgesics to Relieve Pain
Pain medication should be prescribed according to the individual’s symptoms. Medications can be administered as needed to patients experiencing mild to moderate pain. Patients who suffer from severe and persistent pain are more likely to benefit from a regular medication dosage.
Patients with EDS are frequently prescribed nonsteroidal anti-inflammatory medications (NSAIDs), tricyclic antidepressants, and serotonin/norepinephrine receptor inhibitors (SNRIs). Muscular relaxants and magnesium are also effective in the treatment of muscle spasms. Opioids may be given in the case of extreme pain.
Your doctor may prescribe other drugs if you are experiencing additional symptoms. People with vEDS, for example, may require blood pressure-lowering medicines to minimize the risk of burst blood vessels in their legs.
Surgery
The necessity for orthopedic surgery may be required to restore joints that repeated dislocations have injured. Given the increased risk of surgical complications, including severe bleeding, abrupt rips in blood vessels, and issues with wound healing in EDS patients, surgery is only suggested after physiotherapy, and the use of braces have failed to give significant relief.
Lifestyle and Home Remedies
It is necessary to take precautions to avoid Ehlers-Danlos syndrome from becoming a problem. Some essential steps you should take to keep yourself safe are listed below.
- Make well-informed judgments when it comes to athletic endeavors. Walking, swimming, Tai Chi, recreational biking, and the use of an elliptical machine or a stationary cycle are all suggested forms of exercise, as are other forms of physical activity. Exercise caution if you are participating in sports that involve contact, weightlifting, or any other activity that raises your risk of injury. Make your hips, knees, and ankles less stressed by reducing the amount of tension you put on them.
- Take a break from chewing for a few minutes. Gum chewing, hard rolls, and ice should all be avoided if you want to keep your jaw joint healthy. Throughout your dental operation, you should close your mouth for a few minutes at intervals.
- Put on a pair of shoes that are both comfortable and supportive. The use of laced boots with adequate arch support can help to reduce the likelihood of ankle sprains occurring.
- Improve the quality of your sleep by exercising regularly. Body pillows and super-dense foam mattresses can be utilized to provide additional support and cushioning for hurting joints. The ability to sleep on your side may also be useful.
Treatments to Keep Bone Density
Supplementing with calcium and vitamin D can aid in the development of stronger bones. Weight-bearing activities also assist in the maintenance of mineral bone density and muscle tone, in addition to other benefits. A physiotherapist can advise patients on the safe and appropriate exercises for them to perform.
Treatments for Gastrointestinal Issues
Propon-pump inhibitors (PPIs) and H2-blockers are medications that can be used to treat gastroenteritis (inflammation of the lining of the stomach) and gastroesophageal reflux disease (GERD).
Irritable bowel syndrome is treated with dietary changes or, if necessary, with antidiarrheals, laxatives, and antispasmodics to relieve the symptoms.
Cardiovascular Disease Treatments
Sodium supplementation and water consumption to increase the blood volume and electrolyte pills can treat low blood pressure. In certain situations, beta-blockers or other medicines may be required in addition to standard care.
Treatment of Dental Issues
The condition known as looseness (laxity) of the jaw joints is difficult to cure. Intra-oral devices may be beneficial in some situations. Resting the jaw muscles by reducing the amount of chewing and talking can also be beneficial. Muscle relaxants may be effective in providing pain relief.
Treatment for Psychiatric Disorders
Depression is a typical symptom among those who suffer from chronic pain conditions. Individualized psychological counseling or cognitive behavioral therapy may be of use. Antidepressants can also help to alleviate some of the symptoms of mental disorders.
Additionally, in addition to the therapeutic options available to control the symptoms of the disease, numerous investigational therapies are in the works.
Edsivo (celiprolol)
Acer Therapeutics is developing Edsivo, an investigational treatment for vascular EDS in the early stages of development. Efficacy is achieved by stimulating collagen production in blood arteries, which lowers the pressure within those at risk of rupture.
Edsivo had already been given priority consideration by the Food and Drug Administration of the United States (FDA). In June 2019, the FDA released a comprehensive response letter indicating that an appropriate and well-controlled study is required to evaluate Edsivo’s effectiveness in patients with vascular EDS.
Excellagen
Excellagen is a topical gel-based on type 1 collagen created by Olaregen Therapeutix, which Generex Biotechnology later purchased. It is used to treat various human skin conditions. Excellagen applied topically to a wound promotes the generation of growth factors, such as platelet-derived growth factor, which aid in the healing process and promote wound healing.
The FDA has authorized Excellagen to treat 17 distinct types of wounds, and it is now in the process of being designated as an orphan medication for use in EDS patients.
Prolotherapy
Prolotherapy is regarded as a kind of regenerative medicine, and it is frequently employed as a last option when other treatments have been unsuccessful. The procedure injects a solution containing sugar (dextrose), phenolic compounds, and growth factors directly into the connective tissue to encourage tissue development and strengthen tendons, ligaments, and other connective tissue in the treated area. Prolotherapy is classified into three categories:
- Growth factor injection prolotherapy: This treatment entails injecting a growth factor into the patient’s body to encourage the accumulation of particular cells (such as fibroblasts) at the injection location.
- Prolotherapy for growth factor stimulation: This procedure includes injecting low-concentration doses of dextrose into the tissue, causing cells to generate growth factors that aid in developing and migrating specific cell types. The cells subsequently release collagen and other extracellular matrix proteins, which help reinforce the connective tissues around the injection site and the surrounding area.
- Inflammatory prolotherapy: Increased quantities of dextrose, coupled with phenolic chemicals such as procaine, lidocaine, or sodium morrhuate, are injected into the joints during inflammatory prolotherapy. As a result of the low level of inflammation caused by this solution at the injection site, immune cells are drawn to the area in an attempt to restore the joint’s function.
Specific Treatment Of Most Common EDS Types
1. Treatment for Classical Ehlers-Danlos Syndrome
The therapy for classical EDS differs from person to person, depending on the indications and symptoms present in each individual. For example, pediatric physical therapy and occupational therapy may benefit children with hypotonia and delayed motor milestones.
These procedures can also aid in the improvement of joint stability. Depending on the degree of the joint instability, assistive devices such as braces may also be required in some cases. Joint discomfort may need the use of anti-inflammatory medicines. Because classical EDS is linked with fragile skin and irregular wound healing, persons who have the condition, particularly youngsters, may need to wear protective bandages or pads over exposed regions such as the knees, shins, and forehead to prevent skin breakdown and infection.
Monitoring of children and adolescents for the development of aortic root dilatation may be necessary (enlargement of the blood vessel that distributes blood from the heart to the rest of the body).
2. Treatment for Vascular Ehlers-Danlos Syndrome
Treating and managing vascular Ehlers-Danlos syndrome (EDS) aims to alleviate signs and symptoms while preventing catastrophic consequences. People suffering from vascular EDS, for example, have tissue fragility that puts them at high risk of rupturing arteries, muscles, and internal organs, among other things. It is also critical to get medical treatment as soon as possible if you have unexpected, inexplicable discomfort since you may require emergency surgery. Maternal-fetal medicine specialists at a center for high-risk pregnancies should monitor the progress of pregnant women who have vascular EDS.
In order to identify aneurysms or other issues that may not manifest themselves with apparent symptoms, it may be necessary to have periodic screenings performed. Contact sports, hard lifting, and weight training should be avoided if at all possible to reduce the likelihood of injury. Also discouraged is the practice of elective surgery.
3. Treatment for Hypermobile Ehlers-Danlos Syndrome
In each individual, the therapy for hypermobile Ehlers-Danlos syndrome is determined by the indications and symptoms that they are experiencing. Physical therapy, for example, is frequently advised to help patients strengthen their muscles and improve joint stability. Depending on the severity of the joint instability, assistive equipment such as braces, wheelchairs, or scooters may be required to ensure proper mobility. When significant musculoskeletal (muscle and bone) pain is present, pain medicines may be administered to alleviate it. Patients with osteopenia (low bone density) and aortic root dilatation may be observed to develop these conditions in the future (enlargement of the blood vessel that distributes blood from the heart to the rest of the body).
