Summer Fogle is the mother of two Boys who all suffer from a very rare and comex condition called Vascular Ehlers-Danlos Syndrome. The following story starts at the beginning of our journey over two years ago. Since then, both of our boys have been diagnosed and battled Jsut as hard as I have to fight for their life. This year alone, we have already had two out of 3 of us needing major neurosurgeries, including Dakotah who had his first surgery at only 12 years old. He had suegry o February 9th at Penn State in Hersey with our vascular neurosurgeon who specializes in endovasular neurosurgery and has a special interest in venous hypertension and stenosis in EDS patients. He did an emergency angiogram on Dakotah in January after a very rapid decline that showed his transverse sinus vein was severely blocking blood flow and he had severe stenosis Bilaterally but worse on one side. His surgeon informed us blood was already backing up, blood flw but compromised and things were only going to get worse. So 2 weeks later, we were back and they wheeled Dakotah to the Or for neurosurgery #1 to place a stent in his vein to open up blood flow and reduce pressure in his brain and other life threatening effects of this condition. Within 10 minutes, blood flow was successfully returned and our surgeon had saved our sons life. There are many more stories of our journey in the beginning here andin all the updates. Please bare with us as we have to travel almost monthly to the East Coast for medical care so life is very hectic and unpredictable so we have been bad at updating this page. But keep checking back as we have now made this active again. 

This Spring, however, Summer began to develop serious health issues. She was put on oxygen and started having to use a wheel chair due to extreme weakness in her legs and other symptoms thatmade walking difficult. She also had to wear a hard cervical collar due to her neck and head being unstable to prevent serious damage to her neck and spinal cord. After numerous tests and visits to specialists, Summer was finally diagnosed with a very rare and serious genetic connective tissue disorder, called Ehlers-Danlos Syndrome (EDS). 

EDS is a genetic connective tissue disorder causing defects in collagen. Collagen is the most abundant protein in the body. It provides the basis for strong connective tissues and healthy skin, and is a building block for all the body’s systems. Connective tissue covers over 95% of your body; therefore, EDS is a multi-systemic syndrome. Experts say that EDS can profoundly affect quality-of-life. Collagen acts as the “glue” that holds your body together. Faulty collagen creates fragile and stretchy connective tissues in the joints, muscles, skin, blood vessels, organs, and more. The resulting pain and symptoms tend to progress with age and “wear & tear” on the body. 

Ehlers-Danlos Syndrome is a group of disorders that affect connective tissues, which are tissues that support the skin, bones, blood vessels, eyes and other organs. There are many coexisiting conditions that occur with EDS that can be very serious and even life threatening if not detected early enough and treated quickly. Defects in connective tissues cause the signs and symptoms of Ehlers-Danlos Syndrome, which vary from mildly loose joints to life-threatening complications related to tissue structure and fragility. There is no cure for Ehlers-Danlos Syndrome, one can only treat the symptoms and the many coexisiting conditions as they occur. This makes it a very disabling and debilitating condition and always makes one vulnerable to injuries, organ ruptures and multiple health issues that coud arise at anytime

Summer and her family have already had to deal with the devestating affects of EDS and have seen firsthand how rapidly it can progress and affect the entire body. Because EDS is so rare and widely misunderstood, there are only a handful of specialists in the world that are trained and truly understand how to treat patients with Ehlers-Danlos. There are two highly qualified world renowned neurosurgeons in the DC area that both have their expertise in EDS and how it affects different parts of the body. Summer found an excellent neurosurgeon in Maryland who is an expert in Ehlers-Danlos Syndrome and how it affects the neck and spine. Through specialized testing, he was able to determine that Summer had severe instability at several levels of her cervical spine that were causing her spinal cord to be compressed, leading to numerous neurological symptoms. Left untreated, this could have turned into a life threatening situation for her due to how severe the instability and spinal cord compression was. Summer's neurosurgeon urged her to have the surgery immediately to stabilize the cervical spine and relieve the spinal cord compression immediately in order to avoid any more serious complications. Therefore two weeks after her first visit with the neurosurgeon, Summer had the first of many surgeries to address her multiple health issues caused by EDS.



On July 8th, Summer had a cervical fusion done that stabilized the levels in her cervical spine from C2-T1. The cervical spine was also realigned to alleviate all compression from the spinal cord. Some levels were hard fused with screws, rods and plates and some were fused with bone bank bone mixed with bone marrow harvested from the hip during the surgery. This was a very long and detailed surgery and Summer spent 5 days in the hospital aftewards and ended up in the ER once during her recovery period. Finally after getting the all clear from her neurosurgeon, she returned home to her family to start the recovery process. Since this was almost a full cervical fusion and the surgery caused a lot of trauma to her body, Summer has had a very long and painful recovery from the fusion.

Prior to having the cervical fusion, Summer met with another EDS neurosurgeon in Virginia who specializes in the vascular part, specifically how EDS affects the blood vessels and veins in the brain and the neck. He performed a specialized test on Summer's head and neck that showed some veins were flattened and narrowed and could be causing CSF pressure to build up inside her brain, causing symptoms of intracranial hypertension. Therefore, this neurosurgeon recommended that she have a cerebral angiogram to determine how severe the blockages are in her brain and to check how high the pressure is. Despite being less than 2 months post op from her cervical fusion, she will be traveling to have her cerebral angiogram in about a week due to the severity of this condition and the debilitating symptoms it is causing. The results from that test will determine if Summer needs to have a stent placed in her brain. Should she need this venous stent brain surgery to open up the narrowing veins, she and her family are very hopeful this will offer some much needed relief and prevent any permanent neurological damage or further complications.

Although the cervical fusion has stabilized her neck and improved many symptoms Summer was having, and the brain surgery could also prevent further neurological complications, she will still be faced with other surgeries in the near future to address the many coexisiting conditions that come with EDS. Many times in Ehlers-Danlos patients, something called Tethered Cord Syndrome occurs, and can commonly start to cause symptoms after a surgery such as the fusion due to the tightening of the spinal cord. Tethered Cord Syndrome is a condition where the spinal cord tissue abnormally attaches to the bones of the spine. This “tethering” restricts the spinal cord from moving freely inside the spinal canal. This can cause a variety of neurological symptoms. The Tethered Cord release surgery does have many risks,  and there is a chance of CSF leaks, scar tissue or re-tethering of the spinal cord causing issues after surgery. However,  it is important to note that the risks of an untreated Tethered Cord can lead to other health problems such as Chiari I Malformation or permanent damage to the spinal cord due to the stretching of nerves due to the tethering. 

Therefore, Summer will also be meeting with the neurosurgeon that did her cervical fusion to discuss whether or not she will need the Tethered Cord release surgery and the risks versus beneifts to that surgery and whether it will improve her symptoms and quality of life and prevent any permanent nerve damage. Either way, she will need spinal surgery soon to remove a large tarlov cyst on her spine that is causing pain and other symptoms. Tarlov Cysts are very common in EDS patients and this surgery would also have to be performed by her neurosurgeon in Maryland, due to his expertise in handling the complexity of EDS patients during any type of surgery. Those who have progressive and prolonged symptoms run a risk of neurological damage, if the cysts continue to compress nerve structures. At her next visit with her neurosurgeon, Summer will discuss with her neurosurgeon the two spinal surgeries and determine which issues is most serious and needs to be addressed first.

Ehlers-Danlos is a complex disorder, and every health issue surrounding the disorder can be much more complicated due to how weak the body tissues are in EDS patients. Surgery in EDS patients can come with delayed healing and longer recovering times and a variety of complications, therefore, that is the reason it is crucial that Summer must travel to have her surgeries done by neurosurgeons who are highly skilled in the neck, spine and brain and truly understand all that encompasses Ehlers-Danlos Syndrome.

Summer has strived to live each day with a positive attitude despite all the trials and struggles that she is facing, in hopes that she can serve as an inspiration to others. She especially hopes to set an example for her children, so that they will learn how to handle challenges that come their way and learn the true meaning of working together as a family during difficult times. Summer has continued to keep her B&B open as it was always her dream to create a peaceful retreat that others can enjoy. Despite her health struggles, she still is planning on making B&Bs Going Blue a huge success for its second year in January 2017 as a way to honor law enforcement officers and their families.

She and her family are very thankful for the support of family, friends and neighbors during this difficult and trying time. They appreciate any and all help that is received and are very gracious to all those that are able and willing to help them on this medical journey into the unknown.