Welcome to a journey with POTS!
May is Ehlers Danlos Awareness month and that makes it a perfect time to share our story and journey.
I would like to talk to you about 2 rare conditions that often go with each other in patients; mostly young women. Both conditions have made our 25-year-old daughter permanently crippled and disabled in a short amount of time – about 6 years – and unable to leave the 2nd floor of our home on most days.
Ehlers-Danlos syndromes (EDS) is a group of connective tissue gene disorders associated with baseline chronic pain, which affects both physical and psychological wellbeing. the skin tears or bruises easily, they have unstable joints prone to frequent painful dislocations subluxations, and degenerative joint disease. My daughter sounds more like an 80-year-old woman when she gets to the bottom of our stairs than she does a 25-year-old. She lives with a daily pain deep down in the bone that nothing will touch for pain. She is also not being offered anything for her pain that hovers around a 6 or 7 every day, and I find that just a little inhumane.
The most common analogy likens the body of someone with EDS to that of a house built with faulty materials. Connective tissue supplies support in the body, just like the mortar between bricks. Hers seems to be progressing and getting worse and she is being held together with physical therapy tape. There is no textbook treatment or medication regiment for this condition.
POTS (postural orthostatic tachycardia syndrome) is a form of dysautonomia that affects the flow of blood through the body. The autonomic nervous system regulates involuntary actions that allow the body to function. When we stand, the body needs to accommodate a complete set of processes to allow this. Upon standing, a significant amount of blood automatically falls to the lower body.
In POTS, there is an increase in heart rate and light-headedness on standing and exercise intolerance, fatigue, and a multitude of other symptoms. The symptoms of POTS are the equivalent of living with COPD or congestive heart failure. There is no cure for POTS. There is no turning this switch off once it has been activated in the body. It becomes a chronic lifetime condition to live with.
There is a motion picture coming out this month entitled “Behind the Visible” about POTS. I am so excited for the release of this movie and am driving my loved ones a little crazy right now!
Register for the premiere and watch a clip here: https://www.behindthevisiblefilm.com
POTS may be so severe that even everyday activities usually taken for granted such as bathing or walking may be severely limited. These conditions cause an enormous emotional, physical, and financial burden to patients and their families. The high degree of physical disability, cognitive impairment, pain, and disruption to schoolwork and life plans leaves these patients with many daily challenges. 25% of patients are so disabled (like our daughter), that they cannot work, drive, study, or go to school.
My daughter also has chronic hypovolemia (which means she now has low blood volume in her body) and leaking capillaries in her feet and legs because she went too long undiagnosed, untreated, and misdiagnosed by doctors. She now has a port in her chest to receive daily iv fluids and needs home health care to take care of the dressing. At one point, she was swallowing over 40 pills a day to manage all her symptoms.
There are few specialists in this country to treat both conditions, and most patients typically must travel more than 100 miles. Travel by car is extremely difficult for my daughter, so I have been very thankful for telehealth during the pandemic and really hope that will continue for our patients beyond the crisis. The average time for diagnosis is 6 years and our community dreams of a day when it is so easily recognizable that diagnosis happens in 15 minutes.
Her entire academic career had already been very difficult due to her NVLD diagnosis at the age of 9. We did not put pressure on her to also work, but she did try twice. Once working at Kohls, briefly, until a customer belittled her because she could not make a change out of a $100 bill. The second job she loved. She was working at a daycare center and that is when she started her journey to becoming crippled, after being inundated with germs and viruses while working at the daycare center.
On this journey, we have learned that she does not qualify to draw disability benefits because she has not earned enough work credits in her incredibly young life. This is legislature and we need some policy changes. We found the PA state government to be very abusive. She is allotted $500 a month in SSI, because of the value of our home and our monthly mortgage payment. Why does that matter? She will also lose her SSI benefit if she does marry her fiancé. Why is that necessary? It does not change the fact that she is crippled and disabled and needs assistance! The judge in her SSI hearing moved her to tears and completely blindsided all of us when he accused us of being tired of supporting her and wanted rent money. Some policies need to change in this department because of her age, we were not allowed to go with her in this hearing; knowing she would have difficulties advocating for herself because of her cognitive deficiencies from a brain impairment she was born with.
You see, her conditions are invisible, and she looks relatively “normal”, so most people just assume that she is lazy and undriven. She is a warrior who deserves a huge amount of credit for the way that she is handling being disabled for the rest of her life. Do not even get me started on the looks that she gets from others when she gets out of a car parked in a handicapped spot. At the age of 25, she needs Botox injected into her bladder to manage symptoms from a neurogenic bladder. The comorbidities list associated with these conditions is long and no two cases are ever the same. In fact, if you know someone in Arizona who has POTS, and you also know someone in Maine who has POTS….do not compare the two.
We also often meet issues with insurance because our benefits come through a self-insured plan. Even with a letter written by a physical therapist with a doctorate saying that she should really have physical therapy for her conditions 3 days a week for the rest of her life, they refuse to bend beyond the contracted 20 visits per year. I could not get her to physical therapy 3 days a week if I tried, but it would sure be nice if we did not have to pay for this out of pocket at this point in our lives. Another issue is Medicaid cards in PA not being accepted by some professionals that we need to work with. That speaks volumes for what a pain the government must be to work with! The state has declined coverage of two medications because they were being used for something not approved on their list. These conditions do not fit neatly into a box.
Finally, school nurses and gym teachers in public schools need to be made aware of POTS and how difficult it will be for those students to perform the same as their peers. They will need accommodations for these graduation requirements. I’ve heard stories of gym teachers being the bane of existence for some moms because they have decided the student is lazy and often stand between the student and a diploma or progressing to the next grade.
We are in the process of writing a book together about our journey to where we are today. A link was discovered last year between EDS and the autism spectrum disorder. She finds this news incredibly validating! I spend A LOT of time trying to connect the dots with what happened to her and hope that it may help someone else. It may take us a year or five years, but we will get this done!