This is from an open letter that I found online some years ago. I have written it to fit my situation. Feel free if you would like to have a letter to share to use this one or use it as a guide to writing your own.
I am sharing this letter with you because I need your support and understanding about something that makes life very difficult for me.
Many of my friends have known for a long time that something is wrong with me but they didn’t know what and even I didn’t know what it was for a long time. This is part of what is wrong with me. I have a genetic disorder called Ehlers Danlos Syndrome Classical Type 1 or sometimes just known as Classical Type. The type I have effects about 1 in 20,000 to 50,000 people although there are many others out there like I was who have gone undiagnosed. I have another rare genetic disorder called Osteogenesis Imperfecta Type 1. In my younger days, although I had pain, I could do lots of things with my body that I can no longer do. You know the people in Cirque Soleil and the stretchy man at the circus? Well, they are able to bend their bodies because they have EDS that is why they can do what they do. It is good to know what is wrong with me as Ehlers Danlos Syndrome and Osteogenesis Imperfecta OI explains everything that has ever been wrong with me from literally the top of my head to the bottom of my feet since birth. I am however dealing with the reality that my pain and fatigue along with many other things that I deal with will be with me for the rest of my life. My pain greets me in the morning, stays with me in every step I take and every movement that I make, it puts me to bed at night, or earlier, and it haunts my dreams and wakes me from my dreams nightly. This as for now will be the rest of my life.
What is Osteogenesis Imperfecta (OI)?
Osteogenesis imperfecta (OI) or Brittle Bone Disease is a complicated, variable and rare disorder. Its major feature is a fragile skeleton, but many other body systems are also affected. OI is caused by a mutation (change) in a gene that affects bone formation, bone strength and the structure of other tissues. It is a life-long disorder. OI occurs equally among males and females and in all racial groups. It is estimated that approximately 25,000 to 50,000 people in the U.S. have OI. Having OI and EDS Classical type together is extremely rare, it is said that there are less than 30 people in the world with this combination. With good medical management and supportive care, the majority of people who have OI can expect an average life span.
People with OI experience frequent broken bones from infancy through puberty. The frequency typically decreases in the young adult years but may increase again later in life. Respiratory problems including asthma are often seen. Other medical characteristics and issues include:
Bone deformity, and bone pain.
Short stature.
Spine curves.
Low Bone Density.
Loose joints, ligament laxity, and muscle weakness are common.
Distinctive features of the skull including late closing fontanels, and head circumference greater than average.
Hearing loss may begin in the early 20s and by middle age is present in more than 50% of people with OI.
Brittle teeth (called dentinogenesis imperfecta or DI) are seen in 50% of people who have OI
Respiratory problems including asthma; may be aggravated by chest wall deformity and/or spine deformity.
Vision problems including myopia and risk for retinal detachment
Skin hyperlaxity; easy bruising.
Cardiac issues.
Fatigue.
Basilar Invagination a serious neurological problem is seen in some people with the more severe forms of OI.
Skin, blood vessels and internal organs may be fragile.
OI exhibits wide variation in appearance and severity. Severity is described as mild, moderate, or severe. The most severe forms lead to early death. Clinical features (observable signs) such as fracture frequency, muscle strength or extraskeletal problems vary widely not only between types, but within types, and even within the same family. Some features are age-dependent.
Types of OI
Since the 1970’s a list of numbered types has been used to describe the different forms of OI. The original list featured 4 Types. Today, as a result of recent research 15 Types of OI have been identified. Many people with OI do not fit clearly into one of the identified types and not all characteristics are seen in each person. A description of the more common OI Types follows. Understanding the individual’s OI Type provides a starting point for understanding the person’s health care needs. But due to all of the variable features, care for each person needs to be individualized.
People with Type I OI, the mildest and most common form, may have only a handful of fractures or as many as several dozen fractures in a lifetime. They may have few obvious signs of the disorder. There usually is little or no bone deformity. Height is less affected than in other types of OI. People with Type I OI are often similar in height to other family members. Muscle weakness, joint laxity, and flat feet are common. Dislocations and sprains may occur as well as fractures. Life expectancy appears to be average.
Most people with Ehlers Danlos Syndrome live a normal life span but there are parts of this syndrome that can affect your heart and veins that can lead to an early death. There are about 9-13 types of Ehlers Danlos and the type I have is one that should allow me to live a normal life span although I may have some cross over to a type of EDS called Vascular which can cause sudden rupture of organs and arteries. Having OI also puts me at risk for this.
Having EDS/OI means that my joints are very loose and my bones are fragile and doing the simplest things like getting a jug of milk out for my cereal can dislocate a shoulder or a wrist or just standing up can dislocate a knee or break a bone. The good thing is that most of the time I can put my joints back in place and I have thus far broken very few bones. Most of the bones that I have broken are in my feet. I know it may not seem impossible but in a week, I have an average of at least 6 dislocations or subluxations which means an almost dislocation. If you have ever dislocated anything then you know how much pain I am in every day just from my joints not to mention my muscles and tendons and ligaments. I often have bone pain as well.
There is not a part of my body that is not affected by these disorders. Our bodies are 80% collagen which is the glue that holds your body together. I don’t make enough collagen so everything that collagen holds together is compromised or can be compromised in my body from my skin all the way into my organs. This disorder has also affected my blood vessels and the way blood flows through my body, so I often get dizzy by just shifting in my chair or standing up. Most people who have EDS also have POTS Postural Orthostatic Tachycardia Syndrome which causes dizziness and sudden changes in heart rate and blood pressure. This leaves you very fatigued and many times unable to concentrate or stand up.
Having Ehlers Danlos Syndrome means that many things change all the time. As a child, I had many injuries and several surgeries but since we didn’t know about EDS/OI or POTS we all thought that is just the way things are. As a kid I didn’t know that having pain all the time was not normal, I thought that was how everyone was, so I just kept going on with normal activities. As I have gotten older my body is no longer just able to go on with normal activities. On some days washing dishes for 5 minutes causes me to have to rest or recover for several hours and sometimes for days. So, you can see that is what I mean when I say things change all the time. One day I may be able to do lots of things and the next day I may not be able to get off the couch. I want you to know that just because the outside of my body doesn’t look like things have changed, doesn't mean they aren't real.
Most people don't understand much about this disability/disease/syndrome/disorder and its effects, and that is ok, but it would help me for you to know how it affects my everyday life. In the spirit of informing those who wish to understand...
... These are the things that I would like you to understand about me….
I am scared. I don't know what the future holds for me. I already use a wheelchair at times, will I end up in one full time or will I be one of the lucky ones. If you find me being quiet and reflective, please don't think I am upset with you. I am trying to sort out my fears.
I am angry. EDS/OI has taken so much away from me. I can no longer do many of things I enjoy doing. I sometimes have difficulty just completing simple tasks. If I appear angry please understand it is EDS/OI I am angry with, not you.
Please understand that having EDS/OI doesn't mean I'm not still a human being. I have to spend most of my day being very careful about what I do, and if you are around me I might not seem like much fun to be with, but I'm still me stuck inside this body. I still worry about everyday things and most of the time I'd still like to hear you talk about your worries too.
Please understand that there are parts of my body that are very sensitive to heat and cold and smells and loud sounds. If I excuse myself in certain situations that may involve those things please don’t think I am leaving you, I am not, I am just taking care of myself.
Please know that EDS/OI can affect my thinking and my short-term memory so I may not be able to remember things that you tell me or things that I tell you. So, if I repeat myself, or if I seem like I am not paying attention to what you tell me it is not that, it is just that I have a hard time remembering sometimes.
There is not a part of my body that is not affected by these disorders. Our bodies are 80% collagen which is the glue that holds your body together. I don’t make enough collagen so everything that collagen holds together is compromised or can be compromised in my body from my skin all the way into my organs. This disorder has also affected my blood vessels and the way blood flows through my body, so I often get dizzy by just shifting in my chair or standing up. Most people who have EDS also have POTS Postural Orthostatic Tachycardia Syndrome which causes dizziness and sudden changes in heart rate and blood pressure. This leaves you very fatigued and many times unable to concentrate or stand up.
Having Ehlers Danlos Syndrome means that many things change all the time. As a child, I had many injuries and several surgeries but since we didn’t know about EDS/OI or POTS we all thought that is just the way things are. As a kid I didn’t know that having pain all the time was not normal, I thought that was how everyone was, so I just kept going on with normal activities. As I have gotten older my body is no longer just able to go on with normal activities. On some days washing dishes for 5 minutes causes me to have to rest or recover for several hours and sometimes for days. So, you can see that is what I mean when I say things change all the time. One day I may be able to do lots of things and the next day I may not be able to get off the couch. I want you to know that just because the outside of my body doesn’t look like things have changed, doesn't mean they aren't real.
Most people don't understand much about this disability/disease/syndrome/disorder and its effects, and that is ok, but it would help me for you to know how it affects my everyday life. In the spirit of informing those who wish to understand...
... These are the things that I would like you to understand about me….
I am scared. I don't know what the future holds for me. I already use a wheelchair at times, will I end up in one full time or will I be one of the lucky ones. If you find me being quiet and reflective, please don't think I am upset with you. I am trying to sort out my fears.
I am angry. EDS/OI has taken so much away from me. I can no longer do many of things I enjoy doing. I sometimes have difficulty just completing simple tasks. If I appear angry please understand it is EDS/OI I am angry with, not you.
Please understand that having EDS/OI doesn't mean I'm not still a human being. I have to spend most of my day being very careful about what I do, and if you are around me I might not seem like much fun to be with, but I'm still me stuck inside this body. I still worry about everyday things and most of the time I'd still like to hear you talk about your worries too.
Please understand that there are parts of my body that are very sensitive to heat and cold and smells and loud sounds. If I excuse myself in certain situations that may involve those things please don’t think I am leaving you, I am not, I am just taking care of myself.
Please know that EDS/OI can affect my thinking and my short-term memory so I may not be able to remember things that you tell me or things that I tell you. So, if I repeat myself, or if I seem like I am not paying attention to what you tell me it is not that, it is just that I have a hard time remembering sometimes.
Please don't tell me you know how I feel. You don't. Don't offer me sympathy; I don't want your pity. But do offer me support and understanding, which I appreciate. I know sometimes I look perfectly healthy, but looks can be deceiving, it is very possible that I dislocated my shoulder just this morning reaching for a glass in the cabinet or a rib is out of place because I laid down in bed. Please understand that I am dealing with invisible pain and a lot of fatigue. Even on a good day, I feel like you do when you have the flu on your worse day of the flu (tired, achy and sore). Please keep that in mind.
Please don’t be offended if you share with me that you are in pain or you are sick, and I don’t respond right away or at all. It is not that I don’t care, I care a great deal and I certainly know what it is like to be in pain and be sick. It is just that sometimes I just don’t have the ability to help out like I would like to.
Please understand that if I am not very talkative that I am not ignoring you and it is not that I don’t want to be around you, but I hardly ever get a full nights sleep because my pain keeps me awake so I am probably just sleepy.
Please understand the difference between "happy" and "healthy". When you've got the flu you probably feel miserable with it, but I've been sick for years (EDS and OI is genetic, this means I have had it since birth (even if I was only diagnosed in the last few years, I have been suffering from this since I was born)). I can't be miserable all the time, in fact, I work very hard at not being miserable and I work very hard not to show my pain. So, if you're talking to me and I sound happy, it means I'm happy. That's all. I may be tired. I may be in pain. I may be sicker than ever. Please, don't say, "Oh, you're sounding better!" I am not sounding better, I am sounding happy.
Please don’t be offended if you share with me that you are in pain or you are sick, and I don’t respond right away or at all. It is not that I don’t care, I care a great deal and I certainly know what it is like to be in pain and be sick. It is just that sometimes I just don’t have the ability to help out like I would like to.
Please understand that if I am not very talkative that I am not ignoring you and it is not that I don’t want to be around you, but I hardly ever get a full nights sleep because my pain keeps me awake so I am probably just sleepy.
Please understand the difference between "happy" and "healthy". When you've got the flu you probably feel miserable with it, but I've been sick for years (EDS and OI is genetic, this means I have had it since birth (even if I was only diagnosed in the last few years, I have been suffering from this since I was born)). I can't be miserable all the time, in fact, I work very hard at not being miserable and I work very hard not to show my pain. So, if you're talking to me and I sound happy, it means I'm happy. That's all. I may be tired. I may be in pain. I may be sicker than ever. Please, don't say, "Oh, you're sounding better!" I am not sounding better, I am sounding happy.
Please understand that I can’t just get over this or just rest more to feel better. Even asleep I have pain. There is NO cure for EDS or OI or POTS (and until they find the exact genes causing it and technology and medicine get to a point where something can be done about this, there will be no cure), only some of my symptoms and pain can be treated. If there was something that helped, then I would be doing that!
Please know that trying to get help for my pain and the other problems that my disorders cause is very difficult because many doctors don’t know much if anything about any EDS and some of the other conditions I have. Thank goodness for the ones that do, but they are hard to find. That is why I have gone undiagnosed until now. You cannot even imagine what all I have been told they “think” is wrong with me or how often I was just handed pain medication to move me along or even just dismissed because I’m too much of a challenge. If I seem frustrated, I am but I am not frustrated with you.
I want you to know that the pain and instability etc from EDS/OI and yes even POTS move around. If I was able to do something one day I may not be able to do it again the next day. If you want me to do something, ask if I can and I'll tell you. Just because I climbed the stairs yesterday (or an hour ago) doesn't mean I can do it today (or in another hour). Yesterday (or earlier) my shoulder was throbbing; today (now) it is my knee, who knows what it will be tomorrow (or later). Also, understand that being able to stand up for five minutes, doesn't necessarily mean that I can stand up for ten minutes, or an hour. It's quite likely that doing those five minutes has exhausted my resources and I'll need to recover - imagine an athlete after a race. They couldn't repeat that feat right away either. Please repeat the above paragraph substituting, "sitting up", "walking", "thinking", "being sociable" and so on ... it applies to EVERYTHING that I do. Similarly, EDS/OI and POTS and the symptoms of it may vary suddenly, meaning I may need to cancel an invitation at the last minute. I am not able to help do things that involve lifting or bending or cleaning or walking. If you ask me to help out in those ways, please know I may have to turn you down and if this happens please do not take it personally and know if I am turning you down I for sure need help with those things myself.
Please understand that EDS/OI and POTS does cause me depression every day of my life, wouldn't you get depressed occasionally if you had a body that could change suddenly for no reason, caused you pain 24/7 and could spontaneously rearrange itself through no fault of your own. If I seem down I probably am. Please know that I am not feeling sorry for myself. Just saying you understand will help.
Please understand that if I say I have to sit down/lie down/take these pills now, that I do have to do it right now - it can't be put off or forgotten just because I'm doing something else more exciting. EDS/OI and POTS does not forgive its victims easily.
Please understand that I can't spend all of my energy trying to get well from EDS/OI and POTS; it is incurable (and genetic which causes POTS, so unless I can change my gene’s I cannot change my disease/disorder). With a short-term illness like the flu, you can afford to put life on hold for a week or two while you get well. But an important part of having a chronic illness or disabilities like I have is coming to the realization that you have to spend energy on having a life while you're sick/disabled. This doesn't mean I'm not trying to get better. It doesn't mean I've given up. It's just how life is when you're dealing with EDS/OI and POTS or any chronic illness/disability.
Please understand that I truly have pain somewhere in my body every single minute of the day. Please don’t laugh at me or make fun of me or my disorder. Please don’t tell me I hurt because I’m getting old and to wait until I’m such and such age. My body on the inside is like 90 years old already. I am doing my best.
Please know that there are good days and bad days, more bad days lately than good ones, and that I am learning to pace myself and take better care of myself. I can’t always take care of the basic needs of everyday life and sometimes I don’t even have the energy to heat a can of soup, so I am left with lots of frustration and disappointments. I am learning to not be disappointed with myself but since these are a new diagnosis for me I am learning that me “not being able” is not because I am lazy or that I don’t try or that I didn’t take good enough care of me, turns out it is not my fault, it is just the way I was born.
Please know that trying to get help for my pain and the other problems that my disorders cause is very difficult because many doctors don’t know much if anything about any EDS and some of the other conditions I have. Thank goodness for the ones that do, but they are hard to find. That is why I have gone undiagnosed until now. You cannot even imagine what all I have been told they “think” is wrong with me or how often I was just handed pain medication to move me along or even just dismissed because I’m too much of a challenge. If I seem frustrated, I am but I am not frustrated with you.
I want you to know that the pain and instability etc from EDS/OI and yes even POTS move around. If I was able to do something one day I may not be able to do it again the next day. If you want me to do something, ask if I can and I'll tell you. Just because I climbed the stairs yesterday (or an hour ago) doesn't mean I can do it today (or in another hour). Yesterday (or earlier) my shoulder was throbbing; today (now) it is my knee, who knows what it will be tomorrow (or later). Also, understand that being able to stand up for five minutes, doesn't necessarily mean that I can stand up for ten minutes, or an hour. It's quite likely that doing those five minutes has exhausted my resources and I'll need to recover - imagine an athlete after a race. They couldn't repeat that feat right away either. Please repeat the above paragraph substituting, "sitting up", "walking", "thinking", "being sociable" and so on ... it applies to EVERYTHING that I do. Similarly, EDS/OI and POTS and the symptoms of it may vary suddenly, meaning I may need to cancel an invitation at the last minute. I am not able to help do things that involve lifting or bending or cleaning or walking. If you ask me to help out in those ways, please know I may have to turn you down and if this happens please do not take it personally and know if I am turning you down I for sure need help with those things myself.
Please understand that EDS/OI and POTS does cause me depression every day of my life, wouldn't you get depressed occasionally if you had a body that could change suddenly for no reason, caused you pain 24/7 and could spontaneously rearrange itself through no fault of your own. If I seem down I probably am. Please know that I am not feeling sorry for myself. Just saying you understand will help.
Please understand that if I say I have to sit down/lie down/take these pills now, that I do have to do it right now - it can't be put off or forgotten just because I'm doing something else more exciting. EDS/OI and POTS does not forgive its victims easily.
Please understand that I can't spend all of my energy trying to get well from EDS/OI and POTS; it is incurable (and genetic which causes POTS, so unless I can change my gene’s I cannot change my disease/disorder). With a short-term illness like the flu, you can afford to put life on hold for a week or two while you get well. But an important part of having a chronic illness or disabilities like I have is coming to the realization that you have to spend energy on having a life while you're sick/disabled. This doesn't mean I'm not trying to get better. It doesn't mean I've given up. It's just how life is when you're dealing with EDS/OI and POTS or any chronic illness/disability.
Please understand that I truly have pain somewhere in my body every single minute of the day. Please don’t laugh at me or make fun of me or my disorder. Please don’t tell me I hurt because I’m getting old and to wait until I’m such and such age. My body on the inside is like 90 years old already. I am doing my best.
Please know that there are good days and bad days, more bad days lately than good ones, and that I am learning to pace myself and take better care of myself. I can’t always take care of the basic needs of everyday life and sometimes I don’t even have the energy to heat a can of soup, so I am left with lots of frustration and disappointments. I am learning to not be disappointed with myself but since these are a new diagnosis for me I am learning that me “not being able” is not because I am lazy or that I don’t try or that I didn’t take good enough care of me, turns out it is not my fault, it is just the way I was born.
As you can see EDS/OI and POTS really alters my life…
Finally, please remember that I am the same person I was before I was diagnosed with these; EDS/OI and POTS doesn't change the heart and soul, well it does change the physical heart but not my God-given heart. I still laugh, I still cry, I still love, and I still want to be a productive person. I am me, I am not my disease. Please continue to love me just as you did before. I need lots of love, support, and prayer just like you.
But most importantly, I need you to understand me and ask me what I need instead of just assuming or guessing or doing what you think would be best for me. My needs are simple at times but at other times they are as rare as my disorders. Please be patient with me and know that I would much rather talk about you and your life and what you’re doing than anything that is happening to me but there are times that I need to talk about the stress that my illness’ is causing me just like you need to talk about your life stressors. If you find my illness’ too hard to understand or you don’t know how to help me or talk to me, know that I will feel that and feel very abandoned by you. Think about if you were in my place what you would want and then ask me if that would help or not. I need you in my life and I do have something to offer to you as well.
Most people with EDS are very determined and strong-willed. I don’t give us easy and I will overdo either out of determination or because I forget that I will pay for my actions later and sometimes I need to be reminded that I don’t need to do things but that I need to relax and ask for help and receive that help when it’s offered. The last thing that people with EDS want is to be a burden on anyone and as a chronically ill person it is very easy to sense when others see you as a burden so for myself, I tend to stay home because I’ve been forgotten and made fun of or asked to stop talking about my illness so many times that the energy that I expend to deal with the emotional pain from those types of actions is something that I cannot afford. I need gentle hugs and love from you and most of all acceptance from you without me having to hide who or how I am.
Amy
But most importantly, I need you to understand me and ask me what I need instead of just assuming or guessing or doing what you think would be best for me. My needs are simple at times but at other times they are as rare as my disorders. Please be patient with me and know that I would much rather talk about you and your life and what you’re doing than anything that is happening to me but there are times that I need to talk about the stress that my illness’ is causing me just like you need to talk about your life stressors. If you find my illness’ too hard to understand or you don’t know how to help me or talk to me, know that I will feel that and feel very abandoned by you. Think about if you were in my place what you would want and then ask me if that would help or not. I need you in my life and I do have something to offer to you as well.
Most people with EDS are very determined and strong-willed. I don’t give us easy and I will overdo either out of determination or because I forget that I will pay for my actions later and sometimes I need to be reminded that I don’t need to do things but that I need to relax and ask for help and receive that help when it’s offered. The last thing that people with EDS want is to be a burden on anyone and as a chronically ill person it is very easy to sense when others see you as a burden so for myself, I tend to stay home because I’ve been forgotten and made fun of or asked to stop talking about my illness so many times that the energy that I expend to deal with the emotional pain from those types of actions is something that I cannot afford. I need gentle hugs and love from you and most of all acceptance from you without me having to hide who or how I am.
Amy
