Is that an emergency?

I have been followed periodically by Dr. Trese, a specialist in ROP who practices in Detroit, since 1998. However, I don't live near Detroit, so I see someone locally to monitor my general condition. One of the more frustrating aspects of my life with ROP has been trying to manage seeing new eye doctors. Since I have a partially detached retina and a good bit of damage from various eye procedures, inflammation, etc, it is difficult for a doctor who is not very familiar with me to determine when I need to be seen by a specialist. I have some cornea damage; and sometimes when I see a new cornea doctor, he is alarmed to see that I have a retinal detachment and thinks I should go off to Detroit ASAP.

The question I have is not whether I have a detachment but whether it has changed. Sometimes Dr. Trese can assess this by looking at a scan--without me going to Detroit. At other times the scan is not clear, and I need to make the trip. If I went to Detroit every time a doctor was alarmed about the condition of my eyes, I might as well move there. The truth is that most doctors do not often get a chance to regularly follow people whose eyes are in the condition that mine are in--the people simply stop going to the doctor.

I have fallen into that category over the last few years. In 2008, my cornea specialist told me that there was nothing else that could be medically done for my eyes. So I stopped going. The rest of the story is for another post.

long-term complications of ROP: the basics

Today I responded to some emails on the ROP-support list on YahooGroups from a family with a child who was having surgery for advanced ROP. In my responses, I discussed some topics that are often important to families just beginning their journeys with ROP. I want to break my responses up for posting here--and of course make them generic so that they can benefit others. If you are new to the ROP journey, please feel free to post your comments and I will respond in comments or future posts.

What are the long-term effects of ROP that parents should be aware of? This is a question that new parents often bring to the ROP support groups. It is, sadly, not surprising for me to hear that doctors (still) are not giving this information to parents. There may be a number of reasons for this. Certainly doctors want to provide good treatment for the current condition; and in advanced cases of ROP it may be difficult to know whether the retina will even stay detached. It may seem silly to talk about the possibility of long-term retinal detachment when the reality is that the retina may not stay attached at all in the first place. If a child ends up being totally blind due to retinal detachment, complications which threaten vision, are less concerning to a doctor. However, this does not help a new parent to feel that she has been properly informed; and becoming active in a support group and reading posts from adults who talk about various complications can be alarming while one's child is still in treatment as an infant.

There is much information available online in medical terms; and I have collected a good bit of this as well as some personal experiences which are shared with people's permission on my ROP site. For the sake of making things easy, I want to explain the long-term issues associated with ROP in language for non-medical readers. I use my own experience as examples in parts of this explanation. I am only one person; and everyone experiences this differently. That is very important to understand.

ROP does its work during the first few months of a baby's life. This is also when it can generally be treated most successfully, assuming there is opportunity for success. Various things influence the opportunity for success. Treatments have only been available to infants for about 25 years, and within that time they have been refined. Some doctors are more aggressive than others; and some use more innovative procedures than others. This is also true of treatment for adults.

After ROP has stopped its activity during infancy, the baby is left with a damaged eye. Damaged eyes are typically vulnerable to certain types of additional problems, or complications. This is true of other types of eye problems as well; but in the case of ROP there are certain types of problems that are documented to be common. This doesn't mean that every person with ROP will experience them. It means that we know to watch for them. No one knows why they do or do not happen. I was diagnosed with ROP in October 1972, no screening or treatment performed. I had total retinal detachment in the left eye reported at the time and partial detachment in the right eye. I had vision in the right eye, but this was not confirmed until 1973.

Some people develop glaucoma, which is a condition resulting from high pressure in the eye, later in life--sometimes this is in childhood as the eye is growing and sometimes it is much later; sometimes it is very painful and sometimes it is chronic and painless and requires drops to be put in the eye every day in order to keep the pressure in the eye down. I have copies of records from 1974 showing that I was diagnosed with painful glaucoma in the left eye and surgery was recommended. It was never done and the pain eventually subsided. It occurred again in 1981 and I was placed on drops. At this time I was treated also for other problems in the right eye. (You see, ROP is complicated and the same problem may not affect both eyes.)

Some people develop cataracts--again this can be in childhood or much later. If a person has no vision, cataracts are often not treated. The "other problem" that I referred to above was a rapidly growing cataract in the right eye. The surgical report states that the cataract not only caused rapid vision loss but also could cause me to develop painful glaucoma. The doctor took the opportunity to also perform a retinal reattachment. At the time the procedure he used had only been performed on children for four years. It was quite successful on me.

I later developed glaucoma anyway, but it was the painless type. Because I didn't understand the importance of using my medications, I had chronic high pressure which damaged my cornea--this is not a normal complication of ROP. I have had three failed cornea transplants in my right eye, and this is the primary reason for my reduced vision. My retina is partially attached and still healthy, but light is unable to reach it well.

In 1998, I had another surgery for retinal detachment as a combined procedure with my first cornea transplant. This was also initially successful--the retina is still attached. I had what is called a traction detachment, which means that the retina was slowly being pulled away from the back of the eye. This is different from a detachment that occurs from a blow to the head, which is what parents are typically warned about watching for in children with ROP.

My situation is different from that of babies who have the opportunity for treatment now in numerous ways, and the long-term outcome that they experience medically will be very different. I spent many years of my life having surgeries and doing things that would help me to keep hold of some usable vision. I am now at a state where I cannot have more surgeries--my eye bled very badly during the last procedure and is considered too fragile for further procedures to be performed successfully. I now live with what remains and monitor for pain. The one complication that I have not experienced is phthisis. This is a condition in which the eye is dying. It is extremely painful and leads to removal of the eye. When the eye is removed, it is replaced with an implant which is then covered with a shell that is painted to look like a real eye. I have known people who have had this done and they have been very relieved afterward in terms of pain levels. It is a situation I hope never to find myself in; but if I do, I will face it. That is the important thing about my approach to coping with ROP complications: I cannot stop them. I either treat them or face them and learn to live with them.

What is the impact of ROP?

When I was about nine years old, I was rather proud of the fact that I could spell retrolental fibroplasia, the term used back then for what is now called retinopathy of prematurity. I was recovering from surgery on my right eye--a very complicated surgery to remove a cataract and reattach my retina, which had become partially detached--and my visits to the eye doctor were so frequent that it seemed that RLF was taking over my life.

How much impact does RLF/ROP really have on my life? Today, my perspective is quite different. I am comfortable discussing it openly; and I have had several surgeries and therefore plenty of opportunities for learning how I want to balance the focus on ROP. I don't claim that other people will always agree with my choices in this.

I separate my discussion of the impact of ROP/RLF as a medical condition from my discussion of the impact of blindness in general. Blindness is something I consider to be an outcome of ROP; for all people with ROP do not develop blindness, but they do face complications that must be managed medically in order to prevent further vision loss. Furthermore, those who are considered legally blind must still often manage complications in order to prevent vision loss. Blindness, on the other hand, impacts life in ways that present other management needs: the need for alternative reading techniques, the need for new ways of getting around, the need for new ways of doing any number of other things... Whether or not I think about these management tasks depends on how deeply I have integrated the management of blindness into my life and how much my need is dependent on the assistance of other people. So the impact of blindness varies; and I share this impact with people who have other eye conditions.

I had a severe case of ROP, and from the time I was a little girl I had only a small amount of vision. I learned to read braille when I was five; and I began using a cane when I was seven. Numerous other skills were likewise normal to me. For me, blindness does not have the same impact that it has on people who lose their sight later in life. It also does not have the same impact that it has on people who never learned these skills.

Having said that, there were times when I struggled with my feelings about blindness. Relating socially has not always been easy for me; and this caused me great emotional stress at some points in my life. Chronic unemployment has also been a problem for me, like a number of other people who are blind, despite the fact that I am educated and well-prepared to work. These things remain significant impacts of blindness.

Finally, the impact of blindness varies as other things in my life take on more significance. These things include social relationships that may be positive or negative and may ease or aggravate the stress of blindness-related problems, other medical conditions that need to be managed, projects that I have taken on,, etc. At this stage of my life, I don't perceive blindness or ROP to be the most significant thing impacting my life.

new parents and decisions about surgery

Parents often ask about how to decide when to cease efforts to restore sight to a baby with ROP via advanced surgical procedures such as vitrectomy. This is a very personal matter that only the family can decide. Many parents are afraid that their child will grow up and be resentful that more effort was not made if they do not continue attempts. On the other hand, parents who consider continuing on with surgeries are afraid that the procedures will have lasting psychological outcomes for the child.

Surgeries were not available for infants in the early 1970s, when I was born. However, I was subjected to numerous examinations as doctors attempted to evaluate the state of my eyes and provide my parents with an idea of my prognosis. During my early childhood, I was diagnosed with glaucoma in my left eye which was determined to be painful; and my parents were advised that the eye might someday need to be removed. In later childhood, I lost a significant amount of vision in my right eye very suddenly due to a rapidly growing cataract. Emergency surgery was scheduled to remove the cataract; and in the same procedure my retina was also reattached.

My mother experienced a great amount of anxiety regarding this procedure, much like what I described above. In the end, she decided that she must try and that it was best to know that she had done all that she could do. In other circumstances this might not have been the best decision. As an adult, I have had to repeat this decision several more times when surgery has been necessary to treat additional complications of ROP. During the last procedure, my eye bled profusely; and I now know that surgery is not a wise decision. This makes things easy for me now, but I empathize strongly with parents who struggle so deeply with these decisions about their babies' eyes.

Growing up with some vision has been useful. It is important for me to acknowledge this, even though I have had only a small amount of vision. I must also say that vision is not necessary for living a full life; and when dealing with advanced cases of ROP it is not always possible to predict the outcome of surgery. The art of hoping for the best while preparing for the possibility of unsuccessful outcomes is very important to master. For your child's sake, it is important to be at peace no matter what the outcome is. Your child will grow up and experience that outcome as normal. Your attitude will help your child to develop his/her perspective about blindness.

Welcome

When I was a little girl, retinopathy of prematurity was called by the name retrolental fibroplasia... My parents were very open about discussing my eye condition and prematurity with me; and I could pronounce and even spell retrolental fibroplasia by the time I was nine years old. I also knew that I was at risk of losing my vision due to the possible onset of glaucoma and that my eye might need to be removed if it became painful. However, there were many things I did not know. I learned a few along the way. For instance, I learned what a cataract was when I had one removed at the age of eight. There were other things I did not learn until much later.

In 1998, I began to educate myself intentionally about ROP. Several things prompted this journey: the sudden loss of my vision, a cross-country move and a search for a new doctor who could provide treatment, the availability of information online, and ability to scan and read articles at the medical library using relatively new technology. I became an empowered patient;. Twelve years later, as I face the fact that there is no more that can be done for my eyes, I am confident that I have made responsible choices in my treatment, even when risk was required. I am also confident that I am able to live well as a blind person. This has always been the case; for my parents chose educational options that ensured I would have the tools to live fully without sight from early in my life. My search for treatment has never been a fight to avoid blindness; but instead it has been a search prompted by my belief that I must use all of my senses to their fullest for the time available to me. I am thankful to my parents for both of these philosophies.

In this blog, I will share the things that have helped me along the way. Technology has changed a great deal over my lifetime; and I discuss this in detail on my technology blog rather than here. Here, I will discuss instead the philosophies that have caused me to make particular choices with regard to blindness: whether to use my vision or tactual functioning in a particular situation, when to seek help, and common lifestyle questions that face people with ROP.

I hope that you who are reading will leave comments and even discuss these things in response to my posts. Your comments will help me to know what kind of new posts will be useful or interesting to you. Please sign your posts so that I know how to address you in replies or new posts.

Welcome to the ROP Exchange.

introductory post

I've been a blogger for several years. I have considered transferring old blog entries related to ROP to this blog. However, as I read through my old entries, I found that it would be difficult to separate ROP-related material from other material in my blog. ROP is a piece of my life--a very important piece, but still only a piece. I am accustomed to discussing ROP on email lists confined to this discussion; but I felt that it was important to set up a blog where I could discuss more openly issues that I have lived with and wrestled with related directly to ROP.

I was born 12 weeks early in May, 1972, weighing 2 pounds and 2-1/2 ounces. My family was in the midst of a cross-country move from Oklahoma to Gulfport, MS, where my father would serve as a pastor for the next year or so. I remained hospitalized for 80 days, and my parents visited me once a week. They did not hold me until they took me home. According to Mom, thye got a call one day telling them to "come get her." They know very little about my NICU stay. There are many things Mom and I wish we knew.

I was diagnosed with ROP when I was five months old. Back then there were no screening procedures or treatments. ROP was then known as retrolental fibroplasia, a term describing the membrane characteristic of advanced ROP which often causes partial or total retinal detachment. Most people with this membrane were legally blind. Many were totally blind or had virtually no usable vision. The extent of my blindness was unknown until I was old enough to behave in ways that indicated I could see: pointing at lights, etc. I eventually learned to distinguish the differences between a few colors and read extremely large print.

People with ROP are vulnerable to a number of complications due to growth and changes in the eye. These complications are treatable if detected early. I have been treated for a number of complications:

• retinal detachment and cataracts at age 8
• surgical management of chronic (non-painful) angle closure glaucoma at age 20
• retinal detachment and cornea transplant due to corneal clouding at age 26
• 2nd cornea transplant due to failure of first transplant at age 28
• artificial cornea following failure of 2nd transplant at age 32
• removal of membrane (immune reaction to transplant) at age 35

I am on medication for glaucoma as well. The corneal clouding is not a documented complication of ROP but may have been related to persistent elevated pressure or repeated surgeries. Currently I am able to distinguish a small number of variations in color contrast and to locate some objects in the environment. This vision is useful in navigation but not for reading. For reading, I use braille and auditory materials. I have a program on the computer that provides synthetic speech output of text on the screen and can also display it on a braille display. I also travel with a dog guide.

I attended public schools in Texas throughout my entire school career, spending one summer at the Texas School for the Blind as a part of their talented and gifted program. After graduating high school in 1990, I attended Anderson University in Anderson, Indiana, a college affiliated with the Church of God Reformation Movement. I had grown up in a Church of God-affiliated congregation and desired to study music business at AU. I experienced some difficulties at AU, which I will post about separately. These were only partially related to blindness. In 1992, I transferred to Stephen F. Austin State University in Nacogdoches, TX. I studied elementary education at SFA, spending one semester as an intern in a classroom for children with learning disabilities and another in a fifth-grade ESL classroom. While I enjoyed working with children, I found that the school environment was difficult for me to handle. (I later learned that I had uncontrolled partial seizures as well as sleep apnea, and these likely contributed to my difficulties with maintaining alertness.) I dropped out of the education program without having student taught but having completed over 60 credit hours in education and special education courses; and I changed my major to psychology. I graduated in 1999, taking my final two courses back at AU following a family move to Anderson.

Following graduation, I held several part-time jobs. I worked part-time in a child care environment for three years before moving to Florida to work as a contractor providing technical writing services to Freedom Scientific, Inc., a manufacturer of adaptive technology for people with disabilities, in 2002. In 2004, I returned to Indiana following the active hurricane season. In 2006, I enrolled in AU's School of Theology to work toward my Master of Divinity. I have been a full-time student and anticipate finishing by the fall of 2009 or spring of 2010 depending on course offerings.

I mentioned seizures above. The seizures began during my teens; but getting treatment for them was a tremendous challenge. I have since been diagnosed with a number of other conditions as well: asthma, migraines, arthritis, chronic sinusitis, aspirin sensitivity, and possible mitral valve prolapse. Staying healthy is not easy for me; and often dealing with ROP takes a back seat these days. Still, I encounter blindness issues on a regular basis; and I remember that for people who are new to life with ROP, this is something that provokes many questions. That is why I want to open a dialogue about ROP. I will post about common questions that have cocme to me over the years, and I will also post in response to questions readers ask in comments. I can't promise regular posting--a lot depends on how much energy school and health stuff is taking out of me. But perhaps having something to write about will get my juices flowing.

I recognize that blogs develop community, and I want to encourage that. If you like to comment, please do. My only request is that you be respectful in your comments. My personal preference would be that if you use anonymouse posting you at least sign your first name or something that we can use to address you. It is confusing to me to read multiple "anon said" posts.

I may at times post general prematurity topics; but I need to state from the outset that my blog is not a forum for ethical debates. In the event that I post things that result in sensitive discussions via comments, I expect those discussions to remain respectful. I have worked very hard over the years to learn to respect both sides of sensitive issues. It has not always been easy. I expect anyone who reads this blog to take a step away from their personal agenda before posting a comment, especially if they feel upset by something that is said. Please remember that the person you are replying to is a human being with feelings. Also please remember that I can and will close discussions that become too heated.