RP and Cataract Surgery

People with Usher Syndrome face progressive hearing and vision loss.  The vision loss is due to Retinitis Pigmentosa (RP) that causes increasing loss of peripheral vision, while the central vision - for the most part - is unaffected by the RP.  That doesn't mean that the central vision can not be affected by other visual acuity issues.  Near- and far-sightedness can usually be corrected (or somewhat corrected) with glasses or contact lenses.  But cataracts are more common with RP at a younger age than with normal-sighted folks (like me!)

There is controversy and lack of consensus as to whether or not cataract surgery is advisable at all for patients with RP.  In fact, our daughter's retinal specialists have STRONGLY advised against it!  However, the retinal specialists she's seen, while long well-known in their field of research, are just that.  They are not clinicians; they don't deal with the reality of one's life or the functionality of vision based on the personal, professional, social/emotional life of their patients.  So....after consulting with many, by phone, email, in person, and doing extensive online research, she went to a highly recommended cataract surgeon who has experience with such surgery on RP patients and has, in fact, done a number of them against the recommendation of our daughter's retinal specialists - with success!

Roberta made this decision herself, deciding to go forward with the surgery and take the risks associated with it to have a chance at extending her work life, which had become severely impacted quickly over the last 8 months, due to loss of acuity, extreme sensitivity to lights and glare, and distorted images from the affected eye.  I think when a hearing-impaired person who relies heavily on lip-reading can no longer do that across a table, it's natural to want to do anything to improve that.  Imagine the frustration of struggling to hear AND see when just months before, you had a good handle on the disabilities imposed by this condition.  Usher Syndrome is an inherited condition, caused by both parents carrying a recessive gene for it.  Our daughters both have this syndrome, Type IIC, with absolutely no known history of it on both sides of our families.

So....the surgery took place last Wednesday, a simple procedure that had us in and out of the surgery center in very little time.  She was alert (no conscious sedation) and pain-free when we left there, and she was immediately thrilled with the results.  A follow-up appointment the next day revealed that her vision was 20/30 unaided in that eye as opposed to the 20/200 pre-surgery with her contact lenses.  WOW!  WOW!  WOW!

We left her yesterday to return home, feeling comfortable that she was OK to be on her own and to return to work today.  So far, so good.  Stay tuned, however, for the follow-up to this story, for there is an issue today for which she is going to see the surgeon.  Not sure what's up, but if you're reading this, please continue to pray for her.  I'll report once I know more, or you can follow along on FB.

A Special Book and Author

Many thanks to a new neighbor / court reporter who took a workshop on transcription for the hearing impaired and then loaned me a book, highly recommended, on which the workshop was designed and the author was the presenter.  The author describes her personal journey from hearing to profound deafness and back into the world of hearing as a result of a Cochlear Implant (CI).  Author Arlene Romoff calls it a "modern day miracle" in her second book, Listening Closely.  This is actually the second part of her journey, documenting the demise of her first CI and a reimplantation as well as her progression to becoming a binaural hearing person once again.  I have been moved to order her first book, Hear Again.

Ironically, the hearing aid dealer we dealt with in RI, when we were in the throes of FOUR hearing-impaired ears, called his business "Hear Again!"  While visits there were always traumatic for me, he and his wife and staff ran the most welcoming service, always made me laugh, even while dealing with difficult life and life term  events.  I will always be grateful to the late Reno Bellafiore and his family.

And it's important to realize that while we were struggling to deal with the realities of our children's hearing loss in the 70s, others were in the process of developing the so-called "bionic ear."  The results of those scientists, researchers, and medical personnel are that CIs have become more and more perfected and available to restore "hearing" to deaf and hearing-impaired individuals across the world.  I had always hoped there would be something in my lifetime - and certainly within my daughters' lifetime - that would make it possible for them to "hear!"

It's important to realize that CIs do NOT correct hearing, not in the way that eyeglasses can correct vision (sometimes), but that one does actually have to learn to hear again.  Arlene Romoff, in her well-written and informative books, describes sensitively and in great detail the process by which one relearns to hear and communicate with the hearing world.  The social/emotional components of the transition are handled equally as well.  It is a fascinating read for anyone considering a CI, or for those, like myself, who travel the journey along with their afflicted offspring.

As one of my daughters has committed to CI implantation next June, I am most interested in reading Arlene's story.  I posted about her books on FB yesterday and then searched for her within that venue.  I found that she has a personal profile but not a business page.  I sent her a message along with a friend request, which she immediately accepted, visited my Scarf It Up FB page (and "liked" it).  As we began a dialog via FB messages, I informed her of my interest in her story and told her of my daughters' Usher Syndrome and that they manage a FB Community called, Usher Me In.  She immediately "liked" that page too, and I hope she'll be a follower and contributor to that effort.  Thank you, Arlene!

Also somewhat ironically, as I was driving home from work at my co-op gallery, I heard on the radio a report about a bionic eye that has been developed in Australia, for use in RP patients.  Somehow I knew 40 years ago when we first started this journey that there would be assistance for the likes of my daughters, and hopefully I will personally see and hear and experience all this for my daughters!

The Frustration Continues!

Life can sometimes feel like a living nightmare soap opera!  Just when you think things are status quo - after accepting what is over a period of time - a new kick comes into play.  In the last week, there have been TWO such knock-downs, one for each of our daughters:

• One learned that her hearing has declined significantly in one ear.  Consideration of cochlear implant has been advised, which she is very reluctant to consider.
• The other learned that cataracts are the cause of her diminished vision.  Surgery in the case of RP patients is riskier than the norm, and the opinions on their treatment are varied and horribly confusing.

Both have jobs they can't afford to lose and parents who can never stop worrying, particularly as WE get older and realize that we can't take care of their needs forever!  I guess this is a constant for most parents.  We've spent the last week researching the best retina specialists, cataract specialists, and comparing the two major brands of cochlear implants.  Confusing and exhausting!

Any personal recommendations will always be appreciated!

There IS Hope!!!

I've been remiss and haven't posted here for quite a while.  Life and other stuff (like the stuff I make!) have gotten in the way.  But always I come back to the issue of Ushers Syndrome - because it affects my life through my daughters.  They are both amazing women with fulfilling lives who seem to deal well with their condition(s).  It takes just an incident or posting on FB or blog posting or conference call to bring the whole thing home again - for me!  But I don't live it EVERY single day of my life.  I admire my girls and all those who have vision and/or hearing issues - for the struggles they most often endure silently every single day of their lives.

I listened on Monday to a conference call/visual presentation by Heidi Rehm of the Harvard Medical School, on the "Why's" of genetic testing for Usher Syndrome, the reasons for the cost, and the time it takes to secure the results.  Is it worthwhile?  Absolutely!  Very costly, yes!  But just today I was alerted to a new research study approved by the FDA for US Type 1b.  So that means that it is very important to KNOW what type of US one has - to be able to be included in the study.  We know that our daughters have Type 2, but we DON'T know which one.  It will be important to know in order to be able to avail themselves of studies down the road.

So much has transpired in the world of genetics for US in the last 22 years since Roberta's diagnosis.  Progress comes slowly, but there is hope, and I always want to hold onto that.

Then I read on FB of Roberta's mishap at school and was devastated for her.  No long-lasting physical ill-effects but the emotional ones wear on and on.  It hurts so much when a mom can't FIX what hurts!

And this morning I caught up on my reading of DoubleVisionBlog, in which twins are undergoing an unusual Eastern medicine treatment for RP, and so far the results are outstanding.  And scary!  And so well-written.  Fear of hope is explored as well as some details of this extraordinary treatment process.  I urge you to read it if you have any interest at all in RP (retinitis pigmentosa).

Let there be hope - as fearful as that is!

Vision Walks! Fundraising for the Foundation Fighting Blindness!

My girls are amazing!  I am always proud of them but now more than ever.  They both decided it was time to get involved in fighting for their own cause.  The Foundation Fighting Blindness' Vision Walks to raise funds for research on a variety of blindness diseases and conditions seemed to be right up their alley.  They BOTH committed to doing something they've never done before - establish a "walk team," set fundraising goals, and solicit funds to support their respective walks.

Andrea is walking in Greensboro, NC this Saturday; Roberta is walking in Boston in late October.  BOTH have met their $$$ goals, raising a combined total of almost $4000.00, and both will walk with family and friends.  Andrea and her family made tie-dyed tee-shirts for their team members with the Usher Me In logo on the front.

My only regret:  I can't participate in either walk with them for a variety of reasons, but I will be there in spirit.  I've committed a percentage of my Scarf It Up! sales to this effort through the holidays.  If you'd like to see more of my work, please view my flickr pages, and if you're interested in purchasing anything, contact me via Facebook.  Many thanks to all those who have supported Andrea and Roberta in this effort and who have purchased my creations!

Musings of a Mom!

I've always tried to maintain a positive attitude towards most everything in life.  It is not because I am a person of faith or that my family of origin was positive in nature, or that I married a positive person.  These were NOT the case!  I guess, despite all that, I have my own sense of looking at life to uncover the best possible means to be happy or improve my own situation or that of my loved ones.  Considering the environment in which I've lived 60+ years, I manage to be positive most of the time.  And so it is in dealing with disability and family health issues that have been part of my adult life for 40+ years.

I do get depressed from time to time!  And I do take medication for periods of time when I need it.  And I do talk with a therapist on occasion - also when I know I need it.  In other words, I recognize the signs and symptoms, and I take care of myself.  I have to....because I am needed.  I can't help those in the family who need it if I am not whole and sane. (Tennis and my fiber art help too!)

Somehow, and this seems rather ironic, I've gained strength from the many setbacks our family has encountered.  For example.....the knowledge that one daughter would fail her DMV vision test and have to give up her driver's license - although we knew this was coming - was initially devastating.  A life-altering experience that has been characterized by relief, rather than angst.  I worried more about this lack of driving permission than her driving itself.  And she has dealt with it beautifully - hiring drivers for work, appointments, and errands, and prevailing upon friends and neighbors for other needs and entertainment.  I should have been worried about how safe she was (and others!), and so now I realize why she's experiencing this as a HUGE relief, rather than a HUGE burden.  And she's handled it with aplomb!  So proud!  I pray that our other daughter will accept her fate when it happens with similar equanimity.

Each person handles life's experiences differently.  That's a function of personality and one's personal philosophy.  How do I handle the various tribulations of my life?  Not always with aplomb!  Not always with positivity!  Not without falling sometimes!  And not without failing sometimes.  But I realize that I have a process that seems to work.  If shared, this process can perhaps help others in dealing with their own trials.  But that's where personality comes into play, I think.

Here's what I've done - even before I realized it was indeed MY process!

My initial reaction to adversity is not always good or positive or effective.  In an emergency (such as the many cardiac issues encountered with my DH), I am usually OK through the crisis and fall apart after the critical period is over.  With disability issues for my children, I am initially devastated, shocked, frightened, and immobilized.  I ALLOW myself 24 hours (but generally no more) of self-pity.  Sometimes it's hard to "snap out of it" (as some have advised!)  If it persists for more than a few days, then I know it's time to resort to other means - medication first and then arrange to meet with my wonderful, compassionate but direct LISW.  Generally by the time I get to the appointment several days later, I have snapped out of it, and embark on a plan to DO and COPE rather than STEW and MOPE!  This has proven effective in so many instances!  But this is MY personal plan - it will not work for everyone.

The most important thing is to find the resources and help to develop strategies that WORK for you!  For some, this might be faith in God, prayer, support groups, involvement in disability organizations, writing, helping others, presenting, educating, or any number of other engagements that WORK!  Helping others always works wonders, so if you're reading this and having difficulty dealing with Usher Syndrome, low vision, hearing loss, any other form of disability, disenfranchisement, disaster, or distress, consider these possibilities and SHARE them with others.

This blog is proving therapeutic for me but with the hope that it will perhaps help others too.  Comments welcome!

What can you see?

I've never asked my daughters this question before!  Since one of them is visiting us this week, I decided to now ask this question and one other.  We were sitting in our office area - she on the floor; DH and I in our respective chairs at our computers.  I turned to look directly at her and said: "Look straight at my nose and tell me what you see."  My elbows were resting on the arms of my chair, and I was about 5' away from her.  She could see my face and my extended elbows, the tops of my knees, and she was aware of the light from the window behind me and the stained glass disk hanging in the window.  She could NOT see my legs below the knees, the floor, the top of my computer cabinet, or anything on the wall above it.

Retinitis Pigmentosa
ONE of the aspects of Usher Syndrome

The limits of her field of vision shocked me.  I'm not really sure why because we knew that she had recently had to give up her driver's license because she could not pass the visual field test.  She "never saw the moving light at all!"  Normal peripheral vision is close to 180 degrees.  Hers is about 30, so we're told.

This prompted my next question - one I have been reluctant to ask, but she willingly answered.  "Are you relieved to no longer be driving?"  I had thought her need to give up driving would be a crisis, a critical life-altering experience to which she would VERY slowly adjust, find her drivers as necessary, and go into some type of immediate - and understandable - depression.  Instead, she has been more pro-active than usual, found people willing to provide services in her home (the hairdresser, the dog groomer, the speech pathologist), found drivers for work, back-up drivers when needed, friends and neighbors to drive her to shop and to attend to daily needs, and SHE IS VASTLY RELIEVED!!!

I had no idea how stressful driving had become for her.  She had for several years given up driving at night and in rain, but honestly, she and I had driven several times in recent years between NC and New England, sharing the driving almost equally.  She never drove in the NY - DC stretch, but who likes that stretch of 95 anyway?  I NEVER felt uncomfortable with her driving or concerned for her or my safety.

Well, all that's behind her now, and she has handled the changes with aplomb.  It has not been a crisis; it has been a gift.  By her own admission, she manages things in her life much better on a deadline and so her driving needs have been addressed and arranged - all before her vacation in NC!  I am immensely proud!