Riley's Story- Pars Planitis

The short story:

Riley was diagnosed with an eye disease called Pars Planitis at the age of 9. He has had 4 procedures on his eye including one major eye surgery. He can see out of both eyes at this time.

Current Status:

Right eye: Pars Planitis, Glaucoma, Astigmatism, Cataract, Near Sided, Snowbank (Macular Pucker Removed), Beginning damage to his cornea, Afferent Pupillary Disorder. Afferent Pupi

Left: Pars Planitis, Lazy Eye, Amblyopia, Far sided, Snowballs, Recent significant Loss of vision in this eye

The Very, Very Long Story:

In May 2009 Riley and I were walking up the stairwell for bedtime tuck-ins when he covered his left eye with one hand and said "Mama, if I do this, it's blurry." This wasn't alarming to me because my brothers and I all three wear glasses. I asked him a few questions about how long had it been blurry to him, and with each question he answered "I don't know." To me, blurry meant he needed glasses. I called the ophthalmologist the next day and scheduled an appointment for him. I told the person on the phone what Riley had said, no one was alarmed, and the appointment was scheduled a few weeks out.

Three weeks later, the day before the last day of school, I took Riley to his appointment. We were with the doctor for a very long time. He was asking Riley questions that were alarming to me. He asked me if he had taken a fall, or had a blow to the head. He brought in one machine after another testing things. When the senior doctor in the practice came in, I knew whatever was going on was a big deal.

We were told at that visit that Riley's right eye had hemorrhaged and was full of blood. His left eye did have some infection in it, but the right eye was the primary concern. Some names of genetic disorders were tossed around with some speculations. We were told to go right then and get a prescription for glasses filled and that Riley needed to be still for the next two weeks. Then, we would need to come back for them to check his eye again.

The next two weeks were hard. We left on vacation two days after his visit, a trip to the beach, and we were mainly worried and stressed with the unknowns. We kept Riley as still as possible for a nine year old boy.

Two weeks later we went back and met directly with the senior staff doctor. He looked in Riley's eyes with some fancy equipment and said he could see some improvement, but wanted to see some more. He talked more about genetic disorders and then mentioned a disease specifically that he believed Riley to have.

After showing no improvement at the third visit, he referred us to a retina specialist.

Two days later we sat in another office hoping for some answers. This doctor was very nice and thorough. At the end of the visit he said he needed to be honest and tell us that he really did not know exactly what was going on in Riley's eyes, but that they were definitely both affected. Since Riley's eyes were not improving he wanted to take Riley's case to a National Retina Conference in New York over the weekend and get the opinions of some more retina specialists.

He called me on my cell phone the next day and asked me for permission to present Riley's case to an audience. I agreed.

The following Monday he called to say that he presented Riley's case and it was believed that Riley may have Coats Disease. We were referred to Emory and given an appointment a few months out. The wait was very difficult.

Once at Emory, we met Dr. Hubbard. He examined Riley and within minutes diagnosed Riley with Pars Planitis in both eyes. He used instruments to view Riley's eyes like no one else had before. He took pictures of both eyes so that we would have them for comparison later.

He explained to us that pars planitis affects the pars plana in the eye. If you were to stand inside of the eye and look out from the inside you can see the ring of color around the pupil. The ring around that is called the pars plana. Whenever pars planitis is present, the pars plana produces balls of infection called "snowballs" The accumulation of infection creates a "snowbank." Snowbanks can block the vision or even cause the retina to detach. It can also cause sudden blindness. The cause of pars planitis is unknown and no cure has been found. Treating pars planitis is an on-going process.

From there we were told that Dr. Hubbard wanted to collaborate with some other doctors before making a decision on treatment, because of Riley's young age. Generally this disease strikes in adulthood.

A week later he called with a plan. He wanted to place Riley under general anaesthesia and inject his eye with a steroid shot to help to fight the infection. After the infection was at bay we would know how much damage had been done in Riley's right eye. This procedure was scheduled for November, the week of Thanksgiving.

The procedure itself went well and we went back in January to see if the steroid shot had done it's job. Dr. Hubbard was not pleased, but recommended that maybe we should wait a few more weeks to give the steroid shot time to work.

In the meantime, Riley saw a pediatric ophthalmologist who started Riley on patching his left eye for one hour each day to strengthen his right eye. He had a case of lazy eye.

A few days later Dr. Hubbard called and said that he had met with a team of specialists and they had a very aggressive plan to present. It would be difficult for Riley, but may save his vision. If we chose not to go through, his retina was likely to detach. He wanted to do three more procedures on Riley's right eye, each six weeks apart. The first was in February, one in late March and the last in May. The first two would include the same steroid shot with the addition of cryotherapy. Cryotherapy is the freezing of the eye. The hope was to kill as much of the infection as possible.

The last procedure would be to surgically remove the layer of infection that had hardened over Riley's macula causing a macular pucker. As it was, when Riley looked out of his eye it was the equivalent to trying to look through a white dryer sheet. He could see some light and specks of color, but mostly the white infection in his eye.

The next two procedures went well. We heavily depended on the love and support of our family, church and friends during this time. They provided financial support, prizes for Riley, meals and emotional support.

In May 2010 Riley went in to have the last surgery, but was sick with an upper respiratory infection, so surgery was rescheduled for June.

In June, Riley did have the surgery to remove the macular pucker from his right eye. He was given additional steroid shots in his eye to prevent infection and to kill the infection that still lingered in his eye. After the surgery he struggled with chest pains for some time and was in recovery longer than expected. He was released to stay in a local hotel room and went back the next morning to have his eye patch removed. Everything looked great. He was released to go home (3 hours away).

A few days later Riley's pressure in his eye sky-rocketed to 50. (Normal is between 9-21). Unaware of what the problem was we took off to Emory. Dr. Hubbard was concerned with the high pressure and we decided to stay a few days so that he could recheck it. He was prescribed two pressure drops to use twice a day. We went home a few days later with everything back to Riley's state of normal.

In February 2011, Riley developed glaucoma. Glaucoma can be a side-effect of the steroid shots, but in Riley's case, it is now believed that he just has glaucoma, not as a result of the shots. He has also developed a cataract, which is a side effect of the surgeries. Riley will continue to develop cataracts for the remainder of his life. He is on three drops to keep his pressure regulated at this time.

In August 2011, Riley's check-up showed his vision in his left and right eyes has decreased. In the right eye the decline has been steady, but the left eye took a significant hit. The reason for this is contributed to his eye disease although no additional infection was found.

In February 2012 Riley's vision has decreased to 20/400 in the right eye and 20/100 in the left eye. His pediatric opthalmogist has indicated that he can no longer be of help to Riley. He will remain on board for emergencies, pressure checks and vision updates only. We left without a follow-up visit. Riley's eye had suffered significant damage from Pars Planitis and his vision is deteriorating rapidly. July 2011: Eye disease progression continues. The cornea in the right eye now displays some damage.

April 2013: Riley has APD added to his list of concerns in his left eye. HE also has some calcium build-up on his cornea and more inflammation and snowballs.

June 2013: Riley's right eye has taken another hit from the disease. His vision is decreasing. He has developed some swelling in the back of his eye that is posing threat to his retina as far as detachment. There are many other new concerns as well, so many that I can not remember them all, so surgery has been scheduled for July.

July 2013: Steroid injection and cryotherapy. We were advised after surgery that he would likely need this repeated 2-3 more times for it to be effective in light of the trauma his eye was under.

August 2013: Returned for surgery follow-up. The procedure was effective and his vision in his right eye had greatly improved. The edema had decreased and the was overall pleased. Enough so, that he released him for three months.

November 2013: Riley returned for his second post surgery follow-up. He received the best report he has had since the onset of this disease. His eye was pictured as almost completely clear and his vision was the best it had been in five years. Next visit scheduled for February 2014.

November 2014: Riley's eye continues to slowly decline, but in a fashion that is considered "stable". The "life" of his last steroid injection is expected to expire soon, so he is currently on a plan to return to Emory every three months to keep check on his eye due to the increased risk of a flare up.

May 2015: Stable! Yippee!

June 2015: Some vision loss in right eye. Corrected by new lenses. (Riley turned 15 this month!)

March 2016: Eyes are stable with some notable growth on the cataract in the right eye.

May 2016: Riley is examined by a neurologist due to some "ticking" that he is doing. Will return in December 2016 for diagnosis.

October 2016: No eye disease found! Damage still evident, but healing is also evident!

March 2017: Eyes still stable, no evidence of disease in the right eye

May 2017: Diagnosed with Tourette's Syndrome