My Wife's Cornea Transplant - Fuchs' Dystrophy

My wife has defective eye condition called Fuchs' Dystrophy, on both eyes, a very rare genetic cornea condition that required grafting. This procedure was performed about a year ago and the following is a write up of it. There's more of what she had to go through and I will post them here soon. This was one of the reasons I devote more of my time to her and less and less time being active in work and career as I mentioned in the Introduction yesterday.

RAM’S CORNEA TRANSPLANT

The day finally came. It's early morning, Monday 19th January, 2004. I did not sleep well the night before. I also noticed that Ram was tossing and turning most of the night. I guess both of us were silently praying for Allah’s guidance and providing us strength to face the event of the following day with confidence. We were praying that the event will go to pass successfully. We performed our morning ‘subuh’ prayers, Ram packed light clothing, toiletries and other essentials, had breakfast of cereals and some fruits, and got ready to go to the hospital. We left for the hospital at just before 9.00 am.

Four days earlier, on the morning of Thursday 15th January, 2004, Ram received a call from The Tun Hussein Onn National Eye Hospital (THONEH) that the operation on her right eye is scheduled for the afternoon of the 19th. She was to come in to the hospital at about 9.00 am. She should only take light breakfast that morning, after which she had to fast until the operation is performed. It was a short telephone conversation. We had questions and wanted more information, and decided to go to the hospital then. At the hospital we were told that a donor cornea is available from the US and allocated for Ram. She should come in as scheduled. She would be expected to stay in the hospital for a few days and that a room is reserved for her. Since she is already at the hospital would she like to get the necessary pre-operation tests done? No need to do them on Monday morning, they said. Ram agreed. All the tests were performed and their results were positive. Meanwhile I informed my children and other kinfolks of the impending operation. Siti and Rashidah (Ram's sisters) had previously planned to visit for the weekend and join us in a ‘tazkirah’ session meant for PETRONAS retirees that I was hosting at home. So also also our children, Tessa, Sita and Fahrul. It was too far for Shaffik to come home, we told him no need to, and furthermore it was the Chinese New Year holiday period and flights were mostly full. The ‘tazkirah’ session was indeed timely as we took the opportunity to have a ‘do’a selamat’ after the session and there were about fifty retirees present then. Siti decided to stay on until after the operation while Rashidah had to return to Johor Bahru as she had not planned to take leave from work beyond the weekend.

It was about twelve years ago, circa 1992. Ram was working in the Finance Department of University Hospital. She drove to work daily. She started work in the UH in July 1969 and had been in Finance all the time, starting as an Executive Accounting Officer and rose to be the Finance Manager until her retirement in 1999. In 1992 she was the officer-in-charge of billings and income follow-ups, policies and procedures and as secretary to the committee for waiver of bills. In this position she has to deal with piles and piles of papers and documents. She was also instrumental in developing computerized billings, working closely with the computer section and had to face the computer screen alot. She had had difficulties then to discern what’s written on the computer screen and had always put the blame on these difficulties to her glasses. Outside of the office she had difficulties reading billboards, maps, fine prints and the like, more so under the glare of sunlight or spotlights. Her vision had been cloudy under these conditions. She had her glasses remade and retested several times over the next few years but the problems persist. Once she drove through a stop sign at a road junction not realizing it until she had gone far beyond the junction. On another occasion she almost ran into a policeman directing traffic in the middle of a traffic light T-junction. Luckily the policeman just glared at her and did not do anything beyond that. All these were blamed on her glasses. In addition to all these she also, at times and in the beginning, feel pain in the eyes especially in the mornings. She felt as though her eyes were swollen but the pain subsided as the days progressed. As time went on the pain got more and more frequent.

On one visit to her regular optician to get her glasses checked, sometime in 1995, the optician encountered difficulties rationalizing the refraction in both her eyes. The optician advised her to see an eye specialist as she suspected there was something in her eye but could not identify what it was. She made an appointment with the eye specialist in UH, and on examining the eye confirmed that there was something in the eyes but could not identify what it was. Yet another specialist took a look at her eyes but they still could not diagnose the problem. Finally the eye specialists decided to bring Ram’s case to the departmental conference to discuss her case. An eye specialist from the Tun Hussein Onn National Eye Hospital is usually in attendance at these conferences.

On the day of the conference Ram was invited to be present. The THONEH specialist, Dr Pall Singh, took a close look at Ram’s eyes and confirmed that she has “Fuchs’ Dystrophy”. All the eye doctors present at the conference took turns examining her eyes. To all of them it’s something they have not seen before. From that day on, she had been requested to attend session after sessions of displaying her eyes for eye doctors to see. She had become a study piece!! It does not help her morale at all. She was often moody, phobic and stressed.

Fuchs’ Dystrophy, as explained by Dr Pall Singh then, was discovered by a German Doctor named Dr. Fuchs. It’s a slow, degenerative disease of the cornea and genetically inclined prevalent amongst Caucasians of age group 60 onwards. Not much is currently known to correct the disease nor is there a cure although there are medications that help slow down its degeneration. The pain she felt, in the mornings especially, is due to this disease. It is due to water being retained in the eyes. One way of relieving the pain is by running a hair dryer and blowing hot air against the eyes. However, as the disease progresses, the patient’s vision will deteriorate further and the eye will ultimately become blind!! To Ram it was a very scary thing to discover. How long will it take before she becomes blind? It can be one year, it can be ten, only God knows. What can be done when she is blind? “A transplant,” Dr. Pall Singh said. Transplant? Where can we get the eye from? Can I give her one of my eyes? What about compatibility, blood relationship, and many other questions were playing in our minds. I tried talking to my family doctor about Fuchs’ Dystrophy but he was not aware of the disease and promised to look it up for me. I tried the library, medical journals, the Readers’ Digest Family Medical Reference book, and whatever else I can get my hands on to learn more of the disease and could not get any. My family doctor also confirmed that he too could not get more information on the disease. Finally I went into the internet and there it was, one website that described the disease in two paragraphs!!

One does not see anything wrong on a person with Fuchs’ Dystrophy. There are no outward signs nor any indication that the eyes are diseased. Only through an eye doctor’s instruments can the disease be seen. It is the failure of the tiny capillaries on the cornea of the eye to release water. Water is retained in the cornea and vision is impaired. (Imagine looking at objects through water). As the disease progresses the cornea would appear as though it is peeling off. It is as though the capillaries have burst at the seams, much like when holes are punched in a tin can. The pain now will be severe. Simple tasks will be difficult to perform. Even reading will become a major task. The disease is also very rare, less than 1% of the worlds’ population has the disease and a majority of them are above 60 years of age. Studies on the disease have not been conclusive, no cure has been found, and for the time being only ways to relieve and lessen the pain are available. The only way permanent is a transplant of the cornea. When to perform the transplant will depend very much on the deterioration of vision; when it has detrimental impact on the person’s quality of life. More and more details on Fuchs’ Dystrophy has, over the years, been available on the internet and for those who are interested the site www.fuchs-dystrophy.com is one to visit.

Ram continued to see the eye specialists in UH and had eye drops prescribed to be applied daily. However she had to respond to requests after requests from the Medical Faculty Eye Department for her to be present now and then for eye doctors to view her eyes until she got tired of it. She even had to present herself at the eye doctors’ graduate examination as an exhibit or specimen!! Moreover the specialist at UH kept changing and each time she had to go through the same procedures all over again but ending with no expert advise. At the same time and as the days, weeks and months go by, she was encountering more and more difficulties doing her daily routine things. She likes reading but could not read properly. She read the newspapers but only discern the headlines while the text appear like patches of black ink. Sometimes her vision is so bad that she felt like looking through her glasses smeared with a layer of Vaseline or oil. She once missed pouring a spoonful of milo into the cup, when making the drink, spreading the milo on the floor instead. Cutting her fingernails would always be a feel and cut job. She would not be able to see where to put the nail clipper before cutting. She decided to shift to The Tun Hussein Onn National Eye Hospital in early 1999 to consult Dr. Pall Singh directly as his patient. The slow deterioration of her eyes and vision continued until the middle of 2003 when she was almost blind in her right eye. She was referred to a cornea specialist, Dato’ Dr. Veera Ramani, who confirmed that she needed a cornea transplant and from then on continued to be under her consultation. Ram was registered as a donor recipient with the International Eye Bank. Reality began to slowly sink in our minds. The inevitable will take place.

So it was, in the morning of Thursday, 15th January, that confirmation of the inevitable arrived through that phone call. On Monday, 19th January, Ram and I arrived at THONEH at about 9.15am, registered at the ward on level 2 of the hospital and was assigned to room 213A, a single room but with an extra bed available for a boarder. A single room was advised and preferred so as to have some isolation and also to avoid contamination of the eye after the operation. The nurses started preparing Ram for the operation; eye lashes of the right eye were removed, eyebrow trimmed, blood sugar level tested, while other necessary tests prior to the operation had already been done a few days ago. Ram was then ready for the operation and we waited. Siti, Tessa and Sita came to the hospital at about 11.30am to lend support to Ram, and indirectly to me. Ram’s doctor nephew and niece, Anis (Dr. Salleh) and Fizah (Dr. Sabariah Faizah) with husband Md Diah also came to lend support. Tessa, on her way to the ward met Dr. Sanjeev, a close friend of hers. He is understudying to be a cornea specialist under Dato’ Dr. Ramani. He has seen Ram once and is aware of the scheduled transplant but did not know who Ram was until meeting Tessa. We were wondering why we were still waiting in the ward when it was well after lunch time. Dr. Sanjeev explained that they were still waiting for the DHL courier service to deliver the cornea from KLIA; the plane carrying the cornea having landed in KLIA in the morning. From then on Dr Sanjeev was more than helpful and dropped in to see Ram as often as he could.

At 3.15pm, the nurses came and asked Ram to proceed to the operating theatre. All of us trooped in behind her into the lobby of the operating theatre, said our last words, encouragement and prayers for her and then left the room. Anis left for his office (he was working) while the rest of us waited. We felt hungry and went for some food in the canteen located in the basement of the hospital. Eat? What did I eat? I cannot remember what I ate. I was more thirsty than hungry. My mind then was in the operating theatre. We later went back into the room and waited. They wheeled Ram back into the room at about 5.45pm. Her right eye was bandaged and patched-up. She was conscious and obviously quite sedated still. Her face disheveled, lips dry and she complained of a headache. The nurses changed the bandage on the right eye and replaced the patch. A little while later Dr Sanjeev came to see her and prescribed something to relieve her of the headache and pain. She was given porridge to eat but vomited it all out after eating; after effects of the sedation I guess. All family members, Siti, Tessa, Sita left at about 8.30pm, including Fahrul who came just before Maghrib from his office meeting. Faizah and Md Diah had left a little earlier. I stayed on to accompany her. Afraid that I may not be able to immediately respond to her needs during the night, I moved the single chair next to her bed, sleeping in it instead of the spare bed. The nurses came in twice to clean her eyes, change dressings and administer medicines that night. Ram, restless the whole night, slept little and so did I.

The next morning Dr Sanjeev and Dato Dr Ramani saw Ram in the consulting room. She explained that the operation went on smoothly and the new cornea, a very good cornea, is set very neatly in its place. They say it looks very good and do not expect any problem. There is however some swellings on the eyelids and the eye is red but these will go off in due course, according to the doctors. Ram is to be cautious though, and not exert herself. She should not bend forward, nor carry heavy loads, nor do strenuous work and not exert pressure on the right side of the face. Neither should she wash the eyes with ordinary water for the time being. The eye is to be protected from dust and contaminations, and a plastic eye shield was provided for this purpose. It is to be cleaned using saline solution and three types of eye-drops were prescribed to be applied 4 times a day.

On Wednesday 21st. January 2004, after the Doctors had seen her in the morning, she was allowed to go home with a list of precautions she has to take. So began the routine of cleaning her eyes and applying the medicine and three types of eye-drops every six hours. To avoid contamination, all utensils used for cleaning the eye have to be cleaned with disinfectant soap and placed in a covered receptacle all the time together with the cotton wool and cotton buds. The hands should be regularly washed with soap and especially when cleaning the eyes.

Subsequent visits made to the doctor in the hospital showed positive improvements in the eye. Focus and vision slowly improved. However, according to the doctor, full recovery may take up to one year. The sutures may be taken out in stages in about four to six months and meanwhile she has to continue taking the necessary precautions. One week after operation she could not read anything but three weeks later she could read the letters on the second line of the eye refraction-chart. She could read the headlines of newspapers but the full texts are still blurred. Focus is slowly taking place. Vision on the right eye as compared to the left eye is bright, as though a spotlight or torch is directed to what she sees. Does this mean that her vision has been dimmed all these while?

Ever since returning from the hospital there had been a steady stream of visitors, relatives, friends and also neighbours to see her, comfort her and wishing her steady recovery. Afraid that she may get overly excited receiving these visits and thereon exerting herself in explaining the operation and the eye condition, I printed out brief descriptions of Fuchs’ Dystrophy and related pictures of the part of the eye that is operated on and how the new cornea is sutured, for all to see and read. I kept reminding Ram to speak little, and not move about too much. She did very well and the visitors understood the need for her not to move about as best possible. The visitors helped uplift her morale as well and did good for her wellbeing. Shaffik and family came home for the Hari Raya Haji holidays, 10 days after the operation, and the sight of the grandchildren, lifted Ram’s spirits. “Kesian Tok Mi, eye painfullll….” Said Hifzhan…. And you can certainly see the glow in the grandmother’s eyes….

She is now in the fourth week after the operation. The swellings in the eye, the redness on the eyeball and the pain in her head and temple have subsided almost completely. The eye appears to have a ring around the edge of the cornea and taking a closer look at it, the sutures can be seen quite clearly on the ring, 360o around the cornea. It is neat and even. Frequency of cleaning the eye, of applying the eye drops and taking the prescribed medicine is maintained, while the number of eye drops to be applied can be reduced to only one type after the fourth week. She has to continue to be cautious, not to exert herself, not to carry or lift heavy objects, not to bend forward, in short, not to strain herself in anyway for yet some months. She is well, except for the eye, has good appetite since she is at home all the time, cannot exert therefore cannot exercise, all these does not appear good for maintaining her body weight!! I keep reminding her to watch what she eats. We do not want other problems to creep in do we?

We still have many questions in our minds about Ram’s condition for which only time will provide the answers. Questions like, will the cornea adhere completely to the eye in within the period mentioned? (We did hear of a case where the cornea came off when the person accidentally touched the eye, and that was after 5 years of transplant!!) What happens if it does not? After all there are no blood vessels to assist!! What about the left eye? The left eye is also afflicted with Fuchs’ Dystrophy. Will another cornea transplant be necessary?

The donor? …… All we know is that the donor is from the US. It is not the practice of the International Eye Bank to release details of the donor. It shall remain anonymous. There was an earlier allocation of a cornea from Canada for Ram during the Christmas/New Year holidays but it was not feasible then as there were delays in the flights out of the US/Canada during that holiday period partly due to the restrictions imposed by the US Government on flights into and out of the US as precautions against terrorism and also partly due to the repeat outbreak of SARS. These delays would only make the cornea exceed its permissible exposure limits rendering it to be clinically dead by the time it arrives in Malaysia and hence not acceptable for grafting or transplant. The doctors decided not to ship out the cornea then. We hope that that cornea found a beneficial recipient in the US or Canada.

We thank Almighty Allah for His blessings, His guidance to us in making the decisions we made, His compassion towards providing us the comforts in these stressful times, and most of all His allowance to Ram, for the strength He gave her to face these trials and tests. We continue to seek and to pray for His guidance to us to face the trials and tests with humble allegiance and confidence, instilling and maintaining in our hearts the will to be humble to Him and the will to accept whatever He bestows on us with rightful thanks and ‘syukur’. To Allah we owe our allegiance and from Him we seek forgiveness.

Kampong Tunku,
Petaling Jaya.
February 15, 2004