A UDS / VKH EXPERIENCE

The GHC would like to thank the owners and Bea Pitts for allowing us to use this article on our website.  This dog lived in Great Britain.   This is a factual report of events relating to a much loved, young male Akita suffering from Uveo-Dermatological Syndrome (often referred to as VKH), and is written with the full knowledge and collaboration of the owner in the sincere hope that it may be a step towards a greater understanding of the problem.  ©Bea Pitts, abridged version used with permission of the author. 

By Bea Pitts

The story starts with a much loved, very fit and apparently healthy 19 months old male Akita who was full of vitality and enjoying life in the company of a spayed female Akita and two Rottweilers. 

About the time of his first birthday, in the spring of 1997, he started to blow coat and, as was the case with many other Akitas, continued to do so for several months throughout the summer, thus curtailing his show career temporarily.  No other health problems, other than a bout of enteritis were reported.  

21 September His owner was offered the opportunity to share a holiday with friends going to the USA.  

22 September - 23 September For the first two days of his owner’s absence the male Akita  was reluctant to eat normal meals.   However, tasty supplements and natural hunger eventually overcame the problem and two days later he was eating and playing normally again.  

ON SATURDAY 27 SEPTEMBER (DAY 1)  Caretaker noticed Akita had “red eyes”.   Severe conjunctivitis was diagnosed and chloramphenicol drops prescribed.  ( DAY 3)  Caretaker horrified to find Akita  with huge “staring” blue, milky eyes. He was very obviously unable to see

(NOTE:  At this point I think I should add that I have known and been closely associated with this puppy since he was only a few weeks of age and I have seen him at least once almost every week of his life since then. As a result he has always greeted me like a member of his own family, either mugging me for biscuits in my pocket, or piddling all over the yard in his excitement to see me. The reader can therefore imagine my own concern when the dog I met on the vet’s car park was not only unable to see me, but neither did he recognize my voice, or the scent of my hand when I placed it right over his nose.Yes, he was very confused and probably frightened too. His nose was very dry and felt rough like sandpaper, and he was losing pigment around the edges of his mouth – previously black flews were now a pinkish-liver in color. I did not want to believe it then, but I suspected I recognized these symptoms). 

Our vet was equally shocked by the rapid deterioration in the Akita's eyes and after lengthy examination was of the definite opinion that this animal was suffering from Uveo-Dermatological Syndrome. For this he prescribed Prednisolone 5mg strength 4 tablets twice daily and continuation of drops as previously prescribed, with a request for return for further examination on Day 7.

“The VKH syndrome in human beings (Vogt-Koyanagi-Harada Syndrome) is a strange disease in which there is whitening of hair (poliosis), depigmentation of skin (vitiligo), internal inflammation of the eye (uveitis), painful hearing and meningitis. It is thus sometimes referred to as “uveominingoencephalic syndrome” and it primarily affects 30-40 year old people with black or heavily pigmented skins. The cause is not completely understood, but auto-immune reactions to pigment containing cells, nerve tissues and retinal cells are considered to be important. Neurological signs usually precede the uveitis, and blindness due to retinal detachment may occur. The uveitis may persist for years, and cataract (lens opacity) and glaucoma (high fluid pressure within the eye) will cause blindness in such patients. Alopecia (loss of hair), poliosis and vitiligo develop 2-3 weeks after the uveitis has appeared. Treatment is difficult – in short, VKH is a rotten disease. “A similar disease occurs in dogs, pigs and chickens; the list of dog breeds involved includes the Samoyed, the Irish Setter, the Golden Retriever, the Chow-Chow, the Shetland Sheepdog, the Siberian Husky, the Old English Sheepdog and the St Bernard, as well as the Japanese Akita. The neurological features of human VKH are not seen, the lesions being restricted to the skin, hair and eye (vitiligo, poliosis and uveitis). The term VKH is thus not entirely appropriate, and “uveodermatological syndrome” is preferred. The incidence of the disease would appear to be increasing in the USA, and [at the time of writing in 1990]it has occurred in the Japanese Akita in the UK. The incidence figures are unknown, but currently it would appear to be of rare occurrence.” PGC Bedford

Bear in mind that throughout this time the owner was away in the United States but had been informed, long-distance telephone calls were routine.

(DAY 7)  It had been really heart-breaking to see this dog:   He had been confused and blind, and was therefore walking into things.  Hitting the problem hard and fast with the steroid treatment had obviously done the trick, because when we returned to the vet, the blue milky stare was completely gone.   The Akita's sight appeared to be totally restored and he was getting back to his old self again.   The vet decided to continue the drops for one further week only, and continue the steroid tablets on the high dose for one more week, then reduce to half the dose for the second week, by which time mum would be back home again.

The good news was communicated to Professor Peter Bedford, whom we had called  previously.   Arrangements were made to see him at one of his clinics in a few weeks time.

“It is important to remember that canine patients undergoing treatment for auto-immune disease are very often not going to be cured but rather have the clinical signs of the disease suppressed by use of the drugs. The aim of this palliation is to ensure a reasonable quality of life for the dog, but a great deal of owner dedication is required in the management. Some cases, e.g. rheumatoid arthritis, will not be suppressed for very long.

“The drugs used are themselves not without side effects; many will reduce the dog’s ability to deal with minor surgery, make the animal more susceptible to other infections or produce side effects which cannot be controlled and therefore make their use unsuitable in that particular patient. Regular monitoring is essential for the dog’s well-being. Veterinarians will advise regarding suitable monitoring regimes. It is really important to observe these and to take your dog back to the vet when requested to do so.  

“Postscript: Without doubt our knowledge of the role of the immune system in the progression of many diseases is still in its infancy. As our knowledge increases so our attitudes to the prevention and treatment of disease is going to change rapidly. ”Veterinary Notes for Dog Owners, edited by Trevor Turner.

( DAY 21)  The Akita's eyes were still clear, and his sight appeared to be good. In fact, apart from slight loss of pigmentation on the end of his nose, and more general loss of pigmentation around the edges of his mouth and gums, the dog seemed fit and well.  He was certainly a happy dog!

 One of the side effects of steroid treatment is that the animal will drink profuse quantities of water, and consequently will pass similar amounts out of the other end.  His coat was not at its best either.  It now felt quite coarse and lacking in substance. He was beginning to lose muscle tone and body weight too, yet he was eating with a ravenous appetite, scavenging for any scraps which he could find in the house to supplement his normal meals.

 The vet was very pleased with this Akita's progress, to the extent that he prescribed the half dose reduction of the steroid tablets to continue for a further two weeks.

( DAY 31)  It was noticed that the conjunctivitis seemed to be returning, so a quick phone call to the vet confirmed that the steroids should be increased back to the higher dose, recommence the drops, and the Akita was to be brought in the next day.  By now the loss of pigmentation which had been noted at the end of his nose had returned to its normal color, but the flews and gums were now a very definite pink.

(DAY 32) The Akita is again blind!   With large, “staring” blue, milky eyes, and he was now using his nose to find his way from the car!  An obviously dismayed vet told us that both retinas appeared to have detached and an urgent referral to an Eye Specialist was needed.

 It was felt that the BOA Hospital and the Animal Health Trust would be unlikely to be able to accommodate us at such short notice, and that Hereford was a geographically better option than Bristol or Liverpool.

“The disease is not life threatening, but blindness is not unusual. The UV varies in severity and prompt diagnosis can result in relatively effective treatment. Acute blindness is due to retinal detachment, whereas chronic uveitis can lead to blindness as the result of cataract formation or the development of glaucoma. Poliosis and vitiligo usually begin within 2 weeks of the uveitis, but it may take several months to develop. Poliosis involves the face, and the eyelids, the nose, the lips,“the scrotum and the footpads may be involved in vitiligo. Occasionally, the vitiligo may involve the whole body, and the depigmentation areas may ulcerate. Loss of hair may occur, but not all patients will be involved.” PGC Bedford

During the course of the day we all noticed that the blueness which had existed on our arrival, seemed to be visibly fading. It was thought that this may have been either because the lights had been turned out during some of the tests, or as a result of the drops used in the tests.

Similarly, it was explained that whilst it may be thought that the disease was under control and stabilized, it can, and does, recur (or “peak”) when least expected. When the disease is “under control” it is possible to gradually reduce the steroid dosage to what could be described as a steady regular top up dose, thus reducing the continued side effects and allowing the resumption of normal function of organs and glands generally. However, at “peak” times the only course of action is to again hit hard with steroids.

“As with man the precise nature of the cause of the disease is unknown, and treatment can be difficult. Prompt diagnosis is essential and topical and systemic corticosteroids are usually used. Long-term treatment is often essential to prevent recurrent attacks, and other treatments involving immunosuppressive and cytotoxic drugs may have to be tried. "There is a lot to learn about this disease, and once the cause is established an improvement in treatment will be possible. The literature indicates a breed disposition but the mechanism of inheritance, if any, remains unknown. Awareness of the disease should lead to prompt treatment with, hopefully, the prevention of blindness. At this moment the avoidance of affected animals in breeding programmes is a sensible step.”  PGC Bedford

We had also begun to notice over the past two or three days that when the dog was sitting beside you he would lean against your legs and literally close his eyes, as though resting them against the light.  During all of this time one of the side effects of steroid treatment had become more and more apparent. The animal will drink gallons of water, and consequently gallons will come out of the other end.  

1 NOVEMBER The Akita  was obviously in great discomfort. He had cried all night and his owner had kept getting out of bed to let him go outside to relieve himself. His bladder was full but nothing would come out of the other end, no matter how he tried and he kept crying. With human assistance in the form of gentle massage with the finger tips, the waterworks began to operate again.  Poor soul, he had been full to bursting.

Another urgent early morning visit to the vet, together with a urine sample, to confirm that he had now developed a urine tract infection. The PH and protein levels were high and the emergency vet felt that a 24 hour stoppage of the steroid tablets, would allow a massive injection of antibiotics to clear up the urine infection and ease his bladder. He injected chloramphenicol, which is not commonly used in injection form nowadays, but it was felt to be the best and most effective alternative in view of current medications.

(DAY 37) His bladder was now operating normally but a further urine sample showed high alkaline content. A further antibiotics injection (chloramphenicol) was administered, and directions to refrain from steroid tablets until next day, but continue with eye drops as before.

The early morning low cloud and drizzle had not lifted at all and fading daylight was making visibility difficult for driving. However, after a quick call to stop the vets going home, we were off again to be told that he had developed Addisons disease. He was suffering withdrawal symptoms from the sudden withdrawal of steroids and his adrenal system had not kicked back into gear-- Addison's disease. He had a temperature of 102° and a rapid heart beat.

Apparently Medrone is a steroid which can usually be given without the necessity of reducing the dose gradually before coming to a stop. In this case, however, it had been given as a continuation of treatment immediately following on from large doses of Prednisolone, and had been given in one large dose rather than being split into two doses in the day. Therefore the sudden stoppage, albeit temporarily, had meant the adrenal gland had not begun to function normally.

 We were told to watch the dog carefully, as there was always the possibility of him becoming comatose. If indications to this effect were noticed, then he must be taken back to the vet immediately. Tests would be taken at such a stage and thyroid could be included at that time.

 In view of the happenings of the weekend, our vet decided to put him back onto the Prednisolone 5mg, 4 tablets twice daily, and also Synulox 3 tablets daily for 10 days to control the urine infection.

The vet explained that the dog’s own immune system has now been totally shattered by the high dosage of steroids and that infections, such as the urine one experienced over the weekend, can now be expected, as there is now very low natural resistance.

SATURDAY 8^TH NOVEMBER (DAY 46) The owner reported that the Akita was playing happily with his girlfriend and that his eyes had a normal healthy appearance.

Supplements of cod liver oil and multi-vitamin capsules had been added to his food over the past two weeks and there was a very noticeable difference in the quality of his coat now.

(DAY 48)    Return to see the specialist at Hereford. We were asked how he had been during the intervening weeks and we explained about the setback with the urine infection the previous week.  Numerous tests were carried out as on our previous visit. When all the tests were completed we were relieved to be told that there was no doubt his retinas were beginning to reattach.   

It was decided that the steroid tablets should continue on the dosage of 4 tablets twice daily. Also the drops (Pred Forte 1%) six times daily to both eyes were to continue. However, Atropine 1% drops were now prescribed twice daily for both eyes. In addition to the above, it was also decided to give the Akita diuretics, to speed up the reduction in the inflammation. Frusemide 40mg daily was prescribed and the Synulox was to continue for a further ten days against the urine infection.

It was also suggested that we might consider giving Aloe Vera to him.

We asked about the long term prognosis for him.  For instance, if he did eventually become permanently blind, what would be his expectancy of quality of life?   It was explained that the disease would continue to attack the mucous membrane parts of the system – e.g. eyelids, nose, mouth, anus etc. It would be necessary for him to remain on steroids, hopefully a reduced dose, for the remainder of his life. If the steroid treatment were stopped when total blindness was diagnosed, this would not prevent the continued degeneration of the eyes and could more than likely result in glaucoma, of a very painful nature.

(DAY 49) The vet was certainly pleased and relieved with the progress of the Akita and confessed he could see a marked improvement in the general appearance of the dog before he even examined him.   Throughout the course of the following days there was absolutely no doubt whatsoever that the sight of this dog was gradually returning. Not only was he a happy dog, but he was actually playing with toys again and could see where they were. He would also look up into the trees where the squirrels were playing. He was no longer bumping into things but was able to avoid objects in his way.

  As a result of his loss of body weight the Akita's spine has seemed very prominent, and his head had lost any flesh that might have been there, so he appeared to have a very bony skull, particularly on the top between his ears. His coat was also in bad condition. His owner had continued to give the dog cod liver oil capsules, and had been adding freshly cooked pasta to each of his normal meals. The results were unbelievable. He had bodied up again (without being fat) and his coat was blooming. In fact, he looked the picture of health, apart from his nose and his spectacles.

26 NOVEMBER (DAY 64)  The dog appeared to have normal vision, was his usual mischievous self, but had a very definite crusty nose end, and slightly paler pink spectacles. On examination, vet confirmed that the retinas were almost totally reattached again. He said there was definite damage to the retina, which had been totally detached, but the one which had remained slightly attached throughout, seemed to be in better condition.

 The vet also confirmed that the nose was ulcerated and that his owner could only continue to treat as before, by applying the Aloe Vera product as she had been doing, though he personally did not think it would do much good.

8 DECEMBER (DAY 75)  The day of our scheduled visit to the specialist at Leominster and we were both optimistic that the outcome would be good. In recent days the bright pink of his “spectacles” and the “new” nose had begun to change to a liver color. His eyes were no longer bulbous and staring, but were back to the size and shape we all know and love in a “normal” Akita.

 We were told that the retinas were now completely re-attached but that they were slightly thinner than previously. We were told that his sight is now approximately 70-80% of what it should be if normal, but if he were a human and had had detached retinas for as long as this dog, there would be no sight at all.   She  decided to stop the Frusemide diuretics as there was a risk of developing glaucoma if too much fluid was removed from his eyes.

 The Atropine was to be stopped for the time being as he was showing no pupil response and his pupils were permanently dilated. The vet showed us how to use a small torch to see the bright green of the large dilated pupil. As long as it showed so large then no Atropine should be used. If however the pupils became small again then use Atropine once every three days or so to force them to dilate and make the muscle work again.

 The Pred Forte drops were to be reduced to four times daily instead of the previous six times daily. Ultimately, the ideal dose would be a half or one tablet on alternate days, but this was some time away, yet. 

We were warned that there was always the possibility that the problem would peak again, and if that were the case then immediately the tablets should go back up to 8 per day, and the drops return to six times daily.

On our previous visit, it had been suggested that we might consider using Aloe Vera which is proving to be an effective anti-inflammatory natural product which does not conflict with traditional medications. I had therefore written to a veterinary advisor retained by an Aloe Vera producing company for his recommendations. In his reply he confessed to having no experience of UDS in dogs, but had seen many cases of recurrent uveitis in horses (periodic ophthalmia). From his experience and correspondence:

"The main Aloe Vera preparations used to help conditions of the eye are dilute Aloe activator or dilute Aloe veterinary formula. We tend to dilute the Aloe preparation 50:50 with sterile water and then add 2/3 drops to the eye 4/6 times per day. At the same time I add up to 60mls of oral Aloe Vera gel to the diet each day. The benefit of the Aloe preparations is that it appears to be naturally anti-inflammatory and does not interfere with any other medication which is being used.”  David Urch, BSc MA VetMB MRCVS

 Throughout all of these problems the Akita still from time to time had bouts of diarrhea, but we knew that this was part of the side effects of the medication he was receiving, so a good stock of Kaobiotics was always maintained in case the situation worsened when it was not convenient to see a vet. 

10 DECEMBER (Day 77) Formal examination and closer inspection with the various ophthalmic instruments confirmed that his retinas were completely re-attached and whilst there was obviously slight damage resulting from the detachment, our own vet agreed that he had no doubts that the Akita was stabilizing nicely.

 Tablets and drops were again ladled out and a request to return again in two weeks time, for a further check-up before Christmas.

22 DECEMBER (DAY 89)  Another trip to our vet where we were greeted by:  “Hey, look at his eyes! Aren’t they great. He looks fantastic!” There was absolutely no doubt that he was pleased with what he saw even before he gave a closer examination. It was confirmed that the retinas had definitely returned to normal position, and that whilst there was obviously some damage as a result of the detachment, this was not as bad as might have been expected.

 His owner explained the agreement with the specialist that the Akita's steroid tablets be reduced by one to 6 per day from 1st January and by a further one per day at two weekly intervals thereafter. The “spectacles” had now become a monocle which looked a little odd, but since the pink was now slightly more liver in color it was not quite so remarkable.

 In fact his general appearance was so good that he received lots of admiring comments and glances whilst we were in the reception area waiting for our vet. It was on this particular visit that we showed our vet a copy of the article we had seen about a new auto-immune problem in the States – Lymphoplasmacytic interfacial dermatitis (from a Shiba Inu publication in the states regarding theories of pre and post Hiroshima breedings)  He read it through very carefully and expressed a great interest in the information, asking if he might keep the copy for his own records.

19 JANUARY (DAY 117)  For our routine visit and he was obviously very pleased with the progress and confirmed that the retinas now seemed stabilized and we should return in one month’s time, continuing to progressively reduce the tablets as previously instructed.

4 FEBRUARY (DAY 133)   The tablets had been reduced to four per day and the Atropine drops were being used only every third or fourth day.  We agreed that the safe course of action would be to now reduce by half of a tablet at each interval, because this had been the crucial dosage when things had gone wrong previously.  

Both his owner and I had agreed some time ago that we did not think he would last until Christmas, and here we were on SUNDAY 8TH MARCH 1998, 165 DAYS later with a dog able to celebrate his second birthday.

Lots of tender loving care has been lavished on this dog, he has been carefully monitored not only by his owner but by vets who, by their own admission, were not very familiar with the problem at the outset, but who were determined to save the sight of this beautiful Akita.  

AUTHOR’S POSTSCRIPT:

We all say we know our own dog and this was no exception. Everything had been as normal, absolutely nothing had changed, the Akita appeared to be fit and well.  His owner started to administer the eye drops as usual to find his eyes were “bloodshot” again. We both agreed that since it was a Sunday, we would follow earlier advice. The drops and tablets were stepped up again and we took him to the vet again on MONDAY 30THMARCH.

 Needless to say, our vet confirmed our suspicions that the inflammation had definitely returned, though fortunately at this time the retinas were still attached. He agreed that the tablets should be increased to 4 twice daily and that the Pred Forte drops also be increased again. He recommended that the increased medication continue until the Friday when we should return to confirm that a reduction of medication at four day intervals could resume, until we achieved the level we had previously achieved at the beginning of March.

THE EXPERTS ARE RIGHT – NOBODY CAN AFFORD TO FEEL COMPLACENT ABOUT UDS/VKH.

ADDENDUM:

After many sleepless nights of soul-searching, this grand Akita's owner eventually came to the decision that the time had come to end this boy's suffering, and on Monday 15th February 1999 (one month before his third birthday) we made that tearful, final journey to the vet.

He had started to develop cataracts on both eyes. His body was covered in small, lumpy calcium deposits, which the vet had explained was a direct result of the high dosage of medications, and is known as Cushing's Disease. The once beautiful coat had been progressively shedding leaving large bald patches where it refused to grow back. What remained of his coat had become quite coarse and gave the impression of total neglect.

The dog had gradually lost so much body-weight and muscle that he was now only a shadow of his former self. He had become totally food and drink obsessed and would steal absolutely anything if it smelled of food. On one occasion he actually stole and ate the plastic feet off the bottom of the electric sandwich toaster. This action was not normal – it was the disease taking him over.

Our vet agreed that no matter how guilty his owner felt about making the decision, she had made the right decision at the right time. The dog's body could not take any more medications. He had always told us that the Akita would die from the medications before he would die from the disease. We all agreed, however, that before the euthanasia was administered, we wanted a blood sample to be taken from him, to be donated to research to find the DNA marker for this awful disease. This he did with his last breath …. …. ….  

Updated on 09/18/2008