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Dirk
By Chris Chapman

In October of 2001, when my first Deerhound (Dirk) was about 14 months old, I heard about the problems caused by cystinuria in Deerhounds. I also heard that the U. of Penna. had a screening test for the defect, so I submitted a urine sample to Penn. That’s how I found out that Dirk excretes cystine in his urine and how he became part of Dr. Paula Henthorn’s study.

After my initial panic subsided, I started my research. Christie Keith’s website and email discussion group became a major source of information on what I could expect and what others found had worked for their dogs. I decided to take a very conservative approach to Dirk’s situation. I did nothing more than to be diligent in my observations of his ability to urinate properly and to encourage him to drink more water to dilute the cystine in his urine.

On-leash walks became our hallmarks for each day: A morning walk before I went to work, first thing done when I got home and the last thing before bed, all to be sure that he could urinate. In between those walks, of course, we did lots of wonderful things together. Romps in the woods, dog shows, obedience classes, lure coursing, watching TV, and body slams are all things we shared. I preferred the TV watching. Dirk held a preference for body slams a little longer than I did. He lived a wonderful, healthy life. No signs of blockage or urinary tract infections, no blood tinged urine. No sign of any urinary problem at all. If I hadn’t had him tested I would NEVER have known.

That situation held for more than 4 years.

Then on Wednesday, June 28, 2006 while on an afternoon walk in the woods, I noticed Dirk taking an awfully long time to urinate and he was looking up at me with a “Mom, this isn’t right,” look on his face. His urine stream was virtually non-existent. He was passing urine but only in a long session of drop-by-drop with straining. So, off to the Veterinary Hospital of the University of Pennsylvania’s (VHUP) Emergency Room we went.

Once there, he was triaged immediately and the vet tech determined that his bladder was small, so the urgency of his case decreased slightly. After taking his history and doing their physical exam, the possible diagnoses were: 1) blockage by uroliths (stones), possibly/probably cystine stones, 2) a mass at the outlet of the bladder, 3) urinary tract infection (UTI). He was catheterized and the catheter passed easily, hinting that it might be a mass or an infection. Blood and urine were submitted to the lab. I was offered the option of doing ultrasounds and/or radiographs to check for stone or masses. Since his bladder was small and he had urinated normally that morning, I opted to wait until the already submitted urinalysis (UA) and culture came back. I knew from my research that cystine stones are often not seen on radiographs and sometimes don’t show up on ultrasound. The UA would be in the next morning, the culture in several days, and if a UTI was the problem the expensive imaging wouldn’t tell us anything at all.

The next day, Thursday, Dirk was fine. He got to come in to work with me for extra walks and observation, had no trouble urinating, no blood in his urine, happy as a lark, in fact, all day.

Friday wasn’t as happy a day. In the morning Dirk’s urine stream was reduced to a thin thread and he was straining again, so we headed back to the ER. His bloodwork from Wednesday had come back all within normal ranges and the preliminary urine culture was showing no growth but had a few more days for a definitive answer, while the UA reported crystals. Unfortunately, the kind of crystal wasn’t reported. Dirk was unblocked again via catheter and an ultrasound was done to rule out an obstructing mass in the bladder. The ultrasound showed several stones, some approximately 5 - 6 millimeters in diameter (about ¼ inch). Now the plan escalated into a surgical approach as he could only continue to block as long as those stones were in his bladder. Because of the influx of cases over the holiday weekend, Dirk was put on the Monday schedule for a cystotomy to remove the stones and he was allowed to come home with me for the weekend with orders to return to VHUP before 6 AM on Monday for admission. We were to return immediately if he blocked again. Thankfully, Dirk was fine all weekend.

So, on Monday Dirk was admitted to VHUP for his surgery. Accompanying him was a letter I had written over the weekend asking for the following things in addition to the cystotomy: 1) that a urethrostomy be done to reduce (and hopefully remove) the potential for Dirk to block again if/when he continued to form stones with the required castration to allow for the urethrostomy, 2) the stones be sent for analysis to confirm that they were, in fact, cystine stones, 3) that, in the event that things went badly and Dirk did not make it off the surgical table, kidney be taken for Dr. Henthorn’s study. I knew that if he did die, I wouldn’t be in any state to think of what should be done at that point. I feel this research is important to the breed. It’s better to prepare for the worst and not need it.

Discussions with his surgeon that morning got a little tense, since she wasn’t convinced he should have the urethrostomy and she initially wanted me to dive in with the internal medicine department to put him on expensive and hard to get drugs, alkalinizers and a new diet instead. Unfortunately, none of these, alone or in combination, have proven to *completely* eliminate the formation of stones in all Deerhounds. Some therapies help some dogs, to a greater or lesser degree, but none are a guarantee that if you do XYZ, your dog will never form another stone, and some have significant side effects. Ultimately it came down to my belief that without the urethrostomy he would be back at VHUP for more stone removal surgeries and the only question would be how soon and how often. I wanted the additional surgery now, while he was healthy and strong, rather than when he was sick or debilitated from either being blocked severely or from just too many surgeries (and with their attendant anesthetic risks). I guess I convinced her because she agreed to do the urethrostomy once she heard my reasoning.

After surgery, Dirk was on pretty hefty pain medications. No surprise there, since the surgery is invasive and in a VERY tender region. Unfortunately, the combination of hydromorphone and fentanyl or the dose levels made him extremely anxious. By Tuesday evening they had his dosages adjusted and he was calmer. He was released on Wednesday, July 5th with cephalexin as his antibiotic, deramaxx for pain and the fentanyl patch that was placed pre-surgery to stay on until Sunday. Other post-op orders were no stairs, no free roaming in the house, only short leash walks to eliminate and no wiping away the clots at the incision sites. He was awake and aware, but not really himself when he got home. He was distant, not very responsive, had a sort of “stare-iness” to his eyes, and was having trouble coping with the combination of a “Bite-Not” collar AND an E-collar to keep him from reaching his incisions. He couldn’t reach his water bowl or his food bowl without help (but he didn’t want help), and he got stuck in doorways more times than I care to remember. The bleeding from the surgeries was pretty impressive but well within what I expected thanks to Christie’s web site. Every time he was walked to relieve himself his urine was obviously bloody, with numerous, large drops of blood after each stream ended. Occasionally the drops of blood were more like a squirt that left him dripping blood from the site as well and from what ran down his leg. He can’t really “aim” like he’s used to. Sometimes the bleeding would take 2 – 3 minutes before it stopped completely. He also left bloody patches everywhere he decided to lay down. Again, because of Christie’s site I was prepared for this and so all my furniture had been covered with plastic underneath layers of old blankets. A mop and bucket was kept handy for cleaning up floors as needed and saw a lot of use at first but reduced as the healing went on. Remarkably, the odd look he’d had all week disappeared as soon as his pain meds were stopped and his wonderful, loving personality re-emerged. It’s probable that the pain meds were still too much or too many, but his surgery sites were enough to expect a considerable amount of pain that he shouldn’t have to just “endure”. The bleeding seemed to stop entirely by Sunday, July 9th. He had a few bleeding episodes on Wednesday July 12th and again on the following Monday, the day his sutures were removed, but nothing in between, so I consider us lucky on that front. The collars came off the following week. The stone analysis came back as all stones being 100% cystine.

Why did I bother to write Dirk’s story? Well, for a few reasons, in no particular order:

  • to add to the pool of “working” knowledge for owners of cystinuric Deerhounds.
  • to emphasize the need for testing. If I had not been so diligent about making sure Dirk could urinate normally, not only once but multiple times a day, HE COULD HAVE DIED and I would NEVER have been so diligent if I hadn’t tested him. Yes, the screening test is not perfect. Yes, many Deerhounds may test negative that are, in fact, affected, just not passing cystine at the time the sample was collected. But Dirk’s test result made me aware, enabled me to catch the problem before it made him seriously ill and allowed me to make an informed decision about his care before we got into a crisis situation.
  • to raise awareness of the consequences of this disease. Any animal that blocks and cannot void its urine ultimately has urine back up into the kidneys, and then into the blood stream (uremia) and can not only suffer incredible pain but progress on to seizures and death. The estimate for Dirk’s care was $4,000.00 and that’s without needing ICU care to stabilize him so he could tolerate surgery. That said, owning a cystinuric dog is not the end of the world and catastrophic consequences can be avoided by careful observation of the dog and immediate response to any change in urination.
  • to point out that Dirk’s 4 years of absolutely no symptoms can easily happen to any other affected Deerhound. How many undiagnosed affected dogs does the breed have? How many of them are bred between the ages of 2 – 4 years? If I hadn’t tested Dirk and he’d been bred within his asymptomatic stage, we’d now have puppy owners to contact to tell them their pups are at an even greater risk because their sire is *now* known to be affected and that we didn’t find out through a pre-breeding test but through a crisis. That isn’t a way to inspire confidence in a breeding program or the breed in general.
Overall, Dirk has come through this ordeal with flying colors thanks to good information being available, an excellent surgeon and good nursing care by VHUP. I still expect occasional bleeding episodes for the next few weeks and was advised that the healing process itself can cause a stricture in the urethra and impair urination. Someday I may have to consider putting him on drugs or urinary acidifiers if he forms stones large enough to cause another blockage but for now we’re back to romps in the woods and obedience classes. So while I will always have to monitor his urination, life is good again. At least now he shares his body slams with his nephew, Ian, instead of me.

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