Copper Storage Disease in Dalmatians
What we know, what we don’t know, and the role of breeders
What is Copper (and why is it an issue?)
Copper is a trace element that is mainly ingested with the food the dogs eat. Copper is an essential micronutrient necessary for all living organisms. Copper helps with electron transport, energy production, nerve transmission, antioxidant function, iron metabolism and is an important factor in blood coagulation.
What is Copper Storage Disease (CSD)?
The liver is the main place where Copper is stored, it is then excreted by bile. As the copper concentration increases beyond what the hepatocytes can transport and bind, free copper will cause oxidative stress damage which will lead to hepatocellular degeneration and necrosis. This will the lead to acute or chronic inflammation of the liver, or sometimes both. In a normal liver, the copper-concentration should be less than 400 ppm.
Primary copper-associated chronic hepatitis is primarily due to a genetic defect in copper metabolism.
Dalmatians have a primary form of Copper Storage Disease, but we do not yet understand the background - the mode of inheritance and responsible genes have not yet been identified. Research is ongoing, in a large part funded by Dalmatian Club of America’s efforts with DCAF, the Dalmatian Club of America Foundation.
To date, the DCAF funded researcher’s manual assessment of variants has ruled out several gene variants linked to CT in other breeds. This means that Dalmatian breeders will not benefit from currently available genetic tests for Copper Storage in other breeds. Despite the frustration of not being able to use currently available genetic tests, this is ultimately encouraging as the research is narrowing in on the genetics of Copper Storage in Dalmatians.
Signs of Copper Storage
Early signs of Copper Storage are often signs of gastrointestinal disease, such as anorexia and vomiting. Definitive diagnosis includes liver biopsy, which comes with it’s own risks, depending on the overall health of the patient.
Typical presentation is fast and in a dog that was previously healthy. By the time clinical signs present, the dog may be too ill for treatment to work. This is a scary and potentially deadly disease. One that every reputable Dalmatian breeder is taking very seriously.
Early Detection
Early detection is critical; often the liver will have toxic levels of copper by the time symptoms arise which makes positive treatment outcome more difficult. Since CSD in the Dalmatian is believed to be associated with a genetic defect, acquiring the disease may not be preventable but the onset of “critical life threatening” symptoms may be avoided if some specific steps are taken early in the life of the dog.
Treatment includes Copper chelation (Penicillamine), Zinc (too slow acting to be primary treatment), low copper diet, and added antioxidants.
What can we do?
For owners? The best defense we have is to catch any issue early. The current recommendation is to run a CBC and Chem panel (standard bloodwork) at 12 months, 18 months, 24 months, then annually. There is some speculation that Dalmatians may have two versions of Copper Storage Disease, a more acute and deadly version that presents in younger dogs (often <2), and a more gradual and easily manageable version that presents in older dogs (>7) - this has not been substantiated yet, but we DO know that in young dogs CSD is an aggressive and frequently deadly diagnosis.
For breeders? The BEST DEFENSE is a deep dive into pedigrees to help minimize doubling up on risks. We don’t yet understand the heritability behind Copper Storage Disease in Dalmatians. Throwing out every pedigree that has Copper Storage Disease lurking would effectively leave us with….no Dalmatians. Instead, we need to work together to ensure we all have the best understanding of dogs who may pose a risk, ensure we aren’t choosing pairings that have risks on either side….but this only works when everyone is transparent.
Honesty & Transparency - the only path forward
Breeders committed to producing healthy, sound, stable pups spend a lot of time reviewing pedigrees. An ideal pairing is so much more than a dog-to-dog comparison - it is a consideration of all the dogs that are behind these two dogs - for conformation (size, movement, structure, spotting), for temperament (stability, confidence, biddability), and for health - looking beyond our minimal requirements for health testing and looking at all the things we can not (yet) test for. Are there dogs in the pedigree that have produced cardiac issues? Do dogs in this line have Epilepsy? Were there dogs that died suspiciously young and could have been CSD?
The challenge with a deep dive into pedigrees is that it is only as useful as people are honest. Every Dalmatian breeder can tell you a story about a breeder they know who had dogs die suspiciously young, that never came out with the truth and never shared a diagnosis. These dogs become question marks in pedigrees, and we wonder about not just avoiding them, but avoiding their relatives, or their predecessors. This sort of dishonesty makes it so hard to make the best choices to give us the best chance of healthy pups.
Every breed deals with issues, often ones that can not be screened for (yet). The only path forward is to be honest and up front. We KNOW Copper Storage Disease exists in Dalmatians. If breeders are not honest about the tragedy of producing a dog with Copper Storage Disease, another breeder could do a similar pairing with tragic results - a loss to both the breeder and the dog’s owners that could have been avoided.
Kudos to the breeders who have spoken out and been honest and truthful about producing CSD. Copper Storage Disease is, arguably, behind every single pedigree. No one is without risk, and by working together we can ALL minimize and manage the risk until that wonderful day that an accurate genetic test is available.
Has Bedlam Acres produced CSD? Not yet, in a large part thanks to our mentors helping us with the deep dive into pedigrees and being honest and transparent with the risks within their own lines. Much like producing a deaf dogs=, the likelihood is one day it will happen despite our best efforts - our hope is a genetic test is available before that happens so we can definitively avoid the risk.
References:
Copper associated hepatitis Lina Hedman Borg Supervisor Dr Sterczer Ágnes PhD, associate professor. University of Veterinary Medicine Budapest. Department of Small Animal Medicine. Budapest 2023; https://huveta.hu/server/api/core/bitstreams/5e48f90d-daac-4a25-978c-b743fa012121/content
Copper-Associated Liver Disease in Dalmatians:A Review of 10 Dogs (1998–2001)Craig B. Webb, David C. Twedt, and Denny J. Meyer; https://onlinelibrary.wiley.com/doi/epdf/10.1111/j.1939-1676.2002.tb02405.x
Hepatitis and Increased Copper Levels in a Dalmatian
V. L. Cooper, M. P. Carlson, […], and N. R. Schneider+1View all authors and affiliations
Volume 9, Issue 2; https://journals.sagepub.com/doi/abs/10.1177/104063879700900217
Copper Storage Disease in Dalmatians, DCA brochure; https://dalmatianclubofamerica.org/wp-content/uploads/2019/04/CopperStorageDiseasePamphlet-12102018.pdf
Pedigree study of the heredity of copper-associated hepatitis in Dalmatians in Japan Munekazu Nakaichi 1,✉, Toshie Iseri 1, Hiro Horikirizono 1, Harumichi Itoh 1, Hiroshi Sunahara 1, Yuki Nemoto 1, Kazuhito Itamoto 1, Kenji Tani; https://pmc.ncbi.nlm.nih.gov/articles/PMC9112360/#b11-cvj_06_633
