When I (Perry Jameson) arrived at the hospital on a recent morning, one of my several transfers stood out from the others. Gumbo bounced up as I approached his kennel, standing with his front paws against the door and looking up at me. He was distinctive because of his thick, folded skin, short ears and curved tail. He was a Chinese Shar-Pei, a breed I do not get to see every day.
Following the establishment of the People’s Republic of China, it was harder to get Shar-Peis. In the 1970s, interest in this breed of dog increased in the United States. This meant there were not a lot of dogs to choose from to meet the growing demand, resulting in many of them being closely related.
Further narrowing the gene pool was the fact that selective breeding of the existing dogs was occurring. As with most breeds of dogs, humans were taking the ones with the characteristics we liked and breeding them together to get more of the ones that look or act like we want them to. While bringing out the qualities we like, it also makes it more common for negative genetic qualities to occur as well.
What gives the Chinese Shar-Pei its characteristic look is an increased amount of hyaluronan (HA) in their skin. This makes their skin thick, which gives them their cute folded, vesiculated look. All of this hyaluronan comes at a price.
Chinese Shar-Peis may suffer from a condition called Familial Shar-Pei Fever (FSF). This is a periodic fever syndrome characterized by random episodes of high fever, and sometimes swollen joints and muzzle. These usually last for 12-36 hours.
Shar-Peis who suffer from this syndrome have a genetic mutation that predisposes them to an inappropriate inflammatory response.
At the same time, the hyaluronan in their tissues is constantly being broken down and turned over. Since they have so much HA, it is suspected the increased amount of HA fragments contributes to their increased inflammatory response.
Unfortunately, there is no specific test to diagnose this syndrome. This means I have to eliminate everything else that can cause the same serious of symptoms. We call this a diagnose by exclusion.
Fevers usually start before they are 18 months of age but adult onset is not uncommon. Fortunately, the episodes often lessen as they age. In most dogs, the fever will resolve without treatment in one to two days, however they are miserable while it occurs.
They often have swelling of the muzzle and around the lower joints, with the ankle most commonly involved. Most will be reluctant to move, acting stiff and sore. Some will experience vomiting and diarrhea. The inflammatory cascade makes them feel the same way you and I would with the flu.
Besides feeling poorly, the chronic low-grade inflammation predisposes them to amyloidosis. This is a disease resulting from the abnormal deposition of the breakdown products of the chronic inflammation into the extracellular matrix (space between cells). Not every Shar-Pei will develop this, but those who do will die early from kidney failure.
So we treat not only to keep them from having these terribly uncomfortable episodes but to prevent amyloidosis from developing. Even between the clinical episodes, these dogs are experiencing low-grade, potentially damaging inflammation. This means treatment is constant and for life.
Treatment involves a variety of drugs and supplements to modulate the immune response as well as stabilize the HA so it is not broken down as rapidly.
The drug colchicine is the mainstay of therapy. It works by blocking the movement of white blood cells, decreasing inflammatory messengers, not letting mast cells release histamine and blocking the formation of amyloid protein.
High doses of omega 3 fatty acids also are started for their anti-inflammatory properties. I will often add vitamin C, vitamin D3 and lecitihin. My FSF patients are also started on HyVitality, a combination of vitamins, minerals, antioxidants and phytochemicals that were chosen for their HA health-promoting effects.
Most dogs also will be treated with low doses of aspirin for its anti-inflammatory and anti-thrombotic properties.
We are realizing more and more the significance of the intestinal bacterial population, the microbiome, in the overall health of the body. There is increasing evidence it affects our immune responses. For this reason, I recommend a high, quality, limited ingredient dog food and probiotics.
Gumbo checked all the right boxes for FSF. So I have started him on the all the medications discussed with the hopes of preventing any more ER visits, flu-like episodes and an early death from amyloidosis.