When I (Perry Jameson) arrived at the hospital one recent morning, one of my many transfers stood out from the rest. Gumbo bounced as I approached his kennel, standing with his front paws against the door and looking at myself. He was distinguished by his thick, wrinkled skin, short ears and curved tail. It was a Chinese Shar-Pei, a breed that I don’t see every day.
After the establishment of the People’s Republic of China, it was more difficult to obtain Shar-Peis. In the 1970s, interest in this breed of dog increased in the United States. This meant that there weren’t many dogs to choose from to meet the growing demand, so many of them were closely related.
The fact that selective breeding of existing dogs was occurring further reduced the gene pool. As with most dog breeds, humans would take those with the characteristics we liked and breed them together to get more of those who look or act the way we want them to. While bringing out the qualities that we love, it also makes the appearance of negative genetic qualities more frequent.
What gives Chinese Shar-Pei its distinctive appearance is an increased amount of Hyaluronan (HA) in its skin. This makes their skin thick, which gives them their lovely folded and blistered appearance. All that hyaluronan comes at a price.
Chinese shar-peis can 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 typically last 12 to 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 broken down and returned. Since they have so much HA, it is suspected that the increased amount of HA fragments contributes to their increased inflammatory response.
Unfortunately, there is no specific test to diagnose this syndrome. This means that I have to rule out everything else that can cause the same serious symptoms. We call this exclusion diagnosis.
Fevers usually start before the age of 18 months, but onset in adulthood is not uncommon. Fortunately, episodes often decrease with age. In most dogs, the fever will go away without treatment in one to two days, but they are miserable while it does.
They often have swelling of the muzzle and around the lower joints, with the ankle most commonly affected. Most will be reluctant to move, acting stiff and sore. Some will suffer from vomiting and diarrhea. The inflammatory cascade makes them feel the same as you and I would with the flu.
In addition to feeling unwell, chronic low-grade inflammation predisposes them to amyloidosis. It is a disease resulting from the abnormal deposition of degradation products of chronic inflammation in the extracellular matrix (space between cells). Not all Shar-Pei will develop this, but those who do will die prematurely from kidney failure.
We therefore treat not only to prevent them from having these terribly uncomfortable episodes, but also to prevent amyloidosis from developing. Even between clinical episodes, these dogs suffer from low-grade, potentially damaging inflammation. This means that the treatment is constant and for life.
Treatment involves a variety of drugs and supplements to modulate the immune response and stabilize HA so that it doesn’t break down as quickly.
The mainstay of treatment is the drug colchicine. It works by blocking the movement of white blood cells, decreasing inflammatory messengers, preventing mast cells from releasing histamine, and blocking the formation of amyloid protein.
High doses of omega 3 fatty acids are also initiated for their anti-inflammatory properties. I will often add vitamin C, vitamin D3, and lecithin. My FSF patients also receive HyVitality, a combination of vitamins, minerals, antioxidants and phytochemicals that have been chosen for their health benefits.
Most dogs will also be treated with low doses of aspirin for its anti-inflammatory and anti-thrombotic properties.
We are increasingly realizing the importance of the gut bacterial population, the microbiome, in the overall health of the body. There is a growing body of evidence that this affects our immune responses. For this reason, I recommend high quality, limited ingredient dog food and probiotics.
Gumbo ticked all the right boxes for the FSF. So I put him on all the medications I discussed in the hopes of avoiding further emergency room visits, flu-like episodes, and premature death from amyloidosis.