When Protein Turns Toxic: Jesse’s Story

Jesse’s story is one of poor genes, devoted pet-owners, and high-end veterinary intervention.  It offers insight into the clinical decision-making surrounding management of a rare disease that makes protein toxic to a carnivore. Balancing potential rewards of success, against risks of surgical misadventure, and costs of  treatment, Ron and Margo are still living with the highs and lows, and difficult decisions to this day.

When Jesse entered the world, she drew the genetic short straw.  Born of Schnauzer and Maltese heritage, two breeds known for their predisposition to her problem, Jesse suffered a portosystemic shunt. This microscopic, mutant, blood vessel, bypassing the liver, flooded her body with unfiltered nutrients and toxins from the bowel.

Deprived of its normal workload, detoxifying and regulating chemicals from the gut, her liver never fully developed. Her entire body followed, remaining scrawny and underweight, more than could be explained by her extreme fussiness with food.

After meals, toxins liberated from the digestive process would sometimes affect Jesse’s brain. She’d become sleepy, sometimes salivate, sometimes tremble, sometimes drunk. The symptoms were never severe enough to rouse suspicion of disease, but after 3 years of hand-feeding and nurturing, a testament to the patience of her owners, she was finally presented to the vet for investigation. Blood tests and abdominal ultrasound readily diagnosed Jesse’s problem.

Presented with two treatment options, an expensive surgical cure, or special diet, drugs and a shortened life, her parents decided to go-for-broke with the operation. Performed by specialists, the surgery split Jesse from sternum to belly button, and cellophane wrapped the mutant blood vessel, deep within her abdomen.  Over several months post-op, the cellophane would slowly strangle the flow of toxic blood.

Skilfully dodging all the predictable risks (anaesthesia, infection, haemorrhage) and those unique to shunts (portal hypertension and fitting), the surgery was a resounding success. The liver immediately started to respond, taking on it’s long intended role.  Jesse’s appetite, however, failed to follow.

During her marathon surgery, leakage of stomach acid had burned her oesophageal lining, leaving Jesse with scarring that refused to let food pass from mouth to stomach. Having taken it this far, Ron and Margo refused to give in, overextending their resources further.

A temporary feeding tube was inserted, bypassing the oesophagus, so a liquefied diet could be syringed directly through abdominal wall, and into stomach. After six weeks, Jesse had reached sufficient body weight to cope with the next round of surgery: stretching and dilation of the oesophagus. This misaventure doubled the cost of her shunt tratment.

After nearly six months of medical intervention, and six thousand spent, Jesse was a new dog. There were still some issues with the food textures Jesse could get down, but neurological symptoms had disappeared, liver function was improved, and Jesse was fitter than ever.  With daily, 10km beach runs, she easily reached a healthy body weight of 6kg.

For two years, Ron and Margo enjoyed the rewards, and gradually paid-off their substantial investment in veterinary care, before Jesse started losing weight again. There was only a 15% chance of the shunt reopening, but it happened. Jesse’s blood was bypassing the liver once again and high-protein meals or excitement would tip Jesse into full body tremor, or depressed stupor.

Even just brief hospital procedures, like sedation for ultrasound, would leave Jesse anorexic and groggy for days, and deplete 5-10% of her body weight.  If Jesse was to survive another round of repeat surgery, Ron and Margo needed to build up her body reserves, while minimising the toxic effects of protein on her body.

Jesse is now awaiting news from the surgeons.  Her story will be continued……

Latest news, April 2010: Jesse will be undergoing repeat cellophane banding, desexing, and cystotomy to remove urate bladder stones, a common complication of her condition, once she gains sufficient weight to endure the procedure.  Estimate: $4000, at a university hospital.


Homemade diet for a failing canine liver

The main aim of the diet was to provide Jesse a high quality protein, with balanced amino acids, in quantities just sufficient to meet requirements for weight gain. Too much protein, or protein types that liberated lots of toxins needed to be avoided.

The raw meat and bone philosophy of dog feeding would have been a very unhealthy choice for Jesse. There were some prescription foods available (Hills L/d) but her fickleness and narrow oesophagus made these less attractive.  Her owners had the energy and time to invest, so a home-prepared recipe was devised.

Formulating the daily ration:

Protein: Cottage cheese had the highest digestibility, ideal amino acid balance, and more importantly, a texture Jesse could swallow. 100g of cheese would provide 12g of protein per day (2 grams/kilogram of target body weight of 6kg).

Fat: Short chain animal fats were not ideal, long-chain seed or fish oils were preferable. Her daily requirement of 8g of fat came partly from cottage cheese, the reminder as Omega 3 and 6 oils.

Carbohydrates: Steamed white rice and vegetables made up the carbs. Minimally contributing to the formation of toxins, these could be offered ad-lib.

Vitamins and minerals: are often depleted in liver failure, and Vitamin B’s, E, A and C are worth supplementing.  Zinc can slow the progression of some liver disease, and a dog of Jesse’s size would take about 100mg twice daily. Copper can accumulate to toxic levels in the liver failure patient, exacerbating disease, and must be avoided.

The cottage cheese had many of these nutrients but not in the desired quantities. A regular multivitamin, made for man or animal, would contain insufficient Zinc, and too much Copper. Here is the breakdown of the twice daily, zinc supplement:

Zinc amino acid chelate                  125 mg
Magnesium phosphate                   122 mg
Manganese amino acid chelate      20 mg
Vitamin A                                         2500 IU
Vitamin B6                                       50 mg

Steamed vegetables, vegemite and egg yolk, would complement other B vitamins.  The Vitamin E-rich avocado was a tempting ingredient, and the perfect texture for Jesse to swallow, but possible toxicity of this fruit for animals limited its use.

Antioxidants: important for mopping up all the nasty free-radicals Jesse’s liver was failing to catch, and protecting the liver itself, Vitamins A, C and E, aswell as various phytonutrients like grape seed extract and milk thistle (silymarin).


The pet owner’s experience

Despite spending well beyond their means, requiring a loan to fund Jesse’s care, Ron and Margo have few regrets. Their devotion and sense of responsibity continues to ensure Jesse has the best possible chance at a long and healthy life.  Their perseverence highlights the fact: those most willing to invest in veterinary care are not necessarily those with the resources to do so.  For more on this topic see child versus tool.

Ron and Margo understand oesophageal stricturing, as a complication of anaethesia, is something their vets would have difficulty predicting.  Rare with brief procedures, reflux is more likley during protracted abdominal surgery. Irresepective, they now partly take control of their own veterinary care, premedicating with Losec before any GA.

If there is one message Ron would like to communicate to pet owners, it’s a warning of the dangers of selective breeding.  Jesse’s disease would be far less prevalent, or may not even exist, in an outbred world of mongrels and crosses. Ironically, their choice of Schnauzer crossed Maltese unwittingly placed them in the firing line of an inherited disease seen in both these breeds.

Ron expresses dismay that more isn’t done to regulate the ‘puppy industry’ or discourage the breeding of diseased animals when so many healthy ones are homeless and euthanased.  There’s enough misery in the world already, without adding to the pile.

For a more thorough description of this disease, it’s symptoms, diagnosis, and treatment options available, see what the University of Tennessee has to say.

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