Collie Cyclic Neutropenia - Tessie the Border Collie

Report for the Border Collie Society

Tessie the Border Collie -29.05 05 - 01.04.08

A Sufferer from Collie Cyclic Neutropenia & the only UK Border Collie Recipient of Lentivirus-mediated Gene Therapy as a Treatment.

Tessie the Border Collie Tessie was purchased on July 11th 2005 from an experienced breeder on a farm in mid-England. She was small, pot-bellied & had one blue eye & one brown but otherwise appeared healthy. However, the night after she was first vaccinated her first characteristic symptoms appeared of high fever, joint pain that made her lame & cry out if you picked her up, upset stomach & other eye, mouth & chest infections.

This was able to be alleviated by pain control, but antibiotics did not seem to help much, it was as though she got herself better with time, so long as she was given highly supportive veterinary care & home-nursing. This continued in a puzzling pattern of good health & high activity followed by crashes of severely ill health. She gained & lost weight, could not eat for several days at a time, listless yet still wanted to interact if at all possible.

Blood tests were regularly taken until a diagnosis of Collie Cyclic Neutropenia was made in November 05. It was made on the basis of the cyclic nature of the presence or absence of neutrophils in her blood. With Tessie, this was about a 3 week cycle, though often it is a 10-12 day cycle. Without sufficient, mobilised, neutrophils in the bloodstream, it is impossible to fight infection adequately. Therefore, illness would follow about 5 days after her low point of neutrophils, which would last about 5 days, then she would become bright & energetic again; then the cycle would start all over again, although sometimes there were longer or shorter gaps between illness & good health.

She evidenced many phobias during this time with fears of electronic beeps, metal gates clanging, pouring of dry food - that she had encountered while hospitalised. We learnt that her morale was vital in her care & several young people became crucial to her ability to eat, take medication, in that she allowed them to encourage her to eat etc, while she trusted less those of us who had to be involved in her personal care of cleaning, injections etc. She developed a trademark 'shiver' of greeting for those she particularly trusted.

After changing veterinary practice, it was recommended that Tessie spent the Christmas of 2005 in a local veterinary hospital; Tessie was afflicted with such severe mouth ulcers that it had caused previous local vets to reach an impasse and so recommend ending her life. However we had also begun correspondence with a paediatrician, Professor Dr W Osborne in Seattle, who had spent his life developing a cure for CN in the children in his care. He generously offered his treatment, of gene therapy, which he had perfected in North American Gray Collies (referred to here as "Lassie collies") & therefore we decided that far from ending her life, along with the continued support of our new local vet, who took Tessie under his devoted care, Tessie should be given this potentially-lifesaving opportunity.

She responded well to IV treatment at the veterinary hospital & came home. We then continued her weekly blood tests, which she withstood most stoically and gently. Her health however was still failing in the continued fluctuating manner, and the programme for gene therapy was initiated despite lack of time in mapping her own exact gene sequences from bloods sent earlier to the USA. The STC from the Veterinary Medicines Directorate was fast tracked by a lot of wonderful people in the GMO Regulatory Authority, aided by Professor Dr. Osborne and the VMD Staff. As soon as the lentivirus-vectored c-GCSF was received in the UK under the STC, the strict, established procedure for treatment was swung into action by Tessie's vet under the personal guidance by Dr Osborne in the second half of 2006.

There was no immediate improvement & indeed she further developed a problem with her over-production of saliva, that caused swallowing problems & frequent regurgitation of food. We learnt to feed her from a high plate & to puree her food, on the advice of a speech therapist friend. Then a local shepherd suggested this might be a fish allergy, which seemed to help.

Then within 4 months, to our joy, we realised that we were no longer visiting her vet to deal with her high fevers etc. She began to gain weight, going from 10kgs to 17 Kg in 6 months. She became jaunty, ate well - at least 2 chicken breasts a day, played a lot & became a part of farm life.

We run a centre for young people with autism to learn countryside skills. Our animals are all part of this; Tessie took her place with the girls with Asperger's syndrome, who had helped to look after her when she was ill & now became her trusted companions out on the farm. While she was too scared to mix with the noisier boys, she could go anywhere with the girls, relaxed & confident together. There were many walks & cuddles together.

Dr Osborne visited her in the Summer of 07 & examined the blood test charts that had been exhaustively prepared by a scientist colleague, from the original practice blood sample results. He could see that, although the cyclic pattern persisted , the lowest point ( nadir ) that her neutrophils reached was no longer as low as before treatment. From his experience, Dr. Osborne knew that this meant that Tessie was now able to deal with the everyday infections that had previously assailed her. It was a very happy day. But because he needed to use Lassie collie DNA to urgently create her treatment rather than using her own precisely-targeted gene therapy, Dr. Osborne knew that he had only been able to give her a close-targeted gene therapy treatment, but at least & amazingly this had raised her neutrophil levels above critical, so giving her the chance for growth and development, and real quality of life.

Tessie continued in good health apart from a cough & an occasion of shortness of breath. This seemed to be contained by treatment & we continued to have a happy & exuberant little dog who joined in all farm activities with great joy. She also brought great joy to us all, as we watched her experience all the things her illness had prevented; splashing in floods to create ripples to bark at, developing her herding instinct by following piglets & hens ; chasing squeaky balls & balancing them on my father's wheelchair arm so that he just had to throw it again - & again, to both of their delights.

We had just got used to having her without worry, when very sadly this January her cough increased & she became very short of breath. She was hospitalised again & diagnosed with pneumonia via radiography. She again responded to IV treatment & came home, but suddenly the infection spread to her other lung & she was hospitalised again. This time there seemed to be no antibiotic combination able to help her fight this virulent infection, which created reactive fluid in the pleural cavity & we brought her home to hospice-nurse her amongst her familiar things, smells, sounds & particularly her friends.

Most sadly but most peacefully, she was allowed to pass on by elective euthanasia on April 1st, valiant to the end in trying to overcome her body's damage, still walking outside just hours before she died. Her spirit will never be forgotten, & many tears have been shed at the loss of this extraordinary little dog whose fortitude & joyfulness created such a special place in so many people's lives. And above all the love that she created all around her, as people became so selfless in their care for her, & to which she reciprocated with such trust & devotion.

But this was a devastating illness, which has caused so very much suffering to her & all her friends & family. It would surely be a fitting memorial if it became possible for this disease to be eradicated from the breed, by careful selection & screening for unaffected dogs for breeding, & the prevention of inbreeding which can be the unwitting cause of such Collie Cyclic Neutropenia.

Lydia Otter, Pennyhooks Farm.
Tel 01793-782436
pennyhooks dot farm at virgin.net
www.pennyhooks.com