(Ez a cikk magyarul megtalálható ezen a blogon - Az epilepszia arcai)
Each of
these beautiful Mudis in the above photos were created with a great deal of
love and concern for not only their future, but also the future of the Mudi
breed.
They were
born into a litter of 5 puppies on June 28, 2018.
The
breeder carefully considered which male to use to make the litter, what owners
to place the puppies with and kept in close contact with the owners after they
went home.
The
breeder did everything right, but still it went wrong, very wrong.
How did
this happen if such great attention to so many important details was carefully considered? We can ask many questions, unfortunately, there
is just one answer: idiopathic epilepsy is the devil that hides in the
details.
Idiopathic
epilepsy is quite possibly the most dreadful disease that affects purebred and
mixed breed dogs alike: it is genetic, there is no DNA test or any other
indication a parent carries the genes, it appears in unexpected places, it
doesn’t appear in the expected places, it has variable seizure types and presentation,
it terrifies both breeders and owners, and in the end the dogs are the biggest
losers as they often pay with their lives. Those “lucky” dogs that don’t die
from the seizures or medications used to treat them, pay with a much lower
quality of life. The owners incur costly
vet bills, experience high stress levels and heartbreak. There is no cure, euthanasia is often the
best, if not only, option.
Further
insult to injury comes from announcing to the Mudi community that your Mudi has
idiopathic epilepsy, this is far too often almost as bad as the disease itself,
and this is a burden the owners should not have to additionally face. The Mudi
community should be ashamed of itself for the way it handles difficult
realities – this must change.
These are
the details of the litter:
- Kennel
name: Bohemia Paws, in the Czech Republic, this was their first Mudi litter
- Mother (Tiszai
Hullámtörô Ágyas) was 4 years old, father (Wartownik Wiatru Versor) was 6 years
old; this was the mothers first litter and the fathers second, fathers first litter
was born earlier in 2018 in Finnish kennel Takkutukan
- COI is 4.50%
based on a 32-generation pedigree, 4 full generations; the genetic COI given by
Embark for “Kafka” (one of the puppies in the litter) is 10%
- The
epilepsy risk score for this litter, given before the litter was bred, was 38
moderate, epi risk was mainly on one side of the pedigree – the mothers
- An
updated score, knowing what is known now about the carriers on the father’s
side that I did not know then, but should have, would have given a minimum
epilepsy risk score of 44 moderate; not a large increase, but now the pedigree
for the pups has risk on both sides (mothers and fathers) which increases the
chances to produce an epileptic; and additionally, a second score type that I
now also provide, because of what happened with this litter, would have been 69
high; if these risk scores on the father’s side had been known before the
litter was bred, another sire would possibly have been chosen that offered a
lower risk
- The
puppies were placed in homes in 4 different countries, Czech Republic, USA,
Italy and Belgium
- “Kafka”
in the USA started to have seizures in December 2018 at almost 6 months of age;
he was euthanized a year later due to complications of his epilepsy treatment
- “Abby”
in Italy started to have seizures in June 2020 at almost 2 years of age, she is
currently on anti-seizure medication, her future is uncertain
- “Ean”
in Belgium suffered a medical event, similar to a seizure, while under
anesthesia, in June 2020, he did not survive, he was being castrated, this was
his first time under anesthesia, he showed no signs of epilepsy prior to this;
it is important to note that his brother Kafka also had a full body seizure
while under sedation
Kafka and
Abby were diagnosed by veterinarians to have idiopathic epilepsy after
significant testing. Ean’s vet could not
be sure what happened, but it is highly suspected that he suffered a seizure
while under anesthesia and could not be revived.
Kafka’s
seizure presentation and age of onset was very similar to another Mudi in
Finland named “Vinka" (Mustantassun Mauno). Vinka and Kafka
are related via sisters from one kennel on their father’s sides and via several
shared relatives on their mothers sides; however without a DNA test, I cannot
be exactly sure which grandparents gave the required genes to the parents, but
as these two litters have shared relatives that are connected to other
diagnosed epileptics, a pattern of inheritance is clearly evident. The path of
transmission is more clear on the fathers sides, than the mothers.
There are
rumors that a sister of the mother of Kafka died from a seizure (this would be Kafka’s
aunt). Her son is also rumored to have seizures (this would be Kafka’s cousin).
If one or both of these rumors are true, the epilepsy in this line is
particularly fierce. Now more than ever,
it would be vital to know how many other Mudis, not only in this line, but in
other lines, have epilepsy. Sharing your
Mudi’s epilepsy will help stop the stories that are yet to come by providing
wiser litter planning.
The confirmed
epileptic count is now 39. There are 16
possible epileptics as well. There are
rumors of many others. In the confirmed cases, males outweigh the females by 22
to 17. In the possible cases, females outweigh the males 11 to 5. But as we do not know all the affected Mudis,
there is no way to know if one sex is more affected than another. COI is also not seen to have an effect at
this point, but more research is needed in that area before any connection can
be ruled out.
How
common is it that more than one puppy in a litter has seizures? I can only answer that with regards to the
Mudi. The Bohemia Paws A litter (born
2018) and two separate (with different parents) litters in one Hungarian kennel
each had at least two epileptics (born in 2000 and 2002), and a Finnish kennel also
had one litter with two epileptics (1988 litter). Several kennels have had related litters with
epileptics as well: Hungary (3 separate litters in one kennel in 2006-2007), Sweden
(1998 and 2009 litters), another kennel in Hungary (2002 and 2009 litters) and
Finland (1987, 1988,
1989 litters). Of course, these are only the epileptics that have come forward,
there are surely others. At this point
in time, there is no kennel or line that does not have some connections to
epileptics in their 5 generation pedigree.
I have
also been told by several owners that their epileptics also had fear issues and
other behavioral problems, particularly in this Kafka-Vinka line. Is this a sign, is there a connection? I do not know without more information being
shared.
Kafka and Vinka both began to show a very similar type of seizure at almost 6 months of
age. Vinka’s epilepsy progressed more slowly than Kafka’s. At 2 years of age Vinka’s seizures became
worse and more frequent and he was put on anti-seizure medication. Kafka’s seizures progressed more quickly,
while Abby’s seizures were serious from the very first one.
Another
relative named “Leo” also suffered from odd unexplainable behaviors, and severe
fear issues that progressed over time, eventually resulting in euthanasia. It
was denied by his breeder that it was epilepsy and there were many rumors that
it was or wasn’t true. I believed the breeder, this mistake haunts me to this
day. It was not until after I heard
about Kafka (who had similar seizures to Vinka and was related to Leo, as is
Kafka) that I did serious investigation which concluded that Leo was indeed epileptic,
especially in light of his family connections to Kafka and Vinka and another
relative from Leo’s kennel that also exhibits odd behavior and was diagnosed as
likely being epileptic. If I had done the proper investigation sooner, this litter
may not have been sired by the father that was finally chosen because he
appeared to be the least risky. We now know that was not the case.
While the
tragedy of this litter is unimaginable, it could have been worse and the
knowledge that was gained, provides a little bit of solace. This litter clearly shows that waiting till
the parents are older to breed them, and not breeding litters in rapid
succession, prevents more affected puppies and carriers being born that will
continue the spread of this disease. Hypothetically speaking, if the breeder
chose the most common age (2 years old) to start breeding her bitch (instead of
waiting till she was 4 for her first litter), this could have been the likely result:
-Mother Ágyas
is bred at 2 years of age to Father Buksi, they have 5 pups
-Mother Ágyas
is then bred at almost 3 years of age to Father Samu, they have 4 pups
-Mother Ágyas
is bred at 4 to Versor, they have 5 pups, some pups in this litter are
diagnosed with idiopathic epilepsy
-All pups
in first two litters are now likely carriers, and should not be bred, nor
should the pups in the affected litter, nor any in any following litters;
litters the father of the epileptics has produced with other bitch’s should
also not be bred
-In the
meantime, Mother Ágyas may have even had another litter AND the pups in the
first litter may also have had a litter.
All this in just the span of 2-3 years.
How many Mudis are instantly removed from the gene pool due to this
speed breeding style?
This is why
breeding too young (before 4 years old for both males and females) and breeding
litters closer than 2 years apart is harmful to a breed that has epilepsy. Breeding
the Mudi in the traditional or ultra-modern ways will not work to slow the
spread of epilepsy. We must choose another
method which is: waiting till breeding dogs are 4 years of age and spacing
litters 2 years apart.
It’s time
for you to meet Vinka and the Bohemia Paws littermates that have epilepsy and hear their
stories and see their videos, I hope it will help you to understand epilepsy
from the closest perspective and realize why it is so important to not let
epilepsy take our dogs and this breed away from us. There is still a chance, but the clock is
ticking, and the window will close, please don’t let that happen. If you have an epileptic Mudi, please tell
someone about it and let them share the story of your epileptic Mudi if you
cannot do so yourself. Do it for your Mudi, because they would do it for you.
This is a video of Vinka experiencing focal type epileptic seizures:
This is a video of Kafka experiencing focal type epileptic seizures:
Abby and
Ean’s stories will be posted as soon as the owners are able to write them, it
is a very difficult task for them at this time.
This
article was written with the approval and collaboration of these owners and the
breeder of these pups. I hope that more
owners and breeders will work together to raise epilepsy awareness. It is not your private shame, it is our
shared burden and we must all carry it together.
Bohemia Paws Alpine Leopard, ‘Kafka's’ story:
As a puppy, Kafka was uncertain about strangers, not unlike many Mudis, despite being very well-socialized and adored by his breeders. He would generally warm up after a little bit or he could be desensitized to odd objects with a little click-and-treat work. Over time, however, his fear of other people became worse and he would try to scare them away. I noticed a sharp decline in the month or so before his seizures started at 5.5 months.
In late December 2018, Kafka started having repeating focal seizures involving a head tremor without loss of consciousness. You could interrupt him with food. But, the seizures kept coming and kept getting worse. After a day or two, we went to the emergency clinic at a veterinary school where they did a basic neurological exam and bloodwork, including thyroid and tick-borne disease testing. Kafka had not had any vaccinations in the weeks before the seizures started, nor was he taking tick or heartworm preventatives at the time. Nothing showed up on his exams and the tremors kept happening. Taking him to any vet was hard, because he was so afraid of contact with strangers.
After all the basic testing
was done, we were referred to one of the top neurological programs in the
country, at University of Wisconsin Veterinary School. In the exam room, Kafka
had seizure after seizure. We subsequently did an MRI and a spinal tap to
analyze spinal fluid for evidence of any infection that had crossed the
blood/brain barrier. Nothing was found. Because he was so young, no one wanted
to jump to the diagnosis of idiopathic epilepsy. Instead, we kept looking for
alternative causes. We tested his blood to see if he might have had lead
exposure as a puppy; we examined his ears to see the head tremor might have
been caused by an inner ear issue; we examined his eyes to see if he had a
vision problem. Again, nothing. We tried changing his diet to a prescription
food formulated for neurological issues. No improvement. Other veterinarians
around the country were consulted about his case.
One alternative diagnosis
that was considered was "idiopathic head tremor syndrome" which also
involves focal seizures, but does not respond to anti-seizure medications. But
Kafka had a full-body seizure while under sedation, and responded to
anti-seizure medications. Subsequently, he was started on Keppra
(levetiracetam) which worked for about a month to stop the seizures, and then
failed. (This is apparently typical of refractory cases.) We switched to a
combination of Zonisamide and Gabapentin, along with behavioral medications for
his fear, which continued to intensify. The Zonisamide/Gabapentin would work
for a while, and then the seizures would start again. When they restarted, we
would increase the dosage. After a few months, 28-pound Kafka was taking enough
drugs to leave dogs twice his size groggy and immobile. That was still not
enough, so we tried CBD oil too. His seizures could be controlled for a while,
but if you watched him carefully, you could see slight head movements or
shaking of the head that suggested to us that seizure activity was on-going,
just at a lower level. Because he did respond to medications, even if
imperfectly, the neurologists ultimately decided to diagnose Kafka with
idiopathic epilepsy, meaning that there were no known physical causes and it
was likely to be genetic in origin.
Tragically, the constant seizure activity seemed to heighten his fear, and his fear heightened his seizure activity, creating a terrible downhill cycle. We consulted with professional veterinary behaviorists across the country, including Dr. Karen Overall. He was sweet and loving and a perfect dog with his family, but very suspicious of anyone but his people. He could not go outside our property without a muzzle. Even when we had worked with a trainer for weeks, and he seemed to have grown accustomed to her, he would suddenly turn without any apparent cause and try to attack her. He started having problems with dogs inside the household too. After a year of fighting the diagnosis and doing everything we could for him, we ended up making the painful decision that he was too much of a risk for others, and that the confinement that was necessary for him was exacerbating his epilepsy and his behavioral problems. We spent his last months trying to make sure that he experienced joy every day, that he played with the one dog who could manage him, that he had off-leash adventures on our property, and special one-on-one time with us. And then we said good-bye.
Though, of course, it was not really a good-bye because a dog like that will stay in your heart forever, and the scars of losing him will never leave you. The memories are still painful, but I hope that making his story public will help save some other family from repeating the same pain.
