Spina Bifida- how cute puppies can lead to healthier children

When I tote my wagon full of diaper clad puppies, people often think that this is a fashion statement or because the puppies are not house trained.  But the truth is they do not wear diapers simply because they are cute, but the puppies have Spina Bifida.  When I tell people that they have Spina Bifida, most of the time, the response is “I didn’t know dogs can get that!” Fostering special needs dogs, I hear this often, but the truth is – animals can pretty much be born with every birth defect that people can be born with. The difference is, most animals are euthanized, whereas, in the US (unlike countries like the Netherlands which allows parents to euthanize infants with significant birth defects), we spend a significant amount of money on the medical needs on children born with Spina Bifida and other birth defects.  It is estimated that the costs of healthcare for children with Spina Bifida is $200 million every year.

In the US, over 1,600 babies are born every year with Spina Bifida.  There is currently no cure for Spina Bifida, research shows that folic acid before and in the first few weeks of pregnancy may help to prevent Spina Bifida, but there is no cure for it. Spina Bifida often is diagnosed before birth and the current best practices for care would be fetal surgery, where the surgeon makes an incision into the mother’s uterus, exposing the fetus, and then they close the open spine of the developing fetus and then return it to its nice waterbed to finish cooking.  The reason fetal surgery is done is because after 20 weeks of gestation, amniotic fluid is mostly made up of urine, which is very acidic.  The amniotic fluid then continues to do damage to the spine of the forming fetus, and there is currently no surgery that can repair that damage. So the goal is to limit the amount of damage done, to hopefully provide the baby a better quality of life.  Also, I should mention that babies who have this surgery have a 10% chance of having hydrocephalus, whereas, those who do not have the surgery also have about a 80-90% chance of having hydrocephalus, which is another set of issues and problems.  So the standard of care is some amazingly talented surgeons, do this miracle of a surgery, to help minimize damage done.

According to the CDC, there are approximately 1,621 children being born in the US every year with unrepaired Spina Bifida. This is because  fetal surgery is only performed at 20 hospitals nationally and I can find no statistics on how many of these are performed a year, but  the consensus is, that not many are done . It can be for lack of skilled doctors, and also potential complication, as well as having proper medical care to be able to afford such an expensive treatment.  So this desire to help children born with Spina Bifida, led to trying to figure out (first) the best animal model for research.  The problem with trying to come up with an animal model to look at regeneration (post birth) is that most species of animals cannot survive Spina Bifida.  Many animals, such as rodents will actually eat their deformed babies alive. This form of recycling is horrifying when considering it an option for humans.  Other species are not quite as cruel, but are not willing to care for their babies. A ewe will deliver one (or more) babies but if they are unable to walk, they will not care for them and the lamb will die of starvation. Plus gestation lengths also play a part in considering the best possible animal model for research as well.

Precision is required with fetal Surgery, this is why only 20 surgery centers in the United States have the the capabilities to provide surgery for Spina Bifida before birth.

Dogs are the perfect animal model for this research project for many reasons.  Mostly because most dogs are very loyal to their humans and their babies, and so they do not mind that their babies poop all the time and generally continue to raise and care for these special babies.  Where lambs are expected to walk immediately, pups don’t really start walking until 3-4 weeks.  Most dog breeders tend to not realize that there are issues until the pups reach 4-6.  At this point, many breeders (often at the recommendation of their vets who have minimal experience here) suggest euthanasia for these babies.  So first and foremost we want people to know, EVEN UNTREATED – SPINA BIFIDA DOGS USUALLY LEAD LONG AND HAPPY LIVES.

Why does this study focus on English Bulldogs? That is easy; clearly it is because they are the cutest.  Ok, that isn’t the reason, but I think it is good enough reason.  The research focuses on English Bulldogs because they have a higher incidence of naturally occurring Spina Bifida. Different breeds are more prone to certain diseases, defects or injuries.  Some examples you may know is there is a higher incidence of Cancer in Golden Retrievers, we see a lot more Hip Dysplasia in German Shepherds and we have a higher incidence of Cleft Palate in French Bulldogs and American Bullies. Of course, Chihuahuas can have hip dysplasia and I have raised a Border collie and a Rat Terrier with cleft palate.  When looking at breeds with Spina Bifida, we see a higher incidence of Spina Bifida in English Bulldogs – which I appreciate as their lack of tails makes diapering much easier.   I should point out that cats are another animal that  we could use for this study as felines also suffer from Spina Bifida, but in our feline friends, it is commonly called “Manx Syndrome,” however,  I run a dog rescue and my frenchies are enough like cats for me.

The only treatment currently for Spina Bifida in babies is surgery to close the spine. This ideally is done as a fetal surgery (as mentioned before) otherwise  the surgery is done shortly after birth. But as I mentioned there are not many doctors or hospitals have the capability to do this complex surgery. We happen to work in an amazing area. Sacramento has the benefit of having an amazing Medical School and Veterinary School, which makes  this the perfect area and experts to allow for this quality of research.   Dr. Diana Farmer is one of the Principal Investigators and she is a pioneer in the field of fetal surgery.  Dr. Aijun Wang kind of put it all together with his bioengineer expertise and is one of those super happy and down to earth people who has been very successful as a researcher.  Of course, we need an amazing  dogtor as well, and that would be Dr. Beverly Sturges, the chief of Neurology at the UC Davis School of Veterinary Medicine.  She is also very sweet and passionate and wanting to help all the animals – she is what I would like to be if I had the ability to understand algebra.

Right: Dr. Beverly Sturgis, DVM, looking over Ariel to determine whether she would be a candidate for surgery. Ariel was not a candidate but she is now an IG sensation. Follow Mermaid_Ariel_Madison
Left: Dr. Aijun Wang and Kaitlin Clark escort their patient to UC Davis. You can follow this cutie on IG at: Slughornthebulldog

Then there is me, Research Manager at Shriners Hospital for Children Northern California and I have been running a dog rescue for nearing a decade.  I have basically zero science background; in college, I took a writing intensive astronomy class for my science requirement in order to avoid math.  I wrote my 18 page research paper on whether asteroids caused dinosaurs to become extinct.   I foster special needs dogs galore, do a ton a research and try to know everything about the issues I deal with (Spina Bifida, Hydrocephalus, Cleft Palate, Megaesophagus, and so many others) and so I was contacted early on by one of the research assistants in Dr. Wang’s lab asking about the correlation with Spina Bifida dogs and English Bulldogs.  One of my jobs is looking at grants and I was pulling for Shriners to fund this, as I saw it, revolutionary grant.  Two bulldogs had the surgery before I really came into the picture.  I saw the results and they looked promising. But actually seeing the results with dogs you spend every day with, is another thing.

Who is a candidate for the surgery?

Inclusion requirements for the study includes English bulldog puppies that are between 8-10 weeks of age.
The puppies must be mobile, not suffer from contracture (leg is unable to bend), but also has a significant enough of impairment that the surgery will benefit them.  Incontinence is not believed to be helped, so the goal is to improve mobility.  I hope to soon be able to include puppies with both Spina Bifida and hydrocephalus (that would open to other breeds and allow for a bit older puppies to have surgery up as well). Find out more at: https://studypages.com/s/assessing-a-stem-cell-therapy-for-spina-bifida-in-english-bulldog-puppies-405288/?ref=gallery

To determine whether a puppy may be a candidate usually we ask for short videos, from a side view, where we can see the movement.  If there is an X-ray, we appreciate that to be able to see whether we can see a bifid (or cleft) in the spine.

What happens after puppies are chosen?

After the puppies make it to Dogtor Sturgis, then the next step, before surgery, is for the pup to get an MRI.  This happens before and 2 months post-surgery.

The program is really testing the benefits of using stem cells alongside of surgery to improve outcomes so this is done by having two different groups. There is one group that only receives surgery to close the spine, this is also the current course of treatment for children born with Spina Bifida. * As of 6/19, there has only been one puppy who was not treated with stem cells.  While it benefits him in that an open spine is easier to damage (which can cause worse paralysis so this is a benefit for long term prognosis), he did not show the same improvement to his gait as the puppies who received stem cell treatment.

The stem cells come from the placentas from donor dogs (every puppy has a placenta so there are lots of placentas to choose from when pups are born C-section, otherwise mom eats them). With the plethora of placentas, stem cells are grown at the UC Davis Veterinary Institute of Regenerative Cures (VIRC).

If you just add the stem cells freely, they will migrate to vascular areas, like the bladder.  So basically the puppy will pee pee the plethora of placental cells.  Well that means probably there would be little help, so the FDA has approved the use of Porcine Small Intestine Submucosa (SIS) –aka pig gut– as a scaffold to help keep those stem cells in place. The SIS is sutured in to tuck in all the magic.

To break it down: hole in spine closed, stem cells grown from dog placenta and then scaffolding is used to hold all of it in place.

What’s next?

Besides looking to enroll more Spina Bifida pups, WE NEED NORMAL ENGLISH BULLDOG PUPPIES as well.  We need puppy owners with pups less than 10 weeks old. Total there will be 5 appointments (2 include MRIs and 5 have gait analysis) so they can compare normal bulldogs to that of the Spina Bifida pups.  There are some benefits, other than just helping children. If you enroll your normal English Bulldog puppy, they will get 2 free MRIs (which can be a helpful baseline), they will get free vaccines, heartworm preventative, flea preventative, and free spay  or neuter.  You can contact Lisa Even at: lmmahakian@ucdavis.edu to discuss enrolling.

There are always potential complications, but the joys of this study being done at the #1 Vet School in the world, that there have been only minor issues.  Sometimes there is an immune response to stem cells and so they may have a fever for a few days, but the pups are up and walking immediately.  The worst complication I have had, is that they get spoiled rotten while at UCD (they can be there for 3 days to 2 weeks depending on any issues) and they come home expecting to get round the clock attention.  I guess there are worst things to happen.  For me, as the person who has located, collected and fostered the last 3 puppies – it is hard for me to really recognize the major milestones, but they are getting better and better about collecting gait and other data, but it is pretty clear

from the videos alone- it really works!  Generally, if they are incontinent before (and most are), then they will likely need diapers forever.  But pups learn to adjust to whatever level of ability they have and will overcome.  There are dog wheelchairs if needed, but they always find a way around.  So mostly, you can expect (with or without surgery) they are mostly normal dogs who might walk a little different and have to wear diapers. The good news is since they do not have tails, human diapers can be used at a fraction of the cost.

Recycled Pets NorCal and our role

Puppies  that are a part of this program need to be local to the Sacramento, CA area.  RPNC happens to be one rescue in the local area, and we like special needs dogs and the joys of working at Shriners Hospital (the funding source for the grant), it is easier for me to foster, do follow ups, and then adopt puppies out to local families.  But local breeders, owners and other rescues who have Spina Bifida dogs that want to be a part can also be involved as long as they are committed to doing follow ups and the new families have to continue to be willing to as well (our adoption contract states if the adopters are not willing to participate in follow up MRIs, gait analysis or other necessary tests, then they need to pay back the entire cost of the medical treatment (which is approximately $15,000). Of course, if breeders or rescues want to surrender puppies, even if they are not candidates for the study, we will always try to accommodate them.   You can reach me at: elsie@recycledpetsnorcal.org  if you have a potential candidate for the surgery or are looking to surrender a special needs pup.

Together, we can help future generations of babies born with Spina Bifida!

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