Why Early Treatment Matters
Exacerbation can relapse and remit. They tend to increase in duration and intensity with each episode. Untreated PANDAS/PANS can cause permanent debilitation and in some cases can become encephalitic in nature. Repeat strep infections can cause serious problems. It is important to eradicate strep completely. Subsequent episodes can be caused by environmental and infectious triggers different from the original infection. Treated early and in a timely fashion, PANDAS/PANS can remit entirely.
These have been very good for arresting most strains of strep. See remarks on Cephalexin superiority over penicillin regarding post-strep illnesses at page (3) of by Dr. R. Hahn, et al (2008) Evaluation of Post-streptococcal Illness.
The Cephalosporin alternative is also discussed in the American Academy of Pediatrics Journal, p. 1609 – Prevention of Rheumatic Fever and Diagnosis and Treatment of Acute Streptococcal Pharyngitis.
Azithromycin is a front-line, broad based antibiotic that protects against many forms of bacteria. A study of azithromycin being helpful in treating youth with acute onset OCD can be found here
It has been recommended by physicians that the PANDAS child remain on prophylactic antibiotics in accordance to the RF (Rheumatic Fever) guidelines established by the American Academy of Pediatrics Journal. The RF guideline by the AAP is for 5 years after last attack or until age 21 (whichever is longer). According to the World Health Organization, the duration of prophylaxis for ARF is 5 years after last attack until 18 years old (whichever is longer).
Children who have had Sydenham’s Chorea, Rheumatic Fever or PANDAS have a risk of developing a more severe reaction upon reinfection with strep. Prophylaxis prevents reinfection.
Long term prophylactic antibiotic use for PANDAS has been shown in the study Antibiotic Prophylaxis with Azithromycin or Penicillin for Childhood-Onset Neuropsychiatric Disorders to “…play a role in the management of children in the PANDAS subgroup, as well as provide support for the assertion that GAS plays an etiologic role in some children with tics and/or obsessive-compulsive Disorder”. In that same study, it also states that “There was a 61% overall reduction in neuropsychiatric symptom exacerbations during the year of antibiotic prophylaxis and a 94% reduction in GAS triggered neuropsychiatric symptom exacerbations.”
According to the NIMH page , prophylactic antibiotics “may be helpful to use antibiotics as prophylaxis (prevention) against strep infections. Prophylactic antibiotics have proven to be quite beneficial to patients with rheumatic fever and Sydenham chorea”.
How PANDAS/PANS Children May React to Antibiotics
A Child That Improves With Antibiotics
The parents and provider will see improvement in the child and an easing of symptoms. Some children rapidly improve with antibiotics, while others experience improvement over a period of time. Children may have mild setbacks with viruses, other infections, etc. as the healing process continues.
A Child That Does Not Improve With Antibiotics
When a child has no improvement with antibiotics, the child’s episode may be encephalitic in nature and the inflammatory reaction may not only increase over time, but take many months to stop. A significant number of the original PANDAS cases PANDAS Network followed fit into this category.
For some children, antibiotics are needed for a minimum of 6 weeks to see a large reduction. Even then, there may not be a 100% reduction in symptoms. The autoimmune process is well in place and if after several months the child seems to be reactive to many illnesses at school or at home, it may be time to consider other treatment alternatives.
IVIG is an intravenous pooled blood product comprised of immunoglobulins, that is used in treating immune deficiencies, encephalitis, and other medical conditions. The Immune Deficiency Foundation has more information on IVIG here
Drs. Perlmutter and Swedo used IVIG in the 1999, Lancet, Therapeutic plasma exchange and intravenous immunoglobulin for obsessive-compulsive disorder and tic disorders in childhood, where all of the children benefited from its use.
IVIG is endorsed for treating PANDAS by a consortium of physicians and researchers, as it is an autoimmune irregularity that causes encephalitic-like inflammation. The PANDAS Physicians Network gives recommended dosing.
Healing is gradual over several months. Any form of infection will exacerbate a child while healing so manage exposures and illnesses carefully. Guidelines have been published in the Journal of Child and Adolescent Psychopharmacology (July 2017).
Doctors in the consortium are seeing that some children need a few IVIGs, but some need it on a continual basis. Our understanding of this is not clear. Typical dosing is 2gram/1kg). Occasionally a child is found to be clearly immune deficient (PID or CVID). Consultation with an immunologist is important and IVIG follow up may be different. Learn more about immune deficiency here.
How Does IVIG Work?
The exact mechanisms of IVIG are not thoroughly understood.
IVIG has been shown to be helpful with the harmful inflammation caused by autoimmune illnesses, but the exact causative actions are not clear yet. Donor antibodies may “retrain” the abnormal antibodies in the patient or the large amounts administered may simply overwhelm the harmful antibodies – thereby removing them from the PANDAS patient.
2016 NIMH & 2021 Independent IVIG Studies
NIMH IVIG study shows 60% mean reduction in symptoms for PANDAS patients. A paper (Oct 2016) submitted to the Journal of the American Academy of Child & Adolescent Psychiatry describes the outcome of the NIMH double-blind placebo controlled study of IVIG for treatment of symptoms in children who met the criteria of PANDAS. Read more about it on the Pandas Physicians Network.
In 21 patients, with moderate to severe PANS, results demonstrated significant reductions in symptoms from baseline to end of treatment. Read the full 2021 paper here.
Plasmapheresis (Apheresis) or Plasma Exchange (PEX) is a process during which the harmful auto-antibodies are removed from the blood system. This procedure is done in a hospital setting (you can read more about the procedure here).
There are not many providers that offer Plasmapheresis for PANDAS and PANS, but those that do have cited seeing symptoms improve even while the procedure is still occurring. When a child is presenting with very severe symptoms that would be considered life-threatening, Plasmapheresis may be the preferred method of treatment due to the quick response rate. In some cases, PEX has had to be repeated (as with IVIG) . Again, prophylactic antibiotics should be maintained.
The American Society for Apheresis lists Plasma Exchange as an accepted 1st-line therapy, either alone or with other treatment for a PANDAS exacerbation. The 2019 Guidelines from the Journal of Clinical Apheresis can be found here.
The full ASFA guidelines, as established in the 2013 Journal of Clinical Apheresis, appeared in their ‘Special Issue’ that is published every 3 years. The guidelines can be found at: Guidelines on the Use of Therapeutic Apheresis in Clinical Practice—Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Sixth Special Issue.
Merck Manual on Therapeutic Apheresis also lists Plasma Exchange as a first line therapy at http://bit.ly/1JcvSth
CBT (Cognitive behavioral therapy) and/or ERP (Exposure and Response Prevention) may be beneficial for a recovering PANDAS child. Medical interventions, such as antibiotics, IVIG, etc, are needed to lay a foundation for therapy to be introduced.
Some information on CBT as it pertains to PANDAS and PANS, can be found in Dr. Eric Storch’s presentation CBT for PANDAS and PANS(2012).
Steroids likely reduce the inflammation occurring in the child’s brain and have been shown to reduce severity of symptoms in patients with Sydenham’s chorea. Active infections need to be addressed with the use of steroids. Some children with PANDAS/PANS can experience a worsening of tics and/or aggression. It is important to discuss with your provider the pros and cons of using the steroid.
A tonsillectomy may be considered by an experienced ENT. Some research has shown marked improvement post tonsillectomy, including full cessation of symptoms in some patients. This has been noted in both a 2008 case study and 2015 JAMA Case Series. If a full remission of symptoms does not take place immediately following the procedure, it does not mean the surgery will not have any benefits. Removing the tonsils should lower the chances of a person contracting strep. A child may also see a more gradual improvement post-surgery.
A treatment course of antibiotics prior to surgery and antibiotics post-surgery can be beneficial. Requesting the tonsils be biopsied post-surgery may also show whether strep, staph, or other bacteria was hiding in the tonsillar crypts.
Some children are advised to take an Omega 3 supplement because of its known positive effect on brain function. It is also known to reduce inflammation and reduce hyperactivity and increase focus in children with ADHD.
Ibuprofen is classified as a NSAID (nonsteroidal anti-inflammatory drug). Some children have a temporary easing of symptoms with Ibuprofen.
If a child does not show any improvement with Ibuprofen, this does NOT dismiss a possible PANDAS or PANS diagnosis. Ibuprofen should only be given under the direction of a physician.