Jump to content

Menu

A long shot - anyone with experience with D+ HUS?


Cammie
 Share

Recommended Posts

{I accidently posted this in the General Education Board first!}

 

I have American expat friends here whose very young daughter has been hospitalized with D+ HUS (a syndrome most often associated with ecoli bacteria.)  She has acute renal failure.  This is such a rare thing to have that they are having trouble finding anyone to speak to who have been through this.  They could use some encouragement.

 

If you have been through this, please PM me or respond here.  We are really trying to figure out how this has worked out for real families (we have done ALL the research online but that doesn't provide the comfort actual personal experiences might.)

 

Thanks!

Link to comment
Share on other sites

I'm a medical professional, but had not heard of that and had to look it up. Sounds very serious. Are they comfortable with the where she is being treated? My only suggestion is to have her providers be in contact with a children's hospital or other experts on this condition to ensure they are on the right track with treatment. In my area, when something rare is encountered, they will contact the experts for advice, even if the experts are in other parts of the country. 

 

She will be in my prayers for healing.  :grouphug:

Link to comment
Share on other sites

I'm a medical professional, but had not heard of that and had to look it up. Sounds very serious. Are they comfortable with the where she is being treated? My only suggestion is to have her providers be in contact with a children's hospital or other experts on this condition to ensure they are on the right track with treatment. In my area, when something rare is encountered, they will contact the experts for advice, even if the experts are in other parts of the country. 

 

She will be in my prayers for healing.  :grouphug:

 

 I understand it is a rare complication of ecoli.  She is in the biggest and best hospital in the city.  It is a place that people come to from other parts of the world for medical procedures.  That being said, while the doctors are top notch they are having a hard time with the nursing staff.  She is in the icu in an isolation room.  I am not sure we have experts in HUS in India.  Would you have a way of finding out if there are experts in HUS in the US?  If you can point me towards a facility or doctor I will do my best from this end to get a connection made.

 

She did have a little urine output last night which was very good news.  From what we are hearing this is probably a weeks long battle.

 

Thank you for the prayers.

Link to comment
Share on other sites

 I understand it is a rare complication of ecoli.  She is in the biggest and best hospital in the city.  It is a place that people come to from other parts of the world for medical procedures.  That being said, while the doctors are top notch they are having a hard time with the nursing staff.  She is in the icu in an isolation room.  I am not sure we have experts in HUS in India.  Would you have a way of finding out if there are experts in HUS in the US?  If you can point me towards a facility or doctor I will do my best from this end to get a connection made.

 

She did have a little urine output last night which was very good news.  From what we are hearing this is probably a weeks long battle.

 

Thank you for the prayers.

 

PM'd you.  :001_smile:

Link to comment
Share on other sites

We will certainly add your friend’s daughter to our prayers.  As far as avenues for treatment or US HUS experts, most US trained pediatric nephrologists and pediatric ID doctors are quite competent with managing D+ HUS and I would imagine that would also be the case in India.  If your friend’s daughter is at a hospital without pediatric nephrologists or at a hospital that is not in a position to initiate acute dialysis then those would be reasonable reasons to request a transfer and those would be the resources that should be available at the receiving facility.  If she had D- HUS and was not at a hospital where plasmapharesis and or Eculizamab therapy were available those would also be reasons to request transfer.  I’m honestly not sure if Eculizamab is available in India but from what you are describing it would not be indicated for your friend’s daughter anyway.

I’m including some additional HUS information in case it is helpful to you, your friend, or someone else reading.  Please understand this [and really everything I share on this forum] is not intended as medical advice but rather background information facilitate further discussion with the physicians involved.  I’ll also offer the disclaimer that I am doing this off the top of my head and I am not a pediatric nephrologist but rather an EM Physician and a mom to a child with her own [non HUS related] renal issues.

HUS is Hemolytic Uremic Syndrome
The classic form manifests with  triad of:
1.)Hemolytic Anemia (the red blood cells are destroyed)
2.)Thrombocytopenia
3.)Acute Renal Failure/ Renal Insufficiency

Now we can we can break HUS down into groups and subgroups:
D+ is Diarrhea associated.  This is the more common grouping and associated with the O157 strain of E.coli.  Approximately 10-15% of afflicted patients will go on to develop HUS which is a toxin mediated syndrome.  The risk of HUS increases with the use of antibiotics and antimotility agents (ie. Lomotil etc) which seem to increase the Shiga toxin.

D- is not associated with diarrhea.  Strep pneumoniae associated cases of HUS are group in here but are really their own subgroup and because they are toxin mediated [with neuraminidase] their clinical course and prognosis is much more in line with D+ HUS than the rest of the D- HUS group.  It is now understood that many D- HUS cases are actually due to an abnormalities in certain components [Factor H, Factor I, Membrane Cofactor Protein, etc] of the complement pathway of the immune system.  These are genetically linked and there are more mutations that are currently being investigated and identified.  Non strep D- HUS usually has a more chronic and indolent course.  Generally the risk of renal failure is lower but the risk of relapse and recurrence is also generally higher.

Treatment for HUS is largely supportive:
-Judicious blood product [PRBCs, platelets] transfusion.  
-Maintenance of euvolemia [so initial volume resuscitation in D+ patients who are volume depleted but then careful attention to not volume overload the child]
-Renal replacement strategies [ie. hemodialysis, peritoneal dialysis] as needed.  Dialysis does not alter the disease process but can be used to manage the life threatening complications of renal failure.  Often D+ HUS patients who need dialysis recover, regain their renal function, and are able to come off dialysis.
-Management of hypertension
-Although antibiotics are generally avoided with D+ E.coli associated cases, antibiotics are favored with documented Strep pneumoniae cases.
-With D- disease plasmapharesis is beneficial.  The monoclonal antibody Eculizumab is also beneficial and has now been FDA approved.  Steroids may also be beneficial in certain immune conditions associated with HUS.  The details of the latter is beyond my scope as an EM physician.
-In patients who develop end stage renal disease, D+ HUS patients are good transplant candidates because their disease is unlikely to recur.  D- HUS patients are likely to have disease recurrence even after transplant and are poor candidates.

*Many HUS trials have taken place over the last few decades.  Sadly toxin binding therapies, antiplatelet agents, and antithrombotic agents have not been beneficial.*

 

Link to comment
Share on other sites

My nephew has Atypical HUS (not from e-coli, from something genetic possibly). He receives care at Children's Healthcare of Alanta. He takes some miracle drug that keeps in remission. He went through dialysis, and we thought he would need kidney and liver transplants at one time. The medicine is called Soliris. Good luck!

Link to comment
Share on other sites

My nephew has Atypical HUS (not from e-coli, from something genetic possibly). He receives care at Children's Healthcare of Alanta. He takes some miracle drug that keeps in remission. He went through dialysis, and we thought he would need kidney and liver transplants at one time. The medicine is called Soliris. Good luck!

 

Atypical HUS is the D- HUS mentioned above.  Most of these cases are explained by some breakdown or problem with part of the complement system of the immune system.  As you have mentioned there is a strong genetic component for these patients and some of that is still being worked out.  Soliris is the trade name for the monoclonal antibody Eculizumab. I'm glad it is working for your nephew.

Link to comment
Share on other sites

Thanks everyone for the info and the prayers.

 

Yes, she has "typical" D+ HUS.  She is in the best possible hospital here, good doctors.  Just, I think, very little experience with this particular syndrome. Also communication between doctor and patient-parents is very different here.  I think the family feels very in the dark not having access to doctors who have treated HUS many times or families that have been through this.

Link to comment
Share on other sites

Join the conversation

You can post now and register later. If you have an account, sign in now to post with your account.

Guest
Reply to this topic...

×   Pasted as rich text.   Paste as plain text instead

  Only 75 emoji are allowed.

×   Your link has been automatically embedded.   Display as a link instead

×   Your previous content has been restored.   Clear editor

×   You cannot paste images directly. Upload or insert images from URL.

 Share

×
×
  • Create New...