The Foundation for Children with Atypical HUS

Symptoms

courtesy of Bill Biermann

In most cases, Atypical HUS does not begin with a violent illness. Instead, the child starts off ill, fatigue, irritable, and lethargic to a point where hospitalization is needed. Supportive treatment is absolutely necessary in order to prevent further damage.
Typical HUS cases begin rather violently, with a severe bout of gastroenteritis that may be accompanied by bloody diarrhea.
Thereafter, symptoms of both typical and Atypical HUS may be similar. HUS causes clotting, and therefore results in vascular enlargement. Clotting tends to affect the kidneys, and may result in acute kidney failure, requiring dialysis or kidney tranplants.
A wide variety of symptoms can occur. Life threatening intestinal problems may occur. Neurological problems such as seizures, blindness and coma could develop. Profound intestinal or neurological diseases are indicative of a more severe HUS, and have poor prognosis. Nearly 5% of children will die during the acute phase of the disease as a result of renal failure or multiple organ complications. However, one the acute phase of the disease passes, most patients will recover eventually in full.
However, a small percentage of HUS patients are diagnosed with Atypical HUS. This group is especially proned to recurrences of the disease and are much more likely to develop chronic renal failure and other complications such as chronic high blood pressure. Atypical patients can have HUS episodes set off by routine colds and infections.

The initial symptoms may come gradually, and may differ from case to case. In most cases, Atypical HUS does not begin with a violent illness. Instead, the child starts off ill, fatigue, irritable, and lethargic often with fever. Usually, loss of appetite also occurs, Hospitalization usually occurs at this point.

Once hospitalized, the disease is usually identified through a routine blood exam. The blood exam will show:
1) Reduced platelet counts
2) Reduced Red Blood cell counts
3) Shattered red blood cells (this is called Shistocytes)

Children with Atypical HUS are much more likely to develop complications. These complications may be:
• Recurrance
• Kidney failure
• Seizures/Neurological problems
• No response to supportive treatments
• Return of HUS even after s kidney transplant occurs
• Severe Hypertension (high blood pressure)

To understand why these symptoms occur, we first must understand more about the disease.

To understand atypical HUS you first have to understand the role of blood and vascular system as well as the kidneys and how they work together.

  • The average adult has about five liters of blood, the average child less, living inside of their body, coursing through their vessels, delivering essential elements, and removing harmful wastes.
  • Blood is the fluid of life and growth, transporting oxygen from the lungs to body tissue and carbon dioxide from body tissue to the lungs, transporting nourishment from digestion and hormones from glands throughout the body. Blood is the fluid of health, transporting disease fighting substances to the tissue and waste to the kidneys.
  • Red blood cells and white blood cells are responsible for nourishing and cleansing the body. Since the cells are alive, they too need nourishment. Vitamins and minerals keep the blood healthy. The blood cells have a definite life cycle, just as all living organisms do.
  • Approximately 55 percent of blood is plasma, a straw-colored clear liquid. The liquid plasma carries the solid cells and the platelets which help blood clot. Without blood platelets, you would bleed to death.
  • The kidneys play an important roll in making sure we have enough red blood cells and in removing toxins, waste products of metabolism, from the body.
  • Factor H, is a protein found in the plasma which protects the endothelium tissue located in the lining of your blood vessels of the kidney. A factor H deficiency can resulting in damage to the endothelium. This triggers a cascade of events that result in platelets aggregating in the endothelium, clots which block arterioles and capillaries of the kidney. The platelets clump together (aggregate) and cause blood clotting and low platelet counts also called consumptive thrombocytopenia.

IN REGARD TO MEMBERSHIP REQUESTS

To ensure proper processing of your membership, please make sure to set your email filters to accept emails, from Linda@atypicalhus.org.

The Foundation for Children for Children with Atypical HUS is an all-volunteer organization. Please allow at least 72 hours for an email response confirming your membership request.  If you do not receive an email, please check your spam folder or email directly to Linda@atypicalhus.org

Membership is open to patients, family, friends, caregivers research and medical personnel.

WELCOME - JOIN US!

The Foundation for Children with Atypical HUS encourages patients and investigators to share information and explore options/resources as we work together to gain insight into this rare complement disorder. By increasing contact opportunities with researchers and medical personnel interested in helping the aHUS community, our stories foster a better understanding of atypical hemolytic uremic syndrome.

Sharing information, inspiration and support for one another, we seek to gather together people and knowledge as we strive to improve the lives of patients and families dealing with a diagnosis of aHUS.


NEW DIAGNOSIS OF aHUS?
Be proactive! Get the medical basics of aHUS, what lab values to monitor, and areas of concern...check out the "aHUS Bootcamp" and "About aHUS" tabs at the top of this page!
If your doctor has never treated a case of aHUS, please print out our 'Doc to Doc Registry' and ask him/her to contact a physician versed in the complexities of aHUS and new options for 2011 genetic testing and treatment.

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NOTE: Our 'Send a Message" function on each Member's page allows for private discussion of personal content. As with any social network, be cautious about giving personal contact information (home email info, phone number) until you have an established relationship with another person, organization, or associated website.

 

Did you know...

CFH (Serum Complement Factor H) is a regulatory protein. The secreted protein product of CFH consists of 20 repetitive units named "short consensus repeats" or SCRs (each approximately 60 amino acids). In patients with aHUS the last 5 "pearls" in the twenty pearl strand protein, SCR16 - SCR20, should bind to protect cells but do not- they are defective in one or more of the last 5 SCR locations. If they cannot bind or stick to the kidney to protect that tissue, the platelets clump into clots that affect the glomeruli of the kidney -potentially causing acute renal failure.
  
• • • • • • • • • • • •
  
It is estimated that there are about 300 cases of aHUS in the U.S., and it is most common with young children. The condition is life threatening and either can be chronic or can recur at intervals.
  
more factoids...

Help us fight the battle

Your tax-deductible purchase of one or more bracelets will directly fund research to help that aHUS families live with every day. Each bracelet has an appraised value of $825, but your purchase price is only $295.

  

  
Or you can donate a specific dollar amount-every dollar will help our research efforts of the Foundation for Children with atypical HUS at www.atypicalhus.org and www.atypicalHUS.net


Donations may be made via credit card or Paypal. If you prefer, checks made payable to The Foundation for Children with Atypical HUS may be mailed to:

Foundation for Children with Atypical HUS
C/O Deborah Lewis
PO Box 333
Barnhart, MO 63012


For pearl bracelet orders, please allow extra time for processing checks. Questions about aHUS donations? Please email Linda@atypicalhus.org


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