Jerod's First Steps
Jack Barghausen, C.P.
This report presents the story of an infant, Jerod M., who at the age of 11 months was given treatment for a sore throat by his family doctor. No improvement occurred in two days and a diagnosis of pneumonia was made. The child became progressively more lethargic and was taken to Kittanning Hospital and subsequently transferred to Children's Hospital of Pittsburgh with severe hyperglycemia and coma. Despite intensive hospital care the child came to bilateral below-knee amputations approximately four weeks after admission. The final diagnosis was given as:
- Juvenile diabetes mellitus
- Severe hyperglycemia without ketosis
- Bilateral below-knee amputations
Jerod's father was 30 years of age, his mother 27, and he had a brother five years old. All were in good health. The patient's birth, delivery, and neonatal life were normal. Immunizations included diphtheria-pertussis-tetanus (DPT) and poliomyelitis. He had had no illnesses in early infancy and in fact had been well until one week prior to admission to the hospital.
On admission to Kittanning Hospital the child's blood glucose was found to be 2400. Immediately, Normisol-M was given intravenously. He received 425 cc.'s of this solution within three hours and then was begun on Hart-mann's solution. Ten units of regular insulin were administered subcutane-ously and ten units of regular insulin were given intravenously. Within three hours blood sugar was down to 1800. The child was then transferred to Pittsburgh Children's Hospital.
On examination at Children's Hospital the patient appeared lethargic, with the eyes partially open. Spontaneous movements of all extremities were noted. Temperature was 37.2 deg. C, pulse was 150 and respirations 48 per min. Weight was 8.32 kgs.
The head circumference was 48 cms. and the anterior fontanelle was flat and sunken. The pupils were of equal size and reacted sluggishly with no focusing on objects. Fundi were benign.
The oral pharynx was slightly dry with some dark brown vomitus. Neck was supple without masses. The lungs revealed fine rales over the lower lung fields, anteriorly and laterally with deep inspiration. No rhonci were evident and there appeared to be good air exchange.
The heart had a regular rhythm with no rubs, murmurs, or gallops apparent. The abdomen was protuberant and soft, but tympanitic. The spleen was not palpable; the liver was 2 cm. and firm. Bowel sounds were hypoac-tive. Genitalia were normal.
Radial and dorsalis pedis pulses were weakly palpable. The nailbeds were slightly dusky. There was slightly decreased vascular filling of the nailbeds and this reaction became very poor after arrival. No clubbing or edema was evident. A cutdown for intravenous feeding was present in the left ankle. The skin on the palms and the soles was pale and pink. No petechiae were noted.
The patient was lethargic but vocalized occasionally. There were symmetrical DTR's, which were rated as 2+. Babinski plantar flexion reflexes were evident bilaterally. There was withdrawal to pain.
Laboratory Data and Blood Chemistry
Hemoglobin (HB) was 13.6, and Hematocrit (HCT) was 40. White blood cell count was 8100 with 45 polymorphonuclear leukocytes, 41 lymphocytes and 5 monocytes with 10 bands. Platelets were normal. Urinalysis revealed a pH of 5, and a trace of protein. Glucose was 4+, acid was negative, and acetone small. Occult blood was moderate. Microscopic examination showed the blood loaded with red blood cells, 4-5 WBC's per hpf., and no casts. Subsequent urinalysis showed high glucose and no acetone. On admission, the blood sugar was 850, osmolarity was 465, and a trace of acetone was evident in the blood. The pH was 7.20, PCO2 was 31.5, CO2 was 12.7, and stand, bicarb, was 13. Base X was - 15.
Sodium was 135, potassium was 3.1 and chloride was 125. Partial thromboplastin time (PTT) and prothrombin time were normal.
Cultures of the nose, throat, and blood were obtained. A spinal tap was considered at the time of admission but it was felt that the patient was too sick to be subjected to this procedure. The sodium reading of 135 was considered to be inconsistent with a chloride indication of 125. It was felt at the time that the sodium should probably have been in the 160 range. The base deficit was restored with sodium bicarbonate, with half the calculated need replaced over a 12-hour period. Potassium acetate was added to the intravenous fluids. Saline (.45 per cent) was also used. The administration of fluids was calculated on the basis of a 10-per-cent dehydration, but within four hours the amount was recalculated on the basis of a 5-per-cent dehydration. Antibiotics were not ordered.
Within 2 hours of his admission the patient was given insulin again. This time the dose was 10 units subcutaneously, and 10 units intravenously. Further insulin was given at 4-6 hour intervals. An electrocardiogram was taken and was normal. Disc margins were felt to be blurred and the patient was started on Decadron. It was not known whether the patient had superimposed septicemia.
Because of the rising PCO2 (56) it was elected to put the patient on the Emerson respirator, and transfer him to the Intensive Care Unit. Ten hours after admission the sodium reading was 156, potassium 9.6, and chloride 127. The pH was 7.19, PC02 was 64, C02 26.1, and Base X was -4. The glucose reading was 858, and urine output was poor. The administration of fluids and electrolytes was continually adjusted to the demands of the patient's blood status.
Surgical consultation was obtained after 12 hours of hospitalization. Both legs were cold and mottled with faint femoral pulses. No popliteal or dorsalis pedis pulses were evident. A decision was made to attempt a spinal block in order to open up arterial circulation in the lower limbs. However, no relief of the ischemia of the legs was obtained from the spinal block.
After 20 hours of hospitalization, bilateral arteriothrombectomies were done. Considerable clot was removed from both limbs, both proximally and distally. However, the small vessels were still greatly occluded with no runoff. Papaverine was injected in the operating room in an attempt to relax the arteries but without effect. After 40 hours of hospitalization, the blood sugar was still 802 mgs. per cent. The legs were beginning to show evidence of gangrene, and the patient was kept in the Intensive Care Unit and observed to determine the lower limit demarcation of the gangrene.
After 48 hours of hospitalization, the patient was still on the Emerson respirator, with a PCO2 that was being kept in the range of 20-26. At this time the blood glucose was 200, osmolarity was 298, acetone was 0, and calcium was 7.3. Electrolytes and blood gases were within normal limits. Sensor-ium activity showed no change.
On the fourth day of hospitalization, the patient was extubated with some difficulty. He was transferred back to the Metabolic Ward from the Intensive Care Unit. At this time the sensorium was still abnormal with some probable seizure activity and staring spells.
Below-Knee Amputations Performed
In the period following transfer from the Intensive Care Unit, the patient occasionally made attempts at vocalization. He was placed on phenobarbital at 5 mgs. per kg. per day. Nasogastric feedings were begun. Over the following month, the patient's sensorium improved gradually and very slowly returned to normal. He began eating, and his legs showed progressive demarcation, and at a lower level. On March 18, 1970, the patient came to bilateral amputations. The surgeons attempted to save as much tissue as possible, and were able to achieve below-knee amputations on both sides. The right stump was 3 1/4 in. and the left 1 1/4 in- (measured from the medial tibial plateau to the end of the tibia). The healed stumps are shown in Fig. 1 . The patient was immobilized in casts postoperatively.
On the first postoperative day the patient spiked fevers to 39.2 deg. C. On March 25, 1970, he was placed on Cloxacillin and Ampicillin because of the presence of pus in the legs. Cultures grew heavy hemolytic Staphylococcus coagulase positive, and heavy Klebes. The fever came down quickly and the patient remained afebrile after the antibiotics were stopped. His sensorium showed marked improvement and the amputated stumps healed well.
Ophthalmology consultation revealed that the discs were within normal limits. No hemorrhages or exudates were seen. The patient's diabetes came under good control with insulin NPH at 3 units plus 2 units of regular insulin, subcutaneously. Examination of the tissue of the amputated legs revealed dif-
fuse thrombi from the arteries. During a period of bilateral femoral thrombi, complete blood studies were carried out and revealed a relatively low fibrinogen level. Otherwise the blood appeared normal. During this period the child received several blood transfusions.
The pathogenesis of bilateral femoral thrombi was felt to be caused by the extreme hyperosmolarity of the blood.
Emotional factors played a major role in the care of this child and the Social Service Department and Psychiatric Clinic were intimately involved. The parents were initially quite depressed but rallied to face their problems courageously. On April 7, 1970, the casts were removed and Ace bandages were applied. The patient was referred to the Home for Crippled Children for rehabilitation, with an appropriate intellectual assessment to be done at a later time.
Towards the end of May 1970 the J.E. Hanger Co. of Pittsburgh was contacted for prosthetic evaluation and counsel regarding the type of prostheses that would best suit Jerod's early needs. It was particularly desired, of course, that with the prostheses the child should be able to ambulate at a level appropriate to this age.
Jerod's stumps had healed well. They were well padded with tissue and without bony prominences. There seemed to be good control about the knee joints although there was considerable doubt about mediolateral stability since the child had never walked independently before amputation. The decision was made to fit him with bilateral below-knee prostheses with side bars and corsets. In view of the patient's history it was decided that an end-bearing saddle or foam pad should be avoided until the stumps had become fully mature.
Studies of juvenile amputees have indicated that the very young child spends much of his time on his knees or in a crouched position. This activity pattern was a contributing factor in the selection of the thigh corset, side-bar approach, since this type of fitting allows more complete knee flexion.
SACH-type feet were selected since they would provide a smoother heel-to-toe roll-over. To afford maximum stability, a fairly hard heel wedge was used, thus compression of the heel wedge and heel strike was limited since the child's amputations were bilateral and he only weighed some 18 lbs. at the time of fitting. It was felt that single-axis feet would not benefit him in any way. Moreover, as a bonus, the SACH-type foot allows much easier access to the shin for lengthening of the prosthesis as this becomes necessary.
During the preprosthetic period, Jerod was given balance training in the Physical Therapy Department of the Children's Hospital (Fig. 2 ). Postoperative and preprosthetic counseling was also given the parents and at no time were any problems encountered with patient or parent cooperation or acceptance of the limbs. This is not a universal experience in dealing with juvenile amputees and their parents. Preparing them for casting and fitting visits and for acceptance of the prostheses are matters which are apparently too often overlooked in total counseling.
Casting was very carefully done in order to capture as much of the medial tibial flair as possible for weight-bearing. With the exception of the fibula head, the landmarks usually used in below-knee fittings were difficult to establish because of the fleshy stumps. Tracings of the limbs were made and stump and thigh circumferences were measured at one-inch intervals. The medial tibial plateaus were clearly marked in order to establish the prosthetic joint centers in relation to the anatomical knee centers. Medial tibial plateau to ischial tuberosities measurements were taken and the length of the lower limbs determined by reference to anthropometric charts.
The sockets were designed to obtain maximum weight-bearing on the medial tibial flairs. Posterior trim lines were held high in order to keep the short stumps from slipping out of the sockets at toe-off and during the initial swing-through period. Careful attention was paid to fitting as much of the distal stumps as possible to avoid creating any problems of edema.
Both sockets were set in about 2 deg. of medial tilt and about 3 deg. of flexion. It was felt that since this child had never walked, setting the feet slightly out of midline would increase his balance without creating any future gait problems.
The prostheses were delivered to the Physical Therapy Department at the Home for Crippled Children and the critical patient-parent-physical therapist relationship in gait training was begun. Donning and care of the prostheses were discussed together with the possible lacing and belt adjustments that might be necessary (Fig. 3 ).
Parallel-bar and single-bar training was rapidly accomplished by the patient (Fig. 4 ). Jerod was trained on an out-patient basis because of the close parent-child relationship. It was felt that the home atmosphere would be conducive to easier acceptance by both the child and his parents.
Future treatment plans for Jerod include:
- Intelligence testing.
- Provision of end contact in the prostheses with foam pads.
- Changing the prostheses to the bilateral patellar-tendon-bearing type possibly incorporating the supracondylar technique.
- Regularly scheduled follow-up visits to the Amputee Clinic at the Home for Crippled Children.
The rehabilitation of this child was a team venture involving the doctor, physical therapist, and prosthetist, plus a great deal of acceptance and cooperation on the part of the patient and his parents.
After approximately 80 days of ambulation the stumps have not shown any sign of tissue breakdown nor has there been any prosthetic rejection by the child or his family.
Despite his history of diabetes, the prosthetic treatment of this patient has not been significantly different than that of any other bilateral below-knee amputee. The case is noteworthy, however, in that the first independent steps Jerod took were with his prostheses and that at 18 months of age he is only a few months behind his normal growth level.